Congenital extrahepatic portosystemic shunt (CEPS) is a rare condition in which the portomesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial shunt. Most often, the diagnosis is made primarily with Doppler ultrasonography. Computed tomographic angiography and magnetic resonance angiography are used for further classification of the shunt and assessment of accompanying anomalies. Conventional angiography is necessary when results of the other tests disagree or are inconclusive. CEPS is classified into two types according to the pattern of anastomoses between the portal vein and systemic vein. In type 1, intrahepatic portal venous supply is absent; in type 2, intrahepatic portal venous supply is preserved. Type 1 usually occurs in girls with associated malformations, such as situs ambiguous with polysplenia and congenital heart defects. Associated anomalies are less frequent in type 2, and symptoms usually develop later without a gender preference. Hepatic encephalopathy and liver dysfunction are possible complications of both types and usually develop during adulthood. Both types are also associated with regenerative hepatic nodules. The clinical setting and imaging appearance of these nodules can help one avoid misdiagnosis. Definitive treatment of CEPS is determined by the type of shunt. Liver transplantation is the only effective treatment for symptomatic type 1 CEPS; surgical closure or embolization of the shunt is the therapeutic approach for type 2.

Congenital anomalies of the gastrointestinal tract are a significant cause of morbidity in children and, less frequently, in adults. These abnormalities include developmental obstructive defects of the small intestine, anomalies of the colon, anomalies of rotation and fixation, anorectal anomalies, and intestinal duplications. Neonates with complete high intestinal obstruction do not usually require further radiologic evaluation following radiography, whereas those with complete low obstruction should undergo a contrast material enema examination. An upper gastrointestinal series must be performed in all patients with incomplete intestinal obstruction because management is different in each case. In low intestinal obstruction, ultrasonography (US) may help differentiate between small bowel obstruction and colonic obstruction. In addition, US can help correctly identify meconium ileus and meconium peritonitis and is useful in the diagnosis of enteric duplication cysts. In malrotation and anorectal anomalies, computed tomography (CT) and magnetic resonance (MR) imaging can provide superb anatomic detail and added diagnostic specificity. Intestinal duplications manifest as an abdominal mass at radiography, contrast enema examination, or US. At CT, most duplications manifest as smoothly rounded, fluid-filled cysts or tubular structures with thin, slightly enhancing walls. At MR imaging, the intracystic fluid has heterogeneous signal intensity on T1-weighted images and homogeneous high signal intensity on T2-weighted images. Familiarity with these gastrointestinal abnormalities is essential for correct diagnosis and appropriate management.

A wide spectrum of congenital anomalies may affect the upper gastrointestinal tract, including anomalies of the esophagus (e.g., atresia, fistulas, webs, duplications, vascular rings), stomach (e.g., congenital gastric outlet obstruction, duplications), and duodenum (e.g., atresia, annular pancreas, duplications, malrotation). The evaluation of affected patients can require multiple imaging modalities for diagnosis and surgical planning. Radiography is often diagnostic and specific and can usually provide important clues to help determine the optimal diagnostic procedure. Neonates with complete gastric or upper intestinal obstruction do not usually require further radiologic evaluation after radiography: Barium studies are usually contraindicated, and complementary procedures (e.g., ultrasound [US], computed tomography [CT]) are not usually helpful and may even delay surgery, resulting in death. Nevertheless, US has become important in the evaluation of the pediatric gastrointestinal tract and is being used in an increasing number of applications. CT and magnetic resonance imaging are unsuitable for general screening but provide superb anatomic detail and added diagnostic specificity. They are especially useful in demonstrating esophageal duplications and vascular rings as well as associated abnormalities. However, the decision to perform a given imaging examination should be considered carefully to avoid inconvenience or unnecessary radiation exposure to the patient or delays in surgical correction. Quality control programs should be in place to ensure safe, effective radiologic practice through use of up-to-date equipment and good imaging technique.

Orthotopic liver transplantation is currently the treatment of choice in patients with end-stage liver disease for which no other therapy is available. In children, segmental liver transplantation with living donor, reduced-size cadaveric, and split cadaveric allografts has become an important therapeutic option. However, the resulting expansion of the donor pool has increased the risk for postoperative vascular and biliary complications, which affect children more frequently than adults. Early recognition of these complications requires radiologic evaluation because their clinical manifestations are frequently nonspecific and vary widely. Doppler ultrasonography (US) plays the leading role in the postoperative evaluation of pediatric patients. Current magnetic resonance (MR) imaging techniques, including MR angiography and MR cholangiography, may provide a wealth of pertinent information and should be used when findings at US are inconclusive. Computed tomography is a valuable complement to US in the evaluation of complications involving the hepatic parenchyma as well as extrahepatic sites and is commonly used to guide percutaneous aspiration and fluid collection drainage. Familiarity with and early recognition of the imaging appearances of the various postoperative complications of pediatric liver transplantation are crucial for graft and patient survival.

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