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.

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.

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.

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.

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.

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.

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.

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.

Interleukin 6 and interleukin 8 play important roles in systemic inflammatory response syndrome of meconium peritonitis.

PURPOSE: Meconium peritonitis is caused by an intestinal perforation that may occur in the fetus, followed by severe chemical peritonitis, resulting in high morbidity. METHODS: We have experienced six patients with meconium peritonitis. Cystic drainage was performed soon after birth for all patients. We investigated the concentrations of several cytokines and a chemokine (interleukin 8) in the ascites from the six patients with meconium peritonitis. In two patients we also measured the serum cytokines and chemokine level just after birth. RESULTS: Interleukin 6 and interleukin 8 concentrations were very high in the cyst or ascites just after birth. In the serum taken from two patients, the levels of interleukin 6 and interleukin 8 were also high. In five patients who underwent drainage of cysts after birth, systemic inflammation could not be completely suppressed before curative surgery. CONCLUSIONS: Interleukin 6 and interleukin 8 play important roles in the inflammatory response syndrome associated with meconium peritonitis, and drainage of cystic fluid did not completely suppress this inflammation. To lessen the high morbidity of meconium peritonitis, efforts should be made to suppress the inflammatory response using new treatment strategies, such as administration of steroids or anti-cytokine therapy to supplement cystic drainage.

New idea of intestinal lengthening and tailoring.

INTRODUCTION: Both the Bianchi and the serial transverse enteroplasty (STEP) procedure have been reported to be successful in short bowel syndrome, but both have their limitations. The Bianchi technique is surgically challenging and can only tailor the diameter to half and double the length. The STEP procedure is easy, adjustable, but it changes the orientation of the muscle fibres making the circular; longitudinal and the longitudinal; circular. We have created a model to test our idea of Spiral Intestinal Lengthening and Tailoring (SILT). MATERIAL AND METHODS: A double layer intestinal simulator was used as the bowel model. The orientation of the circular muscle fibres was marked. The simulator was cut spirally at a 45° and 60° angle, stretched longitudinally and retubularised. The procedure was adapted to porcine small bowel ex vivo and mucosal microcirculation was observed in three separate points by intravital videomicroscopy in vivo. RESULTS: The simulator was lengthened by 60% and the diameter was tailored by 33% using the 45° spiral cut, 73% lengthening and 44% tailoring was achieved at 60°. The circular muscle fibres showed oblique orientation. The porcine bowel was lengthened by 136 ± 21% and the diameter was tailored by 56 ± 8%. The linear regression analysis of variants showed significant linear regression R = 0.9689 (R(2) = 0.9388), p = 0.0014. The angle of the spiral cut (α) showed the relation: α ≥ 90-arc sin R(2)/R(1). (R(1) is the radius of the original segment and R(2) the desired radius of the tailored and lengthened bowel). The bowel remained viable macroscopically 90 min after the procedure. The median red blood cell velocity reduced from 570 (control) to 558 µm s(-1) in point 1, to 382 µm s(-1) in point 2, and to 482 µm s(-1) in point 3. Oscillation of the capillary flow has not been observed. CONCLUSION: The SILT may be an easy, more physiological and adjustable alternative to the existing techniques.

Reliability of the lung to thorax transverse area ratio as a predictive parameter in fetuses with congenital diaphragmatic hernia.

PURPOSE: An accurate prenatal assessment of the patients' severity is essential for the optimal treatment of individuals with congenital diaphragmatic hernia (CDH). The purpose of this study was to clarify the reliability of the lung to thorax transverse area ratio (L/T) as a prenatal predictive parameter. METHODS: A multicenter retrospective cohort study was conducted on 114 isolated CDH fetuses with a prenatal diagnosis during the period between 2002 and 2007 at five participating centers in Japan. The relationship between the gestational age and the L/T was analyzed. The most powerful measurement point and accurate cutoff value of the L/T was determined by an analysis of a receiver operating characteristic curve, which was verified by comparing the patients' severity. RESULTS: There was a negative correlation between the gestational age and the L/T in the non-survivors, and no correlation in the survivors. There were significant differences in the parameters which represented the patients' severity including the respiratory and circulatory status, the surgical findings, and the final outcomes between the groups divided at 0.080 in the minimum value of the L/T during gestation. CONCLUSION: The L/T was not strongly influenced by the gestational age, and it was found to be a reliable prenatal predictive parameter in fetuses with isolated CDH.

The Japanese experience with prenatally diagnosed congenital diaphragmatic hernia based on a multi-institutional review.

PURPOSE: To review the recent Japanese experience with prenatally diagnosed congenital diaphragmatic hernia (CDH) based on a multi-institutional survey. METHODS: A multicenter, retrospective cohort study was conducted on 117 patients born between 2002 and 2007 with isolated prenatally diagnosed CDH. All patients were managed by maternal transport, planned delivery, immediate resuscitation and gentle ventilation. The primary outcome measurements were the 90-day survival and intact discharge. The examined prenatal factors included gestational age (GA) at diagnosis, lung-to-head ratio (LHR), lung-to-thorax transverse area ratio (L/T) and liver position. Physical growth and motor/speech development were evaluated at 1.5 and 3 years of age. Data were expressed as the median (range). RESULTS: The mean GA at diagnosis was 29 (17-40) weeks. The LHR and L/T were 1.56 (0.37-4.23) and 0.11 (0.04-0.25), respectively. There were 48 patients with liver up. The mean GA at birth was 38 (28-42) weeks. The 90-day survival rate and intact discharge rate were 79 and 63%, respectively. Twelve patients had major morbidity at discharge, and 71% of these patients had physical growth or developmental retardation at 3 years of age. CONCLUSION: This multicenter study demonstrated that the 90-day survival rate of isolated prenatally diagnosed CDH was 79%, and that subsequent morbidity remained high. A new treatment strategy is needed to reduce the mortality and morbidity of severe 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.

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.

Sonic hedgehog gene expression in nitrofen induced hypoplastic lungs in mice.

BACKGROUND: The pathogenesis of congenital diaphragmatic hernia (CDH) is unknown. The sonic hedgehog (Shh) cascade is crucial for the patterning of the early respiratory system in mice. Optical Projection Tomography (OPT) is a new tool for 3D imaging small biological specimens that enables us to visualise both the anatomy of developing organs and gene expression localised in the context of normal or abnormal anatomy. We visualised Shh gene expression patterns in the nitrofen treated and control mouse lung buds at early stages of lung development. MATERIALS AND METHODS: Time-mated CD1 female mice (n = 5) received oral administration of 100 mg of the herbicide 2,4-dichlorophenyl-p-nitrophenylether (nitrofen) (WAKO Chemical, Osaka, Japan) in 1 ml of olive oil or olive oil alone at 7 days of gestation. Embryos were harvested on gestation days 9-12, and stained following whole mount in situ hybridisation with labelled RNA probes to detect Shh transcripts at each stage. Embryos were scanned by OPT to obtain 3D representations of gene expression domains in the context of the changing morphology of the embryo. RESULTS: OPT analysis of Shh transcript distribution clearly revealed gene expression in both groups. In treated embryos, there were no significant changes in Shh transcript distribution in lung buds in comparison with control embryos. CONCLUSION: Although altered Shh expression in the hypoplastic lung has been reported in late gestation, the present study did not reveal any significant alterations in pulmonary Shh spatial transcript distribution or gene expression level during the early gestation in nitrofen CDH model. It would be of great interest in future studies to use OPT approach to investigate pulmonary expression of Shh and other regulatory genes both during early and late stages of lung development in order to provide new insights into the pathogenesis of pulmonary hypoplasia.

Repeat STEP procedure to establish enteral nutrition in an infant with short bowel syndrome.

The serial transverse enteroplasty (STEP) procedure is a safe and successful way to lengthen the small bowel in patients with short bowel syndrome. However, postoperative dilatation of the intestine may occur, which induces bacterial overgrowth and malabsorption leading to liver failure. We describe the case of an infant boy with short bowel syndrome caused by jejunal atresia requiring the STEP procedure twice. The first STEP improved the liver function, and the second STEP allowed 80% of the total calorie intake to be tolerated enterally. One should not hesitate to perform a second STEP if after the initial bowel lengthening procedure the patient develops small bowel dilatation that interferes with enteral nutrition.

A novel association of duodenal atresia, malrotation, segmental dilatation of the colon, and anorectal malformation.

We describe two female neonates who suffered from four gastrointestinal anomalies, including duodenal stenosis or atresia, malrotation, segmental dilatation of the colon, and anorectal malformation. Each patient was managed by two or three operations, resulting in good bowel movements. Since this is the first report of four gastrointestinal anomalies, these cases may provide clues to elucidate the etiology of gastrointestinal tract developmental abnormalities.

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.

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.