Optical Trocar Access for Retroperitoneal Robotic-Assisted Pyeloplasty in Children with Ureteropelvic Junction Obstruction.

Purpose: Retroperitoneal robotic-assisted pyeloplasty (ret-RAP) for ureteropelvic junction obstruction (UPJO) requires a larger retroperitoneal space (RS) to maintain specified distances between robotic (da Vinci) trocars and between trocars and the region of interest. A modified closed technique (MOT) and conventional closed technique (COT) were compared for creating an adequate RS with optical trocars. Methods: RS access in children with UPJO who underwent ret-RAP (n = 30) was MOT (n = 15) and COT (n = 15). All patients were positioned laterally. For MOT, a 5 mm optical trocar was inserted at the angle formed between the 12th rib and the erector spinae muscles. As the trocar was advanced under direct vision, it pierced the superficial subcutaneous layer, Scarpa's fascia, lumbar fascia, internal/external oblique and transversus abdominalis muscles, and the posterior renal fascia. Once in the RS, the tip of the scope was used for blunt dissection of perirenal fat, the tip was withdrawn until it was outside the perirenal fascia, and used to dissect toward the anterior abdomen in the pararenal fat layer. Results: Ages and weights at ret-RAP were similar (MOT: 5.6 ± 1.8 years versus COT: 7.8 ± 4.6 years; MOT: 20.6 ± 10.1 kg versus COT: 27.6 ± 13.9 kg). Times for RS access were similar (MOT: 1.6 ± 0.5 minutes versus COT: 1.9 ± 0.7 minutes), but RS expansion was significantly quicker in MOT (32.3 ± 8.7 minutes versus 52.0 ± 15.1 minutes; P < .001). Peritoneal injury caused carbon dioxide leakage in 4 of 15 COT cases and 0 of 15 MOT cases. Conclusion: RS expansion with MOT was safer because there were no peritoneal injuries and MOT was quicker than COT.

Retroperitoneoscopic Simple Nondismembered Pyeloplasty with Da Vinci Si Assistance to Prevent Alignment Shift.

Aims: Retroperitoneoscopic simple nondismembered pyeloplasty (SNDP) with da Vinci Si assistance was developed because of a possible risk for alignment shift after retroperitoneoscopic diamond-shaped bypass pyeloplasty (Diamond-Bypass; DP). Outcomes of SNDP and DP were compared. Materials and Methods: For SNDP, a small longitudinal incision is made on the border of the dilated pelvis and narrowed ureter at the ureteropelvic junction (UPJ). Extending this incision toward the pelvis allows identification of mucosa while maintaining the integrity of surrounding tissues that are so thin and fragile that they will not influence lumen alignment. Data for DP were obtained from a previously published article. Results: For SNDP (n = 3), mean age at surgery was 2.67 years (range: 1-4), mean operative time was 176 minutes. Mean postoperative Society of Fetal Urology (SFU) grades for hydronephrosis were 1.2, 0.7, and 0.6, 1, 2, and 3 months after stent removal, respectively. Postoperative diethylenetriaminepentaacetic acid (DTPA) was normal (n = 3). For DP (n = 5) mean age at surgery was 4.3 years (range: 1-14), mean operative time was 189 minutes. Mean postoperative SFU grades were 2.8, 2.2, and 1.6, respectively. Postoperative DTPA was normal (n = 4) and delayed (n = 1). All SNDP and DP were asymptomatic by 3 months after stent removal. Conclusion: Both SNDP and DP have favorable outcomes. If the UPJ is located at the lowest end of the renal pelvis, SNDP may improve hydronephrosis more quickly.

Pulmonary artery diameter ratio as a prognostic indicator of congenital diaphragmatic hernia.

Background: Following on from an earlier study published in 2008 about left pulmonary artery (LPA) flow measured on serial echocardiography being strongly prognostic in left-sided congenital diaphragmatic hernia (CDH) and the ratio of LPA to right pulmonary artery (RPA) diameters being a simple and reliable indicator for commencing nitric oxide (NO) therapy, the ratio of LPA:RPA diameters (PA ratio or PAR) was hypothesized to possibly reflect cardiopulmonary stresses accompanying CDH better. Methods: Subjects with isolated left-sided CDH treated between 2007 and 2020 at a single pediatric surgical center were recruited and classified according to survival. Data obtained retrospectively for subject demographics, clinical course, LPA/RPA diameters, and PAR were compared between survivors and non-survivors. The value of PAR for optimizing the prognostic value of PA diameter data in CDH were analyzed with receiver operating characteristic (ROC) curve analysis. Results: Of 65 subjects, there were 54 survivors (82.3%) and 11 non-survivors (17.7%); 7 of 11 non-survivors died before surgical repair could be performed. Mean PAR for survivors (0.851±0.152) was significantly higher than for non-survivors (0.672±0.108) (p=0.0003). Mean PAR for non-survivors was not affected by surgical repair. Characteristics of survivors were: LPA ≥2 mm (n=52 of 54; mean PAR=0.866±0.146) and RPA ≥3 mm (n=46 of 54; mean PAR=0.857±0.152). Non-survivors with similar LPA and RPA diameters to survivors had significantly lower mean PAR. ROC curve cut-off for PAR was 0.762. Subjects with high PAR (≥0.762) required high-frequency oscillatory ventilation/NO less than subjects with low PAR (<0.762) (p=0.0244 and p=0.0485, respectively) and subjects with high PAR stabilized significantly earlier than subjects with low PAR (1.71±0.68 days vs 3.20±0.87 days) (p<0.0001). Conclusions: PAR would appear to be strongly correlated with clinical outcome in CDH and be useful for planning management of cardiopulmonary instability in CDH.

Advantages of Urethroplasty Terminating at the Corona for Severe Hypospadias: Complications, Uroflowmetry, and Esthetics.

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.

A Pilot Study to Determine the Role of Spatulating the Ureter During Pyeloplasty in Children for Ureteropelvic Junction Obstruction in the Robotic Era.

Purpose: Spatulation during ureteropelvic junction obstruction repair was evaluated in children treated by robot-assisted retroperitoneal pyeloplasty anastomosis (RRPA). Methods: Intraoperative video recordings (IVRs) of RRPA (n = 22 ureters) performed at a single institute between 2018 and 2022 were reviewed blindly by 5 independent surgeons for perceived difficulty of suturing (DOS; 5 = impossible; 4 = difficult; 3 = tedious; 2 = slow; 1 = easy) and spatulation ranking as superior (+1), inferior (-1), or unnecessary (0). The retroperitoneal space was accessed in the lateral decubitus position using a closed technique under direct vision to avoid air leakage and subcutaneous emphysema. All subjects had a Double-J stent (4.7F) placed. Results: Subjects had similar demographics and preoperative ureter diameters. IVRs were RRPA with spatulation of the ureter on the lateral side (RRPA +SP) (n = 13) and RRPA without spatulation of the ureter (RRPA -SP) (n = 9). Overall DOS scores and coefficients of variation for time taken to place one suture were similar. Total anastomotic time was significantly shorter for RRPA -SP; 67.9 ± 8.4 minutes versus 57.9 ± 9.2 minutes, P = .01. Overall spatulation ranking was 0. Postoperative scanning showed improved drainage in 12 of 13 (92%) in RRPA +SP and 8 of 9 (88%) in RRPA -SP; differences were not significant. One anastomotic stricture in RRPA -SP required open repair. Conclusions: RRPA was quicker and more precise without spatulation. Outcomes of scanning 1 year after RRPA were similar for RRPA -SP and RRPA +SP.

Premalignant/malignant histology in excised choledochal cyst specimens from children. Experience and literature review.

BACKGROUND: Dysplasia, carcinoma in situ, and other malignant transformation or premalignant/malignant histopathology (PMMH) seem uncommon in pediatric choledochal cyst (CC). A literature review and the authors' experience are presented. METHODS: All reports about PMMH in CC patients 15 years old or younger published in English and all cases of PMMH in specimens excised from CC patients 15 years old or younger by the authors were reviewed. RESULTS: Of 20 published reports, PMMH was adenocarcinoma (n = 4), sarcoma (n = 4), and dysplasia (n = 12). Treatment for malignancies was primary pancreaticoduodenectomy (PD; n = 2) or cyst excision/hepaticojejunostomy (Ex/HJ; n = 6). Outcomes at the time of writing for malignancies: 2 deaths, 4 survivors after follow-up of 2 years, and 2 lost to follow-up. No dysplasia case has undergone malignant transformation. The authors have experienced 7 cases of PMMH; adenocarcinoma in situ (AIS; n = 1) and dysplasia (n = 6). CONCLUSIONS: The present study identified the youngest cases of AIS and dysplasia from specimens excised when they were 3 years old and 4 months old, respectively. Both are published for the first time as evidence that PMMH can complicate CC in young patients. Long-term protocolized postoperative follow-up is mandatory when PMMH is diagnosed in pediatric CC.

Comparison of robotic assistance and laparoscopy for pediatric choledochal cyst: advantages of robotic assistance.

AIM: Surgery for pediatric choledochal cyst (CC), complete excision (CE), and Roux-en-Y hepaticojejunostomy anastomosis (HJA) can be performed using laparoscopy (Lap), robotic-assistance (Rob; da Vinci Xi/Si), or both (Lap/Rob). METHODS: Lap was used exclusively between 2009 and 2021 (n = 31) and Rob was introduced in 2017 (n = 23). All subjects were matched for age, weight, BMI, and episodes of preoperative pancreatitis. For Rob, the first 15/23 were Lap-CE/Rob-HJA and the last 8/23 were Rob-CE/Rob-HJA. RESULTS: Total anastomotic time (TAT), TAT per suture during HJA, and time taken for dissection during CE were significantly shorter with less variance for Rob, although overall operative times were similar. Serum amylase on postoperative days 3, 5, and 7 were significantly higher for Lap. Times taken to ambulate, for return of bowel sounds, and discharge home were all significantly shorter for Rob. All postoperative complications occurred after Lap; HJA leak (n = 1; 3.2%), HJA stricture (n = 1; 3.2%), both treated by open re-HJA; and pancreatic fistula (n = 6; 19%), all treated conservatively. CONCLUSION: Dissection and recovery were faster with Rob while overcoming Lap-associated shortcomings to prevent complications associated with suturing. Both CE and HJA were safer and more reliable with Rob, a reflection of Rob's superiority.

Prospective evaluation of common hepatic duct histopathology at the time of choledochal cyst excision ranging from children to adults.

PURPOSE: To evaluate common hepatic duct just distal to the HE anastomosis (d-CHD) prospectively for mucosal damage, inflammation, fibrosis, dysplasia, carcinoma in situ, malignant transformation, effects of serum amylase, and symptoms at presentation in CC cases ranging from children to adults. METHODS: Cross-sections of d-CHD obtained at cyst excision 2018-2023 from 65 CC patients; 40 children (< 15 years old), 25 adults (≥ 15) were examined with hematoxylin and eosin, Ki-67, S100P, IMP3, p53, and Masson's trichrome to determine an inflammation score (IS), fibrosis score (FS), and damaged mucosa rate (DMR; damaged mucosa expressed as a percentage of the internal circumference). RESULTS: Mean age at cyst excision ("age") was 18.2 years (range: 3 months-74 years). Significant inverse correlations were found for age and DMR (p = 0.002), age and IS (p = 0.011), and age and Ki-67 (p = 0.01). FS did not correlate with age (p = 0.32) despite significantly increased IS in children. Dysplasia was identified in a 4-month-old girl with cystic CC. Serum amylase was elevated in high DMR subjects. CONCLUSIONS: High DMR, high IS, and evidence of dysplasia in pediatric CC suggest children are at risk for serious sequelae best managed by precise histopathology, protocolized follow-up, and awareness that premalignant histopathology can arise in infancy.

Prednisolone administration monitored by postoperative stool color achieves high jaundice clearance after laparoscopic portoenterostomy for biliary atresia.

PURPOSE: Stool color (SC) for monitoring prednisolone use in biliary atresia (BA) patients after laparoscopic portoenterostomy (LPE) was reviewed. METHODS: Subjects were 47 post-LPE BA patients given a reducing dose course of intravenous prednisolone. The course started at 4 mg/kg/day and gradually reduced, ultimately reaching a final total dose (TD) of 31.5 mg/kg. Normal SC indicated a course could progress until finished and was repeated until jaundice clearance (JC) was achieved. Abnormal SC persisting for two consecutive courses was the absolute indication for redo or liver transplantation (LTx). RESULTS: JC was achieved in 38/47 (80.9%) LPE cases and 4/6 redos to give an overall JC rate (JCR) of 42/47 (89.4%). Outcomes after one course (n = 5; JCR: 80.0%; median TD: 30.0 mg/kg, interquartile range [IQR: 26.0-31.5]), two courses (n = 10; JCR: 90.0%; median TD: 62.5 mg/kg [IQR: 60.8-66.0]), three courses (n = 13; JCR: 92.3%; median TD: 90.0 mg/kg [IQR: 86.0-90.0]), four courses (n = 10; JCR: 80.0%; median TD: 120.0 mg/kg [IQR: 116.7-123.3]), five courses (n = 7; JCR: 100%; median TD: 156.0 mg/kg [IQR: 154.3-157.5]), six courses (n = 1; JCR: 100%; TD: 189.0 mg/kg), ten courses (n = 1; JCR: 100%; TD: 308 mg/kg). CONCLUSION: Indications for repeat prednisolone and timing of redo/LTx based on SC monitoring appeared effective based on high JCR and successful redo/LTx. LEVELS OF EVIDENCE: III.

Comparative assessment of fully laparoscopic Duhamel-Z with minimal rectorectal dissection vs. laparoscopy-assisted Duhamel-Z with blunt manual rectorectal dissection for total colonic aganglionosis.

Aims: Early postoperative outcome (EPO) was compared between fully laparoscopic Duhamel-Z (F-Dz) and laparoscopy-assisted Duhamel-Z (A-Dz) anastomoses performed for total colonic aganglionosis (TCA). Methods: EPO was assessed quarterly for the first year after F-Dz/A-Dz using a continence evaluation score (CES) based on stool frequency (motions/day) and stool consistency (0 = liquid, 1 = soft, 2 = formed), presence of anal erosion (0 = severe, 1 = moderate, 2 = mild), and incidence of enterocolitis.Surgical technique involved taking the ileostomy down, dissecting the colon laparoscopically, and preparing the pull-through ileum through the stoma wound. In F-Dz (n = 3), a working port (SILS trocar) was inserted, and laparoscopic retrorectal dissection with forceps used to create a retrorectal tunnel from the peritoneal reflection extending downward as narrow as possible along the posterior wall of the rectum to prevent lateral nerve injury and preserve vascularity. After completing the tunnel, the ileum was pulled-through from an incision on the anorectal line and a Z-shaped ileorectal side-to-side anastomosis performed without a blind pouch. In A-Dz (n = 11), the retrorectal pull-through route was created through a Pfannenstiel incision using blunt manual (finger) dissection along the anterior surface of the sacrum. Results: Subject backgrounds were similar. Mean quarterly data were: frequency (F-Dz: 4.67, 4.67, 4.67, 3.33) vs. (A-Dz: 7.27, 7.09, 6.18, 5.36) p < .05; consistency (F-Dz: 0.33, 0.67, 0.67, 0.67) vs. (A-Dz: 0.27, 0.45, 0.70, 0.73) p = ns; anal erosion (F-Dz: 0.33, 0.33, 0.33, 0.67) vs. (A-Dz: 0.18, 0.36, 0.45, 0.64) p = ns; and enterocolitis (F-Dz: 1 episode in 1/3 cases or 33.3%) vs. (A-Dz: 7 episodes in 6/11 cases or 54.5%) p = ns. Conclusions: Overall, EPO after F-Dz was better than after A-Dz.

Remote cadaveric minimally invasive surgical training.

Objective: The aim of the study is to discuss the efficacy of live vs. remote cadaver surgical training (CST) for minimally invasive surgery (MIS). Methods: A cohort of 30 interns in their first and second years of training were divided into three groups: live observers (n = 12), live participants (n = 6), and remote observers: (n = 12). The interns had the opportunity to either observe or actively participate in two different surgical procedures, namely, laparoscopic lower anterior resection, performed by a colorectal surgical team, and laparoscopic fundoplication, performed by a pediatric surgical team. The procedures were conducted either at a base center or at a remote center affiliated with the institute. Some of the interns interacted directly with the surgical teams at the base center, and others interacted indirectly with the surgical teams from the remote center. All interns were administered questionnaires before and after completion of the CST in order to assess their understanding of various aspects related to the operating room layout/instruments (called "design"), accessing the surgical field (called "field"), understanding of anatomic relations (called "anatomy"), their skill of dissection (called "dissection"), ability to resolve procedural/technical problems (called "troubleshooting"), and their skill in planning surgery (called "planning") according to their confidence to operate using the following scale: 1 = not confident to operate independently; 4 = confident to operate with a more senior trainee; 7 = confident to operate with a peer; and 10 = confident to operate with a less experienced trainee. A p < 0.05 was considered statistically significant. Results: All scores improved after CST at both the base and remote centers. The following significant increases were observed: for remote observers: "field" (2.67→4.92; p < .01), "anatomy" (3.58→5.75; p < .01), "dissection" (3.08→4.33; p = .01), and "planning" (3.08→4.33; p < .01); for live observers: "design" (3.75→6.17; p < .01), "field" (2.83→5.17; p < .01), "anatomy" (3.67→5.58; p < .01), "dissection" (3.17→4.58; p < .01), "troubleshooting" (2.33→3.67; p < .01), and "planning" (2.92→4.25; p < .01); and for live participants: "design" (3.83→6.33; p = .02), "field" (2.83→6.83; p < .01), "anatomy" (3.67→5.67; p < .01), "dissection" (2.83→6.17; p < .01), "troubleshooting" (2.17→4.17; p < .01), and "planning" (2.83→4.67; p < .01). Understanding of "design" improved significantly after CST in live observers compared with remote observers (p < .01). Understanding of "field and "dissection" improved significantly after CST in live participants compared with live observers (p = .01, p = .03, respectively). Out of the 12 remote observers, 10 participants (83.3%) reported that interacting with surgical teams was easy because they were not on-site. Conclusions: Although all the responses were subjective and the respondents were aware that observation was inferior to hands-on experience, the results from both centers were equivalent, suggesting that remote learning could potentially be viable when resources are limited.

Upper Ureteral Reconstruction with a Tapered Descending Colon after Failed Pyeloplasties in a 13-Year-Old Boy.

An 11-year-old boy was referred for further management of a 6-cm-long grossly stenosed ureter following two failed left ureteropelvic junction (UPJ) obstruction repairs elsewhere. A tapered segment of the descending colon (TDC) was used successfully for ureteral reconstruction. The UPJ was exposed through a left flank incision. The stenosed segment was excised; both ends appeared severely inflamed and thickened. Tissue interposition was required and ureteroplasty with a TDC was performed by incising the peritoneum adjacent to the excised ureter to mobilize the descending colon to the retroperitoneal space. To prepare the TDC, an 8-cm segment of the colon with intact blood vessels was isolated, tapered, and sutured into a funnel shape using a 14-Fr catheter as a temporary stent. After colocolostomy, the colon was returned to the abdominal cavity, the peritoneum was closed carefully to prevent vascular compromise, and the TDC was anastomosed to the ureter and renal calyx with interrupted absorbable sutures. A double J stent (DJS) and percutaneous nephrostomy tube were placed. Postoperative recovery was uneventful. The DJS was removed on day 50 after confirming smooth urine flow through both the ureter-TDC and calyx-TDC anastomoses. Diuretic renography performed 68 days postoperatively was unobstructed. The patient is currently well after 12 months follow-up. This would appear to be the first report of a TDC being used to create a funnel-shaped segment to reconstruct a long, grossly stenosed ureter. The TDC is simpler than the re-tubularizing colon but requires monitoring for postoperative mucus-related complications and malignant transformation.

Retroperitoneoscopic Pyeloplasty for Ureteropelvic Junction Obstruction in Children: Value of Robotic Assistance.

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.

Robot-Assisted Retroperitoneoscopic Diamond Bypass Pyeloplasty.

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.

Biopsy Diagnosis of Hirschsprung's Disease Using a Single Excisional Biopsy Based on the Anorectal Line.

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.

Successful all robotic-assisted excision of highly malignant mediastinal neuroblastoma in a toddler: A case report.

An otherwise well 28-month-old girl presented with fever/left thigh pain. Computed tomography identified a 7 cm right posterior mediastinal tumor extending to the paravertebral and intercostal spaces with multiple bone and bone marrow metastases on bone scintigraphy. Thoracoscopic biopsy diagnosed MYCN non-amplified neuroblastoma. Chemotherapy shrank the tumor to 5 cm by 35 months of age. Robotic-assisted resection was chosen because the patient was large enough and public health insurance coverage was available. At surgery, the tumor was well-demarcated by chemotherapy and dissection posteriorly from the ribs/intercostal spaces and medially from the paravertebral space and azygos vein was facilitated by superior visualization/instrument articulation. The capsule of the resected specimen was intact on histopathology, confirming complete tumor resection. Despite minimum distance specifications between arms, trocars, and target sites with robotic assistance, excision was safe without instrument collisions. Robotic assistance should be actively considered for pediatric malignant mediastinal tumor provided the thorax is of adequate size.

Intraoperative Bronchoscopic Inspection Facilitates Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula.

Aim: The value of intraoperative bronchoscopic inspection (IBI) for accurate confirmation of the location and distance between the distal tracheoesophageal fistula (TEF) and the proximal blind end of the esophagus (GAP) was evaluated in Type C esophageal atresia (EA)+TEF. Methods: IBI involved inserting the tip of a bronchoscope into the TEF and a nasogastric tube into the blind end of the EA and measuring GAP with fluoroscopy. EA+TEF patients (n = 23) treated thoracoscopically between 2007 and 2020 were classified according to IBI as IBI+ (n = 16) and IBI- (n = 7) to compare demographics, operative time, and time taken for TEF division. Results: Demographics were similar. Mean time for TEF division (15.4 ± 4.6 minutes for IBI+ versus 38.6 ± 20.9 minutes for IBI-; p < .05) and mean operative time (215.3 ± 48.9 minutes for IBI+ versus 286.4 ± 51.7 minutes for IBI+; p < .05) were significantly shorter. Mean GAP measured radiographically was 0.5 cm (range: 0-1.2 cm); mean GAP measured with IBI was 0.9 cm (range: 0-2.2 cm). Postoperative complications were 3 anastomotic leakages (1/16 in IBI+ and 2/7 in IBI-) that resolved without surgery and 8 strictures (3/16 in IBI+ and 5/7 in IBI-) treated by dilatation. Conclusions: IBI was effective for measuring GAP and is recommended for improving the efficiency of thoracoscopic repair.

Native liver survivors of portoenterostomy for biliary atresia with excellent outcome: redefining "successful" portoenterostomy.

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; p < 0.001). Durations of follow-up were similar: 13 years in EO, 18.5 years in NLS-2, 20 years in NLS-3, and 15 years in NLS-4. CONCLUSION: Incidence of "successful" PE or EO is low and correlated with early onset of symptoms and quicker JC.

Japan's comprehensive undescended testis screening program: incidence of ascending testis after screening.

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

Oncologic safety of Carrel patch hepaticojejunostomy for treating cystic-type choledochal cyst in children based on 20-plus years follow-up.

AIM: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: CPA appears to be oncologically safe because of the absence of malignant transformation for at least 20 years.