John J. Buchino

Gastrointestinal perforation following indomethacin therapy in very low birth weight infants.

Over an 18-mo period patent ductus arteriosus (PDA) was diagnosed in 112 (50%) of 222 very low birth weight infants (

Acute Hepatic Failure Associated with the Use of Sodium Valproate

SODIUM valproate, a recently introduced anticonvulsant, is particularly useful in the management of absence seizures and has gained widespread popularity.1 Initial European studies reported infrequ...

Pancreatoblastoma. A histochemical and ultrastructural analysis.

A case of pancreatoblastoma in a 4‐year‐old boy is presented. This tumor was studied by histochemical and ultrastructural techniques and was found to have both exocrine and endocrine components. This analysis and a review of the literature helps to further define this entity, which has a significantly better prognosis than other pancreatic malignancies.

The histologic similarity between craniopharyngioma and odontogenic lesions: A reappraisal

The histologic similarities between the craniopharyngioma and the ameloblastoma are well recognized and supported by their common embryologic origin from oral ectoderm. Differences in these lesions include a greater tendency for craniopharyngiomas to be cystic and form ghost cells and calcifications. The keratinizing and calcifying odontogenic cyst (KCOC), a lesion that features proliferating ameloblastic epithelium, ghost keratin, calcification, and cyst formation, may more precisely mimic the craniopharyngioma. The histologic features of twenty-seven craniopharyngiomas were studied. Twenty cases resembled KCOC microscopically. Two examples duplicated the histologic features of infiltrative ameloblastoma, while five showed characteristics of both lesions. This study shows that the range of histologic features in craniopharyngioma includes and spans both odontogenic lesions but more often simulates KCOC. The results suggest that the KCOC and the ameloblastoma may be closely related developmentally.

Tracheal agenesis: A clinical approach

Tracheal agenesis, though seemingly rare and fatal to date, has been reported from multiple institutions with increasing frequency. Establishment of the diagnosis necessitates a high index of suspicion in an infant in respiratory distress at birth, without a cry and in whom intubation is difficult. Following definitive diagnosis by endoscopy, infants have been given a chance at survival on four occasions by prompt surgical intervention. The procedure proposed by Altman is adequate for initial stabilization. Postoperative management is aided by the use of continuous positive airway pressure (CPAP) and rigorous pulmonary toilet. Definitive correction should be considered at an early stage in order to prevent fatal complications.

Hirschsprung's disease with skip area (Segmental aganglionosis)

Hirschsprungs disease is characterized by a single aganglionic segment of colon extending distally to the anal margin. Well documented reports of segmental aganglionosis have been rare. We report a case of segmental aganglionosis in which there were two distinct aganglionic segments resected. The entire transverse colon between the two aganglionic segments was normally ganglionated, preserved, and utilized and functions in a normal fashion.

Open lung biopsy in pediatric patients on extracorporeal membrane oxygenation

Open lung biopsy has proven beneficial in the treatment of life-threatening pulmonary diseases. Its safety and efficacy in infants and children placed on extracorporeal membrane oxygenation (ECMO) for severe respiratory failure is not known. The authors reviewed eight cases (4 neonates, 3 infants, 1 child) who underwent open lung biopsy while on ECMO. The primary diagnoses were pneumonia (4), lymphoma (1), primary pulmonary hypertension (1), and complex congenital heart disease (2). The patients underwent biopsy after they had been on ECMO an average of 9.6 days (range, 1 to 14 days). Biopsy results confirmed the clinical diagnosis in five patients, two of whom had irreversible alveolar destruction resulting in ECMO withdrawal. Three patients had pathological diagnoses, which resulted in major therapy revisions (1 fungal infection and 2 noninfectious lesions that required steroid treatment). The overall average duration of ECMO treatment was 16.3 days (range, 10 to 24 days). Three patients were weaned successfully from ECMO, but only one infant survived to discharge. One nonlethal bleeding complication occurred after biopsy. Open lung biopsy is well tolerated during ECMO. It accurately determines pulmonary pathology and provides valuable prognostic information. Earlier biopsy for patients whose diagnoses are uncertain or who are not responding to ECMO may improve the mortality rate for this high-risk group.

Prognostic value of pre- and postoperative cardiac troponin I measurement in children having cardiac surgery.

ABSTRACT The objective of this study was to determine if perioperative elevation of cardiac troponin I (cTnI) predicts mortality in infants and children after surgical correction of congenital heart defects. One hundred infants and children having open heart surgery were studied. Blood samples for cTnI analysis were collected before cardiopulmonary bypass (CPB) and at 4, 8, 12, and 24 h after initiation of CPB. Demographic information, cardiac defect, repair performed, duration of CPB, complications, and outcome were recorded. Cardiac defects were categorized as atrial septal defect (ASD), ventricular septal defect (VSD), hypoplastic left heart syndrome (HLHS), complex, and “other.” Baseline cTnI was significantly lower in survivors (mean 0.42 ng/ml, median 0.35 ng/ml) than in nonsurvivors (mean 1.89, median 1.30), p= 0.0001. Baseline cTnI was significantly higher in the HLHS group (mean 1.47, median 1.10) than in all other subgroups (mean 0.62, median 0.35), p≤ 0.009. There were no significant differences between survivors and nonsurvivors at the remaining sampling times. Children who died from cardiac failure (n = 2) were more likely to have 4 h cTnI >125 ng/ml compared to survivors (2 of 90). Within cardiac defect subgroups, 4 h cTnI was significantly higher in the complex group (mean = 53.51, median = 32.30) than in the ASD (mean = 23.84, median = 19.85) and other (mean = 21.59, median 21.50) subgroups. Perioperative measurement of cTnI identifies children within specific cardiac defect subgroups at risk of mortality after cardiac surgery. We speculate that detection of myocardial injury may decrease mortality and morbidity in children with complicated congenital cardiac lesions by leading to improvements in perioperative management.

Aggressive Osteoblastoma: A Case Report Involving a Unique Chromosomal Aberration

Osteoblastomas are rare bone-producing neoplasms that generally occur in the young and can be misdiagnosed as an osteosarcoma if correlation with clinical history, radiology, and histology is not carefully considered or if the several variants of osteoblastoma are not recognized. These variants lie on a morphologic spectrum between conventional osteoblastoma and osteosarcoma. Aggressive osteoblastoma is one such subtype. As the name implies, the histologic features of aggressive osteoblastoma may appear malignant, and its biologic behavior may separate it from conventional osteoblastoma. We report a case of aggressive osteoblastoma occurring in the femoral diaphysis of a 12-year-old girl; this osetoblastoma was dyssynchronous from the radiologic appearance and a diagnostic challenge. Cytogenetic evaluation of the neoplasm revealed a pseudodiploid clone with a balanced translocation involving chromosomes 4, 7, and 14. Using the premise that cytogenetics might be useful as a diagnostic tool for a more specific classification, we reviewed the literature in order to compare our findings with known chromosomal aberrations.

Large-Cell Calcifying Sertoli Cell Tumor

We report on a 20-year-old man with a left testicular mass clinically believed to be a malignant neoplasm but subsequently identified as a large-cell calcifying Sertoli cell tumor. This is a rare form of Sertoli cell tumor with unique clinical and pathological features only recently described in the literature. The neoplasm has a low malignant potential with only 1 patient known to have had metastasis. The tumor occurs primarily during the first 2 decades of life and has been associated with endocrine disorders and cardiac myxoma. The clinician should be mindful of this tumor because of its low malignant potential and the necessity to evaluate individuals for endocrine and cardiac abnormalities.