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Background and Aims: We performed thoracoscopic costal cartilage excision (TCCE) combined with the Nuss procedure to correct asymmetrical pectus excavatum (PE). We reviewed the efficacy of combined TCCE and Nuss procedure for asymmetric PE. Patients and Methods: Overall, 8 patients with asymmetrical PE underwent TCCE with the Nuss procedure. The Haller index, asymmetry index, and angle of sternal rotation were calculated using preoperative computed tomography. The procedure was performed using bilateral 2.5-cm incisions at the same level of the deepest chest wall depression. The most depressed three to four costal cartilages were partially resected through a right mini-thoracotomy. Subsequently, one or two titanium bars were implanted and secured with stabilizers. The cosmetic outcome was evaluated on the following four ratings: excellent, good, fair, and failure ( = recurrence). Results: The median age at surgery was 14.5 years (8-20 years). The number of bars was one in 3 cases and two in 5 cases. The preoperative Haller index, asymmetry index, and angle of sternal rotation were 4.3 (3.5-5.9), 1.15 (1.04-1.26), and 21.5° (15°-31°), respectively; 2 patients had scoliosis before the Nuss procedure. Complications included surgical site infection and hemothorax. Median follow-up time was 25.5 months (3-63). Bars were removed in 3 patients, 3 years postoperatively. Cosmetic results were excellent, 4; good, 2; fair, 1; failure, 1. Both patients with scoliosis had poor outcomes (fair, 1; failure, 1). Conclusions: Combined TCCE with Nuss procedure is considered safe and effective for patients with asymmetrical PE. Careful long-term follow-up is required, especially in cases with scoliosis. [ABSTRACT FROM AUTHOR]

Thoracoscopic repair (TR) of esophageal atresia (EA) has been performed with increasing frequency over the last two decades, with the expectation of improved outcomes by avoiding thoracotomy. To understand the current practice and outcomes of TR of EA, we reviewed the relevant literature, including 15 case series, 7 comparative studies, and 3 meta-analysis comparing TR with conventional open repair (COR). Most of the studies had a retrospective design and small numbers of patients. Although the evidence level is low because of the lack of prospective studies, this review found that TR is as safe as COR, with comparative outcomes. Moreover, there were several advantages of TR over COR, such as less blood loss and a shorter hospital stay. The long-term outcomes of TR remain unclear because of limited data. Moreover, there is a significant learning curve over the first 10–20 TRs performed. We conclude that TR of EA, when conducted by experienced surgeons, is a safe and minimally invasive alternative to COR and may yield better results than COR in appropriately selected patients. [ABSTRACT FROM AUTHOR]

Congenital esophageal stenosis (CES) is rare and usually manifests in infants as dysphagia, failure to thrive, and food impaction. Dilatation is considered to be the first-line therapy for CES, but the incidence of complications (perforation and mediastinitis) is relatively high. We report two cases of CES treated by thoracoscopic resection without prior dilatation. Both infants recovered without recurrent stenosis and were able to eat solid food soon after surgery. One had postoperative gastroesophageal reflux and eventually required fundoplication. Thoracoscopic resection could be a valid option for CES. [ABSTRACT FROM AUTHOR]

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, aged >15 years. The long-term morbidities included neurodevelopmental abnormality, nutritional impairment (short height <−2SD, low BMI <18.5), subjective symptoms, and musculoskeletal deformities. Comparisons of the results were made between Gross A-type EA ( n = 6) and Gross C-type EA ( n = 63). Results: All patients underwent esophageal anastomosis without esophageal replacement. Cardiac anomalies and long gap were present in 26 and 18%, respectively. Esophageal dilatation, fundoplication, and aortopexy were performed in 40, 34, and 18%, respectively. The incidence of long gap and esophageal stenosis was higher in Gross A-type EA than in Gross C-type EA. The long-term morbidities included neurodevelopmental abnormality (13%), nutritional impairment (62%: as short height in 34% and as low BMI in 46%), subjective symptoms (14%), and musculoskeletal deformities (59%). There were no differences in the long-term morbidities between Gross A and Gross C. Conclusions: The incidence of the long-term morbidities is high in adolescents and young adults, regardless of the type of EA. Early detection of morbidity is important to improve the long-term outcomes of EA. [ABSTRACT FROM AUTHOR]

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) (≤48 h) and delayed repair (DR) (>48 h). The primary outcome was 90-day survival, with treatment duration (ventilation, oxygen, and hospitalization) being a secondary outcome. To adjust for disease severity, patients were stratified into three severities by Apgar score 1 min ("mild" 8-10, "moderate" 4-7, and "severe" 0-3), and outcomes were compared between ER and DR within each severity.Results: Although 90-day survival was significantly different among the three severities ("mild" 97%, "moderate" 89%, and "severe" 76%, p = 0.002), there were no differences in 90-day survival between DR and ER within each severity. In "mild", there were no differences in treatment duration between ER and DR. In "moderate", treatment duration was shorter in ER than DR (ventilation 11 vs. 16 days, oxygen 15 vs. 20 days, and hospitalization 34 vs. 48 days). In "severe", treatment duration was shorter in ER than DR, while the best OI was higher in DR than ER.Conclusions: Timing of CDH repair seems to have no influence on 90-day survival regardless of disease severity. Patients with moderate severity may benefit from the early repair by reducing treatment duration. [ABSTRACT FROM AUTHOR]

Background Very low-birthweight (VLBW) infants (VLBWI) are at increased risk for surgical intestinal disorders including necrotizing enterocolitis (NEC), focal intestinal perforation (FIP) and meconium-related ileus (MRI). The aim of this study was to identify disease-specific risk factors for surgical intestinal disorders in VLBWI. Methods A retrospective multicenter case-control study was conducted at 11 institutes. We reviewed VLBWI who underwent laparotomy for intestinal disorders including perforation and intractable bowel obstruction. The surgical disorders were classified into four categories (NEC, FIP, MRI, others) based on the macroscopic findings at operation. In order to identify risk factors, two matched controls for each subject were chosen based on gestational age and birthweight. OR and 95%CI were calculated using a conditional logistic regression model and a multivariate model. Results A total of 150 cases (NEC, n = 44; FIP, n = 47; MRI, n = 42; others, n = 17) and 293 controls were identified. The cases and controls were similar in terms of gestational age and birthweight (cases/controls, 26.7 ± 2.5/26.5 ± 2.6 weeks; 790 ± 256/795 ± 257 g). On multivariate modeling, disease-specific risk factors were as follows: female (OR, 0.23; 95%CI: 0.06-0.89), respiratory distress syndrome (OR, 35.7; 95%CI: 2.48-514) and patent ductus arteriosus (OR, 10.9; 95%CI: 1.51-79.3) for NEC; outborn delivery (OR, 5.47; 95%CI: 1.48-20.2) for FIP; and twin pregnancy (OR, 4.25; 95%CI: 1.06-17.1), PROM (OR, 6.85; 95%CI: 1.33-35.4) and maternal steroid (OR, 0.23; 95%CI: 0.07-0.79) for MRI. Conclusions Different risk factors were identified for NEC, FIP and MRI, suggesting that each disease has a different etiology, and that different strategies are required to prevent these diseases. [ABSTRACT FROM AUTHOR]

Background: In order to better understand the current practice and outcomes of thoracoscopic repair of esophageal atresia (EA)/tracheoesophageal fistula (TEF), a multi-institutional analysis was conducted among seven Japanese institutes. Materials and Methods: A survey was sent to the seven institutes regarding the surgical technique, postoperative management, and outcomes of thoracoscopic repair of EA/TEF. Results: The operation was uniformly performed via an intrapleural approach in the 0-45° prone position. The TEF was occluded with suture ligature in four (57.1%) institutes and clips in the remaining three (42.9%) institutes. Anastomosis was performed using the extracorporeal knot-tying technique in four institutes and the intracorporeal technique in three institutes. Patients were routinely left intubated and paralyzed for 3-7 days postoperatively in four institutes. In total, 58 patients underwent thoracoscopic repair of EA/TEF. Fifty-two (89.7%) of the patients underwent successful thoracoscopic repair. Six (10.3%) operations were converted to open thoracotomy because of a long gap ( n=4), right aortic arch ( n=1), and intraoperative instability ( n=1). The body weight at operation ranged from 1.2 to 4.6 kg, and the operative time ranged from 115 to 428 minutes. There were no major intraoperative complications. Eleven patients (19.0%) suffered from anastomotic leakage. Twenty-eight patients (48.3%) developed anastomotic stricture. One patient died during the postoperative period because of an unrelated disease. Recurrent TEF developed in three patients (5.2%). Thirteen patients (22.4%) later required fundoplication. Conclusions: The outcome of thoracoscopic repair of EA/TEF was comparable to that of the open procedure. As considerable variability was observed among the seven institutes with respect to the surgical technique and management, standardizing the surgical management may improve the outcome. [ABSTRACT FROM AUTHOR]

Background: There are few reports of the laparoscopic findings of the internal inguinal ring (IIR) in patients with hydrocele. The purpose of this study was to assess the safety and efficacy of laparoscopic percutaneous extraperitoneal closure (LPEC) for hydrocele in comparison with that of open repair (OR) and compare the findings of the IIR between cases of hydrocele and inguinal hernia (IH). Materials and Methods: We analyzed 69 consecutive patients with hydrocele who underwent surgery at our institution between April 2009 and February 2014. The patients were divided into two groups (LPEC and OR) according to the procedure. Age, length of operation/anesthesia, and complications were compared. Concerning the findings of the IIR, we classified the features into three categories, as follows: Type 1, flat; Type 2, narrow patent processus vaginalis (PPV) with a peritoneal veil; and Type 3, widely opened PPV. We then compared these findings between the cases of hydrocele and IH treated with LPEC during the study period. Results: Among a total of 69 patients, 40 underwent LPEC, and 29 underwent OR. There were no significant differences in the length of operation/anesthesia and complications. No recurrences were observed in either group. The findings of the IIR were mostly classified as Type 2 (59.1%) among the cases of hydrocele and Type 3 (92%) among the cases of IH. Conclusions: LPEC is a safe and effective procedure for treating hydrocele. The findings of the IIR differ between cases of hydrocele and IH. [ABSTRACT FROM AUTHOR]

Introduction: Laparoscopic procedures for inguinal hernias and hydroceles in children have become widespread in the past few decades. The purpose of this study was to perform a retrospective analysis of our experience in order to assess the safety and efficacy of laparoscopic percutaneous extraperitoneal closure (LPEC) and to compare the findings with those of conventional open repair (OR). Subjects and Methods: We analyzed the medical records of 488 patients who underwent LPEC or OR for inguinal hernia or hydrocele at our institute between April 2008 and December 2012. The indications for the operation, length of the operation, complications, day surgery, contralateral patent processus vaginalis, and incidence of metachronous contralateral hernia were investigated. The chi-squared test, unpaired t test, and Steel-Dwass test were used to analyze the significance of the data. Results: Among a total of 488 patients, 326 patients underwent LPEC (125 males and 201 females), and 162 underwent OR (140 males and 22 females). There was no significant difference in the incidence of recurrence (three in the LPEC and none in the OR group, P=.55) or in the success rates of day surgery (97.8% in LPEC versus 97.6% in OR). The incidence of metachronous contralateral hernias in the LPEC group was lower than that in the OR group (LPEC 0%; OR 2.2%, P=.03). Seventeen subjects with hydroceles were treated by LPEC without any complications. Conclusions: LPEC is safe and effective for inguinal hernias and hydroceles in children, regardless of age, sex, and incarceration and could reduce the incidence of metachronous contralateral hernias. [ABSTRACT FROM AUTHOR]

Introduction: We report four cases of long-gap esophageal atresia (LGEA) treated with thoracoscopic esophagoesophagostomy (TEE) after sequential extrathoracic esophageal elongation (SEEE: Kimura's technique). Methods: All initially had gastrostomy, then SEEE. The proximal end of the esophagus was introduced into the apex of the thorax under direct vision during TEE. Results: Mean birth weight was 1.9 kg. Mean gestational age was 35.0 weeks (range 30-39); mean age at initial esophagostomy was 43.2 days (range 15-110); SEEE was performed for a mean of 3 times (range 2-4) at mean intervals of 5.3 months (range 2-10), with the upper esophageal segment lengthened by a mean of 2.1 cm each time (range 1.1-3.5). Mean age and weight at TEE were 22.3 months and 9.8 kg, respectively. Gap was initially 4.5 vertebrae (range 4-5). Mean operating time was 9.6 h. TEE was successful in 3; 1 required thoracotomy, then re-anastomosis after 11 months of anastomosis leakage; 2 cases with post-TEE anastomosis leakage were treated conservatively. Postoperatively, all cases required fundoplication and esophageal dilatation (2, 3, 5, 8 times, respectively). At mean follow-up of 3.6 years, 2 eat normally and 2 eat minced food. Conclusions: TEE after SEEE appears to be a feasible option for treating LGEA. [ABSTRACT FROM AUTHOR]

The article presents a case study of a female infant who has multiple associated anomalies such as left pulmonary agenesis, total anomalous pulmonary venous return (TAPVR), and anovestibular fistula. It uses expandable metallic stents (EMS) with a silicon stent to treat extrinsic tracheal obstruction in an effort avoid the occurrence of serious respiratory complications. The use of tracheoscope is discussed.

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. [ABSTRACT FROM AUTHOR]

The lung to thorax transverse area ratio (L/T ratio) and the lung area to head circumference ratio (LHR) have been widely used for the assessment of pulmonary hypoplasia in fetal congenital diaphragmatic hernia (CDH). The aim of this study was to evaluate the relationship between the L/T ratio and the LHR, and to clarify the characteristics of these two indicators as prognostic predictors by means of retrospective concurrent measurements from the same subjects with prenatally diagnosed fetal CDH. The medical records of 55 fetuses who had undergone a prenatal evaluation of isolated CDH from 1988 to 2006 were studied. The L/T ratio and the LHR were determined as the early values (earliest measurement performed earlier than 33 weeks of gestation) and as the late values (latest measurement performed later than 34 weeks of gestation) and analyzed, as well as the clinical data. Of the 55 infants, 13 died resulting in a 76.4% survival rate. A correlation expressed in the linear equation [(LHR) = 14.4 × (L/T ratio) − 0.11] was recognized between the early L/T ratio and the early LHR. All cases with an early L/T ratio of less than 0.08, or with an early LHR less than 1.2, died. Of the 13 cases, 5 with an early L/T ratio not lower than 0.08, but less than 0.13, died. Of the 17 cases, 4 with an early LHR not lower than 1.2, but less than 2.0, died. All cases with an early L/T ratio not lower than 0.13, or with an early LHR not lower than 2.0, survived. In 24 cases, the late values, which were measured at an interval of more than 4 weeks, were compared with the early values. Although the L/T ratio was consistent, the LHR increased in the late value compared to the early value. A good linear correlation was recognized between the L/T ratio and the LHR in the early phase of gestation, and the cutoff point of the prognostic prediction was determined in both indicators. In contrast to the L/T ratio, a definite cutoff point throughout the gestation may not be available in the LHR, because there is a natural increase of the LHR in the late phase of gestation. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 4-year-old child with tracheal agenesis where the esophagus was reconstructed with a jejunal pedicle graft. The patient underwent esophageal reconstruction and laparotomy to take down the esophageal banding and to transect the esophagus at the esophagocardiac junction. The article discusses the classification of tracheal agenesis based on the degree of distal tracheobronchial development and the fistulous connection to the esophagus.

We report a case of tracheal obstruction caused by an expandable metallic stent. A 3-month-old girl with severe tracheomalacia had a placement of a Palmaz stent. At 3 years of age, she developed progressive dyspnea. The CT scan showed tracheal obstruction caused by granulation tissue over the stent. At operation, the stent was found to have penetrated the posterior tracheal wall. Under partial cardiopulmonary bypass, the stent was removed along with the membranous wall of the trachea, and the trachea was reconstructed using slide tracheoplasty. Tracheal obstruction is one of the serious complications caused by an expandable metallic stent. Direct open approach to the trachea under cardiopulmonary bypass is thought to be a safe way to manage this problem. [ABSTRACT FROM AUTHOR]

Purpose: Congenital esophageal stenosis (CES) associated with esophageal atresia (EA) is rare, and no standard treatment has been established. We reviewed cases of EA-associated CES to assess the clinical characteristics and treatment outcomes, especially the feasibility of endoscopic dilatation. Methods: We retrospectively examined patients with EA-associated CES. We also compared treatment outcomes of EA-associated CES with those of EA patients without CES who developed postoperative anastomotic stricture. Results: Among 44 patients with EA, ten had CES (23%). Postoperative complications were not significantly different between EA patients with CES and those without CES but with anastomotic stricture. All CES patients underwent balloon dilatation as initial treatment. Eight of nine patients (89%) were successfully treated by dilatation only, and one patient underwent surgical resection. The median number of balloon dilatations for CES was five (2–17), which was higher than that for anastomotic stricture in patients without CES (p = 0.012). Esophageal perforation occurred in five patients with CES (5/9, 56%) after dilatation, but all perforations were successfully managed conservatively with an uneventful post-dilatation course. Conclusions: Twenty-three percent of patients with EA had CES. Although balloon dilatation for EA-associated CES required multiple treatments and carried a risk of perforation, balloon dilatation showed an 89% success rate and all perforations could be managed conservatively. [ABSTRACT FROM AUTHOR]

The treatment of long-tracheal lesion is difficult because there are currently no viable grafts for tracheal replacement. To solve this problem, we have developed an autologous Tissue-Engineered Trachea (aTET), which is made up of collagenous tissues and cartilage-like structures derived from rat chondrocytes. This graft induced successful long-term survival in a small-animal experiment in our previous study. In this study, we investigated the regeneration process of an aTET to attain reproducible success. We prepared an aTET by using a specially designed mold and performed patch tracheoplasty with an aTET. We assigned twenty-seven rats to three groups according to the three types of patch grafts used: aTET patches (the aTET group), fresh tracheal autograft patches (the Ag group), or polylactic acid and polycaprolactone copolymer sheets (the PPc group). In each group, gross and histological evaluations were performed at 1 month (n = 3), 3 months (n = 3), and 6 months (n = 3) after implantation. We obtained high survival rates in all groups, but only the PPc group attained thick tracheal walls with granular tissues and no tracheal regeneration. On the other hand, the aTET and Ag groups reproducibly achieved complete tracheal regeneration in 6 months. So, an aTET could be a promising candidate for tracheal regeneration grafts. [ABSTRACT FROM AUTHOR]

Patients who undergo pediatric living donor liver transplantation (LDLT) sometimes develop graft fibrosis. Recently, Mac-2 binding protein glycosylation-modified isomer (M2BPGi) was developed as a new marker of hepatic fibrosis progression. We performed this study to examine the relationship between serum M2BPGi levels and liver histologic findings in patients after LDLT for biliary atresia. Patients aged <19 years who underwent LDLT for biliary atresia at our institution and followed up for at least 1 year after LDLT were eligible. There were 56 patients in this study. Pathologic findings of the last available biopsy were assessed. Portal vein (PV) stenosis was confirmed with angiography. M2BPGi levels were compared with pathologic fibrosis scores and PV stenosis findings. The mean age at transplant was 4.3 years. The mean observation period was 8.6 years. In terms of the degree of liver fibrosis, F0 was observed in 7 patients, F1 in 36, and F2 in 13. The median serum M2BPGi value was 0.8 cut-off index (COI) overall and 0.60 COI for F0, 0.74 COI for F1, and 1.07 COI for F2. The mean M2BPGi value in F2 was higher than that in F0 (P =. 016) and F1 (P =. 012). Mean serum M2BPGi values were 1.57 COI (0.29 COI) in patients with PV complications (n = 5) and 0.72 COI in patients without PV complications (n = 51) (P =. 0001). M2BPGi is a novel marker for liver fibrosis in patients after pediatric LDLT. It is especially useful for follow-up of pediatric patients after LDLT to support liver biopsy interpretation. [ABSTRACT FROM AUTHOR]

The COL4A1 (collagen Type 4 alpha1) pathogenic variant is associated with porencephaly and schizencephaly and accounts for approximately 20% of these patients. This gene variant leads to systemic microvasculopathy, which manifests as brain, ocular, renal, and muscular disorders. However, only a few patients with surgical interventions have been reported and the potential surgical risks are unknown. Here, we present the cases of two female patients between 7 and 8 years of age who were diagnosed with the COL4A1 variant and underwent laparoscopy‐assisted percutaneous endoscopic gastrostomy (LAPEG) for oral dysphagia. Their primary brain lesions were caused by porencephaly and paralysis, which are caused by multiple cerebral hemorrhages and infarctions, and both patients had refractory epileptic complications. Although LAPEG was successfully performed in both patients without any intraoperative complications, one patient developed alveolar hemorrhage postoperatively and required mechanical ventilation. Thus, careful perioperative management of patients with the COL4A1 variant is important. [ABSTRACT FROM AUTHOR]

Abstract: 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 cases that had undergone TR of EA/TEF at our institutes were included in this study. The medical charts were reviewed retrospectively. To determine the learning curve for TR of EA/TEF, a logarithmic curve‐fitting analysis was performed. The data were expressed as medians with ranges. Results: The median age and birth weight were 1 day (range, 1–3 days) and 2.8 kg (range, 2.5–3.7 kg), respectively. TR was completed in all cases without any complications. The median operative time was 230 min (range, 164–383 min). There were no cases of anastomotic leakage. One patient with a long gap required repeated balloon dilatation for refractory anastomotic stricture. No mortality or recurrence of tracheoesophageal fistula occurred. The operative time was significantly longer in patients with a long gap (>20 mm) than in those with a shorter gap. Once the three cases with a long gap had been excluded, the operative time decreased as the number of treated cases increased. The relationship between the operative time and case number fit a logarithmic function curve well (operative time in minutes = 300 – 62 × log (case number), R2 = 0.8359, P = 0.0015). Conclusions: Our results suggest that TR of EA/TEF is a safe procedure. It has a considerable learning curve, but requires advanced endoscopic surgical skills. [ABSTRACT FROM AUTHOR]

Liver failure and gastrointestinal bleeding occur in the end-stage of biliary atresia (BA). Living-donor liver transplantation (LDLT) is a standard treatment in Japan. Our program actively provides pre-transplant total parenteral nutrition (TPN) for such patients, and here we report its efficiency and safety. Patients with BA for whom LDLT was indicated were identified. Those with a long-term external central venous catheter and TPN, longer than 4 weeks before LDLT, were analyzed. Ascites was controlled with diuretics. TPN indications, efficacy, and complications were assessed along with patient growth, biochemical markers, and gastrointestinal bleeding. Fourteen patients were included in the study, of whom 8 were girls and 6 were boys. The median age at LDLT was 0.9 years. Body weight (BW) at TPN initiation averaged 6799 g, and the median serum total bilirubin was 9.5 mg per dL. The median catheterization duration was 54 days, and 1 patient received home TPN. Indications for TPN were gastrointestinal bleeding and/or massive esophageal varices in 4 patients and poor nutritional status in 10 patients. No complications were observed except for 1 catheter infection and 1 catheter occlusion. The median final body weight before LDLT was 7906 g. The mean rate of BW gain was significantly higher after TPN than before (149 vs 32 g/wk, respectively, P =.0002). Mean prothrombin time and levels of albumin, cholinesterase, and total bilirubin were not significantly different at the start and end of TPN. Pre-transplant TPN was safe and effective for patients with end-stage BA. [ABSTRACT FROM AUTHOR]

Pediatric living-donor liver transplantation (LDLT) candidates often receive long-term antibiotic treatment. Micafungin has been used as an antifungal agent after LDLT, but the adequate dose after pediatric LDLT was unknown. Here, we report micafungin blood concentrations after pediatric LDLT and discuss its safety and adequate dosing. Pediatric patients with data on micafungin concentrations after LDLT were identified. Those with surgical complications were excluded. All patients received standard tacrolimus-based immunosuppression. A micafungin dose of 1 mg/kg was administered once daily for 10 days starting on postoperative day (POD) 1. The trough and peak micafungin blood concentrations were evaluated on PODs 1, 4, 7, and 10. Beta D glucan levels and liver function tests were assessed to determine micafungin effectiveness and safety. Ten patients were enrolled, with a median age of 1.2 years. The median graft vs body weight ratio was 2.7%. The primary diseases were biliary atresia (n = 7), Alagille syndrome (n = 2), and progressive familial intrahepatic cholestasis type 2 (n = 1). Mean peak micafungin levels were 4.47, 6.27, 5.47, and 5.47 µg/mL on PODs 1, 4, 7, and 10, respectively. Mean trough levels were 2.03, 1.88, and 2.66 µg/mL on PODs 4, 7, and 10, respectively. The micafungin half-lives were 13.7, 14.7, and 14.0 hours on PODs 4, 7, and 10, respectively. Beta D glucan levels were 4.4 pg/mL and 3.7 pg/mL before and after transplantation, respectively, indicating no significant difference (P =.3). No clinical fungal infections were observed. Micafungin administration is safe and effective after pediatric LDLT. [ABSTRACT FROM AUTHOR]

Introduction: Intestinal transplantation (ITx) is the ultimate treatment for intestinal failure (IF). In Japan, most cases of IF are a result of pediatric disease, including secondary or congenital intestinal disease or allied disorders of Hirschsprung's disease. Here, we report the results of the Japanese ITx registry. Methods: A web-based survey form was completed. We investigated the number, age, sex, indication, surgical procedure, immunosuppressants, postoperative course, and the effects of transplantation in patients who underwent cadaveric or living-donor ITx. Results: By the end of 2022, 42 cases of ITx have been performed in 38 patients in Japan. The donor sources included cadavers (29 cases) and living donors (13 cases). The surgical method was isolated ITx (N = 40) and combined liver and ITx (n = 2). Survival rates were 92%, 73%, and 59% at 1 year, 5 years, and 10 years, respectively. Ninety percent of patients completely discontinued parenteral nutrition. Approximately 80% of the patients had a performance status of 1 or less, indicating that the QOL of patients after ITx was extremely good. Conclusion: The results of ITx are acceptable to treat IF patients and the QOL after transplantation is also good. [ABSTRACT FROM AUTHOR]

Background: Pediatric endoscopic surgery has become an alternative to conventional techniques with the development of medical equipment. However, there is no formal, standardized curriculum for pediatric endoscopic surgery, and its requirement remains elusive. The purpose of this study is to determine the baseline knowledge of pediatric surgeons that is considered essential for pediatric laparoscopic and thoracoscopic surgery. Methods: Sixteen web-based multiple-choice questions based on the fundamental cognitive knowledge of pediatric endoscopic surgery were administered. The questions were created based on the fields covered by the Fundamentals of Laparoscopic Surgery ™ (FLS) certification examination blueprints and eight specific diseases of pediatric surgery. Pediatric surgeons and pediatric surgical trainees participated in this study voluntarily. Results: A total of 122 surgeons participated through the Japanese Society of Pediatric Surgeons. The response rate was 95% (122/128). The total mean examination score of all participants was 79.4% (77.3–81.4%). There were no significant differences in total scores between the board-certified pediatric surgeons without an endoscopic surgical skill qualification and the non-board-certified pediatric surgeons (80.4% vs. 77.1%, p = 0.12). The endoscopic surgical skill-qualified surgeons had significantly higher percentages of correct responses in specific subjects than board-certified pediatric surgeons and surgeons without pediatric board certification (94.3% vs. 82.9%, p = 0.02; 94.3% vs. 77.5%, p = 0.0002). The FLS original subjects' scores were not significantly different among them. The mean score of surgeons who had experienced more than 200 cases of endoscopic surgery, including adult cases, was 83.2% (80.4–85.9%). Conclusions: A knowledge gap exists between surgeons, board-certified pediatric surgeons, and endoscopic surgical skill-qualified surgeons in Japan. In the field of pediatric surgery, an effective formal curriculum, such as FLS, is required to help address this vast knowledge gap for the safe conduct of endoscopic surgeries. [ABSTRACT FROM AUTHOR]

• The timing of liver transplantation in biliary atresia remains controversial. • Liver-spleen volume ratio (LSR) reflects splenomegaly and liver atrophy. • LSR could predict long-term native liver prognosis. • LSR may guide decisions about liver transplantation in biliary atresia patients. The appropriate timing of liver transplantation (LT) in patients with biliary atresia (BA) who survived with their native livers until adolescence remains controversial. The liver–spleen volume ratio (LSR) has been reported to be efficacious in predicting the prognosis of chronic liver disease. We investigated whether LSR could predict long-term native liver prognosis and serve as an indication for LT in patients with BA. Patients with BA who survived with their native liver until the age of 15 years were included. These patients were classified into 2 groups. The unfavorable prognosis group included patients who underwent or were awaiting LT or developed complications such as refractory cholangitis or gastrointestinal bleeding due to esophagogastric or intestinal varices. The favorable prognosis group included patients who survived with their native liver without complications. We compared the 2 groups regarding LSR, hematological, and histologic data. Of 19 patients, 8 were in the unfavorable prognosis group, and 11 were in the favorable prognosis group. LSR was significantly lower in the unfavorable prognosis group (P =. 009). Analysis of the receiver operating characteristic curve showed that the area under the curve of the LSR was 0.891, which was higher than the area under the curve of liver fibrosis markers. The optimal LSR cut-off value for predicting poor native liver prognosis was 1.97, with a sensitivity of 75.0% and a specificity of 87.5%. The LSR reflects splenomegaly and liver atrophy. The LSR might be a reliable predictor of native liver prognosis and could guide decisions about LT in patients with BA. [ABSTRACT FROM AUTHOR]

Purpose: Thoracoscopic repair (TR) of congenital diaphragmatic hernia (CDH) is associated with a higher recurrence rate than the conventional open method. We evaluated the effectiveness of our strategy for quality improvement, named "tension-free TR of CDH". Methods: The subjects of this retrospective analysis were 11 consecutive patients with CDH who underwent TR at our hospital between 2017 and 2021. Tension-free TR of CDH included the proactive use of an oversized patch for dome-shaped reconstruction and gapless suturing. We developed a percutaneous extracorporeal closure technique for secure suturing using a commercially available needle. Results: Patch repair was performed in 8 (73%) patients and none required conversion to open surgery because of technical difficulties. Recurrence developed in one patient (9%), who underwent successful reoperation via TR. All patients had an uneventful postoperative course. Conclusion: Tension-free TR combined with extracorporeal closure could reduce the difficulty of suturing and the risk of recurrence of CDH. [ABSTRACT FROM AUTHOR]

Congenital pulmonary airway malformation is a rare congenital lung lesion. This report documents the successful application of thoracoscopic segmentectomy in a 1‐year‐old boy prenatally diagnosed with congenital pulmonary airway malformation. Preoperative imaging studies revealed a microcystic lesion with a region of consolidation in the middle area of the right lung. Elective thoracoscopic resection was performed when the patient was aged 1 year and 4 months. Intraoperatively, the superior segment of the right lower lobe was partially separated from the normal lower lobe by a superior accessory fissure. A solid mass, fused with this segment, shared the visceral pleura. Thoracoscopic segmentectomy was successfully performed, and the patient had an uneventful postoperative course. Histological examination revealed congenital pulmonary airway malformation stocker type 2. Thoracoscopic segmentectomy is a viable surgical option that preserves the normal lung parenchyma in pediatric patients with congenital pulmonary airway malformation. [ABSTRACT FROM AUTHOR]

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. Haller index and concave rate were used as objective indices of the deformity. The questionnaires were used to evaluate satisfaction with the chest appearance by a linear scale including five markers (1: dissatisfaction, 5: satisfaction). The patients were divided into two groups: the low satisfaction (score = 1, 2) and the high satisfaction (score = 3–5). Results: The median age during the Nuss procedure was 7.6 (interquartile range, 5.8–12.8) years. Out of 133, 65 patients replied, and the mean postoperative satisfaction score was 3.8 ± 0.2. Out of the 65 respondents, 16 patients (24.6%) were classified as low satisfaction group. Haller index and concave rate were significantly higher and the previous instances of chest operation history were more frequent in the low satisfaction group than in the high satisfaction group, although there was no significant intergroup difference in terms of the postoperative concave rate. Conclusions: Severe deformity and previous chest operation history were considered to be factors for low satisfaction. [ABSTRACT FROM AUTHOR]

Purpose: Mac-2 binding protein glycosylation-modified isomer (M2BPGi) is a new marker for hepatic fibrosis progression. We examined the relationship between serum M2BPGi levels and liver histological findings in intestinal failure (IF) patients without IF-associated liver disease (IFALD). Methods: This study included IF patients without IFALD followed at our hospital. All patients underwent routine liver biopsies per protocol every 1–2 years. We examined M2BPGi levels and histological findings in relation to aspartate aminotransferase (AST) to platelet ratio index, fibrosis-4 index, and AST/ALT ratio. Liver fibrosis was evaluated based on the METAVIR score. Results: Total 18 liver biopsies out of eight patients were included. The median age was 11.5 years. Mean M2BPGi was 0.44 cutoff index (COI) in patients with F0 fibrosis, 0.78 COI in patients with F1 fibrosis and 1.63 COI in patients with F2 fibrosis. Mean M2BPGi was significantly higher in patients with F2 versus F1 or F0 fibrosis (P < 0.016 and P < 0.028, respectively). M2BPGi levels were more strongly correlated with fibrosis stage than with other conventional fibrosis markers. Conclusion: Serum M2BPGi is a novel marker of liver fibrosis in patients with IF. It is useful for follow-up prior to IFALD. Serum M2BPGi levels can support the interpretation of liver status. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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 CDH neonates over 15 years. In our multicenter retrospective study, 464 cases of left-sided isolated CDH with prenatal diagnoses were divided into two groups: NG tube below the diaphragm (BD; n = 190) or above the diaphragm (AD; n = 274). The primary outcome was the 90-day survival rate, and the secondary outcomes were mechanical ventilation duration, hospitalization duration, and recurrence rate. Results: The BD group had a significantly higher 90-day survival rate (98.4 vs. 89.4%, p < 0.001), shorter mechanical ventilation (11 vs. 19 days, p < 0.001), shorter hospitalization (38 vs. 59 days, p < 0.001), and lower recurrence rate (p = 0.002) than the AD group. A multivariate analysis showed that BD (adjusted odds ratio, 3.68; 95% confidence interval 1.02–13.30) was a favorable prognostic factor for the 90-day survival. Conclusion: The assessment of the NG tube position revealed it to be a reliable prognostic factor of left-sided isolated CDH. Therefore, it should be included as a routine assessment. [ABSTRACT FROM AUTHOR]

Congenital pulmonary airway malformation (CPAM) is a developmental malformation that affects the lung parenchyma, especially terminal respiratory bronchioles. This paper reports a case of an infant diagnosed with CPAM who underwent stapleless thoracoscopic lobectomy using Hem-o-Lok clips®. Computed tomography showed cystic pulmonary lesions in the left lower lobe. Thoracoscopic lobectomy was performed at the age of 1 year and 3 months. During surgery, the hilar vasculature was treated using either Hem-o-Lok® clips or a LigaSure vessel sealing system. The lower lobe bronchus was divided using double Hem-o-Lok® clips proximally. The surgery was successfully completed. The patient's postoperative course was uneventful, and there were no complications. This technique can be easily performed as a thoracoscopic lobectomy with the potential benefits of safe and effective procedures for bronchus closure and vascular sealing in the small working space of pediatric patients. [ABSTRACT FROM AUTHOR]

Extracorporeal photochemotherapy (ECP) is one of the more effective cell therapies for graft‐versus‐host disease (GvHD). ECP is a widely recommended therapeutic approach for the treatment of chronic GvHD, particularly steroid‐refractory GVHD. In recent years, the use of a light emitting diode (LED) in the clinic has attracted considerable interest. In this study, we examined the issue of whether an ultraviolet A1‐light emitting diode (UVA1‐LED) can be used as a light source in ECP. To compare the efficacy of ECP with conventional UVA lamp and a UVA1‐LED, we established an in vitro ECP model. Treatment efficacy was evaluated by measuring the % apoptosis and the inhibition of T‐cell proliferation. To investigate the effect of ECP on the innate immune reaction, THP‐1 cells with a luciferase reporter gene driven by a NF‐kB response element (THP‐1 luc NF‐kB) were treated with ECP. The LED‐ECP induced apoptosis and inhibition of T‐cell proliferation as efficiently as a conventional ECP. However, LED‐ECP induced less innate immunity in THP‐1. Since LED devices are more compact compared with conventional UVA irradiation devices, the use of a UVA1‐LED in the treatment of ECP may be a better alternative to conventional ECP therapy. [ABSTRACT FROM AUTHOR]

Background: Hydrocele of the canal of Nuck (HCN) is a rare cause of inguinal swelling in women. The optimal surgical procedure for HCN in children remains unclear. To assess the safety and efficacy of laparoscopic percutaneous extraperitoneal closure (LPEC) for HCN in a pediatric population, a retrospective study was conducted. In addition, to clarify the pathogenesis of HCN, we assessed the morphological findings of the internal inguinal ring (IIR). Materials and Methods: We retrospectively analyzed 10 consecutive female patients with HCN who underwent LPEC at our hospital between January 2010 and May 2020. Age, operative time, and complications were recorded. Concerning the findings of the IIR, we classified the morphological features as follows: Type 1 (flat), Type 2 (narrow patent processus vaginalis [PPV] with a peritoneal veil), and Type 3 (widely opening PPV). Results: The median age of patients who underwent LPEC was 3 (1-12) years. Although 2 patients showed contralateral inguinal hernia (IH), there were no cases of ipsilateral IH. All patients showed ipsilateral PPV, and the morphological features of the IIR were mostly classified as Type 3 (70%). In total, 6 of 8 HCN cases without preoperatively diagnosed contralateral IH had contralateral PPV (75%), and all were closed by LPEC. All operations were accomplished laparoscopically, and the postoperative course was uncomplicated, with no recurrences observed during the study period. Conclusions: LPEC is a safe and simple surgical approach to repair the HCN in children with minimal complications. [ABSTRACT FROM AUTHOR]

Purpose: Short bowel syndrome (SBS) with intestinal failure (SBS-IF) requires long-term parenteral nutrition (PN). This study investigated the real-world etiologies of SBS, treatment patterns, and PN-related outcomes among adult patients with SBS-IF in Japan. Methods: This retrospective, observational cohort study was based on data from April, 2008 to January, 2020 from one of the largest hospital-based claim databases in Japan. Analyzed patients were aged ≥ 16 years, had received continuous PN for ≥ 6 months, and had SBS or undergone SBS-related surgery with a diagnosis of a causative disease. The primary endpoint was PN weaning. Results: We analyzed data for 393 patients. The most frequent causes of SBS-IF were ileus (31.8%), Crohn's disease (20.1%), and mesenteric ischemia (16.0%). Of 144/393 (36.6%) patients who were weaned off their PN, 48 (33.3%) were subsequently restarted on PN. Of 276/393 (70.2%) patients whose PN was initiated in hospital, 156 (56.5%) transitioned to home management. The mean duration of initial PN was 450.4 and 675.5 days for patients who were able or unable to be weaned off PN, respectively. Sepsis (67.4%), catheter-related bloodstream infections (49.1%), and liver disorders (45.0%) were the most reported PN-related complications. Conclusions: Most patients with SBS-IF in Japan could not be weaned off PN and suffered life-threatening complications. [ABSTRACT FROM AUTHOR]

Background The aim of this study was to clarify how extracorporeal membrane oxygenation ( ECMO) is used to treat congenital diaphragmatic hernia ( CDH) in Japan. Methods We completed a nationwide survey of CDH involving 614 infants. The subjects included 43 patients who underwent ECMO. We compared the clinical data of the patients who did and did not survive ≥90 days, and analyzed the 24 h blood gas data in isolated CDH cases in both groups. Results Of the 43 CDH patients, non-isolated CDH associated with other life-threatening or chromosomal anomalies was diagnosed in six patients. Only one of these six patients was able to discontinue ECMO and survived, and the other five died shortly after birth. The other 37 patients all had isolated CDH. The reason for initiating ECMO in 31 of these patients was persistent pulmonary hypertension of the newborn ( PPHN). In the 37 patients with isolated CDH, ECMO was initiated within 24 h after birth. Sixteen patients (37%) survived ≥90 days, and intact discharge was possible in eight cases. Among the isolated CDH patients, on ROC analysis of the lowest oxygenation index ( OI) to predict 90 day survival, the cut-off was 15. Conclusions ECMO is used to treat PPHN starting from an early period after birth, but the mortality and morbidity are not favorable. For lowest OI, the index used to predict survival following ECMO, the cut-off was 15. [ABSTRACT FROM AUTHOR]

• Everolimus could be used safely and effectively after intestinal transplant. • Early use of everolimus after intestinal transplant did not affect wound healing. • Renal function after intestinal transplant was well maintained with everolimus. In patients with intestinal transplantation (ITx), renal function is easily impaired because of long-term parenteral nutrition and side effects of tacrolimus. Everolimus was used in patients with renal insufficiency in our study. We administered everolimus as a third immunosuppressive agent in addition to tacrolimus and steroids for renal sparing in patients who received ITx. We assessed everolimus levels, complications, and renal function. Two patients received everolimus after ITx. Patient 1 was a 13-year-old boy who underwent ITx for an allied disorder of Hirschsprung's disease. After induction therapy with rabbit antithymocyte globulin, maintenance therapy consisted of tacrolimus and steroids. Everolimus was introduced 3 months after ITx for renal sparing. Seven months later, the patient required partial intestinal graft resection owing to bowel obstruction. Everolimus was suspended for only 2 weeks. Four years after ITx, the trough level of tacrolimus was maintained at 3 to 5 ng/mL. The trough level of everolimus was maintained at 3 to 5 ng/mL. Patient 2 was a 32-year-old man who underwent deceased ITx for short gut syndrome. Induction and maintenance immunosuppression was the same as for patient 1. Everolimus was introduced 1 month after surgery. Two years after ITx, trough levels of tacrolimus and everolimus were the same as in patient 1. No rejection was observed in either patient, and renal function was well maintained. We observed no side effects caused by everolimus. Everolimus could be used safely and effectively after ITx. Early use of everolimus after ITx did not affect wound healing. [ABSTRACT FROM AUTHOR]

• Here we present a rare case report of a successful case of hepatoblastoma with pulmonary metastases in which recurrences were treated with repeated lung resection, chemotherapy, and living donor liver transplantation. • Liver transplantation following resection of pulmonary metastases was a feasible option. • Resection of recurrent pulmonary lesions after liver transplantation was a feasible option when lesions were located only in the lung. 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, chemotherapy, and LTx. This study strictly complied with the Helsinki Congress and the Istanbul Declaration regarding donor source. Our case was a 1-year-old boy who developed pre-treatment extent of disease (PRETEXT) Ⅲ HB with multiple pulmonary metastases. The liver tumor was unresectable because it involved all hepatic veins. After 3 cycles of chemotherapy (cisplatin/carboplatin plus doxorubicin), the remaining 2 pulmonary metastases were resected and living donor liver transplantation (LDLT) was performed. Five months after LDLT, a tumor recurrence was detected in the right lung. Repeat lung resection was performed followed by 1 cycle of chemotherapy (carboplatin plus doxorubicin). There has been no recurrence for 18 months since the last lung resection. Previous reports revealed that 14 patients, including the present case, underwent LTx after resection of metastatic HB pulmonary lesions. Of these patients, the 2-year survival rate after LTx was 91%. Recurrence was reported in 5 patients, 2 of whom were successfully treated with repeated resection of the metastatic lesions. LTx after resection of lung recurrence may be a potential treatment for unresectable HB with pulmonary metastases. [ABSTRACT FROM AUTHOR]

• Estimated graft volume in infants varies widely. • Grafts were functioning well with the exception of one graft loss due to antibody-mediated rejection. • Monosegment grafting can be useful for infants as well as newborns. Left lateral segment grafts are generally used for very young pediatric patients undergoing living donor liver transplantation (LDLT). Recently, graft reduction techniques were developed for LDLT. Monosegment grafting has been used in newborns. The aim of this study was to determine the usefulness of monosegment grafting for infants. Recipients <2 years of age who underwent LDLT with a monosegment graft between 2010 and 2020 were gathered. Parents comprised all LDLT donors. A segment 2 monosegment graft was resected as a graft from the donor. Standard liver volume (SLV) was estimated using Urata's equation. Graft type, graft weight (GW), and native liver weight were assessed. Eight patients were included in the study. Original diseases consisted of biliary atresia (n = 6) and fulminant hepatitis (n = 2). Final graft type included monosegment (n = 5) and reduced monosegment (n = 3). Median final GW/body weight after reduction was 3% (range, 2%-3.4%). Median native liver weight/SLV was 134% except in patients with fulminant hepatitis. Median pre-reduction graft volume (GV)/estimated GV was 113% (range, 60%-208%). Median pre-reduction GV/SLV of monosegment grafts that required reduction (n = 3) was 109% (range, 106%-121%). Median final reduced graft GV/SLV was 80% (range, 74%-91%). Complications due to large-for-size grafts were not observed. One case of bile leakage due to graft reduction occurred as a complication. Grafts were functioning well with the exception of one graft loss due to antibody-mediated rejection. Estimated GV in infants varies widely. Monosegment grafting can be useful for infants as well as newborns. [ABSTRACT FROM AUTHOR]

• Tacrolimus-induced encephalopathy was observed in 5% of patients after pediatric living donor liver transplantation. • White blood cell count elevation was observed in all patients at the time of onset. • Patients can resume tacrolimus safely without further neurologic symptoms. Tacrolimus-related neurotoxicity is a serious complication. Posterior reversible encephalopathy syndrome, which is severe neurotoxicity after pediatric living donor liver transplantation (LDLT), is a medication-induced complication related to calcineurin inhibitors. The purpose of this study was to evaluate the long-term outcome of tacrolimus-related neurotoxicity after pediatric LDLT. Pediatric patients who underwent LDLT between 2007 and 2020 at our institution and developed neurologic symptoms with tacrolimus were included in the study. Tacrolimus-related encephalopathy was defined as encephalopathy that resolved after tacrolimus was discontinued. All patients received tacrolimus and a steroid for immunosuppression starting just after LDLT. During the study period, 128 patients underwent LDLT. All patients received tacrolimus and a steroid. Six patients (5%) developed tacrolimus-related encephalopathy. The median age at transplant was 1.6 years. The original diseases were biliary atresia (n = 5) and progressive familial intrahepatic cholangiopathy type 2 (n = 1). Patients developed encephalopathy at a median of 9 days after LDLT. All patients recovered with conversion to cyclosporine. Posterior reversible encephalopathy syndrome was confirmed by magnetic resonance imaging in 3 patients. The mean tacrolimus level at encephalopathy was 11 ng/dL (range, 5.6-14.6 ng/dL). White blood cell count elevation was observed in all patients. One patient died of pancreatitis. Surviving patients (n = 5) were followed for a median of 9 years. All patients resumed tacrolimus a median of 8 months from onset. No neurologic complications were observed after resuming tacrolimus. We observed tacrolimus-induced encephalopathy in 5% of patients after pediatric LDLT. Patients can resume tacrolimus safely without further neurologic symptoms. [ABSTRACT FROM AUTHOR]

• It is rare for hepatopulmonary syndrome (HPS) to occur without cirrhosis/liver disease or portosystemic shunting. • It is also rare for HPS to occur in liver transplant recipients. • HPS should be considered in a patient with hypoxemia with hepatic or biliary abnormalities who does not have cardiopulmonary disease even if there are no apparent causes of HPS. Hepatopulmonary syndrome (HPS) is a disease of gas exchange caused by intrapulmonary shunting secondary to liver disease-associated intrapulmonary vascular dilation. HPS is characterized by the triad of cirrhosis, chronic liver disease, or portosystemic shunting (PSS); arterial hypoxemia; and intrapulmonary arteriovenous shunting in the absence of a primary cardiopulmonary anomaly. We encountered a rare case of HPS without liver disease or PSS. The patient was an 8-year-old girl who underwent living donor liver transplantation (LDLT) shortly after developing fulminant hepatitis at 11 months of her age. Eight years after LDLT, hypoxemia and shortness of breath developed. The shunt ratio on 99mTc-macroaggregated albumin (MAA) lung perfusion scintigraphy (99mTc-MAA lung scan) was 32%. The patient had no cardiopulmonary disease, so we diagnosed her illness as HPS. We did not find cirrhosis, chronic liver disease, or PSS as a cause of HPS. We thought the graft was the cause of HPS. A second transplantation was planned. One year after the diagnosis of HPS, the shunt ratio on 99mTc-MAA lung scan worsened to 42%, digital clubbing appeared, and hypoxemia was worsening. Thus, we performed a second LDLT. After LDLT the shunt ratio on 99mTc-MAA lung scan normalized (6%) and cyanosis resolved. We determined that the graft was the cause of HPS; the typical causes of HPS were not clearly revealed in the histologic examination of the second liver explant. Acute rejection occurred twice after LDLT, so we speculated that HPS occurred because the graft became stressed over the long term. [ABSTRACT FROM AUTHOR]

Background: MicroRNAs (miRNAs) play an important role in regulating fibrogenesis in the liver. The current study examined the ability of microRNA-214 (miR-214) level in liver and serum samples obtained from patients with BA to predict progressive liver fibrosis in patients with biliary atresia (BA). Methods: We examined miR-214 level in relation to conventional markers of liver fibrosis, with liver and serum samples from BA patients. Fifty-two patients with BA who underwent Kasai portoenterostomy and four control patients underwent liver biopsy. In 28 patients with BA, blood samples were collected to analyze circulating serum miR-214. Results: MiR-214 levels in liver tissue were significantly upregulated in patients with BA who had severe liver fibrosis (F3–4) compared to those with none to mild fibrosis (F0–2), whereas suppressors-of-fused homolog (Sufu) mRNA levels were significantly suppressed in F3–4. Serum miR-214 levels were significantly higher in patients with F3–4 compared with F0–2. Area under the curve analysis showed that the serum miR-214 cut-off level for predicting F3–4 was 0.805 (p = 0.0046). Conclusion: Hepatic overexpression of miR-214 is associated with progression of liver fibrosis in patients with BA, and the circulating miR-214 level may serve as a non-invasive predictor of liver fibrosis. [ABSTRACT FROM AUTHOR]

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 diagnosed with isolated unilateral CDH (born 2006–2020). Patients who required ECMO due to respiratory insufficiency were included in this study. Patients who underwent ECMO due to transient disorders were excluded from analysis. Blood gas analysis data within 24 h of birth were compared between survivors and non-survivors. Predictive abilities were assessed for factors with significant differences. Results: Overall, 34 patients were included (18 survivors and 16 non-survivors). The best pre-ductal PaO2 was significantly lower in non-survivors than in survivors (50.4 [IQR 30.3–64.5] vs. 67.5 [IQR 52.4–103.2] mmHg, respectively; p = 0.047). A cutoff PaO2 of 42.9 mmHg had a sensitivity, specificity, and positive predictive value of 50.0%, 94.4%, and 88.9%, respectively, to predict mortality. Conclusion: The best PaO2 within 24 h after birth predicted mortality following ECMO initiation. This should be shared to families and caregivers to optimize the best interests of the infants with CDH. [ABSTRACT FROM AUTHOR]

Purpose: In 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 Osaka prefecture. We assessed the efficacy of the 18MS in screening unfavorable cases. Methods: Public health centers in Osaka prefecture, excluding the Osaka city area, provided test kits to the guardians of infants who received a check-up at 18 months of age between 2004 and 2017. For patients whose standardized urinary levels of vanillylmandelic acid or homovanillic acid exceeded the threshold, they were further examined and treated in two specific hospitals Osaka University Hospital and Osaka Women's and Children's Hospital. Screening-positive patients with and without neuroblastoma were retrospectively reviewed. Results: Among 142,423 children screened during the 18MS, 85 tested positive, and 14 were diagnosed with neuroblastoma. Twelve patients were classified as very low risk, while 2 were classified as high risk, based on the International Neuroblastoma Risk Group risk classification. Conclusion: The 18MS did not screen unfavorable cases with neuroblastoma efficiently, although few participants benefited from it. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

In Japan, mortality due to intestinal perforation has been increasing for the last 15 years, as the survival rate of extremely-low-birth-weight neonates (ELBWs) has been increasing. In our NICU, although the incidence of necrotizing enterocolitis has been decreasing, that of focal intestinal perforation (FIP) has been increasing. To elucidate the pathogenesis of FIP, a histological study was performed. For the last 20 years, in our NICU, 11 ELBWs with FIP, including one with multiple perforations and impending rupture, underwent laparotomy and enterostomy. The intestinal wall around the perforation was resected for histological study. Histological studies revealed well-developed ganglion cells in all the cases. Although some lymphocytic infiltration and hemorrhage were observed, no apparent neutrophilic infiltration, or necrotic change was identified in any case. No evidence of microcirculatory impairment was identified. The intestinal musculature discontinued abruptly with thinning in three cases, and without thinning in the others. The histology of impending rupture showed an absence of the muscularis with preservation of the remaining components of the bowel wall. The findings that the punched-out perforations had the appearance of Hirschsprung's disease, FIP exclusively occurred in ELBWs, and that neither mechanical obstruction nor necrotic change could be identified as the cause of the perforation suggest that immature bowel movement and congenital muscle defects may be involved in the pathogenesis of FIP. [ABSTRACT FROM AUTHOR]

Purpose: It 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 the medical records of pediatric cancer patients who underwent CT-guided marking procedures in our institutions between Jan 2011 and Apr 2020. After 2015, these procedures were combined with an indocyanine green (ICG) navigation-guided surgery for hepatoblastoma cases. Results: We targeted a total of 22 nodules in 12 patients. Of these, marking was successful in 18 (81.8%) nodules, 10 of which contained viable malignant cells. Complications caused by the marking procedures included mild pneumothorax and mild atelectasis in two patients, respectively. Of the eight resected nodules in patients with hepatoblastoma, four were ICG-positive and contained viable malignant cells. Two additional ICG-positive nodules, which were unidentified before surgery, were observed intraoperatively. Conclusion: CT-guided marking is a feasible and safe method that can be used to identify and resect tiny pulmonary lesions in pediatric cancer patients. An ICG navigation-guided surgery is useful when combined with CT-guided marking, particularly in hepatoblastoma cases. [ABSTRACT FROM AUTHOR]

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 neuroblastoma by pediatric surgical trainees. Subjects and Methods: In this multicenter, retrospective study, including 18 children with abdominal neuroblastoma indicated for 19 laparoscopic resections (1 with bilateral neuroblastomas) from 1999 to 2018, the clinical data were retrospectively reviewed and compared between trainee and attending surgeons. Results: None of the cases had image-defined risk factors (IDRFs) at surgery. All patients successfully underwent complete laparoscopic resection without blood transfusion, open conversion, or intraoperative or postoperative complications. Of the 19 cases, 6 were performed by pediatric surgical trainees under the guidance of attending surgeons, and 13 were performed by attending surgeons. With comparable tumor, largest diameter, operative time, and bleeding amount were not significantly different between the two groups. In the trainee surgeon group, one local recurrence occurred at a secondary resection site in the bilateral neuroblastoma patient with Stage L2. Conclusions: Laparoscopic resection of neuroblastoma could be safe and feasible when limited to absent IDRFs at surgery by pediatric surgical trainees under the guidance of experienced attending surgeons, as well as by attending surgeons. [ABSTRACT FROM AUTHOR]

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 diagnosis and postnatal care has led to an increase in the survival rate of patients with CDH. However, some survivors experience mid‐ and long‐term disabilities and complications requiring treatment and follow‐up. In recent years, the establishment of clinical practice guidelines has been promoted in various medical fields to offer optimal medical care, with the goal of improvement of the disease' outcomes, thereby reducing medical costs, etc. Thus, to provide adequate medical care through standardization of treatment and elimination of disparities in clinical management, and to improve the survival rate and mid‐ and long‐term prognosis of patients with CDH, we present here the clinical practice guidelines for postnatal management of CDH. These are based on the principles of evidence‐based medicine using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. The recommendations are based on evidence and were determined after considering the balance among benefits and harm, patient and society preferences, and medical resources available for postnatal CDH treatment. [ABSTRACT FROM AUTHOR]