Biomedical subjects
Publications and source records attributed to T E Herman.
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.
After noting several occurrences of cystic kidney disease in Turner's syndrome we retrospectively reviewed the renal sonograms of 12 patients with this syndrome. Single renal cysts occurred in two patients and a multicystic dysplastic kidney in another. Our experience suggests that single renal cysts are a relatively frequent finding in Turner's syndrome and should be recognized within the scope of renal abnormalities associated with it.
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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.
Surfactant is a complex structure primarily composed of phospholipids, but containing essential proteins as well. Congenital deficiency of Surfactant Protein-B (SPB) has recently been documented for the first time in two siblings. The pathologic findings in these infants was that of congenital pulmonary alveolar proteinosis and the radiographic manifestations were strikingly similar to hyaline membrane disease.
The Schinzel-Giedion syndrome is a rare autosomal recessive condition with typical facies, skeletal manifestations and congenital hydronephrosis. We report an infant with characteristic findings who had bilateral congenital megacalyces. Congenital megacalyces is believed to be a developmental abnormality, occurs in other malformation syndromes and has not previously been described in the Schinzel-Giedion syndrome.
A phenotypically female infant with 46-XY chromosomes was found to have Smith-Lemli-Opitz syndrome, type II a rare congenital malformation syndrome with many features of the more common classic Smith-Lemli-Opitz syndrome. The patient's skeletal survey revealed characteristic and previously undescribed skeletal anomalies which are reported. In addition a lipoma of the pituitary gland was found on magnetic resonance imaging. This lesion is particularly interesting given the hypothesized steroid abnormality in Smith-Lemli-Opitz, type II syndrome, the sexual ambiguity of males with this syndrome and the similarity of this syndrome to the Pallister-Hall syndrome which characteristically has a hamartoblastoma of the hypothalamus.
Langerhans cell histiocytosis of the head and neck is an uncommon histiocytic proliferative disorder that often clinically resembles an inflammatory process. A pseudoaneurysm of the external carotid artery was found by contrast-enhanced CT and confirmed by Doppler ultrasonography and angiography in a 9 year old girl with a lytic lesion of the mandible, a prior severe tracheal inflammatory process, and sclerotic lesions of the sphenoid wings. Biopsies of the mandibular lesion, pseudoaneurysm, and trachea demonstrated Langerhans cell histiocytosis. In cases of LCH with head and neck involvement, contrast-enhanced CT allows adequately detailed initial evaluation of the protean manifestations of this disorder.
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Smith-Lemli-Opitz syndrome, type II, is a very rare congenital condition which has been fatal in all reported cases. The imaging findings can be diagnostic. A rapid and accurate diagnosis is very important to the effected infant and the parents in this autosomal recessive condition.
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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