Liver Mitochondrial Morphology and Gene Expression as Markers of Liver Reserve: Prognostic Implications for Native Liver Survival in Biliary Atresia.

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.

Intraoperative surgical complications of open surgery for congenital diaphragmatic hernia: a multicenter, observational study in Japan.

PURPOSE: This study aimed to clarify surgical complications associated with open surgery for congenital diaphragmatic hernia (CDH). METHODS: We performed an exploratory data analysis of the clinical characteristics of surgical complications of neonates with CDH who underwent laparotomy or thoracotomy between 2006 and 2021. Data of these patients were obtained from the database of the Japanese CDH Study Group. RESULTS: Among 1,111 neonates with left or right CDH, 852 underwent open surgery (laparotomy or thoracotomy). Of these 852 neonates, 51 had the following surgical complications: organ injury (n = 48; 6% of open surgeries); circulatory failure caused by changes in the organ location (n = 2); and skin burns (n = 1). Injured organs included the spleen (n = 30; 62% of organ injuries), liver (n = 7), lungs (n = 4), intestine (n = 4), adrenal gland (n = 2), and thoracic wall (n = 2). Fourteen of the patients who experienced organ injury required a blood transfusion (2% of open surgeries). The adjusted odds ratio of splenic injury for patients with non-direct closure of the diaphragm was 2.2 (95% confidence interval, 1.1-4.9). CONCLUSION: Of the patients who underwent open surgery for CDH, 2% experienced organ injury that required a blood transfusion. Non-direct closure of the diaphragmatic defect was a risk factor for splenic injury.

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.

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.

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.

Intraoperative Transpyloric Tube Insertion for Congenital Diaphragmatic Hernia: Analysis of Japanese Study Group Data.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a severe congenital disease. Some CDH infants suffer from gastro-esophageal reflux disease (GERD), even after surgical correction of gastric position. A transpyloric tube (TPT) is inserted into CDH patients under direct observation intraoperatively in some hospitals in Japan to establish early enteral feeding. This strategy avoids gastric expansion to maintain a better respiratory condition. However, it is unclear whether the strategy has a secure effect for patient prognosis. This study aimed to evaluate the effectiveness of intraoperative TPT insertion on enteral feeding and postoperative weight gain. METHODS: The Japanese CDH Study Group database was used to identify infants with CDH born between 2011 and 2016, who were then divided into two groups: the TPT group and gastric tube (GT) group. In the TPT group, infants underwent intraoperative TPT insertion; postoperative insertion/extraction of TPT was irrelevant to the analysis. Weight growth velocity (WGV) was calculated using the exponential model. Subgroup analysis was performed using Kitano's gastric position classification. RESULTS: We analyzed 204 infants, of which 99 and 105 were in the TPT and GT groups, respectively. Enteral nutrition (EN) in the TPT and GT groups was 52 ± 39 and 44 ± 41 kcal/kg/day (p = 0.17) at age 14 days (EN14), respectively, and 83 ± 40 and 78 ± 45 kcal/kg/day (p = 0.46) at age 21 days (EN21), respectively. WGV30 (WGV from day 0 to day 30) in the TPT and GT groups was 2.3 ± 3.0 and 2.8 ± 3.8 g/kg/day (p = 0.30), respectively, and WGV60 (WGV from day 0 to day 60) was 5.1 ± 2.3 and 6.0 ± 2.5 g/kg/day (p = 0.03), respectively. In infants with Kitano's Grade 2 + 3, EN14 in the TPT and GT groups was 38 ± 35 and 29 ± 35 kcal/kg/day (p = 0.24), respectively, EN21 was 73 ± 40 and 58 ± 45 kcal/kg/day (p = 0.13), respectively, WGV30 was 2.3 ± 3.2 and 2.0 ± 4.3 g/kg/day (p = 0.76), respectively, and WGV60 was 4.6 ± 2.3 and 5.2 ± 2.3 g/kg/day (p = 0.30), respectively. CONCLUSION: Intraoperative TPT insertion did not improve nutritional intake and WGV30. WGV60 in TPT was less than that in GT. In Grade 2 + 3 subgroup analysis, TPT also had no advantage. We could not recommend routine TPT insertion at surgery. LEVEL OF EVIDENCE: III.

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.

The efficacy of the postnatal nasogastric tube position as a prognostic marker of left-sided isolated congenital diaphragmatic hernia.

PURPOSE: The prenatal diagnosis of the stomach position in congenital diaphragmatic hernia (CDH) has been a reliable prognostic factor, but few studies have focused on the postnatal position. We therefore evaluated the significance of the nasogastric (NG) tube position just after birth. METHODS: The Japanese CDH Study Group database enrolled 1037 CDH neonates over 15 years. In our multicenter retrospective study, 464 cases of left-sided isolated CDH with prenatal diagnoses were divided into two groups: NG tube below the diaphragm (BD; n = 190) or above the diaphragm (AD; n = 274). The primary outcome was the 90-day survival rate, and the secondary outcomes were mechanical ventilation duration, hospitalization duration, and recurrence rate. RESULTS: The BD group had a significantly higher 90-day survival rate (98.4 vs. 89.4%, p < 0.001), shorter mechanical ventilation (11 vs. 19 days, p < 0.001), shorter hospitalization (38 vs. 59 days, p < 0.001), and lower recurrence rate (p = 0.002) than the AD group. A multivariate analysis showed that BD (adjusted odds ratio, 3.68; 95% confidence interval 1.02-13.30) was a favorable prognostic factor for the 90-day survival. CONCLUSION: The assessment of the NG tube position revealed it to be a reliable prognostic factor of left-sided isolated CDH. Therefore, it should be included as a routine assessment.

Prenatal predictors of mortality in fetuses with congenital diaphragmatic hernia: a systematic review and meta-analysis.

PURPOSE: This study aimed to evaluate prenatal predictors of mortality in fetuses with congenital diaphragmatic hernia (CDH). METHODS: A systematic literature search was performed to identify relevant observational studies that evaluated the ability of lung-to-head ratio (LHR), observed-to-expected LHR (o/e-LHR), observed-to-expected total fetal lung volume (o/e-TFLV), lung-to-thorax transverse area ratio (L/T ratio), intrathoracic herniation of the liver and the stomach, and side of diaphragmatic hernia, using a threshold for the prediction of mortality in fetuses with CDH. Study quality was assessed using the QUADAS-2 tool. Hierarchical summary receiver operating characteristic curves were constructed. RESULTS: A total of 50 articles were included in this meta-analysis. The QUADAS-2 tool identified a high risk of bias in more than one domain scored in all parameters. Among those parameters, the diagnostic odds ratio of mortality with o/e-LHR < 25%, o/e-TFLV < 25%, and L/T ratio < 0.08 were 11.98 [95% confidence interval (CI) 4.65-30.89], 11.14 (95% CI 5.19-23.89), and 10.28 (95% CI 3.38-31.31), respectively. The predictive values for mortality were similar between the presence of liver herniation and retrocardiac fetal stomach position. CONCLUSIONS: This systematic review suggests that o/e-LHR, o/e-TFLV, and L/T ratio are equally good predictors of neonatal mortality in fetuses with isolated CDH.

Long-Term Outcomes of Congenital Diaphragmatic Hernia: Report of a Multicenter Study in Japan.

BACKGROUND: Treatment modalities for neonates with congenital diaphragmatic hernia (CDH) have greatly improved in recent years, with a concomitant increase in survival. However, long-term outcomes restrict the identification of optimal care pathways for CDH survivors in adolescence and adulthood. Therefore, we evaluated the long-term outcomes within the Japanese CDH Study Group (JCDHSG). METHODS: Participants were born with CDH between 2006 and 2018 according to the JCDHSG. Participants were enrolled in the database at 1.5, 3, 6, and 12 years old. Follow-up items included long-term complications, operations for long-term complication, and home medical care. RESULTS: A total of 747 patients were included in this study, with 626 survivors (83.8%) and 121 non-survivors (16.2%). At 1.5, 3, 6, and 12 years old, 45.4%, 36.5%, 34.8%, and 43.6% developed complications, and 20.1%, 14.7%, 11.5%, and 5.1% of participants required home care, respectively. Recurrence, pneumonia, pneumothorax, gastroesophageal reflux disease, and intestinal obstruction decreased with age, and thoracic deformity increased with age. CONCLUSIONS: As CDH survival rates improve, there is a need for continued research and fine-tuning of long-term care to optimize appropriate surveillance and long-term follow-up.

Long-term outcomes of male imperforate anus with recto-urethral fistula: laparoscopy-assisted anorectoplasty versus posterior sagittal anorectoplasty.

PURPOSE: A five-parameter fecal continence evaluation questionnaire (FCEQ) and incidence of complications were used for long-term assessment of laparoscopy-assisted anorectoplasty (LAARP) and posterior sagittal anorectoplasty (PSARP) for treating male imperforate anus (MIA) with rectobulbar (RB) or rectoprostatic (RP) fistulas. METHODS: Subjects were 64 consecutive Japanese MIA patients with RB or RP fistulas treated at a single institution between 1995 and 2021. FCEQ data collected retrospectively were used to calculate a fecal continence evaluation (FCE) score (best = 10) and coefficient of variation for FCE (FCECV). The statistical significance threshold was defined at p < 0.05. RESULTS: Fistulas were RB (n = 40; LAARP = 25/40, PSARP = 15/40) or RP (n = 24; LAARP = 17/24, PSARP = 7/24). Mean ages at surgery and status of the sacrum were similar (p = 0.06, 0.05 and 0.51). FCE scores in RP-LAARP were consistently higher with less FCECV but differences were only statistically significant from 7 years postoperatively (p < 0.05). While FCE scores for RB-LAARP and RB-PSARP were similar (p = 0.99), FCECV were lower for RB-LAARP compared with RB-PSARP. LAARP was associated with less-wound infections, but greater incidence of anal mucosal prolapse unrelated to preoperative status of the sacrum. CONCLUSION: Long-term postoperative FCEQ assessment favored LAARP for treating MIA with either RB or RP fistulas.

A useful tip for preventing surgical site infections after hypospadias repair. A single surgeon's experience of 376 cases.

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.

Lysosomal overloading and necrotizing enterocolitis.

PURPOSE: Intestinal absorption in premature infants occurs via direct epithelial cellular endocytosis and degradation by intracellular lysosomes. Autophagy is a mechanism by which cytoplasmic organelles contribute to lysosomal degradation. However, excessive autophagy can lead to cell death. The purpose of this study was to investigate whether autophagy and endocytosis are present in the small intestinal mucosa during experimental necrotizing enterocolitis (NEC). METHODS: NEC was induced by gavage feeding of hyperosmolar formula, lipopolysaccharide and hypoxia between P5 and P9 (ethical approval 44032). Breastfed mice were used as control. Distal ileum was harvested on P5, P7 and P9 and analyzed for intestinal epithelial cellular morphology as well as autophagy/lysosomal activity, and cell death. Groups were compared using Student's t test. RESULTS: During NEC, giant lysosomes were present in the intestinal villi, with some exceeding their degradation ability leading to their rupture. The NEC group had significantly increased inflammation and autophagy activity, decreased lysosome activity, and increased apoptosis compared to control. CONCLUSIONS: NEC induction causes excessive autophagy and endocytosis leading to lysosomal overloading, lysosomal membrane permeabilization and rupture which results in cell death. These novel findings may help to clarify the pathogenesis of NEC. Reduction of lysosome overload and assisting in their degradation capability may reduce the burden of NEC.

Atypical rectoscrotal and rectoperineal fistula in male anorectal malformations: a case series of non-detaching strategies for preventing urethral injury.

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.

Indications for Nonoperative Management of Uncomplicated Appendicitis in Children: A Prospective Analysis at a Single Institution.

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-appy cases presenting younger than 16 years between 2015 and 2018 who had NOM (one dose of intravenous analgesia and intravenous piperacillin/tazobactam 112.5 mg/kg 8 hourly) and were followed up for at least 3 months were reviewed prospectively (n = 146). Perceived pain and fever were assessed 12 hourly, biochemistry daily. If predetermined cutoff results were not achieved at each assessment, NOM was abandoned and urgent laparoscopic appendectomy (ULA) performed. Results: NOM succeeded in 48.6% (S-NOM; n = 71) and failed in 51.4% (F-NOM; n = 75). Mean age at presentation (10.7 ± 2.5 versus 8.6 ± 3.7 years old; P < .0001) and duration of preadmission fever (1.0 ± 0.9 versus 2.1 ± 1.2 days; P < .0001) were the only significantly different criteria between S-NOM and F-NOM. Optimal cutoff values using receiver operating characteristic curve analysis were 7.0 years old (32% sensitivity and 93% specificity) and 1.0 day (95% sensitivity and 25% specificity), respectively. NOM was abandoned for persistent pain, prolonged fever, or raised white blood count at 12 hours in 20/75 (26.7%), 24 hours in 31/75 (41.3%), 36 hours in 14/75 (18.7%), and 48-72 hours in 10/75 (13.3%). At ULA, perforation was identified in 14/75 (18.7%). Complications arising within 3 months of ULA were residual abscess (n = 7/75; 9.3%) and transient ileus (n = 1/75; 1.3%). Conclusion: It would appear that surgery may be more appropriate for children with UC-appy when they are younger and febrile before admission for longer.

The role of autophagy in intestinal epithelial injury.

PURPOSE: Autophagy is a natural mechanism aimed to degrade and recycle cellular components within cells. Previous studies reported that autophagy in the intestinal epithelium can be activated and that excessive autophagy can have negative consequences. However, the mechanism by which autophagy is regulated during intestinal epithelial injury remains unclear. This study aimed to investigate the mechanism of autophagy regulation during intestinal epithelial cells (IEC) injury. METHODS: Rat IEC18 were exposed to hypoxia and Lipopolysaccharide (LPS) (200 µg/ml) to induce injury. IEC18 were treated with autophagy initiation inhibitor, Wortmannin or with autophagy degradation inhibitor, Bafilomycin A1 were added for 24 h. We assessed the number and diameter of autophagic vacuoles, Cell viability, inflammation and apoptosis. RESULTS: Hypoxia and LPS administration increased the number and diameter of autophagic vacuoles in IEC18. Wortmannin administration reduced the number and diameter of autophagic vacuoles. On the contrary, Bafilomycin A1 administration increased the number of autophagic vacuoles. Cell viability increased following administration of Wortmannin and decreased following administration of Bafilomycin A1. CONCLUSIONS: We found that accumulation of autophagic vacuoles which characterize excessive or incomplete autophagy was detrimental to cell survival. This was shown by an increase in the number and size of the autophagic vacuoles with Bafilomycin A1treatment after hypoxia and LPS stressors relative to hypoxia and LPS alone. Conversely, there was a decrease in the number of autophagic vacuoles with Wortmannin treatment after hypoxia and LPS stressors relative to hypoxia and LPS alone. Therefore, reducing autophagosomes accumulation may represent a novel therapeutic strategy for intestinal injury.

Undermining the epidermis around the U-shaped skin incision preserves thick, well-vascularized tissue that effectively prevents posturethroplasty complications.

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 ±â€¯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.

Surgical management of hypospadias in cases with concomitant disorders of sex development.

INTRODUCTION: To review the surgical treatment of hypospadias (HP) associated with disorders of sex development (DSD). PATIENTS AND METHODS: 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. RESULTS: 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). CONCLUSIONS: Although UP for HP + DSD was formidably challenging, we achieved a significant decrease in post-UPC through a combination of surgical techniques and experience.

How best to expose the entire surgical anal canal in the operative field during transanal pull-through for Hirschsprung's disease: a crucial step that determines success.

PURPOSE: During transanal pull-through (TAPT) for Hirschsprung's disease (HD), exposing the entire surgical anal canal (SAC) including the squamo-columnar junction, or anorectal line (ARL) is a crucial step for minimizing problematic postoperative bowel function. We present a hint for exposing the entire SAC. METHOD: Histologically, the ARL represents the junction of proximal unilayer columnar colorectal mucosa with distal stratified squamous epithelium and is the proximal limit of the SAC. It is an obvious landmark; proximal mucosa is vivid pink and distal mucosa is more whitish. We use the Lone Star (LS) self-retaining retractor system to expose the ARL. Before we attach the LS hooks to the anal sinuses on the dentate line full-circle, we place 3/0 sutures at 0, 3, 6, and 9 o'clock around the anus to expose the anal sinuses. If the patient's buttocks cannot be positioned as described or the patient is too high on the table, the LS ring does not sit well, resulting only in dilatation and lengthening of the SAC without prolapse. By hanging the patient's buttocks 5 cm over the end of the table, the LS ring sits snugly and the ARL and entire SAC prolapse to the anal verge. RESULTS: Good positioning, as described, greatly facilitated dissection in 61/68 TAPT cases, while poor exposure hindered treatment in 7/68. CONCLUSION: Thorough exposure of the entire SAC, which is crucial for adequate TAPT, is greatly facilitated by patient positioning.