Neonatology - term newborn and introduction into the area of pathological newborn Ivan Peychl The speciality of neonatology Neonatology is the speciality oriented on the newborn care. During the last 20 – 30 years, it has become the independent subspeciality of pediatrics throughout the developed world. The main reason, why this subspeciality has been set aside, was especially in the on-going development of more and more complicated specific field of intensive care of the premature and pathological newborns. Nevertheless, neonatology as a whole includes the care of healthy, term newborns (approximatelly 95% of all born babies), as well as moderately premature babies or babies with moderate disturbances of postnatal adaptation (4%), and above mentioned intensive care of hardly premature babies or hardly diseased babies (0,5 – 1%). Of course, these groups differ much in theor demands for medical care after the delivery. Term newborn care consists of the observation of normal postnatal adaptation, professional treatment and some preventive measures, including support of breastfeeding, routine screening sampling and first vaccinations. Healthy newborn stays several days in the neonatal department of the maternity hospital, in the Czech Republic the stay usually takes about 4 – 5 days. On the other hand, extremely preterm babies can be immediatelly after the delivery in the critical status requiring extreme intensive care measures, which can last long days or weeks. Then, it can take several months until the baby achieves the degree of postnatal adaptation enabling his/her discharge home. On the base of this postnatal course diversity there was created in the Czech Republic (similarly as in some other countries) so called three-degree-system of the newborn care. Neonatology Ist degree departments provide care for healthy term newborns, IInd degree departments in addition to that care of newborns with moderate adaptation problems (e.g. babies born between 32nd to 37th week of gestation and/or babies requiring short-term ventilatory support), and, IIIrd degree departments also called perinatology centers, which provide, except for the care typical for lesser degrees, the care of newborns with all types of postnatal disturbances, including long-lasting intensive care of the most serious cases. There are presently (September 2000) 122 neonatal departments in the Czech Republic, 12 of which are perinatology centers. This is the three-degree system what makes neonatal care more effective from professional and financial point of view. The minority of more serious cases (about 1000 cases/per year presently) is treated predominantly in the IIIrd degree facilities. Concentration of these more or less rare cases in the centres make possible for doctors to gain higher erudition and better skills and for hospitals to use the expensive medical equipment more economically. Neonatology as a part of pediatrics influences its primary speciality: intensive care caused progressive fall of infant mortality and improved dramatically the prospects of premature babies for survival and good quality of life. On the other hand, the increased survival rates of prematures causes so far the increase of absolute number of children with long-term developmental defects. In addition to pediatrics, neonatology is in close interrelationship with the speciality obstetrics and gynecology. The care of premature and pathological newborns starts mostly before delivery and so close co-operation between obstetricians and pediatricians is an essential precondition for finding the optimal treatment, e.g. decision about maintenance or 1 terminating the pregnancy, about pharmacology treatment of pregnant women etc. Interconnection between obstetric and neonatology fields in care of pathological pegnancy cases results in calling this area perinatology or perinatology care. Relatively new is also the term fetal medicine. It refers to the narrow specific part of obstetric care aimed at direct treatment of the fetus in the mother´s womb (e.g., intraumbilical blood transfusions to anemic fetuses with severe Rh-isoimmunization). Basic terms and definitions Gestational age is the time elapsed from the conception. It represents the age of the fetus in weeks or, more exactly, in weeks + days (e.g. formula 28 + 5 means 28 completed weeks + 5 days of gestation). Normal pregnancy lasts on average 40 weeks, presumed term of the delivery is counted usually by the last menses period according to the following formula (TD … term of delivery, DM … date of the 1st day of the last menses): TD = DM – 3 months + 1 week. (For example: if the date of the start of the last menses is June 3rd, the presumed term of delivery is March 10th). Exact expression of the gestational age of the baby born just in the presumed term is 40 + 0. Relative term postconceptional (or postmenstrual) age is used in babies after delivery: this is the sum of the gestational age and the chronological postnatal age. (E.g., baby born at the age of 31 weeks has 4 weeks after delivery the postconceptional age 35 weeks). The delivery the expulsion of the fetus from the womb. Normally vital signs are present in the fetus after the delivery and the infant is what we call live- born baby. If there are no vital signs (heart beat, breathing, spontaneous movements) at birth, this is the case of fetal death. We distinguish between fetal death and abortion (miscarriage). The latter term refers to the expulsion of the fetus lacking vital signs before completion of 28 weeks of gestation or with the birthweight lower than 1000g. If the birthweight is lower than 500g, the expulsion of the fetus is called abortion even if the vital signs disappeare until 24 hours after delivery. These definitions of the delivery and abortion are formal and used for statistics needs in the Czech Republic. These definitions may vary a little in different countries and can change with the progress of the speciality. Developing capabilities of the medical care decrease the viability limits of the fetus, i.e. the lowest gestational age at the delivery in which there is the chance for the long-term survival of the newborn. Presently (2000), completed 23 gestational weeks are considered the viability limit. Earlier in the gestation born babies are virtually all born dead or dye soon after the delivery. Newborn is the baby síince the moment of the delivery until the finished 28 days after delivery. This neonatal period includes early (since the delivery until the end of the 7th day) and late neonatal period (since 8th until the completed 28th day). Newborn classification according to the gestational age at delivery: Term (mature) newborn is the baby born after the completion of 38 weeks and before completion of 42 weeks of gestation. The baby born earlier, i.e. before completion of 38 weeks of gestation, is preterm (premature) newborn, the baby born after the completion of 42 weeks is post-term newborn. In some countries the baby that completed 37 weeks of gestation is considered mature. 2 Newborn classification according to the birthweight: The group of newborn babies with birtweight lower than 2500g is called low-birth-weight babies (LBW). The birthweight lower than 1500g defines the group of very-low-birth-weight infants (VLBW) and babies with birthweight lower than 1000g are extremely-low-birthweight infants (ELBW). Newborn classification according to the relation of birthweight and gestational age at birth: Newborn, whose birthweight at birth is adequate to his/her gestational age, is called normotrophic newborn. If the birthweight is inappropriatelly low for the certain gestational week (g.w.), the newborn is hypotrofic, if the birthweight is inappropriatelly high, the newborn is hypertrofic. The birthweight of term normotrophic newborn is approximatelly in the range 2500g-4200g. To classify the newborn exactly in any gestational week the tables or centile graphs are used, that vary a little accordint to the sex of the newborn. See graph 1. Healthy, term newborn is a normotrophic term newborn with normal postnatal adaptation. Newborns mortality statistics Is one of the indicators of quality of the newborn care. Following terms are used: Neonatal mortality is the rate of newborns (infants younger than 28 days), that died, out of all live-born. Early neonatal mortality is the rate of newborns younger than 7 days, that died, out of all live-born. Late neonatal mortality, the rate of newborns, that died between the age of 8 – 28 days, out of all live-born. Fetal death rate, the rate of fetal death out of all deliveries. Perinatal mortality is the sum of fetal deaths and early neonatal mortality. Postneonatal mortality, the rate of infants that died after 28th day, but until the age of 1 year, out of all live-born. Infant mortality is designated as a rate of a infants that died between birth and 1 year of age, out of all live-born, and is the sum of the neonatal + postneonatal mortality. Sometimes the term „in-hospital“ mortality is used, which means the sum of the neonatal mortality and the rate of infants, that died after the 28th day of age, but earlier than discharged from the hospital, out of all live-born. The values are expressed usually either in percent or per mile. Instead of the term mortality sometimes the reciprocal value is used, called survival. For instance, mortality 30% corresponds to the survival 70%. Mortality in specific birthweight groups is called specific mortality. See some statistical values in the tables. Table 1: Low-birth-weight rate in the Czech Rep. in 1997 (rounded): birthweight rate out of all newborns (%) < 2500 g < 1500g < 1000 g 5,7 0,9 0,4 3 Table 2: Approximate values of infants mortality, neonatal mortality and specific neonatal mortality in certain birthweight groups in CR, 1997-98. % out of all live-born infant mortality 0,52 neonatal mortality 0,32 specific neonatal mortality in the group with 22,0 birthweight < 1500 g < 1000 g 36,0 < 750 g 58,0 Prenatal period from the pediatrician´s point of view Pediatric care starts in the moment of birth of the baby. Nevertheless, number of neonatal diseases and postnatal complications have its origin in the prenatal period, i.e. pregnancy. Proper obstetric follow-up is of primary importance. It includes clinical follow-up, follow-up of the fetal growth, measuring of the growth parametres by repeated ultrsound examination and ultrasound screening of malformations. The next part is genetic screening – the examination of biochemical markers and ultrasound. The monitoring of blood pressure during pregnancy is routine. The aim is to diagnose the hypertension of pregnancy and/or the late gestosis (preeklampsia) as soon as possible. This disease can cause a damage of the placenta, the early diagnosis can lead to the decision to terminate the pregnancy thus saving the fetus. Blood group and mother´s Rh is also examined in obstetricians office. Then, pregnancies jeopardized by immunologic colision (incompatibility) of blood groups are followed very properly. Especially if Rh-negative mother is pregnant with Rh-positive fetus, the fetus is endangered by so called Rh-isoimmunisation. Especially in the prenatal period it is important to diagnose early the acute hypoxia, mainly according to fetal bradycardia examined by auscultation or by cardiotocography (CTG) monitoring. In certain special cases, when genetic screening is positive, amniocentesis, chorion villi examination or chordocentesis (fetal blood sampling via transabdominal umbilical puncture) may be performed. When there is serious intrauterine growth retardation (IUGR) present, looking for its cause may be needed (small placenta, intrauterine infection etc.). Also umbilical blood flow monitoring by Doppler ultrasound examination may be necessary. Bad blood flow threatens the fetus and may be also the reason for pregnancy termination. Above mentioned and some other information about pregnancy circumstances are important for neonatologist´s decisions and comprises the main part of newborn´s medical history. See table 3. 4 Table 3: Some pregnancy disorders with impact on fetus preeclampsia diabetes mellitus hypertension other diseases of heart and circulation: right-left shunt, ischemia Rh-sensitisation epilepsia thyroid gland diseases – thyreotoxicosis, hypothyreosis autoimmune diseases TORCH infections (toxoplasmosis, rubela, cytomegalovirus, herpes ) sexually transmitted diseases (gonorrhea, syfilis, AIDS) trauma or surgery in pregnancy drug addiction autoimmune trombocytopenia myastenia gravis With regard to the above mentioned 3-degree system of newborn care it should be emphasised, that immediate availability of equipped intensive care unit after delivery is critically important for the prognosis of the ill newborn. If transfer from one to the other hospital is necessary, the outcome is significantly worse. Obviously, efforts are made to concentrate the care of high-risk pregnancies in perinatology centres, i.e. to hospitalize pregnant women in which signs potentially endangering the fetus were found (e.g., imminent premature labour, IUGR) preferentially in these centres before the delivery. With regard to the fetus we speak about the in utero transfer. Delivery The circumstances of the labour and delivery are important for the neonatal course. The most frequent is the spontaneous delivery in occipital position, breech delivery occurs in approximatelly 5%. This position is more often associated with complications and may be the reason for cesarean section. Cesarean section for differing reasons is performed in 5-10% of deliveries, in some centres with higher occurrence of high-risk pregnancies the frequency may be as high as 15-20%. The forceps (or vacuumextractor) delivery because of the not-proceeding labour is also associated with higher risk of birth trauma. The labour and the delivery of the newborn is the natural process, which most usually proceeds without problems even in the absence of medical care. Nevertheless, up to date obstetric care brings a lot of possibilities of monitoring the mother and the fetus in the antenatal period and during the labor and delivery and prompt treatment of complications. Owing to the obstetric care the occurence of pathological conditions in the developed countries decreased significantly and/or their consequences were alleviated: e.g., the perinatal maternal mortality decreased dramatically, the frequency of perinatal sepsis was decreased to the minimum, it is sometimes possible to save the fetus even in the case of acute intrauterine hypoxia, the outcome of premature babies has improved etc. Therefore, the delivery in the hospital under professional supervision has become standard in developed countries. On the other pole of the universally active strategy there is a philosophy of so called natural delivery emphasising uselessness of many of the obstetrician´s interventions, prefers to allow the labor and delivery theirs free course and leads sometimes to extreme strategies such as the delivery in the water basin or deliveries at home without professional assistence. 5 The reasonable balance of both attitudes is optimal from neonatologist´s standpoint. The delivery in the absence of the obstetrician exposes the baby (and the mother) to serious unnecessary risks. The presence of experienced obstetrician and midwife decreases the risks of the labour and delivery and may occassionally save mother´s and baby´s life. On the other hand, it is part of obstetrician´s responsibility to decide judiciously which interventions to use, and to be rigorous in using them only if indicated. Some too frequently used procedures and methods can be reduced this way (e.g., immobilization of the mother, enemas, early membranes dirruption, episiotomy or external pressure on the uterus – which should be definitely avoided). Unwarranted use of some of these procedures may cause inconvenience to the mother, who may experience the postnatal period less comfortably, and/or cause some of complications of postnatal course. Problems of perinatal analgesia are specific. In accordance with up to date knowledge, the intensity of experienced pain of labor can be significantly decreased by the use of noninvasive methods like allowing free motion (walking) before the delivery, looking for the appropriate position for relief (with the aid of apecial tools – soft balls, shapeable bags) or administering warm (warm shower or bath before the delivery). Only if the pain is too intensive and the mother wishes more potent analgesia, the use of pharmacological analgesia is appropriate. General analgesia (parenteral administering of opioids or analgesic mixtures) should be avoided, because it may worsen mother´s coopoeration during labor and/or cause respiratory depression in the newborn. Hence, epidural analgesia should be preferred. Even the epidural analgesia should be used only if strictly indicated, because it´s use may lead to head- or backaches in mother after delivery, thus worsening the cooperation of the mother after delivery and interfering with the good start of lactation. In the immediate postnatal period, the philosophy of non-separation of the mother and the baby is critically important for the lactation commencement. The practice of separated health-care for both mother and child used to be a big disadvantage of in-hospital deliveries during past decades. Later, it was found that such practice causes significantly worse and delayed start od lactation and less successful breastfeeding in the population as a whole. Therefore, in up to date hospitals the health-care professionals aim to enable the close mother and child´s „skin-to-skin“ contact and nursing from the very first minutes after delivery and afterwards frequent breastfeeding and common care for both in one room (rooming-in system) throughout their stay in the maternity hospital. Immediate postnatal adaptation of newborn In the fetal period lungs are collapsed and oxygen is delivered in the body by the placental circulation. It comes via the umbilical vein into the inferior vena cava (either directly or through the venous duct and portal vein). Only smaller part of blood from the right heart (less than 10% of the whole blood flow) go through the lungs, although the majority aims directly into the systemic circulation through the shunts (foramen ovale and ductus arteriosus). Placental oxygen suply is less effective than postnatal lungs ventilation. That is why hematocrit of the fetus is higher (45 – 65% before delivery) and hemoglobin saturation is lower (about 45%). The delivery changes the conditions in the circulation. The placental oxygen supply is interrupted. The newborn responds by the start of regular spontaneous breathing. It is started with the first inhalation. The latter is more vigorous than later breaths, with lower negative pressure in the pectoral cavity, because this first breath „opens„ the lungs. The air is inhaled into the lungs and the transport of oxygen into the pulmonal capilaries starts. The fetal type of circulation is becoming disadvantageous and necessary redirections occur: umbilical 6 vessels stop working, right-to-left shunts are closed. The close of foramen ovale and ductus arteriosus is functional at first but later (after days to weeks) becomes anatomic. Owing to the above mentioned changes the pulmonal and systemic circulations are connected after one another, 100% of blood go through the lungs and effective oxygenation of blood and fast delivery of oxygen into the organs is made. The hemoglobin saturation increases to normal postnatal values above 95%. These patophysiologic changes are associated by characteristic clinical manifestations. Usually, the newborn is born with peripheral cyanosis or mild body cyanosis, which disappears quickly after breathing was started. If the peripheral vessels are filled well, the skin colour changes to pink (or even red, if the hematocrit is higher). The first inspiration is usually, but not always, associated with crying. Baby, that does not cry, can also breath regularly and adapt normally. The remnants of amnial liquor in the lungs, that stay in the lungs and airways after lungs´ expansion, cause „wet lung auscultation“ – rales, but they are absorbed during first minutes after delivery. In the period of the functional close of shunts, small parts of blood flow can be directed through these shunts. This flow i hemodynamically insignificant, but can lead to changeable heart murmurs. So, the skin of the normally adapting newborn becomes pink during the first minutes after delivery, the baby is breathing regularly, has good muscle tone, responses to painful stimuli and is able to suck the breast. Simultaneously, changes in the gastrointestinal and urinary tracts, termoregulation and metabolism are under way during the first hours after delivery. These changes are parts of complex baby´s adaptation to extrauterine life. Care of the healthy term newborn After the delivery, the umbilicus is interrupted (ligated and cut) and the newborn is usually taken to the newborn-care room, where the process of adaptation is quickly evaluated and the first necessary treatment is performed by midwife, pediatric nurse or pediatrician. One of these responsible persons assess, whether the adaptation is normally progressing, with use of clinical experience as well as objective clinical signs: for each newborn, so called Apgar score is counted and documented (see later in the chapter about asphyxia). The care of the normally adapting healthy newborn consists of: 1. providing thermal comfort (among others, the temperature of the environment 20 - 22°C) 2. drying the skin from the amnial liquor (gentle touching of the skin with clean warm towel, not rubbing) 3. proper check of the umbilicus ligation, to ascertain umbilicus cannot bleed 4. treatment of the umbilicus tip with aseptic solution and it´s wrapping with sterile soft cloth to avoid the injury of the stem 5. weighing and measuring the length of the baby, measuring head and chest circumference 6. treating eyes with aseptic or antibiotic eye drops – so called „credeisation“ 7. administering vitamin K 8. labeling the baby such securing unambiguous later identification To provide thermal comfort to the healthy term baby, it is enough if there is appropriate temperature of the environment (delivery room), the skin is gently dried and the baby is put on the abdomen of the mother, whose body affects as a warm source in that case. The back of the baby is covered with warm towel. If abnormal adaptation is suspected and there is need of close observation of the baby, or there is even the apparent need of resuscitation, the baby 7 has to be transfered to the resuscitation room, where machines serve as a source of the warm (electric heater or incubator). Mentioned above, skin drying diminishes temperature loss. It is good not to rub the skin and not to clean vernix from the skin surface, because the newborn´s skin is very sensitive during the first days after delivery, there in no resistant superficial layer and vernix constitutes it´s natural protection. Too vigorous rubbing irritates the skin and contributes to the development of the neonatal rash called „erythema toxicum“ on next days – benign maculous exantema, which is itching and provokes scratching and sometimes secondary infection of the skin. Measuring of the length of the body should be performed in the special measurign device, where the baby is laying on his/her back and touches the margin of the device with his/her head. The attempts to measure the length on the table just with tape measure are usually very inaccurate. The length measuring can be postponed in those babies, whose hip joints are not movable enough and limbs extension could be painful for them – especially after deliveries in breech position. then, the lenth is measured on the day 2 or 3. Normal length of the healthy term newborn ranges between 47 – 55 cm. Range of normal birthweight of healthy term newborn is 2500g – 4200g. Head circumference measuring is performed with tape measure in the longest occipitofrontal circumference – over the forehead just above eyes and over the most prominent part of occiput. Normal head circumference ranges between 33 – 37cm. If there is apparent subcutaneous swelling (caput succedaneum), the measuring could be also inaccurate and it is necessary to repeat it after several days, when the swelling disappeares. Normal chest circumference in newborn should be between 30 – 37cm and it is shorter than head circumference in the first half-year of life. It is measured in lying baby at the level of breast nipples. The aim of credeisation is the prevention of gonoccocal and/or other bacterial coniunctivitis. Eyes can be infected while going through the birth canal. In the Czech Republic, aseptic drops (Opthalmo-septonex) are presently usually used. In some countries drops with erythromycin are preferred. Preventive administering of vitamin K decreases the risk of so called haemorrhagic disease of the newborn. The dose 1 mg i.m. or 1 – 2 mg. p.o. is used. Preferrable way to label the baby is a plastic stripe clasp on which the name is written and it is buckled around the wrist or ankle. Instead, sometimes small circlets with the number of delivery are used, both for the mother and the baby. Frequently too different ways of labelling are used. In some countries, as definite way for later unequivocal identification, baby´s heelfootprint in the chart is used. The appropriate moment to interrupt umbilical stem is a little controversial. The cut immediately after the delivery is usual. It allows the prompt transfer of the baby to the resuscitation room. Nevertheless, when there is optimal technical and personal equipment, it is mor appropriate to put the baby on mother´s abdomen and allow the umbilicus to work one or two minutes more. This way, slowlier and more continual transition from prenatal to postnatal type of oxygenation is allowed and early skin-to-skin contact of the mother and the baby is ascertained, usually with the first breastfeeding. The routine first treatment is several minutes delayed then. The obvious precondition of this approach is reliable assessment of normal course of adaptation and no need of resuscitation, and optimal environment of the delivery room, especially appropriate temperature. 8 Perinatal asphyxia About 90% of newborns undergo normal postnatal adaptation and are cared for routinely as described above. In smaller part (5-10%) the adaptation in the first moments after delivery is disturbed, hypoxia occurs (perinatal asphyxia) and immediate resuscitation is necessary instead of routine care. Asphyxia can be caused by prenatal (placental abruption, cord compression and others) or postnatal (immature lungs, meconium aspiration and others) reasons. Apgar score is used to assess the progress of postnatal adaptation: Table 4: Apgar score heart rate breathing 0 asystole no muscle tone very hypotonic, limbs extension skin colour general cyanosis or pale no response to irritation 1 < 100/min slow, irregular, or gasping hypotonic, but certain limbs flexion present peripheral cyanosis weak, grimace 2 > 100/min regular, or crying normal tone, limbs flexion, active movement pink defensive movement, or cry The score assigns 0 to 2 points to 5 different parameters. The resulting score is the sum, so the result can have values 0 to 10. Routinely, Apgar score is assessed 1, 5 and 10 minutes after delivery. The score 8 or more is considered normal. Typical Apgar score of healthy newborn is 9-10-10 (there is usually distinct peripheral cyanosis present one minute after the delivery). The score below 8 reflects usually perinatal asphyxia. The lower the score, the more serious is the adaptation disturbance. 3 degrees of perinatal asphyxia are usually described: 1st degree asphyxia (Apgar score 5-7), 2nd degree asphyxia (score 3-4) or 3rd degree asphyxia (score 0-2). This is a traditional concept of perinatal asphyxia. However, it must be considered that low Apgar score does not equal the asphyxia. Sometimes the low score can have different reasons than perinatal hypoxia or stress of the labour. For example, the newborn with congenital disorder of neuromuscular transmission will suffer hypotonia and/or respiratory distress and will have low Apgar score, reason of which is not asphyxia. Similarly, premature newborn has usually weaker muscle tone than term baby and lower Apgar score: again, hypotonia is not caused by asphyxia, but rather by general immaturity of the baby. (On the other hand, obviously asphyxia may also influence Apgar score of the premature newborn). The evaluation with the use of Apgar score helpa us to document the progress of immediate postnatal adaptation and has also prognostic significance: it was found that even very low score values in the 1st minute are not associated with developmental disturbances (i.e., long-term sequelae and handicaps) if the baby is resuscitated successfully. On the contrary, still low Apgar scores in 5th or 10th minute after the delivery are signs of increased risk of irreversible brain tissue damage. Apgar score is a tool for clinical evaluation of asphyxia. It is contributing to perform also laboratory examination – umbilical blood pH. Low umbilical pH is also evidence of asphyxia. 9 pH 7,25 and more can be considered normal in newborn, pH 7,15 – 7,25 shows 1st degree asphyxia, pH 7,05 – 7,15 means 2nd degree asphyxia and pH under 7,05 is sign of serious 3rd degree asphyxia. Very low pH value (less than 7,0) is sign of poor long-term neurologic outcome, especially if associated with low BE, meaning that acidosis has it´s significant metabolic component, i.e. acidosis is probably already long-lasting. Using both approaches to evaluate asphyxia, Apgar score as well as umbilical blod pH, can contribute to finding etiology of asphyxia: if Apgar score and umbilical pH are decreased simultaneously, the cause of asphyxia is probably prenatal (ubmilical cord complication, placental abruption, intrauterine infection). If pH is normal, while Apgar score is low, the cause is more likely postnatal (for example, pharmacological depression with opioids administered to mother during the delivery, meconium aspiration or RDS). So, assignment to those 3 degrees of asphyxia is not necessarily corresponding with both methods. If the baby suffering asphyxia is not promptly efficiently resuscitated, hypoxic damage of organs occurs. The most significant consequence in surviving children is a brain damage, (hypoxic-ischemic encephalopathy, HIE). Morphological corelate of the latter may be cortical atrophy or brain cysts, whereas functional corelate is a disturbance of a motor development (cerebral palsy), sensoric malfunction (vision or hearing deficits) and/or cognitive deficit. Resuscitation of the newborn Data shows, that each year approximatelly 5 million of newborns die worldwide. In about 19% of them, i.e. about 1 million, the reason of death is perinatal asphyxia. These numbers underline the importance of resuscitation techniques knowledge in all health-care givers involved in newborn care. The primary precondition for the successfull resuscitation is optimal thermal comfort. Hypothermia increases tissue oxygen consumption, such decreasing the chance for succes of the resuscitation. On the other hand, it is also imperative to avoid hyperthermia, which may cause the respiratory depression. Ideally, newborn should be cared for in the electric heater with the opportunity of the fine heating intensity adjustment. (It is possible, that in the future the selective hypothermia of the brain will be used as a part of treatment protocol for perinatal asphyxia. Some data shows potential of this treatment for improving outcome. Nevertheless, the method is in the early stage of clinical experiments). The first step of the resuscitation is to secure the patency of the airways. This is provided by positioning and suctioning. The adequate position of the baby is on his/her back, head aiming to the resuscitating person. The head must not be hyperextended (difference from older children) nor in anteflexion, nor bowed to side. The right position is neutral or slightly extended („sniffing position“). Suctioning is important particularly if there is content in the mouth which obturates airways and hampers breathing (bigger amount of amnial liquor) or may cause aspiration (liquor with solid meconium). It is necessary to suction quickly and as gently as possible, from the mouth, upper airways and nose. If there is meconium in the liquor, it is necessary to suction repeatedly, including stomach. One has to avoid too aggressive suctioning: rough irritation of the palate can lead to vagal reflex with bradycardia and laryngospasm thus causing more harm than benefit. Next steps depend on the newborn´s status. If the asphyxia is mild, circulation works (heart rate more than 100/min) and at least irregular spontaneous breathing is present, we can start resuscitation with tactile stimulation. It means rubbing of the skin in lumbar area or on the plants. This technique may contribute to physiologic reflexes triggering normal adaptation and regular breathing. Next step may be oxygen inhalation via mask: mask is put on the 10 mouth and nose of the baby, which then inhales 100% oxygen and oxygenation of his/her body is made easier. If the status of the baby improves quickly, the cyanosis is disappearing and regular breathing begins, next resuscitation may be unnecessary. If the asphyxia is more serious, the baby struggles for air (gasping) or does not breath at all, the intermittent positive pressure ventilation with bag and mask is necessary. The mask is put over the mouth and nose again, but his time the mask has to adhere tightly to the skin of the face, encircled with fingers of one hand (the left one in right-handed persons). The gas mixture cannot escape then. Thus the establishment of positive pressure is enabled. The other hand compresses the bag rythmically with the rate about 40-60/min. The most important while resuscitating this way is to maintain the right position of the body and head and the patency of airways, and the same time to create sufficient pressure to ventilate the lungs. Nevertheless, the pressure must not also be too high, because this could cause lung injury including pneumothorax. For the first inspirations higher pressures are usually necessary. Later, when the alveoli are already filled with air (oxygen), lower pressures may be enough. The efficacy of the positive pressure ventilation is confirmed by rythmical movements of the chest and/or lungs auscultation. (The adequate strength of bag compressions can be practiced with the bag with affixed manometer. The right pressure is about 25 – 30cmH2O, for the first inspirations 30 – 40 cmH2O. If there are no heart sounds at all or serious bradycardia is present (less than 60/min), we perform chest compressions together with positive pressure ventilation. The chest is compressed rythmically in the area of lower third of sternum, approximatelly to one third of the distance sternum-backbone. 2 techniques may be used. a) hands are put under the body of the baby and thumbs are crossed on sternum (picture 1), or b) we cross second and third finger of both hands on sternum. Recent data seem to show that the first described technique is more efficient and should be preffered. Also while compressing sternum the sufficient but not too big strength is important. The right compressions rate is 120/min. Because chest compressions and positive pressure inspirations are performing simultaneously, the rythm is importnat. The right rate compressions : inspirations rate is 3:1, usually 120 compressions and 40 inspirations per minute. The optimal rythm looks like this: inspiration – 1st compression – exhalation – 2nd compression – 3rd compression. If the baby´s status is so serious that bag and mask ventilation does not seem to be efficient enough, we perform intubation. We have to prepare properly for intubation. Again, the above described position of the baby is important. The intubating person needs assistant (nurse or colleague) which holds the appropriate, straight position even when the intubating doctor concentrates on the view of larynx. Further, the appropriate laryngoscope has to be prepared (Miller – with straight blade, number 0) with good batteries, adequate endotracheal tubes (see table 5) and stripes of wellsticking adhesive plaster for tube fixation. The procedure itself starts with introducing the tip of the blade of the laryngoscope into the right mouth corner and push the blade right to the vallecula glossoepiglottica. The tip of the blade is pressed gently against the bottom of the vallecula. Thus, the epiglottis is pushed away and the view into the larynx is uncovered: white vocal cords aiming down and diverging obliquely and the laryngeal entrance between them. Then, the larynx is positioned as to be in the center of the view, with slight laryngoscope movements and/or with the slight external pressure on the cricoidal area (with the middle finger of the other hand). After that, the tip of the endotracheal tube is inserted into the trachea (enough, but not too deep, see table 5). 11 Table 5: Recommended diameter and deepness of introduction of endotracheal tube (ETC) according to the weight and gestational age of the newborn baby. Valid for orotracheal intubation. Weight (g) < 1000 1000 - 2000 2000 - 3000 > 3000 gestational age (week) < 28 28-34 34 - 38 > 38 ETC diameter (mm) 2,5 3,0 3,5 3,5 – 4,0 insertion deepness (cm from upper lip) 6,5 - 7 7-8 8–9 >9 Usually, the right-handed person uses his/her right hand to intubate. After finding the good larynx view the laryngoscope is passed to the left hand and the right hand inserts the tube. Some right-handed people use the left hand to hold the laryngoscope from the beginning of the procedure. Soft endotracheal tube may twist such making the insertion just between the vocal cords more difficult. Sometimes it helps if the tubes are stored in the environment with low temperature, where the material becomes tougher. Or, the introducer can be used – sterile aid passed through the endotracheal tube such making it stiffer. After the successful insertion teh introducer is pulled back. Adequate deepness of the insertion is of critical importance. Insufficient deepness can lead to undesirable spontaneous extubation. On the other hand, if the tip of the tube is too deep, it aims already into the right main bronchus and the baby is endangered with collaps of the left lung and emphysema of the right lung. After insertion of the tube it´s adaptor is connected with the adaptor of the ambu-bag. The appropriate deepness of the insertion is confirmed according to the centimeter scale marked on the tube and checked with the lung auscultation: the mechanical inspirations should be heard symetrically on both sides. If the breathing sounds are very quiet above both lungs and, conversely, the loudest above the stomach, it is likely that the tube has been inserted incorrectedly into the oesophagus. Then, the breathing movements of the chest are lacking. If the breathing sounds are heard well on the right side but clearly weakened on the left side, the insertion is too deep and the tip of the tube aims into the right main bronchus. After the right position of the tube is confirmed, the tube is fixed to the face with two plaster stripes. After the fixation another check of the position is needed, because sometimes one can shift the tubeto the incorrect position while fixing it. If we are in doubts about the right position of the tube, it can be confirmed also by x-ray. The tip of the tube should be under the level of the vocal cords but above the level of carina. In the x-ray picture it means the position between the medial ends of clavicles or slightly under this level. Recently developed aid contributing to confirmation of the correct position of the tube is the device measuring CO2 in the exspired air. The intubation has it alternative in introducing of the laryngeal mask. However, this technique is not equivalent of the intubation comes into consideration only if the equipment for intubation is not available or the attempts to intubate were unsuccessful. The combination of intermittent positive ventilation (with bag or via endotracheal tube) and chest compressions, i.e. complete cardiopulmonal resuscitation, is a technique enabling successful resuscitation of absolute majority of asphyctic newborns. summary of the procedure see in the picture 12 Schema 1: Steps in resuscitation of the newborn approximate time Birth Clear of meconium? Breathing or crying? Good muscle tone? Color pink? Term gestation? YES ----------→ ROUTINE CARE - provide warmth - clear airway - dry ↓ 30s ↓ NO ↓ Provide warmth Position, clear airway (as necessary) Dry, stimulate, reposition Give O2 (as necessary ) 30s 30s ↓ ↓ Breathing Evaluate respirations, ----------------------→ SUPPORTIVE CARE heart rate and color HR > 100 and pink ↓ ↓ Apnea ↓ or HR < 100 ventilating Provide positive pressure ventilation * ---------------------→ ONGOING CARE ↓↑ HR > 100 and pink ↓↑ HR < 60 ↓ ↑ HR > 60 Provide positive pressure ventilation* Administer chest compressions HR < 60 Administer epinephrine* * … ENDOTRACHEAL INTUBATION MAY BE CONSIDERED AT SEVERAL STEPS 13 Pharmacological resuscitation is of secondary importance. It is beginned, if cardiopulmonal resuscitation is unsuccessful for longer period of time (1-3 minutes). See the table 6 for reccomended drugs, doses and ways of administration. Table 6: Drugs used for resuscitation: drug epinephrin volumexpander – saline, Ringer-lactate – 0 negat.ery koncentrate naloxon bicarbonate indication dose asystole,bradycardia< 60/min 0,1–0,3ml/kg sol. 1:10 000 hypovolemia 10 ml/kg in 5 – 10 minutes depression with opioids 0,1mg/kg administered to the mother during last 4 hours before delivery prolongated resuscitation 1 – 2 mmol/kg Some aspects of pharmacological resuscitation are controversial. It is considered appropriate to administer epinephrin in asystolic or seriously bradycardic baby and to administer naloxon if the depression with opioids is suspected. If hypovolemia is likely, volumexpansion is indicated. Bicarbonate does not belong to primary drugs used in resuscitation of the newborn and should be considered only if the resuscitation is not successful for extended period of time or if metabolic acidosis is confirmed by laboratory evaluation. Tthe use of calcium and magnesium preparations is controversial. The fastest way to administer the drug is the intratracheal one. It is possible in epinephrin and naloxon. Naloxon can be administered also subcutaneously or intramuscularly, if the peripheral perfusion is still satisfactory. To secure the vein we perform the umbilical vein cannulation. Also possible, but not frequently used is intraosseal way. It is good to practice the technique of resuscitation on the mannequin. Newborn examination Early after the delivery the baby is usually examined by pediatrician, either in the delivery room or during the first hours after the delivery in the neonatal department. The examination is preceded by taking the detailed history of the family, pregnancy and delivery. Detailed physical examination serves to the assessment of the general status of the baby and exclusion of congenital malformations. The newborn should be examined when naked, in warm environment on well enlightened place, preferably in parent´s presence, enabling to ask for peculiarities of the history. It is also advantageous to allow to the parents to také part in the examination, for example by undressing the baby, holding the baby on their knees during certain parts of the examination etc. The examination is easier if the baby is quiet and happy. It may be contributing to allow the baby to hold the parent´s or doctor´s hand (finger). The firm surface of the table and the feeling of stable position are also important for the baby. It is necessary to remember, that the muscles of the neck of the baby are not able to support the head. Therefore, if we pick up the baby, we hold him/her with one hand under the back and with the other one support the head, holding it along the body axis. See picture 2. 14 The correct evaluation of all findings in newborn examination is a matter of clinical skills and experience and cannot be fully explained by words. It is true for instance for assessment of the maturity of the baby, normal findings in lungs auscultation, assessment of the muscle tone etc. From this pint of view, the following text is the instruction which can lead to the knowledge of newborn examination only together with clinical practice. The examination begins with observation of the baby. We observe the behaviour – does the baby sleep or is alert, is he/she crying, is the anomalous irritability present or, conversely, is the baby lethargic and generally hypotonic? We evaluate, how regular the breathing is and exclude rspiratory distress – jugulum, intercostal spaces and/or hypgastrium retractions, grunting. We observe spontaneous movements of extremities and head. The intensity and the tone of cry should be noticed – healthy newborn has a strong and clear voice, congenital anomaly of larynx may cause stridor, CNS damage may lead to high-pitched voice. We evaluate the proportions of body parts, face features, presence of apparent congenital malformations, deformations or deformits. If we suspect anomalous facies, firstly we compare the face features of the baby with features of the parents. Seemingly unusual features of the face could be rather familial variation than pathological findings. However, sometimes real signs of hereditary syndrome can be found, e.g. anomalous size or shape of the head, low position of external ears, eyes anomalies, „Gothic“ palate etc. Also babies with congenital palsy of n. facialis have characteristic facies, which is most apparent in crying baby: on the side of palsy the mouth corner is not raising and the nasolabial crease is wiped out. See the most frequent visible syndromes and malformations in the tables 7 and 8. Table 7: The most frequent hereditary syndromes which can be diagnosed by the first newborn examination with their signs. Syndrome M. Down, 21 trisomy most usual phenotypical signs in newborn mongoloid shape of eyes, epicanthal folds, flat occiput, transversal palmar creases, bigger distance between the 1st and 2nd toes, sometimes heart murmur M. Edwards, 18 trisomy hypotrophy, crossed 2nd and 3rd fingers and 5th and 4th toes, microcephaly, micrognathia cleft of the palate, low position of external ears, microphtalmia M. Patau, 13 trisomy Turner syndrome, XO hypotrophy, shorter length, lymphedema of the backs of hands and feet insteps Table 8: Some more frequent isolated malformations which can be diagnosed in the first newborn´s examination cleft of the lip and palate polydactylia, syndactylia reduction deformities of extremities microcephaly, macrocephaly, hydrocephalus choanal atresia 15 omphalocele, gastroschisis, urinary bladder exstrophy spina bifida, meningomyelocele, encephalomyelocele hypospadia, epispadia pes equinovarus, pes calcaneovalgus Assessment of the maturity (gestational age in delivery) is a part of newborn´s examination. This is a clinical estimate which can be used as a correction of inaccurate prenatal evaluation according to the last menstrual period of the mother. Several body signs are used for approximate estimate. The external ear of the mature newborn is fully shaped and has a stiff and elastic cartilage. The cartilage of premature baby is soft and lacks flexibility. Mature infant has fully developed breast nipple prominating over the level of the skin and palpable gland with diameter about 5 mm under the areola. The more premature the delivery, the less developed the nipple and the gland are and so in very premature babies born before the 28th week the nipple is flat and the gland not palpable. The skin of the mature newborn on plants is richly creased. Premature babies have shallow creases, in very premature ones the plant is flat and free of drawing. Genitalia of the mature boy is developed to the stage of fully descnded testicles, the scrotum is pendulating and the skin is creased. In premature boys the incomplete descensus of testicles is more usual, scrotum is smaller with flat skin. Labia majora of mature girls are clearly bigger than labia minora and cover the latter, while in premature girls the difference is not so clear. Except of these basal signs there are complex scoring systems (Dubowitz, Brazelton) based on examination of many physical and neurological signs of maturity. These systém are time-consuming and not widely used. The nutritional status is evaluated according to the layer of the subcutaneous fat (thinner in hypotrophic babies, where the skin is folded on thighs, face is of „old man“ appearance) and classification according to exact growth parameters in centile graphs. The most informative is the relation of birthweight and gestational age. We observe the skin colour and peripheral perfusion quality. Newborn´s epidermis is covered with slimy and whitish layer of vernix caseosa. Vernix protects the skin of the fetus in the intrauterine life where the skin is exposed to the action of amnial liquor, and goes on to protect the skin during the first hours after the delivery, when the skin of the newborn is very sensitive. The skin of the healthy newborn is pink and without peripheral cyanosis severeal tens of minutes after the delivery. In some babies the skin is rather red than pink, because the hematocrit is high. (Due to the increased hemolysis during the first days of life hematocrit decreases quickly so that after 7 – 10 days the skin is much paler.) Persistent significatn peripheral cyanosis and especially central cyanosis is anomalous. The term central cyanosis refers to the blue colour of circumoral skin, body and mucous membranes of the mouth. This is the evidence of circulatory maladaptation (e.g. as a consequence of congenital heart defect or pneumopathy). Erythema or angiomatosis on the skin of the forehead, face or the eyelids are very frequen and usually disappears during the first days. Even more frequent are so called milia, whitish or yellowish dots visible usually on the nose or cheeks. They are also benign. „Harlequin´s skin“ is the skin divided sharply in 2 halves with different colours: one half is white and the other one is red. It is a striking transient sign with no specific underlying pathology and probably no prognostic value. There is no jaundice on the skin of healthy newborn during the first 24 hours. Normally, the jaundice appears not earlier than on the day 2. If it appears sooner or even is present at delivery, it is early jaundice caused most usually by Rh or ABO isoimmunization. Peripheral perfusion can be basically assessed by performign short pressure with a finger on the small skin area: after relieving the skin we observe the speed of blood refill, which should 16 last not more than 2 seconds. If the peripheral perfusion is worsened, the peripheral parts of extremities use to be colde, too. Sometimes the hemangioma of the skin can be found, usually planar, and most frequently benign, sometimes with tendency to involute spontaneously. However, bigger hemangiomas neccessitate proper follow-up in next months and prospective dermatologic treatment. Exceptional is Sturge-Weber disease with a large planar hemangioma of one half of the face and head: this can be associated with intracranial and intraocular hemangiomas, developmental disorder, seizures and vision disturbance. Brown nevi can be also found on the skin, usually benign. It is good to describe the size of the nevus properly for later compartion. Also petechie can be revealed. The most probable reason is an increased venous pressure during the labour and delivery. Then, petechie are located on the face or head, sometimes very rich, with appearance of continual blue areas. If petechie are generalised, trombocytopenia must be considered (caused by intrauterine viral infection, action of antitrombocytic antibodies etc.). Hematomas can be found on the head, extremities,in the pelvic area or in other locations. Sometimes they are becoming to be apparent only several hours after delivery. Large sufusions lead to suspicion of coagulation disorder. Frequent finding during the first week is erythema toxicum. It can begin as several small red spots, sometimes confluencing and covering bigger surfaces, and not rarely generalised. This is a response of the sensitive skin to first postnatal contacts with extrauterine environment (touches of napkins, hands etc.) It ´s evolvement is less likely if the skin is cared for gently during the first hours, without rough rubbing, and if vernix is left on the skin at least several hours. This is an itching exantema but disappears spontaneously until the end of the first week. It is good to avoid scratching of the skin which could support the secondary infection. 1- 2 chamomile bathes per day seems to relieve itching. So called „mongolian spot“, red-violet area of the skin in lumbosacral location, is insignificant finding, more usual in asian races. The order of examination uses to be described craniocaudally. However, if the baby is quiet, it is advantegeous to begin the examination with heart and lungs auscultation. Later, baby usually start to cry and the auscultation may be more difficult. Lung auscultation gives information about normal postnatal expansion of the lungs: one should hear symmetrical alveolar breathing. We assess regularity and rate. Normal rate of breathing in newborn is about 40/min, with range about 30 – 60/min. The brathing of a healthy newborn is not necessarily absolutely regular, but time intervals between breathes should not exceed 10 seconds. Longer interval is called apnea. One-sided weakening of breathing sounds may be caused by atelectasis, emphysema or pneumothorax. Breathing is weakened bilaterally in RDS or pneumonia. Grunting is a specific expiratory sound which may be listened in respiratory distress. Inspiratory stridor may be caused by inborn feebleness of ligamentous laryngeal structures (stridor laryngis congenitum), usually transient, but it may also have it´s origin in vascular ring compressing trachea or in tracheomalacia. While auscultating heart, the heart rate is counted (normal range in quiet baby 100160/min) and the regularity of heart beat has to be assessed. Respiratory arrhytmia is not present in newborn but mild undulations of the rate is normal. If the irregularities are more pronounced, the ecg examination and/or ecg monitoring is indicated to rule out serious ventricular dysrythmias. We are looking for murmur. The heart sounds are distinct in healthy newborn, but sometimes quiet changeable murmur associating physiologic postnatal adaptation of circulation may be present transiently. Rough or unusually loud murmur may lead to suspicion of congenital heart defect and indication of echocardiography. The shape of the head should be noticed: mesocephalic shape is usual in babies born by cesarean section or in breech position. After the head-position delivery the shape may be modified during the first hours, sometimes quite a lot – usual elongation of the scull to the 17 vertex is caused by the compression of the head in the birth canal and the flexibility of the sutures. The shape changes to normal in several hours. The anterior fontanelle should be palpated. It is open in healthy newborn, the most usual diameter is 2-3cm. However, the diameter of only several mm orconversely, 4-5cm can be also physiological. Open sagittal suture and open posterior fontanelle may be signs of delayed scull ossification. If the anterior fontanell is extraordinarily large and/or the head circumference is increased, the ultrasound examination of the brain is appropriate to rule out hydrocephalus. Whole scull should be palpated thoroughly. The finding of fractures is rare, but possible, especially after the forceps delivery. Caput succedaneum is usual finding: it is a soft doughy edematous subcutaneous infiltration, most often in the occipital or parietal area. This is caused by the lower pressure in the vessels of just delivered head of the newborn (before the delivery of the rest of the body) and is transient, disappears after several days. Sometimes one can find kefalhaematoma, subperiostal bleeding, which is tough, fluctuant and located usually in parietal areas, often bilaterally. It´s consistence is different from caput succedaneum and it does not cross the borders of the sutures. It resorbs longer time, goes through the period of partail calcification and may persist to the second month of age. We examine the external ears. Soft and inflexible cartilage suggests immaturity of the baby, anomalous shape or low position of external ears may be sign of genetic disease. These findings should lead to looking for other morphological anomalies. There can be found skin outgrowths in front of the ears, usually benign, but occassionally also as a part of genetical syndromes. It is usually possible to remove the outgrowths by simple strangulation with sterile thread. However, it is probably preferable to refer the patient to plastic surgeon. The eyelids use to be swollen after birth. It is better, then, not to open the eyes violently, but to postpone the eye examination one or two days. Also next time the baby may have closed eyes while examined. Vestibular reflex may be helpful: we hold the baby vertically and perform several rotational movements around the vertical axis of the body, in the angle about 90, back and forth several times. The baby usually opens the eyes during this maneuvre. Newborn is able to recognize main features of the face of the examining physician and to follow the face again approximately in the angle 90. But, the accommodative ability is limited in the newborn, and so the face has to be in optimal distance , approximately in adult´s arms distance. If the baby follows our face with movements of his/her eyes, the amaurosis can be practically ruled out. Very frequent finding is a bleeding under corneal conjunctiva (subconiunctival suffusion or episcleral hemorrhage), which may be apparent on one or both eyes as very conspicious red spots on sclera. It is also caused by increased pressure when the head goes through birth canal and it is harmless. Usually it disappeares spontaneously after one or two weeks. The congestion of conjunctiva can occur in the first days or even infectious conjunctivitis. From the half of the first week the scleral jaundice can be found. Chorioidal koloboma, enlarged bulbus in congenital glaucoma or dim whitish pupils in congenital cataract are rare findings. The size of bulbi should be noticed. They should be symmetrical, comparation with adult´s eyes may be helpful. We ask the mother to hold the baby on her arm, with her own cheek next to the cheek of the baby. The increased bulbus of the infant may seem bigger than adult´s eye. It is possible to examine newborn´s red reflex using opthalmoscope or retinoscope, thus confirming the translucency of eye media. We can find cleft of the lips, more usually paramedial, either unilateral or bilateral and more often associated with a cleft of the palate than isolated. We observe the mouth where the isolated cleft of the palate can be sometimes found. The palate should be palpated with a clean finger to rule out the hidden submucous cleft. If the cleft was found, it is important to be careful with feeding in the beginning. Some babies are able to suck and to be breastfed, but sometimes the bottle feeding is necessary. We have to prevent milk aspiration. Parents are 18 usually terrified by the finding and it is important to reassure them and to offer the information about the possibilities of successful surgery. It is advantegeous to be prepared to show the parents the pictures of older children who underwent such surgery. See picture 3. When the baby has a cleft, we are looking for the associated anomalies. If teh cleft is isolated finding, we refer the baby to plastic surgeon. The surgery is performed in several steps during the first years of life. The lip is usually operated during the first months. Short sublingual frenulum is usually variation of physiology and should not be operated in newborn – it is likely, that it will improve to normal function. The finding of neonatal tooth is rare. It is necessary to extract the tooth gently to avodi the aspiration. Mild salivation is quite usual, especially during the first days, when thegastrointestinal peristalsis only begins and the baby spits up the remnants of amnial liquor. Enormous salivation, however, should not be omitted. It can be sign of swallowing difficulties in neuromuscular disorder, for exaomple in congenital myotonia, or esophageal atresia. If atresia of esophagus is present, the salivation is often associated with episodes of cyanosis. The suspicion of esophageal atresia leads to sondage of the esophagus, which confirms whether the esophagus is clear to the stomach. If the atresia is confirmed the permanent tube should be put into the blind stump of the esophagus, the saliva should be suctioned continually and on the stable infant surgery should be performed. We examine the nose of the baby, confirm patency of both nostrils: with mouth closed, we press externally one and then the other half of the nose. If atresia is suspected, patency is confirmed by pulling tube through nostrils. Atresia is indicated to immediate surgery. Especially bilateral choanal atresia can lead to acute respiratory distress and cyanosis and may necessitate the use of plastic airway aid until the operation is done. We observe the shape of chest , assess the maturity of breast nipples and mammal glands and examine properly clavicles. Fractured clavicle belongs to the most frequent birth injuries, especially of big fetuses, after complicated deliveries associated with anomalous position or forceps deliveries. The lateral third of the clavicle is mostly involved. During the first hours and days one can palpate crepitation above the place of fracture. The palpation may be painful for the baby. The fracture may be associated with brachial pseudoparalysis – situation, when active movement of the extremity is possible but the baby avoids movement because of pain. It can resemble real brachial palsy, but pseudoparalysis is transient and resolves spontaneously after several days. After the fracture is found, it is important to reassure the parents. The fracture is almost always benign. It is recommendable to spare the extremity for one or two weeks, not to palpate the place again and again uselessly nor to move the extremity qithout purpose. The fracture is usually healed without treatment in several weeks. After several days crepitation disappeares, then palpable lump (calus) occurs and afterwards, until the 2nd month of age, the bone is fully reconstructed. Mammal glands may be increased sometimes and even mild milk production may be apparent. Maternal hormones cause this hormonal reaction. No treatment is necessary, spontaneous resolution is a rule. It is easier to palpate abdomen in a baby who, lying on his/her back, is a little bended forward. The abdominal wall is softer then. Margin of newborn´s liver may normally be 1 2cm below the costal arch, spleen is either not palpable or one can feel the lower pole. Both kidneys may be palpable, especially in smaller babies. tumor must be ruled out (increased multicystic kidney, ovarian tumor etc.). Good intestinal peristalsis may be confirmed by auscultation. Umbilicus is always examined, bleeding and inflammation must be ruled out. After the umbilicus is cut, umbilical vessels should be checked. Usually one vein and two arteries are seen. If one artery is missing, one should suspect increased risk of associated congenital organ anomalies, especially of urinary tract, which should be examined by ultrasound. Omphalocele is one of congenital anomalies visible by a first view. This is abdominal organs herniation through the enlarged umbilical orifice, sometimes large and not easily treatable by 19 operation. Similar malformation is gastroschisis – herniation through the paramedial orifice besides the umbilicus, usually smaller than omphalocele and with better prognosis. The other malformation involving abdominal wall is urinary bladder exstrophy. The examination of femoral arteries is a part of examination. It is screening for coarctation of aorta – weak or absent pulse should lead to indication of echocardiography. Palpation is always possible, but sometimes difficult when the baby is restless or crying. One has to be patient and, if uncertain, to repeat the examination later. The examination should be performed not sooner than on day 3 or 4. Until then, the prenatal physiological shunts may be still open and the femoral pusle may be palpable even in patients with coarctation. In the lumbal area of the back impaired skin surface can be found when herniation of menings is present (meningocele, meningomyelocele). Or, the spinal cord dysraphism may be limited to dorsal parts of vertebrae, without herniation. This split (spina bifida) may be palpable under the intact skin. The finding of pilonidal sinus is not rare. this is a dimple in sacral area. If the bottom of the dimple consists of intact skin, the finding is usually benign and no other examination is needed. The dimple sometimes disappeares after several months. In rare cases, where the sinus communicates into the deeper layers and the bottom is not covered with skin, next investigation is necessary. The dimple may be part of fistula and of more complex spinal cord malformation. The examination of hips is important. It is performed by pediatrician (neonatologist) or by specialist – pediatric orthopedist. The examination is reliable only if the baby is quiet. The newborn is laid on firm surface on his/her back. Thighs are flexed to vertical position and the lenth of both thighs is checked under gentle vertical pressure. Then we take the thighs in our hands: thumb touches the inner part of the thigh in it´s upper third while middle finger touches the area of trochanter major. We perform maneuvre of reposition (Ortolani) by gentle rotation with adduction and pressure of the middle finger on trochanter. Then maneuvre of luxation (Barlow) follows with rotation in opposite direction and pressure of the thumb in lateral direction. In healthy newborn, both fenomena are negative, articulations are firm in rotations and the adduction is free at least to 45 from the table. If dysplasia is present, reposition fenomena is positive as a clunk with a shift of thefemoral head back to the articulation and luxational fenomena – head „jump out“ the articulation. In milder degree of dysplasia, the head does not luxate spontaneously, but luxation by luxational maneuvre is possible – „luxable hip“. On the contrary, in more pronounced asymmetrical dysplasia, also some other findings may be present: limited adduction, length of thighs asymmetry (Bettman´s sign: exarticulated femur is shifted higher and „shortened“) and skin folds asymmetry on thighs and buttocks. Every pathological finding is and indication to refer the baby to pediatric orthopedist for ultrasound examination and early treatment, enabling normal development of the articulations. Missed diagnosis may lead to fixed articulations damage and delayed development of walking. While examining extremities we observe the shape and motion, articulations, number and shape of fingers. Syndactylia may be found, sometimes as isolated and benign sign (most frequently patial adhesion of 2nd ad 3rd finger on feet) but some other time associated with other morphological organ malformations as a part of genetic disease. Also redundant fingers, partial or total finger aplasia or reduction deformities of the whole extremities are possible findings. The examination of genitalia is also important. In boys, glans should be fully covered by praeputium (which cannot be totally pulled back and neither examining doctor nor parents should not be trying that). Scrotum and testicles findings were partially described above as a part of maturity assessment. Diameter of testicles may be 1-2cm, mild asymmetry is common. Among anomalous findings hydrocele is not rare, usually without need of any treatment. It is recommendable for the community pediatrician to follow the baby during the 20 following months – hydrocele usually resolves spontaneously. Only if hydrocele is very large, filling major part of the relevant half of scrotum, covered with tense skin, it can threaten the nutrition of the testicle and be indicated for surgery. To examine glans, pulling back praeputium a little is needed, to make clear that outer end of uretra is normally positioned and shaped – it should be slit and in the apex of glans. If possible, it is contributing to see the baby urinating and to assess the quality of the stream: very thin or oblique stream may suggest uretral anomaly. Anomalous position of uretral ostium is also not rare. Hypospadia is more often found, i.e. ostium located on dorsum of glans or penis or even on perineum. It may be difficult to find the exact position of uretra visually and again observation of urination may be of help. Epispadia is less frequent. If epispadia or hypospadia has been found, an ultrasound examination of urogenital systém is indicated to rule out associated anomalies and the baby should be referred to pediatric urologist and/or plastic surgeon. Fining of undescended testicles is significant as well. More commonly it is partial, sometimes one-sided. If at least one testicle can be palpated, in scrotum or in inguinal duct, the baby should be only followed by community pediatrician. (If the descensus of testicles is not finished until 1 year of age, irreversible damage of testicles may occur and hormonal treatment or surgery may be warranted to avoid it.) If we are unable to palpate even a single testicle (total cryptorchism), one must take possible intersex into consideration, especially if other morphological anomalies of genitalia are present. It is critically important to rule out congenital adrenal hyperplasia, because the baby could be endangered by mineral inbalance, hyperkalemia and sudden death! Also history of unexplained sudden deaths in older siblings in neonatal period may support the diagnosis. Mature genitalia of newborn girls was also described above. The finding of yellowish skin tails, partially covering the introitus of vagina is not rare and is benign. Also mild whitish or haemorrhagic vaginal discharge may be seen quite often and is result of maternal hormones influence. Newborn girl´s clitoris may seem diproportionatelly large, especially in premature ones, but it should not resemble fallic appearance. Also in girls anomalous position of uretral ostium on perineum etc. is possible. Hernias may be apparent in newborn. Inguinal and umbilical hernia are most commonly involved. In inguinal hernia, especially in boy, it must be checked, whether it is reponible. If the herniation is large surgery is sometimes necessary. If the hernia cannot be given back (strangulated hernia), the immediate surgery is indicated. Umbilical hernias usually do no need surgery, but rarely, for example in prematures, even umbilical hernia may have potential to strangulate and operation may become necessary. Anal ostium is always examined. We observe the position and outer appearance, the patency is confirmed by the first temperature measuring and the first passage of meconium. Manual rectal examination is almost never perfomed as a part of pediatric examination of newborn. Basic neurological examination should be performed. It consists of assessment of muscle tone, usually with repeated passive flexion and extension of upper extremities, or according to lower extremities and general posture while picking up the trunk. In mature baby the palmar grasp reflex may be elicited by pressing on the palm – the baby grasps the pressing fingers, usually firmly enough that it is possible to pick up the baby partially above the table. Sucking reflex is also present: it can be elicited if one puts his/her finger in the mouth of the baby. Also startling reflexes are easy to examine, mostly Moro reflex: in a quiet baby lying on it´s back the napkin baby is lying on is quickly shifted. The normal reflex has 2 phases. In phase one the baby stretches out his/her arms, in phase 2 he/she put hands to the chest and legs to the abdomen and starts crying. We evaluate especially the symmetry of Moro reflex. Also „walking“ reflex can be elicited in newborn: a baby holded vertically above a table, whose heels touches gently the table, starts to change feet as if walking. More detailed examination of neonatal reflexes, if indicated, should be performed by specialist. 21 Postnatal adaptation in general Except of the pediatric physical examination, continual observation of the behaviour of the baby throughout the whole time of stay in maternity hospital is useful. Healthy newborn uses to sleep major part of the day, 18 – 20 hours on average. During the first days or weeks he usually does not distinguish between day- and nighttime, sleeps and falls asleep irregularly and for different periods of time. The phase immediately after his waking up is the most active one, with active movement, interest for the environment and breastfeeding. It is preferable not to try to rule newborn´s activities according to watch and not to wake him up actively. For instance, if we decide to wake the baby up each 3 hours because of breastfeeding, it leads often to interrupting his/her deep sleep, after which the baby is not necessarily in the best mood for eating. Bad temper and restlessnes may complicate breastfeeding then. If we allow natural course (the baby is breastfed anytime he/she wakes up spontaneously), the baby will at first wake up rather irregularly and the intervals between feedings may be once 1 hour while next time, for example, 5 hours. If the interval is not extraordinarily long (e.g. more than 6 hours), we do not wake up the baby actively. The fact, that newborn wakes up for breastfeeding at night the same way as during the day is tiring for mother but it must be taken into consideration. The rhythm of sleeping at night and vigil during a day develops gradually and the process may last weeks or months. Thus, mother has to sleep with a baby part of a day. The period of activity immediately after awakening may last different piece of time, sometimes 5 minutes, other time one hour or more. Therefore, it is important, especially during the first weeks, to allow the baby to breastfeed early after awakening so that he/she can use the most active time for sucking the breast. This way, good cooperation of the mother and the baby is formed and good advancement of lactation is supported. The cry of the baby may become important psychological problem of some parents. Although the cry can be considered, to certain extent, natural way of communication in small infants and staff of neonatal wards is used and „resistible“ to it, for newborn´s mother the inconsolable crying can represent months lasting ordeal. For parents, who are worried by frequent crying, reassurance from nurse or doctor (after baby´s examination), that the reason of crying is not serious one. It is necessary to make sure, that no apparent discomfort causes the reason for crying (pain, heat or cold, beeing wrapped too firmly, not allowing to move, feeling unstable etc). If we are unable to find any pathological finding or discomfort, crying should not be reason for everlasting anxiety. Usually working approach to appease the baby is: crying baby wants to suck the breast! The vast majority of babies quiet down after breastfeeding is allowed. Also for this reason it is not reasonable to delay breastfeeding just because short time elapsed from the last breastfeeding. If the baby does not want to suck, is leaving the breast and turning away from it and goes on crying, bad technique of breastfeeding or, rarely, anomalous shape of the nipple can be a reason. We have to accept the fact, that in some cases the reason of cry will not be found and it will not be easy to appease the baby. Then, it is imporatant to explain to the parents, that frequency and intensity of crying is to certain extent associated with high spirits of the baby and a right approach is to do their best to comfort him/her, to try to find the most comfortable position etc. (In older infants, the problems evolves into the problem of infant „colics“, when baby is crying with his/her legs pulled to abdomen. Not in this case the underlying problem must be usually blamed abdominal pains and „gassy stomach“. Attacks of cry and colics begin usually 22 disappear at latest in the second half of the first year and they do not interfere in any way with normal development of the infant.) Jitternis is frequent associating sign of neonatal period. These are irregular movements of extremities or the whole body either in vigil or in sleep state. They may alarm parents as well as to become a diagnostic problem for a physician. to diiffer jitterness from real seizures is sometimes difficult, especially because seizures in newborn may be nonspecific – for example, look like singel movements, face grimaces or apneas. To differ jitterness from seizures several features can be used. Jitterness is usually symmetric, not one-sided. On the other hand, only one half of the body may be involved in seizures. Jitterness is not associated with abnormal eye movements. And, simple maneuvre can be performed: we try to hold the extremity with suspected movements in our hand and try to stop the movements. If jitterness is the problem, it is possible to stop it. On the contrary, the extremity involved in seizure attack, the movements go on in spite of our effort to stop it. Weight development A newborn decreases his/her weight during the first days after birth. This decrease is temporary and under normal circumstances lasts not more than 3-4 days and is not bigger than 10 – 15% of the birthweight. In premature babies the decrease may be deeper and may last longer. The underlying process corresponding to the postnatal weight decrease is the transition of circulation from fetal into postnatal type. It is associated with contraction of intravascular compartment and temporary over-abundance of body fluids. A newborn excrete this part of his fluids and his weight decreases. The initiation of lactation is related to the weight decrease. The baby is nurse during the first hours after birth several times „ for zero“ (no milk is drunk), first small doses of milk are drunk optimally about 24 hours after delivery. The body undergoes short transient catabolic period. Gradually, the milk production begins, the drunk doses increase, anabolism predominate, the infant start to increase his weight (from day 3 – 4) and in the 2nd week drinks already 100 cc per dose or more. Normal speed of weight gain throughout the first year of life is approximately 100-250g per week. See graph 2. Postnatal weight decrease is a natural phenomenon and it is useful to alert parents in before and then to reassure them when decrease occurs, that the weight is evolving normally. The efforts to avoid weight decrease by early offer of bottle with water or milk should be discouraged. The decrease will occur anyway and the bottle feeding may interfere with breastfeeding. That is why in healthy and mature newborn no fluids are offered during the first 24 hours after birth. If the lactation initiation is delayed and it is necessary to offer some fluids, water or tea should be preferred (not milk) given from a cup, a spoon or a syringe. After lactation begins (usually not later than on day 2 – 4) we stop offering these fluids immediately and allow exclusive breastfeeding. Gastrointestinal system adaptation Gradual initiation of lactation and breastfeeding were described above. In accordance with them, functional adaptation of gastrointestinal system is under way. Intestines of the fetus are filled with a black content, meconium, consisting from intestinal epithelium and swallowed amnial liquor. An intestinal peristalsis is slow before delivery and there is normally no passage of meconium. (finding of meconium in amnial liquor is abnormal and suggests intrauterine hypoxia. The hypoxia itself as well as meconium aspiration bring risk to the fetus, then.) After birth, peristalsis strengthens, the first passage of meconium occurs usually during the 1st day, at latest until 48 hours after birth. The lack to pass meconium may suggest congenital gastrointestinal tract (GIT) obstruction. Under normal circumstances, black 23 meconium changes into normal stools of breastfed baby of yellow colour and mushy consistence. Natural strat of breastfeeding enables to increase step by step the load of milk. On the first day GIT is not prepared to accept milk: peristalisis is only beginning, meconium is beginning to pass and the upper parts of GIT are cleaned from remnants of amnial liquor, process often associated with mild spitting-up. Bottle-feeding the baby with formula from the very first hours may bring problems. The baby, fully capable of sucking reflex, drinks eagerly from the bottle, is able to drink 30-40 cc or more, but this „success“ is often folowed by vomiting and risk of milk aspiration. GIT is not ready and does not tolerate too early feeding with tto large doses. Also other signs then delayed meconium passage may alert anomalies of GIT. Finding of intestinal dilatation on prenatal ultrasound examination of the fetus may lead to intestinal atresia suspicion. After birth, atresia may manifest by vomiting (the earlier the vomiting, the more orally the obstruction uses to be in esophageal atresia the baby is vomiting from birth, in atresia of ileum or rectum the vomiting may occur only after several days). The warning sign is abdomen overdistension or general sepsis symptoms (from peritonitis after perforation). Meconium ileus is a specific case, where GIT obstruction is caused by increased viscosity of meconium. It may suggest cystic fibrosis. Urinary tract adaptation The urine is formed in small amounts in kidneys before birth and is passed into amnial liquor. After birth the first urination should be observed. This is an evidence of patency of urinary tract and should occur not later than 24 hours after birth. If the first voiding have not occured, the urinary bladder should be examined (with palpation and ultrasound) and the reason of the delay should be searched. The amount of urine may be very small initially, about 5 cc for one voiding and the urine is usually transparent, almost without yellowish coloration. Therefore, the napkin mut be observed thoroughly, because otherwise the first voiding could be missed and this mistake could lead to useless investigation of the baby. during the next days, with increasing doses of milk, also diuresis increases. We do not measure exact amount of urine in healthy newborns, but i should be at least about 1-2cc/kg/hour. Hemopoiesis adaptation, neonatal jaundice Normal hematocirt of healthy newborn is higher than normal hematocrit anytime later in life, 0,55 – 0,65 on average. (A newborn with hematocrit less than 0,40 on day 1 after birth should be considered anemic.) The increased rate of reticulocytes (up to 60 per mile) is an evidence of increased stimulation of erythropoiesis. At birth, the way of oxygenation changes: oxygenation by gas exchange in the lungs is more efective than oxygenation via placental circulation and the baby will need less erythrocytes for successful oxygen tissue supply in it´s extrauterine life. Therefore, the activity of erythropoiesis attenuates soon after birth (reticulocytes decreases to 1-5 per mile). this process is associated with an increased hemolysis. The balance between erythrocytes production and degradation is shifted to the side of degradation (lower need of erythrocytes, and their shorter survival) and hematocrit decreases. This decrease is quite quick during the first days and hematocirt may fall to 0,40 – 0,45. (Later, the decrease gets slower, but the period of erythropoiesis depression and hematocrit fall continues until the 2nd – 3rd month. Then usually the development turns, erythropoiesis 24 intensifies and hematocrit gradually increases to the level usual in older children.) See graph 3. The development of neonatal jaundice is related to hemopoiesis adaptation. Yellow coloration of the newborn´s skin, usually appearing during the first week of life, is a normal physiological associating feature of the above described accelerated hemolysis. While normal plasma bilirubin level of older child is less than 20 μmol/l, bilirubinemia in newborn on it´s peak (usually on the day 3 or 4) is often 200-300 μmol/l, occasionally even more than 300. Nevertheless, it is important for the level not to increase to extreme values. Levels above 400μmol/l are associated with a risk of brain damage. This can happen most probably when isoimmunization is an underlying problem, i.e. immunologic colision between Rh or ABO blood groups of the maother and the baby. One of the substantial tasks of pediatrician is to follow neonatal jaundice properly and to diagnose non-physiological course as soon as possible. Normal physiological jaundice has several important features: 1. It is not present at birth and it appears not sooner then after 24 hours. 2. It´s intensity does not exceed common limit. 3. It peaks on day 3-4 and spontaneously disappears afterwards. The said „common limit“ is estimated firstly visually by the pediatrician with use of his/her clinical experience. There is an optical tool capable to partly objectify this visual estimate, called transcutaneous icterometer (or bilirubinometer). This aid uses arbitrary units for rough quantification of intensity of yellow skin colour and it´s trend. Nevertheless, the reliability is rather dependent on natural coloration of the skin; it is very inaccurate in babies with dark skin. In caucasians, if measured with the only available machine Minolta, findings of 20 and more arb. units usually require further investigation. If pathological jaundice is suspected, the objective method to evaluate it´s intensity is only measuring bilirubinemia from blood sample, repeated as needed. Not only absolute bilirubin level is important, but also a factor of time – the age of the baby in hours. During the first 4 days, the earlier the bilirubin level increases and the steeper is the increase, the more likely is an achievement of dangerous levels. In other words, the same level (for example, 220 μmol/l) may be absolutely normal on the 4 th day after birth, but at the age of incomplete 24 hours it may be the reason for intensive treatment. Also, the increased level must be considered as more serious in babies with evidenced isoimmunisation, especially Rh, where higher dynamics of increase must be assumed. The assessment is also different in premature babies, in which phototherapy is usually started sooner, with lower levels. To evaluate how serious jaundice is and for indication of phototherapy special graphs or tables are used (in the Czech Republic, for instance, graph Hodr-Polacek). See graph 3 and table 9. Table. 9: Indication of jaundice and bilirubinemia monitoring and indication of hyperbilirubinemia treatment according to the zone in Hodr-Polacek graph. Valid in term newborns without Rh or ABO isoimmunisation and in term newborns with Rhisoimmunisation. (PT… phototherapy, ET … exchange transfusion, bili … sampling for bilirubinemia, TCI … transcutaneous icterometry) zone without Rh or ABO Rh V PT, if no success, ET ET IV PT PT, if no success, ET III bili, TCI repeatedly PT II TCI 2 times a day bili I TCI once a day bili 25 For better evaluation of the dynamics of bilirubinemia it is useful to know the bilirubin level of umbilical blood, i.e. in the moment of birth. Normal value is less than 40 μmol/l, 40-50 is borderline. Umbilical blood bilirubin above 50 is an clearly increased „starting“ value suggesting pathological hemolysis in fetus and predicting abnormal speed of increase of bilirubinemia during the first hours after birth. Except of assigning in graph also evaluation of speed of increase of bilirubinemia is helpful. In healthy baby, the speed is not higher than 5 μmol/l per hour, while serious isoimmunisation leads to the speed exceeding 10 μmol/l per hour. The strongest reaction of this type is Rh-isoimmunisation originating from constelation of Rh-negative mother and Rh-positive fetus. Rh-antigen on erythrocytes of the fetus penetrates into the circulation of the mother (during pregnancy, labour and delivery), stimulates production of anti-Rh (anti-D) antibodies, which after their penetrance back to the circulation of the baby hemolyse his/her erythrocytes. Similar reaction may be caused when conflict in other blood groups exists (ABO or some rare blood groups) but hemolysis is usually not so serious Rh-isoimmunisation used to be the most frequent reason of extreme hyperbilirubinemia in newborn, which jeopardised the baby seriously. In extreme levels, bilirubin may transfer hematencephalic barrier and form deposites in brain tissue, mainly in basal ganglia (kernicterus). This process causes irreversible brain damage and may lead even to death. In surviving babies, permanent neurological and developmental handicap called bilirubin encephalopathy evolves. With up to date knowledge and prevention and treatment possibilities kernicterus can be always prevented. Rh-isoimmunisation prevention involves passive immunisation of Rhnegative mothers immediatelly after delivery of Rh-positive baby (administration of anti-D antibodies attenuates the immunological reaction and endogenous antibodies production, thus attenuating reaction in possible next pregnancy). Also, it is important to assess the blood groups in all pregnant women in an early stage of their pregnancy and to follow closely all Rh-negative ones. Evaluation of anti-D antibodies plasma level should be part of this followup. If these antibodies are found, the development of the fetus has to be closely monitored and if serous intrauterine course is revealed, the early intervention is necessary. In the past, evaluation of bilirubinoids in amnial liquor used to be performed, nowadays early percutaneous umbilical blood sampling (chordocentesis) for evaluation of fetal blood count and bilirubinemia, and intrauterine transfusion if indicated. Serious anaemia adn fetal hydrops can be avoided this way. Treatment of pathological jaundice Normal physiological jaundice in healthy newborn does not require treatment. In pathological cases early intervention is required. The most frequently used treatment is phototherapy, method based on interference of electromagnetic waves of specific wave-length (420-470nm) comprised in violet and green part of light spectrum with bilirubin molecules stricken by the light in the blood of superficial skin capillaries. The efficacy of the method depends on the body surface on which the light falls and on the intensity of the light. As a source of light special lamps are used which provide concentrated light af desired wave-length. Treated baby must be naked in and incubator or an electric heater. In accordance with how serious the hyperbilirubinemia is intensive phototherapy may be used, i.e. use of several lamps together. Also combination with bili-blanket enabling underneath exposure is useful. Bilirubinemia increase is influenced also by the degree of hydration. Therefore adequate fluids offer is needed. In many cases breastfeeding itself is enough, sometimes we can add the offer of tea or water from spoon or syringe, not from bottle), in serious cases of isoimmunisation fluids are given intravenously. 26 If the treatment with phototherapy is not successful in maintaining bilirubinemia in normal physiologic range of values, exchange transfusion is a next step. To perform the latter, we have to insert catether in umbilical vein and then, during several hours lasting procedure to take up from circulation part of baby´s own blood and to replace it with a blood of the same group and Rh-negative (recommended volume is 180-200 cc/kg). This way part of bilirubin as well as antibodies is removed and bilirubin level increase slow down sharply. Rarely, it may be necessary to repeat the procedure twice or even three times with intervals of several hours. Other treatments are only auxiliary: administration of albumin binding bilirubin in the blood such decreasing it´sability to cross hematencephalic barriere. Some other treaments were used in the past, as administration of phenobarbital; however, this treatment does not influence progress of hyperbilirubinemia significantly and may be even complicating factor because of it´s sedative effect. It should not be recommended anymore. In future, management of neonatal hyperbilirubinemia might include administration of mesoporphyrin, drug decreasing bilirubin production. Other forms of pathological jaundice Occasionally some other form of pathological jaundice may be found in newborn. Increased hemolysis in newborn may be associated with benign hyperbilirubinemia (Gilbert disease) or, rarely, with serious hereditary disorder of bilirubin metabolism (e.g.,Criggler-Najjar disease). Common feature of all hemolytic forms of hyperbilirubinemia in newborn is that increased level of unconjugated bilirubin is an underlying problem. Conjugated bilirubin level may be normally slightly increased in newborn, but it should not exceed approximately 25μmol/l. If the level is significantly higher, congenital hepatopathy should be considered (e.g., from intrauterine viral infection, cholestasis in biliary atresia etc.). If significant part of bilirubin is it´s conjugated component, phototherapy is not indicated (it is not effective and leads to produciton of bilirubin metabolite discolorating the skin in so called „bronze icterus“). Postnatal adaptation of metabolism, hypoglycemia Metabolic changes are complex. Problems of glycemia should be noticed. Normal glucose level is in intrauterine life maintained by supply from mother. Permanent overload of glucose maintains the fetus in the state of relative hyperinsulinemia. In the moment of umbilicus interruption the external supply of glucose is stopped. Remaining hyperinsulinemia causes fall of glycemia during approximately 1 hour after birth to borderline values about 3,0 mmol/l. This glycemia fall stimulates adaptive mechanisms, insulin level falls, glycogenolysis and gluconeogenesis begins and glycema begins to increase again. during the second hour after birth metabolic circumstances changes to permanent normoglycemia. Postnatal fall of glycemia may be faster and deeper and may lead to serious symptomatic hypoglycemia under certain pathological conditions. For example, in an infant of diabetic mother. Especially if mother´s diabetes mellitus is not adequately compensated during her pregnancy, the fetus undergoes repeated hyperglycemias, compensated by pathological hyperinsulinemia. Remaining increased level of insulin during the first tens of minutes after birth may cause serious hypoglycemia (even less than 0,5 mmol/l!) with seizures or apnea. That´s why obstetrician has to notify pediatrician before delivery about mother´s diabetes and the baby has to be monitored after birth. During the first hours glycemia is evaluated each 30-60 minutes and, if necessary, parenteral supply of glucose is ascertained early enough. Also premature and hypotrophic babies are prone to postnatal hypoglycemia, as well as asphyctic babies or babies with congenital bacterial sepsis. 27 Adaptation of thermoregulation When in a womb, fetus need not to maintain it´s body temperature by it´s own thermoregulatory mechanisms, because it is under influence of mother´s thermoregulation. In the moment of birth, the body temperature tends to fall till the moment, when this fall is counteracted by initiation of baby´s own adaptive mechanisms. In healthy term newborn the temperature falls to borderline values about 36,0 °C during the first hour after birth and then increases to normal values. Risk of hypothermia is bigger, for example, if the temperature of environment in the delivery room is low or, if the skin surface is not dried or in some pathological situations like asphyxia, congenital sepsis, prematurity, intrauterine growth retardation etc. Breastfeeding Problems of breastfeeding are everyday destiny of neonatologist. In an environment with well-established system of breastfeeding support there is not a lot of work for physician left and a practical education of mothers is more pediatric nurses´and midwives´ concern (or, in some countries, concern of „lactation consultants“). However, it requires a lot of hard work of doctors to create such environment. The period, when formula feeding got overestimated (1950 – 1970) are already the past. Multiple scientific evidence was published about advantages of breatfeeding for baby and mother and it is difficult to disprove. Every pediatrician and obstetrician should be aware of this knowledge. See table 10. Table 10.: Advantages of breastfeeding for baby and mother Advantages for baby evidenced: decreased risk of infant diarrhea decreased risk of respiratory and other infections in infant period including sepsis, meningitis and UTI decreased risk of SIDS decreased risk of necrotising enterocolitis in prematures decreased risk of food allergy in childhood decreased risk of Crohn´s disease and ulcerose colitis decreased risk of dental caries in childhood decreased risk of obesity in childhood b) likely or possible decreased risk of hypertension and atherosclerosis in childhood and adult age more favourable cognitive development (higher IQ) decreased risk of some malignancies, e.g. lymphoma decreased risk of diabetes mellitus type I Advantages for mother evidenced: shortened period of postpartum bleeding, quicker postpartum uterus 28 involution decreased blood loss during lactational amenorrhea improvement of postnatal bone remineralisation decreased risk of malignant breast and ovarian tumours General advantages psychologically significant for mother-infant bonding practical convenience of breastfeeding economic advantage for the family As the table shows, breastfeeding represents an important preventive factor not only for infancy but really for whole life. Most of world significant health-care authorities (for instance, WHO or American Academy of Pediatrics) recommends exclusive breastfeeding until 6 months of age and then partial breastfeeding until different age with a step by step introduction of other food. Exclusive breastfeding refers to the offering of food exclusively by nursing, without any combination with formula or other feeding. Even single doses of formula fed unnecessarily in the period of lactation initiation may lead to lifelong sensitization. Furthermore, premature introduction of bottle feeding complicates establishment of good cooperation between mother and baby and may be reason of unsuccessful breastfeeding efforts. The younger the infant, the more significant are advantages of breastfeeding. So, breastfeeding is most important during the first months. The specific age of weaning is not so much important and depends on mother, family and baby´s preferences. The optimal age in weaning is perhaps between 12 a 24 months. Success of breastfeeding as measured by percentage of infants breastfed exclusively until 6 months of age is presently the highest in scandinavian countries (80% and more). The exact information about the smae value in the Czech Republic is lacking but it seems to be about 30% and increasing. Several factors support success of breastfeeding: 1. a family should have enough information about advantages of breastfeeding before delivery (prenatal classes, prenatal visit to pediatrician) 2. a philosophy of non-separation of mother and child after birth cannot be overemphasised – mother and child should be in a skin-to-skin contact from the moment of birth and the first nursing should take place not later than 30 minutes after birth (early initiation) 3. a baby should be breastfed anytime when wakes up, independently on time, and allowed to suck as long as he/she is sucking 4. if the progress of lactation is normal (baby drinks the first doses of breastmilk approximately 24 hours after birth) and the baby is healthy, offering other fluids is not needed, we wait for breastfeeding 5. if the lactation is delayed (baby drinks the first doses of breastmilk on day 2-4), we offer after nursing fluid supplement (tea or water) as the baby wishes, from syringe, cup or spoon (never from bottle), we do not offer formula, we wait for breastfeeding 6. offering formula should come into consideration only in rare special occassions, when lactation does not progress in spite of all efforts, and not sooner then on day 5. If only temporary formula feeding is presumed, hypoallergenic formula should be used 7. unnecessary use of plastic nipple-shields should be avoided. It may be necessary only rarely if nipples are much excavated such making sucking impossible. It´ s unnecessary use leads to the short-term feeling of success but from a long-term view it usually causes insuficient progress of lactation and breastfeeding termination. 29 8. it is useful, if mother is taught in the maternity, how to express milk, most efficiently with her own fingers. Especially women during the first 2 weeks after delivery, who feel pressure or pain in breasts even after breastfeeding, should express their milk. Under special circumstances, breastfeeding may be contraindicated. See table 11. Table 11: Contraindication of breastfeeding drug-abused mother HIV-positive mother (only in developed countries) mother on cytostatic treatment mother with untreated active tbc child with galactosemia Probably more than in healthy newborns, breastfeeding is important in premature babies. For example, ofthe lethal complication necrotising enterocolitis is up to 6 times more frequent in formula-fed than in breastfed prematures. To achieve exclusive breastfeeding may be much more difficult in premature babies and requires a lot of efforts from mother and pediatric nurses. If cooperative staff is able to support mother, the achievement of high rate of success in exclusive breastfeeding is possible even in premature babies. Other practical aspects of newborn care in neonatal ward During the first day after birth blood sampling for screening of certain diseaes is performed. Presently, congenital hypothyreosis and phenylketonuria is screened. Both are congenital diseases which can be diagnosed soon after birth and due to early diagnosis and treatment the baby avoids permanent developmental deficit. Several drops of capillary blood are took up from baby´s heel, on special suctorial paper. Afterwards, dry drops of blood are sent to the central screening laboratory for evaluation. Optimal age for sampling is a little controversial. While hypothyreosis could be diagnosed immediately after birth, the sensitivity of phenylketonuria screening increases until about 6 days of age. Most usually, samples are drawn on day 4, or, if milk-feeding has been delayed, one or two days later. In future the spectrum of dieseases screened in neonatal period may widen. Every baby in the Czech Republic, except of babies with significantly lower birthweight, is vaccinated against tuberculosis (BCG vaccination) before discharge from maternity. The vaccination is performed intradermally, on left shoulder, dose 0,3 BM. Umbilicus must be kept clean after birth (wrapped in sterile gauze) and is usually cut on day 3 – 4. After this procedure, umbilicus should be closely observed for possible bleeding and later cleaned with desinfection until complete healing. Inflamation of umbilicus, omphalitis, manifests with reddening and swelling and may be dangerous. Central circulation is anatomically near to umbilicus and spread of the infection and sepsis is possible. Therefore, if above mentioned signs are found, early laboratory examination is necessary and systemic antibiotic treatment may come into consideration. Umbilicus is usually completely healed until the age of 2 weeks. In the Czech Republic, umbilicus is cut usually. Nevertheless, it is not necessary to perform this procedure. The alternative way is to discharge the baby with dry umbilicus left in place. It will fall away spontaneously after several days. 30 Statistics on national numbers of births and postnatal course and it´complications is important for physicians. In the Czech Republic, unified protocol called „Zpráva o novorozenci“ (Newborn report) is used. It serves as both discharge report and information for center for health-care statistics, institution held as a part of Ministry of health, which receives one copy. It depends on a helath status of the mother and baby, how long they stay in neonatal ward. In the Czech Republic, a discharge on about day is most usual. 4 days after delivery are usually enough to assess adequately normal postnatal adaptation, to be sure that lactation and breastfeeding initiates normally and umbilicus heals well. Also the baby is old enough for screenings and physician is usually sure, whether the intensity of jaundice does not progress in abnormal way. In some countries, newborns are discharged earlier, for instance 48 hours after birth, or even sooner. This approach is usually welcomed by families but is more demanding for pediatric care in the community and leads to increased rate of rehospitalizations. Introduction into the area of pathological newborn Although all organ functions of healthy newborn are fully developed and ready for extrauterine life, the development has not bee finished and that´s why newborn is more vulnerable to unfavourable influences. In the following text, some of more frequent pathological circumstances possibly associating neonatal period are discussed. Perinatal asphyxia was discussed above. It´s course and consequences may vary. Milder degrees of asphyxia, when a newborn resolves spontaneously or after short resuscitation, are more common. More rarely, serious asphyxia requiring several minutes of resuscitation and occassionally also following ventilatory support lasting hours or days until achievement of full cardiopulmonary adaptation occurs. Lethal course is possible in extremely serious cases. Birth trauma may occur under certain circumstances. Less serious are kefalhaematoma and fracture of clavicle. More serious may be brachial plexus palsy, caused by mechanical damage of nerve roots. There are two types, upper and lower. Upper type (Erb-Duchenne) involves motor activity of upper extremity apart from hand, lower type (Klumpke) involves fine hand motor activity. Upper type is more frequent. Characteristic feature is a flaccid extremity which stays in addcution to the body and prone position, without motion. Grasp and Moro reflexes cannot be elicited on involved side. The course uses to be favourable with gradual (weeks lasting) resolution of motor function. However, permanent palsy is also possible. Treatment consists of physical therapy and rehabilitation. Birth trauma may occur during and after delivery. Kefalhaematoma and fractrue of the clavicle are among less serious ones. Brachial plexus palsy may be more serious. It is causes by mechanical damage of nerve roots. Upper and lower type are known. Upper type (ErbDuchenne) deteriorates motor function of the upper extremity except of the hand, lower type (Klumpke) involves fine motor function of the hand. Upper type is more frequent: typicla manifestation is lack of motor function of the whole arm and forearm – the extremity stays in adduction to the trunk and prone position. Moro and grasp reflex cannot be elicited on the involved side. The course uses to be favourable with gradual (weeks) resolution. However, permanent damage is also possible. Treatment consists of physical therapy and rehabilitation. Long bone fracture (femur or humerus) may occur occassionally, requiring orthopedic treatment, but usually with good outcome. Infraction of skull may be caused by forceps delivery. During operative delivery (caesarean section) may occur skalpel skin incisions, occassionally requiring suture. Extraordinary (in term newborn) is intracranial haemorrhage cuased by birth injury, subarachnoidal, subdural or intraventricular, subcapsular liver haematoma or, in boys, contusion of genitalia or even torsion os testicle. 31 Problems of postmature newborn (postmaturity, dysmaturity), i.e. baby born after completed 42 weeks of gestation, are specific. It is not necessarily different from term baby, but sometimes, characteristic signs are present, as poor vernix covering of the skin, dry and peeling skin, increased birthweight. Nevertheless, postmaturity may also lead to fetal hypoxia: then, the fetus may become relatively hypotrofic and/or meconium may be found in amniotic fluid. Green coloration of the umbilicus is characteristic, too. Postmature babies are in higher risk of neonatal mortality, caused by hypoxia or meconium aspiration. Congenital developmental anomalies (malformations) may lead to various pathological states. In summary, congenital heart defects may cause serious symptoms during the first hours after birth (cyanosis, respiratory distress, heart failure), lungs or airways malformations may underlie disturbed adaptation of breathing, large congenital diphragmatic hernia may lead to serious adaptation disturbance. Also CNS malformations or congenital neuromuscular disorders may be reason for need of intensive care. The spectrum of congenital anomalies is wide and out of scope of this text. Neonatal jaundice exceeding above said features of normal physiological course, i.e. hyperbilirubinemia, is treated with phototherapy, or exceptionally with exchange transfusion. Congenital infection is dreaded complication of neonatal period. In nurseries with good standard of hygiene and aseptic measures group B Streptococcus (GBS) is presently the most prevalent etiologic factor of bacterial infections in term babies, while in premature ones the most common are Staphyloccoccus epidermidis. Also intestinal gramnegatives or other bacteria may be involved. Due to immature immunity, neonatal infection is usually not local one but generalised, i.e. progressing with clinical pattern of neonatal sepsis. Except of bacterial infections, the fetus may become infected by microorganisms of so called TORCH group (toxoplasmosis, rubeola, cytomegalovirus, herpes simplex), which may induce varying symptoms in newborn. Problems of infants of diabetic mothers were discussed above. Meconium aspiration is a serious complication. Preconditions are intrauterine or perinatal hypoxia causing preterm passage of meconium into the amniotic fluid. Then, risk of aspiration into the airways is significant during the first inspirations after birth. It leads to respiratory distress or even suffocation and respiratory failure. Postmature or hypotrophic babies are most commonly involved. It may be extremely difficult to stabilize the baby. Risk can be decreased with proper obstetric follow-up (early recognition of hypoxia and pregnancy termination) and readiness of the neonatologist to resuscitate. If solid pieces of black meconium are present in amniotic fluid during delivery, or if the fluid is dark and thick, it is necessary to start intensive suctioning of upper airways and stomach immediately after delivery of the head to avoid aspiration of the meconium. If aspiration occurs, one has to start resuscitation with suctioning as much of meconium as possible from upper airways and, if needed, also from trachea (after intubation), before positive pressure ventilation is commenced. Hypotrophia (small-for-date baby), refers to the birthweight under 3rd centile in specific gestational age. It is proportionate (head circumference is decreased proportionately to body weight) or disproportionate (head circumference is on higher centile than body weight). Hypotrophia of proportionate type is a result of long-lasting chronic hypoxia (insufficient nutrition) of the fetus in the womb and it is associated with a worse developmental outcome, because damage of brain development is more likely under these circumstances. On the other hand, hypotrophia of disproportionate type results from worsened nutrition of the fetus during the last weeks before delivery (the fetus „slims“), when there is a bigger chance that CNS has been saved. 32 Small-for-date baby´s adaptation is usually better than adaptation of normotrophic baby of the same birthweight, because it´s gestational age is higher, i.e. the baby is more mature. Furthermore, lung maturity is favourably influenced by intrauterine stress (maturation of adaptive mechanisms is accelerated. Also in these cases monitoring of glycemia is necessary, because the baby is more in risk of hypoglycemia, and to provide optimal thermal comfort. Persistent pulmonary hypertension of newborn (PPHN) is a specific circulatory disease out of the range of congenital heart defects. It is a functional problem resulting from intrauterine stress or perinatal asphyxia, or, sometimes occuring withou any apparent reason. It can be seen mostly in term or near-term babies. Underlying problem is a functional spasm or a morphological contraction (hypertrophy) of pulmonary vessels. As a consequence, resistance in pulmonary circulation does not decrease after delivery and does not allow normal lungs perfusion. With increased ventilation/perfusion rate part of blood continues to be shunted fromt the right to the left through foramen ovale, ductus arteriosus and intrapulmonal shunts and goes to the systemic circulation. Cyanosis, respiratory distress and possible serious or even lethal state may develop. Management consists of mechanical ventilation, oxygen administration and inhalation of NO (nitric oxide), which has capability of selective pulmonary arteries dilatation. Exceptionally, ECMO (extracorporeal membrane oxygenation) is used. Premature newborn Mild degree of prematurity (33 – 37th gestational week) is associated with no or mild problems of adaptation, for example periodic breathing, sometimes with apneas (apneic intervals longer than 20 seconds) or transient tachypnea of newborn (TTN, „wet lung“), i.e. temporary postnatal tachypnoe and/or mild dyspnea, disappearing during the first hours after birth, sometimes requiring short-term treatment with oxygen. Sometimes, commencement of feeding may be more difficult in prematures and achievement of exclusive breastfeeding may last several days longer and it may be preceded by period of partly feeding with expressed mother milk or parenteral nutrition. The weight fall may be deeper and last longer, peak of bilirubin level may be postponed and jaundice may persist until higher age (1-2 months). The full postnatal adaptation, allowing discharge to home, is usually achieved at latest about 36th postconceptional age. Last 3 decades brought radical changes in diagnostic and treatment possibilities of very prematurely born babies ( 32 completed gestational weeks or less). Fundamental pathophysiological problem of premature newborn´s adaptation with a lot of consequences is lung immaturity. The reasons of the latter are complex, but lack of lung surfactant is substantial. Surfactant is a substance which, under normal circumstances, equals surface tension of alveoli and it´s deficiency leads to RDS (respiratory distress syndrome, hyaline membranes disease,HMD). It is characterised by development of multiple small atelectasis, i.e. patial lung collaps and decreased aeration. Clinically, the disease manifests by dyspnea (jugulum, intercostal spaces and epigastrium retractions), grunting (typical sound originating when exspired air passes the narrowed place under partially closed epiglottis), increased oxygen demand and gradual development of respiratory failure. X-ray pattern is typical. See picture 4. Management of RDS consists of oxygen administration and, if needed, ventilatory support, together with parenteral fluids and nutrition. Ventilatory support is either continuous positive pressure (CPAP) or artificial ventilation with respirator. Intratracheal instillation of comercially produced surfactant, repeated as needed, is used routinely as a causative treatment of RDS, which can bridge the period until sufficient production of endogenous surfactant (usually not more than several days). Mechanical ventilation may be required for several hours to several weeks. 33 A lot of complications may be associated with RDS, which are also more or less specific for premature babies. They include persistent ductus arteriosus, intraventricular and periventricular haemorrhage into the brain, retinopathy of prematurity, acute necrosis of part of intestinal wall called necrotising enterocolitis, and, regarding pulmonary pathogenesis, RDS may be followed by long-term pulmonary changes called bronchopulmonary dysplasia BPD). Problems of RDS and it´s complications and other diseases asociated with premature delivery, as well as problems of term ill newborn, are objects of subspecialty neonatal intensive care. Long-term development of premature babies Enormous increase of survival of very- and extremely-low-birth-weight infants raises the question about their chances for good quality of life. Therefore, recently the quality of newborn care is not only appraised along the survival statistics. More crucial are data on longterm development of premature babies. So, every hospital providing high-degree newborn care should have it´s own programme of long-term follow-up. There are efforts in developed countries to collect these data on national basis. Premature or ill newborn is exposed to increased risk of CNS and other organs damage in postnatal period. That´s why especially among premature children higher rate of those with long-term handicaps is found. The defects include motor (cerebral palsy), cognitive (lower developmental quotient or IQ) or sensoric ones (deficit of hearing or vision). Risk of long-term handicap is the higher, the lower is the birthweight and gestational week. In the group of babies with birthweight under 1500g the risk of serious handicap is about 5 – 7%, in the group under 1000g the risk is about 20%. The probability of serious long-term handicap in relation to gestational age at birth see in talbe 12: Table 12: Schema, how to remember risk of serious developmental deficit in premature babies along with gestational age at birth. g.w. survive at (% of all birth born alive) 30 23 40 24 50 25 60 26 70 27 80 28 90 29 > 95 30 and more (g.t. = gestační týden) without severe handicap (% of all surviving) 30 40 50 60 70 80 90 > 95 34 Sudden infant death syndrome Sudden infant death syndrome ( SIDS, „cot death“) affects infants during the first year of life, but usually (95%) during the first 6 months, mostly at home. Thus, this is not primarily neonatal problem. However, parents of all babies should be informed about SIDS and possibilities of it´s prevention at latest after birth of a baby and knowledge of the problem is important for all neonatologists as well as community pediatricians. SIDS is a situation, when until then absolutely healthy infant is found dead in his/her bed in the reason is not enlightened even with autopsy. Thera are lots of theories about possible reasons (peracute infection, inborn dysrhytmia, unrecognizde inborn metabolic error with hypoglycemia and others). Prevalence of the syndrom in developed countries is about 1 : 1000 of all born alive. Aboslutely reliable prevention is not known. Nevertheless, several risk factors were identified, which increase the risk of SIDS. They include smoking of the mother during pregnancy and smoking in the family generally, early introducing of formula instead of breastfeeding, sleeping in the other room than where parents are sleeping, sleeping in the same bed with parents and sleeping in the prone position. Campaigns providing families with need to avoid these risk factors the decreased incidence of SIDS was achieved in some countries. So, parents should be provided by pediatrician with these advises: avoid smoking in the environment, where the baby lives do your best to breastfeed your baby exclusively until 6 months of age when the baby is sleeping unattended, put him to his/her own bed close to your bed, and positioned on his/her back Some used and recommended sources: Pediatric Clinical Skills. Schaffer, Avery: Diseases of the Newborn.( Saunders) Fanaroff, Martin: Neonatal-Perinatal Medicine.(Mosby) Sinclair, Bracken: Effective care of the newborn infant.(Oxford University Press) Royal College of Midwives: Succesfull Breastfeeding. (Churchill Livingstone) Nelson: Textbook of pediatrics. (Saunders) American Academy of Pediatrics, Work Group on Breastfeeding: Breastfeeding and the use of human milk. (Pediatrics 6/97, str. 1035-1039) International Guidelines for Neonatal Resuscitation: An Excerpt From the Guidelines 2000 for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care: International Consensus on Science. (Pediatrics 3/2000, str. e29) Postup resuscitace na videokazetě „Péče o novorozence I.“ (vyd. Centrum podpory zdraví, k dispozici ve videopůjčovně Státního zdravotního ústavu, Praha 10, Šrobárova 48, tel. 02 6708 2986). Avery, Fletcher: Neonatology. (Lippincott comp.) 35