Thomas E. Herman

Brachytelephalangic chondrodysplasia punctata with marked cervical stenosis and cord compression: report of two cases

Severe cervical spine stenosis with cord compression has not been well documented in brachytelephalangic chondrodysplasia punctata. We report two boys with phenotypic features of brachytelephalangic chondrodysplasia punctata who had severe cervical spine stenosis secondary to dysplastic cervical vertebrae, and discuss the significance of this association and its relation to the phenotypically similar Binder phenotype.

Pseudotumoral sarcoid granulomatous nephritis in a child : case presentation with sonographic and CT findings

Abstract Granulomatous nephritis with renal masses is a very uncommon complication of sarcoidosis. Renal sonography in a patient with pathologically proven granulomatous nephritis demonstrated echogenic masses. CT demonstrated low-density lesions with mottled contrast enhancement.

Solitary Hepatic Hemangioendothelioma with Extensive Cystic Necrosis and Markedly Elevated α-Fetoprotein

Solitary Hepatic Hemangioendothelioma with Extensive Cystic Necrosis and Markedly Elevated α-Fetoprotein

Asplenia Syndrome with Congenital Microgastria and Malrotation

A 2795-g infant was born to a 20-year-old gravida 3, para 0 mother at 40 weeks gestation by Caesarean section following failed induction. The child was noted to be profoundly cyanotic within minutes of birth, was started on Prostaglandin E1 and transferred to this hospital. An initial chest and abdominal radiograph were obtained (Figure 1). A cardiac catheterization demonstrated pulmonary atresia, double outlet right ventricle with a large VSD with single ventricle physiology, total anomalous pulmonary venous return to the inferior vena cava, bilateral superior venae cavae and pulmonary artery branch stenoses. Because of this, the child was taken to the operating room where a right modified Blalock Taussig shunt, left atrial reimplantation of total anomalous pulmonary veins and repair of bilateral branch pulmonary artery stenoses. Subsequently, an abdominal sonogram (Figure 2) and upper gastrointestinal series (Figure 3) were performed.

Central dot sign on CT of liver cysts

The central dot sign on CT in Caroli disease has been described previously. We describe a patient with the central dot sign who had periductal cysts but did not have Caroli disease.

Feingold Syndrome: Microcephaly, Esophageal Atresia, Type III Laryngeal Cleft, Malrotation, Limb Anomalies

Feingold Syndrome: Microcephaly, Esophageal Atresia, Type III Laryngeal Cleft, Malrotation, Limb Anomalies

Posterior mediastinal capillary hemangioma with extradural extension resembling neuroblastoma.

Abstract We present two patients with posterior mediastinal capillary hemangiomas that were paraspinal and had intraspinal extension. Computed tomography demonstrated the strikingly hypervascular nature of these tumors, distinguishing them from neuroblastoma.

CT manifestations of ileal dysgenesis

Ileal dysgenesis is an uncommon condition of unknown etiology occurring in the distal ileum in the region of the vitelline duct. The CT appearance of this lesion, although not previously described to our knowledge, is characteristic. We report a patient with ileal dysgenesis who had an abdominal CT scan to evaluate chronic iron deficiency anemia and protein-losing enteropathy. Recognition of this lesion by pediatric radiologists is important; so that surgical treatment, which is simple and effective, can be initiated quickly.

Accelerated phase of Chédiak-Higashi syndrome diffuse white-matter-enhancing lesions.

Abstract We present the CT and MRI findings of a patient with Chédiak-Higashi syndrome in the accelerated phase with marked white-matter abnormalities. Pathologic and clinical features allow diagnosis of this condition, which in this case had dramatic neuroradiographic manifestations not well described in previous reports.