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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.

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.

Ultrasonography (US) is a rapid, accurate, noninvasive method of evaluating ocular and orbital disease. A wide spectrum of pediatric ocular and orbital disorders can be demonstrated with US: ocular congenital malformations, vitreoretinal disorders, primary and metastatic ocular and orbital neoplasms, inflammatory and congestive disease (focal or systemic), cystic disease, trauma, and foreign bodies. US may be the method of choice for studying ocular congenital anomalies and vitreoretinal disorders owing to its high definition and noninvasiveness. In ocular neoplasms, US demonstrates the morphology of the lesion, its solid or liquid nature, and the presence of calcifications. Its use is limited when the tumor extends outside the globe; computed tomography (CT) or magnetic resonance (MR) imaging must then be performed. In ocular trauma, US precisely demonstrates the presence of hemorrhage, retinal and choroidal detachment, and foreign bodies. CT and MR imaging are superior in evaluation of orbital disease. Color Doppler US often allows differential diagnosis of vascular, inflammatory, and neoplastic disease.