Navroop Johal

Ureterocystoplasty: long-term functional results.

PURPOSE Many institutions have reported varied levels of success with ureterocystoplasty. Recently, there have been concerns regarding the efficacy of ureterocystoplasty as a form of bladder augmentation. We report our long-term functional outcomes with the procedure based on the experience of a single surgeon. MATERIALS AND METHODS A total of 17 procedures were performed during a 12-year period. Of the patients 10 had posterior urethral valves, 3 had bladder exstrophy, 2 had a neuropathic bladder, 1 had a cloacal anomaly and 1 had a left ectopic ureter. Mean patient age at operation was 5.9 years (range 0.3 to 14.2). All patients underwent preoperative ultrasound, urodynamic studies and radionuclide scanning. Postoperative urodynamics were performed at 6 months. RESULTS Followup ranged from 0.5 to 11.5 years (mean 4.5). Nine patients underwent ureterocystoplasty with preservation of the ipsilateral kidney, and 1 underwent ureterocystoplasty alone and had a solitary right kidney. The remaining 7 patients underwent ureterocystoplasty with ipsilateral nephrectomy. Postoperatively, the mean bladder capacity improved from 125 to 292 ml, while the mean end filling pressure decreased from 72 to 22 cm H(2)O. Mean preoperative system compliance was 2.1 ml/cm H(2)O, which increased to 16.2 ml/cm H(2)O postoperatively. A total of 13 patients did not require further augmentation surgery. The remaining 4 patients had high pressure and poorly compliant bladders, and underwent ileocystoplasty. Subsequent postoperative nucleotide scans in these 4 patients demonstrated stable upper tracts with good drainage. CONCLUSIONS Based on our results, ureterocystoplasty provides durable functional urodynamic improvement in patients with a megaureter.

Is it safe to use aluminum in the treatment of pediatric hemorrhagic cystitis? A case discussion of aluminum intoxication and review of the literature.

In pediatric oncology patients, hemorrhagic cystitis can be a life-threatening complication of bone marrow transplantation, chemotherapy, and radiation therapy. The treatment of this condition is often challenging and includes intravesical irrigation with aluminum, embolization, endoscopic laser coagulation, hydrostatic pressure, use of hyperbaric oxygen, instillation of formalin, prostaglandins, and oral sodium pentosan polysulfate. Although the efficacy of aluminum irrigation is well documented for the management of hemorrhagic cystitis in adults, there are limited reports describing its use in children. The potential multisystem toxic effects of aluminum are well described and the range and progression of aluminum toxicity can be devastating. We report a case of a 9-year-old girl suffering from acute lymphocytic leukemia with hemorrhagic cystitis. Although the symptoms resolved after intravesical aluminum treatment, she developed significant aluminum toxicity. We have reviewed the literature relating to aluminum toxicity in the pediatric age group and present our recommendations for the effective and safe use of aluminum in this cohort of patients.

The role of unilateral nephrectomy in the treatment of nephrogenic hypertension in children.

To define the efficacy of unilateral nephrectomy in a large series of patients presenting with renal disease and hypertension, as the latter may be a prominent finding in children with nephrourological disease (renal parenchymal disease, renovascular disease, obstruction, renal dysplasia and cancer).

Neonatal Imperforate Hymen Causing Obstruction of the Urinary Tract

An imperforate hymen associated with urinary retention, bilateral hydronephrosis, and bilateral hydrosalpinx is extremely rare in the neonatal period. We present a case of a 2-day-old neonate with a marked interlabial swelling causing urinary retention. Imaging revealed hydrometrocolpos, hydrosalpinx, and bilateral hydronephrosis. Her symptoms resolved after hymenectomy.

Secondary hypospadias repair in two stages.

INTRODUCTION Following failed primary reconstruction, various single-stage reconstructive techniques have been proposed for secondary hypospadias repair. Unfortunately, these frequently fail to achieve a satisfactory cosmetic and functional outcome. We review a single surgeons experience of two-staged reconstruction in this scenario. PATIENTS AND METHODS Of 104 patients with hypospadias treated in two stages, between July 1998 and June 2003, 42 were secondary reconstructions. The site of the meatus after initial failed repair was glanular (5), subcoronal (11), distal shaft (12), mid-shaft (6) or peno-scrotal (8). Factors associated with the choice of two-stage reconstruction included moderate/severe chordee (14), excessive scarring (15), shallow/no groove or poor glans (8), and balanitis xerotica obliterans (4). The grafts used were post-auricular skin (PAWG, 29) buccal mucosa (BMG, 6), inner prepuce (IP, 6) and one composite graft. The mean age of our patients was 131 months and the average follow up after the second stage was 26.3 months. RESULTS In 40 of the 42 cases the graft took well. In one patient a PAWG developed keloid scarring which was excised and later successfully replaced with a BMG. In another, severe shrinkage of a BMG led to revision with a PAWG. Following the second stage, complications were experienced in five: the repair broke down in two BMG patients and was successfully reconstructed with a PAWG in both, and three developed fistulae that were repaired (1 PAWG, 1 BMG, 1 IP). CONCLUSIONS The two-staged reconstruction is a versatile and technically straightforward secondary procedure for this difficult patient cohort. Excellent cosmetic and functional results can be achieved with only a small risk of a revision operation. Four of our six BMG patients experienced problems and PAWG appears more satisfactory with superior results.

Transpubic radical prostatectomy: a novel approach for rhabdomyosarcoma of the prostate in children.

Rhabdomyosarcoma is the most common soft tissue sarcoma, representing 10–15% of all malignant solid tumours in children and can occur in any site. The prognosis for rhabdomyosarcoma of the prostate has improved significantly in the last few decades. The management involves a combination of chemotherapy, radiotherapy and surgery. Several approaches to resect the prostate in adults have been described. These include radical retropubic, transperineal, posterior sagittal as well as the transpubic approach. In the child, the transpubic approach allows direct access to the prostate and proximal urethra whilst obviating problems noted in adults. We describe this approach to the prostate in a 3-year-old boy.

An unusual case of a prenatally detected large mullerian duct remnant.

Mullerian duct remnants presenting in childhood are often diagnosed incidentally and when symptomatic, typically present with recurrent infection and/or voiding disturbances. We present a rare unusual case of a male neonate who presented with a prenatally detected large pelvic cyst that turned out to be a giant prostatic utriculus.

Functional properties and connective tissue content of pediatric human detrusor muscle

The functional properties of human pediatric detrusor smooth muscle are poorly described, in contrast to those of adult tissue. Characterization is necessary for more informed management options of bladder dysfunction in children. We therefore compared the histological, contractile, intracellular Ca2+ concentration responses and biomechanical properties of detrusor biopsy samples from pediatric (3-48 mo) and adults (40-60 yr) patients who had functionally normal bladders and were undergoing open surgery. The smooth muscle fraction of biopsies was isolated to measure proportions of smooth muscle and connective tissue (van Gieson stain); in muscle strips, isometric tension to contractile agonists or electrical field stimulation and their passive biomechanical properties; in isolated myocytes, intracellular Ca2+ concentration responses to agonists. Pediatric detrusor tissue compared with adult tissue showed several differences: a smaller smooth muscle-to-connective tissue ratio, similar contractures to carbachol or α,β-methylene ATP when corrected for smooth muscle content, and similar intracellular Ca2+ transients to carbachol, α,β-methylene ATP, raised K+ concentration or caffeine, but smaller nerve-mediated contractions and greater passive stiffness with slower stress relaxation. In particular, there were significant atropine-resistant nerve-mediated contractions in pediatric samples. Detrusor smooth muscle from functionally normal pediatric human bladders is less contractile than that from adult bladders and exhibits greater passive stiffness. Reduced bladder contractile function is not due to reduced smooth muscle contractility but to greater connective tissue deposition and to functional denervation. Significant atropine resistance in pediatric detrusor, unlike in adult tissue, demonstrates a different profile of functional neurotransmitter activation. These data have implications for the management of pediatric bladder function by therapeutic approaches.

Dual Pathology Causing Congenital Bladder Outlet Obstruction

Anterior urethral syringocele is an uncommon congenital deformity characterised by cystic dilatation of bulbo-urethral gland ducts and is usually asymptomatic. We present a case on 4-day-old male neonate who presented with bilateral antenatal hydroureteronephrosis and renal impairment and found to have urethral syringocele and posterior urethral valves (PUV).

Cloacal Exstrophy Repair with Primary Closure of Bladder Exstrophy: A Case Report and Review of Literature

Cloacal exstrophy is the most complex congenital, ventral, abdominal wall defect. Traditionally surgery consists of a staged approach to repair which takes place on many separate theatre visits. In this case a primary approach was undertaken resulting in a relatively short inpatient stay and a reduced risk from multiple surgical procedures under general anaesthesia.