Chapter 322 Intestinal Atresia, Stenosis, and Malrotation

Approximately 1 in 1,500 children is born with intestinal obstruction. Obstruction may be partial or complete, and it may be characterized as simple or strangulating. Luminal contents fails to progress in an aboral direction in simple obstruction, whereas blood flow to the intestine is also impaired in strangulating obstruction. If strangulating obstruction is not promptly relieved, it can lead to bowel infarction and perforation.

Intestinal obstruction can be further classified as either intrinsic or extrinsic based on underlying etiology. Intrinsic causes include inherent abnormalities of intestinal innervation, mucus production, or tubular anatomy. Among these, congenital disruption of the tubular structure is most common and can manifest as obliteration (atresia) or narrowing (stenosis) of the intestinal lumen. More than 90% of intestinal stenosis and atresia occurs in the duodenum, jejeunum, and ileum. Rare cases occur in the colon, and these may be associated with more proximal atresias.

Extrinsic causes of congenital intestinal obstruction involve compression of the bowel by vessels (e.g., preduodenal portal vein), organs (e.g., annular pancreas), and cysts (e.g. duplication, mesenteric). Abnormalities in intestinal rotation during fetal development also represent a unique extrinsic cause of congenital intestinal obstruction. Malrotation is associated with inadequate mesenteric attachment of the intestine to the posterior abdominal wall, which leaves the bowel vulnerable to auto-obstruction due to intestinal twisting or volvulus. Malrotation is commonly accompanied by congenital adhesions that can compress and obstruct the duodenum as they extend from the cecum to the right upper quadrant.

Obstruction is typically associated with bowel distention, which is caused by an accumulation of ingested food, gas, and intestinal secretions proximal to the point of obstruction. As the bowel dilates, absorption of intestinal fluid is decreased and secretion of fluid and electrolytes is increased. This shift results in isotonic intravascular depletion, which is usually associated with hypokalemia. Bowel distention also results in a decrease in blood flow to the obstructed bowel. As blood flow is shifted away from the intestinal mucosa, there is loss of mucosal integrity. Bacteria proliferate in the stagnant bowel, with a predominance of coliforms and anaerobes. This rapid proliferation of bacteria, coupled with the loss of mucosal integrity, allows bacterial to translocate across the bowel wall and potentially lead to endotoxemia, bacteremia, and sepsis.

The clinical presentation of intestinal obstruction varies with the cause, level of obstruction, and time between the obstructing event and the patient’s evaluation. Classic symptoms of obstruction in the neonate include vomiting, abdominal distention, and obstipation. Obstruction high in the intestinal tract results in large-volume, frequent, bilious emesis with little or no abdominal distention. Pain is intermittent and is usually relieved by vomiting. Obstruction in the distal small bowel leads to moderate or marked abdominal distention with emesis that is progressively feculent. Both proximal and distal obstructions are eventually associated with obstipation. However, meconium stools can be passed initially if the obstruction is in the upper part of the intestinal tract or if the obstruction developed late in intrauterine life.

The diagnosis of congenital bowel obstruction relies on a combination of history, physical examination, and radiologic findings. In certain cases, the diagnosis is suggested in the prenatal period. Routine prenatal ultrasound can detect polyhydramnios, which often accompanies high intestinal obstruction. The presence of polyhydramnios should prompt aspiration of the infant’s stomach immediately after birth. Aspiration of more than 15-20 mL of fluid, particularly if it is bile stained, is highly indicative of proximal intestinal obstruction.

In the postnatal period, a plain radiograph is the initial diagnostic study and can provide valuable information about potential associated complications. With completely obstructing lesions, plain radiographs reveal bowel distention proximal to the point of obstruction. Upright or cross-table lateral views typically demonstrate a series of air-fluid levels in the distended loops. Caution must be exercised in using plain films to determine the location of intestinal obstruction. Because colonic haustra are not fully developed in the neonate, small and large bowel obstructions may be difficult to distinguish with plain films. In these cases, contrast studies of the bowel or computed tomography images may be indicated. Oral or nasogastric contrast medium may be used to identify obstructing lesions in the proximal bowel, and contrast enemas may be used to diagnose more-distal entities. Indeed, enemas may also play a therapeutic role in relieving distal obstruction due to meconium ileus or meconium plug syndrome.

Initial treatment of infants and children with bowel obstruction must be directed at fluid resuscitation and stabilizing the patient. Nasogastric decompression usually relieves pain and vomiting. After appropriate cultures, broad-spectrum antibiotics are usually started in ill-appearing neonates with bowel obstruction and those with suspected strangulating infarction. Patients with strangulation must have immediate surgical relief before the bowel infarcts, resulting in gangrene and intestinal perforation. Extensive intestinal necrosis results in short bowel syndrome (Chapter 330.7). Nonoperative conservative management is usually limited to children with suspected adhesions or inflammatory strictures that might resolve with nasogastric decompression or anti-inflammatory medications. If clinical signs of improvement are not evident within 12-24 hr, then operative intervention is usually indicated.

322.1 Duodenal Obstruction

Christina Bales, Chris A. Liacourus

Congenital duodenal obstruction occurs in 2.5-10/100,000 live births. In most cases, it is caused by atresia, an intrinsic defect of bowel formation. It can also result from extrinsic compression by abnormal neighboring structures (e.g., annular pancreas, preduodenal portal vein), duplication cysts, or congenital bands associated with malrotation. Although intrinsic and extrinsic causes of duodenal obstruction occur independently, they can also coexist. Thus, a high index of suspicion for more than one underlying etiology may be critical to avoiding unnecessary reoperations in these infants.

Duodenal atresia complicates 1/10,000 live births and accounts for 25-40% of all intestinal atresias. In contrast to more-distal atresias, which likely arise from prenatal vascular accidents, duodenal atresia results from failed recanalization of the intestinal lumen during gestation. Throughout the 4th and 5th weeks of normal fetal development, the duodenal mucosa exhibits rapid proliferation of epithelial cells. Persistence of these cells, which should degenerate after the 7th week of gestation, leads to occlusion of the lumen (atresia) in approximately two thirds of cases and narrowing (stenosis) in the remaining one third. Duodenal atresia can take several forms, including a thin membrane that occludes the lumen, a short fibrous cord that connects 2 blind duodenal pouches, or a gap that spans 2 nonconnecting ends of the duodenum. The membranous form is most common, and it almost invariably occurs near the ampulla of Vater. In rare cases, the membrane is distensible and is referred to as a windsock web. This unusual form of duodenal atresia causes obstruction several centimeters distal to the origin of the membrane.

Approximately 50% of infants with duodenal atresia are premature. Concomitant congenital anomalies are common and include congenital heart disease (30%), malrotation (20-30%), annular pancreas (30%), renal anomalies (5-15%), esophageal atresia with or without tracheoesophageal fistula (5-10%), skeletal malformations (5%), and anorectal anomalies (5%). Of these anomalies, only complex congenital heart disease has been associated with increased mortality. Annular pancreas has been associated with increased late complications, including gastroesophageal reflux disease, peptic ulcer disease, pancreatitis, gastric outlet and recurrent duodenal obstruction, and gastric cancer. Thus, long-term follow-up of these patients into adulthood is warranted. Nearly half of patients with duodenal atresia have chromosome abnormalities; trisomy 21 is identified in up to one third of patients.

Clinical Manifestations and Diagnosis

The hallmark of duodenal obstruction is bilious vomiting without abdominal distention, which is usually noted on the 1st day of life. Peristaltic waves may be visualized early in the disease process. A history of polyhydramnios is present in half the pregnancies and is caused by inadequate absorption of amniotic fluid in the distal intestine. This fluid may be bile stained due to intrauterine vomiting. Jaundice is present in one third of the infants.

The diagnosis is suggested by the presence of a “double-bubble” sign on a plain abdominal radiograph (Fig. 322-1

Only gold members can continue reading. Log In or Register to continue

Share this:

• Click to share on Twitter (Opens in new window)
• Click to share on Facebook (Opens in new window)

Related

Related posts:

1. Rumination, Pica, and Elimination (Enuresis, Encopresis) Disorders
2. Adolescent Pregnancy
3. Neisseria gonorrhoeae (Gonococcus)
4. Blastomycosis (Blastomyces dermatitidis)

Stay updated, free articles. Join our Telegram channel

Join