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Inguinal hernia repair is one of the most common operations performed worldwide. No consensus currently exists regarding the most appropriate operation for inguinal hernia in adolescent and young adult (AYA) patients. This study aimed to evaluate the outcomes in AYA patients undergoing high ligation or mesh repair under laparoscopy by examining the location and size of the hernia orifice defect.We retrospectively reviewed all patients aged 15 to 40 years old who underwent laparoscopic hernia repair. Under single port laparoscopy, we classified the anatomic location (lateral, medial, or femoral) and size of the hernia orifice according to the classification by the European Hernia Society (EHS). A laparoscopic percutaneous extraperitoneal closure (LPEC) was performed on the patients with a lateral hernia with a hernia orifice defect size of ≤1.5cm (L1). Transabdominal preperitoneal (TAPP) repair was performed on the patients with a lateral hernia with a hernia orifice defect size of1.5 cm (L2 or 3).Overall, 40 patients underwent the mentioned surgical procedures. We performed LPEC on 22 patients, and TAPP on 18 patients. There were no intraoperative or postoperative complications and recurrences.This is the first report that evaluated the outcomes of AYA patients who underwent high ligation or mesh repair under laparoscopy by examining the location and size of the hernia orifice defect. And our data indicated that LPEC were effective and safe for AYA patients with small hernia orifice defect.

To examine pathophysiology of parameatal urethral cyst (PUC) with comparison between the surgical excision group and the spontaneous resolution group.We retrospectively reviewed all patients diagnosed with PUC and aged ≤15 years. Initially, all the patients received observation without any treatment. The indication of surgical excision was family preference. The patients were analyzed according to their sex, age, symptoms, clinical course, and pathological examination findings.Among the 54 boys visited our hospital for PUC. The median age at presentation was 35 (IQR: 12-50) months, including 7 (13.0%) neonates cases. In total, 38 (70.4%) patients were underwent surgical excision, 12 (22.2%) had spontaneous resolution during observation, 1 (1.9%) had observation without resolution, and 3 (5.6%) were lost to follow-up. The median size of PUC at the first visit was significantly larger in the surgical excision group (5 mm median, IQR 3-5) than in the spontaneous resolution group (1.5 mm median, IQR 1-2.5) (P.0001). In this study, PUC ≥5 mm in diameter did not have spontaneous resolution.This is the largest study of PUC conducted at a single institute. This study indicated that 22% of PUCs had spontaneous resolution, and PUCs5 mm had potential for spontaneous resolution. The results recommend an observation of at least 5.5 months for patients with asymptomatic PUC5 mm. By contrast, we recommend the surgical excision for symptomatic PUC ≥5 mm according to the family preference.

Purpose In recent years, many studies have made considerable progress in the development of stem cell-based therapies for Hirschsprung’s disease (HD). However, the question of whether enteric neural crest-derived cells (ENCCs) that are transplanted into aganglionic gut can migrate, proliferate and differentiate in a normal manner remains unanswered. Thus, we designed this study to compare the behavior of ENCCs transplanted into the aganglionic gut of endothelin receptor B knockout (Ednrb-KO) mice versus wild-type (WT) mice. Methods ENCCs were isolated from the fetal guts of Sox10 transgenic mice, in which ENCCs were labeled with an enhanced green fluorescent protein, Venus, on embryonic day 18.5 (E18.5). Neurospheres were generated and transplanted into the aganglionic region of either Ednrb-KO mice gut, or WT mice gut that had not yet been colonized, on E12.5. Time-lapse imaging of the transplanted ENCCs was performed after 24h, 48h and 72h of culture. Neuronal differentiation was evaluated using whole-mount immunohistochemistry. Results Sox10-positive ENCCs were seen to successfully migrate into the myenteric region of the aganglionic gut following transplantation in both the Ednrb-KO and WT mice. The ratio of Tuj1-positive/Sox10-positive cells was significantly increased after 72h of culture compared to 24h in the Ednrb-KO mice, which suggests that the transplanted ENCCs differentiated over time. In addition, at the 72h timepoint, neuronal differentiation of transplanted ENCC in the aganglionic gut of Ednrb-KO mice was significantly increased compared to that of WT mice. Conclusions The results of our study demonstrated that transplanted ENCCs migrated into the myenteric region of aganglionic recipient gut in mice. The increased neuronal differentiation of transplanted ENCC in Endrb-KO mice gut suggests that the microenvironment of this region affects ENCC behavior following transplantation. Further research to explore the characteristics of this microenvironment will improve the potential of developing cell therapy to treat HD patients.

Screening for undescended testis (UDT) in Japan is performed as a neonate, then at 1, 3, 10, and 18 months old, and 3 years old. Incidence of ascending testis (AT) after screening was reviewed.All orchiopexy/orchiectomy at a single institute between July 2005 and June 2022 were reviewed retrospectively.376 boys had 422 procedures; 54/422 (12.8%) were in 48 boys ≥ 4 years old (mean age: 6.7 years; range: 4-13); testes were normal (n = 22; 40.7%), small (n = 25; 46.2%), or atrophied (n = 7; 1.3%). There were 47 orchiopexies and 7 orchiectomies for atrophy. Incidence of AT in boys ≥ 4 years old was 24/422 (5.7%). Of these, 16/422 (3.8%) developed after normal descent and 8/422 (1.9%) were associated with retractile testis (AT + RET). Other indications included delayed treatment for UDT (n = 13), late referral by pediatricians (n = 10), and iatrogenic UDT (n = 6). Surgical intervention in boys ≥ 4 years old (12.8%) was less than that reported in the West (range: 30-50%) as was AT: (5.7% versus 15.4%) and AT + RET (1.9% versus 13.8%).Comprehensive UDT screening probably contributed to the lower incidence of surgery and AT (especially AT + RET) in boys ≥ 4 years old.

Introduction: A modified pull-through (PT) distinguished by complete full-thickness removal of the posterior rectal cuff, initially developed as an open procedure in 1980, has been performed with l...

Interactions between enteric neural crest-derived cells (ENCC) and the surrounding intestinal microenvironment, such as the extracellular matrix (ECM), are critical for regulating enteric nervous system (ENS) development. Integrins are the major receptors for ECM molecules, such as laminin, which have been reported to be involved in the pathogenesis of Hirschsprung’s disease. In this study, we examined the expression of β1 integrin in the endothelin receptor B (Ednrb) knock out (KO) mouse gut, which presents with an aganglionic colon. A Sox10-Venus-positive Ednrb KO mouse, where ENCC is labeled with fluorescent protein, ‘Venus’, was created. Sox10-Venus-positive Ednrb wild type (WT) were used as controls. Small intestine, proximal colon and distal colon were dissected on E13.5 and E15.5 and β1 integrin expression of the gut tissue was examined by immunohistochemistry and real time RT-PCR. The cells of the gut dissected on E11.5 were isolated and cultured for 2 days. Venus-positive ENCC were immunostained with β1 integrin and Tuj-1, which is a marker for neurons. The expression of β1 integrin was not significantly different between KO and WT in all parts of the gut examined. However, the β1 integrin expression in the isolated ENCC was significantly decreased in KO compared to WT. The average threshold area was 42.98 ± 17.47% in KO and 73.53 ± 13.77 in WT (p

The incidence of perforation during antibiotic therapy (AT) of children triaged as non-complicated acute appendicitis (NC-Ap) was investigated. Abdominal ultrasonography (US) and/or computed tomography (CT) scans from cases of perforation identified at appendectomy for failed AT were reassessed blindly by a panel of board-certified specialists for any evidence of pre-AT morbidity suggestive of perforation. Of 521 cases triaged as NC-Ap, symptoms resolved with AT in 452 cases (86.8%). All 69/521 (13.2%) cases with persistent symptoms had urgent appendectomy, and 12/521 (2.3%) were found to have perforated. Blind reassessment of US and/or CT scans from these cases identified seven with evidence of perforation when they were triaged as NC-Ap. Thus, the actual incidence of perforation during AT for NC-Ap was actually 12–7 = 5/521 (0.95%). Perforation is generally believed to be a complication of AT, but inappropriate triaging of cases for AT can bias results by artificially inflating the number of perforations, in this study, by more than double. We are the first to assess the unbiased incidence of perforation during AT for NC-Ap, by reassessing pre-AT US and/or CT scans. The incidence of perforation during AT is actually negligible.

Purpose To report the sequelae of and preventive strategies for selected lower urinary tract (LUT) complications, i.e., posterior urethral diverticulum (PUD), intraoperative LUT injuries, postoperative dysuria, and fistula recurrence in male imperforate anus (IA) with rectourethral/rectovesical (RU/RV) fistula after laparoscopy-assisted anorectoplasty (LAARP) or posterior sagittal anorectoplasty (PSARP). Methods 153 boys with IA and RU/RV fistula treated 1986–2019 by LAARP (n = 56) or PSARP (n = 97) at two unrelated institutes were studied retrospectively. Results After mean follow-up of 17.0 years (range: 36.5 days-32.0 years), the overall incidences of LUT complications were: LAARP (6/56; 10.7%); PSARP (7/97; 7.2%); p = 0.55, comprising PUD: LAARP (n = 5), PSARP (n = 0); p = 0.006; injuries: LAARP (n = 0), PSARP (n = 5); p = 0.16; dysuria: LAARP (n = 1), PSARP (n = 1); p>0.999; and recurrence: LAARP (n = 0), PSARP (n = 1); p>0.999. Mean onset of PUD was 5.1 years (range: 1.0–15.1 years). Treatment: PUD: surgery (n = 2/5), conservative (n = 3/5); injuries: intraoperative repair (n = 5/5); dysuria: conservative (n = 2/2), and recurrence: redo PSARP (n = 1/1). Conclusions Strategies devised to improve dissection accuracy resolved the specific technical issues causing LUT complications (remnant RU fistula dissection in LAARP and blind posterior access in PSARP). Currently, the incidence of new cases of PUD and LUT injuries is zero. Level of Evidence: Level III

Dysgerminoma is a malignant ovarian germ cell tumor, and unlike sex-cord stromal tumors, endocrine manifestation is considered rare. Here, we report the first case of dysgerminoma presenting precocious puberty. The patient is a 7-year-old girl who presented with a breast development in Tanner stage 3. Serum estradiol (E2) was markedly elevated while luteinizing hormone (LH) and follicle-stimulating hormone (FSH) were suppressed below the detection limit. Microscopically, the right ovarian mass displayed nests of large polygonal cells and fibrous septa which were focally concentrated by theca-like plump spindle cells. Immunohistochemistry revealed that the spindle cells expressed various steroidogenic enzymes involved in estrogen biosynthesis including P450 aromatase. The tumor was diagnosed with pure dysgerminoma with estrogen-producing functioning stroma. After the operation, serum E2 declined below the detection limit; LH and FSH returned within the normal range. This case demonstrates that even a conventional dysgerminoma can present endocrine manifestation through functioning stroma.

Aim: We reviewed intraoperative video recordings (IVRs) of laparoscopic percutaneous extraperitoneal closure (LPEC) for inguinal hernia in children blindly to assess performance. Methods: IVRs of 1...

Objective Three criteria (age at first pale stool, age at portoenterostomy, and duration from the first pale stool to portoenterostomy) were assessed for prognostic value in biliary atresia. Methods The medical records of 116 consecutive biliary atresia patients treated by portoenterostomy after liver transplantation became available in Japan in 1989 were identified and data from 96 were analyzed retrospectively for this study. The impact of each criterion on clearance of jaundice to normal levels (total serum bilirubin ≤1.2 mg/dL) and survival with the native liver as indicators of outcome were compared according to time (≤30 days, 31−60 days, and ≥61 days). Results Age at first pale stool was ≤30 days in 53, 31−60 days in 26, ≥61 days in 17; age at portoenterostomy was ≤30 days in 7, 31−60 days in 35, ≥61 days in 54, and duration pre-portoenterostomy was ≤30 days in 50, 31−60 days in 36, ≥61 days in 10. Survival with the native liver was not significantly influenced by age at first pale stool or age at portoenterostomy, but prolonged duration (≥61 days) reduced survival with the native liver significantly (p = 0.003). Clearance of jaundice to normal levels was not affected by any criterion at any time.

Detailed implications of age at laparoscopic transanal pull-through (LTAPT) on postoperative bowel function (POBF) in Hirschsprung’s disease (HD) are somewhat obscure because of a spectrum of factors. Age at surgery was used to categorize 106 consecutive postoperative HD cases treated by our modified LTAPT (JLTPAT) between 1997 and 2015; group A

Aim The purpose of this study was to determine the incidence of ureteric obstruction (UB) and requirement for Cohen antireflux surgery (CAS) after Deflux Ⓡ treatment (DT) for vesicoureteric reflux (VUR). Methods Between 2011 and 2017, 494 ureters (VUR severity ≤ grade III: N=291 or >grade IV: N=203) were treated by DT at a mean age of 4.5 (range: 0.2–24) years. Epidural Catheter Assistance (ECA) was used to exclude UB by injecting diluted indigo carmine solution (1–3mL) into an epidural catheter inserted into a ureter after DT and confirming dye flow within 15min. ECA+: N=181 ureters; ECA−: N=313 ureters. Results In ECA+, UB was detected in 5/181 (2.7%) ureters (grade II: N=1, III: N=3, and IV: N=1) treated by leaving the ECA catheter in situ overnight (N=4) or double J stent (DJS) insertion for 1month (N=2). After mean follow-up of 1.9years, one grade III DJS case has residual grade II VUR. In ECA−, 3/313 (0.9%) cases developed UB. One resolved, and one required DJS. CAS was required for 17/494 (3.4%) ureters and hindered by DT in 5/17 (29.4%) ureters. All are sequelae-free after mean follow-up of 1.8years. Conclusions UB may be more frequent than reported (3.3% versus 0.6%–1.8%). ECA identifies potential UB. Type of study Treatment study. Level of evidence Level III.

Aim Early diagnosis is essential for the successful management of biliary atresia (BA). We assessed the efficacy of our diagnostic strategies for BA in the laparoscopic era. Methods A retrospective review of the medical records of 132 infants presenting with suspected BA between 1998 and 2018 was performed to assess the efficacy of “basic” tests (blood biochemistry, abdominal ultrasound, and Tc-99m N-pyrydoxyl-5-methyltriptophane liver scintigraphy) and the value of laparoscopic assessment of the porta hepatis and/or cholangiography for choosing between Kasai portoenterostomy (PE) or cholangiodrainage (CD) for treatment. Results BA was excluded on “basic” tests in 7 cases: 109/125 had BA diagnosed laparoscopically and proceeded immediately to PE. Nonatrophic gallbladder cases (n = 10) had CD and other morbidity diagnosed on liver biopsy (Bx; n = 6). Sensitivity, specificity, and accuracy of diagnosis using “basic” tests were 100%, 30.4%, and 87.9%, respectively. Accuracy of laparoscopy without Bx was 100%, allowing immediate progression to PE without waiting for Bx results or trauma from Bx to heal. “Basic” tests and laparoscopy can be completed within one week. Conclusions A combination of “basic” tests and laparoscopy can diagnose BA promptly and accurately without Bx. Bx is essentially unnecessary for diagnosing BA if laparoscopy is performed. Type of study Study of diagnostic test. Level of evidence Level III.

The behavior of enteric neural crest-derived cells (ENCC) during enteric nervous system (ENS) development is being gradually understood with the introduction of live-cell imaging. However, many of the analyses to date are two-dimensional and the precise multidirectional migration of ENCC has been challenging to interpret. Mice lacking the endothelin-B receptor gene, Ednrb −/− mice, are widely used as a model for Hirschsprung’s disease (HD). We have recently developed a Sox10 transgenic (Tg) mouse to visualize ENCC with enhanced green fluorescent protein (Venus). By breeding these two models, we have created a Venus-positive, Sox10 Tg mouse with a deletion of the Ednrb gene, Sox10-Venus+/Ednrb −/− mouse, to investigate the ENS in HD. The aim of this study was to investigate the behavior of migrating ENCC in the hindgut of the Sox10-Venus+/Ednrb −/− mouse using three-dimensional and four-dimensional image analysis software. To compare the ENCC behavior when the wavefront of ENCC reaches the mid-hindgut between HD mouse and control, we harvested the fetal hindguts of Sox10-Venus+/Ednrb −/− mice on embryonic day 15.5 (E15.5) and Sox10-Venus+/Ednrb +/+ mice on E12.5, which was used as control. Dissected hindguts were cultured for 360 min and the time-lapse images were obtained using a confocal laser-scanning microscope. Each ENCC at the wavefront was tracked after adjusting the longitudinal axis of the gut to the Y axis and analyzed using Imaris software. Track displacement (TD)-Y indicates ENCC advancement in a rostral-caudal direction. TD-X and TD-Z indicate ENCC advancement perpendicular to the rostral–caudal axis. Mean TD-Y was 34.56 µm in HD, but 63.48 µm in controls. TD-Y/TD-XZ was not significantly different in both groups. However, the mean track speeds were significantly decreased in HD (72.87 µm/h) compared to controls (248.29 µm/h). Our results showed that the track speed of ENCC advancement was markedly decreased in the HD mice compared to controls. This technique provides added information by tracking ENCC with depth perception, which has potential for further elucidating the altered behavior of ENCC in HD.

Total bilirubin (T-bil) is used universally for monitoring post-portoenterostomy (PE) biliary atresia (BA) patients although other biochemical markers [BM; AST/ALT and platelet count (PC)] are also prognostic. We compared open PE (OPE) with laparoscopic PE (LPE) using T-bil, AST/ALT, and PC (3BM) as more comprehensive indicators of postoperative clinical status.Subjects were 31 PE cases (LPE: n = 17; OPE: n = 14). BA classification was type III (n = 16), type II (n = 1) in LPE and type III (n = 12), type I (n = 1), type II (n = 1) in OPE.Mean ages and weights at PE were similar: 65.5 days, 4.4 kg (LPE) versus 69.3 days, 4.1 kg (OPE); and mean follow-up was 2.5 years for both LPE and OPE. Jaundice clearance (T-bil ≤1.2 mg/dL) was achieved in 16/17 (94.1 %) after LPE versus 10/14 (71.4 %) after OPE (p = NS), but 3BM were closer to normal after OPE. At the time of review, 13/17 LPE cases (76.5 %) were alive with native livers and 4/17 had received LTx (23.5 %) and 10/14 OPE cases (71.4 %) were alive with native livers and 4/14 had received LTx (28.6 %).Although JC was better after LPE, 3BM were better after OPE. Further follow-up will prove the comprehensive prognostic value of 3BM.

Purpose We reviewed our post-Kasai portoenterostomy biliary atresia (BA) patients who required liver transplantation (LTx) for deterioration in native liver (NL) function to investigate mortality in relation to age at LTx. Methods BA patients indicated for LTx when less than 18years old (U18; n=17) and when 18 or older (18+; n=13) were compared. All achieved jaundice clearance postoperatively (TBil ≤1.2mg/dL (≈20μmol/L)). Results In U18, living-donor (LD) LTxs were performed at a median of 6.1years (range: 0.5–16.7; n=14) and cadaveric (CD) LTxs at a median of 1.3years (1.1–1.5; n=3). In 18+, LDLTxs were performed at a median of 28years (18–37; n=8), and 1 case died from graft versus host disease. CDLTxs were indicated in 5, but 4 died at a median of 30years (26–32), a mean of 1.4years (0.7–1.8) after NL deterioration commenced. One case is awaiting CDLTx. At the time of review, all U18 and 7 LDLTx cases in 18+ were clinically stable. Mortality rates were 0% in U18 and 38% in 18+ (P=.006). Conclusion Our results highlight the extremely grave prognosis for long-term BA patients requiring LTx when 18 or older because of poor donor availability in Japan. Level of evidence Level III.

Laminin-1 regulates neurite outgrowth in various neuronal cells. We have previously demonstrated that laminin-1 promotes enteric neural crest-derived cell (ENCC) migration by using Sox10-VENUS transgenic mice, in which ENCCs are labeled with a green fluorescent protein, Venus. Mice lacking the endothelin-B receptor gene, Ednrb −/− mice, are widely used as a model for Hirschsprung’s disease (HD). The aim of this study was to investigate the effects of laminin-1on ENCC migration in Sox10-VENUS+/Ednrb −/− mice, a newly created HD mice model. Fetal guts were dissected on embryonic day 12.5 (E12.5). Specimens were incubated either with, or without laminin-1 for 24 h and images were taken under a stereoscopic microscope. The length from the stomach to the wavefront of ENCC migration (L-E) and the total length of the gut (L-G) were measured. Changes in the ratio of L-E to L-G (L-E/L-G) after 24 h were calculated. On E12.5, the wavefront of ENCC migration in the HD gut samples was located in the midgut, whereas the wavefront of ENCC in Sox10-VENUS+/Ednrb +/+ (WT) samples had reached the hindgut. After 24 h, L-E/L-G had increased by 1.49%, from 34.97 to 36.46%, in HD gut and had increased by 1.07%, from 48.08 to 49.15%, in HD with laminin-1, suggesting there was no positive effect of laminin-1 administration on ENCC migration in HD. Our results suggest that laminin-1 does not have a positive effect on ENCC migration in HD mice on E12.5, in contrast to the phenomenon seen in normal mice gut specimens, where laminin-1 promotes ENCC migration during the same period. This suggests that there is an impairment in the interaction between ENCC and extracellular environmental factors, which are required for normal development of the enteric nervous system, resulting in an aganglionic colon in HD.

Background In 2007, we began using the anorectal line (ARL) as the landmark for commencing rectal mucosal dissection (RMD) instead of the dentate line (DL) during laparoscopy-assisted transanal pull-through (L-TAPT) for Hirschsprung's disease (HD). We conducted a medium-term prospective comparison of postoperative fecal continence (POFC) between DL and ARL cases to follow our short-term study. Methods POFC is assessed by scoring frequency of motions, severity of staining, severity of perianal erosions, anal shape, requirement for medications, sensation of rectal fullness, and ability to distinguish flatus from stool on a scale of 0 to 2 (maximum: 14). Results Patient demographics were similar for ARL (2007–2014: n = 33) and DL (1997–2006: n = 41). There were no intraoperative complications and 2 cases of postoperative colitis in both ARL (6.1%) and DL (4.9%). Mean annual medium-term POFC scores for the 4–7 term of this study were consistently better in ARL: 9.7 ± 1.4*, 10.1 ± 1.6*, 10.6 ± 1.6, and 11.3 ± 1.4* in ARL and 8.6 ± 1.5, 9.1 ± 1.6, 9.8 ± 1.9, 10.0 ± 1.6 in DL (*: p Conclusions Medium-term POFC is better when the ARL is used as the landmark for RMD during L-TAPT for HD.