PERITONEUM, MESENTERY, AND EXTRAPERITONEAL SOFT TISSUES
Complex familial inheritance patterns exist for heterotaxy. An X-linked recessive inheritance appears to account for a male preponderance.
These patients usually have complex congenital cardiac abnormalities that tend to overshadow abdominal findings. Complex bowel rotation anomalies are encountered with both right and left isomerism. Aortic and gastric positions are variable. Right-sided isomerism results in asplenia. The liver extends across both upper quadrants of the abdomen. Some of these patients have a left-sided inferior vena cava. Gallbladder duplications occur in right-sided isomerism.
Heterotaxy with left abdominal isomerism results in polysplenia. Cardiac anomalies tend to be less severe than in asplenia. Most patients have azygous continuation with interruption of the inferior vena cava. The spleen(s) is (are) on the same side as the stomach. An absent gallbladder is more common in left-sided isomerism. An association exists between polysplenia and biliary atresia. Midgut malrotation is a common associated finding, and midgut volvulus develops in some of these infants.
Traditionally, heterotaxy has been evaluated with angiocardiography. While both CT and pulse and color Doppler US are helpful in outlining some anomalies, MRI is evolving as the preferred modality.
Liver/spleen scintigraphy detects splenic tissue in suspected heterotaxy; in some infants with negative planar imaging, the presence of splenic tissue is shown by SPECT.
Abdominal Wall Defects
Bladder exstrophy is discussed in Chapter 11. Cloacal malformations are discussed in Chapter 12.
Omphalocele
Failure of the abdominal wall to close normally results in several defects.A defect cephalic to the umbilicus results in a supraumbilical ventral hernia, anterior diaphragmatic defects, and related conditions. Additional failure of lateral wall fusion results in an omphalocele. The degree of visceral herniation depends on the size of the defect. Peritoneum covers the viscera,
and the defect is obvious. Associated bowel malrotation is common.
Prognosis in these neonates is often limited by other associated abnormalities, at times major. The abdominal organs tend to be malpositioned, leading to unusual imaging findings.
Gastroschisis
Defects in gastroschisis involve the lateral abdominal wall. The gross appearance is similar to that of an omphalocele, but with gastroschisis the umbilicus is in its normal position. Among infants with gastroschisis, about two thirds have a simple defect, and in one third complex defects are identified, ranging from bowel atresia, stenosis, and perforation, to volvulus (4); survival of those with a simple defect was 100%, but those with a complex defect had a mortality rate of 28%.
Neonates with gastroschisis are often premature. Small bowel dysmotility and a prolonged transit time are common. Conventional radiography often reveals bowel wall thickening and lumen dilation. Delayed barium transit suggests obstruction, although actual small bowel obstruction is uncommon. Bowel atresia, if present, does result in a high mortality.
After surgical repair of gastroschisis these infants are at increased risk for necrotizing enterocolitis, although even then overall survival rate is quite high.
Prune Belly Syndrome (Eagle-Barrett
Syndrome)
The prune belly syndrome, named after the lax, wrinkled abdominal wall seen in this condition, consists of abdominal wall hypoplasia, genitourinary anomalies—including bilateral cryptorchidism in males—and other, at times major, systemic abnormalities. Occurring mostly in males, the more severely affected neonates die shortly after birth. An incomplete expression of this syndrome exists, and some neonates have only mild manifestations. Unilateral abdominal wall hypoplasia also occurs. An association between congenital cytomegaloviral infection and prune belly syndrome has been raised, but the precise etiology is unknown.
A minority of these neonates have a urethral obstruction such as atresia or valves; most, however, have functional bladder outlet