Juan Prieto

Straightening ventral curvature while preserving the urethral plate in proximal hypospadias repair.

PURPOSE We report the efficacy of an expanded algorithm for penile straightening in proximal hypospadias surgery to preserve the urethral plate for urethroplasty. We also compared ventral corporotomy with grafting to multiple superficial ventral corporotomies without grafting for straightening greater than 30-degree ventral curvature. MATERIALS AND METHODS The need for urethral plate transection was compared in 2 groups comprising consecutive patients with proximal shaft to perineal hypospadias repair done by one of us (WS). The 47 patients in group 1 underwent surgery from 2000 to 2005 and had ventral curvature greater than 30 degrees after degloving, leading to urethral plate transection, while in 23 in group 2 from 2006 to 2008 mobilization of the corpus spongiosum/urethral plate and proximal urethra were also performed before urethral plate transection. Patients in group 1 with greater than 30-degree ventral curvature after urethral plate transection underwent ventral corporotomy with grafting (7) or multiple transverse corporotomies without grafting (4), while those in group 2 with greater than 30-degree ventral curvature after corpus spongiosum/urethral plate and urethral mobilization underwent multiple transverse corporotomies without grafting. RESULTS Excluding 10 group 1 and 3 group 2 boys without ventral curvature after degloving the rate of urethral plate transection significantly decreased from 54% to 15% using the expanded algorithm (p = 0.005). At a mean followup of 11 months in those with corpus spongiosum/urethral plate and urethral mobilization there was no recognized recurrent ventral curvature. Seven patients with greater than 30-degree ventral curvature underwent ventral corporotomy with grafting, while 11 underwent multiple transverse corporotomies without grafting. At a mean followup of 27 and 19 months, respectively, no patient had recurrent ventral curvature. CONCLUSIONS Mobilization of the corpus spongiosum/urethral plate and the urethra in proximal hypospadias cases with greater than 30-degree ventral curvature after penile degloving decreases the need for urethral plate transection. Ventral lengthening to correct corporeal disproportion can be achieved by corporotomy with grafting or by multiple transverse incisions without grafting.

Ureteroureterostomy via Inguinal Incision for Ectopic Ureters and Ureteroceles Without Ipsilateral Lower Pole Reflux

PURPOSE We report outcomes from ureteroureterostomy performed through an inguinal herniorrhaphy type incision for definitive management of ectopic ureters or ureteroceles in the absence of ipsilateral lower pole reflux. MATERIALS AND METHODS Records were reviewed for 23 consecutive patients who underwent lower ureteroureterostomy for 26 duplicated systems with ectopic ureter or ureterocele and no ipsilateral lower pole reflux. In 21 patients surgery was performed through an inguinal incision similar to that for herniorrhaphy, while a Pfannenstiel incision was used initially in the series in 2 others with bilateral duplex systems. Median patient age at operation was 10 months (range 2 to 56). RESULTS At a mean followup of 26 months preoperative upper pole hydronephrosis and distal upper ureteral dilatation had resolved or improved in all cases. Preliminary cutaneous ureterostomy was performed on 4 ureters (3 patients), 2 because the recipient lower pole ureter was thought to be too small for incision and anastomosis, 1 because of purulent drainage and 1 following inadvertent transection of the lower pole ureter. Mean surgical time for inguinal ureteroureterostomy was 101 minutes and mean postoperative hospitalization was 0.6 days. Except for dextranomer/hyaluronic acid injection in 1 patient with symptomatic grade I ipsilateral lower pole reflux, no additional surgical procedures were performed after ureteroureterostomy. CONCLUSIONS Lower ureteroureterostomy offers potentially definitive treatment for ectopic ureter or ureterocele without ipsilateral lower pole reflux, and can be performed through an inguinal herniorrhaphy type incision.

Comparing outcomes of slings with versus without enterocystoplasty for neurogenic urinary incontinence.

PURPOSE We compared 2 cohorts of children with neurogenic urinary incontinence undergoing bladder neck sling with and without augmentation to determine relative continence outcomes, catheterization intervals, anticholinergic requirements and health related quality of life improvement as perceived by the patients and their parents. MATERIALS AND METHODS Consecutive patients followed through our spina bifida program underwent a structured postoperative interview by a research nurse to assess continence, interval between catheterizations and anticholinergic use. In addition, the child and parent together answered a health related quality of life satisfaction survey to determine the impact of surgery from their perspectives. RESULTS There were 18 patients undergoing sling with augmentation and 23 with sling alone. Overall improved continence rate was 83%, with no difference between outcomes in patients with vs without augmentation. However, the interval between catheterizations was longer and the use of anticholinergics was less following augmentation. Nevertheless, health related quality of life responses differed significantly in only 1 area, independent care, with both cohorts reporting similarly improved overall health, and increased ability to participate in social and leisure activities. CONCLUSIONS We directly compared results in patients undergoing slings with and without augmentation. Both procedures were similarly successful in achieving improved continence, with patients undergoing augmentation having a longer interval between catheterization and requiring fewer anticholinergics. However, health related quality of life responses revealed that both cohorts were similarly satisfied with the outcomes.

Screening ultrasound in follow-up after pediatric pyeloplasty.

OBJECTIVES To investigate whether an initial ultrasound (US) adequately identifies those patients who need further investigation and possibly intervention while decreasing instrumentation and radiation exposure. The recommended imaging after pediatric pyeloplasty has included intravenous pyelography, radionucleotide renography, and ultrasound US. METHODS We retrospectively reviewed pediatric open pyeloplasty cases performed between 1999 and 2007. Of 116 patients reviewed, 49 met the inclusion criteria of unilateral pyeloplasty with pre- and postoperative US and mercaptoacetyltriglycine (MAG-3). Hydronephrosis was judged by anterior posterior pelvic diameter and caliectasis. Change in renal function on MAG-3 was defined as increase or decrease in function >5%. RESULTS Of 49 patients, 42 (85.7%) showed improved or stable hydronephrosis postoperatively and 7 (14.3%) showed increased hydronephrosis. Forty-six (93.8%) patients had either improved or stable renal function. Of 42 patients with stable or improved hydronephrosis, 41 (97.6%) also had stable or improved function. Comparatively, of 7 patients with increasing hydronephrosis, 2 (28.6%) showed deteriorated renal function, P = .05. CONCLUSIONS Postpediatric pyeloplasty imaging should aim to identify those who require further intervention. Our data demonstrate that at-risk patients can be identified with a sentinel US and selectively determine who needs further MAG-3 investigation. This should increase compliance while decreasing costs, instrumentation, and radiation exposure.

Spontaneous bladder rupture in non-augmented bladder exstrophy

Childhood spontaneous bladder rupture is an uncommon event, usually associated with bladder augmentation. Occasionally it occurs in normal bladders or non-augmented bladders with a predisposing factor. We present a unique case of misdiagnosed spontaneous bladder rupture in a non-augmented bladder exstrophy patient without evidence of lower urinary outlet obstruction. His acute abdomen mimicked appendicitis and was managed with laparoscopic exploration, intraperitoneal fluid drainage, appendectomy and bladder drainage. This case highlights the need for pediatric urological consultation by surgeons in the case of an acute abdomen with free fluid in a patient with an abnormal bladder.

Childhood renal lymphangiectasia

heterogenously hyperintense and hypointense on with a renal lymphangiectasia. At 2.5 years of followup, she remains asymptomatic without radiological change. Renal lymphangiectasia is a rare, benign tion of the perirenal lymphatic system. The lymphatic structures that surround the kidney fail to establish normal communication with the rest of the lymphatic system. The physiopathological process is ectasia of

Surgical Technique for Use of Autologous Buccal Mucosa Vaginoplasty in Children: Video

Abstract Purpose Vaginal replacement surgeries often require donor materials such as skin grafts, myocutaneous flaps, or bowel, each with significant disadvantages. We describe our technique using autologous buccal mucosa grafting for partial or complete neovagina creation. Material and Methods Our indications for buccal mucosa vaginoplasty have included primary repair of congenital defects such as vaginal agenesis (Mayer-Rokitansky syndrome), cloacal abnormalities, and intersex disorders as well as secondary repair of postsurgical vaginal defects like vaginal stenosis after vaginoplasty or pelvic extenteration. This video demonstrates our surgical technique in a Mayer-Rokitansky syndrome patient. Results A transverse incision is made over the mucosal prominence at the level of the expected location of the hymen. Blunt dissection and electrocautery are used to dissect between the urethra and anorectum to depth 8 cm and width 3 fingerbreadths. Bilateral buccal mucosa is harvested, prepared on the bench and sewn end to end. The graft mid-portion is secured with interrupted 4-0 chromic to the apex of the previously dissected vaginal vault. The meshed graft is then stretched and tacked into position with interrupted 4-0 chromic sutures to achieve 360 degree coverage of the entire neovaginal area. A spongy vaginal mold is left in-situ for 5-7 days. Conclusions Buccal mucosa generates a moist, hairless, nonkeratinized neovaginal mucosa with excellent color and texture matching the genital/vaginal skin. It leaves no visible surgical scars, avoids abdominal bowel surgery and has no excess mucous production. It is an ideal replacement material for primary or secondary vaginoplasty with excellent early results.

Comparison of Pediatric Stone-Formers with and without mutations in Soluable Adenylate Cyclase

Abstract Purpose Two-thirds of children with urolithiasis have hypercalciuria. Recently, a candidate gene for absorptive hypercalciuria (AH) was mapped to chromosome 1q23.3-24 in the adult population. In adults, the presence of up to 6 identified base substitutions in the soluble adenylate cyclase gene (sAC) was associated with a 2.2- to 3.5-fold increased risk for AH. We screened a pediatric population of stone formers for sAC sequence variations. Material and Methods Pediatric patients with stone disease were offered study participation if they met the following criteria: family history of stones; hypercalciuria (urine calcium/creatinine ratio > 0.21 mg/mg creatinine or 24 hour calcium excretion > 4 mg/kg/day); and/or calcium stones on stone analysis. Serum laboratories, spot and 24-hour urine, and bone mineral density (BMD) were collected. Patients were excluded for metabolic conditions predisposing to stone disease. Probands were genotyped for 6 previously identified base substitutions in the sAC gene. Comparisons were made between probands with and without sAC substitutions using Fishers exact test. Results Of 46 children recruited, sAC substitutions were identified in 19 (41%). Clinical parameters were similar in patients with and without substitutions: positive family history in 14/17 (82%) vs. 21/27 (78%); hypercalciuria in 13/19 (68%) vs. 22/27 (82%); and mean number of stone events 2.1 vs. 2.8. Overall, 5/16 (31%) had abnormal BMD (age-adjusted Z-scores Conclusions Of 46 pediatric stone formers, 31% had abnormal BMD scores, and 41% of probands had sAC gene base changes previously identified in adults to increase risk for stones and osteoporosis. This warrants further investigation for potential genetic screening and therapeutic interventions to prevent pediatric stone formation and long-term osteoporotic risk.