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To assess mid-/long-term postoperative quality of life (QOL) of esophageal atresia (EA) patients.Modified gastrointestinal quality-of-life index surveys were administered to postoperative EA patients who were at least 7 years old at evaluation to assess three topics about general lifestyle (GL), five topics about EA, and four topics about mental health (MH). For MH, caregivers were also interviewed, but separately. Subjects were divided according to age: children (7-12 years old), teenagers (13-19), and adults (20 and over) and compared according to Foker or Kimura elongation (FK) or bougienage stretching (BS).There were 22 patients evaluated. Responses for GL, EA, and MH did not differ significantly between age groups, but MH responses by caregivers for subjects who were children or teenagers scored significantly lower than responses they made themselves. For primary esophageal elongation technique (PET), age at esophagoesophagostomy was significantly higher in FK. Despite FK scoring 15.1 versus 12.4 for BS during EA evaluation, this difference was not statistically significant.Changes in QOL responses according to age were unremarkable. However, discrepancies in MH indicate that subjects felt better than their caregivers thought. PET did not appear to influence QOL.

Open portoenterostomy (PE) for biliary atresia (BA) is currently more extended (EP) than the original (OP). Typical OP techniques, shallow transection of the biliary remnant and shallow suturing, both lost in EP, were revived as a modified procedure (MP). Postoperative outcomes of EP and MP were compared.Subjects were 55 consecutive BA patients treated by EP (n = 18) or MP (n = 37) at a single center between 2004 and 2021.Mean follow-up duration was: MP: 15.5 years (range 0.1-12.3 years) and EP: 15.5 years (range 0.38-17.1 years). The ratio of jaundice free (JF; total bilirubin ≤ 1.2 mg/dL) subjects was significantly higher in MP (78.4%) versus EP (50%); p 0.05, the incidence of bile lakes at the porta hepatis was significantly higher in MP (7/37: 18.9%) versus EP (0/18: 0%); p 0.05, and Kaplan-Meier analysis showed JF survival with the native liver (JF + SNL) was significantly better in MP (26/37: 70.2%) versus EP (4/18: 22.2%); p 0.05. All other criteria were similar. Of note, time taken to become JF and the incidence of cholangitis were not significantly different.Shallow transection and shallow suturing would appear to influence postoperative outcome. The etiology of bile lake formation in MP requires urgent confirmation.

The levator ani (LA) complex in high-type imperforate anus (H-IA), low-type imperforate anus (L-IA), and Hirschsprung's disease (HD) patients as controls were documented using magnetic resonance imaging (MRI) and compared for symmetry.Mean left:right LA thickness ratio (LA ratio), and deviation of the LA from the pubococcygeal line (PCL; LA angle) were calculated from thin-slice MRI images (axial 2 mm, coronal 2 mm, and sagittal 3 mm) of the puborectalis and pubococcygeus taken parallel to the PCL under sedation in H-IA (n=14), L-IA (n=16), and HD (n=9).MRI scans were performed between January 2018 and June 2021. LA were significantly thinner in H-IA (1.78±0.46 mm) compared with L-IA (2.97±0.55 mm) and controls (2.87±0.32 mm), p0.0001. LA ratio was significantly lower in H-IA (0.71±0.15) compared with L-IA (0.93±0.04), and controls (0.91±0.06), p0.0001. Mean LA-angle was significantly different in H-IA, 10.8° (range 6°-19°), versus L-IA and controls, both zero degrees (range 0°-5°), p0.0001, respectively.LA was confirmed to be significantly asymmetric in H-IA. Because outcome of surgical repair involving a midline incision, such as posterior sagittal anorectoplasty could be impaired, pediatric surgeons are advised to plan surgical intervention for H-IA carefully and appropriately.

Introduction A biopsy protocol for diagnosing Hirschsprung's disease (HD) in children using the anorectal line (ARL). Materials and Methods The ARL was adopted for diagnosing HD in 2016 using two excisional submucosal rectal biopsies performed at different levels, sequentially; the first just above the ARL and the second, further proximal (2-ARL). Currently, only the first-level biopsy is performed (1-ARL) and examined intraoperatively. Management was observation if normoganglionic, pull-through if aganglionic, and a second-level biopsy if hypoganglionic. Hypoganglionosis was considered physiologic if the second-level biopsy was normoganglionic and pathologic if hypoganglionic. Colon caliber change and bowel obstructive symptoms reflect the severity of hypoganglionosis. Results For 2-ARL (n = 54), results were: normoganglionosis (n = 31/54; 57.4%), aganglionosis (n = 19/54; 35.2%), and hypoganglionosis (n = 4/54; 7.4%); physiologic (n = 3/54; 5.6%) and pathologic (n = 1/54; 1.9%). Normoganglionosis and aganglionosis were always duplicated in 2-ARL (kappa = 1.0). For 1-ARL (n = 36), results were: normoganglionosis (n = 17/36; 47.2%), aganglionosis (n = 17/36; 47.2%), and hypoganglionosis (n = 2/36; 5.6%). Second-level biopsies were normoganglionic (physiologic: n = 1) and hypoganglionic (pathologic: n = 1). All normoganglionic cases, except one, resolved conservatively. All aganglionic cases had pull-through with HD confirmed on histopathology. Both pathologic hypoganglionic cases had caliber change and severe obstructive symptoms as definitive indications for pull-through with hypoganglionosis of the entire rectum confirmed on histopathology. Physiologic hypoganglionic cases were observed and currently have regular defecation. Conclusion Because the ARL is an objective functional, neurologic, and anatomic demarcation, normoganglionosis and aganglionosis can be diagnosed accurately with a single excisional biopsy. Only hypoganglionosis requires a second-level biopsy.

Aim: To report the value of adult cadavers for training in minimally invasive surgical (MIS) techniques for pediatric surgery (PS). Materials and Methods: Three teams, each consisting of a board-certified consultant pediatric surgeon (CS), a senior trainee (ST), and a junior trainee (JT), attended a cadaver surgical training (CST) course involving five procedures: thoracoscopic esophagoesophagostomy (TEE), thoracoscopic right lower lobectomy (TRL), laparoscopic fundoplication (LFN), laparoscopic hepaticoduodenostomy (LHD), and laparoscopic ureteroureterostomy (LUU). The same teams also performed LFN on live pigs. Attendees (3 CSs, 3 STs, and 3 JTs) were administered a questionnaire to rate their CST experience according to five criteria (tissue texture, organ size, operative field, "feel," and anatomic relationships) using a 4-point scale with 0 being the worst response. Scores were averaged per procedure per attendee groups and compared. LFN was also compared between a cadaver and a pig. Results: End-point (1): For LFN, cadaver scores were significantly higher than pig scores for anatomic relationships (P = .0001), operative field (P = .0053), and organ size (P = .0481). End-point (2): TRL and LFN were ranked together as being most realistic, followed by TEE and LUU, then LHD. End-point (3): Anatomic relationships and operative field consistently scored highly for all attendee groups. End-point (4): CSs and STs tended to award higher scores than JTs although differences were not statistically significant. Conclusions: CST is a valuable opportunity for PS trainees to experience highly realistic training in minimally invasive surgery. Pig training was inferior. IRB Number: 2019173.

During choledochal cyst (CC) excision, the hepaticojejunostomy anastomosis (HJA) can be performed conventionally (CHJA) or with a Carrel patch (CPA). CPA can increase CHD diameter to 10-13 mm, preventing anastomotic stenosis and intrahepatic bile duct (IHBD) stones but may be at risk for malignant transformation.The medical records of 83 cystic-type CC with CHD ≤ 9 mm followed up for at least 20 years were reviewed retrospectively. Available excised CC specimens (70/83) were re-examined blindly for pre-malignant changes. A questionnaire about suturing narrow lumens was conducted.All 83 had pancreaticobiliary maljunction. Group data were similar. Anastomoses were CPA (n = 43) and CHJA (n = 40). Mean diameter for CPA was 11.4 mm (range: 10-13 mm); for CHJA was 7.4 mm (range: 5-9 mm). Mean follow-up was 27.7 years (range: 20-42). Postoperative anastomotic stenoses were less after CPA: 1/43 (2.3%) versus 5/40 (12.5%) (p = 0.10), but CHJA had significantly more postoperative IHBD stones: 0% versus 4/40 (10.0%) (p 0.05). All IHBD stone patients had anastomotic stenosis. Excised specimens showed no pre-malignant cytology. Lumen diameter ≤ 9 mm was considered challenging by 10/10 surgical trainees and ≤ 7 mm by 16/22 pediatric surgeons.CPA appears to be oncologically safe because of the absence of malignant transformation for at least 20 years.

Purpose To engraft bladder organoids (BO) on de-epithelialized mouse colon using an epithelial replacement technique. Methods BO cultured using bladder specimens from enhanced green fluorescent protein (EGFP) transgenic mice were engrafted to replace proximal colon epithelium stripped from an approximately 1cm long target site in syngeneic wild-type recipient mice (n = 9) by exposure to ethylenediaminetetraacetic acid by infusion and flushing with phosphate buffered saline. Target sites were harvested on postoperative days 2, 7, and 28 for hematoxylin-eosin staining and immunofluorescence. Results Histology on postoperative days 7 and 28 showed BO derived EGFP + cells forming multiple layers on the luminal surface of the colon. Immunohistochemistry showed that EGFP + areas were positive for CK5 and CK14, markers for basal and immature subtype urothelium, respectively, but negative for CA2, a marker for colonic epithelium. Ki67 was detected predominantly in the basal parts of EGFP + areas on post-operative day 7 and day 28. Conclusions This is the first report of successful engraftment of BO in de-epithelialized colon with urothelial tissue reconstituted by actively-proliferating cells. This technique could be developed for augmentation cystoplasty to prevent bladder calculi formation and malignant transformation.

Purpose: Native liver survivors (NLS) after portoenterostomy (PE) for biliary atresia (BA) with normal biomarkers defined as total bilirubin (T-Bil), aspartate aminotransferase (AST)/alanine aminotransferase (ALT) for liver function (LF), cholinesterase (ChE), platelet count (PC), and absence of portal hypertension (PHT) were reviewed to redefine “successful” PE. Methods: 92 post-PE BA patients were classified as NLS-1: normal biomarkers, PHT (-); NLS-2: at least one abnormal biomarker, PHT (-); NLS-3: normal biomarkers, PHT (+); NLS-4: abnormal biomarkers, PHT (+) and reviewed for a maximum 32 years. Results: As of June 2022, 55/92 (59.8%) had received liver transplants and 37/92 (40.2%) were NLS. NLS patients were classified as excellent outcome (EO): NLS-1 (n=10; 27.0%) or non-EO: NLS-2: (n=8; 21.6%), NLS-3: (n=6; 16.2%), and NLS-4: (n=13; 35.1%). Compared with non-EO, EO had PE earlier (50.5 versus 65 days; not significant; p=0.08), significantly earlier onset of symptoms (13 days versus 32 days; p=0.01) and significantly shorter jaundice-clearance (JC; 34.5 days versus 56.0 days; pConclusions: Incidence of “successful” PE or EO is low and correlated with early onset of symptoms and quicker JC.

To assess mid-/long-term quality of life (QOL) of total colonic aganglionosis (TCA) patients.Modified pre-existing QOL assessment tools for general lifestyle (GL), bowel function (BF), and mental health (MH) were administered to postoperative TCA patients from five institutions, who were at least 7 years old to compare Duhamel (with pouch) and Swenson/Soave (without pouch) techniques between children (Ch 7-12 years old), teenagers (Tn 13-19), and adults (Ad 20 and over). For MH, caregivers were also interviewed, but separately. Maximum scores were 12 for GL/MH and 18 for BF.There were 32 subjects. GL and BF scores increased significantly from Ch (GL 4.8 ± 2.5, BF: 11.3 ± 4.6) to Tn (GL 7.8 ± 2.6, BF 16.2 ± 3.0); scores for MH did not change significantly. Mean caregiver MH scores were significantly lower than mean subject MH scores for all age groups (subject scores: 10.1, 10.7, 10.7 versus caregiver scores: 6.8, 7.8, 8.1 for Ch, Tn, Ad, respectively). PT technique/presence of a pouch did not influence the incidence of enterocolitis or QOL scores.MH responses showed subjects felt better than caregivers believed. This discrepancy could cause conflict despite steadily improving GL/BF. QOL was unaffected by PT technique/presence of a pouch.

Purpose: We report the efficacy of pre- and post-operative showering for preventing surgical site infections (SSIs) and urethrocutaneous fistula after hypospadias surgery. Materials and Methods: In 2006, standardised pre- and postoperative showering was introduced for hypospadias patients. Showering involves washing the genitals and groin 2 h preoperatively as well as immediately after the removal of a stent postoperatively. Data from 520 procedures performed on 376 hypospadias patients by a single surgeon from 1996 to 2015 were collected prospectively. The shower (S) group comprised 258 patients (372 procedures) and the nonshower group comprised 118 patients (148 procedures). Management protocols were identical for two groups. Results: Patient demographics were similar. SSIs were significantly less in the S group (0% vs. 2.0%; P < 0.05). The incidence of fistulas was lower in the S group (2.0% vs. 6.3%). The mean duration of follow-up was significantly shorter in the S group (3.6 vs. 12.8 years; P < 0.05) but longer than the mean time taken for complications to develop (0.4 years). Conclusions: Our results suggest that pre- and post-operative showering may contribute to preventing SSIs and fistulas in hypospadias patients.

To determine the outcomes of patients with cystic biliary atresia by correlating the anatomy of the hepatic ducts with the choice of biliary reconstruction surgery.The Kasai hepatoportoenterostomy (Kasai) is the initial surgical procedure offered to most patients with biliary atresia. In contrast, a hepatic-cyst-jejunostomy has been reported to be effective in patients with the cystic form of biliary atresia.We performed an international multicenter retrospective review. Two hundred eighty-seven patients were included, and 33 cases of cystic biliary atresia were identified. Outcomes were the serum total bilirubin level 3 months post-surgery and native liver survival at 2 years of age and were compared between cases who received the Kasai versus hepatic-cyst-jejunostomy in correlation to the anatomy of proximal hepatic ducts. The patients were categorized into 3 anatomical groups: patent intact hepatic ducts (n = 10), patent hypoplastic hepatic ducts (n = 13), and obliterated hepatic ducts (n = 10). All 10 patients with patent intact hepatic duct group underwent hepatic-cyst-jejunostomy, and 9 experienced bile drainage and native liver survival. Among the 13 patients with hypoplastic hepatic ducts, 11 underwent the Kasai procedure, and 9 had bile drainage, whereas 2 underwent hepatic-cyst-jejunostomy, and one survived with the native liver. All of the patients with obliterated hepatic ducts underwent the Kasai procedure; 5 established biliary drainage and survived with the native liver. Of 5 who did not drain, 3 underwent liver transplantation.In patients with cystic biliary atresia, the subset with a connection between cyst and intrahepatic bile ducts via intact proximal hepatic ducts had favorable clinical outcomes following hepatic-cyst-jejunostomy.

Purpose: The high incidence of scarring and vascular compromise after failed hypospadias repair greatly compromises salvage urethroplasty. Pedicled soft tissue reinforcement (STR) is presented for enhancing the potential for success.Methods: The impact of lateral reinforcement of the scarred urethral plate to boost thickness and perfusion at the time of curvature correction (called CHARGE by the authors) and covering the neourethra at the time of urethroplasty (NUC) used as STR during salvage tubularized incised plate urethroplasty performed between 2003 and 2019 were assessed by retrospective review of the medical records of 8 cases of failed hypospadias repair. Results: Types of hypospadias were perineal/penoscrotal (n=7), and mid-shaft (n=1). Indications for salvage surgery included residual penile curvature (n=5), urethral diverticulum >15mm (n=3), urethral dehiscence (n=1), and urethral stricture (n=1). Salvage surgery was single-stage (urethroplasty at the same of curvature correction; n=1) or multi-stage (n=7; two-stage: n=4, three-stage: n=3). All cases had NUC and 4/8 had CHARGE. All salvage repairs were successful with no incidence of postoperative complications after mean follow-up of 3.9 years (range: 1.6-6.4 years) after the last procedure.Conclusion: STR most likely enhanced tissue perfusion/pliability which ensured the success of salvage urethroplasty for failed hypospadias repair.

Highlights • Undifferentiated carcinoma of the liver is reported in a child for the first time. • Neoadjuvant chemotherapy with cisplatin/doxorubicin was outstandingly effective. • After chemotherapy, total excision was possible by resecting segments 4b and 5. • Aggressively malignant, recurrence caused death 9 months after successful surgery. • Neoadjuvant chemotherapy is indicated in aggressive malignancies to enable surgery., Introduction Undifferentiated carcinoma (UC) of the liver has only been reported in three adults in the English language literature and is so rare it has never been reported in a child. Our management is presented to improve knowledge of its treatment. Case presentation A 3-year-old previously well Japanese girl was referred for further assessment/management of an abdominal mass. On examination an obvious right hypocostal mass was visible extending across the midline. Diagnostic imaging identified a 12.5 cm mass on the ventral surface of the liver containing multiple cystic lesions extending along Glisson’s capsule with invasion to the portal vein. Open biopsy eventually led to a diagnosis of poorly differentiated or UC of the liver with embryonal features. Resection of hepatic segments 4b and 5 after a remarkable initial response to cisplatin/doxorubicin that shrank the tumor substantially, separating it from Glisson’s capsule enabled total excision. Surgery was successful and tolerated well with unremarkable postoperative recovery. Unfortunately, ascites due to peritoneal carcinomatosis developed 4 months postoperatively and she died 5 months later. Conclusion The initial impressive response to neoadjuvant chemotherapy and successful surgery was unexpectedly fortuitous but inadequate for controlling such an aggressive malignancy. Our case demonstrates the value of neoadjuvant chemotherapy.

Anorectal malformations (ARM) represent a broad spectrum of patients with different level of the rectum and type of a fistula. Standardized approaches are usually successful, but patients occasionally present with an unusual course of fistula which requires a modified surgical strategy. We present here three male ARM patients with an atypical fistula which did not have connection with the urinary tract, but ran near the fistula. Case 1 has a low-type ARM with a rectoscrotal fistula running deep and partly involved in the corpus spongiosum. Anorectoplasty was performed through an anterior sagittal incision and the anterior wall of the fistula was laid open leaving the posterior wall undetached. Case 2 was diagnosed with an intermediate-type ARM with a long rectoscrotal fistula running near and parallel the urethra. Posterior sagittal anorectoplasty (PSARP) was performed leaving the fistula untouched. Case 3 presented with an intermediate-type ARM with a rectoperineal fistula adherent to the urethra. The patient was treated by PSARP leaving the fistula and part of the muscle coat of the rectum in situ. All the cases were smoothly discharged and no urological complication nor problem associated with the residual fistula was observed at the latest follow-up (17 months-2 years). Preoperative distal colostography with the aid of diverting colostomy was importantly useful for deciding surgical procedure.

Near-infrared spectroscopy (NIRS) was used to monitor intraoperative regional oxygen saturation (rSONIRS sensors were applied to the forehead and flanks for cerebral rSOMean ages at surgery were N: 5.2 ± 8.2 days, MOp: 2.4 ± 2.9 years, and MMI: 3.8 ± 4.3 years. Despite significantly shorter operative times in N (169 ± 94 min; p 0.0001), PD was significantly worse; PD(C-rSO

Aim: To present the use of an additional trocar (AT) in the lower thorax during thoracoscopic pulmonary lobectomy (TPL) in children with congenital pulmonary airway malformation.Methods: For a lower lobe TPL (LL), an AT is inserted in the 10th intercostal space (IS) in the posterior axillary line after trocars for a 5-mm 30° scope, and the surgeon's left and right hands are inserted conventionally in the 6th, 4th, and 8th IS in the anterior axillary line, respectively. For an upper lobe TPL (UL), the AT is inserted in the 9th IS, and trocars are inserted in the 5th, 3rd, and 7th IS, respectively. By switching between trocars (6th↔8th for the scope, 4th↔6th for the left hand, and 8th↔10th for the right hand during LL and 5th↔7th, 3rd↔5th, and 7th↔9th during UL, respectively), vital anatomic landmarks (pulmonary veins, bronchi, and feeding arteries) can be viewed posteriorly. The value of AT was assessed from blood loss, operative time, duration of chest tube insertion, requirement for post-operative analgesia, and incidence of perioperative complications.Results: On comparing AT+ (n = 28) and AT– (n = 27), mean intraoperative blood loss (5.6 vs. 13.0 ml), operative time (3.9 vs. 5.1 h), and duration of chest tube insertion (2.2 vs. 3.4 days) were significantly decreased with AT (p < 0.05, respectively). Differences in post-operative analgesia were not significant. There were three complications requiring conversion to open/mini-thoracotomy: AT– (n = 2; bleeding), AT+: (n = 1; erroneous stapling).Conclusions: An AT and switching facilitated posterior dissection during TPL in children with congenital pulmonary airway malformation enhancing safety and efficiency.

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...

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.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.LapCC was performed in 37 age/weight/HJA diameter matched children. Scores for DOD (p0.001) 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.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.III.

In children with choledochal cyst (CC), the diameter at the hepaticojejunostomy anastomosis (HJA) is usually less than 10 mm. The da Vinci surgical system (dV) has been used successfully for treating CC in children, but lack of haptic feedback can hinder suturing at times. We describe a simple maneuver that allows dV-assisted HJA (dV-HJA) to be completed successfully without haptic feedback. The maneuver involves placing the final three 5/0 or 6/0 absorbable sutures between the incision in the Roux-loop jejunum and the common hepatic duct for closing the anterior wall, without tying any of them. Only after all sutures have been placed, they are tied. The tie should never be performed before all the sutures have been placed, because if any one of the last 3 sutures is tied prior the others being placed, the surgeon will not be able to “see” where to place the subsequent suture. Even the smallest HJA should be possible to be completed confidently using this maneuver. We treated 13 consecutive children with CC during a recent 3-year period using this maneuver during dV-HJA. Mean age at surgery: 5.8 years (range 1.8–11.2 years); mean weight at surgery: 18.5 kg (range 9.4–35.6 kg). Mean HJA diameter was 7.9 ± 3.2 mm (range 4–15 mm). All HJA were performed successfully with no reports of anastomotic leakage or anastomotic stenosis after a mean follow-up of 22 months (range 4–34 months). The described maneuver provides the surgeon with visual cues to complete suturing successfully and confidently when haptic feedback is lacking.

To document the recovery of bowel function (BF) in children after transperitoneal (TP) or retroperitoneal (RP) laparoscopic pyeloplasty. Data were obtained retrospectively from four centers between 2008 and 2019 for TP (n = 51) and RP (n = 58). Each surgeon chose which technique to perform. Subject demographics were not significantly different. Differences in operative times were not significant (RP: 241 min versus TP: 225 min). Mean duration/requirement for postoperative epidural/intravenous analgesia were not significantly different (TP: 1.4 days versus RP: 1.3 days) and (TP: 66.7% versus RP: 67.2%), respectively. Postoperative nasogastric (NG) intubation was more common in RP (TP: 19.6% versus RP: 44.8%; p

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 Conclusions Undermining the edges of the U-shaped incision during mUIT would appear to prevent post-UPC. Level of evidence Level III.

Aim: In children, the diameter at the hepaticojejunostomy anastomosis (HJA) can range from 10 mm to less than 5 mm, irrespective of the type of choledochal cyst (CC). We developed a maneuv...

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.

We describe our modified retroperitoneal laparoscopic pyeloplasty (MRLP) and assess the incidence of anastomotic stricture and torsion of the ureter in the mid-term. We reviewed 12 patients with ureteropelvic junction obstruction (UPJO) we treated with MRLP between 2013 and 2018. MRLP involves: identifying the lowest point of the renal pelvis and the lateral aspect of the ureter to ensure correct orientation of the anastomosis; placing the first suture between the lower edge of the incised pelvis and the distal end of the spatulated ureter to avoid crushing tissue that will be anastomosed; and complete excision of the narrow segment. All MRLP were completed successfully without conversion. UPJO was resolved completely, both clinically and radiologically, in all cases. Mean age at surgery was 6.8 years (range: 1.7–8.8 years); mean operative time was 212.1 min (range: 170–333 min); mean estimated blood loss was 4.2 mL (range: 2–8 mL); mean follow-up was 36.7 months (range: 6–65 months); mean post-operative differential renal function on the affected side was 53.1 ± 13.7% (range: 37.0–87.2%), increased from 44.9 ± 16.0% (range: 27.0–84.3%), pre-operatively (p = 0.02). All remain completely cured after mean follow-up of 36.7 ± 19.7 months (range: 6–65). Our MRLP is safe and effective despite limited retroperitoneal space.

Refinement of organoid technology is important for studying physiology and disease of the intestine. We aimed to optimize defined serum-free conditions for human infant small intestinal (SI) organoid culture with predetermined doses of Wnt3a and Rspo1 from surgical specimens. We further assessed whether intestinal specimens could be stored before use as a source of organoids. Different doses of Wnt3a and Rspo1 in a serum-free medium were tested to establish a condition in which surgically resected SI cells grew as organoids over multiple passages. The expression of marker genes for stem and differentiated cells was assessed by quantitative polymerase chain reaction. We also investigated the organoid-forming efficiency of cells in degenerating intestines stored at 4 °C for various intervals post-resection. We determined the doses of Wnt3a and Rspo1 required for the continuous growth of infant SI organoids with multi-differentiation potential. We revealed that, despite the time-dependent loss of stem cells, tissues stored for up to 2 days preserved cells capable of generating amplifiable organoids. SI cells can be grown as organoids under defined conditions. This could provide a reproducible and customizable method of using surgical specimens for the study of intestinal maturation and their relevance to pediatric diseases.

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

It has long been considered surgical dogma that the length of the shared common wall (CW) between a fistula and the urethra in males with anorectal malformation (ARM) and rectourethral bulbar fistula (RUBF) is considerably longer than in males with ARM and rectourethral prostatic fistula (RUPF). This belief has led surgeons who perform laparoscopic-assisted anorectoplasty (LAARP) for RUPF to avoid LAARP for RUBF for risk of potential injury to the urethra or incomplete removal of the fistula. In this study, we compared CW between RUBF and RUPF using distal colostography (DCG) and direct intraoperative measurements. DCG of rectourethral fistula patients (n = 63; RUBF: n = 44; RUPF: n = 19) were used to measure CW retrospectively. Results were expressed as a ratio of the height of L4; i.e., CW:L4. If less than 0.7, the CW was classified as being “short”; if 0.71–1.4, as being “medium”; and if greater than 1.41, as being “long”. CW that could not be measured was classified as indeterminate. 24 of these patients also had CW measured intraoperatively during LAARP as previously described. The results obtained using both techniques were also compared. Surprisingly, CW:L4 in RUBF patients was short in 47.7%, medium in 27.3%, long in 20.5%, and indeterminate in 4.5% on DCG, equivalent to mean lengths of 7 mm, 8.5 mm, and 10.3 mm obtained using direct intraoperative measurement for short, medium, and long CW:L4 categories, respectively. CW:L4 in RUPF was short in 73.6%, medium in 10.5%, and long in 5.2% on DCG, while mean intraoperative measurements were 5 mm, 7 mm, and 10 mm, respectively. Differences in CW measured intraoperatively were not significantly different between RUBF and RUPF (p = NS). From our findings, 47.7% of CWs in RUBF were short using two independent methods, with only 20.5% being long. Thus, LAARP should be considered actively for treating selected RUBF cases and not be excluded on the basis of CW length.

Colon vaginoplasty (CV) is often performed for cloacal malformation (CM). We used mice to study the vitality of spermatozoa after contact with colonic mucosa as a factor contributing to infertility. Spermatozoa isolated from the epididymides of C57BL/6J male mice (n = 23) were syringed directly into the vaginas (Vag-group) or colons (Colo-group) of female mice (n = 45). Vitality was determined by assessing motility using computer-assisted sperm analysis, viability by staining with SYBR-14 and propidium iodide, and fertility by in vitro fertilization, prior to deposition, and at 5, 10, 30, and 60 min after deposition. Motility was significantly decreased in Colo only at 10 and 60 min. Viability of Colo spermatozoa was significant at all assessment times, except at 10 min. Normal fertilization was observed with all Vag spermatozoa, but with Colo, there was arrest of embryo development with spermatozoa collected at 5 and 10 min, and no fertilization with spermatozoa collected at 30 and 60 min. The vitality of spermatozoa is compromised by contact with colonic mucosa which could contribute to infertility in CM after CV, because their ovaries and fallopian tubes are considered to be normal.

A 10-year-old Japanese boy presented the day after a bicycle handlebar injury with mild abdominal pain. Computed tomography (CT) confirmed pancreatic transection and possible main pancreatic duct (MPD) injury because of a fluid collection around the pancreas. Conservative management (fasting, antibiotics, total parental nutrition, protease inhibitor) was initially successful, but an oral challenge with plain water on day 23 caused a relapse. CT identified a pancreatic pseudocyst. Endoscopic trans-papillary drainage was attempted but failed because the MPD was completely obstructed. Endoscopic ultrasound (EUS)-guided drainage of the pseudocyst with a lumen apposing metal stent (LAMS) was performed on day 29 via the stomach. On day 36, EUS-guided pancreatic drainage (EUS-PD) with a 5Fr plastic stent was performed via the stomach with rapid improvement in symptoms and elevated enzyme levels. Repeat oral challenge on day 39 was successful, the LAMS was removed on day 59 without recurrence. He was discharged well on day 69 and remains asymptomatic 20 months after treatment. Our case highlights the potential of minimally invasive endoscopic procedures as options for the non-surgical management of complete MPD disruption in a child. However, further research to develop dedicated pediatric endoscopic devices is required.

Aim: To assess nonoperative management (NOM) of uncomplicated appendicitis (UC-appy) in children to determine factors influencing prognosis and review the literature. Materials and Methods: All UC-...

Bacterial overgrowth commonly occurs and favors bacterial translocation in short bowel syndrome (SBS). Glucagon-like peptide-2 (GLP-2) is effective for treating SBS, but is rapidly inactivated by dipeptidyl peptidase IV (DPP4). DPP4 inhibitor (DPP4I) is known to be effective for treating SBS. Here, we investigated cell junction protein function following DPP4I administration in a mouse model of SBS. Mice were divided into four groups: naive (n = 5), naive + DPP4I (n = 6), control (n = 6), and DPP4I (n = 5). All control and DPP4I mice had 50% of their proximal small bowel resected. DPP4I or normal saline was administered orally twice daily from days 1–7 postoperatively. The functions of cell junction proteins were assessed by RT-PCR and immunohistochemistry. Body weights and blood glucose levels were recorded. E-Cadherin was significantly higher in the DPP4I group than in the control group. E-Cadherin, occludin, and claudin-4 were significantly higher in the naive group than in the control group. Positive staining for E-cadherin and occludin varied widely between the control and DPP4I groups. Up-regulation of E-cadherin and occludin by DPP4I may be correlated with the anti-inflammatory action of DPP4I. Therefore, DPP4I may reduce bacterial translocation in SBS.

We compared robotic hepaticojejunostomy anastomosis (RHJA) with laparoscopic hepaticojejunostomy anastomosis (LHJA) in children undergoing complete excision of choledochal cyst. Difficulty of suturing (DOS) during anastomosis was scored blindly, from intraoperative video recordings, using: 5 = impossible; 4 = difficult; 3 = tedious; 2 = slow; and 1 = easy. A panel of fiveindependent surgeons was also asked to compare RHJA with matched LHJA and score + 1 if RHJA appeared superior to LHJA, 0 if RHJA appeared equivalent to LHJA, and − 1 if RHJA appeared inferior to LHJA. RHJA (n = 10) was performed between 2017 and 2019; LHJA (n = 27) was performed between 2009 and 2018. LHJA cases were matched for age, weight, and anastomosis diameter to RHJA cases. Complete excision was performed laparoscopically in both groups. DOS was lower in RHJA with less variance. The panel all scored RHJA as + 1. Total anastomotic time (TAT) and TAT per suture were significantly shorter for RHJA. Times taken to ambulate and for return of bowel sounds postoperatively were significantly shorter for RHJA. There was one anastomotic leak with LHJA (3.7%) and no anastomotic complications with RHJA. RHJA is a more stable anastomosis that can be performed quicker, and thus, would appear to be superior to LHJA.

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.

We investigated the relationship between the affected lobe and symptom onset in prenatally diagnosed congenital pulmonary airway malformation (CPAM). 53 CPAM patients diagnosed prenatally were reviewed retrospectively by creating 2 groups according to symptom onset. Group Sneo: (symptomatic during the neonatal period; n = 13) and group S > neo: (symptomatic after the neonatal period; n = 40) were compared for type of CPAM, affected lobes, types of symptoms/infections, treatment, duration of follow-up, and histopathology. Requirement for surgery (Sx) was then used to create three subgroups: Sneo + Sx, S > neo + Sx, and Sx−. Some cases had multiple affected lobes. In Sneo, symptoms developed in 55.6%, 50.0%, 0%, 0%, and 36.8% of right upper lobes (RUL), right middle lobes (RML), right lower lobes (RLL), left upper lobes (LUL), and left lower lobes (LLL) diagnosed with CPAM, prenatally. In S > neo, symptoms developed in 0%, 0%, 6.3%, 55.6%, and 33.3% of RUL, RML, RLL, LUL, and LLL diagnosed with CPAM, prenatally. In prenatally diagnosed CPAM, RUL and RML lesions are more likely to become symptomatic in neonates, and LUL lesions in infants. Surgery is recommended before the onset of respiratory infections after 1 year of age.

To review the surgical treatment of hypospadias (HP) associated with disorders of sex development (DSD). HP cases were assessed for DSD by gross examination for atypical external genitalia, and assessment of hormone levels and karyotype. There were 58 HP cases with concomitant DSD treated between 1999 and 2017. DSD classification, type of HP, sex assignment, hormonal abnormality, surgical strategy, and post-urethroplasty complications (post-UPC) were reviewed. DSD were sex chromosome abnormalities (n = 4), 46,XY (n = 51), 46,XX (n = 1), and 47,XY + 21 (n = 2). HP was perineal: (n = 26), scrotal: (n = 16), penoscrotal: (n = 15), and midshaft: (n = 1); repair was primary (n = 6) or staged (n = 52). Mean age at final urethroplasty (UP) was 4.12 ± 0.21 years; all cases had soft tissue interposition at UP. At mean follow-up 5.16 ± 0.56 years after final UP, observed post-UPC (n = 8; 13.8%) were urethral stenosis (n = 3), urethral diverticulum (n = 2), urethrocutaneous fistula (n = 2), and curvature (n = 1). Mean onset of post-UPC was 1.24 ± 0.77 years (range 0.1–6.3). The second half of our cases (n = 29; treated 2015 ~) had significantly less post-UPC (0/29; 0%) than the first half (8/29; 27.6%) (p = 0.0075). Although UP for HP + DSD was formidably challenging, we achieved a significant decrease in post-UPC through a combination of surgical techniques and experience.