Surgery Board Review
advertisement
March 29, 2011 SURGERY BOARD REVIEW RESPIRATORY DISTRESS UPPER AIRWAY Attempt to pass NG tube Esophageal atresia Choanal atresia Half have other congenital anomaly CHARGE Syndrome Coloboma Heart Atresia of choanae Retarded development Genital hypoplasia Ear CHOANAL ATRESIA Membranous 90% Bony 10% Gavage feed until repair Maintain oral airway MACROGLOSSIA True enlargement of tongue Vs. Glossoptosis in which normal tongue protrudes from abnormally small oral cavity Focal (pebbly, vescicle-like blebs) Congenital tumors (lymphangioma, hemangioma) One-half Hemihypertrophy MACROGLOSSIA Generalized (smooth surface) Beckwith-Wiedemann, Generalized (papillary, fissured) Down syndrome Also hypothyroidism poor tone Progressive Mucopolysaccharidoses QUESTION 1 This child was most likely to have which of the following metabolic abnormalities as a newborn? A Hyperkalemia B Hypokalemia C Hyponatremia D Hypoglycemia E Metabolic Acidosis BECKWITH WIEDEMANN SYN Features Macroglossia Macrosomia Hypoglycemia (islet cell hyperplasia) Hemihypertrophy Hypospadias Omphalocele 18 month old with B.W. presents with gross hematuria: Wilms Tumor PIERRE ROBIN SEQUENCE Unusually small mandible (micrognathia) Posteriorly displaced tongue Upper airway obstruction U-shaped cleft palate May be part of a larger syndrome LARYNGEAL LESIONS Hoarseness Faint cry Aphonia Associated dyspnea THORACIC LESIONS QUESTION 2 Which is the most common abnormality? A B C D E 2 1 TE fistulae: Most common types… 1 Esophageal atresia and distal fistula 2 Esophageal atresia and no fistula 3 H-Type (only one without atresia) Manifestation may not be as severe 3 TE FISTULA Inability to pass NG tube Use to drain secretions Resp distress secondary Fistula Reflux of pouch contents Inability to tolerate initial feed Excessive drooling May see distension of stomach (air fistula) Avoid positive pressure ventilation Antenatal U/S: Microgastria, polyhydramnios PURE ESOPHAGEAL ATRESIA Dilated proximal pouch Gasless abdomen Scaphoid abdomen BRONCHOPULMONARY FOREGUT MALFORMATION BRONCHOPULMONARY FOREGUT MALFORMATION Congenital Lobar Emphysema Congenital cystic adenomatoid malformation Pulmonary sequestration Bronchogenic cyst PULMONARY SEQUESTRATION Portion of lungs perfused by systemic arteries Lacks normal connection with tracheobronchial tree Intralobar: Most common lower lobes Anywhere in thorax Extralobar: Subdiaphragmatic or retroperitoneal Associated with other anomalies Dx: MRI or angiogram PULMONARY SEQUESTRATION BRONCHOGENIC CYST Occur any point along the tracheobronchial tree Centrally located Does not communicate, fluid fill with wall composed of tissue resembling large airways Typically present 2nd decade of life with recurrent wheezing, cough, pneumonia BRONCHOGENIC CYST CONGENITAL PULMONARY AIRWAY MALFORMATION (CPAM) Previously known as Congenital Cystic Adenomatoid Malformation (CCAM) Hamartomatous lesion Cystic and adenomatous elements Resp distress, recurrent PNA, asymptomatic Severe forms can be lethal after birth Surgical resection if symptomatic Controversial if asymptomatic CONGENITAL PULMONARY AIRWAY MALFORMATION (CPAM) CONGENITAL LOBAR EMPHYSEMA Results from area of poorly developed or absent cartilage Check-valve effect Air trapping Lung hyperexpansion Possible acute decompensation with positive pressure ventilation GI OBSTRUCTION GI OBSTRUCTION Bilious emesis is always considered a red flag for mechanical obstruction. Surgical emergency until proven otherwise NONBILIOUS EMESIS IN INFANTS Most common cause is overfeeding Second most common: GE Reflux HYPERTROPHIC PYLORIC STENOSIS Fairly common 1:300 Unknown genetic link Vomiting starts by 2wks old Progressively projectile Age at presentation Most commonly b/f 1 month Remember: 2wks-2months Male predominance Firstborn PYLORIC STENOSIS Clinical Manifestations: Abdomen probably will not be distended Can feed them to look for signs Distended over stomach immediately after feeding Peristaltic waves Palpable olive After vomiting Epigastric b/w midline and R midclavicular line PYLORIC STENOSIS Diagnosis Palpable Olive (no imaging required) Hypochloremic, hypokalemic metabolic alkalosis Ultrasonography Pyloric muscle Thickness 3-4mm Length 15-16mm DIAGNOSIS Upper GI the "string" sign the "double-track" sign the "beak" sign the "shoulder" sign PYLORIC STENOSIS Surgical treatment (pyloromyotomy) Fluid and electrolyte correction are most important Surgery after these are corrected QUESTION 3 You are working up a 4 month old infant with bilious vomiting. Findings on barium enema reveal cecum located in left upper abdomen. The most likely diagnosis is: A B C D situs inversus malrotation intussusception intestinal duplication BILIOUS VOMITING IN INFANT Malrotation with midgut volvulus MALROTATION WITH MIDGUT VOLVULUS Proximal obstruction at distal duodenum/ proximal jejunum Bilious emesis May not have significant abdominal distention until late Late finding: hematemesis, hematochezia Bowel ischemia leading to perforation and death MALROTATION WITH MIDGUT VOLVULUS Diagnosis Suspect in setting of: Bilious vomiting in infant (or older child) Abdominal films: Gastric distention and duodenal dilatation Otherwise gasless abdomen MALROTATION Surgical correction Ladd Procedure Division of Ladd bands Appendectomy Widening of mesentery Securing intestines in position of nonrotation DUODENAL ATRESIA Bilious vomiting shortly after birth May occur later Annular pancreas Duodenal stenosis Duodenal web Ladd bands Associated with Down Syn and congenital heart disease in 30-50% MECONIUM ILEUS 20% of patients with CF Meconium ileus is CF until proven otherwise Intraluminal obstruction by thickened meconium Distal ileum is small, may have microcolon Possible proximal bowel dilitation “soap bubble” appearance MECONIUM ILEUS Contrast enema (may be temporarily therapeutic) Microcolon Inspissated meconium “rabbit pellets” May perforate Intraperitoneal calcifications and ascites prenatally QUESTION 4 You are seeing a new 9 month old who has had difficultly stooling since birth. You see in the records that this imaging has recently been performed • Which information is FALSE? • A Rectal suction biopsy should be done • B Identification of transition zone is helpful • C Development of explosive diarrhea fits with diagnosis • D Barium enema should be performed following GI cleanout HIRSCHSPRUNG DISEASE Absence of ganglion cells in distal rectum Bilious emesis and abdominal distention BE: proximally dilated bowel (normal) Transition zone Distal decompressed segment (abnormal) Barium study should preceed rectal exam or enemas Definitive: rectal suction biopsy HIRSCHSPRUNG DISEASE May be diagnosed in older child with chronic constipation Requiring rectal stimulation to evacuate Lifethreatening hirschsprung-related enterocolitis Explosive diarrhea Toxic megacolon Sepsis INTUSSUSCEPTION 6mos-2yrs Older children more likely to have lead point Meckel Polyp Lymphoma Intestinal duplication cyst Hematoma/inflammation Hemophilia, HSP INTUSSUSCEPTION Classic location: ileocolic (may be ileo-ileal if there is a lead point) Presentation Paroxysmal bouts of severe colicky abdominal pain Drawing up legs Period of somnolence Normal activity between episodes Unexplained lethargy or seizure-like episode INTUSSUSCEPTION Emesis may turn bilious late in course Current jelly stool: late finding “sausage-shaped mass” RUQ INTUSSUSCEPTION Evaluation: Start with plain films Crescent sign, target sign If suspicion high, can go straight to air contrast or barium enema Diagnostic Emergent and therpeutic surgery if enema unsuccessful May recur in first 12 to 24 hrs GI BLEEDING NEWBORN Swallowed maternal blood Apt-downey test Mix serum with sodium hydroxide Pink: newborn Brown: swallowed maternal blood Vitamin K Anal stenosis (fissures) Hirschsprung Severe gastritis or AGE NEC NEC Acute illness of unclear etiology associated with intestinal necrosis 1-3/1000 live births Incidence decreases with increasing gestational age 13% occur in term infants Inflammation Invasion of enteric gas forming organisms Dissection of gas into the muscularis and portal venous system Preexisting illness is common Mortality rate 20-30% NEC Presentation Nonspecific systemic signs in a fed, well infant Apnea Respiratory failure Lethargy Poor feeding Temp instability Abdominal signs Distention Gastric retention Tenderness Vomiting Diarrhea Gross bleeding NEC Diagnosis Abdominal distention Rectal bleeding Abdominal radiographs Early - abnormal gas pattern with dilated loops c/w ileus Pneumatosis intestinalis Portal air: late finding If perforation suspected Bubbles of gas in the bowel wall Cross-table lateral Left lateral decubitus Sentinel loop Abdominal ultrasound NEC Diagnosis Nonspecific Blood studies CBC Coags Chemistries Stool analysis Occult blood Sepsis DIC evaluation 20-30% NEC Management Supportive care Bowel rest and decompression with intermittent suction TPN Fluid replacement CV support Resp support Hematologic support Metabolic support Surgical intervention Reserved for perforation, obstruction NEC Management Antibiotic therapy Vanc, gent, clinda Vanc, gent, flagyl Vanc, gent, zosyn Term infants Amp, gent and clinda 10-14 day course Unless complication TODDLER GI BLEEDING Painless rectal bleeding Meckel diverticulum Can have massive bleeding Dx: technetium-99m pertechnetate isotope scan False negatives possible May present with complications: Volvulus Internal hernia Intussusception Acute inflammation (similar to appendicitis) ESOPHAGEAL VARICES Massive upper GI bleeding Portal HTN Portal vein thrombosis h/o UVC Hepatic cirrhosis Chronic liver failure TPN-related liver disease Biliary atresia s/p Kasai Cystic fibrosis a1- antitrypsin deficiency Viral hepatitis ESOPHAGEAL VARICES Treatment Endoscopy Direct visualization Banding Sclerosant techniques Transjugular intracaval portosystemic shunting DON’T FORGET HSP (Henoch-Schonlein Purpura) Vasculitic conditions Arthralgias Palpable purpuric rash Buttocks Lower extremities NORMAL CBC HUS (Hemolytic Uremic Syndrome) Renal failure, anemia, thrombocytopenia Very sick!! OLDER CHILDREN Benign hamartomatous juvenile polyps Age 2-8 Painless, low volume, streaks on stool Autonecrosis and slough into stool May prolapse through anus Surgical or endoscopic resection is therapeutic QUESTION 5 This 10 y/o child has had recurrent lower GI bleeding. What is your diagnosis? A B C D Peutz-Jeghers Familial adenomatous polyposis Gardner’s Syndrome Hereditary Hemorrhagic Telangectasias FAMILIAL POLYP DISORDERS Gardner’s Polyps of small and large bowel: premalignant Extra teeth Osteomas AD inheritance Surgical resection Peutz-Jeghers Hamartomatous polyps: premalignant Melanotic mucocutaneous pigmentation lips, gums AD inheritance Surgical resection DON’T FORGET Maintain high index of suspicion for appendicitis Rare in toddlers, but 75% rupture prior to dx Don’t expect them to fit classic picture Obturator and psoas signs may help In Children, classic features (pain followed by vomiting, fever, anorexia, migration to RLQ, rebound) neither sensitive nor specific Lack of migration to RLQ in 50% Absence of anorexia 40% No rebound tenderness 52% DON’T FORGET Imitators of appendicitis Yersinia Mesenteric adenitis Sickle crisis Caution in adolescent female!!! Other causes of abdominal pain: Lower DKA lobe pneumonia ABDOMINAL MASSES ABDOMINAL MASSES What is it? Age Location Characteristics Firm Cystic Mobile Fixed Initial diagnostic studies History usually no significance ABDOMINAL MASSES Location Most important factor Characteristics Cystic Solid Usually benign Predominantly malignant Age Second most important factor ABDOMINAL MASSES Work up Plain films Displacement Obstruction Calcification Ultrasound Initial diagnostic tool of choice Solid vs cystic ABDOMINAL MASSES Work up CT Study of choice Extent of local disease Sites of distant metastases Character of the lesion Contrast MRI Careful analysis of abdominal and pelvic structures QUESTION 6 On examination of a newborn female, you palpate an abdominal mass. Ultrasound reveals a 6cm ovarian cyst. What should you tell these anxious parents? A. Most ovarian cysts resolve spontaneously and therefore no treatment is needed B. Large cysts >5cm are at risk for torsion and should be aspirated C. These are very rare and have a high risk for malignant transformation D. They are caused by hormones produced by the fetus in utero
