Thoracoscopic surgery in children in the Kyushu area of Japan.

BACKGROUND: Recently, minimally invasive thoracoscopic surgery has offered several options for both the diagnosis and surgical treatment in the field of pediatric surgery. This report reviews the surgical experience in order to assess the problems including the complications, encountered during thoracoscopic surgery in children under 15 years of age in the Kyushu area, Japan. METHODS: From 1993 to 2005, 153 boys and 103 girls underwent thoracoscopic surgery for chest disease. Pertinent information included number of operations for each year, sex, age, indication, treatment procedure, follow-up duration, cause of conversion to thoracotomy, method of treatment, and complication. RESULTS: There has gradually been an increase in the number of operations every year. These included procedures for funnel chest, n= 121; pneumothorax, n= 25; mediastinal tumor, n= 25; palmar hyperhydrosis, n= 23; neuroblastoma, n= 11; and cystic lung disease, n= 10. A large portion of the surgery was for patients under 1 year of age excluding neonates. Approximately half of the procedures were for the excision of a neuroblastoma. Approximately half of the patients older than 5 years of age underwent Nuss procedure for funnel chest. Almost all of the pneumothorax and palmar hyperhydrosis surgery was for patients 14 or 15 years old. Three patients required a conversion to a standard thoracotomy. Six complications occurred. There was no mortality associated with the thoracoscopic surgical procedures. CONCLUSIONS: Thoracoscopy is a useful diagnostic and therapeutic tool for both infants and children. Various thoracoscopic complex procedures have been performed both safely and effectively.

Neonatal gastrointestinal perforation.

Infants, especially extremely low birth weight infants (ELBWIs, birth weight <1,000 g) continue to have a high mortality after gastrointestinal (GI) perforation. In Japan, the overall mortality rate for neonates under 30 days having GI perforation was 31.6% in 2003. From 1974 to 2003, 34 cases of GI perforation in neonates were treated surgically in Fukuoka University Hospital. The overall mortality rate was 50% (17 of 34). Etiologies included necrotizing enterocolitis (NEC) (35.3%), meconium peritonitis (25%), idiopathic (25%), and gastric perforation (11.8%). The present series was divided into four groups: survival and non-survival neonates of the early (1974 to 1997) and recent (1998 to 2004) periods. Several prognostic factors of neonatal GI perforation were compared between several groups. The gestational week (GW) at birth, birth weight (BW) and weight at operation were significantly lower for non-surviving neonates in the recent period compared with the other three groups. Although a real improvement in surgical outcome was noted with improved neonatal intensive care management, the mortality rate was still high, especially in extremely premature cases under both 1,000 g and 29 GWs. The vast majority of these extremely premature babies thus comprised the NEC patients. It is therefore necessary to substantially improve the medical treatment level for such premature babies.

An antenatal appearance of megacystis-microcolon-intestinal hypoperistalsis syndrome.

We herein present a megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) case followed by ultrasound (US) examinations before birth. During the prenatal US examination of a 34-year-old woman, an enlarged bladder with bilateral hydronephrosis and hydroureter of the fetus were detected. The amniotic fluid was normal in the second trimester but polyhydramnios was observed in the third trimester. A female baby was born by cesarian section weighing 2632 g. Imaging studies detected an enlarged bladder with bilateral hydronephrosis, hydroureter and microcolon. Laparotomy on the 9th day confirmed a short small bowel with caliber change and the existence of ganglion cells and plexus of the nerve in the intestine. These findings correlated with the previously reported characteristics of MMIHS. An enlarged bladder in the second trimester and polyhydramnios in the third trimester have been reported in many cases of MMIHS. These findings may thus help to accurately diagnose MMIHS before birth.

Modification of the endoscopic management of congenital duodenal stenosis.

This report documents a new endoscopic management modality for congenital membranous stenosis in the third portion of the duodenum. Standard approaches to duodenal stenosis in newborns include a laparotomy with an enteroenterostomy, bypassing the obstruction, or a duodenoduodenostomy with excision. We successfully developed a modification of the endoscopic treatment modality for congenital duodenal diaphragm.

Pulmonary endocrine cells in hypoplastic lungs due to foetal urinary tract obstruction: a microscopic immunohistochemical study.

METHODS: We performed a urinary tract obstruction (UTO) surgical procedure at 93-107 days' gestation in lambs to investigate the relationship between pulmonary hypoplasia and the appearance of pulmonary endocrine cells by quantitative analysis of respiratory tract cells using light microscopic immunohistochemistry. RESULTS: UTO produced a significant reduction in lung weight, lung/body weight ratio, air capacity, air capacity/body weight ratio (p < 0.01) and radial alveolar count (p < 0.05), which indicated the presence of lung hypoplasia. These foetuses also showed a significant increase in the number of neuron-specific enolase (NSE)-positive pulmonary endocrine cells, expressed as the number of NSE-positive cells per bronchus (p < 0.01) or bronchiole (p < 0.05), the number of NSE-positive cells per unit perimeter of bronchus or bronchiole (p < 0.01), and the number of NSE-positive cells per unit bronchial or bronchiolar surface area (p < 0.01). CONCLUSION: These results suggest that UTO significantly retards and modifies the structural growth and functional development of pulmonary endocrine cells in NSE expression. We speculate that pulmonary endocrine cells and their mediators may play a role in the problems associated with UTO during intrauterine life.

Fetal case of congenital cystic adenomatoid malformation of the lung: fetal therapy and a review of the published reports in Japan.

We herein report a case of type I congenital cystic adenomatoid malformation of the lung (CCAML) with non-immune hydrops fetalis (NIHF), a mediastinal shift and polyhydramnios diagnosed at 24 weeks' gestation by ultrasonography. The fetus was treated with a cyst-amniotic shunt at 29 weeks' gestation. Following a postnatal whole resection of the right lung, postpneumonectomy syndrome appeared and, as a result, the infant died 13 months after delivery due to respiratory failure. Only 19 cases demonstrating CCAML associated with NIHF have been reported previously in Japan. Four cases showed a spontaneous resolution of NIHF, while 5 cases with type I CCAML, which all underwent fetal intervention, demonstrated an excellent outcome.

Biliary atresia associated with jejunal atresia and a review of the literature in Japan.

An unusual case of biliary atresia with jejunal atresia is herein described. Only 12 cases demonstrating biliary atresia associated with a jejunal atresia have been previously reported in Japan. The pathogenesis of biliary atresia is thought to be secondary to the influence of jejunal atresia.

Quantitative study of pulmonary endocrine cells in fetal, postnatal and adult sheep.

Using light microscopic immunohistochemistry, neuron-specific enolase (NSE)-positive endocrine cells were quantitatively analyzed in the sheep lung during different stages of development from the canalicular stages to adulthood. In all stages, NSE-positive endocrine cells were usually located in the bronchi and bronchioles as solitary cells, although a few NSE-positive cell clusters, the so-called neuroepithelial bodies, were found in some places. The number of NSE-positive endocrine cells decreased with advanced stages of gestation. In the late alveolar stage, the number of NSE-positive endocrine cells reached its bottom during the fetal period. There was a gradual upturn after birth. The overall pattern of growth and differentiation of the endocrine cells is most likely species-related and depends on the state of airway development; the number of the endocrine cells of almost all animals, excluding the sheep, in relation to the size of the lung reaches a peak in the late fetal and early neonatal periods and decreases shortly thereafter. NSE-positive endocrine cells were also predominantly located in the large airways during the early stage of development (canalicular stage), and were found more frequently in the small peripheral airways towards the term. These results show the number of NSE-positive endocrine cells in the sheep to be different from that seen in other species.

Double atresia of hindgut with ileal stenosis: a case report.

An unusual case of imperforate anus with sigmoid colonic atresia and ileal stenosis is described. Only six cases of imperforate anus associated with colonic atresia have previously been reported in Japan. The possibility of other alimentary obstructions should thus be considered when anorectal anomalies are observed.

Immunohistochemical distribution of surfactant apoprotein-A in congenital diaphragmatic hernia-II.

Morphometric analyses of the immunohistochemical expression of surfactant apoprotein A (SP-A) were carried out on the bronchioles of human congenital diaphragmatic hernia (CDH) neonates and then compared with those in a gestational and postnatal age-matched control group. There was no difference in SP-A expression between lung specimens of the control group and unaffected lungs in the CDH group. However, compared with both these lungs, the ipsilateral lungs of the CDH group showed a significant increase in SP-A expression, namely, the number of SP-A-positive cells per bronchiole, the number of SP-A-positive cells per unit perimeter of bronchiole, and the number of SP-A-positive cells per unit bronchiolar surface area. These results suggest that in lungs of CDH cases, especially ipsilateral lungs, there is a possible delay in the functional maturation or development of SP-A synthesis by the bronchiole, and this retardation may play a role in the postnatal respiratory insufficiency observed in CDH patients.

Iatrogenic ileal perforation: an accidental clamping of a hernia into the umbilical cord and a review of the published work.

This report describes the case of an ileal perforation secondary to clamping of the exomphalos minor in 3-day-old girl. Eighteen accidental clamping or cutting cases were found in the published work. It is thought that this defect resulted from either the clamping, ligation or cutting of the bowel in a small unrecognized omphalocele. These complications are extremely rare, but should be held in mind when performing an umbilical ligation.

Ultrastructural evaluation of type II pneumocytes in the hypoplastic lung of rabbit fetuses induced by oligohydramnios.

AIM: The present study was carried out using electron microscopy to evaluate the expression of lamellar bodies in type II pneumocytes of fetal rabbit lungs with hypoplasia induced by oligohydramnios. METHODS: Using an amniotic shunting rabbit model, the lungs obtained from 32 fetuses, including 16 experimental fetuses (shunting group) and 16 control fetuses (control group), were used for electron microscopic studies. At least 250 type II pneumocytes per fetus were photographed. The number of lamellar bodies per type II pneumocyte was counted. RESULTS: The number of lamellar bodies per type II pneumocyte of the shunting group was consistently and significantly less than that of the control group (5.49 +/- 2.07 vs 7.58 +/- 2.08; P < 0.01). CONCLUSIONS: These results suggest that the occurrence of hypoplastic lungs induced by oligohydramnios is associated with an immaturity of the surfactant system, especially the expression of the lamellar bodies of type II pneumocytes.

Effect of maternal dexamethasone treatment on the type II pneumocytes in hypoplastic lung by oligohydramnios: an ultrastructural study.

A previous study documented the effects of maternal corticosteroid treatment on structural growth and functional development in fetal lungs associated with pathogenic conditions such as oligohydramnios using immunohistochemical and morphometric analyses. The purpose of the present study was to examine the effect of maternal dexamethasone treatment the expression of lamellar body in type II pneumocytes of the fetal rabbit lungs with hypoplasia induced by oligohydramnios using electron microscopy. Using an amniotic shunting rabbit model, pregnant rabbits were injected intravenously with either 0.1 ml of saline or 0.25 mg/kg/day of dexamethasone in 0.1 ml of saline 48 and 24 h before the delivery of fetuses, at day 30 of gestation. The number of lamellar bodies per type II pneumocyte was counted in each group using electron micrographs. The number of lamellar bodies per type II pneumocyte in the lungs of the shunted group that received saline was consistently and significantly less than that of the other three groups (5.49 +/- 2.07 vs. 7.34 +/- 2.27: shunted group that received dexamethasone, 7.58 +/- 2.08: non-shunted group that received saline, 7.79 +/- 1.90: non-shunted group that received dexamethasone; P < 0.01). These results suggest that maternal dexamethasone treatment accelerates the maturation of the surfactant system, especially the expression of lamellar bodies in type II pneumocytes, even in hypoplastic lungs induced by oligohydramnios.

Endoscopic extraction of a peritoneal loose body: a case report of an infant.

A 2-month-old female infant with a peritoneal loose body presenting as an abdominal mass and urinary tract infection is described. Peritoneal loose bodies are usually found in the pelvis of adult patients. A peritoneal loose body should be considered in the differential diagnosis of a mobile lesion found in the abdomen.

A case of transverse colon volvulus in a child and a review of the literature in Japan.

A case of transverse colon volvulus in a child with mental retardation and epilepsy is described. Previously reported cases in Japanese children are reviewed. A 540 degrees, counterclockwise volvulus of the transverse colon caused ischemia requiring resection. Possible factors related to pathogenesis are discussed, and diagnostic and therapeutic measures are outlined. Volvulus of the transverse colon is extremely rare in children.

Intestinal obstruction caused by congenital cytomegalovirus infection: report of a case.

We report a case of coexistent congenital cytomegalovirus (CMV) infection with pseudo-Hirschsprung's disease in a female infant born at 39 weeks' gestation. The serologic data, urinary viral culture, and gene expression by in situ hybridization all suggested a CMV infection, and the pathologic findings of resected bowel specimens showed aganglionosis. Thus, congenital CMV infection may be etiologically associated with Hirschsprung's disease in some children.