* Within 8 hours after birth, it is noted that a baby has excessive salivation. Answer is esophageal atresia, likely tracheoesophageal fistula. Could also say there is a choking episode with first feeding. A small soft nasogastric tube is inserted and the child is taken to x-ray to have a “babygram” done; called a babygram because the child is the size of the x-ray plate so they shield the gonads and do an x-ray of the entire baby. Film shows the NG tube coiled back upon itself in the upper chest. There is air in the GI tract. Answer is blind pouch at upper end of the esophagus and a fistula between tracheoesophageal tree and lower fistula. Answer for next step is not immediate operation. This is one sign of a constellation of symptoms that exist, so we must rule out the others. Mnemonic is VACTRR, vertebral anomalies, anal anomalies, cardiac anomalies, tracheoesophageal fistula, and renal and radial anomalies. So next step is examine radial bones, examine vertebrae, examine for anal imperforation, sonogram, and echocardiogram. If no other anomalies preventing fistula prevention, repair is done at first day of life. If other anomalies exist, we place a gastrostomy tube.
* A newborn baby is found on physical exam to have an imperforate anus. Answer is not go to OR. Answer for next step is insert a soft NG tube, babygram to see if tube is in stomach, sonogram for renal anomalies, echocardiogram for heart, x-ray for bony anomalies. If no other anomalies, is there an anal fistula nearby. If a fistula exists such as to the vagina, we do not need emergency surgery. If no fistula exists, we need to know how high up the blind pouch is. This is done via x-ray with the child upside down, little marker taped to anus, measure distance from gas to marker. If gap is short, we can do simple repair immediately. If gap is large, might opt for diverting colostomy with repair at a later, more developed date, before toilet training.
* Newborn baby is tachypneic, cyanotic, abdomen scaphoid, bowel sounds over left chest. X-ray shows bowel in left thorax. Shortly later the baby has hypoxia and aspirates. Answer is congenital diaphragmatic hernia, always on the left side. This is a problem as the lung on the left is hypoplastic, thus answer is not go to the OR and fix diaphragm. Answer is wait 2-3 days for pulmonary maturation, give extensive respiratory support, put on respirator with care taken to prevent over-inflation. Most likely, this mother had no prenatal care else this would be diagnosed in utero. If diagnosed in utero, planning could be made and birth could occur at a location with ECMO (extra-corporeal membrane oxygenation).
* At the time of birth, it is noted that a child has a large abdominal wall defect to the right of the umbilicus. There is a normal cord, but protruding from the defect is a matted mass of angry-looking edematous bowel loops. Answer is gastroschisis, defect with no membrane protecting it. Answer is go to OR and create a silastic container to house and protect the bowel (looks like a silo), gradually squeezing the bowel back over a few days. Answer also includes providing vascular access for long-term parenteral nutrition.
* A newborn baby is noted to have a shiny thin membranous sac at the base of the umbilical cord, with the cord going to the sac and not the baby directly. Inside the sac one can see part of the liver and loose abnormal bowel. Answer is omphalocele. Answer for next step is create silastic silo and move bowel in over a few days.
* Newborn is noted to have a moist medallion of mucosa occupying the lower abdominal wall, above the pubis and below the umbilicus. It is clear that urine is constantly bathing this congenital anomaly. Answer is exstrophy of the urinary bladder. Associated with epispadias. Answer for management is repair within the first two days of life, possibly transporting to a highly specialized facility to perform this.
* 30mins after first feed, baby vomits greenish fluid. Normally, vomiting is white from babies. Mother has polyhydramnios and baby has Down syndrome. X-ray shows a double-bubble sign, with large air-fluid level in stomach and smaller level in duodenum. No gas can be seen in the rest of the bowel. Answer could be three conditions, duodenal atresia, annular pancreas, or malrotation. Answer is barium enema (or swallow). If not malrotation, operate without urgency.
* If baby has green vomiting after first feed, double-bubble sign, and gas in small loops of distal bowel, answer is malrotation. This is rotationally the most devastating of the three (duodenal atresia and annular pancreas). Answer to diagnose malrotation is barium enema or barium swallow. Barium enema is safer, but less diagnostic, likely the better answer as it has less risk. If malrotation diagnosed, operate immediately.
* Newborn baby has repeated green vomiting during first day of life and passes no meconium. Baby is normal except for distension. X-ray shows multiple air-fluid levels and distended loops of bowel. Answer is intestinal atresia. This occurs via vascular accident in utero, so no need to search for other congenital anomolies. Answer is to operate to fix this.
* Very premature baby develops feeding intolerance, abdominal distention, and rapidly dropping platelet count. Baby is 4yo and was treated with indomethacin for a patent ductus. Answer is necrotizing enterocolitis. Answer for next step is stop feedings, intravenous broad-spectrum antibiotics, and surgical intervention only if there are signs of death of the bowel. Signs of bowel death are abdominal wall erythema, air in biliary tree, and pneumoperitoneum. * 3-day-old full term baby is brought in for feeding intolerance and bilious vomiting. X-ray shows multiple loops of dilated small bowel, ground glass appearance in lower abdomen, mother has cystic fibrosis. Answer is meconium ileus, we also know baby has cystic fibrosis from this. Answer for next step is gastrografin enema to make diagnosis by showing unused microcolon and pellets of meconium. Gastrografin is a hypertonic solution, drawing fluid into lumen, so it can help dissolve the meconium pellets, thus it is also therapeutic.
* 3-week-old baby has trouble feeding, is not growing well, has bilious vomiting. X-ray shows double-bubble with normal gas pattern in rest of the bowel. Answer is malrotation. Answer is barium enema then surgery.
* Three-week-old baby, first born baby boy, full term, vomited three days ago and is projectile. Projectile vomiting in adults is a sign of brain tumor. Projective vomiting at age three weeks is hypertrophic pyloric stenosis. Vomiting here has no bile, follows each feeding, and baby is hungry and eager to keep eating, looks dehydrated, has visible gastric peristaltic waves, and palpable olive-sized mass in right upper quadrant (the is the hypertrophic pylorus). No more diagnostic tests are needed, but if one was needed pick sonogram and not barium. Answer for treatment is not surgical first, answer is fix hypochloremic hypokalemic metabolic alkalosis and dehydration. Answer after that is operation, Fredet-Ramstedt pyloromyotomy.
* 8-week-old baby brought in because of persistent, progressive jaundice. Bilirubin is significantly elevated, about 2/3rds is conjugated direct bilirubin, serology negative for hepatitis, sweat test is normal. Answer is biliary atresia. Answer for diagnostic test is IDA radionuclide scan done one week after phenobarbital therapy. Phenobarbital is a powerful choleretic, stimulating liver to create bile. IDA scan showing material only in liver is diagnostic for atresia. If bile if found in liver, ducts, and duodenum, it is not atresia. Answer is operate, and we find during surgery that 1/3 cannot be operated and required liver transplant. Of the 2/3 that can be operated on, half remain well after the repair, so another 1/3 of the original require transplant.
* 2-month-old baby boy brought in because of chronic constipation, has distended abdomen, x-ray shows gas through dilated loops of bowel in the abdomen. Rectal exam is followed by explosive expulsion of stool and flatus, with remarkable improvement to the distension. Answer is aganglionic megacolon (Hirschsprung disease). Answer for diagnosis is full-thickness biopsy of the rectal mucosa.
* If kid has chronic constipation, goes to bathroom once a week, rectal exam shows rectal vault full of feces, episodes of fecal soiling, answer is likely psychological problem and not aganglionic megacolon.
* 9-month-old chubby healthy-looking boy who has episode of colicky abdominal pain that make him double-up and squat. The pain lasts for one minute and kid looks happy until he gets another colic. Physical exam shows a vague mass on the right abdomen, empty right lower quadrant, and current jelly stools. Answer is intussusception. Answer for next step is contrast enema, which is also therapeutic in most cases.
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