Debra Beneck

Obstetric antecedents of intraventricular hemorrhage and periventricular leukomalacia in the low-birth-weight neonate☆☆☆★

OBJECTIVEnNeonatal 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.nnnSTUDY DESIGNnSeven 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.nnnRESULTSnAbnormal 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.nnnCONCLUSIONSnThe 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.

Histologic chorioamnionitis, antenatal steroids, and perinatal outcomes.

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, χ2, 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.

Atypical morphologic presentation of biliary atresia and value of serial liver biopsies

Background Liver biopsy findings are important in diagnosing extrahepatic biliary atresia. Diffuse ductular proliferation is a characteristic finding. We describe four patients with conjugated hyperbilirubinemia in whom the initial liver biopsy findings showed a lack of ductular proliferation, despite subsequent development of biliary atresia. Results On initial biopsy, paucity of intrahepatic bile ducts was present in three of four patients, with a bile duct to portal space ratio of 0.3 to 0.4 (normal, 0.9–1.8). A normal bile duct to portal space ratio of 1.0 was observed in the fourth patient. Ductular proliferation became apparent in three subjects between 9 and 12 weeks of age, and biliary atresia was noted at the time of a Kasai portoenterostomy. The fourth child had well-developed biliary cirrhosis at liver transplantation. Conclusions Changes characteristic of biliary atresia may appear even after 9 weeks of age. Bile duct paucity and normal bile duct to portal space ratio do not preclude the subsequent development of biliary atresia. Infants with unexplained conjugated hyperbilirubinemia and acholic stools should undergo sequential liver biopsies until clinical improvement occurs or until biliary atresia can be excluded from the differential diagnosis.

Campylobacter pylori-related gastrointestinal disease in children

Over a one-year period, 95 children and adolescents presenting with epigastric pain and/or vomiting, and without associated risk factors for development of peptic disease, underwent endoscopic antral biopsies for pathologic diagnosis and to detect presence of Campylobacterss. pylori (C. pylori). Additional biopsies of the esophagus, stomach, and duodenum were obtained for histologic evaluation. C. pylori was identified in 16 patients (16.8%), all of whom had evidence of acute and/or chronic gastritis. Significant discriminating factors between C. pylori-positive and -negative subjects included age at presentation (positive vs negative=14.6 vs 9.9 years, P<0.01, biopsy-confirmed gastritis (100% vs 30.4%, P<0.001), and diagnosis of duodenitis alone (0% vs 46.8%, P<0.001). Risk for bacterial colonization was significantly higher in the presence of endoscopic gastritis (P<0.001). Among C. pylori-positive patients, none responded to standard antiulcer therapy (H2-receptor antagonists, antacids). Symptomatic and histologic remission was achieved utilizing combined therapy with bismuth subsalicylate and antibiotics. Seven of 79 C. pylori-negative patients with biopsyproven gastritis who responded poorly to antisecretory therapy had the organism identified in follow-up antral biopsies; these patients improved clinically following treatment for C. pylori. These data suggest that C. pylori is a significant factor in the etiology of upper gastrointestinal tract inflammatory disease in pediatrics, and presence of the organism should be evaluated, particularly in children with evidence of acute and/or chronic gastritis.

Congenital pulmonary myofibroblastic tumor : A case report with cytogenetic analysis and review of the literature

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.

Combined occurrence of chyloperitoneum and chylothorax after surgery and chemotherapy for Wilms' tumor.

Chyloperitoneum is an extremely rare complication of abdominal surgery in children and a combined occurrence of chylothorax and chyloperitoneum after abdominal surgery has never been reported in children. Chylous ascites usually occurs as a result of operative trauma to the thoracic duct, cisterna chyli, or its tributaries. About one third of all patients with chylous ascites after retroperitoneal lymph node dissection also develop secondary chylothorax. Diaphragmatic defects have been shown to be responsible for the occurrence of chylothorax secondary to chyloperitoneum. Congenital diaphragmatic weakness may result in evagination of the peritoneum causing diaphragmatic blebs, the rupture of which results in the movement of the peritoneal fluid into the pleural cavity. In the authors patient, the rent in the diaphragm that occurred during surgery was probably responsible for the chylothorax. The role of chemotherapy, if any, in the pathophysiology of this complication is unknown. Total parenteral nutrition (TPN) is a simple and effective treatment for postoperative chylous effusions. Surgical treatments such as abdominal exploration for the repair of leaking lymphatics and peritoneovenous shunt should be reserved for patients who fail TPN.

Resolution of nonsyndromic paucity of intrahepatic bile ducts in infancy

N o n s y n d r o m i c pauc i ty of in t rahepat ic bile ducts (NPIBD) during infancy is of ten assoc ia ted with pe rs i s t ence of h is topa thologica l abnormal i t i es on repea ted l iver b iops ies (1). Some infants wi th this condi t ion deve lop p rogress ive l iver d i sease resulting in c i r rhosis and l iver failure. H o w e v e r , bile duct pauc i ty m a y pers is t even under condi t ions of clinical improvemen t or resolut ion of choles ta t ic symptoms. We descr ibe a pat ient d iagnosed with N P I B D at 7 w e e k s of age, who had normal iza t ion of l iver enzymes by 5 yea r s of age. At that t ime, a percut aneous l iver b i o p s y d e m o n s t r a t e d no rma l bi le ducts in v i r tua l ly all por ta l t rac ts . This unusual case demons t ra t ing clinical and h i s topa thologica l resolut ion of N P I B D is d i scussed with regard to the pa thogenes i s of this form of choles ta t ic l iver disease.

Renal Tubular Dysgenesis in an Hydropic Fetus with Trisomy 21: a Case Report with Literature Review

Renal tubular dysgenesis (RTD) is a rare form of noncystic renal disease characterized by paucity or absence of proximal renal tubules. Always lethal in the perinatal period, it has been associated with Potter sequence and with other congenital malformations. An autosomal recessive inheritance has been suggested. We present a case of renal tubular dysgenesis associated with fetal hydrops and trisomy 21, with a review of relevant literature.

Confirmation of Borrelia burgdorferi Spirochetes by Polymerase Chain Reaction in Placentas of Women with Reactive Serology for Lyme Antibodies

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.

Intrascrotal hemangioendothelioma in infancy

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.