Noriaki Usui

Osaka Women's and Children's Hospital · Department of Pediatric Surgery

Purpose This study aimed to clarify surgical complications associated with open surgery for congenital diaphragmatic hernia (CDH). Methods We performed an exploratory data analysis of the clinical characteristics of surgical complications of neonates with CDH who underwent laparotomy or thoracotomy between 2006 and 2021. Data of these patients wer...

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 i...

Purpose: This study aimed to clarify surgical complications associated with open surgery for congenital diaphragmatic hernia (CDH). Methods: We performed an exploratory data analysis of the clinical characteristics of surgical complications of neonates with CDH who underwent laparotomy or thoracotomy between 2006 and 2021. Data of these patients we...

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. W...

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....

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. Patie...

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. Patie...

Objective To assess the reliability of pulmonary artery (PA) parameters as a prognostic marker in neonates with isolated left-sided congenital diaphragmatic hernia (IL-CDH). Study design A retrospective cohort study conducted by the Japanese CDH Study Group (JCDHSG). Results 323 IL-CDH patients registered with the JCDHSG were included. 272 patien...

This systematic review and meta-analysis aimed to review the optimal timing of delivery at term for neonates with prenatally diagnosed congenital diaphragmatic hernia (CDH). We reviewed the literature up to December 19, 2022 using MEDLINE and the Cochrane Library databases. The inclusion criteria were original articles, comparative studies of CDH n...

Objectives To evaluate the survival and intact-survival rates among preterm infants with congenital diaphragm hernia (CDH). Study design Multicenter retrospective cohort study of 849 infants born between 2006 and 2020 at 15 Japanese CDH study group facilities. Multivariate logistic regression analysis adjusted using inverse probability treatment w...

Background: Congenital diaphragmatic hernia (CDH) is a severe congenital disease. Some CDH infants suffer from gastro-esophageal reflux disease (GERD), even after surgical correction of gastric position. A transpyloric tube (TPT) is inserted into CDH patients under direct observation intraoperatively in some hospitals in Japan to establish early e...

PurposeThis 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.Methods This single-center retrospective study included patients who underwent laparoscopic tumor resection, between January 2000 and June...

Objective: To assess the reliability of pulmonary artery (PA) parameters as a prognostic marker in neonates with isolated left-sided congenital diaphragmatic hernia (IL-CDH). Study design: A retrospective cohort study conducted by the Japanese CDH Study Group (JCDHSG). Results: 323 IL-CDH patients registered with the JCDHSG were included. 272 patie...

Background: Patienets with congenital diaphragmatic hernia comorbidity with congenital heart disease have a more severe prognosis than those with either condition alone. Here we report the clinical outcomes of congenital diaphragmatic hernia patients with congenital heart disease who have either two ventricles or a functional single ventricle. Meth...

Purpose To develop a predictive score for small diaphragmatic defects in infants with congenital diaphragmatic hernia (CDH) for determining thoracoscopic surgery indication. Methods The Japanese CDH Study Group cohort was randomly divided into derivation (n = 397) and validation (n = 396) datasets. Using logistic regression, a prediction model and...

Objectives To evaluate the survival and intact-survival rates among preterm infants with congenital diaphragm hernia (CDH). Study Design Multicenter retrospective cohort study of 849 infants born between 2006 and 2020 at 15 Japanese CDH study group facilities. Multivariate logistic regression analysis adjusted using inverse probability treatment w...

Purpose The prenatal diagnosis of the stomach position in congenital diaphragmatic hernia (CDH) has been a reliable prognostic factor, but few studies have focused on the postnatal position. We therefore evaluated the significance of the nasogastric (NG) tube position just after birth. Methods The Japanese CDH Study Group database enrolled 1037 CD...

PurposeThis study aimed to evaluate prenatal predictors of mortality in fetuses with congenital diaphragmatic hernia (CDH).MethodsA 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 vo...

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 lun...

Purpose:The prenatal diagnosis of the stomach position in congenital diaphragmatic hernia (CDH) has been a reliable prognostic factor, but few studies have focused on the postnatal position. We therefore evaluated the significance of the nasogastric (NG) tube position just after birth. Methods: The Japanese CDH Study Group database enrolled 1037 CD...

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...

Background: Mortality prediction of congenital diaphragmatic hernia is essential for developing treatment strategies, including fetal therapy. Several researchers have reported prognostic factors for this rare but life-threatening condition; however, the optimal combination of prognostic factors remains to be elucidated. Objectives: This study a...

Purpose: To develop a predictive score for small diaphragmatic defects in infants with congenital diaphragmatic hernia (CDH). Methods: The Japanese CDH Study Group cohort was randomly divided into derivation (n = 397) and validation (n = 396) datasets. Using logistic regression, a prediction model and weighted scoring system for small diaphragmatic...

Purpose: We examined patient satisfaction with postoperative chest appearance after Nuss procedure and analyzed the factors for postoperative low satisfaction. Methods: We retrospectively reviewed data of 133 patients who underwent the Nuss procedure from 2000 to 2016. Their medical records, X-rays, and computed tomography scans were evaluated....

Background: Treatment modalities for neonates with congenital diaphragmatic hernia (CDH) have greatly improved in recent years, with a concomitant increase in survival. However, long-term outcomes restrict the identification of optimal care pathways for CDH survivors in adolescence and adulthood. Therefore, we evaluated the long-term outcomes with...

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 e...

Purpose To assess the risk factors for surgical site infections (SSIs) post-abdominal surgery in neonates. Methods 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. Results SSI occurred in 39/406...

Background Liver transplantation (LTx) is indicated for unresectable hepatoblastoma (HB) without distal metastasis. However, to our knowledge, there is no consensus on the management of unresectable HB with pulmonary metastases, or on the treatment of recurrent HB. We report a successful case of metastatic HB treated with repeated lung resection, c...

We present the case of a female patient with hepatoblastoma associated with trisomy 18, who was successfully treated using a combination of chemotherapy and surgical resection. She was born after 37 weeks of gestation with a body weight of 1,470 g and was diagnosed after birth as having trisomy 18 and esophageal atresia with tracheoesophageal fistu...

Purpose Predicting lethal pulmonary hypoplasia in infants with congenital diaphragmatic hernia (CDH) before extracorporeal membrane oxygenation (ECMO) initiation is difficult. This study aimed to predict lethal pulmonary hypoplasia in patients with CDH prior to ECMO. Methods This was a multicenter cohort study involving neonates prenatally diagnos...

PurposeIn 2004, the Japanese government halted nationwide mass screening for neuroblastoma in 6-month-old infants as it led to overdiagnosis of localized tumors with favorable prognoses and failed to reduce neuroblastoma-related mortality. However, a new mass screening program for neuroblastoma in 18-month-old infants (18MS) was conducted in the Os...

Background Recent studies have reported the high incidence of undescended testis (UDT) in patients with gastroschisis. Although various mechanical, hormonal, and genetic theories have been postulated to describe testicular descent, the mechanism contributing to this condition remains controversial. We aimed to investigate the incidence and risk fac...

Purpose: We investigated the clinical characteristics of congenital esophageal stenosis and whether balloon dilation is useful as first-line therapy for all types of congenital esophageal stenosis. Methods: We retrospectively analyzed the medical records of patients with congenital esophageal stenosis, who were treated at three hospitals including...

The pathogenesis of gonadotropin-independent precocious puberty (PP) includes both congenital and acquired forms, the latter of which may be associated with neoplasms, such as sex-steroid hormone-producing tumors. Beta-human chorionic gonadotropin (β-hCG)-producing tumors also cause gonadotropin-independent PP by stimulating the production of testo...

PurposeIt is important to confirm the existence of pulmonary metastases in pediatric patients with malignancies. Therefore, we aimed to investigate if computed tomography CT-guided marking is a feasible and safe method for the identification and resection of tiny pulmonary lesions in pediatric cancer patients. Methods We retrospectively reviewed th...

To evaluate the optimal timing of neonates with prenatally diagnosed congenital diaphragmatic hernia (CDH). Data from a retrospective cohort study conducted by the Japanese CDH Study Group between 2011 and 2018 were divided into two groups according to delivery timing: 36–37 and 38–41 weeks of gestation (wg). Death before 90 days as the primary out...

PurposeThe etiology of cholestasis in neonates is associated with several factors including gastrointestinal disease and surgery. We aimed to identify the potential risk factors for perioperative cholestasis in patients with duodenal atresia and determine specific cutoff values for the risk factors.Methods This retrospective cohort study included 7...

PurposesCongenital 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 c...

The risk of intussusception related to percutaneous endoscopic gastrostomy with jejunal extension (PEG-J) in patients with severe motor and intellectual disabilities (SMID) remains unknown. In a cross-sectional study, a review of 26 patients (mean age, 11.6 ± 6.4 years) with SMID who underwent PEG-J was performed. During the follow-up period, 6 of...

Background Observational studies have shown that fetoscopic endoluminal tracheal occlusion (FETO) has been associated with increased survival among infants with severe pulmonary hypoplasia due to isolated congenital diaphragmatic hernia on the left side, but data from randomized trials are lacking. Methods In this open-label trial conducted at cen...

Introduction: Laparoscopic resection has gradually been adopted for neuroblastoma patients; however, some authors reported that, due to its technically demanding procedures, this operation should be performed only by highly experienced surgeons. The aim of this study was to evaluate the safety and feasibility of laparoscopic resection of abdominal...

PurposeWe compared the efficacy of thoracoscopic repair (TR) with that of open repair (OR) for neonatal congenital diaphragmatic hernia (CDH).Methods The subjects of this multicenter retrospective cohort study were 524 infants with left-sided isolated CDH, diagnosed prenatally, and treated at one of 15 participating hospitals in Japan between 2006...

Congenital diaphragmatic hernia (CDH) is a birth defect of the diaphragm in which abdominal organs herniate through the defect into the thoracic cavity. The main pathophysiology is respiratory distress and persistent pulmonary hypertension because of pulmonary hypoplasia caused by compression of the elevated organs. Recent progress in prenatal diag...

To determine the appropriate initial ventilatory mode for neonatal congenital diaphragmatic hernia (CDH) by comparing patient prognosis following conventional mechanical ventilation (CMV) versus high-frequency oscillatory ventilation (HFO). This multicenter retrospective cohort study was performed at 15 participating hospitals in Japan between 2011...

Purpose Growth retardation is a severe morbidity in infants with congenital diaphragmatic hernia (CDH). This study aimed to determine when catch-up growth starts in infants with CDH and to determine the adequate amount of nutrition required during catch-up growth. Methods This was a multicenter retrospective cohort study involving neonates with is...

This is the first report of an immature sacrococcygeal teratoma with inguinal lymph node metastasis, providing the histologic transformation of an immature teratoma in association with chemotherapy. Incomplete tumor resection with coccygectomy was performed, and the histopathologic diagnosis was a grade 3 immature teratoma. Following the initial su...

PurposeTo date, there is no compelling evidence of the optimal timing of surgery for congenital diaphragmatic hernia (CDH). This study aimed to establish the optimal timing of surgery in neonates with isolated left-sided CDH.Methods This multicenter cohort study enrolled 276 patients with isolated left-sided CDH at 15 institutions. Based on the tim...

Aim: To present the feasibility, safety and outcomes of fetoscopic endoluminal tracheal occlusion (FETO) for the treatment of severe congenital diaphragmatic hernia (CDH). Methods: This was a single-arm clinical trial of FETO for isolated left-sided CDH with liver herniation and Kitano Grade 3 stomach position (>50% stomach herniation into the r...

Congenital pulmonary airway malformation (CPAM) is a congenital disease in which cystic lesions form within the pulmonary parenchyma, because the development of the airway or lung are partially interrupted in the fetal lung development. When considering the indications, timing, procedure, and other aspects of surgery for CPAM, it is imperative to a...

PurposeThis study aimed to elucidate the clinical characteristics of neonates with congenital diaphragmatic hernia (CDH) associated with pneumothorax and evaluate the risk factors for the development of pneumothorax.MethodsA retrospective cohort study was conducted in the 15 institutions participating in the Japanese CDH Study Group. A total of 495...

Localization of a microcystic lymphatic malformation within the diaphragm is an extremely rare vascular anomaly of the lymphatic system. We report the case of a 3-year-old boy who had a localized intrathoracic mass that was detected after birth. As the feeding artery to the mass was detected on computed tomographic images before surgery, we suspect...

Purpose To establish and validate risk models of mortality and morbidity associated with 12 major pediatric surgical procedures using the National Clinical Database-Pediatric (NCD-P) data. Methods We used the NCD-P data for the development and validation datasets. By using multivariate logistic regression to analyze the development dataset, we cre...

Background/purpose: The aim of this study was to evaluate our prenatal risk stratification system for risk-adjusted management in fetuses with isolated congenital diaphragmatic hernia (CDH). Methods: Ninety-four infants prenatally diagnosed with isolated CDH treated between 1998 and 2017 at our institution were included in this retrospective sin...

Introduction We aimed to establish and validate a risk score for fetuses with congenital diaphragmatic hernia (CDH) using only prenatal ultrasound findings. Material and Methods Derivation (2011–2016, n = 350) and validation (2006–2010, n = 270) cohorts were obtained from a Japanese CDH study group database. Using a logistic regression analysis, we...

Purpose The aim of this study was to evaluate the indications and the clinical outcomes of the fetuses managed with ex utero intrapartum treatment (EXIT) procedures. Methods We retrospectively reviewed the medical records of all fetuses who underwent EXIT procedures between 2003 and 2018. Results EXIT procedures were performed in nine cases. The...

Background The optimum enteral (EN) and parenteral nutrition (PN) regimes during acute management of congenital diaphragmatic hernia (CDH) remain unclear. We aimed to examine the effects of EN and PN on weight gain in CDH patients. Methods Multicenter retrospective cohort study including neonates with CDH (born 2006–2010; n = 105) who survived to...

Background Sacrococcygeal teratoma (SCT) is the most common extragonadal germ cell tumor in neonates and infants. Although most cases of infantile SCT are benign tumors by nature, some develop into extremely large lesions, leading to massive bleeding, high‐output heart failure, disseminated intravascular coagulation, and even fatal outcomes during...

Purpose Airway obstruction caused by lymphatic malformation (LM) in the head and neck may require a tracheostomy. We present the results of our analysis of a nationwide survey on the indications for tracheostomy in children with head and neck LM. Methods We analyzed data in relation to tracheostomy based on a questionnaire about 518 children with...

Purpose: Clinical guidelines on lymphatic malformation (LM) influencing the airway have been crafted in the Research Project for Intractable Diseases. We herein report an analysis of a nationwide survey of mediastinal LM and the therapeutic recommendations. Methods: Eighty-seven registered cases with mediastinal involvement were analyzed with a...

Purpose: The standard treatment for Wilms tumor (WT) is primary resection. However, in cases with unresectable tumor or tumor spillage, which are considered to have high surgical risks, more intensive chemotherapy and radiotherapy are required. In the present study, we retrospectively analyzed preoperative image parameters to identify factors asso...

Purpose: To clarify the characteristics of gastroesophageal reflux (GER) in pediatric patients with neurological impairment (NI). Methods: Twenty-six patients with NI, 1-17-year-old, fed via a nasogastric tube were categorized based on the absence (group I) or presence (group II) of abnormal GER, defined with multichannel intraluminal impedance-...

Aim: Thoracoscopic repair (TR) of esophageal atresia with tracheoesophageal fistula (EA/TEF) remains a considerable challenge, even for the most experienced pediatric surgeons. The aim of this study is to report the outcomes of our experience with TR of EA/TEF and to determine the learning curve for this procedure. Methods: Eleven consecutive ca...

Objectives: To determine the characteristics, outcomes and prognostic factors of fetal hydrothorax (FHT) with trisomy 21. Methods: A nationwide survey was conducted on FHT fetuses with trisomy 21 delivered after 22 weeks' gestation between January 2007 and December 2011 at perinatal centers. Results: The 91 cases of FHT with trisomy 21 include...

Ehlers-Danlos syndrome (EDS) is an inherited connective tissue disorder that is often associated with pectus excavatum (PE). The Nuss procedure, which is a minimally invasive approach for the treatment of PE achieves good functional and cosmetic outcomes. We experienced a case of EDS-associated asymmetric PE that was corrected by the Nuss procedure...

Objective: To establish a simple risk stratification system for patients with congenital diaphragmatic hernia (CDH) based on postnatal information within 24 h after birth. Study design: A multi-institutional retrospective cohort study was conducted including 348 neonates who had isolated CDH born between 2006 and 2010. Based on the two most powe...

Purpose: This study was aimed to evaluate the influence of timing of surgery on patient outcomes, and to clarify appropriate timing of surgery in neonates with congenital diaphragmatic hernia (CDH). Methods: A total of 477 neonates with isolated CDH were included. Patients were classified into two groups by timing of surgery: early repair (ER) (...

Purpose: To investigate the long-term morbidity of surgically treated esophageal atresia (EA) in adolescents and young adults and establish whether these long-term morbidities are affected by the type of EA. Patients and methods: We reviewed the medical records, including backgrounds and associated conditions, of 69 long-term survivors of EA, ag...

Objective: To seek a simple approach for prenatally classifying congenital diaphragmatic hernia (CDH) severity using fetal magnetic resonance imaging (MRI) markers. Study design: A retrospective, multicenter study using questionnaires to investigate fetal MRI findings. We included fetuses prenatally diagnosed with isolated left-sided CDH and del...

Objectives: To determine the prognostic factors and outcomes of primary fetal hydrothorax (FHT) and investigate the effects of fetal therapy. Methods: A nationwide survey was conducted on fetuses with primary FHT delivered after 22 weeks of gestation between January 2007 and December 2011 at perinatal centers. Results: Among 287 cases of prima...

Purpose: The current study aimed to assess the perinatal risk and clinical features of congenital cystic lung diseases (CCLD). Materials and methods: Of the 874 CCLD patients identified in a nationwide survey, 428 patients born between 1992 and 2012 and treated at 10 high-volume centers, were retrospectively reviewed. Results: Fetal hydrops wa...