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PURPOSE: To compare histopathology with ADC values in strictured bowel segments in pediatric patients with known Crohn's disease and surgical bowel resection. METHODS: Magnetic resonance enterography (MRE) images of 14 subjects with Crohn's disease who had surgical bowel resection for strictures were retrospectively reviewed. Five of 14 subjects had DWI (b=0, 500, 1000) sequences included in the MRE study. ADC measurements were made by placing ROI's in the strictured bowel wall and compared to full-thickness histologic analysis of resected specimens. ADC values were also compared to control ADC measurements (in normal and inflamed-nonstenotic bowel segments) as well as the mean ADC values of Crohn's patients published in the literature. RESULTS: All five subjects had transmural fibrosis. The mean ADC value with b = 500 was 0.92 ± 0.10 × 10(-3) mm(2)/s and with b = 1000 was 0.8 ± 0.05 × 10(-3) mm(2)/s. There was a significant difference in ADC values between strictures and inflamed-nonstenotic segments (p=0.0143) and between normal and diseased bowel segments (p=0.009-0.0143). CONCLUSIONS: Quantitative ADC measures of transmural fibrosis are lower compared to the reported values of inflammation in Crohn's disease. To our knowledge, this is the first pediatric pilot study to investigate the correlation of quantitative DWI with histology of surgical specimens in pediatric patients with Crohn's disease. Our results are comparable to a recently published study in adult Crohn's patients showing a significant correlation between a decrease in ADC values and fibrosis.

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An unusual case of cavitary Rhodococcus equi pneumonia with endobronchial granulomas in congenital HIV infection is presented. The clinical features and radiological manifestations of pulmonary R. equi infection are discussed.

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OBJECTIVE: To determine the perinatal effects of histologic chorioamnionitis on preterm neonates and the effectiveness of antenatal steroids in the presence of histologic chorioamnionitis. METHODS: We studied neonates at our institution who weighed 1750 g or less at birth from January 1990 through December 1997. The population was stratified primarily by presence of histologic chorioamnionitis and secondarily by exposure to antenatal steroids. Subgroups were compared by various perinatal outcomes and confounding variables. Student t test, chi(2), Fisher exact test, and logistic regression were used for analysis. RESULTS: Among 1260 neonates entered, the placentas of 527 had evidence of histologic chorioamnionitis and 733 did not. Those with histologic chorioamnionitis had a lower mean gestational age, lower birth weight, and higher rate of major neonatal morbidities than those without it. After adjusting for confounding variables, histologic chorioamnionitis independently associated with lower gestational age, lower birth weight, and neonatal death. Among neonates exposed to antenatal steroids who had histologic chorioamnionitis, there was a significantly lower incidence of low Apgar scores (18% compared with 33.5%, P <.001), respiratory distress syndrome (RDS) (39.6% compared with 55.9%, P <.001), intraventricular hemorrhage and periventricular leukomalacia (21.9% compared with 36.9%, P <.001), major brain lesions (7.7% compared with 18.4%, P <.001), patent ductus arteriosus (14.8% compared with 23.7%, P =.018), and neonatal death (8.3% compared with 16.2%, P =.02), with no increase in rate of proven neonatal sepsis (18.3% compared with 14%, P =.24). CONCLUSION: Histologic chorioamnionitis increases major perinatal morbidity through its association with preterm birth and is independently associated with neonatal death. In the presence of histologic chorioamnionitis, antenatal steroids significantly decreased the incidence of RDS, intraventricular hemorrhage and periventricular leukomalacia, major brain lesions, and neonatal mortality, without increasing neonatal sepsis.

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We report the cytogenetic findings in a Wilms tumor from a 4-year-old boy. Karyotypic analysis revealed isochromosomes of 7q and 17q as coexisting clonal aberrations. The finding is notable in view of recent reports of i(7q) as a nonrandom event in Wilms tumor and the emerging evidence for genetic heterogeneity in this tumor.

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Intrascrotal and testicular masses in the pediatric population do not usually present as treatment dilemmas. Herein, we report an unusual case of an enlarging, intrascrotal capillary hemangioendothelioma in a 3-month-old male infant. Conservative management including watchful waiting in the case of purely cutaneous scrotal hemangiomas is the treatment of choice. However, scrotal lesions with a palpable testicular or scrotal mass do not lend themselves to conservative treatment and, as in this case, exploration with intraoperative evaluation and excision is warranted.

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BACKGROUND: Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years, and there are now a few reviews based on up to 21 cases. The purpose of this article is to review the clinical and biologic features of prenatally diagnosed neuroblastoma based on a review of 55 cases. METHODS: A review was conducted of 3 cases seen at the study institution and 52 other cases reported thus far in the literature. RESULTS: Prenatal diagnosis was made usually after 32 weeks of gestation. Approximately 93% of the tumors were adrenal in origin, and 44% of these were cystic. Thirty-seven patients (67%) had Stage I disease, 12 (22%) had Stage IV-S disease, and only 3 (5%) had Stage IV disease. The DNA index was favorable (> 1) in 14 of 16 patients studied. None of these 16 patients studied had amplification of the N-myc oncogene. Catecholamines were elevated in only 33% of the patients. The liver was the most common site of dissemination, which was observed in 25% of patients; bone involvement was not observed in any patient. Ultrasonography failed to detect existing hepatic metastasis in three patients. Primary surgical resection was performed in 47 patients (85%). Chemotherapy was given to five patients and radiotherapy to three. Of the 50 patients for whom follow-up information was available, 45 (90%) were alive at a range of 2-120 months from diagnosis. CONCLUSIONS: Prenatally diagnosed neuroblastomas are predominantly adrenal in origin and frequently cystic. The liver is the most common site of dissemination and bone involvement is notably absent. The vast majority of these infants have a favorable stage of disease (I, II, and IV-S) and favorable biologic features, and consequently have an excellent prognosis. Although surgery alone is curative for most patients, a period of observation may avoid surgery in some individuals who may achieve spontaneous regression.

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Polypoid lesions of the gallbladder in children are rare. We report a case of a gallbladder polyp in a 14-year-old boy who presented with recurrent right upper quadrant abdominal pain. Ultrasound examination of the abdomen revealed a polypoid lesion of the gallbladder. His symptoms resolved after laparoscopic cholecystectomy. Histological examination of the gallbladder demonstrated a benign adenomatous polyp. Although the experience with polypoid lesions of the gallbladder in children is limited, we currently recommend cholecystectomy because these lesions are associated with acalculous cholecystitis, and because their long-term effects are unknown.

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We report a case of congenital pulmonary myofibroblastic tumor, and review prior reports of this rare neoplasm to demonstrate its clinically benign behavior despite histologic features previously interpreted as sarcoma. The patient, a female neonate, presented with severe respiratory distress after cesarean section delivery. A large radio-opaque mass was detected in the right hemithorax and resected by right bilobectomy. The tumor mass, confined to the lung, was composed of interlacing fascicles of plump spindle cells showing myofibroblastic differentiation and complex cytogenetic abnormalities. Though sarcomatous in appearance, with highly cellular areas and numerous mitoses, there has been neither tumor recurrence nor metastases. The patient remains alive and well 1 year after surgery. Review of the few other reported cases confirms the uniformly benign behavior of this tumor.

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OBJECTIVE: Neonatal intraventricular hemorrhage and periventricular leukomalacia have a strong correlation with eventual neurologic deficit. Our objective was to correlate obstetric factors with the development of these lesions. STUDY DESIGN: Seven hundred forty-five consecutive inborn neonates with birth weights from 500 to 1750 gm were divided into three clinical groups: premature rupture of membranes, refractory preterm labor with intact membranes, and delivery initiated by the physician for maternal or fetal indications. Neonatal neurosonography was performed on days 3 and 7 of life and results were described as normal or abnormal. Abnormal scans included intraventricular hemorrhage seen within 3 days and echodense or echolucent periventricular leukomalacia seen within 7 days of life. Major abnormalities included intraventricular hemorrhage grades 3 and 4, intraventricular hemorrhage with periventricular leukomalacia, and echolucent periventricular leukomalacia. Abnormal scans were correlated with groups of origin and clinical and histologic chorioamnionitis. RESULTS: Abnormal scans occurred in 33% of cases of premature rupture of membranes and in 38.9% of cases of preterm labor compared with 17.7% of physician-initiated cases (p < 0.000001). Major lesions occurred in 17.6% of cases of premature rupture of membranes, 21.4% of cases of preterm labor, and 1.1% of physician-initiated cases (p < 0.0000001). Clinical chorioamnionitis occurred in 19.7% of cases of premature rupture of membranes, 11.9% of cases of preterm labor, and 1.1% of physician-initiated cases (p < 0.001) and was associated with a significant increase in the incidence (p < or = 0.005) and severity (p < or = 0.007) of these lesions. Histologic chorioamnionitis occurred in 59.9% of cases of premature rupture of membranes, 43.2% of cases of preterm labor, and 8% of physician-initiated cases and did not correlate significantly with the incidence or severity of abnormal scans. These findings were independent of gestational age. CONCLUSIONS: The incidence and severity of intraventricular hemorrhage and periventricular leukomalacia were significantly increased in premature rupture of membranes and preterm labor compared with the physician-initiated cases. Clinical chorioamnionitis increased the incidence and severity of these lesions.

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PURPOSE: We report the unrecognized association of Langerhans cell histiocytosis (LCH) with partial DiGeorge syndrome. PATIENT AND METHODS: A 7-week-old infant with endocrine and immunologic characteristics of DiGeorge syndrome displayed multisystem involvement of Letterer-Siwe disease at birth. RESULTS: Despite vigorous medical support and chemotherapy, she died at 9 months of age with multisystem failure. CONCLUSIONS: This case supports the role of the thymus n the pathogenesis of LCH.

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PURPOSE: This report describes a female infant with stage 4 multifocal ganglioneuroblastoma with gastric involvement. PATIENT: The patient had a right cervical tumor, a left posterior mediastinal tumor, bilateral adrenal tumors, and bony and bone marrow metastases. The tumor cells were diploid and lacked N-myc gene amplification. The gastric involvement, which did not produce clinical symptoms, was only detected by meticulous exploration during laparotomy. RESULTS: Our patient achieved only a partial response to alternating cycles of cyclophosphamide, vincristine, and adriamycin; and etoposide and cisplatin. She currently has stable, unresectable disease with elevated catecholamines. CONCLUSIONS: Multifocal ganglioneuroblastomas may arise from either neuroblastic rests or aberrant deposits of neuroblasts. The latter mechanism may have accounted for our patient's gastric tumor. Patients with multifocal ganglioneuroblastomas warrant meticulous radiographic and surgical evaluation to completely document the full extent of disease, and to ensure appropriate staging and therapy.

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We describe a pediatric patient with dyskeratosis congenita, whose symptoms included abdominal pain, vomiting, dysphagia, and hematochezia. Gastrointestinal symptom are prominent in this rare genetic disorder.

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The purpose of our study was to determine whether Borrelia burgdorferi spirochetes were present in placentas of asymptomatic women with reactive Lyme serology using a silver stain, and to confirm the identity of the spirochetes by polymerase chain reaction (PCR). Sixty placentas of asymptomatic women with ELISA-positive or-equivocal serology for Lyme antibodies during pregnancy were examined for spirochetes using a silver stain. The results of the ELISA serology were confirmed by Western blot analysis. PCR amplification for B. burgdorferi was performed on placentas identified to have spirochetes and on a group of placentas negative for spirochetes. Spirochetes were identified by silver staining in 3 (5%) of the 60 placentas. PCR confirmed B. burgdorferi nucleotide sequences in 2 of the placentas. The 5 women had equivocal Lyme ELISA and negative syphilis serology. The results of the Western blot analysis were negative in 2 cases and indeterminate in 1 case. Six controls were negative for spirochetes by silver staining and PCR. A normal perinatal outcome was observed in all cases. Spirochetes identified in placental tissue of pregnancies with reactive Lyme serology were confirmed by PCR to be B. burgdorferi. There was no relationship between the presence of placental spirochetes and the results of Lyme serology or the pregnancy outcome.

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The incidence of pneumothorax in HIV-infected children has not been reported. In adults with AIDS, pneumothorax has been described exclusively in association with Pneumocystis carinii pneumonia (PCP). We report the cases of three children with AIDS, one with lymphoid interstitial pneumonitis (LIP) without evidence of PCP and two with PCP, all of whom developed spontaneous pneumothorax (SP). On presentation, none of the children had any risk factors for the development of pneumothorax, but all had radiographic evidence of subpleural cystic lesions and bilateral pleural adhesions. None of the patients responded to conservative medical management, which included chest tube thoracostomy and chemical pleurodesis. Two patients underwent pleurectomy that resulted in resolution of the pneumothorax. Both patients with PCP who developed pneumothorax died, but the patient with LIP and SP has had no recurrences of any serious respiratory problems 3 years after pleurectomy and excision of the intrathoracic cysts.

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A newborn with hypoalbuminemia, hematochezia, and hypertrophic gastropathy is described. These features are similar to those reported in association with prostaglandin therapy in newborns, as well as those described in so-called Ménétrier's disease. Very few cases of hypertrophic gastropathy have been reported in children and fewer still verified histologically; only three other cases have been reported in newborns. The clinical and pathologic features of hypertrophic gastropathy in children, as well as its close association with eosinophilic gastroenteritis and with prostaglandin therapy, are discussed.

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The purpose of this study was to determine the underlying cause of abnormal umbilical artery Doppler velocimetry in pregnancies complicated by insulin-dependent diabetes, by evaluating placental morphology and correlating Doppler results with pregnancy outcome. Our study population consisted of 14 pregnancies with normal Doppler results and 11 pregnancies with abnormal Doppler results. Quantitative placental examinations were performed. Maternal and perinatal characteristics were analyzed. Infants from the abnormal Doppler group had a statistically significantly higher incidence than those from the normal Doppler group of hyperbilirubinemia, Cesarean delivery for fetal distress, and neonatal intensive care stay of 2 or more days. Women with abnormal Doppler results delivered earlier (36 vs. 38 weeks) and had infants of lower birth weight (3079 vs. 3629 g). They were also three times more likely to have poor glycemic control (relative risk = 3.2; p = 0.0067). The placentae of both groups showed no statistically significant differences in placental weight, number of tertiary stem villi, number of small muscular arteries, or mean arterial width. Our findings suggest a functional rather than structural placental process as the cause of the abnormal Doppler results in pregnancies complicated by diabetes. An association between glycemic control and Doppler velocimetry is suggested.

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A 4-week-old infant presenting with neonatal cholestasis was found to have congenital toxoplasmosis and biliary atresia. This is the first patient in which their coincidence is reported. Because biliary atresia can coexist with either congenital infection or inborn errors of metabolism, evaluation for an obstructive etiology of jaundice in infants with a recognized cause of intrahepatic cholestasis is necessary.

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