Search

Aneurysmal disease is uncommon in children, and its presence often leads to suspicion of genetic disorders (Loeys-Dietz syndrome, Marfan syndrome, Ehlers-Danlos syndrome, tuberous sclerosis), trauma, and infection. We describe the case of a newborn boy with generalized left lower limb hypoplasia associated with diffuse areas of arteriectasis combined with areas of stenosis and fusiform aneurysms of the iliac, femoral, and popliteal arteries. No additional vascular territories were affected. The patient was asymptomatic, and no therapeutic intervention has been considered. Numerous complementary imaging and laboratory examinations failed to establish a definitive diagnosis. This collection of findings has not been previously reported.

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.

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.

The usefulness of sonography in evaluating the pancreas in infants and children is well known. Its usefulness is due to the optimal sonic window provided by the relative lack of fat tissue and the large left hepatic lobe in children. A wide spectrum of pancreatic pathologic conditions can be identified sonographically in children, including congenital abnormalities; inflammatory, cystic, pseudocystic, and neoplastic lesions in usual and unusual manifestations; rare processes such as nesidioblastosis and Schwachman-Diamond syndrome; and pancreatic involvement in systemic disease. Multiple pathologic processes can affect the pancreas focally or diffusely, making diagnosis difficult in some instances, since the pancreatic response is limited to changes in echogenicity and size independent of the cause. Sonography is rapid, accurate, and noninvasive and does not require patient sedation. It is the modality of choice for evaluating suspected pancreatic disease, since it allows not only direct visualization of the pancreas but also evaluation of adjacent organs.