Upregulated TSP1 and CD47 expression in the lung in nitrofen-induced congenital diaphragmatic hernia.

BACKGROUND: Persistent pulmonary hypertension remains a major cause of mortality and morbidity in congenital diaphragmatic hernia (CDH). The secreted glycoprotein thrombospondin-1 (TSP1), a ligand for receptor CD47, is widely expressed on both systemic and pulmonary vascular cells. TSP1-CD47 signaling has been reported to be one of the pathogeneses of pulmonary hypertension (PH). METHODS: After creating a nitrofen-induced CDH rat model, fetuses were sacrificed on D17, D19 and D21 and divided into a control group and a CDH group. Quantitative real-time polymerase chain reaction was performed to determine the pulmonary gene expression of TSP1, CD47 and Runx3 (a regulator of TSP1). An immunofluorescence study was performed to evaluate the expression and localization of TSP1, CD47 and Runx3. RESULTS: The relative mRNA expression of pulmonary TSP1, CD47 and Runx3 on D21 was significantly increased in the CDH group (p = 0.005, p = 0.001, p = 0.046, and p = 0.002, respectively). The immunofluorescence study also confirmed the overexpression of TSP1, CD47 and Runx3 in the CDH group. CONCLUSION: Our results provide evidence that TSP1-CD47 signaling is involved in the pathogenesis of PH in a nitrofen-induced CDH model. Our data suggest that anti-CD47 antibodies can be novel therapeutic targets for the treatment of PH in CDH.

Optimal First-Line Antibiotic Treatment for Pediatric Complicated Appendicitis Based on Peritoneal Fluid Culture.

PURPOSE: Consensus is lacking regarding the optimal antibiotic treatment for pediatric complicated appendicitis. This study determined the optimal first-line antibiotic treatment for pediatric patients with complicated appendicitis based on peritoneal fluid cultures. METHODS: This retrospective study examined the cases of pediatric patients who underwent appendectomy for complicated appendicitis at our institution between 2013 and 2019. Peritoneal fluid specimens obtained during appendectomy were cultured for the presence of bacteria. RESULTS: Eighty-six pediatric patients were diagnosed with complicated appendicitis. Of them, bacteria were identified in 54 peritoneal fluid samples. The major identified bacteria were Escherichia coli (n=36 [66.7%]), Bacteroides fragilis (n=28 [51.9%]), α-Streptococcus (n=25 [46.3%]), Pseudomonas aeruginosa (n=10 [18.5%]), Enterococcus avium (n=9 [16.7%]), γ-Streptococcus (n=9 [16.7%]), and Klebsiella oxytoca (n=6 [11.1%]). An antibiotic susceptibility analysis showed E. coli was inhibited by sulbactam/ampicillin in 43.8% of cases versus cefmetazole in 100% of cases. Tazobactam/piperacillin and meropenem inhibited the growth of 96.9-100% of the major identified bacteria. E. coli (100% vs. 84.6%) and P. aeruginosa (100% vs. 80.0%) were more susceptible to amikacin than gentamicin. CONCLUSION: Tazobactam/piperacillin or meropenem is a reasonable first-line antibiotic treatment for pediatric complicated appendicitis. In the case of aminoglycoside use, amikacin is recommended.

A clinical review of ovarian tumors in children and adolescents.

PURPOSE: To determine the features which predict torsion and the pre-operative indicators of malignancy in cases of ovarian torsion in ovarian tumors (OTs) in children. METHODS: The medical records of 35 pediatric patients who underwent surgery for OT, except for neonate cases, from 1997 to 2018 at our institution were reviewed retrospectively. RESULTS: The pathological diagnosis was mature teratoma in 17, immature teratoma in 9, yolk sac tumor in 3, and others in 6. The preoperative diagnosis, which was made based on the imaging findings and the serum tumor marker values, matched with the pathological diagnosis in 29/35 (83%). Ovarian torsion occurred in 14/35 (40%). All but one case that presented with torsion had intermittent abdominal pain as the primary symptom. The preoperative white blood cell count was significantly higher in cases where ovary preservation was impossible than where it was possible (p = 0.01) among the cases presenting with torsion. CONCLUSION: Preoperative imaging findings and the serum tumor marker values enabled us to make an accurate preoperative diagnosis. Patients with intermittent abdominal primary symptoms were more likely to have ovarian torsion than those without such symptoms, and leukocytosis may indicate irreversible ischemic changes in the affected ovary.

Long-term follow-up in surgical newborns: A single-institution experience.

BACKGROUND: To assess the actual experiences of long-term follow-up and discuss ways to improve care during the period from childhood to adulthood in newborns who have undergone surgery. METHODS: A total of 306 patients with congenital anomalies requiring newborn surgery who survived to discharge from 1994 to 2013 were eligible for inclusion. Survivors with severe chromosomal and cardiac anomalies were excluded. Patients with myelomenigocele, urogenital anomalies and miscellaneous diagnoses were also excluded. Patients with Hirschsprung's disease were excluded since many of them underwent surgery after the neonatal period. Patients with hypertrophic pyloric stenosis were also excluded since their duration of follow-up was too short for this study. RESULTS: According to the follow-up status, survivors were categorized into 4 groups: under follow-up as an outpatient (UF, n = 67), moved (MV, n = 60), follow-up suspended by doctor (Sus, n = 87), and lost to follow-up (LF, n = 92). The incidence of active medical problems was high, and the duration of follow-up was significantly longer in the survivors with esophageal atresia, congenital diaphragmatic hernia and high-type anorectal malformations than in those with other anomalies. Survivors followed by pediatric surgeons alone, free from active medical problems or free from adverse events during the initial hospitalization were at risk of being LF. CONCLUSIONS: More than 30% of the surgical newborn cases were LF. Disease-specific and standardized multidisciplinary follow-up programs that increase both children's and parents' satisfaction and compliance are needed. (230/250 words).

Pediatric ovarian immature teratoma: Histological grading and clinical characteristics.

BACKGROUND: Ovarian immature teratomas (ITs) are relatively rare among all pediatric ovarian tumors. The histological grading for ovarian ITs, which ranges from 1 to 3, is based on the proportion of immature neuroepithelial component. Higher-grade ITs in adults are treated as malignant neoplasms and require adjuvant chemotherapy. However, there is no consensus on the therapeutic management of pediatric ovarian ITs. The aim of our study was to analyze the histological grades and clinical characteristics of ovarian ITs in pediatric patients. METHODS: This retrospective chart review consisted of seven patients, including one, three, and three patients with histological grade 1, 2, and 3 pediatric ovarian ITs, respectively, who were treated at our institute between 2000 and 2016. Collected data comprised age, alpha-fetoprotein (AFP) level, clinical stage, tumor size, treatment, and prognosis. RESULTS: The median age and AFP levels of patients with grade 1, 2, and 3 ovarian ITs were 8, 7, and 10 years and 37, 112, and 221 ng/ml, respectively. All cases were Children Oncology Group (COG) stage I and International Federation of Gynecology and Obstetrics (FIGO) stage IA. All patients had unilateral tumors in the right ovary. The median tumor sizes of the grade 1, 2, and 3 IT patients were 104, 160, and 100 cm2, respectively. All patients underwent primary open surgery alone. Two patients, including one patient each with grade 2 and 3 ITs, underwent tumor enucleation as ovary-sparing surgery, whereas the remaining five patients underwent unilateral salpingo-oophorectomy. The median follow-up was seven years, and all cases achieved event-free survival. CONCLUSIONS: Clinical characteristics of patients with grade 3 ovarian ITs were relatively older and had higher AFP levels than those with lower-grade ITs. According to our patient's clinical course and prognosis, COG stage I pediatric ITs should be treated by surgery alone and that postoperative chemotherapy is unnecessary even for those with grade 3 ITs as well as patients with rather low AFP levels. LEVEL OF EVIDENCE: IV.

Outcome of early discharge protocol after appendectomy for pediatric acute appendicitis.

BACKGROUND: The aim of this study was to evaluate the outcome of an early discharge protocol for pediatric acute appendicitis. METHODS: The present new early discharge protocol for appendicitis consisted of both postoperative early feeding and reduced-port laparoscopic surgery, to reduce surgical stress. The outcome was studied in patients with acute appendicitis treated at the present institution from 2012 to 2013. RESULTS: Data on 36 acute appendicitis patients (mean age, 10.3 years) were collected. Operation time was 95 ± 27 min. Preoperatively, mean white blood cell (WBC) count was 13 850 ± 3644/µL; mean C-reactive protein (CRP), 2.7 ± 2.9 mg/dL; and mean procalcitonin, 0.25 ± 0.37 ng/mL. After surgery there was a significant decrease in WBC count, which fell to within the normal range; CRP peaked at 4.9 ± 3.2 mg/dL on postoperative day (POD) 1. On POD 7, all of the hematological markers were within the normal range. There were no postoperative complications. Mean hospital stay was 2.1 ± 1.1 days. Mean frequency of oral painkiller use was 3.2 ± 3.3 times per person. CONCLUSIONS: The present early discharge protocol is safe and effective for the management of acute non-perforated appendicitis.

Analysis of risk factors of long-term complications in congenital diaphragmatic hernia: A single institution's experience.

OBJECTIVE: To establish better management practices to reduce morbidities in survivors with congenital diaphragmatic hernia (CDH). METHODS: Of 60 patients treated for CDH at our institution between 1991 and 2011, 49 patients without severe anomalies were retrospectively reviewed. RESULTS: Since 2004, gentle ventilation (GV) has been the main treatment for CDH. Patients were divided into the following two groups: the non-GV group (n = 29) who were treated before GV treatment was implemented, and the GV group (n = 20). The overall survival rate was 62.1% (18/29) and 95% (19/20) in the non-GV and GV groups, respectively (p = 0.016). Despite the high survival rate, the incidence of long-term complications in survivors was still high (14/19, 73.7%) in the GV group. In the GV group, liver-up (p = 0.106) and the need for patch repair (p = 0.257) tended to be associated with the development of long-term complications, but did not reach statistical significance. The presence of perioperative complications was associated with the development of long-term complications (p = 0.045) in the GV group. CONCLUSION: Patients who developed short-term complications seemed to be at risk of long-term complications. Therefore, to minimize long-term morbidities in CDH survivors, the prevention of short-term complications might be important.

Suppressed erythropoietin expression in a nitrofen-induced congenital diaphragmatic hernia.

BACKGROUND: Erythropoietin (EPO), an essential stimulator of erythropoiesis produced by the fetal liver, is important both in vascular remodeling and modulation of the endothelial response in the pulmonary vasculature. In addition, EPO guides alveolar development, along with retinoic acid (RA). EPO is a direct target of RA, and the retinoid pathway is altered in the nitrofen-induced congenital diaphragmatic hernia (CDH) model. In the present study, we tested the hypothesis that the synthesis of EPO is suppressed in a rat model of CDH. MATERIALS AND METHODS: Pregnant rats were treated with either nitrofen or vehicle on gestational day 9 (D9). Fetuses were sacrificed on D19 and D21 and divided into control and CDH groups. Immunohistochemistry and quantitative real-time polymerase chain reaction (RT-PCR) were performed to determine the expression of EPO in the fetal liver and kidney. We also estimated the expression of EPO receptor in the fetal lung. RESULTS: The relative EPO mRNA expression in the liver on D19 and in the kidney on D21 were significantly lower in the CDH group than in the controls (P = 0.0008 and P = 0.0064, respectively). In addition, the results of immunohistochemistry supported the findings from the RT-PCR analysis. No significant changes were noted in the expression pattern or EPO receptor levels in the fetal lungs of the CDH group compared to the controls. CONCLUSIONS: Our results reveal the suppressed EPO synthesis in the CDH fetus, which may contribute to the pathogenesis of lung hypoplasia and modification of pulmonary vasculature in the CDH rat model. Pediatr Pulmonol. 2017;52:606-615. © 2016 Wiley Periodicals, Inc.

Echocardiographic predictors of poor prognosis in congenital diaphragmatic hernia.

BACKGROUND/PURPOSE: The purpose of this study was to investigate echocardiographic parameters in relation to the outcomes of isolated left-sided congenital diaphragmatic hernia (CDH). METHODS: This multicenter, retrospective, observational study was conducted among patients with CDH born between 2006 and 2010. Patients in this study did not have severe cardiac malformations or chromosomal aberrations. Patients with incomplete echocardiographic examinations were excluded. In total, 84 patients with left-sided isolated CDH were included in this study. The prognostic parameters were obtained from postnatal echocardiographic images within 24h after birth. RESULTS: Eight patients died before 90days of birth. Univariate analysis showed that the presence of continuous right to left shunt at the ductus, left pulmonary artery diameter of <2.7mm, right pulmonary artery diameter of <3.3mm, and left ventricular diastolic diameter of <10.8mm, were the predictors of poor prognosis. Multivariate logistic regression analysis showed that right pulmonary artery diameter of <3.3mm (adjusted OR 10.28, 95% C.I.: 1.15-249.19) and left ventricular diastolic diameter of <10.8mm (adjusted OR 7.86, 95% C.I.: 1.01-82.82) were predictors of poor prognosis. CONCLUSIONS: This study revealed that the predictors of poor prognosis associated with CDH include smaller right pulmonary artery and left ventricular diastolic diameters. Retrospective Study-Level II.

The use of a sternothyroid muscle flap to prevent the re-recurrence of a recurrent tracheoesophageal fistula found 10 years after the primary repair.

Recurrent tracheoesophageal fistula (TEF) is still difficult to diagnose and repair. In almost all cases, recurrence appears relatively soon after the primary surgery. We herein describe a case of recurrent TEF that appeared 10 years after the primary repair. At 2 years of age, the patient suffered from mental retardation due to encephalitis and developed a hiatus hernia with gastro-esophageal reflux. He underwent the repair of a hiatus hernia and fundoplication at 3 years of age. However, the hiatus hernia recurred 6 months after the operation. The patient suffered from recurrent pneumonia for 6 years after the appearance of the recurrent hiatus hernia. At 9 years of age, he was hospitalized frequently due to recurrent severe pneumonia. After admission at 9 years of age, an endoscopic study under general anesthesia was performed and revealed subglottic stenosis and a dilated esophagus with a recurrent hiatus hernia. Tracheotomy or laryngotracheal separation was first planned in order to improve his upper airway and facilitate the safer repair of the recurrent hiatus hernia. After laryngotracheal separation, the patient still suffered from severe pneumonia. In addition, a small volume of nutritional supplement was aspirated from the tracheostomy. Thus, recurrent TEF was suspected. Tests using dye under both esophagoscopy and bronchoscopy confirmed recurrent TEF. The fistula recurred in the cervical area because of the elevation of the esophagus due to the recurrent hiatus hernia. The fistula was surgically closed, with a sternothyroid muscle flap to prevent re-recurrence. At 4 months after this operation, the recurrent hiatus hernia was repaired. Thereafter, the patient's respiratory symptoms showed a dramatic improvement. The patient is now doing well and free from further recurrences of TEF and hiatus hernia at 2 years after the final operation.

Pulmonary interstitial emphysema due to respiratory syncytial virus infection.

Pulmonary interstitial emphysema (PIE) primarily affects premature infants on positive pressure ventilation. PIE is rarely reported in infants and children in the absence of mechanical ventilation and/or associated respiratory infection. We report a case of PIE in a 22-month-old girl who had severe respiratory distress due to respiratory syncytial virus infection. Chest computed tomography showed cystic lung lesions mimicking congenital cystic adenomatoid malformation. The cystic lesions spontaneously resolved after conservative treatment. Based on the clinical course and the chronological changes on imaging, the cystic lung lesions were diagnosed as localized persistent PIE.

Hypergastrinemia and a duodenal ulcer caused by gastric duplication.

BACKGROUND: Hypergastrinemia and the resultant peptic ulcer related to an enteric duplication has been quite rarely reported in the literature. CASE PRESENTATION: We herein report the case of a 4-year-old girl who presented with hypergastrinemia and a duodenal ulcer at 2 years of age. She had been followed up with a proton pump inhibitor, which resulted in resolution of the ulcer; however, unexplained hypergastrinemia had continued. A cystic lesion at the antrum was discovered at 4 years of age, which we suspected to be a gastric duplication. After we resected the lesion, the hypergastrinemia resolved without recurrence of the duodenal ulcer. The histology was compatible with a gastric duplication, and the lumen was lined with antral mucosa that strongly stained positive for gastrin. We presumed that the antral mucosa inside the duplication in our case had no hydrogen ion feedback inhibition of gastrin release from gastrin cells and increased release of gastrin from the mucosa inside the duplication led to the duodenal ulcer. Only two cases have been reported in the literature that had hypergastrinemia related to enteric duplication. CONCLUSION: Gastric duplication should be included in the differential diagnosis of sustained hypergastrinemia in children.

Musculoskeletal abnormalities in congenital diaphragmatic hernia survivors: Patterns and risk factors: Report of a Japanese multicenter follow-up survey.

BACKGROUND: The aim of this study was to investigate the natural history and associated predictors of musculoskeletal deformities in congenital diaphragmatic hernia (CDH) survivors. METHODS: A multicenter retrospective survey was conducted among CDH patients between January 2006 and December 2010 in Japan, and a follow-up survey was performed between September 2013 and October 2013. One hundred and eighty-two (79.8%) of the 228 patients were alive. An orthopedic survey of 159 survivors without severe coexisting congenital anomalies was subsequently carried out, and the rates of pectus excavatum (PE), scoliosis and chest asymmetry were evaluated. RESULTS: Scoliosis, PE and chest asymmetry were found in 20 (12.6%), 19 (11.9%) and 12 (7.5%) patients, respectively. In total, 44 patients (27.7%) developed orthopedic abnormalities. Reduction in the oxygenation index within 24 h after birth (P = 0.044), large diaphragmatic defects (P = 0.047) and patch repair (P = 0.014) were predictive for scoliosis. In addition, Apgar score at 5 min was significantly lower in the patients who developed PE (P = 0.034); and stomach herniation (P = 0.004) and liver herniation (P = 0.013) at prenatal diagnosis and large diaphragmatic defects (P = 0.036) were predictive of chest asymmetry. CONCLUSIONS: Approximately one-quarter of the survivors developed musculoskeletal abnormalities in the present survey of CDH patients. These data suggest that each musculoskeletal abnormality has its own specific predictors.

Growth Assessment and the Risk of Growth Retardation in Congenital Diaphragmatic Hernia: A Long-Term Follow-Up Study from the Japanese Congenital Diaphragmatic Hernia Study Group.

INTRODUCTION: Little information exists about the physical growth of patients with congenital diaphragmatic hernia (CDH). This study aimed to assess the growth of patients with CDH during long follow-up periods, and to identify growth retardation (GR) risk factors. PATIENTS AND METHODS: A multicenter retrospective observational study was conducted in 2013. Of the 228 patients with CDH born between 2006 and 2010, 182 (79.8%) survived to discharge, and 174 cases were included in the study. Body weights and heights were measured at 1.5, 3, and 6 years of age. GR was defined as a Z-score relating to the weight or height of < - 2.0. Cases with GR at 1.5, 3, or 6 years of age comprised the GR group. The clinical variables of the GR and non-GR groups were compared using univariate analysis. Multiple logistic regression analyses were conducted successively on the factors that were significant at p < 0.01 in the univariate analysis and had low correlations with other factors (r < 0.7). The numerical data were divided into two groups based on a cutoff value that was calculated from a receiver operating characteristic curve. RESULTS: The GR group comprised 35 cases (22.7%). The rates of GR at 1.5, 3, and 6 years of age were 19.5 (26/133), 14.4 (16/111), and 13.5% (5/37), respectively. The body weight Z-scores improved in cases with GR between the ages of 1.5 and 3 years (p = 0.036). As the patients aged, the wasting type of GR decreased in frequency (31, 0, and 0% at 1.5, 3, and 6 years of age, respectively) and the stunting type of GR increased in frequency (27, 31, and 100% at 1.5, 3, and 6 years of age, respectively). The univariate analysis showed that birth weight and height, liver-up, large defect size of the diaphragm, use of nitric oxide, patch repair, long hospital stay, home oxygen treatment (HOT), and vasodilator administration at discharge were significant risk factors of GR. The multivariate analysis determined that a birth weight of < 2,698 g (odds ratio [OR] = 5.5, 95% confidence interval [CI] = 2.1-16.8, p < 0.001) and HOT (OR = 5.8, 95%CI = 1.6-23.8, p = 0.007) were significant risk factors for GR. CONCLUSION: GR was observed in 22.7% of the CDH survivors. Body weight improved between 1.5 and 3 years of age in the GR cases, but some patients developed chronic malnutrition via acute malnutrition. Low birth weight and the need for HOT were GR risk factors. Aggressive management of acute malnutrition may improve the growth of patients with CDH.

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.

Risk factors for the recurrence of the congenital diaphragmatic hernia-report from the long-term follow-up study of Japanese CDH study group.

AIM OF THE STUDY: Few follow-up studies focused on the recurrence regarding the postoperative course of congenital diaphragmatic hernia (CDH) survivors. The aim of this study was to report on risk factor for CDH patients who had the recurrence during the follow-up. MATERIALS AND METHODS: A multicenter retrospective survey was conducted on neonates diagnosed to have CDH between January 2006 and December 2010. Follow-up survey was conducted between September 2013 and October 2013 (ethical approval: No. 25-222). Nine institutions agreed to participate in this survey. Out of 228, 182 (79.8%) patients were alive and 180 patients were included in this study. Two patients were excluded because the defect had not repaired at the primary operation. The patients were divided into the recurrence group (n=21) and the nonrecurrence group (n=159). Postnatal and postoperative variables were compared between these two groups. Baseline variables which showed significance in univariate analysis were entered into multiple logistic regression analysis for analyzing the recurrence. A value of p<0.05 was considered to be statistically significant by using the JMP software program (version 9; SAS Institute, Inc, Cary, North Carolina, United States). MAIN RESULTS: Out of 180, 21 (11.7%) CDH neonates had the recurrence during the course of the follow-up. Five (2.8%) patients had the recurrence before primary discharge and 16 (8.9%) patients had the recurrence after discharge. Univariate analysis showed that liver herniation (crude odds ratio [OR], 7.4; 95% confidence interval [CI], 2.73-23.68), defect size C and D, proposed by the CDH Study Group (crude OR, 7.09; 95% CI, 2.73-19.99) and patch repair (crude OR, 5.00; 95% CI, 1.91-14.70) were risk factors. Multivariate logistic regression analysis showed liver herniation (adjusted OR, 3.96; 95% CI, 1.01-16.92) was the risk factor for the recurrence. CONCLUSION: A wide spectrum of the disease severity and the rarity of the disease mask the risk of the recurrence for CDH patients. This study showed the only factor to predict the recurrence was the liver herniation. These data will be helpful for providing information for the long-term follow-up of the CDH patients.

Prognostic factors of gastroesophageal reflux disease in congenital diaphragmatic hernia: a multicenter study.

PURPOSE: Gastroesophageal reflux disease (GERD) is one of the concomitant problems in infants with congenital diaphragmatic hernia (CDH). We assessed risk factors of GERD in CDH patients. METHODS: The retrospective observational study for CDH infants was conducted. Cases of CDH who were born between January 2006 and December 2010, were operated in the 9 participating institutions, and survived to discharge were included. Completion of medical therapy for GERD and incidence of surgery were primary outcomes. Kaplan-Meier survival analysis and Cox proportional hazards regression were used. RESULTS: In 182 cases of CDH, the medical therapies for GERD were performed in 23.8% (40/168), and were completed in 60.0% (24/40). Prenatal detection of CDH (HR 5.87, CI 1.6-18.8, p = 0.012) and tube feeding at discharge (HR 5.04, 95% CI 1.3-33.1, p = 0.016) were significantly correlated with unsuccessful weaning from medical therapy. Surgery for GERD was performed in 10.7% (18/169). Gestational age (HR 4.78, 95% CI 1.5-21.1, p = 0.006) and diaphragmatic defect of more than 75% (HR 4.3, 95% CI 1.6-12.9, p = 0.005) were significantly correlated with need for antireflux surgery. CONCLUSION: Diaphragmatic defect of more than 75% was risk factor of future need for antireflux surgery.

Nevoid basal cell carcinoma syndrome with a unilateral giant ovarian fibroma in a Japanese 6-year-old girl.

Nevoid basal cell carcinoma syndrome (NBCCS) is characterized by basal cell carcinoma, skeletal abnormalities, benign tumors including ovarian fibroma, and various other phenotypic expressions. Ovarian fibromas in NBCCS before puberty are very rare. We report a 6-year-old prepubescent girl with NBCCS showing skeletal abnormalities, medulloblastoma, and ovarian fibromas. The patient was referred to our hospital owing to abdominal distension. On admission, a huge elastic hard tumor was palpable and computed tomography showed a huge tumor of the left ovary. We performed a left salpingo-oophorectomy and diagnosed the tumor as a benign fibroma. Further examination of the computed tomography images showed skeletal abnormalities. In addition, the patient had a history of medulloblastoma at the age of 4 years. Therefore, we diagnosed NBCCS. A genetic examination indicated a novel 1 bp deletion in exon 18 (c.3055delG). Sequence analysis of exon 18 using DNA from the ovarian tumor revealed a mutant allele (c.3055delG) dominant to the wild-type allele, thus suggesting loss of heterozygosity in the PTCH1 gene, which is known to be associated with NBCCS. Conclusion On the basis of our experience, physicians treating pediatric ovarian tumors should be aware that such huge benign ovarian tumors may be a phenotype of NBCCS, as shown in our patient. In addition, genetic examination focusing on the PTCH1 gene might be important for diagnosis of NBCCS in pediatric patients.

Surgical technique to prevent spillage of cyst fluid during operation for cystic ovarian tumors.

We describe a new technique to prevent spillage of cyst fluid in patients undergoing surgery for cystic ovarian tumors. The cyst is first covered with a sterilized surgical sheet applied with quick-drying glue and is then punctured. This technique completely prevents spillage of cyst fluid into abdominal cavity.