A congenital abnormality in the arrangement of muscle bundles in a segment of the distal ileum, producing obstruction: a variant of the so-called "giant Meckel's diverticulum".

This 4-year-old male child was diagnosed at birth as having several minor congenital anomalies. X-rays taken during the first year of his life showed a single, massively dilated loop of bowel in the upper abdomen, not appreciated at the time. In 1980 he was admitted to Jackson Memorial Hospital with the diagnosis of small bowel obstruction. An upper gastrointestinal series showed one tremendously dilated loop of distal ileum. At laparotomy, the patient was found to have extreme segmental dilatation of one loop of distal ileum which ended abruptly; there was no evident external cause for obstruction. The resected loop contained in excess of 200 cc of watery brown liquid. The mucosal folds and underlying smooth muscle bundles, in the dilated portion only, were not arranged circumferentially but rather in a distinctive finger-print-like pattern with trifurcations, whorls, and intricate interdigitations which had probably produced contractions of a circus type rather than normal peristaltic waves. We have been able to find only three reports in the literature in which, as was the case here, the so-called "giant Meckel's diverticulum" presented as a single tremendously dilated segment of ileum, sharply demarcated at its distal end. In none of them is there any description of the orientation of muscle bundles. We believe that the abnormal arrangement of smooth muscle in the muscular coat in this specimen, and perhaps in the others, probably represents the underlying cause for the extreme localized dilatation.

Wall thickness of small pulmonary arteries. Its measurement in victims of sudden infant death syndrome.

We attempted to reproduce the work of Naeye with regard to the thickness of the muscular media in small pulmonary arteries among victims of the sudden infant death syndrome, using Naeye's methods. In two separate studies, using two slightly different groups of patients and different ocular grids, we observed a statistically significant difference between crib deaths and accidental deaths in hyperplasia of the arterial muscular media, the only parameter not dependent on point-counting. As no investigator has been able to achieve exactly the same results as Naeye in all three parameters, we concluded that point-counting is probably not the method of choice for this determination. Our data for hyperplasia (in the second phase of the study) correlated with those for the retention of periadrenal brown fat and hepatic erythropoiesis in the same patients.

Sudden infant death syndrome: a review of the medical literature 1974-1979.

In the last five years there have been literally hundreds of articles of the world's medical literature regarding sudden infant death syndrome. This work has shed some light on various epidemiologic aspects of the problem, on pathologic anatomy, and on clinical issues such as the relative importance of spontaneous, protracted, idiopathic apnea and prolongation of the Q-T interval. This relatively comprehensive review treats only a limited number of these subjects and publications in an attempt to bring the reader more or less up-to-date on the major aspects of developments over the last five years.

The question of right ventricular hypertrophy in sudden infant death syndrome.

The relative weight of the free wall of the cardiac right ventricle was ascertained in 177 instances of the sudden infant death syndrome and 30 control infants who had died either in accidents (ten) or of fulminant illness (20). In contrast to the observations of Naeye et al in this regard, these victims of crib death did not exhibit right ventricular hypertrophy. Other evidence to support Naeye et al's contention that such infants are morphologically demonstrably different from normal is well documented. Hence, the significance of this discrepancy between our findings and theirs is unclear.

Extramedullary hematopoiesis in the liver in sudden infant death syndrome.

This study was undertaken to confirm or refute the findings of Naeye with regard to the pathologic continuation of extramedullary hematopoiesis in the livers of infants who die of the sudden infant death syndrome (SIDS). In general, our findings confirm his; however, although the data for SIDS do differ from those of controls, the differences are not statistically significant. Nevertheless, these results would seem to indicate the need for further investigation of the possibility of hypoxemia and/or anemia in affected infants.

Brown fat retention in sudden infant death syndrome.

This study was undertaken to confirm the findings of Naeye with regard to the pathological retention of the so-called brown fat cells in the periadrenal adipose tissue of infants who die of the sudden infant death syndrome. The percent of multilocular fat cells was determined in histological sections of periadrenal adipose tissue obtained from a series of 289 autopsies. In general, our morphological observations confirm his. However, our results indicate that the percent of periadrenal multilocular cells cannot be used as a diagnostic criterion in any single case. Futhermore, we consider that the mechanism for this delay in transformation remains unclear.

Yellow pulmonary hyaline membranes.

Yellow pulmonary hyaline membranes were observed at autopsy in 16 newborn infants between 1972 and 1974 in four hospitals of Philadelphia, Pa., and Newark, N.J. Other pediatric pathologists in this country and in Spain have seen the same lesion within the last decade. Chemical analysis of affected lung tissue, histochemistry, and electron microscopy show the yellow color to be due to the presence of bilirubin. No substantial clues concerning the basic etiology or mechanism for the formation of these unique membranes emerge from a detailed review of clinical and postmortem data nor from comparison of these data with those for 68 control infants with the usual acidophilic pulmonary hyaline membranes. We are left, however, with the impression that prolongation of life, relatively elevated levels of serum bilirubin, and protracted assisted ventilation (with either CPAP or PEEP) are intimately related to their formation.