05 Peds Surg Flashcards
Within 8 hours after birth, it is noted that a baby has excessive salivation.
A small, soft NG tube is inserted, and the baby is taken to x-ray to have a “babygram” done. The film shows the tube coiled back on itself in the upper chest. There is air in the GI tract.
What is it? Tracheoesophageal (TE) fistula, the most common type, with proximal blind esophageal pouch and distal TE fistula.
Management. First, rule out the associated anomalies (VACTER: vertebral, anal, cardiac, TE, and renal/radial). The vertebral and radial will be seen in the same x-ray you already took, you need echocardiogram for the heart, sonogram for the kidneys, and physical examination for the anus. Then off to surgery.
TE fistula associated conditions
I. Maternal polyhydramnios (most commonly seen in pure esophageal atresia without fistula)
II. 40% associated maldevelopments (#1 endocardial cushion defect)
III. VATER complex - Vertebral and Anal defects, TE fistula, Radial limb dysplasia; may be full or partial but must R/O complete complex
IV. VACTERL (VATERL, VATER) association described as group of congenital anomalies with nonrandom tendency for concurrence;
Other associated problems are single umbilical artery, duodenal atresia, Meckel’s diverticulum; cases are preferentially males, higher perinatal mortality rates, higher frequency of fetal loss in previous pregnancies, lower mean birth weights; most patients have normal brain function, growth retardation can be seen during first 3 years of life (Pediatric Surgery Update 1998 Oct;11(4):1)
congenital esophageal stenosis
VACTERL complex stands for?
V = vertebral anomalies (hemi- and bifid vertebrae), A = anorectal malformation (imperforate anus), C = congenital cardiac defects (VSD, ASD, tetralogy of Fallot), TE = trachea-esophageal anomalies (esophageal atresia), R = renal-urinary defects (absent kidney, hypospadias), L = limb defects (radial dysplasia); mesodermic defect traced to third week of intrauterine life.
VATER complex stands for?
Vertebral and Anal defects, TE fistula, Radial limb dysplasia; may be full or partial but must R/O complete complex
A newborn baby is found on physical examination to have an imperforate anus.
Management. This is part of the VACTER group, so rule out the other components. For the anal problem, if there is a fistula to the vagina or peritoneum, repair can be safely done later, as the gastrointestinal tract is not obstructed. If there is no fistula, one has to ascertain the level of the blind pouch. This is done with an x-ray while holding the baby upside down, with a metal marker taped to the anal dimple. Low imperforate anus can be corrected with a very simple operation. High imperforate anus needs a colostomy, and repair at a later date.
A newborn baby is found to be tachypneic, cyanotic, and grunting. The abdomen is scaphoid, and there are bowel sounds heard over the left chest. An x-ray confirms that there is bowel in the left thorax. Shortly thereafter, the
baby develops significant hypoxia and acidosis.
What is it? Congenital diaphragmatic hernia.
Management. The main problem is the hypoplastic lung. It is better to wait 36 to 48 hours to do surgery to allow transition from fetal circulation to newborn circulation. Meanwhile, the trick is to keep the kid alive with endotracheal intubation, low-pressure hyperventilation (careful not to blow up the other lung), sedation, and NG suction)
I. 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
II. A newborn baby is noted to have a shiny, thin, membranous sac at the base of the umbilical cord (the cord goes to the sac, not to the baby). Inside the sac, one can see part of the liver and loops of normal bowel.
What are they? The first one is gastroschisis, the second one omphalocele. Medical school professors love to emphasize differential diagnosis of somewhat similar problems. Chances are all you’ll be expected to do is to identify the correct one.
Management. Intuitive. You’ve got to get those intestines back into the belly, and the technical details are best left to the pediatric surgeons. They will be on the lookout for atresias (which babies with gastroschisis can have) or multiple defects (which are seen with omphalocele), and
they will close small defects directly. Very often, however, the defects are large, most of the bowel
is outside the abdomen, and there is no room to “push it in.” In those cases a silicon “silo” is used to house the bowel and gradually return it to the abdomen. The baby with gastroschisis will also need vascular access for IV nutrition (the angry bowel will not work for about 1
month).
A newborn is noted to have a moist medallion of mucosae occupying the lower abdominal wall, above the pubis and below the umbilicus. It is clear that urine is constantly bathing this congenital anomaly.
What is it? Extrophy of the urinary bladder.
What’s the point of the vignette? These are very rare anomalies that only very highly specialized centers can repair. The problem is that unless the repair is done within the first 48 hours, it will not have a good chance to succeed. It takes time to arrange for transfer of a newborn baby to a distant city. If a day or two are wasted before arrangements are made, it will be too late.
Half an hour after the first feed, a baby vomits greenish fluid. The mother had polyhydramnios, and the baby has Down syndrome. X-ray shows a “doublebubble sign”: a large air-fluid level in the stomach, and a smaller one in the first portion of the duodenum. There is no gas in the rest of the bowel.
What is it? It can be two things, but first some general points. Kids vomit, burp, and regurgitate all the time (ask any parent), but the innocent vomit is clear-whitish. Green vomiting in the newborn is bad news. It means something serious. The two conditions that this could be are duodenal atresia and annular pancreas. Malrotation is also possible, but I expect that one to be presented to you as in the next vignette.
Management. With complete obstruction, surgery will be needed, but these kids have lots of other congenital anomalies, look for them first.
Half an hour after the first feed, a baby vomits greenish fluid. X-ray shows a “double-bubble sign”: a large air-fluid level in the stomach, and a smaller one in the first portion of the duodenum. There is air in the distal bowel, beyond the duodenum, in loops that are not distended.
What is it? Now you have three choices: it could be an incomplete obstruction from duodenal stenosis or annular pancreas, or it could be malrotation.
Management. If you are dealing with incomplete obstruction, you have time to do what’s needed, i.e., it is a lesser emergency. But if it is malrotation the bowel could twist and die, so that one is a super-emergency. How can you tell? A contrast enema is safe but not always diagnostic. An upper GI study is riskier but more reliable.
A newborn baby has repeated green vomiting during the first day of life, and does not pass any meconium. Except for abdominal distention, the baby is otherwise normal. X-ray shows multiple air-fluid levels and distended loops of
bowel.
What is it? Intestinal atresia.
Management. This one is caused by a vascular accident in utero; thus, there are no other congenital anomalies to look for, but there may be multiple points of atresia.
A very premature baby develops feeding intolerance, abdominal distention, and a rapidly dropping platelet count. The baby is 4 days old, and was treated with indomethacin for a patent ductus arteriosus.
What is it? Necrotizing enterocolitis.
Management. Stop all feedings, broad-spectrum antibiotics, IV fluids/nutrition. Surgical intervention
if the baby develops abdominal wall erythema, air in the portal vein, or pneumoperitoneum.
A 3-day-old, full-term baby is brought in because of feeding intolerance and bilious vomiting. X-ray shows multiple dilated loops of small bowel and a ground-glass appearance in the lower abdomen. The mother has cystic
fibrosis.
What is it? Meconium ileus.
Management. Gastrografin enema may be both diagnostic and therapeutic, so it is the obvious first choice. If unsuccessful, surgery may be needed. The kid has cystic fibrosis, and management of the other manifestations of the disease will also be needed.
A 3-week-old baby has had “trouble feeding” and is not quite growing well. He now has bilious vomiting and is brought in for evaluation. X-ray shows a classic “double bubble,” along with normal-looking gas pattern in the rest of the bowel.
What is it? Malrotation. The vignette is repeated here because they can show up at any time within the first few weeks of life. Proceed with urgent diagnostic studies.
A 3-week-old first-born, full-term baby boy began to vomit 3 days ago. The vomiting is projectile, has no bile in it, and follows each feeding, and the baby is hungry and eager to eat again after he vomits. He looks somewhat dehydrated and has visible gastric peristaltic waves and a palpable “olive size” mass in the right upper quadrant.
What is it? Hypertrophic pyloric stenosis.
Management. Check electrolytes; hypokalemic, hypochloremic metabolic alkalosis may have developed. Correct it, rehydrate, and do Ramstedt pyloromyotomy or balloon dilatation.
