Kentaro Ono

Pathogenic implications of remnant vitelline structures in gastroschisis

PURPOSE The pathogenesis of gastroschisis is unknown. It may be helpful in understanding its pathogenesis to know the structural relationships among umbilical components including umbilical vessels, urachus, and vitelline structures, and thus, the authors investigated the remnants of vitelline structures in a series of cases of gastroschisis. METHODS Medical records of 41 cases with gastroschisis treated in our institute from 1979 to 2009 were retrospectively reviewed. RESULTS Paraumbilical bands, possible remnants of vitelline structures, were observed in 4 cases (9.8%). All 4 bands were attached to the skin edge of the abdominal defect without incorporation into the umbilical cord. The band ended at the mesentery in 3 cases and at the antimesenteric site of the ileum in the remaining case. Histologic findings showed fibrous tissues in all cases. One was possibly associated with the development of colonic atresia. Another was noticed after silo reduction when herniated bowels became strangulated by the band. The other 2 cases were uncomplicated. CONCLUSIONS Our findings may support the recently proposed hypothesis that the developmental failure of the yolk sac and related vitelline structures to merge with or to be incorporated into the umbilical stalk might be associated with the pathogenesis of the abdominal wall defect in gastroschisis. Paraumbilical bands derived from vitelline structures may possibly cause intestinal ischemia prenatally or postnatally.

Bile duct duplication as a cause of distal bowel gas in neonatal duodenal obstruction.

PURPOSE The aim of this study is to investigate the clinical characteristics of cases of duodenal atresia (DA) which present with bowel gas distal to a typical double-bubble sign through an anomalous bile duct conduit. METHODS Medical records of 57 neonates with duodenal obstruction (atresia or stenosis), presenting with a double-bubble sign and treated at our institute from 1978 to 2010, were retrospectively reviewed. RESULTS Thirteen (23%) of 57 neonates presented with bowel gas distal to the double-bubble sign. Passage of gas through the duodenal stenosis may have occurred in 3 cases, whereas in 9 cases, gas may have bypassed the atresia through an anomalous bifurcated bile duct termination and through the pancreatic duct from the accessory to the main pancreatic duct in one case. A preoperative upper gastrointestinal series was performed in 9 cases, and an anomalous bifurcated bile duct conduit was demonstrated in 5 cases. Severe and prolonged cholestasis necessitating evaluation for biliary atresia was found in 2 patients with anomalous bile duct anatomy. CONCLUSIONS Neonatal DA presenting with distal bowel gas via an anomalous bifurcated bile duct conduit is more common than initially thought and occurs more frequently than duodenal stenosis. These patients might be at risk for cholestasis, possibly owing to duodeno-biliary reflux through the ampulla.

A case of unusual histology of infantile lipoblastoma confirmed by PLAG1 rearrangement

Lipoblastoma, a relatively rare benign adipose neoplasm, predominantly affects children younger than 3 years of age. We herein report the case of a 7-month-old girl with an unusual myxomatous histology of lipoblastoma. A rapidly growing mass was detected in the subcutaneous area of the left buttock. Histologically, the tumor consisted of abundant myxoid stroma exhibiting cellular atypia and a high mitotic activity. Although the histological findings were unusual, the tumor was diagnosed as a lipoblastoma according to both PLAG1 immunohistochemistry and the presence of PLAG1 rearrangement on fluorescence in situ hybridization.

The outcome of an early discharge protocol after appendectomy in children with acute appendicitis

The aim of this study was to evaluate the outcome of an early discharge protocol for pediatric acute appendicitis.

An incarcerated appendix and the ileocecum within a left inguinal hernia in an infant

An 8-month-old boy with a left-sided incarcerated inguinal hernia involving the appendix, cecum, and terminal ileum was successfully managed via an inguinal approach during an emergency operation. A mobile cecum seemed to have contributed to the left-sided incarceration. Only 13 similar cases with the left-sided Amyand’s hernia have been reported in the literature.

Increased pulmonary RhoA expression in the nitrofen-induced congenital diaphragmatic hernia rat model

PURPOSE Persistent pulmonary hypertension remains a major cause of mortality and morbidity in cases of congenital diaphragmatic hernia (CDH). Recently, RhoA/Rho-kinase-mediated vasoconstriction has been reported to be important in the pathogenesis of pulmonary hypertension (PH). Several recent reports have described that fasudil, a potent Rho-kinase inhibitor and vasodilator, could represent a potential therapeutic option for PH. We designed this study to investigate the hypothesis that the expression level of RhoA is increased in the nitrofen-induced CDH rat model. The expression level of Wnt11, an activator of RhoA, was also evaluated. METHODS Pregnant rats were treated with or without nitrofen on gestational day 9 (D9). Fetuses were sacrificed on D17, D19 and D21 and were divided into control and CDH groups. Quantitative real-time polymerase chain reaction was performed to determine the pulmonary gene expression levels of both Wnt11 and RhoA. An immunofluorescence study was also performed to evaluate the expression and localization of RhoA. RESULTS The relative mRNA expression levels of pulmonary Wnt11 and RhoA on D21 were significantly increased in the CDH group compared with the control group (p=0.016 and p=0.008, respectively). The immunofluorescence study confirmed the overexpression of RhoA in the pulmonary vessels of CDH rats on D21. CONCLUSIONS Our results provide evidence that the RhoA/Rho-kinase-mediated pathway is involved in the pathogenesis of PH in the nitrofen-induced CDH rat model. Our data also suggest that the fasudil, a Rho-kinase inhibitor, could represent a therapeutic option for the treatment of PH in CDH.

Massive pneumatic expansion of lymphatic vessel resulting in cystic lesions in the pulmonary parenchyma: a rare case of persistent interstitial pulmonary emphysema in a non-ventilated infant.

We report the case of 2-week-old female infant with cystic lung disease who presented with mild tachypnea and had no history of mechanical ventilation. Chest CT demonstrated multiple air-filled cystic lesions in right upper lobe, and the patient subsequently underwent a right upper lobectomy. Histology revealed cystic lesions located in the pulmonary parenchyma and showed that the lesions were lined by lymphatic endothelium and were communicating with dilated lymphatic vessels in the interstitium. Additionally, multinucleated foreign body giant cells were attached to the lumen of the cyst. On the basis of these findings, we considered this a case of persistent interstitial pulmonary emphysema (PIPE) with massive pneumatic expansion of the lymphatic vessels, resulting in cystic lesions with lymphatic endothelium in the pulmonary parenchyma. While PIPE is extremely rare in term non-ventilated infants, our case demonstrated that this disease should be added to the differential diagnosis of cystic lung diseases with lymphatic endothelium even in infants without mechanical ventilation. When cystic lesions and symptoms persist despite conservative treatment, open or thoracoscopic resection is an appropriate option for diagnosis and treatment.

Prenatally diagnosed large mediastinal lymphangioma: A case report

Thoroughly planned labor with immediate postnatal resuscitation and percutaneous decompression is mandatory for a large mediastinal lymphangioma that compromises the cardiopulmonary function, and close observation with the administration of Eppikajutsuto (TJ‐28), a Japanese herbal medicine, may be a reasonable treatment, especially when resection of the lesion seems technically challenging.