Minimally invasive surgery (MIS) for congenital diaphragmatic hernia (CDH) has previously been associated with higher recurrence rates and uncertain indications compared with open surgery. Since 2017, the Japanese Congenital Diaphragmatic Hernia Study Group (JCDHSG) has implemented a standardized national protocol, potentially improving perioperative management and surgical outcomes. This multicenter retrospective study analyzed 1,081 infants who underwent definitive CDH surgery at 15 tertiary centers in Japan between 2007 and 2023. Patients were categorized by surgical approach and period: early-period MIS (≤ 2016, n = 62), late-period MIS (≥ 2017, n = 109), early-period open (≤ 2016, n = 536), and late-period open (≥ 2017, n = 374). In-hospital mortality was assessed for the "Intended-MIS" group (including conversions), while surgical outcomes and recurrence were evaluated for the "Complete-MIS" group (successfully completed procedures without conversion). In the Intended-MIS group, the conversion rate dropped significantly from 29

PURPOSE:To elucidate the long-term incidence and risk factors of congenital diaphragmatic hernia (CDH) recurrence in the era of minimally invasive surgery (MIS). METHODS:In a Japanese CDH Study Group database between 2006 and 2023, we included 1,115 patients with CDH who underwent surgery for diaphragmatic repair, with follow-up data available for up to 12 years of age. The following variables were assessed as potential risk factors for recurrence: right-sided hernia, large diaphragmatic defect (international classification C and D), liver herniation, stomach herniation, patch use, and MIS without conversion. RESULTS:A total of 134 recurrences occurred in 98 patients. The cumulative recurrence rate was 11.4% at 10 years of age. In multivariate analyses, large diaphragmatic defect (adjusted odds ratio [OR], 2.26; 95% confidence interval [CI], 1.14-4.47), liver herniation (adjusted OR, 2.39; 95% CI, 1.27-4.47), and MIS without conversion (adjusted OR, 2.56; 95% CI, 1.19-5.50) were significant risk factors. In patients with both non-large diaphragmatic defects and non-liver herniation, the cumulative incidence of recurrence was significantly higher for MIS than for open surgery (p = 0.0288). Patch use was significantly more common in open surgery than in MIS without conversion (45% vs. 18%, p < 0.0001), especially in patients with international classification C diaphragmatic defects (84% vs. 27%, p < 0.0001). CONCLUSION:Herein, the recurrence rate was 11.4%. A large defect size, liver herniation, and MIS without conversion were independent risk factors for CDH recurrence. In MIS for severe CDH, aggressive patch use and appropriate conversion to open surgery may mitigate recurrence.

This study aimed to investigate the microcolon cutoff value for diagnosing intestinal atresia using a neonatal contrast enema. A 20-year retrospective review of contrast enema images of neonates ≤ 4 days of age with abdominal distension was performed. The patients were categorized into five groups: intestinal atresia, meconium ileus, normal, Hirschsprung disease, and others. The maximum diameter of each part of the colon was measured when the splenic flexure, hepatic flexure, or terminal ileum was visualized. Receiver operating characteristic analysis was performed to determine the optimal cutoff value of the colonic diameter (CD) to L1 interpedicular distance (IPD) ratio for predicting intestinal atresia. The area under the curve (AUC) for the diagnostic performance of the CD/IPD ratio for intestinal atresia exceeded 0.8 in all colon parts at all time points. The AUC for the ascending CD/IPD at the time of terminal ileum visualization was the highest (0.907), and its optimal cutoff value was 0.955 (sensitivity, 90.9

BACKGROUND:This study aimed to examine the incidence of undescended testis (UDT) in congenital diaphragmatic hernia (CDH) and clinical risk factors for developing UDT. METHODS:Male patients with CDH born between 2006 and 2021 were retrospectively assessed for UDT using the database of the Japanese CDH study group. The UDT risk factors were examined by comparing patients with and without UDT. RESULTS:Of the 605 boys with CDH, 408 were followed for at least one year and were included in this study. A total of 50 (12.3 %) of the 408 patients experienced UDT and orchidopexy was performed in 41 (10.2 %) patients. Twenty-four cases developed UDT on the same side of CDH, whereas only 6 cases developed UDT on the contralateral side of CDH; the remaining 20 cases were bilateral UDT. Comparison between the patients with and without UDT revealed significantly lower birth weight and lower Apgar Score in the patients with UDT. Furthermore, patients with UDT demonstrated a significantly higher rate of non-isolated, "liver-up", defect with C/D type, and patch closure. Multivariate analysis showed that the C/D type defect was deemed an independent risk factor for developing UDT. CONCLUSIONS:The incidence of UDT and the need for orchidopexy are elevated in boys with CDH. In patients with CDH, UDT may tend to occur on the same side as that affected by CDH. The diaphragmatic large defect was considered to be an independent risk factor for UDT in CDH although different factors are thought to be involved in testicular descent.

BACKGROUND:Vitamin K (VK) prophylaxis refers to the administration of VK to newborns to prevent neonatal VK deficiency bleeding (VKDB), which is characterized by intracranial hemorrhage (ICH). This study investigated the relationship between VK prophylaxis methods and VKDB in biliary atresia (BA). METHODS:The survey targeted 497 cases in the Japanese Biliary Atresia Registry between 2015 and 2019, of which 395 (79.5%) returned the questionnaire. Of the 395 patients, 289 were selected after excluding cases in which the gestational age was <36 weeks or the VK prophylaxis methods/feeding contents were unknown. The patients were categorized into two groups according to VK prophylaxis methods. We conducted a comparative study using propensity score matching. The prognosis of patients with or without ICH was also investigated. RESULTS:In the analysis, no VKDB occurred in patients using the 3-month method. In the propensity score matching analysis, age at first visit and age at surgery were later in the three-times method (p = 0.018 and p = 0.022, respectively); VKDB was higher in the three-times method than in the 3-month method (p = 0.029). ICH, jaundice disappearance, cholangitis, and native liver survival rates (NLSRs) were not significantly different between groups. When examining the prognosis based on ICH occurrence, the two groups showed no significant differences in jaundice disappearance, cholangitis, and NLSRs. The ICH group had a greater number of cases of delayed mental and/or motor development. CONCLUSIONS:In BA, the 3-month method is effective in preventing VKDB, and early diagnosis is crucial.

A neonate with refractory supraventricular tachycardia complicated by necrotizing enterocolitis. Various antiarrhythmic drugs were ineffective, and the tachycardia was controlled with intravenous nifekalant. The accessory pathway was successfully ablated at a weight of 3.0 kg without complications.

This study aimed to investigate the incidence of severe surgical complications among children with hepatoblastoma, identify their risk factors, and evaluate the influence of surgical complications on long-term outcomes. Children with hepatoblastoma who underwent liver resection at our hospital between September 1992 and January 2023 were included in this study. Clinical data were retrospectively reviewed, and patients were categorized into complication and non-complication groups based on the need for radiological or surgical interventions or massive intraoperative blood loss (> 80 mL/kg). Out of the 40 patients, 9 experienced severe complications (massive blood loss, n = 7; bile leakage, n = 3; and common bile duct stricture, n = 1). The participation of experienced liver surgeons was significantly greater in the non-complication group than in the complication group. The median duration from surgery to the start of postoperative chemotherapy was significantly shorter in the non-complication group than in the complication group. The overall 5-year survival rate was significantly higher in the non-complication group than in the complication group. Severe surgical complications were associated with a worse prognosis. An experienced liver surgeon should participate in technically demanding liver resections.

A 4-year-old girl was admitted to our hospital because of precocious puberty. Radiologic findings revealed a fist-sized solid tumor in the left ovary without ascites, peritoneal dissemination, and distant metastasis. The patient underwent left salpingo-oophorectomy without spillage. The size of the excised tumor was 10.0×9.0×4.8 cm. On pathologic examination, the tumor was diagnosed as an ovarian steroid cell tumor, not otherwise specified. In the present case, although the diameter of the tumor (>7 cm) and three mitoses per 10 high-power fields represented some potential for malignancy, we opted for careful observation without chemotherapy as the tumor was of clinical stage Ia.

Background & aims: Growth retardation is a common morbidity in infants with severe congenital diaphragmatic hernia (CDH). This study aimed to investigate the appropriate amount of nutrition and nutrient balance in early infants with severe CDH.Methods: This was a retrospective case-control study. Isolated CDH patients with more than half of the chest wall devoid of diaphragmatic tissue and treated between 2006 and 2022 were included. The patients were divided into positive (n = 16) or negative (n = 8) weight gain velocity groups in early infancy (from 1 to 3 months of age). Clinical variables and amount of nutrition were compared between the two groups.Results: The earlier start of enteral nutrition (EN) and higher replacement rate of the naso-duodenal (ND) tube were observed more frequently in the positive group than in the negative group. Regarding daily intake by both EN and parenteral nutrition (PN), total caloric intake and caloric intake of proteins and lipids were significantly higher in the positive group than in the negative group at 1 month of age. At 2 months, total caloric intake and caloric intake of lipids were significantly higher in the positive group than in the negative group. At 3 months of age, only caloric intake of lipid was significantly higher in the positive group than in the negative group. Conclusions: The early replacement of the ND tube may contribute to the early start of EN, which may subsequently lead to appropriate nutrient supplementation including adequate lipid administration, resulting in early catch-up growth.& COPY; 2023 Published by Elsevier Ltd on behalf of European Society for Clinical Nutrition and Metabolism.

Purpose To identify risk factors for delayed oral nutrition in infants with a congenital diaphragmatic hernia (CDH) and its impact on developmental delay at 18 months of age. Methods This retrospective single-center cohort study compared the clinical parameters in patients with isolated CDH born and treated at our hospital between 2006 and 2020. We evaluated clinical features significantly related to delayed oral nutrition (defined as taking ≥ 30 days from weaning from mechanical ventilation to weaning from tube feeding). Results Twenty-six of the 80 cases had delayed oral nutrition. Univariate analyses showed significant differences. Multivariate analyses were performed on the three items of preterm delivery, defect size (over 50% to nearly entire defect), and ventilation for ≥ 9 days. We identified the latter two items as independent risk factors. The adjusted odds ratios were 4.65 (95% confidence interval, 1.27–7.03) and 6.02 (1.65–21.90), respectively. Delayed oral nutrition was related to a significantly higher probability of developmental delay at 18 months (crude odds ratio 4.16, 1.19–14.5). Conclusion In patients with CDH, a large defect and ventilatory management over 9 days are independent risk factors for delayed oral nutrition, which is a potent predictor of developmental delay that requires active developmental care.

This study aimed to evaluate the feasibility and safety of laparoscopic resection of abdominal neuroblastoma by comparing operative and long-term oncologic outcomes between open and laparoscopic surgeries. This single-center retrospective study included patients who underwent laparoscopic tumor resection, between January 2000 and June 2021, with a maximum tumor diameter of ≤ 60 mm and without image-defined risk factors (IDRFs) at surgery. Data from 35 abdominal neuroblastoma resections were reviewed and compared between the laparotomy and laparoscopic groups. Nineteen patients underwent laparotomy and 16 had laparoscopic tumor resection. All laparoscopic surgeries achieved complete resection without conversion to open surgery. Median blood loss was significantly lower in the laparoscopic group (0.6 mL/kg) than in the laparotomy group (8.4 mL/kg) (P < 0.0001). There were two locoregional recurrences in the laparoscopic group and two metastatic recurrences in the laparotomy group. Five-year overall survival was 93.8

Purpose This study aimed to investigate the incidence and clinical factors associated with undescended testes (UDT) in patients with congenital diaphragmatic hernia (CDH). Methods We retrospectively reviewed the incidence of UDT in male neonates admitted to our institution and underwent surgery for CDH between January 2006 and December 2022. Patients were divided into two groups based on the presence or absence of UDT, and risk factors for UDT were compared between the two groups. Results Among the 66 male neonates with CDH, 16 (24.2%) developed UDT. Patients with UDT had a significantly smaller gestational age (p = 0.026), lower birth weight (p = 0.042), and lower Apgar score at 1 min (p = 0.016) than those without UDT. They had a significantly higher incidence of large diaphragmatic defects (p = 0.005), received more patch closures (p = 0.020), had a longer mechanical ventilation period (p = 0.034), and longer hospital stay (p = 0.028). Multiple logistic regression analysis revealed that large diaphragmatic defect was an independent risk factor for UDT (adjusted odds ratio of 3.87). Conclusion CDH and UDT are strongly correlated. In patients with CDH, the incidence of UDT was related not only to patients’ prematurity but also to the large diaphragmatic defect. Large diaphragmatic defect is an independent risk factor for UDT in patients with CDH.

To assess the risk factors for surgical site infections (SSIs) post-abdominal surgery in neonates. A retrospective, single-center cohort study was conducted using patient data from 2009 to 2018. Patient characteristics and several variables were analyzed to identify independent risk factors for SSI. SSI occurred in 39/406 procedures (9.6%). Univariate analysis showed that the incidence of SSI was significantly higher in patients who had undergone multiple surgical procedures (P = 0.032), prolonged operations (P = 0.016), long-term hospitalization (P < 0.001), long-term antibiotic administration (P < 0.001), with methicillin-resistant Staphylococcus aureus (MRSA) colonization (P = 0.044), contaminated/dirty wounds (P < 0.001), and American Society of Anesthesiologists physical status of 3 or 4 (P = 0.021). Multivariate analysis identified prolonged operations [odds ratio (OR): 2.91 (1.21–8.01)] and contaminated/dirty wounds [OR: 5.42 (2.41–12.1)] as independent risk factors. Patients with SSI had a higher incidence of MRSA colonization (27.8% vs. 14.8%, P = 0.044), longer antibiotic administration (24 days vs. 8 days, P = 0.049), and longer hospitalization times (98 days vs. 43 days, P = 0.007) than those without SSIs. Long operations exceeding 100 min and surgical procedures with contaminated/dirty wounds are independent risk factors for neonatal SSIs after abdominal surgery. SSIs were related to MRSA colonization during hospitalization, long-term antibiotic administration, and long-term hospitalization.

Purpose Fetuses with persistent cloaca are known to develop urine or meconium backflow into the abdominal cavity caused by obstruction of the common channel, thus leading to fetal peritonitis with fetal ascites. We analyzed the impact of prenatal fetal ascites on postnatal clinical features and management. Methods This retrospective single-center cohort study was conducted to compare the perinatal parameters of patients with isolated persistent cloaca who were born and treated at our hospital between 1991 and 2021. The clinical features and management of those with and without fetal ascites were compared. Results Among the 17 eligible patients, fetal ascites were recognized in seven. The occurrence of fetal ascites was significantly related to preterm birth, higher birth weight z-score, birth via emergency cesarean delivery, low Apgar scores at 1 min and 5 min, higher C-reactive protein levels at birth, longer duration of oxygen administration, the need for a urinary drainage catheter at initial discharge, and shorter neonatal hospital stays. Conclusions The postnatal management of patients with persistent cloaca with fetal ascites differed significantly from that of patients without fetal ascites. For patients with unexplained fetal ascites, magnetic resonance imaging may be helpful for determining the definite diagnosis of persistent cloaca.

Purpose This study aimed to evaluate prenatal predictors of mortality in fetuses with congenital diaphragmatic hernia (CDH). Methods A systematic literature search was performed to identify relevant observational studies that evaluated the ability of lung-to-head ratio (LHR), observed-to-expected LHR (o/e-LHR), observed-to-expected total fetal lung volume (o/e-TFLV), lung-to-thorax transverse area ratio (L/T ratio), intrathoracic herniation of the liver and the stomach, and side of diaphragmatic hernia, using a threshold for the prediction of mortality in fetuses with CDH. Study quality was assessed using the QUADAS-2 tool. Hierarchical summary receiver operating characteristic curves were constructed. Results A total of 50 articles were included in this meta-analysis. The QUADAS-2 tool identified a high risk of bias in more than one domain scored in all parameters. Among those parameters, the diagnostic odds ratio of mortality with o/e-LHR < 25%, o/e-TFLV < 25%, and L/T ratio < 0.08 were 11.98 (95% confidence interval (CI), 4.65–30.89), 11.14 (95%CI, 5.19–23.89), and 10.28 (95%CI, 3.38–31.31), respectively. The predictive values for mortality were similar between the presence of liver herniation and retrocardiac fetal stomach position. Conclusions This systematic review suggests that o/e-LHR, o/e-TFLV, and L/T ratio are equally good predictors of neonatal mortality in fetuses with isolated CDH.

BACKGROUND:Sirenomelia is a congenital malformation of the lower body characterized by a single midline lower limb and severe urogenital and gastrointestinal malformations. Sirenomelia is rare (estimated incidence of approximately 1/100,000) and usually lethal in the perinatal period.CASE:A 2,042 g Japanese male infant, one of monochorionic monoamniotic twins, was born at 34 weeks of gestation by elective caesarean section. Sirenomelia was prenatally diagnosed. Single midline lower limb, bilateral dysplastic kidneys, an omphalomesenteric fistula, colon atresia, imperforate anus, indiscernible genital structures, and myelomeningocele were detected at birth. The amniotic fluid volume was normal throughout the pregnancy course, which led to appropriate lung maturation of the twin with sirenomelia. Although renal replacement therapy was initiated soon after birth, stable peritoneal dialysis was difficult because of the limited intraperitoneal space, and the infant frequently developed peritonitis. He died of sudden cardiorespiratory arrest at 6 months of age. Postmortem examination showed bilateral dysplastic kidneys, agenesis of the ureters and urinary bladder, abnormal branching and agenesis of the distal colon, bilateral inguinal hernias, and small testes.CONCLUSION:Infants with sirenomelia, even those with end-stage kidney disease at birth, may survive if they have a stable cardiorespiratory status at birth and renal replacement therapy is appropriately initiated.

PURPOSES:Congenital biliary dilatation (CBD), defined as pancreaticobiliary maljunction (PBM) with biliary dilatation, is a high risk factor for biliary tract cancer (BTC). KRAS and p53 mutations reportedly affect this process, but the mechanisms are unclear, as is the likelihood of BTC later in life in children with CBD. We investigated potential carcinogenetic pathways in children with CBD compared with adults.METHODS:The subjects of this study were nine children with CBD and 13 adults with PBM (10 dilated, 3 non-dilated) without BTC who underwent extrahepatic bile duct resections, as well as four control patients who underwent pancreaticoduodenectomy for non-biliary cancer. We evaluated expressions of Ki-67, KRAS, p53, histone deacetylase (HDAC) and activation-induced cytidine deaminase (AID) in the biliary tract epithelium immunohistochemically.RESULTS:The Ki-67 labeling index (LI) and expressions of KRAS, p53, HDAC, and AID in the gallbladder epithelium were significantly higher or tended to be higher in both the children with CBD and the adults with PBM than in the controls.CONCLUSIONS:BTC may develop later in children with CBD and in adults with PBM, via HDAC and AID expression and through epigenetic and genetic regulation.