Mark R. Zaontz

Surgical Correction of the Buried Penis: Description of a Classification System and a Technique to Correct the Disorder

The concealed penis is a long-standing problem that only recently has begun to receive the attention it deserves. We offer a classification for this general disorder, which facilitates the selection of appropriate surgical procedures for these patients. To correct the most common problem, the buried penis, involves removal of localized deposits of fat from the hypogastrium with open surgical or closed suction techniques followed by anchoring of the skin of the base of the penis to the periosteum of the pubis. During the last year we have used this approach successfully in 7 boys with various forms of penile concealment with good results.

The Influence of Small Functional Bladder Capacity and and Other Predictors on the Response to Desmopressin in the Management of Monosymptomatic Nocturnal Enuresis

PURPOSE The relationship of functional bladder capacity as well as other variables to the responsiveness to desmopressin in children with monosymptomatic nocturnal enuresis was investigated. MATERIALS AND METHODS A total of 95 children 8 to 14 years old with monosymptomatic nocturnal enuresis (6 or more of 14 nights wet) were evaluated in a double-blind study followed by open label crossover extension using 20 to 40 mcg. desmopressin. Evaluated predictors of response included patient age, gender, race, family history, number of baseline wet nights, urine osmolality parameters and maximum functional bladder capacity (as a percent of predicted bladder capacity based on the formula, patient age + 2 x 30 = cc). Responders to desmopressin were classified as excellent (2 or less of 14 nights wet) or good (50% or greater decrease but more than 2 of 14 nights wet) and nonresponders were defined by a less than 50% decrease in wet nights. RESULTS Of the 95 patients 25 (29.5%) achieved an excellent response to desmopressin and 18 (18.9%) had a good response for a cumulative response rate of 45.3%. The remaining 52 patients (54.7%) were nonresponders. There were no significant differences between responders and nonresponders in regard to gender, race, positive family history or baseline urine osmolality parameters. Response to desmopressin was associated with older age, fewer baseline wet nights and larger bladder capacity. Patients with a functional bladder capacity greater than 70% predicted bladder capacity were 2 times more likely to respond to desmopressin. CONCLUSIONS The responsiveness of children with nocturnal enuresis to desmopressin is adversely affected by reduced functional bladder capacity. The results of this study have implications regarding the potential use of combination pharmacotherapy with desmopressin and an anticholinergic for enuretic patients who are nonresponsive to single drug therapy.

KIDNEY TRANSPLANTATION IN CHILDREN WITH URINARY DIVERSION OR BLADDER AUGMENTATION

PURPOSE Urinary tract anomalies or dysfunction leaves the bladder unsuitable for urine drainage in a significant proportion of children presenting for kidney transplantation. We reviewed a multi-institutional experience to determine the ramifications of kidney transplantation in children with bladder augmentation or urinary diversion. MATERIALS AND METHODS During a 28-year period 18 boys and 12 girls 1.7 to 18 years old (mean age 12.1) received 31 kidney transplants. Cause of end stage renal disease was renal dysplasia in 8 cases, posterior urethral valves in 5, obstructive uropathy in 5, neurogenic bladder/chronic pyelonephritis in 4, spina bifida/chronic pyelonephritis in 3, prune belly syndrome in 3 and reflux in 2. RESULTS Of the patients 17 had augmented bladder (ileum 9, ureter 5, sigmoid 2 and stomach 1), 12 had incontinent urinary conduits (8 ileum, 6 colon) and 1 had a continent urinary reservoir. Surgical complications included 1 case each of stomal stenosis, stomal prolapse, renal artery stenosis, urine leak, enterovesical fistula and wound dehiscence. Medical complications included urinary tract infection in 21 cases and metabolic acidosis in 5. A bladder stone developed in 1 patient. There was no correlation between the incidence of symptomatic urinary tract infections and type of urinary drainage. Acidosis was more common in patients with augmented bladder (4 of 17 versus 1 of 14) but there was no correlation between the bowel segment used and the occurrence of acidosis. Graft survival was 90% at 1 year, 78% at 5 years and 60% at 10 years. Etiology of graft loss included chronic rejection in 6 cases, noncompliance in 4 and acute rejection in 1. There were no deaths. CONCLUSIONS Drainage of transplanted kidneys into an augmented bladder or urinary conduit is an appropriate management strategy when the native bladder is unsuitable or absent. Patients with kidney transplants drained into augmented bladder or urinary conduit are at increased risk for urine infection. Graft survival is not adversely affected compared to historical controls when a kidney transplant is drained into a urinary conduit or augmented bladder.

The GAP (Glans Approximation Procedure) for Glanular/Coronal Hypospadias

A modified glanuloplasty is described for the selective repair of glanular and coronal hypospadias with a wide, deep glanular groove and noncompliant urethral meatus. No urinary diversion is required, and cosmetic and functional results are excellent. A total of 24 children underwent the GAP (glans approximation procedure) during 20 months. Followup ranged from 3 months to 1.5 years with no evidence of meatal or urethral stenosis. One distal glanular fistula developed that required division of a 2 mm. skin bridge that separated the fistulous opening from the neomeatus.

Detrusorrhaphy: Extravesical Ureteral Advancement to Correct Vesicoureteral Reflux in Children

We used a modified extravesical technique, coined detrusorrhaphy, to correct surgically vesicoureteral reflux. By detrusorrhaphy the submucosal ureteral tunnel is opened, the ureteral meatus is advanced and anchored onto the trigone, and the detrusor buttress of the ureter is closed (-rrhaphy). The operation is performed extravesically. The procedure was used in the last 5 years in 79 children, or 120 renal units. Reflux resolved in 93 per cent of the renal units. Postoperative morbidity related to bladder spasms and hematuria was minimal compared to conventional transvesical surgical procedures. Detrusorrhaphy is an effective method to correct vesicoureteral reflux and to minimize postoperative morbidity.

Stent-Free Thiersch-Duplay Hypospadias Repair With the Snodgrass Modification

PURPOSE Incision of the glanular urethral plate (the Snodgrass modification) permits tubularization of the neourethra in the Thiersch-Duplay fashion when anatomy would otherwise preclude a tension-free anastomosis and necessitate another operative technique for hypospadias repair. To take advantage of these cosmetic benefits without the potential morbidity associated with indwelling catheters, we performed a stent-free Thiersch-Duplay repair incorporating the Snodgrass modification. MATERIALS AND METHODS The Thiersch-Duplay hypospadias repair was combined with the Snodgrass modification. Hinging the urethral plate was necessary when the glanular groove was too shallow to perform a standard Thiersch-Duplay repair. RESULTS Stent-free repairs were performed in 33 children 0.47 to 2.66 years old (mean age plus of minus standard deviation 0.98 +/- 0.47). Followup was obtained in 31 children. There was no postoperative urinary retention, fistulas or meatal stenosis. No unusual or prolonged discomfort distinguished these children from those who underwent a standard Thiersch-Duplay repair. CONCLUSIONS Excellent cosmetic results can be anticipated irrespective of the preoperative glans configuration. Incision of the glanular urethral plate can be performed safely as an adjunct to a modified Thiersch-Duplay hypospadias repair without postoperative indwelling catheters.

Fate of renal allografts transplanted in patients with urinary diversion

Fifty-five kidneys were transplanted into 50 patients with supravesical urinary diversion at 16 transplant centers between 1970 and 1991. Of the 32 males and 18 females, 40 were adults (≥18 years) and 10 were less than 18 years old at the time of first transplant. Mean follow-up was 7.8 years. At last follow-up, 94% of recipients were alive and 73% of the kidneys were functioning. Fifteen kidneys were lost: 9 to rejection, 3 to noncompliance, and 3 patients died with a functioning kidney. Ten (18%) transplants were followed by surgical complications. Twenty-four (44%) were followed by medical complications of which urinary tract infection was most common. Recipients age 18 or younger had more urinary tract infections than older patients. No patient had urinary stones and no patient required medical treatment for metabolic abnormalities. We conclude that drainage of kidney transplants into a supravesical urinary diversion is an effective treatment for end-stage renal disease patients without adequate urinary bladders.

Surgery for Duplex Kidneys with Ectopic Ureters: Ipsilateral Ureteroureterostomy versus Polar Nephrectomy

Conventional surgery for the ectopic ureter stresses polar nephrectomy, while preserving a functioning upper pole by ipsilateral ureteroureterostomy is performed less commonly. During the last 15 years we operated on 35 children for ectopic ureter. High ipsilateral ureteroureterostomy (15 patients) was performed when the upper pole cortex appeared to be smooth and pink, and the anastomosis was surgically feasible. Upper pole nephrectomy and upper ureterectomy (21 patients) were performed when the involved segment appeared grossly to be pale, cystic or otherwise abnormal. One low ipsilateral ureteroureterostomy was performed concomitant with contralateral ureteral reimplantation. Complications after ipsilateral ureteroureterostomy and partial nephrectomy were similar. Histological evidence of dysplasia of the polar nephrectomy specimens was focal in 7 patients (33 per cent), marked in 2 (10 per cent) and not detected in 12 (57 per cent). Ipsilateral ureteroureterostomy is an appropriate means to manage the ectopic ureter in selected cases because dysplasia in these upper pole renal units seldom is significant, and because ipsilateral ureteroureterostomy and polar nephrectomy have similar postoperative morbidity rates.

Acute focal bacterial nephritis: a systematic approach to diagnosis and treatment.

Acute focal bacterial nephritis, synonymous with acute lobar nephronia or focal nonliquefactive pyelonephritis, represents a localized area of renal inflammation. Clinically, acute focal bacterial nephritis presents as acute pyelonephritis but is distinguishable by the presence of a focal mass on excretory urography. The further distinction between acute focal bacterial nephritis and other renal masses is aided by the appropriate use of renal sonography and computerized tomography. The clinical and imaging manifestations in 9 patients with acute focal bacterial nephritis are described. Our experience coupled with a review of the literature suggests that a systematic approach to the diagnosis and management of acute focal bacterial nephritis allows for the most efficacious use of the noninvasive imaging modalities.

Cryptorchidism, pediatricians, and family practitioners: Patterns of practice and referral

A multicenter study was undertaken to study cryptorchidism and the timing of orchidopexy. A total of 329 children underwent surgery at a mean age of 4.2 years; 17% of the surgery was performed between 6 and 12 months of age, 25% between 5 and 10 years of age, and 9% during or after puberty. Only 30% of the pediatricians and 14% of the family practitioners recommended orchidopexy between 6 and 12 months of age, and 17% of these referring physicians recommended waiting until 3 to 10 years of age. Improved education is needed if current recommendations for early orchidopexy are to be achieved.