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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 2 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%, pConclusion: 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.

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.

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.

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.

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.

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.

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). 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. 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. 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.

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-...

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

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. 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. 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. 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.

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.

To ensure good postoperative bowel function (POBF) following transanal pull-through (TAPT) for Hirschsprung’s disease (HD), transanal dissection should commence just proximal to the anorectal line (ARL), leaving the ARL intact and preserving the anal transitional zone (ATZ). Because the ARL is a readily identifiable landmark, transanal dissection will thus become standardized with reliable and reproducible results. In addition, the posterior rectal muscle cuff above the ARL should be excised completely to fully release achalasia due to an aganglionic rectum while preserving the ARL and the ATZ.

To report our experience of correcting penile ventral curvature associated with minor or no hypospadias. We reviewed 43 penile ventral curvature patients treated by a single surgeon from 1997 to 2015. Of these, 23 had minor hypospadias. Curvature was corrected using degloving, chordectomy, dorsal plication (DP), tunica albuginea incision (TAI), or a combination of these. Outcome was confirmed by induced artificial erection and post-operative appearance. Mean age at curvature correction was 3.2 ± 2.6 years. 17/43 had degloving and chordectomy (DC), 16/43 had DP after DC, and 10/43 had TAI after DC, because of ventral shortening and severe curvature caused by a short hypoplastic urethra. Other procedures required were primary meatoplasty (n = 4) and urethroplasty (UP; n = 1) at the time of curvature correction, and UP after correction of curvature (n = 11). Complications included recurrence of curvature after DP (n = 3/16; 18.8 %) and urethral stenosis after UP with tubed peritoneum (n = 1/10; 10 %). There were no recurrences of curvature in TAI cases. Parents reported penile cosmesis as good (n = 38; 88.4 %), acceptable (n = 4; 9.3 %), or poor (n = 1; 2.3 %). We recommend TAI followed by UP for correcting penile ventral curvature with short hypoplastic urethra. Tubed peritoneum is not recommended for UP.

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. 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. Good positioning, as described, greatly facilitated dissection in 61/68 TAPT cases, while poor exposure hindered treatment in 7/68. Thorough exposure of the entire SAC, which is crucial for adequate TAPT, is greatly facilitated by patient positioning.

INTRODUCTION We prospectively reviewed 41 male infants younger than 12 months old who had presented with severe incarcerated inguinal hernia between 2014 and 2016 and had been treated by laparoscopic percutaneous extraperitoneal closure (LPEC) or conventional open repair (CO). METHODS Operative times and intraoperative findings were evaluated. Scrotal/testicular status were assessed preoperatively, 1 week, and 4 weeks after surgery. RESULTS There were 21 boys treated by LPEC and 20 by CO. Mean ages and mean weights at surgery were similar between the groups. The mean operative time was 19.7 min for LPEC and 45.8 min for CO (P

Soft tissue interposition (STI) during hypospadias repair (HR) purportedly prevents postoperative urethrocutaneous fistula (PUF) by supporting the neourethra. We report our experience.Data from 243 hypospadias patients treated by a single surgeon from 1997 to 2014 by urethroplasty (UP) with STI (n = 229; UP + STI) and UP without STI (n = 14; UP-STI) were collated prospectively and compared for incidence of PUF. Re-operative UP were excluded.Hypospadias was distal (n = 55), mid-shaft (n = 59), proximal/penoscrotal (n = 109), scrotal (n = 15), and perineal (n = 5). UP was single-staged in 86, multi-staged in 157; mean age at UP was 3.1 ± 2.4 years. Soft tissue used for STI was prepucial inner dartos fascia (inner dartos: n = 88), ventral dartos fascia (ventral dartos: n = 15), pedicled external spermatic fascia (ESF: n = 84), adipose tissue surrounding the spermatic cord (pericordal: n = 9), scrotal adipose tissue (n = 8), or a combination of tissues (combined: n = 25). Mean follow-up was 6.4 ± 4.6 (range 0.6-16.8) years. Overall incidence of PUF was 10/243 (4.1 %); 7/229 (3.1 %) for UP + STI and 3/14 (21.4 %) in UP-STI (p0.05); incidence versus type of hypospadias was 1/55 for distal (1.8 %), 3/59 for mid-shaft (5.1 %), 5/109 for proximal/penoscrotal (4.6 %), 0/15 for scrotal (0 %), and 1/5 for perineal (20 %); incidence versus type of STI was 7/88 for inner dartos, 0/15 for ventral dartos, 0/84 for ESF, 0/9 for pericordal adipose tissue, 0/8 for scrotal adipose tissue, and 0/25 for combined. All PUF were repaired successfully. Satisfaction with penile cosmesis was acceptable (10.3 %) or good (89.7 %) without any testicular complications or scrotal deformity.STI, especially ESF, would appear to effectively prevent PUF in HR.

Late-onset congenital diaphragmatic hernia (CDH) is an uncommon subset of CDH and distinct from neonatal CDH with respect to presenting symptoms, diagnosis, management, and prognosis. In particular, CDH diagnosed after 30 days of age (late-onset CDH) is uncommon and has an atypical presentation and a more favorable prognosis. In the present report, an infantile late-onset CDH case that presented with bloody stool and had a severe clinical course is described. In previous reports, no late-onset CDH case developed bloody stool. After diagnosis with image inspections, emergency surgery was performed. At operation, via the hernia orifice, the jejunum was seen to have prolapsed into the thoracic cavity with focally significant intestinal and mesenteric congestion, but no intestinal necrosis. In general, other disorders such as intussusception may be considered in the differential diagnosis of acute abdomen with bloody stool in patients of this age. However, in such late-onset CDH cases, immediate differentiation from other causes of acute abdomen that present with bloody stool is life-saving.

Laparoscopically assisted anorectoplasty (LAARP) was compared to posterior sagittal anorectoplasty (PSARP) in the treatment of male imperforate anus associated with either recto-prostatic fistula (RPF) or recto-bulbar fistula (RBF). 19 RPF patients (12 treated by LAARP and 7 by PSARP) and 26 RBF patients (14 treated by LAARP and 12 by PSARP) between 1995 and 2014 were retrospectively assessed using a fecal continence evaluation questionnaire (FCE) (with a maximum score of 10), an FCE score coefficient variation, as well patients’ MRI scores, anorectal angle values (AA), and incidence of postoperative complications. Statistical significance was determined at p

We monitored arterial blood gases during thoracoscopic (TR) and open repair (OR) of congenital diaphragmatic hernia (CDH) to assess the safety of intraoperative hypercapnia and acidosis.We reviewed 30 neonatal CDH cases (OR = 10, TR = 20) diagnosed prenatally or within 6 h of birth at out institution from 2002 to 2014 not requiring inhaled nitric oxide (NO) intraoperatively. OR, routine until 2006 was replaced by TR in 2007. All subjects were managed identically.Five TR cases requiring conversion to OR were excluded. Prenatal diagnosis, gestational age at birth, gender, birth weight, and side of CDH were similar. Preoperative PaCO2 and pH were not significantly different. However, while intraoperative increase in mean PaCO2 (38.8-62.8 mmHg; p0.01) and decrease in mean pH (7.44-7.25; p0.01) were significant in TR, intraoperative PaCO2 was70 mmHg in 12/15 cases and intraoperative pH was7.20 in 11/15 cases. Both PaCO2 and pH reverted to normal on completion of surgery; pre- and postoperative results were comparable. There were no postoperative complications.It would appear that neonatal cases of CDH not requiring NO can tolerate TR, despite transient reversible deterioration in acid/base balance, indicating that TR is safe for the treatment of selected cases of CDH.

Urethrocutaneous fistula (UCF) complicating hypospadias surgery is associated with compromised tissue and perfusion at the UCF site, especially if recurrent. We report our technique for UCF repair.Between 1997 and 2014, we treated 35 UCF in 26 postoperative hypospadias patients; 12 UCF were recurrent (mean 2.5; range 1-5). Mean age at UCF repair was 9.3 years (range 2-22). Our repair involves making a superficial incision 3-5 mm around the fistula orifice, placing multiple stay sutures in the outer edge of this incision and dissecting only the epidermis under traction for 7-10 mm. This technique does not compromise underlying connective tissue or tissue perfusion. The skin layer of the inner edge of the circumferential incision is trimmed completely and the fistula closed using 7/0 absorbable interrupted sutures. A pedicled external spermatic fascia, or tunica vaginalis flap is then mobilized to cover the repair site through a subcutaneous tunnel and the skin closed. A urethral catheter is placed and removed the next day. Duration of follow-up was calculated as the period from discharge home until the last outpatient clinic attendance.Repair was successful in all cases. Penile cosmesis was acceptable to good without any testicular complications or scrotal deformity. At mean follow-up of 7.4 years (range 0.4-17.3) there have been no recurrences.Our technique allows UCF to be repaired effectively and is also indicated for recurrences.

There are few reports about postoperative outcome of penoplasty (PP). We present the results of mid- to long-term follow-up of PP performed for congenital megaprepuce (CMP). Data from 65 CMP cases treated by PP performed by a single surgeon from 2000 to 2014 were collected prospectively. All cases were treated using the technique reported by Cuckow (Pediatric surgery. Springer, Berlin, pp 543–554, 2006). Mean age at PP was 5.9 years (range 0.4–13.9). All cases presented as infants and some 12 cases (18.5 %) had PP when 10 or more years old. There were no intra- and postoperative complications. Mean duration of follow-up was 3.6 years (range 0.1–17.5). Duration of follow-up was 4 years or less in 48 (73.8 %), 5–9 years in 13 (20.0 %), and 10 or more years in 4 (6.2 %). While postoperative penile cosmesis was good in 63/65 (96.9 %) cases without scrotal deformity, 2/65 (3.1 %) had redundant penile skin excised upon the recommendation of the treating surgeon even though the patients and parents were unconcerned. Mid- to long-term follow-up of our PP cases shows that outcome is cosmetically acceptable and stable.