PYLORIC STENOSIS Supervised By: Professor Doctor Ali Sultan Professor of Pediatric surgery Prepared By: Aser Gomaa 158 Usama Farid 156 (Presentation & Examination) Osama Abo-Reda 157 (Definition & Theories) (Differential diagnosis) Osama Hamed 155 (Investigation) Areej Youssry 152 (Treatment) Definition: Is a developmental narrowing of the pylorus, due to hypertrophy of the muscle surrounding the pylorus. Normally, food passes easily from the stomach into the duodenum through the pylorus. In pyloric stenosis, the muscles of the pylorus are thickened. This prevents the stomach from emptying into the small intestine. Synonyms: Hypertrophic pyloric stenosis, Gastric outlet obstruction Incidence: *2-4:1000 Live birth *The disease is more common in male infants *Male : female = 4:1 *The disease runs in families *Most commonly if first baby *If the parent had it there is a strong possibility that his son will have it *It is more common in the first 4-6 weeks *It rarely presents beyond 6 month Pathologically: 1.Marked hypertrophy of circular muscles. 2.The mucosa is compressed and folded. 3.Narrow lumen. 4.Stomach muscles hypertrophy 5.Rugae hypertrophy with gastritis and ulcers Aetiology: The exact cause of hypertrophic pyloric is still unkown. Many theories has been postulated: 1.Defenitive genetic factors,plays a very important role as it runs in families. Common variants near MBNL1 and NKX2-5 are associated with infantile hypertrophic pyloric stenosis. 2.Ganglion cell,ganglion cells in the region of the pyloric canal are responsible for relaxation,there is immaturity of these cells that leads to failure in relaxation. 3.Nitric oxide synthetase deficiency, Nitric oxide (NO), produced by the neural nitric oxide synthase enzyme (nNOS) is a transmitter of inhibitory neurons supplying the muscle of the gastrointestinal tract. Transmission from these neurons is necessary for sphincter relaxation that allows the passage of gut contents, and also for relaxation of muscle during gastric contraction. There are deficiencies of transmission from NOS neurons in pylorus 4.Hyperacidity 5.Gastrin and other gastrointestinal hormones 6.H.Pylori association. The most widely accepted so far is the Ganglion cell theory. History: Typical presentation of an infant with hypertrophic pyloric stenosis (HPS) is onset of initially nonbloody, always nonbilious vomiting at 4-8 weeks. Although vomiting may initially be infrequent, over several days it becomes more predictable, occurring at nearly every feeding. Vomiting intensity also increases until pathognomonic projectile vomiting ensues. Slight hematemesis of either bright red flecks or a coffeeground appearance is sometimes observed. Patients are usually not ill-looking or febrile. The baby in the early stage of the disease remains hungry and sucks vigorously after episodes of vomiting. Prolonged delay in diagnosis can lead to dehydration, poor weight gain, malnutrition, metabolic alterations, and lethargy. Parents often report trying several different baby formulas because they (or their physicians) assume vomiting is due to intolerance. Physical examination: Careful physical examination provides a definitive diagnosis for most infants with hypertrophic pyloric stenosis. However, some of the classic signs that would lead to diagnosis may be absent due, in part, to the early diagnosis of hypertrophic pyloric stenosis. An enlarged pylorus, classically described as an "olive," can be palpated in the right upper quadrant or epigastrium of the abdomen in 60-80% of infants. In order to assess the pylorus, the patient must be calm and cooperative. A pacifier or small amount of dextrose water may help. If the stomach is distended, aspiration using a nasogastric tube is necessary. With the infant supine and the examiner on the child's left side, gently palpate the liver edge near the xiphoid process. Then displace the liver superiorly; downward palpation should reveal the pyloric olive just on or to the right of the midline. To be assured of the diagnosis, the physician should be able to roll the pylorus beneath the examining finger. The tumor (mass) is best felt after vomiting or during, or at the end of, feeding. The diagnosis is easily made if the presenting clinical features are typical, with projectile vomiting, visible peristalsis, and a palpable pyloric tumor. When diagnosis is delayed, the infant may develop severe constipation associated with signs of dehydration, malnutrition, lethargy, and shock. Differential diagnosis of congenital hypertrophic pyloric stenosis: Adrenal Insufficiency Bowel Obstruction in the Newborn Duodenal Atresia Failure to Thrive Gastroenteritis Pyloric membrane or pyloric duplication Gastroesophageal Reflux Intestinal Malrotation Inborn errors of metabolism * Gastric waves are occasionally visible in small, emaciated infants who do not have pyloric stenosis. Infrequently, gastroesophageal reflux, with or without a hiatal hernia, may be confused with pyloric stenosis. Gastroesophageal reflux disease can be differentiated from pyloric stenosis by radiographic studies. Adrenal insufficiency from the adrenogenital syndrome can simulate pyloric stenosis, but the absence of a metabolic alkalosis and elevated serum potassium and urinary sodium concentrations of adrenal insufficiency aid in differentiation. Inborn errors of metabolism can produce recurrent emesis with alkalosis (urea cycle) or acidosis (organic acidemia) and lethargy, coma, or seizures. Vomiting with diarrhea suggests gastroenteritis, but patients with pyloric stenosis occasionally have diarrhea. Rarely, a pyloric membrane or pyloric duplication results in projectile vomiting, visible peristalsis, and, in the case of a duplication, a palpable mass. Duodenal stenosis proximal to the ampulla of Vater results in the clinical features of pyloric stenosis but can be differentiated by the presence of a pyloric mass on physical examination or ultrasonography. Investigations for Infantile Hypertrophic Pyloric Stenosis: It is clear that for a diagnostic study to become the imaging modality of choice, it must be demonstrated to be, above all, accurate; further, the procedure should be noninvasive and should be able to be performed quickly so that results will be available immediately, without delay in diagnosis. The examination must allow unequivocal differentiation between normal and abnormal conditions. At present, there are two valid and successful methods for the imaging diagnosis of IHPS, the UGI and sonography. UGI Studies Abnormal study.—In patients with IHPS, there is failure of relaxation of the prepyloric antrum, typically described as “elongation” of the pyloric canal. The canal is outlined by a string of contrast material coursing through the mucosal interstices, termed the string sign; or by several linear tracts of contrast material separated by the intervening mucosa. The latter is termed the doubletrack sign. This sign demonstrates the intervening redundant mucosa outlined as a filling defect by the contrast material; it was reported as specific for IHPS by Haran et al in 1966 and, therefore, may aid in differentiation from pylorospasm. Interestingly, the fact that the redundant mucosa in the canal is responsible for the filling defect between the channels of contrast material was not recognized by the authors in the illustration included in the original article. UGI is performed with the infant in the right anterior oblique position, to facilitate gastric emptying. The examination can be successfully accomplished with the child drinking from a bottle; these infants are usually very hungry and will drink with little effort. Insertion of a nasogastric tube is not necessary; however, emptying of an overdistended stomach may help to prevent vomiting, if needed. Fluoroscopic observations include vigorous active peristalsis resembling a caterpillar and coming to an abrupt stop at the pyloric antrum, outlining the external thickened muscle as an extrinsic impression, termed the shoulder sign. Luminal barium may be transiently trapped between the peristaltic wave and the muscle, and this is termed the tit sign Sonography Equipment.—The examination should be performed with high-frequency transducers. We use a linear transducer operating between 6 and 10 MHz, adjusted to the size of the infant and the depth of the pylorus. Because the pylorus rises anteriorly with positioning of the patient, the transducer frequency may be adjusted for greater detail while achieving adequate penetration to the pyloric channel. A sector transducer may be helpful in certain cases. Abnormal study.—Sonographic examination demonstrates the thickened prepyloric antrum bridging the duodenal bulb and distended stomach. In infants with IHPS, the stomach is distended to a variable degree. This gastric distention in a vomiting infant is the first sign available to the examiner that there is a gastric outlet obstruction. Positioning of the patient by using gravity allows the examination to proceed, with the focus on identifying the duodenal cap and its connection to the stomach. Because the variable gastric distension is likely to displace the gastroduodenal junction, the search for the pylorus may prove difficult. It is, therefore, best to begin at a specific site of constant location. We begin by placing the transducer transversely below the xiphoid process, identifying the esophagus as it enters the abdomen anterior to the aorta at the diaphragmatic crus. Caudal movement of the transducer allows identification of the gastric fundus, and continued caudal motion subsequently allows definition of the gastric body, antrum, and duodenal cap, regardless of displacement or position and whether or not IHPS is present. In patients with IHPS, the muscle is hypertrophied to a variable degree, and the intervening mucosa is crowded, thickened to a variable degree, and protrudes into the distended portion of the antrum (the nipple sign) and can be seen filling the lumen on transverse sections .The length of the hypertrophied canal is variable and may range from as little as 14 mm to more than 20 mm. The numeric value for the lower limit of muscle thickness has varied in reports in the literature, ranging between 3.0 and 4.5 mm. In our experience and in that of others . the actual numeric value is less important than the overall morphology of the canal and the real-time observations. The antropyloric canal, as part of the stomach, is a dynamic structure, and it is seen undergoing changes in both length and width during many examinations . The intervening mucosa can often be observed sliding within the canal, usually as a wave of gastric peristalsis washes over the hypertrophied channel. Ressucitation and Preoperative evaluation: Vomiting of gastric contents leads to depletion of sodium,potassium, and hydrochloric acid, which results in hypokalemic, hypochloremic metabolic alkalosis. The kidneys conserve sodium at the expense of hydrogen ions,leading to paradoxical aciduria. In more severe dehydration,renal potassium losses are also accelerated owing to an attempt to retain fluid and sodium. The degree of dehydration can be estimated by clinical examination, urine output, and serum chloride and bicarbonate levels. Resuscitation state is determined by serum electrolytes, skin turgor, moist mucous membranes, and urine output. Serum bicarbonate less than 28 mEq/dL and serum chloride over 100 mEq/dLare generally required for safe anesthesia. TREATMENT Pre-operative Workup and Preparation: -Correction of electrolyte imbalance – infants with pyloric stenosis and vomiting typically have derangements of serum electolytes such as low potassium and magnesium. -Correction of fluid balance –.Infants with electrolyte abnormalities or dehydration require correction of both. Due to the severity of the dehydration, these infants are typically resuscitated with twice the maintenance volume of normal saline solution until they void. Then, potassium is added to the intravenous fluids, which are changed to half-normal saline at 1.5 times maintenance. It may take 48 hours or longer to fully resuscitate an infant and prepare them for surgery. Lactated Ringers Solution is not to be used as an initial resuscitation fluid. Nasogastric tubes should be avoided as they further deplete electrolytes. - -Placement of nasogastric tube – after the diagnosis the infant oral feeds for the infant are suspended. Although prolonged placement of a nasogastric tube is to be avoided as they further deplete electrolytes , sometimes 6-12 hours is needed to prevent further vomiting. -Infants with less than 5% dehydration and no electrolyte imbalance are candidates for surgery without delay -Once an infant is resuscitated, the pyloric stenosis is treated surgically. Operation: Ramstedt Pyloromyotomy Types of surgery Two methods of surgery are used to correct pyloric stenosis—open surgery and laparoscopic surgery. Pyloromyotomy may be performed either as an open procedure, via a right-upper-quadrant horizontal incision or an umbilical incision . Operation is done under general anesthesia.The ring of muscle (pyloric sphincter) is then cut to widen the channel between the stomach and the intestine. During laparoscopic surgery, The laparoscope provides access to the pyloric muscle so the muscle can be cut(seromyotomy), the laparoscopic pyloromyotomy is performed using a peri-umbilical telescope and two stab wounds, one for a grasper and the other for the retractable blade and the pyloric spreader. The surgeon has the choice os whether to grasp the duodenum and incise from duodenum toward the stomach, or to grasp the stomach and incise from the stomach toward the duodenum. Either way, the pyloric muscle is spread and the adequacy of the operation verified Several other small incisions are usually needed.Compared to the open techniques, Recovery from the laparoscopic procedure is quicker than is recovery from a traditional open surgery, and the procedure leaves a smaller scar. -Technique of Balloon dilation does not work as well as surgery, but may be considered for infants when the risk of general anesthesia is high Surgical Details of the open surgery Procedure: 1. A small 3 cm incision is made in the skin with a No. 15 blade just below the right costal margin (the right rib cage) on the anterior abdominal wall, but above the inferior edge of the liver. 2. Care must be taken to place the incision so that it extends laterally from the outer edge of the rectus muscle. 3. Dissection is done through the subcutaneous tissues with Bovie cautery. 4. The muscle layer is carefully divided using Bovie cautery ,with the omentum or transverse colon presenting into the wound. 5. Using very gentle traction on the omentum, the transverse colon if not already visualized through the wound can be presented up into the wound. 6. Gentle traction on the transverse colon will then deliver the greater curvature of the stomach up into the wound. 7. The anterior wall of the stomach is grasped with a moist sponge and gentle traction on the stomach antrum is applied – this will deliver the pylorus into the wound. 8. The avascular (without blood supply) portion of the anterior wall of the pylorus is identified. 9. The pylorus is held between the surgeons thumb and forefinger and a 1-2 cm longitudinal incision (along the plane of the pylorus) is made 10. The incision is taken down through the serosal and muscle layers until the mucosa is exposed 11. Great care must be taken not to incise the mucosa. Extra attention must be given to the duodenal end of the incision as the muscle layer ends abruptly. - In the event of a duodenal perforation,or if mucosa incised .the perforation may be closed primarily or Pylorus sphincter closed and a new pyloromyotomy performed . 12. The incised (cut) muscle is gently spread apart with a hemostat until the mucosa “bulges up” to the level of the cut serosa. 13. The peritoneum and fascia of the transversalis muscle is closed with a running absorbable suture 14. The remaining fascial layers are closed with either running or interrupted slowly absorbable sutures 15. The skin is closed with a subcuticular absorbable suture such as Monocryl and adhesive Steri-strips are placed on the wound. -Possible surgical complications of Pyloromyotomy may include: Perforation of the duodenum Persistent vomiting due to persistent gastritis,inadequate operation. Incisional hernia Adverse reaction to anesthesia dt uncorrected electrolyte imbalance. Bleeding Infection,Scarring -Although pyloromyotomy is safe and curative and performed virtually without operative mortality (< 0.5%) and morbidity (< 10%), it is not without potential complications. Potential intraoperative and postoperative complications include bleeding, perforation, and wound infection. Duodenal or gastric perforation, the most serious complication, rarely occurs; however, if it goes unrecognized before wound closure, devastating or lethal consequences are possible. The infant with an enteric leak develops pain, distention, fever, and peritonitis. Ongoing fluid requirements, generalized sepsis, vascular collapse, and death follow if the enteric leak is not recognized and treated. Suspected perforation postoperatively requires immediate reexploration. Recognition of this complication at the time of surgery is important. Mucosal perforation most commonly results from extending the myotomy beyond the pyloric-duodenal junction. If perforation occurs, the mucosal defect should be repaired and the myotomy completed. An omental patch may be sutured to the perforation site, and a paraduodenal drain may be considered.. Bleeding is a rare complication of pyloromyotomy. Other complications that are more common but less serious include superficial wound infections and postoperative vomiting. Patients with wound erythema, drainage, or both undergo wound opening and debridement and antibiotic therapy. Incomplete myotomy results in ongoing gastric outlet obstruction and requires reoperation. Patients with prolonged preoperative obstruction develop gastric distention and dysmotility, which may cause postoperative emesis for up to 1 week after an adequate pyloromyotomy. -Postoperative : -Crystalloid resuscitation is continued postoperatively until the patient returns to full feeding. Recent data suggest that infants with pyloric stenosis have an increased incidence of postoperative apnea and bradycardia. These infants should be placed on an apnea and cardiac monitor for 24 hours following the operation. -Pain: Intraoperative, infant will receive a pain medication that is injected into the incision.. If necessary, acetaminophen may also be given to help ease discomfort. Slow feeding and gentle burping help prevent wet burps postoperatively, infant can tolerate normal feedings usually one to two days after surgery.Intermittent vomiting persisting through the first postoperative week is sometimes observed in patients with a protracted course of emesis and severe dehydration preoperatively. Although infant often vomits for 24 to 48 hours after surgery, this usually disappears without any further treatment. Occasionally, however, vomiting may persist for 4 to 5 days. Vomiting lasting longer than 7 days postoperatively should alert the physician to the possibility of an incomplete pyloromyotomy. -Incision : incision should be kept clean and dry, and no tub baths should be given for one week after surgery. Steri-strips (bandage-like tape) that are placed over the incision should be left in place and then removed. They are generally left in place for 7 to 10 days. References: 1. Elinoff JM, Liu D, Guandalini S, Waggoner DJ. Familial pyloric stenosis associated with developmental delays. J Pediatr Gastroenterol Nutr. Jul 2005;41(1):129-32. 2. Chung E. Infantile hypertrophic pyloric stenosis: genes and environment. Arch Dis Child. Dec 2008;93(12):1003-4. 3. Kawahara H, Takama Y, Yoshida H, et al. Medical treatment of infantile hypertrophic pyloric stenosis: should we always slice the "olive"?. J Pediatr Surg. Dec 2005;40(12):1848-51. 4. Panteli C. New insights into the pathogenesis of infantile pyloric stenosis. Pediatr Surg Int. Dec 2009;25(12):104352. 5. Alain JL, Grousseau D, Terrier G. Extramucosal pyloromyotomy by laparoscopy. Surg Endosc. 1991;5(4):174-5. 6. Siddiqui S, Heidel RE, Angel CA, Kennedy AP Jr. Pyloromyotomy: randomized control trial of laparoscopic vs open technique. J Pediatr Surg. Jan 2012;47(1):93-8.