Biomedical subjects
Publications and source records attributed to T E Herman.
Congenital syphilis is increasingly being diagnosed in developed countries after many years of decline. The liver is characteristically involved. However, fulminant hepatic failure and subsequent liver calcifications are both rare in patients with congenital syphilis. The infant reported here had both of these rare manifestations of this disease.
Three members of a family in two generations with proximal jejunal atresia and renal dysplasia are described. This association is presumably an autosomal dominant condition.
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Type IV Ehlers-Danlos syndrome (EDS) is characterized by large vessel bleeding, visceral rupture and early death. Pulmonary complications are uncommon. We report an 18-year-old patient with EDS type IV who developed recurrent large, thick-walled lung cavitary lesions, probably a manifestation of focal lung rupture. Such hemorrhagic cavities are probably the basis of the bullous disease and recurrent pneumothoraces occasionally observed in type IV EDS.
Inflammatory myofibroblastic tumor (IMT) is an uncommon quasineoplastic process occurring in young patients. Splenic involvement is uncommon and occurs predominantly in older, adult patients. We present the youngest patient reported to date with splenic inflammatory myofibroblastic tumor and discuss the clinical, pathological and imaging features of this lesion.
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Giant Meckel diverticula type II are very rare. However, in contrast with classic Meckel diverticula the small bowel follow through is often abnormal and suggestive of the diagnosis.
A patient with a recurrent left neck abscess was found to have a branchial fistula from the pyriform sinus. This uncommon anomaly was demonstrated by CT and barium swallow. The embryology, pathology and clinical features of this lesion are discussed and contrasted with those of the branchial cyst.
A marked thoracic deformity associated with intrathoracic tracheal narrowing was seen in a 17-year-old with achondroplasia and dyspnea. The role of chest deformity and its evaluation by CT and MRI in achondroplastic patients with respiratory symptoms are considered.
Occipital horn syndrome, a rare genetic disorder of copper metabolism, was recognized in 2 unrelated patients. Radiographs of these patients at various ages allowed confirmation of previously described radiographic findings. In addition, new radiographic manifestations were encountered. These pathognomonic radiographic findings are presented and the clinical and biochemical features of occipital horn syndrome are reviewed.
Periportal low attenuation, defined as a low-attenuation rim around the portal vein and its branches that is seen on contrast material-enhanced computed tomographic (CT) scans, has been described in a variety of conditions in adults. The authors reviewed the appearance of periportal low attenuation on CT scans of 30 children. An association was found between a rim of low attenuation and hepatic trauma, hepatic transplantation, malignancy (undifferentiated hepatoblastoma, juvenile chronic myelogenous leukemia), and generalized hepatic disorders (acute hepatitis and congenital hepatic fibrosis). The possible mechanisms for development of periportal low attenuation include periportal tracking of blood, obstructive lymphedema, tumor infiltration, perivascular inflammation, or bile duct proliferation.
The case of a three year old boy with a rare extra-renal nephroblastoma is presented. The possible origins of these tumors will be considered as well as the prognosis and differential evaluation by CT.
A 7 year old boy with a prior history of T-cell acute lymphoblastic leukemia developed Sezary syndrome or cutaneous lymphoma of T-lymphocytes (CLTL). His course was rapidly progressive and fatal with the primary manifestation being pulmonary lymphoma. This patient is one of the youngest with CLTL to manifest extracutaneous involvement.
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A case of a 16-year-old girl with a RIO of the T11 neural arch compressing the spinal cord is presented. The radiation therapy had been given after removal of a Wilms' tumor at 1 year of age. RIOs usually occur in the long bones and are diagnosed about 8 years following irradiation. Spinal cord or nerve root compression is usually associated with multiple cartilaginous exostoses.
A cardiac fibroma in a newborn is reported. The infant had skeletal stigmata of Gorlin's syndrome and the mother had known Gorlin's syndrome.
A child with vertebral anomalies and tubular communicating duplications of the esophagus and stomach had a post-natal ultrasound interpreted as normal. This complex anomaly was delineated by barium studies and surgically confirmed.
Osteodysplastic primordial dwarfism, type II manifests typical skeletal features which have not previously been described in the radiographic literature. We present an infant with characteristic findings and discuss related conditions, especially the Seckel syndrome.