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Purpose: Hepatocyte mitochondrial morphology and gene expression were compared between biliary atresia (BA), infantile cholestasis (IC), and normal liver (NL) as prognostic indicators., Methods: Specimens of liver at portoenterostomy (PE) for BA, from intrahepatic bile duct paucity patients for IC, and from choledochal cyst or hepatoblastoma patients for NL were collected prospectively (P) beginning in 2021 (P-BA = 11, P-IC = 9, P-NL = 7) and retrospectively (R) from paraffin-embedded tissue going back to 1981 (R-BA = 25, R-IC = 9, R-NL = 4). The P-cohort had transmission electron microscopy (TEM) to image mitochondria, immunoblotting for heat shock protein 60 (HSP60), and quantitative PCR (qPCR) for HSP60 and mitochondrial functional genes. Both cohorts had immunofluorescence for HSP60 quantified as a ratio to albumin-positive hepatocytes (ALB) with HSP60/ALB<1.0 as a cutoff limit using ImageJ., Results: HSP60 was significantly lower in BA/IC than NL on qPCR (BA: p < 0.01, IC: p < 0.05) and lower in BA than IC/NL on immunoblotting (p < 0.05). HSP60/ALB was significantly lower in BA than NL/IC (p < 0.001). Despite BA subjects being matched for types of BA and ages at PE, HSP60/ALB did not correlate with jaundice clearance (JC; T-Bil<1.2 mg/dL) but was significantly higher in native liver survivors (NLS) after PE compared with liver transplant (LTx) cases (p < 0.05) and significantly lower in LTx cases achieving JC than NLS achieving JC (p < 0.05). TEM showed BA had significantly more mitochondrial inclusion bodies (p < 0.05) and significantly larger cristae (p < 0.01) than IC/NL. qPCR in BA showed significant repression of mitochondrial functional genes for mRNA stabilization and energy facilitation., Conclusion: HSP60/ALB correlates with NLS after PE for BA., Level of Evidence: II., Competing Interests: Declaration of competing interest The authors declare that they have no conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: To investigate whether Leukotriene B4 receptor 2 (BLT-2), an upstream regulator of tight junction protein (TJP) Claudin-4, and TJPs could be etiologic factors in Hirschsprung-associated enterocolitis (HAEC) after pull-through (PT) for Hirschsprung disease (HD)., Methods: Normoganglionic colon (HD-N) and aganglionic rectum (HD-A) specimens from rectal/rectosigmoid (R/RS) or descending/transverse (D/T) HD were assessed using quantitative polymerase chain reaction (qPCR) for Occludin, TJP-1, TJP-2, Junctional adhesion molecule (JAM)-1, JAM-2, Claudin-1, Claudin-3, Claudin-4, and BLT-2 and immunoblotting for Claudin-4 using fresh specimens obtained intraoperatively (2021-2024; n = 17; R/RS = 15 and D/T = 2). Claudin-4 immunohistochemistry was also evaluated quantitatively using preserved (n = 29; R/RS = 20 and D/T = 9; 2009-2021) and fresh HD specimens for comparison with anorectal malformation patients having colostomy closure as controls (n = 42) and between HD-A versus HD-N, R/RS versus D/T, and HAEC (+) versus HAEC (-). Technically inadequate or transitional zone PT were excluded., Results: Subjects were 123 PT cases. Mean ages at PT/colostomy closure (years) were R/RS: 2.7 ± 2.9, D/T: 1.6 ± 2.2, and controls: 1.4 ± 0.7. Postoperative HAEC occurred 18 times in 14 PT cases (grade I = 5, grade II = 13). Post-PT HAEC was significantly more frequent in D/T (50.0% versus 6.4%; p < 0.001); Claudin-4 was significantly lower in HD-N from post-PT HAEC cases, especially D/T (p < 0.05) on immunohistochemistry. Claudin-4 was significantly lower in HD-N/HD-A compared with controls on immunoblotting (p < 0.05) and immunohistochemistry (p < 0.001). qPCR showed TJP-1, TJP-2, JAM-1, JAM-2, Claudin-4, and BLT-2 were significantly lower in HD-N/HD-A compared with controls., Conclusions: Lower Claudin-4 and BLT2 in post-PT HAEC HD-N (especially D/T) suggests generalized epithelial barrier derangement with possible etiologic implications for HAEC., Level of Evidence: Ⅱ., Competing Interests: Conflicts of interest The authors declare they have no conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Fetoscopic endoluminal tracheal occlusion (FETO) improves the survival rate in fetuses with severe congenital diaphragmatic hernia (CDH). We hypothesize that prenatal therapies into the trachea during FETO can further improve outcomes. Here, we present an ex vivo microinjection technique with rat lung explants to study prenatal therapy with nanoparticles., Methods: We used microsurgery to isolate lungs from rats on embryonic day 18. We injected chitosan nanoparticles loaded with fluorescein (FITC) into the trachea of the lung explants. We compared the difference in biodistribution of two types of nanoparticles, functionalized IgG-conjugated nanoparticles (IgG-nanoparticles) and bare nanoparticles after 24 h culture with immunofluorescence (IF). We used IF to mark lung epithelial cells with E-cadherin and to investigate an apoptosis (Active-caspase 3) and inflammatory marker (Interleukin, IL-6) and compared its abundance between the two experimental groups and control lung explants., Results: We detected the presence of nanoparticles in the lung explants, and the relative number of nanoparticles to cells was 2.49 fold higher in IgG-nanoparticles than bare nanoparticles (p < 0.001). Active caspase-3 protein abundance was similar in the control, bare nanoparticles (1.20 fold higher), and IgG-nanoparticles (1.34 fold higher) groups (p = 0.34). Similarly, IL-6 protein abundance was not different in the control, bare nanoparticles (1.13 fold higher), and IgG-nanoparticles (1.12 fold higher) groups (p = 0.33)., Conclusions: Functionalized nanoparticles had a higher presence in lung cells and this did not result in more apoptosis or inflammation. Our proof-of-principle study will guide future research with therapies to improve lung development prenatally., Levels of Evidence: N/A TYPE OF STUDY: Animal and laboratory study., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: Mid-shaft/proximal (msp) and penoscrotal/scrotal/perineal (pssp) hypospadias treated by urethroplasty (UP) terminating at the corona (UPC) or UP terminating at the tip of the glans (UPG) were compared., Methods: UP performed at a single institute (n = 234) between 2003 and 2020 were grouped as: msp-UPC (n = 23), msp-UPG (n = 75), pssp-UPC (n = 81), and pssp-UPG (n = 55) to compare data obtained from medical records for post-UP complications (PUC; urethral stenosis, urethrocutaneous fistula, diverticulum formation, and bleeding; n = 234), post-UP uroflowmetry (PUF; average flow rate (Qave), maximum flow rate (Qmax), voiding time (VT), voided volume (VV) and urine flow curves) in 57 UP patients [msp-UPC (n = 5), msp-UPG (n = 12), pssp-UPC (n = 32), pssp-UPG (n = 8)] and 9 controls, and post-UPC esthetics (EST; n = 104). P < 0.05 was considered significant., Results: Mean ages at UP (years) were: msp-UPC (3.1 ± 3.0), msp-UPG (3.3 ± 1.4), pssp-UPC (4.6 ± 2.4), and pssp-UPG (3.4 ± 1.4); p < 0.0001 by ANOVA test. Overall, there were significantly more PUC in pssp-UPG compared with pssp-UPC except for bleeding. Ages at PUF assessment were similar. Mean Qave (mL/s) for pssp-UPG (4.0 ± 1.0) was significantly less than pssp-UPC (5.9 ± 2.5; p < 0.05) and both were significantly less than controls (6.9 ± 1.8; p < 0.05, p < 0.01, respectively). Mean Qmax (mL/s) for pssp-UPC (11.4 ± 4.8) was significantly better than pssp-UPG (7.8 ± 2.3; p < 0.05) and for controls (14.9 ± 4.4) was significantly better than pssp-UPG (7.8 ± 2.3; p < 0.01). All VT (seconds) were similar to controls; all urine flow curves were normal. For EST in UPC (n = 104), 3 cases requested meatal advancement., Conclusions: UPC may be a valid option for treating pssp hypospadias because of significant differences in PUC/PUF and minimal EST issues compared with UPG., Level of Evidence: Prognosis Study Level-Ⅱ., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Aim: Retroperitoneal pyeloplasty (RP) for pediatric ureteropelvic junction obstruction (UPJO) performed using retroperitoneoscopy (retro-RP) or robotic assistance (robo-RP) were compared., Methods: All subjects were Japanese, matched for age, weight, and RP diameters. All RP were performed in the lateral decubitus position at a single institute by the same team using identical protocols. Five independent surgeons were asked to score intraoperative video recordings for perceived difficulty of suturing (DOS; 5 = impossible; 4 = difficult; 3 = tedious; 2 = slow; 1 = easy) and rank RP as +1 if robo-RP appeared to be superior, 0 if they appeared to be the same, and -1 if robo-RP appeared to be inferior., Results: Robo-RP performed 2018-2022 (n = 22) were matched with retro-RP performed 2011-2019 (n = 34). Mean overall operative times were similar (robo-RP: 305.2 ± 57.8 min versus retro-RP: 340.0 ± 117.9 min; p = 0.19), but securing the larger retroperitoneal space required for robo-RP took significantly longer; 50.8 ± 13.9 min versus 24.3 ± 9.6 min; p < 0.0001. Total anastomotic time (TAT) and TAT per suture were both significantly shorter for robo-RP (p < 0.0001). The coefficient of variation for time taken to place one suture was smaller for robo-RP than for retro-RP. DOS was lower for robo-RP with less variance (p < 0.01). Robo-RP had shorter drainage tube insertion, ambulated quicker postoperatively, and shorter hospitalization. Retro-RP had anastomotic complications; leaks (n = 2) and strictures (n = 2, requiring conventional open re-pyeloplasty). Robo-RP had no anastomotic complications and was ranked +1 unanimously., Conclusions: The RP anastomosis was quicker with less complications and more precise with robotic assistance in matched patients under similar circumstances. Should RP be indicated, robo-RP is recommended., Level of Evidence: III., Competing Interests: Conflicts of interest The authors declare that their research was conducted in the absence of any commercial financial relationships that could be construed as potential conflicts of interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Aim: Robot (da Vinci Si; Intuitive Surgical, Sunnyvale, CA) assisted retroperitoneoscopic diamond bypass pyeloplasty (R-RDBP) performed for ureteropelvic junction (UPJ) obstruction (n = 5) is presented., Methods: Patients were placed affected side up and the retroperitoneal space accessed conventionally using 3-4 trocars. The diamond-shaped anastomosis involved incising the lowest part of the renal pelvis 12-15 mm transversely and the ureter distal to the obstruction 10-12 mm longitudinally. The first two sutures were placed retroperitoneoscopically; one from the mid-caudal line of the renal pelvis to the apex of the ureteric incision (the apex of the diamond) and the other from the corner of the incision in the renal pelvis to halfway along the ureteric incision. Trocars were replaced and the robot system docked. The first robot suture was placed between these two sutures, and the anastomosis completed by suturing from posterior to ventral applying minimal tension to keep the anastomosis close to the renal pelvis. All sutures were interrupted absorbable 5-0 monofilament., Results: Mean age at R-RDBP was 4.3 (range: 1-14) years old. Height/weight were average. Preoperative Society for Fetal Urology (SFU) grading was 4.0 in all cases. All repairs were primary and progressed smoothly without perioperative complications; 3/5 had improved appetite postoperatively. Mean SFU grades 1-3 months postoperatively were 2.8, 2.2, and 1.6, respectively. Diuretic renography that was obstructive in all cases preoperatively was normal in four and delayed in one case, postoperatively., Conclusion: R-RDBP prevented rotation/kinking of the ureter, enhanced precision of suturing, and maximized the diameter at the anastomosis, facilitating smooth urine flow., Level of Evidence: LEVEL IV., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: Outcome studies for patients with anorectal malformation (ARM) have focused on fecal incontinence and quality of life, but a comparison of educational outcomes between ARM cases and controls has not been reported. The purpose of this study was to assess real-world educational outcomes, neurodevelopmental disorders and mental health disorders in ARM patients and compare to an age-matched control group., Methods: We performed a retrospective case-control study of children diagnosed with ARM from 1991 to 2017. We evaluated educational outcomes using an Early Developmental Instrument, Grades 3, 7, and 8 assessments, Grade 9 completion and performance, and high school graduation. Neurodevelopmental and mental health disorders were compared using International Classification of Diseases codes available from a population-based dataset., Results: A total of 96 ARM cases and 960 controls were identified. Cases were at greater risk of failing to meet expectations on Grades 7 and 8 assessments. After entering high school, ARM patients were at no greater risk than their peers of failing to meet expectations. Cases were more likely to have a developmental or intellectual disability (OR 3.59, p < 0.001), anxiety (OR 1.86, p = 0.023), depression (OR 2.35, p = 0.022) or hyperactivity disorder (OR 2.01, p = 0.036)., Conclusions: Our study demonstrated that ARM patients may be more likely to perform poorly in junior high school than controls and may be at greater risk of neurodevelopmental and mental health disorders. It is important for pediatric surgeons to anticipate these challenges and endorse psychosocial supports to optimize educational and mental health outcomes., Levels of Evidence: Level-Ⅲ., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: The optimal age for endorectal pull-through (ERPT) surgery in infants with short-segment Hirschsprung disease varies, with a trend toward earlier surgery. However, it is unclear if the timing of surgery impacts functional outcomes. We undertook the present study to determine the optimal timing of ERPT in infants with short-segment Hirschsprung disease., Methods: The NCBI PubMed database was searched for English-language manuscripts published between 2000 and 2019 analyzing functional outcomes for patient following the initial Soave ERPT for short-segment Hirschsprung disease. Raw data from these studies was obtained from the corresponding author for each manuscript. We combined data from these papers with our own institutional data and performed a meta-analysis., Results: A total of 780 infants were included in our meta-analysis. Constipation occurred in 1.0-31.7%, soiling 1.3-26.0%, anastomotic stricture 0.0-14.6%, and anastomotic leak 0.0-3.4%. Regarding age at ERPT, younger infants at the time of initial corrective surgery had higher rates of soiling, stricture, and leak. On sub-group analysis, patients <2.5 months at their initial corrective surgery had higher rates of soiling (25.9% vs. 11.4%, p<0.01), as well as stricture (10.0% vs 1.7%, p<0.01) and leak (5.5% vs 1.3%, p<0.01)., Conclusion: While age at Soave endorectal pull-through for short-segment Hirschsprung disease has decreased over time, functional outcomes associated with this trend have only recently been examined. Our findings suggest that patients <2.5 months old at the time of endorectal pull-through may have worse functional outcomes, emphasizing the need to consider further study of the timing of surgery in this population., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Aim: The safety and success of laparoscopic choledochal cyst surgery (LapCC) depends upon two critical elements during the hepaticojejunostomy anastomosis (HJA). These may be termed the Difficulty of Differentiation (DOD) and the Difficulty of Suturing (DOS). The type of imaging system (2 or 4 K) used may influence either of these. We compared outcomes of LapCC using 2 or 4 K imaging systems., Methods: LapCC were performed at a single institution by the same team using a 2 K system (2009-2018; n = 26) and a 4 K system (2018-2019; n = 11) were compared. 4 K cases were chosen to match 2 K cases to minimize bias. Five independent senior pediatric surgeons scored DOD and DOS blindly from intraoperative video recordings of LapCC using a subjective 5-point scale (5: impossible, 4: difficult, 3: tedious, 2: slow, and 1: easy) and rated their over all impression as +1 if 4 K was better, 0 if they were the same, and -1 if 4 K was worse. Total HJA anastomosis time (TAT) and TAT/suture were also calculated., Results: LapCC was performed in 37 age/weight/HJA diameter matched children. Scores for DOD (p<0.001) were lower with 4 K with less variance, although there was no difference in DOS (p = 0.08). Operative time (p = 0.03) and duration of hospitalization (p < 0.001) were significantly shorter with 4 K. 4 K was rated +1 unanimously. There was no difference in TAT (p = 0.17) and TAT/suture (p = 0.22). There was one HJA leak with 2 K (3.8%) and no complications with 4 K., Conclusions: Improved resolution with 4 K improved the progress of surgery as reflected by shorter operative time and duration of hospitalization, enhancing the performance of LapCC in children., Level of Evidence: III., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

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., Competing Interests: Declaration of Competing Interest The authors have no financial conflicts of interest., (Copyright © 2021. Published by Elsevier Inc.)

Purpose: We modified the U-shaped skin incision technique (UIT) during urethroplasty (UP) to prevent post-UP complications (post-UPC)., Patients and Methods: Our modified UIT (mUIT) involves undermining the epidermis for 5-7 mm around the U-shaped incision to create a thicker, better vascularized tissue layer for suturing the neourethra compared with UIT. To prevent injury to underlying connective tissue and avoid compromising blood perfusion, the scalpel should be held at a more slanted angle than is conventional. After the urethral plate is incised in the midline, stay sutures are placed to the middle of the penile shaft as a landmark, and three to five sutures are placed through the thick layer created by undermining. To facilitate mobilizing connective tissue during neourethroplasty, relaxing incisions may be used on the edges of the connective tissue. We compared 207 hypospadias patients who had primary or staged UP using either mUIT (n = 110) or UIT (n = 97) between 2003 and 2017 for incidence of post-UPC., Results: mUIT had significantly less post-UPC than UIT after a mean follow-up of 6.44 ± 0.26 years after final UP, 1/110 (0.9%) versus 15/97 (15.5%) (p < .0001)., Conclusions: Undermining the edges of the U-shaped incision during mUIT would appear to prevent post-UPC., Level of Evidence: Level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: Semaphorins are guidance cues for developing neurons, implicated in the determination of the migratory pathway of neural crest-derived neural precursors during enteric nervous system development. Recently, it has been reported that Semaphorin 3A (SEMA3A) expression is up-regulated in the aganglionic colon in Hirschsprung disease (HD) patients, suggesting that increased SEMA3A expression may be a risk factor for HD. Thus, the aim of our study was to determine the expression of SEMA3A using Sox10-Venus mice gut., Methods: We harvested the gut on postnatal day 2 (P2). SOX10-Venus+/EDNRB -/- mice were compared with SOX10-Venus+/EDNRB +/+ mice as controls. QRT-PCR was performed to determine gene expression of SEMA3A (n=8). Fluorescent immunohistochemistry was performed to assess protein distribution., Results: On P2, gene expression levels of SEMA3A were significantly increased in the HD group compared to controls in the proximal and distal colon (p<0.05). Laser scanning microscopy revealed SEMA3A expression was localized within the submucosa and muscle layer of the gut in both HD and controls. In HD, SEMA3A was highly expressed in the proximal and distal colon., Conclusions: In the present study, we demonstrated that SEMA3A expression is increased in the EDNRB-/- HD model on P2, suggesting that SEMA3A may interfere with ENCC migration, resulting in an absence of enteric neurons., (Copyright © 2017 Elsevier Inc. All rights reserved.)

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., (Copyright © 2017 Elsevier Inc. All rights reserved.)

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., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background/purpose: We report the current status of patients who underwent augmentation cystoplasty (AC) at least 20years previously., Methods: Surgical history, incidence of urinary tract infection (UTI) and bladder stones, vesicoureteral reflux (VUR), urine cytology, renal function, a colon cancer tumor marker (carcinoembryonic antigen: CEA), and patient outcomes were assessed., Results: Forty patients who underwent AC (mean age: 34.4years; mean follow-up time: 24.3years) were analyzed. Mean age at AC was 11years. Incidence of bladder stones was 30%. There were no incidences of carcinoma after AC, and CEA levels were not increased. Ureteral reimplantation (URI) was performed in 21 patients. URI performed at the same time as AC was successful in 14 cases (93%) and unsuccessful in 1 (7%) because of persistent VUR. UTI developed after AC in only 1 patient (2.5%) with persistent VUR. This patient required unilateral nephrectomy 18years after the AC because of repeated UTIs. Thirty-four patients (85%) were employed, and 4 (10%) were married. Two of the 19 female patients (11%) had experienced pregnancy and delivery. Five patients (13%) had mental disorders., Conclusion: Ultra long-term follow-up suggests that AC is a safe procedure with manageable sequelae, although some mental health issues remain., Type of Study: Case series with no comparison group., Level of Evidence: Level IV., (Copyright © 2017. Published by Elsevier Inc.)

Background/purpose: We evaluated routine intraoperative residual rectourethral fistula measurement (IRRFM) in 20 consecutive male imperforate anus with recto-bulbar (RB; n=12) or recto-prostatic (RP; n=8) fistula during laparoscopically assisted anorectal pull-through (LAARP) for preventing incomplete fistula excision (IFE) on mid-term follow-up., Methods: Twenty consecutive LAARP performed at a mean age of 10months (range: 3-30) followed-up for a mean of 4.8years (range: 1.5-9) were reviewed. IRRFM involves using a calibrated catheter and a cystoscope to measure the distance between where dissection was ceased at the rectal end and the urethral orifice (Figure). Dissection and IRRFM were repeated until the fistula was <5mm, then tied, and divided. Magnetic resonance imaging (MRI) and pelvic ultrasonography were used to exclude IFE and cyst formation., Results: Residual fistula was 4-18mm on initial IRRFM. Unless measured, dissection cannot proceed to <5mm safely with poentical for urethral injury or IFE. With experience, initial IRRFM were shorter, especially in RP (Table 1). Before the IRRFM era, our incidence of cysts was 2/11 (18%), but here we found no evidence of cyst formation on MRI, no dysuria, and no urinary tract infections., Conclusions: Mid-term review demonstrates that IFE can be prevented successfully by IRRFM during LAARP., Level of Evidence: Case Series with no Comparison Group, Level IV., (Copyright © 2017. Published by Elsevier Inc.)

Purpose: The purpose of this study was to examine whether acute non-perforated appendicitis (ANPA) can be safely triaged by a pediatrician for conservative management (CM) using gray-scale ultrasonography with power Doppler (GSPD)., Method: Seventy five cases of ANPA assessed by a pediatrician with GSPD (2013-2015) were reviewed. GSPD grading for ANPA was: I: slightly irregular wall/normal blood flow; II: irregular wall/increased blood flow; III: irregular wall/decreased blood flow; and IV: absence of wall/blood flow. Grades I/II were managed conservatively with intravenous antibiotics then encouraged to book for interval appendectomy (IA). Grades III/IV were reviewed for emergency appendectomy (EA) by a pediatric surgeon., Results: GSPD grading was I (n=26), II (n=36), III (n=9), and IV (n=4). EA was required for 5 cases, one grade III, and four grade IV cases. One grade IV case was treated conservatively after surgical review but EA was unavoidable. Of the remaining 70 cases discharged well after a mean of 5.7days hospitalization, 25/70 had IA with chronic inflammation on histology, 6/70 had recurrence of ANPA treated successfully by EA, and 39/70 remain asymptomatic at least 10months after declining IA. Overall, GSPD triaging with CM was cheaper than surgery., Conclusions: GSPD performed by pediatricians appears to be safe/effective for triaging ANPA., Level of Evidence: Level III., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: CHARGE is our technique for reinforcing the ventral penile shaft with pedicled pericordal/scrotal fat, pedicled perimeatal connective tissue, or a combination of these at the time of initial hypospadias surgery. Such pedicled grafts "charge" poorly developed urethral plates and thin ventral foreskin prior to urethroplasty to improve compromised vascular perfusion that could prevent post-urethroplasty complications (post-UPC)., Methods: We reviewed post-UPC in 179 staged hypospadias repair patients (1997-2015). CHARGE, adopted routinely in 2010 was used in 39 patients (C-group), not indicated in 7 because ventral connective tissue was thick, and not used in 133 (NC-group). Initial hypospadias surgery included foreskin degloving with or without chordectomy, dorsal plication, tunica albuginea incision, or a combination of these., Results: Subject demographics were similar. NC had significantly more post-UPC than C (25 versus 0; p<.01) comprising stenosis (n=14), fistula (n=7), diverticulum (n=2), and wound infection (n=2) that developed after a mean of 0.7±0.2years (range: 1day-2.8years). Extra time taken for CHARGE was less than 15minutes in all cases. Mean follow-up after urethroplasty (years) was significantly shorter in C (1.5±1.0 versus 5.7±3.8) (p<.01), but almost double the time taken to develop post-UPC., Conclusion: CHARGE would appear to prevent post-UPC., Level of Evidence: Retrospective Comparative Study - Level III., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: We present a first report of the long-term follow-up of biliary atresia (BA) patients who became anicteric with the native liver (ANL; total bilirubin <1.5mg/dL) after redo-Kasai., Methods: Forty-six redo-Kasai cases (1984-2015) were the subjects for this study. ANL ratios were determined using the Kaplan-Meier estimate., Results: BA type was I (n=3), II (n=1), and III (n=42). Mean ages (initial/redo) at Kasai were 60.3/231.9days, respectively. Jaundice persisted after the initial Kasai in 24/46 cases while 22 had recurrence of jaundice after initially becoming anicteric. After redo, 5/24 of the persistent jaundice cases and 14/22 of the initially anicteric cases became anicteric (p<.05). Of these 19, 7 (one type I, six type III) are currently ANL while the remaining 12 had LTx or died. Morbidity/Complications documented in the 7 post-redo ANL cases after a mean follow-up of 16.7years (range: 10.0-31.1) included 4 episodes of cholangitis, 3 episodes of portal hypertension, 4 episodes of esophageal varices, 3 episodes of splenomegaly, one splenectomy, and 4 episodes of thrombocytopenia., Conclusions: Ours is the first long-term follow-up study of redo-Kasai cases. We found that the ANL ratio after redo-Kasai was low at 7/46 (15.2%) and that ANL were prone to multiple morbidity., Level of Evidence: Retrospective comparative study, level III., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Conventional pneumoperitoneum (CP) and automatically maintained pneumoperitoneum using AirSeal Intelligent Flow System (AiFS) were compared during pediatric laparoscopic appendectomy (LA) using intraperitoneal pressure (IPP) and hemodynamic parameters., Methods: A prospective review of 39 children aged 3-14years who had standard 3-trocar LA was performed. Pneumoperitoneum was either AiFS (n=18) or CP (n=21) according to the surgeon's preference. IPP during insertion of trocars in all subjects was initially 8-10mmHg, which was reduced to 5mmHg then maintained until LA was completed. Data were collected every 5min during pneumoperitoneum., Results: Subject demographics were similar for both groups. During pneumoperitoneum, average IPP (AiFS: 7.9; CP: 9.0mmHg), average systolic blood pressure (AiFS: 100.4; CP: 106.9mmHg), and average end-tidal CO 2 (EtCO 2 ; AiFS: 35.7; CP: 38.5mmHg) were significantly different (p<.05, respectively), while pulse (AiFS: 92.1; CP: 96.4bpm), oxygen saturation (AiFS: 98.8; CP: 98.8%), body temperature (AiFS: 37.2; CP: 37.4), urine output (AiFS: 2.7; CP: 2.4mL/kg per hour), operative time (AiFS: 72.2; CP: 76.2mins), blood loss (AiFS: 3.6; CP: 3.5mL), recommencement of oral intake (AiFS: 1.3; CP: 1.4days), and postoperative hospitalization (AiFS: 4.3; CP: 3.8days) were not., Conclusion: Because IPP was significantly lower during LA with AiFS, EtCO 2 and BP were significantly lower., Level of Evidence: Treatment study; prospective comparative study - level II., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: We reviewed renal function after pyeloplasty (PP) in cases of prenatally diagnosed ureteropelvic junction obstruction (PDUPJO) to determine the impact of preoperative urinary tract infection (UTI) on the timing of PP., Methods: We retrospectively reviewed 81 cases of PDUPJO diagnosed between 1998 and 2013. Incidence of UTI was used to divide 37 kidneys requiring PP (3 bilateral) into U(-): UTI≤1; n=25, and U(+): UTI≥2; n=12 to compare age at PP, grade of hydronephrosis (HN) on ultrasonography (US), glomerular filtration rate (GFR) on diethylenetriaminepentacetic acid (DTPA) renography, and uptake on dimercaptosuccinic acid (DMSA) scintigraphy pre/post PP., Results: Age at first UTI and age at PP were similar. HN improved significantly postPP in all cases. Although DTPA and DMSA were similar prePP, improvement postPP was better in U(-) than U(+), but only DMSA in U(-) was statistically significant (15.2%±4.0% vs. 20%±3.7%; p=0.049). There were no complications or UTI postPP., Conclusion: Two or more UTIs would appear to be associated with postPP renal dysfunction in PDUPJO. Thus, PP should be performed after the initial UTI but before the second UTI., (Copyright © 2015 Elsevier Inc. All rights reserved.)

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<0.05)., Conclusions: Medium-term POFC is better when the ARL is used as the landmark for RMD during L-TAPT for HD., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: The association of vaginal atresia (or Mayer-Rokitansky-Kuster-Hauser Syndrome) with imperforate anus is rare and can present significant diagnostic and therapeutic challenges. This study describes clinical characteristics, surgical treatment and outcomes in this group of complex children., Methods: Records of 20 patients were retrospectively analyzed from two pediatric surgical centers., Results: Five patients were excluded from the long-term analysis due to inadequate information, leaving long-term follow-up in 15 patients. Mean follow-up was 10 years (range 1-31.1 years). The diagnosis of vaginal atresia was made pre-operatively in 12 out of 15 patients, and in three patients it was identified during the anoplasty. The anorectal malformations were rectoperineal (N=2), rectovestibular (N=6), recto-bladder neck (N=1) and imperforate anus without fistula (N=6). Satisfactory surgical repair was performed in 13 patients, while one continues to stool through a low perineal fistula awaiting definitive surgery and another underwent a colostomy and mucous fistula. Delayed vaginal reconstruction was due to a failure to identify the problem prior to anoplasty (N=3). Long-term results demonstrated that anorectal continence was much worse than initially appreciated, and many had associated urinary incontinence. Overall stooling score was far lower than in a separate group of children with imperforate anus without vaginal atresia (Levitt and Peña, 2007)., Conclusions: Vaginal atresia with imperforate anus is a rare and an extensive pre-operative workup of females with imperforate anus must include assessment of vagina patency. Vaginal reconstruction and anorectal continuity can be performed in a variety of approaches, but long-term continence is often not optimal. We propose a pathway for management of this difficult genito-anorectal disorder., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Purpose: All reports comparing laparoscopy-assisted anorectoplasty (LAARP) with posterior sagittal anorectoplasty (PSARP) in male high-type imperforate anus include a mix of recto-vesical, recto-prostatic, recto-bulbar, and absent fistula cases without focusing on recto-bulbar fistula (RBF), the most challenging type to treat laparoscopically. We compared LAARP with PSARP for treating only RBF., Method: We used our fecal continence evaluation questionnaire (FCE; maximum score=10), scoring of magnetic resonance imaging (MRI) findings (MRI scores), and the angle between the rectum and the anal canal (RAA) to assess 20 RBF cases (LAARP=12, PSARP=8) treated from 2000 to 2013 prospectively., Results: Mean ages at surgery, MRI scores, mean RAA, and duration of raised C-reactive protein (6.6 vs. 6.7days; p=NS) were similar. In all cases, postoperative MRI showed no residual fistula and normal urination. LAARP had consistently higher FCE (7.9 vs. 7.8 at 3years; 8.6 vs. 8.3 at 5years; 8.9 vs 8.6 at 7years; p=NS, respectively), less wound infections (0 vs. 37.5%; p<0.05), higher incidence of rectal mucosal prolapse (50.0 vs. 0%; p<0.05), and required less analgesia (p<0.05)., Conclusion: Although LAARP and PSARP are comparable for treating RBF, LAARP is associated with less wound infections and higher incidence of rectal mucosal prolapse., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: We reviewed post-portoenterostomy (PE) biliary atresia (BA) patients who became "jaundice-free" (JF; total bilirubin (T-bil) ≤1.2 mg/dL) to determine factors associated with survival with the native liver (SNL)., Methods: We reviewed 76 BA patients treated by PE at our institute between 1989, when liver transplantation (LTx) became available in Japan, and 2012, prospectively. Of these, 60 who became JF and remained JF were divided into two groups, SNL (n=44) and LTx (n=16). Age and weight at PE, pre- and post-PE T-bil, AST, γ-GT, time taken to become JF, corticosteroid requirements, incidence of cholangitis, and micro-bile duct size were compared between the two groups., Results: The SNL patients became JF significantly earlier: 58 vs. 115 days (p<.05). Corticosteroid requirement, cholangitis, and postoperative AST/γ-GT were significantly lower in the SNL patients (p<.05). SNL was significantly higher if patients became JF ≤60 days post-PE (p<.01). LTx was performed from 0.5 to 11 years post-PE (mean=3.4). All patients who had cholangitis within 3 months of PE eventually required LTx (p<.05)., Conclusions: Becoming JF ≤60 days post-PE would appear to be a factor associated with SNL, while cholangitis within 3 months of PE would appear to be associated with LTx. Elevation of AST and γ-GT would also appear to be early indicators of risk for LTx during follow-up of JF patients after successful PE., (© 2013.)

Background: Surgeons are at risk for developing work-related musculoskeletal symptoms (WMS). The present study aims to examine the physical factors and their association with WMS among pediatric laparoscopic surgeons., Methods: A questionnaire consisting of 21 questions was created and mailed to 25 pediatric laparoscopic surgeons (LG). 23/25 surgeons (92%) completed the survey. The questionnaire was analyzed and then split into 2 groups. Group 1 (LG1) included surgeons with greater laparoscopic experience, and group 2 (LG2) included surgeons with less important laparoscopic experience. In addition, we constructed and sent to the same surgeons a similar questionnaire focused on WMS after an open procedure (OG) with the aim to compare results of LG with OG., Results: The prevalence rate of WMS with shoulder symptoms was 78.2% in surgeons that performed laparoscopy for more than 10 years, with 60.8% also reporting other pain. In 66.6% this pain is evident only after a long-lasting procedure. Forty-four percent of these surgeons require painkillers at least twice a week. Fifty percent of these surgeons also suffer at home. Fifty-five and one half percent of surgeons indicate that this pain is related to their laparoscopic activity. Forty-three and a half percent think that laparoscopy is beneficial only for the patient but has a bad ergonomic effect for surgeons. Sixty-five and two-tenths percent think that robotic surgery can be helpful to improve ergonomics. Comparing the groups, WMS occur more frequently in LG (78.2%) than in OG (56.5%), but this difference was not statistically significant (χ(2)=0.05). In addition, WMS occur more frequently in LG1 (84.6%) than in LG2 (70%), but this difference was not statistically significant (χ(2)=0.05)., Conclusions: These results confirmed a strong association between WMS and the number of laparoscopic procedures performed. Skilled laparoscopic surgeons have more pain than less skilled laparoscopic surgeons. WMS in the same group of surgeons are more frequent after laparoscopy than after open procedures. The majority of surgeons refer to shoulder symptoms., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Aim: Liver herniation (LH) in congenital diaphragmatic hernia (CDH) may not be a reliable prognostic indicator. We measured pulmonary artery (PA) diameters in CDH + LH as an alternative., Methods: Of 41 consecutive cases of prenatally diagnosed left-sided CDH treated from 2002 to 2010, 19 had CDH + LH and 22 had CDH - LH. Ultrasonography and magnetic resonance imaging were used to assess LH and echocardiography to measure PA diameters during the third trimester (fetal; 32-34 weeks), at birth, and on day 2 of life., Results: In CDH + LH survivors (9/19; 47%), fetal right PA (RPA) diameters were significantly larger than in nonsurvivors (2.58 ± 0.56 vs 1.82 ± 0.35 mm; P < .01), but left PA (LPA) diameters were not (1.73 ± 0.38 vs 1.59 ± 0.22). In survivors, fetal RPA was greater than 2 mm in all but one case, and both PA diameters increased significantly by birth (RPA, 2.58 ± 0.56 vs 3.52 ± 0.54; LPA, 1.73 ± 0.38 vs 2.60 ± 0.40; both P < .01). Final diameters at birth in survivors were at least 2.5 and 2.0 mm, respectively. In nonsurvivors, both PAs were significantly smaller (RPA, 3.52 ± 0.54 vs 2.04 ± 0.31; LPA, 2.60 ± 0.40 vs 1.68 ± 0.18; P < .01), with no observed increase by birth. Survival in CDH - LH was 82% (18/22)., Conclusion: PA diameter appears to be correlated with prognosis in infants with CDH + LH., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background/purpose: Snodgrass tubularized incised plate urethroplasty (SUP) is versatile and has good cosmesis. However, postoperative meatal/neourethral stenosis (M/N-S) is common enough for some surgeons to add a dorsal inlay graft (DIG) harvested from the inner prepuce and sutured to cover the longitudinal midline incision of the urethral plate. This is the first formal assessment of the effectiveness of DIG for preventing M/N-S., Methods: We reviewed the medical records of 100 consecutive SUP cases performed by a single surgeon between 2003 and 2010 comparing SUP + DIG (S + D group, n = 50) with SUP - DIG (S - D group, n = 50). Mean follow-up was 3.6 years. Data were analyzed statistically using the χ(2), 2-way ANOVA, and Mann-Whitney tests, with P < .05 considered significant., Results: Severity of hypospadias and type of SUP were similar. Mean age at SUP was 3.3 years in S + D and 3.6 years in S-D (P = NS). There were 4 complications in the S + D group: urethrocutaneous fistula (n = 3) and neourethral stenosis without diverticulum (n = 1). There were 15 complications in the S-D group : meatal stenosis (n = 2), neourethral stenosis with or without diverticulum (n = 6), urethrocutaneous fistula (n = 7) (P < .01). M/N-S was significantly less in the S + D group (1 vs 8; P < .05)., Conclusions: We strongly recommend that DIG be performed routinely during SUP., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Enteric neural crest cells (ENCCs) were labeled with VENUS, an enhanced green fluoroscein protein, to record their migration in genetically engineered transgenic (SOX10-VENUS) mice., Materials and Methods: Pregnant SOX10-VENUS mice were killed on day 12.5 of gestation. The colorectum was excised from each embryo (n = 20) and placed in tissue culture medium. Time-lapse images captured using fluorescence microscopy at 10-minute intervals for 3000 minutes were compiled into a video to display ENCC migration., Results: At 0 minutes, VENUS(+) ENCC were observed to be clustered in the cecum and proximal colon (vagal ENCC), and similar cells were also seen in the rectum/sacrum (sacral ENCC). After 500 minutes, vagal VENUS(+) ENCC had migrated caudally from the proximal colon to the midcolon, reaching the distal colon after 800 minutes. Sacral VENUS(+) ENCC had migrated rostrally and transversely by 1250 minutes and had integrated with vagal ENCC by 2500 minutes., Conclusion: We recorded the actual rostral-to-caudal migration of vagal ENCC, caudal-to-rostral migration of sacral ENCC, and their integration in the developing mouse hindgut. Such direct evidence of ENCC migration may further elucidate understanding of ENCC development, thus providing insight into the histopathology of bowel dysmotility disorders., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Aim: The anorectal angle (AA) influences defecation after pull-through (PT) for imperforate anus (IA). We compared postoperative AA and continence after Georgeson's laparoscopy-assisted colon PT (GPT) and Peña's posterior sagittal anorectoplasty (PSARP) for high/intermediate-type IA., Methods: We reviewed 33 high/intermediate-type IA cases (20 GPTs and 13 PSARPs) prospectively. All had colostomy initially as neonates. Anorectal angle was measured as the angle between the rectum and the anal canal on barium enema. A fecal continence evaluation questionnaire (FCEQ) consisting of 5 parameters (frequency of defecation, staining/soiling, perianal erosion, anal shape, and requirement for medication; maximum score = 10) was evaluated in 28 cases (15 GPTs and 13 PSARPs) followed up for more than 3 years., Results: Mean age at PT was similar (6.6 months for GPT and 6.3 months for PSARP; P = not significant). There was no significant difference in mean AA. The FCEQ scores for GPT were generally higher throughout the study and significantly better from 3 years postoperatively (P < .05)., Conclusions: We are the first to confirm that effective AA similar to PSARP can be achieved after GPT, although FCEQ would suggest that GPT has less detrimental functional impact., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Purpose: The piebald lethal mouse with a deletion of endothelin-B receptor gene (EDNRB) is a model for Hirschsprung's disease (HD), whereas the SOX10 gene is vital for the development of intestinal neural crest-derived cells. Recently, we created a SOX10 transgenic mouse with intestinal neural crest-derived cells visible with enhanced green fluorescent protein (VENUS), that is, SOX10-VENUS(+)/EDNRB(sl/sl) to investigate intestinal innervation in HD., Methods: SOX10-VENUS(+)/EDNRB(sl/sl) (n = 30) were compared with wild-type littermates as controls (EDNRB(s/s), n = 30). Mice were killed on days 3, 7, or 12 of age. The entire colorectum was excised, fixed with 4% paraformaldehyde, and examined using fluorescence microscopy alone without staining., Results: In normoganglionic colorectum from controls, a grid network of nerve fibers/glial cells was visualized that connected smoothly with extrinsic nerve fibers running along the colorectal wall. In aganglionic colorectum from SOX10-VENUS(+)/EDNRB(sl/sl) mice, there was no grid network and more extrinsic nerve fibers than controls that invaded the colon wall becoming elongated with branching fibers. Normoganglionic colon from controls and SOX10-VENUS(+)/EDNRB(sl/sl) mice appeared the same. Innervation patterns did not change over time., Conclusion: This is the first time for abnormal enteric innervation in aganglionic colon in a model for HD to be visualized without staining., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Background: The changes in liver blood flow associated with living donor liver transplantation (LDLT) in children have not yet been studied. The aim of the present study was to investigate changes in hepatic hemodynamics before and after pediatric partial liver transplantation., Methods: In 7 pediatric recipients with congenital cholestasis and native liver Child-Pugh classes B and C, portal vein flow (PVF) and hepatic arterial flow (HAF) were measured using an ultrasonic transit time flow meter before removal of the native liver and after transplantation and compared with donor left PVF and donor left HAF., Results: The mean portal contribution to total hepatic blood flow was markedly decreased in the recipient native liver compared with that in the donor (69% +/- 15% vs 32% +/- 15%; P = .0003) and after reperfusion changed to almost the same ratio as that in the donor liver (73% +/- 18%; P < .0001)., Conclusion: The extreme imbalance between PVF and HAF that is common in implanted partial liver in adult LDLT recipients was not observed in pediatric LDLT. After transplantation of an appropriately sized liver graft, the portal contribution to total liver blood flow normalized to the value for normal liver.

Introduction: We routinely perform intraoperative endoscopy (IOE) of the intrahepatic bile duct (IHBD) and intrapancreatic bile duct (IPBD) with a pediatric cystoscope during excision of choledochal cyst (CC). Here we compare extended long-term follow-up patients with a series of recent cases., Methods: We compared 25 CC patients treated over the past 5 years (group 1) with 69 CC patients treated between 1986 and 1998 and followed up for more than 10 years (group 2), focusing on stone formation., Results: In group 1 (mean follow-up, 2.6 years) IOE identified IHBD debris in 7 (28%) of 25 and IPBD protein plugs in 9 (36%) of 25. In group 2 (mean follow-up, 14.6 years) IOE identified IHBD debris in 11 (16%) of 69 and IPBD protein plugs in 17 (25%) of 69. There was no relation between type of CC and incidence of IHBD debris or IPBD protein plugs. The incidence of postoperative stones to date is 0% in group 1 and 2.9% in group 2, rates far lower than those reported in the literature., Conclusions: We attribute our lower incidence of stones directly to IOE and recommend that it be performed routinely during cyst excision. It is simple, is effective, and improves outcome., (Copyright 2010 Elsevier Inc. All rights reserved.)

Introduction: We report a novel technique to measure the length of the rectourethral fistula (RUF) in male patients with high-/intermediate-type imperforate anus during laparoscopically assisted anorectal pull-through (LAARP) to prevent incomplete excision., Method: During LAARP for RUF in 5 male patients (mean age, 4.6 months; prostatic in 3, bulbar in 2), the RUF was dissected carefully close to the urethra and opened; and a fine catheter with 10-mm calibrations was inserted by the laparoscopic surgeon until it was seen to emerge at or near the verumontanum by another surgeon performing cystoscopy. The laparoscopic surgeon then measured the distance from the point where dissection was ceased at the rectal end to the urethral orifice. The RUF was dissected free from the prostate for exactly this length, tied, and excised; and colon pull-through was performed to finish LAARP., Result: Rectourethral fistulae ranged from 5 to 15 mm and were much longer than expected. All dissections were uncomplicated without any injury to the urethra, and postoperative courses were unremarkable. At mean follow-up of 11 months, urination is normal in all without evidence of residual fistula., Conclusion: Knowing the exact length of the RUF facilitates safe and complete excision in an otherwise blind situation., (Copyright 2010 Elsevier Inc. All rights reserved.)

Aim: Immaturity of neurons in normoganglionic colon in Hirschsprung disease (HD), anorectal malformation (AM), idiopathic constipation (IC), and normal controls (C) was assessed using polysialyated neural cell adhesion molecule., Methods: Polysialyated neural cell adhesion molecule immunoreactivity in 3 sections of normoganglionic colon from HD (n = 48), AM (n = 25), IC (n = 36), and C (n = 18) were scored semiquantitatively according to age; 1 day to 11 months (G1), 1 to 4 years (G2), and 5 years and older (G3)., Results: Neurons in all specimens appeared mature irrespective of age on hematoxylin-eosin stain. Polysialyated neural cell adhesion molecule was positive (immaturity) in all specimens during G1 (1.34 in HD, 1.60 in AM, 0.89 in IC, and 1.59 in C) and decreased significantly with age in C (0.34* for G2, 0.25* for G3; *P < .01), decreased after 4 years old in IC (0.93 for G2, 0.10(#) for G3; (#)P < .05), decreased gradually in AM (1.10 for G2, 0.75( section sign) for G3; ( section sign)P < .05), but remained strongly positive in HD (1.34 for G1, 1.26 for G2, and 1.21 for G3; P = not significant), which after 4 years was significantly higher than C (P < .05)., Conclusion: Postoperative colonic dysmotility may be because of persistence of immature neurons in HD and impaired maturation of neurons in AM and IC.

Purpose: Both the dentate line (DL) and anorectal line (ARL) are anatomic landmarks for rectal mucosal dissection during transanal pull-through for Hirschsprung disease. We compared outcome after rectal mucosal dissection commencing above the DL (DL group; n = 8) with outcome after rectal mucosal dissection commencing on the ARL (ARL group; n = 6) with normal babies (Cont group; n = 10)., Methods: Rectal mucosal dissection commenced on the ARL in the ARL group and at various levels (0-10 mm) above the DL in the DL group. Outcome was assessed prospectively for 6 months using a standard structured questionnaire., Results: Subject demographics were not significantly different. Differences in frequency of motions between the ARL and Cont groups were not statistically significant after 3 months of age, but the DL group had significantly more motions than the other 2 groups after 4 months of age (P < .01). At 6 months of age, fecal staining was 17% in the ARL group, 63% in the DL group, and 0% in the Cont group. Anal shape was normal in the ARL and Cont groups, but 50% of the DL group had visible anal mucosa., Conclusion: Bowel function in the ARL group is similar to normal, and because the ARL is easily identifiable without the need for subjective interpretation, we recommend using the ARL as a landmark for rectal mucosal dissection during transanal pull-through for Hirschsprung disease.

Purpose: The aim of the study was to report a new technique for repairing hypospadias with severe chordee (HSC)., Methods: Our new technique involves making a long, wide, U-shaped incision on the ventral penis from the coronal sulcus to very distal to the meatus and dissecting to create a flap (U-flap). During dissection, the urethra is divided just proximal to the meatus. After release of chordee, the U-flap is returned to the ventral penile shaft and sutured in place. A buttonhole made distally in the U-flap is anastomosed to the cut end of the urethra to create a neomeatus. Snodgrass urethroplasty is performed 6 to 18 months later. We have treated 11 patients with HSC (mean age, 22.3 months) using this technique., Results: Postoperatively, all U-flaps were viable. The neomeatus appeared to be more proximal because the penis was straighter. Urethroplasty using the central part of the U-flap was uncomplicated by scar tissue and successful in all cases. After a mean follow-up of 15.7 months, all patients have satisfactory penises without stenosis or diverticulum, although 1 had fistula., Conclusion: Our U-flap technique allows the ventral penis to be preserved intact without scarring for second-stage urethroplasty and as a result is well suited for treating HSC.

Purpose: The aim of the study was to establish the natural history of bile lakes (BLs) in patients with postportoenterostomy biliary atresia (BA) and assess their prognostic implications, in particular, if they are a risk factor for cholangitis., Methods: One hundred twenty-one postsurgical BA patients were divided into 2 groups as follows: period 1, or preliver transplantation era group (n = 56), and period 2, or liver transplantation era group (n = 65). Presence of BL and outcome were examined in relation to the incidence of cholangitis., Results: In period 1, there were more multiple BLs (MBLs) in nonsurvivors than survivors. Non-BL survivors had significantly lower mean total number of episodes of cholangitis than MBL survivors (P < .01). In period 2, 58 survived (native liver in 35; transplanted liver in 23). Bile lakes were present in 9/58 (16%) survivors (single in five, multiple BLs in four), and 1/7 (14%) nonsurvivors had MBLs. Mean total number of episodes of cholangitis in the 4 MBL survivors and the 49 non-BL survivors were not significantly different., Conclusion: Our results suggest that presence of BL is a risk factor for cholangitis in the long-term but not in the short-term.

A 14-year-old adolescent girl with superior mesenteric artery syndrome was referred to us after failure of conservative management. Anterior transposition of the duodenum was performed and appears to be safe for permanently circumventing the duodenal obstruction seen in superior mesenteric artery syndrome, even in a pediatric population.

Purpose: The objective of this study is to analyze the outcome of surgery for high/intermediate-type imperforate anus using anal endosonography (AES), magnetic resonance imaging (MRI), and a continence evaluation questionnaire (CEQ)., Methods: In this study, 24 cases of high/intermediate-type imperforate anus were studied. Fifteen of 24 had Georgeson's laparoscopy-assisted anorectoplasty (GLA), and 9 of 24 had Pena's posterior sagittal anorectoplasty (PPA). All subjects had AES and MRI postoperatively. On AES, differences in thickness of the external sphincter (ES) and puborectalis (PR) at 3 and 9 o'clock were compared, and if pull-through colon was central, AES was 0. On MRI, differences in thickness were analyzed semiquantitatively and scored; if muscles were of even thickness, the score was 0; slight difference was 1, and marked difference was 2. A 5-parameter CEQ questionnaire (maximum score, 10) was administered to 16 of 24 subjects followed up for more than 3 years (9 GLA, 7 PPA). Surgical stress was assessed using mean febrile period, duration of raised white blood cell count, and peak C-reactive protein level., Results: Mean age at surgery and mean postoperative period for both groups were not statistically different. There were no differences in mean muscle thickness for ES or PR on AES according to procedure (ES: GLA = 0.19 +/- 0.15 mm, PPA = 0.16 +/- 0.09 mm, P = .59; PR: GLA = 0.19 +/- 0.19 mm, PPA = 0.22 +/- 0.15 mm, P = .69). Magnetic resonance imaging scores were also not significantly different according to procedure (GLA: 0.77 +/- 0.83; PPA: 0.75 +/- 0.50, P = .97). When CEQ were compared annually, scores for GLA were generally higher throughout the study but only statistically significant at 3 and 4 years (P < .05). Differences in parameters of surgical stress were not significant., Conclusion: Although there were no differences in muscle thickness and parameters of surgical stress observed according to technique, GLA would appear to provide better outcome based on CEQ scores.

Purpose: The outcome of ureteric reimplantation (UR) during bladder augmentation (BA) for high-grade vesicoureteric reflux (HVUR) in patients with neurogenic bladder was assessed to determine the effectiveness of UR. A literature review of HVUR and BA was also performed., Methods: Twenty-two consecutive sigmoidocolocystoplasty patients were reviewed retrospectively; 10 had unilateral HVUR only, 3 had bilateral HVUR, and 9 had unilateral HVUR associated with low-grade VUR in the contralateral renal unit (RU). Preoperatively, VUR was grade V in 3 RU, grade IV in 22 RU, grade III in 5 RU, and grade II in 4 RU. Ureteric reimplantation was performed in the native bladder in 24 RU (16 patients) and in the colon cap in 10 RU (6 patients)., Results: Mean age at sigmoidocolocystoplasty/UR was 8.5 years (range, 2-15 years). Mean follow-up was 12.8 years (range, 2-22 years). Postoperatively, cystourethrography showed residual VUR (grade IV to grade III) in only 1 RU (3%); diethylenetriamine pentaacetic acid scintigraphy showed no obstruction in all RU; (99m)Tc-dimercaptosuccinic acid was improved in 13 RU, unchanged in 18 RU, and worsened in 3 RU; and serum creatinine remained normal in 20 patients and worsened in 2. Urinary tract infection, universal preoperatively, was seen postoperatively in only 2 patients. In the literature, 0% to 16.7% of HVUR persisted after BA alone, and no long-term data were available., Conclusions: Ureteric reimplantation during BA is safe and effective for treating HVUR in patients with neurogenic bladder.

Purpose: Homozygous mutant Ncx/Hox11L.1-deficient (Ncx-/-) mice develop mega-ileo-ceco-colon with a caliber change in the proximal colon. This study investigated the mechanism of intestinal motility in these mice., Method: Five-week-old male and female Ncx-/- mice with mega-ileo-ceco-colon (n = 8) were compared with age-matched male BDF1 mice used as controls (n = 8). All mice were sacrificed, and uniform-sized strips of jejunum, ileum, proximal colon, and distal colon were exposed to electrical field stimulation and pretreatment with atropine sulfate, guanethidine, or tetrodotoxin. Contractile responses were recorded and compared., Results: Longitudinal muscle from strips of jejunum and ileum from all mice (BDF1 and Ncx-/-) did not respond to electrical field stimulation, whereas ileal circular muscle contracted in BDF1 mice and contracted and relaxed in Ncx-/- mice. Pretreatment with atropine sulfate and guanethidine inhibited the responses of circular muscle of distal colon and ileum in BDF1 mice significantly (P < .05), but no effect was observed in Ncx-/- mice., Conclusion: In ileum, BDF1 mice have cholinergic and adrenergic dominant contraction patterns, whereas Ncx-/- mice have relaxation-dominant patterns because of nonadrenergic, noncholinergic nerves. Based on this, there would appear to be some kind of variation in the gastrointestinal nerve supply in Ncx-/- mice.

Aim: The objective of this study is to compare laparoscopy-assisted pull-through (LPT) and open pull-through (OPT) for Hirschsprung's disease with special reference to postoperative fecal continence., Methods: Thirteen OPT patients (1991-1996) were reviewed retrospectively, and 22 LPT (1997-2002) were reviewed prospectively. A continence evaluation questionnaire (CEQ, max score = 10) assessing frequency of motions, severity of staining, severity of perianal erosions, anal shape, and requirement for medications was used. Severity of staining was graded as none = 2, occasional = 1.5, often = 1, always = 0.5, and soiling = 0, and severity of staining less than or equal to 1 was defined as moderate to severe incontinence. Presence of fever (peak and duration), raised white cell count (>10,000/microL), and C-reactive protein (>0.3 mg/dL) were used to assess surgical stress., Results: Pull-through was endorectal in all cases. Mean age at pull-through was not statistically different between the 2 groups. Annual CEQ scores for 7 years after LPT were 6.3, 6.9, 7.3, 7.7, 8.3, 8.9, and 9.0, and after OPT were 5.6, 6.4, 7.0, 7.5, 7.8, 8.3, and 8.4. Although CEQ scores were higher after LPT throughout, the difference was not statistically significant. The incidence of moderate to severe incontinence after 4 years was 54% (7/13) for OPT and 23% (5/22) for LPT, and after 6 years, it was 23% (3/13) for OPT and 0% for LPT. Duration/peak of raised C-reactive protein and duration of fever were significantly less for LPT (P < .01)., Conclusion: Our results suggest that LPT is less invasive and may provide better postoperative bowel management compared with OPT.

Background: The aim of this study was to determine the existence and extent of maternal microchimerism in the livers of biliary atresia (BA) patients., Methods: Two series of investigations were performed based on the sex of our subjects. Subjects for series I were men, of which 6 had BA. Livers were analyzed using X and Y chromosome probes and fluorescent in situ hybridization. Subjects for series II were woman. Nine BA cases and their mothers were HLA typed (class I). Daughter livers were also tested for antibodies to maternal and other HLA. Two cases of neonatal hepatitis, 2 cases of Alagille syndrome, and 1 case of Byler syndrome acted as controls., Results: All male BA livers were found to contain a mixture of cells with 1 and 2 X chromosomes (ie, XY or XX). All livers from male controls had only 1 X chromosome (ie, XY). All female BA subjects had varying intensities of antimaternal HLA class I (HLA-A) antibodies in their bile duct epithelium and hepatocytes (strong, 5; mild, 3; weak, 1). The liver from the female control did not display any antimaternal HLA class I antibodies (HLA-Ab)., Conclusion: Our preliminary data appear to show that maternal microchimerism is present within the livers of patients with progressive postnatal type BA. We suggest that BA could in fact be a graft-vs-host disease masquerading as an autoimmune reaction triggered by maternal microchimerism, and we intend to pursue this hypothesis further to clarify the etiology of BA.

Purpose: We adopted antibiotic (Ab) protocols for managing surgical site infections in children and assessed their effectiveness., Methods: We used our protocols on 1313 children between 2004 and 2005. All wounds were monitored for 30 days and classified as clean, clean-contaminated, contaminated, or dirty-infected. Infections were defined as superficial, deep, or organ/space. A retrospective study involving 721 children who had surgery in 2003 was also performed. Chi2 statistical analysis was performed., Results: Postprotocol, all Abs were administered accurately by anesthesiologists and infections developed in only 22 cases (1.7%): 0.2% (clean), 2.6% (clean-contaminated), 5.8% (contaminated), and 20.8% (dirty-infected), respectively; 21 were superficial or deep and 1 was organ/space. Age at surgery and sex did not influence incidence, neither did length of surgery for clean-contaminated, contaminated, and dirty-infected wounds; clean wounds were excluded because all surgery was minor. Overall, incidence of infections was 1.2% for elective surgery and 4.5% for emergency surgery (P < .01). Preprotocol, only 67% had Ab and infections developed in 27 cases (3.7%), which is significantly higher than in postprotocol (P < .01)., Conclusions: Accurate administration of Ab and careful supervision by an infection control team appear to be effective for preventing wound infections in children.

Purpose: The aim of this study is to investigate the optimal treatment for lymphangioma in children by analyzing the effectiveness and complications of treatment modalities., Methods: We reviewed 128 patients with lymphangioma treated at our institution between 1979 and 2005. Periods of treatment were divided arbitrarily into 2 groups: period I, from 1979 to 1988 (n = 53); and period II, from 1989 to 2005 (n = 75). According to radiological appearance, patients were grouped into 4 types: single cystic (SI; n = 23), macrocystic (MA; n = 11), microcystic (MI; n = 69), and cavernous (CA; n = 25)., Results: Sclerotherapy as primary treatment was performed in only 2 patients (3.8%) in period I using bleomycin but increased significantly in period II to 48 patients (64.0%) using OK-432 (P < .01). Nevertheless, primary surgical excision (69/78 patients, 88.5%) was significantly more successful than sclerotherapy (32/50, 64.0%) (P < .01). The following are the locations and types of lesions: head/neck (n = 69; SI, 11; MA, 5; MI, 42; CA, 11), trunk (n = 34; SI, 6; MA, 6; MI, 15; CA, 7), and extremities/other (n = 25; SI, 6; MI, 12; CA, 7). The effectiveness of sclerotherapy in SI, MA, MI, and CA types was 90.9%, 100%, 68.0%, and 10.0%, respectively. Seventeen patients (SI, 1; MI, 8; CA, 8) who received primary sclerotherapy required surgical excision with good outcome. Complications after primary surgical excision were more serious compared with sclerotherapy., Conclusion: Sclerotherapy with OK-432 was not as effective as reported in the literature. We recommend OK-432 injection therapy alone for SI and MA types and surgical excision after pretreatment with OK-432 for MI and CA types.