Misalignment of lung vessels and alveolar capillary dysplasia: a cause of persistent pulmonary hypertension.

Two infants with fatal persistent pulmonary hypertension are described. Morphologically there was misalignment of the lung vessels, with the veins and the arterioles anomalously related, often sharing the same adventitial sheet. The capillaries did not make contact with the alveolar epithelium. The arterioles had increased medial muscle, and there was extension of the arteriolar muscularization to the precapillary level. The fraction of the parenchyma that was septal and connective tissue was increased. The acini had a decreased complexity, with immature alveoli and with a decreased radial alveolar count. The cause appeared to be related to abnormal capillary and venous plexus formation and migration. This syndrome seems to be identical with that described in three previous reports and probably represents a specific cause of persistent pulmonary hypertension.

Primary B-cell lymphoma of the CNS in an infant.

Primary B-cell lymphoma of the CNS is a rare tumor representing 0.2 to 2% of all primary CNS malignancies. The usual age of presentation is in the 4th to 6th decades. Sporadic cases have been reported in the pediatric population. However, even in this age group the tumor is extremely rare under the age of 5. This is a report of a 3-year-old child with primary lymphoma of the CNS.

Congenital fibromatosis (myofibromatosis) of the orbit: a rare cause of proptosis at birth.

A full-term infant boy had proptosis OS at birth: A large solid tumor mass was found by examination and computed tomographic scan in the orbit and extended intracranially. Biopsy showed spindle-shaped tumor cells that, with electron microscopy, proved to be myofibroblasts. Congenital fibromatosis (myofibromatosis) is a benign localized tumor of which this case is a striking example.

Histology of the intestine in human gastroschisis--relationship to intestinal malfunction: dissolution of the "peel" and its ultrastructural characteristics.

There are conflicting views on the pathogenesis of the intestinal malfunction seen in infants with gastroschisis. It has been variously ascribed to abnormalities of ganglion cells and smooth muscle elements, intestinal ischemia, and the "peel" which invests the serosa of the intestine. Review of the clinical and experimental literature showed only limited information on the histology of the eviscerated human intestine. In order to add to this data base, and to further investigate the pathogenesis of the intestinal malfunction from a histologic standpoint, we reviewed surgical and autopsy material from our experience with 105 neonates with gastroschisis. Ten specimens were satisfactory for evaluation from a standpoint of tissue integrity. The specific mural components of mucosa, submucosa, muscularis, and ganglion cells were examined and found to be either normal, or to show nonspecific abnormalities that varied from case to case, and were related mostly to intestinal infarction due to compromise of the gut at the site of the gastroschisis defect. In six patients, this progressed to atresia formation. The most consistent abnormalities were found in the serosal layer with its peel. Using special stains, the peel was found to be composed largely of fibrin and collagen. Based on this study, we feel that edema and ischemic changes, though often present, are much less prominent than the peel, as the leading histologic abnormality of the intestine of gastroschisis. Squamous epithelial cells were seen in the peel in four cases, suggesting that the peel had been "appliqued" onto the serosa of the herniated fetal gut.

Neonatal pulmonary hypoplasia with premature rupture of fetal membranes and oligohydramnios.

We assessed pulmonary function and compression deformities in 76 preterm infants less than or equal to 34 weeks gestation who had premature rupture of membranes (PROM) for longer than 5 days (mean +/- SD 18.8 +/- 15.4 days, range 6 to 90 days). Twenty-one of the 76 infants had oligohydramnios and positional deformities at birth; however, only two infants met all the criteria for the oligohydramnios tetrad. All 21 required assisted ventilation from the moment of birth. Twenty infants had clinical evidence of pulmonary hypoplasia; 18 of these died. Pulmonary hypoplasia was confirmed by significantly low wet lung weights, low lung DNA content, or low radial alveolar counts in the 13 infants with postmortem examinations. Fifty-five infants with PROM for longer than 5 days did not have positional deformities. Twenty-one required assisted ventilation, of whom 10 had severe oligohydramnios. Eleven of the 21 died; autopsies were performed. All had normal wet lung weights, but seven had significantly decreased radial alveolar counts, implying a less severe but still fatal form of pulmonary hypoplasia. None of the remaining 34 infants had lung disease, and only three had oligohydramnios. We conclude that pulmonary hypoplasia can result from PROM associated with severe oligohydramnios of as short as 6 days duration. Furthermore, fatal pulmonary hypoplasia can occur with little or no external deformation.

Wegener granulomatosis in pediatric patients.

Wegener granulomatosis is more easily recognized as a distinct clinical entity than other vasculitides because the initial clinical features frequently include granulomatous vasculitis of the upper and lower respiratory tract and glomerulonephritis. Although the disease has been lethal in the past, prolonged survival and avoidance of end-stage kidney disease can now be expected when cyclophosphamide therapy is introduced early in the course. We report four children with Wegener granulomatosis in whom the initial clinical findings suggested Henoch-Schönlein purpura. In two of the patients Wegener granulomatosis was not recognized until after end-stage kidney disease had developed. The course in these patients emphasizes the need for attention to even scant evidence of inflammation of the upper or lower respiratory tract in patients with glomerulonephritis. Appropriate diagnostic studies may then lead to recognition of Wegener granulomatosis and the prompt institution of appropriate treatment.

Radiographic chest contour and pulmonary air leaks in oligohydramnios-related pulmonary hypoplasia (Potter's syndrome).

Review of the clinical records, chest roentgenograms, and autopsy material of 17 infants dying from oligohydramnios related pulmonary hypoplasia confirmed that two roentgen signs appeared with high frequency. These were pneumomediastinum/pneumothorax (82%) and a bell-shaped chest contour (59%). The bell-shaped chest is valuable in predicting the most severe degrees of lung growth impairment in these infants, and was present in all cases in which oligohydramnios was caused by bilateral renal agenesis. Either pneumomediastinum/pneumothorax or the bell-shaped chest configuration, when present alone, are of low diagnostic value as they are relatively nonspecific signs; their combination, however, has predictive value for Potter's syndrome and may be important in evaluation infants with less typical clinical manifestations of Potter's syndrome.

Radiographic findings in early onset neonatal group b streptococcal septicemia.

Chest roentgenograms obtained in the first two days of life from 67 infants with respiratory distress were reviewed to determine whether the radiographic features of group B streptococcal septicemia were diagnostic or distinctive. The retrospective review contained 24 infants with proven and 14 with suspected septicemia, as well as 29 patients with other causes of respiratory distress. The films were reviewed in random order by two pediatric radiologists without their prior knowledge of clinical or laboratory data. Typical radiographic appearance of pneumonia was present in only ten of the 24 proven and two of the 14 suspected cases of group B streptococcal sepsis. The radiographic pattern of respiratory distress syndrome (RDS) was just as common among these patients. The most prominent associated radiographic feature of infants with proven septicemia was cardiomegaly which was significantly increased when compared with infants who had other causes of respiratory distress (P less than .001). X-ray recognition of neonatal group B streptococcal septicemia is limited because of superimposition of roentgen patterns probably related to associated disorders. Pediatrics, 59:1006-1011, 1977, NEWBRON, SEPTICEMIA, GROUP B STREPTOCOCCUS.

Possible adverse effect of methylglucamine diatrizoate compounds on the bowel of newborn infants with meconium ileus.

Gastrografin (methylglucamine diatrizoate) enemas were carried out in 2 newborn infants with meconium ileus. Evacuation was slow and incomplete. Both patients died within 72 hours following enemas from bowel necrosis, perforation and peritonitis. Although it is not possible to implicate Gastrografin directly as the cause, it is suggested that it may have contributed substantially to bowel necrosis. Recent experimental evidence of colonic inflammation and occasionally necrosis caused by Gastrografin lends support to this hypothesis. Caution should be exercised to prevent not only the systemic osmotic effects of Gastrografin, but also potential local injury to the bowel, especially when underlying disease interferes with intestinal viability.

Radiologic features of "adult type" polycystic kidney disease in the neonate.

Two cases are reported of adult type polycystic renal disease (autosomal dominant) presenting in the newborn as a unilateral abdominal mass. The radiographic findings in the involved kidney simulated the ectatic tubules of infantile polycystic disease, yet histologic examination was consistent with the adult variety and both infants had other family members with adult type polycystic kidneys. These cases emphasize some of the ambiguities that exist in the definition and classification of polycystic renal disease.