Sacral agenesis and neurogenic bladder: Long-term outcomes of bladder and kidney function.

BACKGROUND: Sacral agenesis (SA) is a rare congenital condition that refers to the absence of part or all of two or more lower sacral vertebral bodies. It can be associated with neurogenic bladder dysfunction that does not necessarily correlate with the level of spinal or skeletal defect. Patients with SA should undergo urodynamic studies (UDS) to guide lower urinary tract (LUT) management. OBJECTIVE: This review aimed to update the present institutional experience since 1981 of this rare patient population with detailed, long-term follow-up of bladder and kidney function. STUDY DESIGN: A single institution, retrospective, IRB-approved review was performed on patients born after January 1, 1981 with an isolated diagnosis of sacral agenesis without spina bifida, and followed with urologic involvement at Boston Children's Hospital. Records were reviewed for demographics, radiologic imaging, UDS including cystometrogram (CMG) and electromyography (EMG), surgery, and blood chemistries. Comparisons were made between groups of patients based on age at diagnosis, with specific focus on renal function and stability of neurogenic bladder lesion. RESULTS: Forty-three patients were identified: 23 female and 20 male. Thirty-seven children (86%) had a known age of diagnosis. Nineteen were diagnosed before 2 months old, including five who were diagnosed prenatally, 11 were diagnosed between 2 and 18 months, and seven were diagnosed after 18 months. All 43 had UDS, with 24 (55.8%) studied at the time of diagnosis (Summary Table). Twenty had serial full UDS, with 30% demonstrating neurourologic instability. None developed end-stage renal disease (ESRD) or required spinal cord detethering. DISCUSSION: Many children with SA appeared to be diagnosed prenatally or early in life; SA was mostly identified during evaluation of associated anomalies. Though UDS aid in urologic management, testing was not routinely utilized at the time of diagnosis. CONCLUSIONS: This review of long-term follow-up in SA patients showed stable LUT and renal function, with minimal risk of progression to ESRD.

Management of functional nonretentive fecal incontinence in children: Recommendations from the International Children's Continence Society.

BACKGROUND: Fecal incontinence (FI) in children is frequently encountered in pediatric practice, and often occurs in combination with urinary incontinence. In most cases, FI is constipation-associated, but in 20% of children presenting with FI, no constipation or other underlying cause can be found - these children suffer from functional nonretentive fecal incontinence (FNRFI). OBJECTIVE: To summarize the evidence-based recommendations of the International Children's Continence Society for the evaluation and management of children with FNRFI. RECOMMENDATIONS: Functional nonretentive fecal incontinence is a clinical diagnosis based on medical history and physical examination. Except for determining colonic transit time, additional investigations are seldom indicated in the workup of FNRFI. Treatment should consist of education, a nonaccusatory approach, and a toileting program encompassing a daily bowel diary and a reward system. Special attention should be paid to psychosocial or behavioral problems, since these frequently occur in affected children. Functional nonretentive fecal incontinence is often difficult to treat, requiring prolonged therapies with incremental improvement on treatment and frequent relapses.

International Children's Continence Society's recommendations for therapeutic intervention in congenital neuropathic bladder and bowel dysfunction in children.

PURPOSE: We present a consensus view of members of the International Children's Continence Society on the therapeutic intervention in congenital neuropatic bladder and bowel dysfunction in children. MATERIAL AND METHODS: Discussions were held by a group of pediatric urologists and gastroenterologists appointed by the board. The following draft review document was open to all the ICCS members via the ICCS web site. Feedback was considered by the core authors and by agreement, amendments were made as necessary. The final document is not a systematic literature review. It includes relevant research when available as well as expert opinion on the current understanding of therapeutic intervention in congenital neuropatic bladder and bowel dysfunction in children. RESULTS: Guidelines on pharmalogical and surgical intervention are presented. First the multiple modalities for intervention that do not involve surgical reconstruction are summarized concerning pharmacological agents, medical devices, and neuromodulation. The non-surgical intervention is promoted before undertaking major surgery. Indicators for non-surgical treatments depend on issues related to intravesical pressure, upper urinary tract status, prevalence of urinary tract infections, and the degree of incontinence. The optimal age for treatment of incontinence is also addressed. This is followed by a survey of specific treatments such as anticholinergics, botulinum-A toxin, antibiotics, and catheters. Neuromodulation of the bladder via intravesical electrical stimulation, sacral nerve stimulation, transcutaneous stimulation, and biofeedback is scrutinized. Then follows surgical intervention, which should be tailored to each individual, based on careful consideration of urodynamic findings, medical history, age, and presence of other disability. Treatments mentioned are: urethral dilation, vesicostomy, bladder, augmentation, fascial sling, artificial urinary sphincters, and bladder neck reconstruction and are summarized with regards to success rates and complications. Finally, the treatment on neuropathic bowel dysfunction with rectal suppositories irrigation and transrectal stimulation are scrutinized.

The management of adolescents with neurogenic urinary tract and bowel dysfunction.

Most children with neurogenic bladder dysfunction arrive into adolescence with reasonably managed lower urinary tract function only to experience bladder and kidney function deterioration after puberty. The aim of this article is to identify issues that contribute to adverse changes in bladder and renal function during adolescence and to highlight strategies to preserve urinary tract integrity, social continence, patient autonomy, and independence. Surveillance of bladder function requires patient attendance at review appointments and compliance with treatment plans. While encouraging independence and treatment compliance the clinician also needs to consider altered mental concentrating ability and fine motor skills of these patients. A keen eye for imminent loss of patient compliance to treatment protocol should be the mainstay of each encounter during puberty and adolescence. Annual surveillance of adolescent neurogenic bladder patients facilitates early identification of risk factors for urinary tract deterioration. Investigations include renal and bladder ultrasonography, urodynamic study when indicated, substantiated by videocystometry when anatomical status dictates. Serum creatinine should be measured and renal scintigraphy performed when upper urinary tract dilation, renal scarring, or atrophy are suspected. Optimal management of adolescents with neurologic disease of the urinary tract included strategies to reduce elevated detrusor pressure, maintain bladder compliance, and maximize dryness. Antimuscarinic medications, botulinum toxin A, and surgical procedures are enhanced by bowel management regimens and regular nurse or urotherapist patient contact. Caring for the patient as a whole requires discussion of sexuality, fertility status, and behaviors that increase the risk of progressive urinary tract damage.

Renal biopsy in congenital ureteropelvic junction obstruction: evidence for parenchymal maldevelopment.

The renal histologic changes associated with congenital ureteropelvic junction obstruction (UPJO) and the relationship to clinical imaging have not been well studied. In order to better understand the histologic alterations of congenital UPJO and their relationship with clinical imaging and outcomes, we examined renal biopsies from 61 patients undergoing pyeloplasty for congenital UPJO. Glomeruli were analyzed for various injury patterns and the tubulointerstitium was examined for tubular atrophy/simplification and fibrosis. Two methods were used to evaluate tubular mass: glomerular density and morphometric measurement of tubular size and density. Control specimens were obtained from age-matched autopsy specimens without renal pathology. Glomerular changes were identified in 73% of all biopsies and were present in a range from 1.7 to 91% of glomeruli in each patient. Overt tubulointerstitial changes were present in 26% of all biopsies. Fibrosis was noted to occur with tubulointerstitial changes in a significantly greater fraction of children over the age of 1 year (P=0.026). Increased glomerular density was associated with severe hydronephrosis (P<0.02). Normal glomerular density was inversely correlated with age (P<0.001), but this relationship was more variable in UPJO (P<0.01). Among patients with intact differential function preoperatively (>45%), postoperative functional decline was predicted only by increased glomerular density. 20 biopsies without overt tubulointerstitial changes were analyzed morphometrically and showed a significant reduction in proximal tubular (PT) size, but unchanged density. Distal tubular (DT) size was unchanged in UPJO, but density was increased. The PT/DT ratio was therefore markedly decreased in UPJO (P<0.0001). Both PT and DT sizes were significantly larger in children with a diuretic renogram washout time less than 20 min than those with greater than 20 min, a common threshold for functionally significant obstruction (P<0.05). Capsular thickness was significantly increased in UPJO. In all, 36% of biopsies had a thickness >0.5 mm and this was associated with greater degrees of tubulointerstitial changes and glomerular alterations. Congenital UPJO produces a variety of renal parenchymal changes, which may in part reflect abnormal development. Some of these alternations are seen in clinical imaging and may help predict outcomes, but there is significant discordance between conventional imaging and histological findings.

The outcome of voiding dysfunction managed with clean intermittent catheterization in neurologically and anatomically normal children.

OBJECTIVE: To describe the tolerability and efficacy of clean intermittent catheterization (CIC) in the management of dysfunctional voiding in patients who are neurologically and anatomically normal. PATIENTS AND METHODS: The medical records were reviewed in 23 patients (16 girls, mean age 9 years, range 6-14.5, and seven males, mean age 8 years, range 5-20.5) with urinary incontinence and/or urinary tract infection (UTI) who were offered CIC because they had a large postvoid residual urine volume (PVR). All had extensive instruction before starting CIC. All patients underwent urodynamic studies, and urinary and fecal elimination habits were recorded. Detrusor hyperactivity, when present, was treated with anticholinergic medication. The follow-up evaluation included tolerance of CIC, continence status and the incidence of UTI. Behavioural modification or biofeedback training was not used in any patient. RESULTS: Of the 23 patients, 13 presented with both UTI and urinary incontinence, five with incontinence only, four with UTI only, one with frequency and no incontinence, and one with haematuria. Associated symptoms included frequency/urgency, constipation or soiling, and straining to void or incomplete emptying (in nine each), and infrequent voiding in six. CIC was performed within 2 days by 15 patients, while four others required up to 2 weeks to master CIC. However, three of the four patients (all older girls) who needed 2 weeks to learn the technique did not tolerate CIC and discontinued it within 3 weeks. Four other adolescents (three girls and one boy) refused to learn CIC. Of the 16 patients remaining on CIC only three had cystitis; no patient had a febrile UTI. Once successfully instituted, all patients became continent while on CIC. Six boys (mean follow-up 4 months) had a marked decrease in their PVR. CIC was discontinued in three girls who voided normally to emptiness within 6 months of starting CIC; they remained dry and infection-free 16 months (two) and 6 years later. CONCLUSION: CIC is a viable therapeutic option for the treatment of dysfunctional voiding, associated with a large PVR, in the absence of any neurological abnormality. CIC is well tolerated in the sensate patient and provides a means for expeditiously achieving continence and improving bladder emptying cost-effectively.

New surgical techniques in pediatric urology.

More sophisticated endoscopic instruments, combined with a better understanding of bladder and urethral pathology, have significantly improved the therapeutic approaches for both posterior urethral valves and ureteroceles. New generation lithotripters have allowed for a safe and efficient method of treating urinary calculi in children, which was once thought too injurious a process with first-generation machines. The rapidly advancing field of laparoscopy, aided by the development of more optically refined and diminutive instruments, has allowed for its application in a wide variety of surgical interventions in pediatric urology. The tubularized incised plate urethroplasty has challenged more traditional approaches to hypospadias repair and is now considered by many pediatric urologists to be the best approach for midshaft and distal hypospadias. The one-stage approach to exstrophy repair may hold the answer to improved continence without a formal bladder neck reconstruction. Finally, the field of tissue engineering leads the way to new advances in autologous biological substitutes in the surgically-challenged patient where there is a shortage of local tissues at the surgeon's disposal.

Rectus fascial sling for the treatment of neurogenic sphincteric incontinence in boys: is it safe and effective?

PURPOSE: While a fascial sling for treating children with intractable urinary incontinence is often successful in girls, its effectiveness in boys remains unclear. We determined the long-term efficacy of a rectus fascial sling in boys with neurogenic sphincteric incontinence and defined its urodynamic characteristics for achieving continence. METHODS AND METHODS: We reviewed the charts of all boys who underwent a rectus fascial sling procedure for neurogenic incontinence to determine urinary continence status at the most recent office visit or by telephone interview, the type and dose of anticholinergic and sympathomimetic medications, the frequency of intermittent clean intermittent catheterization, status of the upper urinary tract and comparative urodynamic findings preoperatively and postoperatively. RESULTS: We evaluated 7 boys 7 to 19 years old, of whom 4 were postpubertal, who fulfilled study criteria and had a followup of 1 to 9 years. In 4 patients a continent stoma was created concurrently at surgery. Postoperatively all patients were dry during the first 3 months after surgery. At the last followup 1 patient was completely dry, 3 had occasional nighttime wetting, 2 had occasional stress incontinence, and 1 had frequent daytime and nighttime wetting requiring subsequent bladder neck closure. Prepubertal and postpubertal males performed catheterization without difficulty and all required less frequent clean intermittent catheterization and medication postoperatively compared to preoperative status. None had hydronephrosis. Postoperatively urodynamic evaluation revealed normal bladder compliance, improved urethral resistance that did not decay with bladder filling and no uninhibited contractions. CONCLUSIONS: The rectus fascial sling is effective for increasing bladder outlet resistance and decreasing the degree of incontinence in prepubertal and postpubertal males with neurogenic sphincteric incontinence. It has no long-term deleterious effects on bladder function and does not impair the ability to catheterize postoperatively. A fascial sling is an effective alternative to bladder neck closure when creating a continent stoma.

Tubularized incised plate urethroplasty: expanded use in primary and repeat surgery for hypospadias.

PURPOSE: We evaluated the impact of tubularized incised plate urethroplasty on primary and repeat hypospadias repair. MATERIALS AND METHODS: We retrospectively reviewed the medical records of all boys who underwent hypospadias repair at our institution during a recent 3-year period. The level of the hypospadias defect, technique of repair, primary repair versus reoperation, age at surgery and complications were recorded. RESULTS: A total of 520 hypospadias repairs were done from May 1996 through June 1999. We began to perform tubularized incised plate urethroplasty in November 1996. During the ensuing consecutive 32 months 181 primary and 25 repeat hypospadias repairs were done using this technique. Mean patient age at surgery was 22 months (range 3 months to 30 years). During the 6 months immediately before we began to use this method the Mathieu flip-flap procedure was the most commonly performed technique, accounting for 38% of all hypospadias repairs. In contrast, during the last 6 months reviewed tubularized incised plate urethroplasty accounted for 63% of all repairs, including 41 of 65 primary operations (63%) and 4 of 6 reoperations (67%), while no Mathieu procedures were performed. Postoperative followup was 6 to 38 months for tubularized incised plate repair. Overall meatal stenosis and a urethrocutaneous fistula developed in 1 and 14 boys, respectively (7% complication rate). CONCLUSIONS: Tubularized incised plate urethroplasty has become the preferred technique of primary and repeat hypospadias repair at our institution. The technique has few complications as well as proved success and versatility that continues to expand its applicability and popularity.

99m Technetium dimercapto-succinic acid renal scintigraphy abnormalities in infants with sterile high grade vesicoureteral reflux.

PURPOSE: Although vesicoureteral reflux associated with bacteriuria may cause renal scarring, sterile reflux is thought not to cause renal injury. We determined the incidence and associated characteristics of renal abnormalities using 99mtechnetium(Tc) dimercapto-succinic acid (DMSA) renal scintigraphy in infants with high grade vesicoureteral reflux but no history of urinary tract infection. MATERIALS AND METHODS: We retrospectively reviewed the results of 99mTc-DMSA renal scintigraphy and renal ultrasonography performed during the first 6 months of life in infants with vesicoureteral reflux detected during the postnatal evaluation of prenatal hydronephrosis or sibling reflux screening. Those with a history of urinary tract infection, or evidence of ureteropelvic junction or bladder outlet obstruction were excluded from study. RESULTS: Of the 28 male and 6 female infants who met study criteria vesicoureteral reflux was bilateral in 25 and unilateral in 9. Reflux grade was IV or V, II or III and I in 38, 18 and 3 of the 59 refluxing renal units, respectively. 99mTc-DMSA renal scintigraphy revealed parenchymal abnormalities in 24 refluxing renal units (41%) in 22 patients (65%), of whom 19 (86%) were male and 15 (68%) had bilateral reflux. We noted differential uptake less than 40% with and without cortical defects in 10 and 7 refluxing units, respectively, and cortical defects only in 7. Of the 24 refluxing units with abnormalities 21 were associated with grade IV or V and 3 with grade II or III reflux. Ultrasound showed evidence of renal injury in only 7 of the 17 patients (41%) in whom 99mTc-DMSA scintigraphy was abnormal. CONCLUSIONS: In our study the majority of infants with high grade reflux had decreased differential function and/or cortical defects. Parenchymal defects detected by 99mTc-DMSA renal scintigraphy were often not identified by renal ultrasound. Therefore, 99mTc-DMSA renal scintigraphy is especially useful for initially evaluating infants with high grade, sterile vesicoureteral reflux.

Initial experience with the transurethral self-detachable balloon system for urinary incontinence in pediatric patients.

PURPOSE: A new endoscopic technique to treat urinary incontinence in children using a self-detachable balloon device was studied. MATERIALS AND METHODS: The study includes 11 patients with a mean age of 14.6 years and all of whom had intrinsic sphincter deficiency due to myelomeningocele in 9, spinal artery bleed in 1 and cloacal exstrophy in 1. All patients were on clean intermittent catheterization preoperatively and postoperatively. Endoscopic balloon treatment was performed on an outpatient basis. A mean of 5 balloons (range 2 to 8) were placed per patient. All patients underwent formal urodynamic study preoperatively and at 6 weeks and 6 months following balloon placement. RESULTS: Of the 9 patients without prior bladder neck surgery 7 had improvement in urodynamic parameters, including urethral pressure profile in all 7 and functional bladder capacity in 6, 4 were markedly improved clinically and 2 were dry. Two patients with prior bladder neck surgery were clinically unchanged following balloon placement, although 1 had urodynamic improvement. CONCLUSIONS: Our initial experience with the transurethral self-detachable balloon system as a minimally invasive outpatient procedure to treat urinary incontinence in children has been encouraging. To date this procedure appears most applicable to the patient who has not undergone surgery and has a neurogenic etiology for urinary incontinence.

Comparative assessment of pediatric testicular volume: orchidometer versus ultrasound.

PURPOSE: Testicular volume measurements obtained with the Prader and Rochester orchidometers were compared to those obtained using scrotal ultrasound. The ability of each orchidometer versus ultrasound in detecting volume differential between 2 testes and the accuracy of orchidometer measurement by a less experienced examiner to that of a urologist were compared. MATERIALS AND METHODS: A total of 65 males were examined by the attending urologist and urology nurse using the Prader and Rochester orchidometers, and scrotal ultrasound was subsequently performed by an attending radiologist. Statistical analysis of the results was performed to determine the correlation of orchidometer measurements between examiners, as well as with ultrasound, and sensitivity and specificity of orchidometer and ultrasound in detecting defined volume differentials between testes of 10%, 15%, 20% and 25%. RESULTS: There was a strong linear relationship between testicular volume measurements using either orchidometer and ultrasound. To detect a defined volume differential as determined by ultrasound orchidometer sensitivity was weak, whereas orchidometer specificity was better. There was a strong correlation between orchidometer measurements of the urology nurse and attending urologist. CONCLUSIONS: Although the orchidometer remains valuable in assessing size of the individual testis, it is too insensitive to volume differentials relative to ultrasound to be used routinely to determine growth impairment. For this reason observation of an adolescent with varicocele should include an annual ultrasound assessment of testicular volume.

Function of hollow viscera in children with constipation and voiding difficulties.

We wished to investigate the urodynamic characteristics and colonic motility in a group of children with severe chronic constipation and lower urinary tract symptoms. We performed colonic manometry using an endoscopically placed catheter. The urodynamic studies consisted of cystometry, electromyography of the external urethral sphincter, measurement of urinary flow rate, and urethral pressure profile. We found abnormal colonic motility in all patients. Findings included: absent gastrocolonic response (N = 8), absent high-amplitude propagated contractions (HAPCs) (N = 4), and abnormal propagation of HAPCs (N = 7). Urodynamic features were abnormal in 10 children. Findings included: uninhibited bladder contractions (N = 6), hypertonic bladder (N = 2), sphincter dyssynergy (N = 2), small capacity bladder (N = 1). In all children constipation improved, in three after a partial colectomy. Urinary symptoms persisted. We conclude that some children with severe constipation may have a neuropathy affecting both the colonic and lower urinary tracts systems. In this group of patients treatment of constipation does not result in resolution of urinary symptoms.

Ketorolac suppresses postoperative bladder spasms after pediatric ureteral reimplantation.

UNLABELLED: We evaluated the efficacy of ketorolac in suppressing postoperative bladder spasms after ureteroneocystostomy (ureteral reimplantation). Twenty-four pediatric patients undergoing intravesical ureteroneocystostomy were enrolled prospectively to receive either ketorolac or placebo via double-blinded randomization. Twelve patients in each group shared similar preoperative characteristics. All were maintained on an epidural infusion of bupivacaine (0.1%) with fentanyl (2 microg/mL) throughout the study. Patients were given either ketorolac (0.5 mg. kg(-1). dose(-1)) or placebo (equivalent volume saline) IV after surgery and every 6 h thereafter for 48 h. Parents were instructed to record bladder spasm episodes prospectively by using a standardized time-flow diary. Three patients (25%) in the ketorolac group experienced bladder spasms, compared with 10 patients (83%) in the placebo group (two-sided P < 0.05). The median severity score for the ketorolac group was 1.2 (mild = 1.0, severe = 3.0), compared with 2.6 for the placebo group (P = 0.003). We conclude that IV ketorolac reduces the frequency and severity of postoperative bladder spasms after intravesical ureteroneocystostomy. IMPLICATIONS: We studied the efficacy of ketorolac, a prostaglandin synthesis inhibitor, in the treatment of bladder spasm after ureteroneocystostomy (antireflux operation). Patients were randomized in a double-blinded manner to receive either ketorolac or placebo after the surgery. We demonstrate that ketorolac reduces the frequency and severity of postoperative bladder spasm.

The effect of surgery for split spinal cord malformation on neurologic and urologic function.

The split spinal cord malformation (SSCM) is an occult spinal dysraphism which causes tethering of the spinal cord. We performed a retrospective analysis of 15 patients who had split cord malformations (without associated open neural tube defect) who underwent both pre- and postoperative urodynamic studies (UDS) in order to determine if a significant percentage of these patients, even in the absence of overt urologic symptoms, had evidence of urologic dysfunction. Eleven patients presenting in early childhood and 4 patients presenting later in life are reviewed. Despite the lack of preoperative urologic symptoms in almost all patients, 73% of patients had voiding abnormalities on formal testing. UDS on these patients before and after surgery was a useful adjunct to perioperative management and decision making, helped define the success of surgery, and gave objective information for cases in which retethering was suspected.

The overactive bladder in childhood: long-term results with conservative management.

PURPOSE: Idiopathic detrusor overactivity has not been thoroughly investigated and its natural history remains largely anecdotal. Bladder overactivity resulting from a neurogenic, anatomical or medical condition has been well described. Therefore, we assessed the long-term results of conservative treatment of children with idiopathic symptomatic refractory detrusor instability. MATERIALS AND METHODS: We reviewed the records of 58 patients who had an isolated finding of uninhibited contractions on urodynamics performed for refractory enuresis and daytime wetting between 1988 and 1994. Study exclusion criteria were chronic urinary tract infection, neurological lesion, anatomical abnormality of the lower urinary tract and less than 12 months of followup. RESULTS: Of the 30 children who met our study inclusion criteria 26 (87%) had complete (21) or significant (5) symptom resolution. Average time to resolution was 2.7 years (range 0.2 to 6.6). Patients with a 50% to 90% bladder capacity expected for age were more likely to benefit from therapy than those with a bladder capacity outside of this range. Age and gender were not significant predictors of resolution although girls were more likely to have resolution than boys. CONCLUSIONS: Idiopathic detrusor instability is amenable to conservative management in the majority of patients during a prolonged period. We advocate thorough urological and urodynamic evaluation to identify idiopathic detrusor instability as an etiology of enuresis and daytime wetting in complicated cases.

Normal urodynamics in patients with bladder exstrophy: are they achievable?

PURPOSE: Urodynamic study was performed in patients with exstrophy to determine the effect of bladder neck reconstruction and the ability to achieve normal urodynamics following surgery. MATERIALS AND METHODS: A total of 30 exstrophy cases at different stages of reconstruction were retrospectively reviewed in terms of bladder capacity, compliance, stability and presence of detrusor contractions following urodynamic study. RESULTS: Bladder capacity increased from a third predicted volume for age to half after reconstruction. Approximately 80% of patients had compliant and stable bladders before bladder neck reconstruction. Following bladder neck reconstruction approximately half of the patients maintained normal compliance with a smaller number maintaining normal stability. A quarter of patients maintained normal filling dynamics following bladder neck reconstruction, and 19% maintained normal filling and voiding dynamics after reconstruction. CONCLUSIONS: The majority of closed exstrophy bladders have normal filling dynamics before bladder neck reconstruction. Compliance and stability are impaired following bladder neck reconstruction. Approximately 25% of patients with exstrophy may maintain normal detrusor function following reconstruction. However, less invasive alternatives to the Young-Dees-Leadbetter bladder neck reconstruction should be sought.

The incidence of intersexuality in children with cryptorchidism and hypospadias: stratification based on gonadal palpability and meatal position.

PURPOSE: The combined findings of cryptorchidism and hypospadias often indicate the existence of an intersex state. Testicular maldescent and incomplete tubularization of the urethral plate occur in a spectrum with the severity of the 2 processes likely dependent on the degree of pathophysiology in the androgenic hormonal axis. The incidence of intersexuality in children with undescended testes, hypospadias and otherwise nonambiguous male genitalia has been reported to be 27%. Although the likelihood of genotypic or gonadal ambiguity has previously been associated with meatal position in this population, to our knowledge our study is the first to evaluate the incidence of intersexuality relative to whether the undescended testis is palpable or nonpalpable. MATERIALS AND METHODS: The database at our hospital was searched for all cases of undescended testes (2,105) and hypospadias (1,057) between 1982 and 1996. Radiographic, histological and karyotypic data were compiled for all patients presenting with both diagnoses. Gonadal palpability (palpable versus nonpalpable) and meatal position (anterior versus mid versus posterior) were recorded and correlated with the likelihood of identifying an intersex condition. Ten boys with a diagnosis of undescended testes subsequent to inguinal hernial repair were excluded from study. Patients with congenital adrenal hyperplasia or complete testicular feminization were also excluded from study due to the clearly female appearance of the external genitalia. Statistical significance was assessed using Fisher's exact test. RESULTS: We identified 79 patients presenting with undescended testes, hypospadias and a phallus that was believed to be a possible penis. Intersex conditions were identified with nearly equal frequency in the 44 cases of unilateral (30%) and 35 of bilateral (32%) cryptorchidism. In the unilateral undescended testes group patients with a nonpalpable testis were at least 3-fold more likely to have an intersex condition than those with a palpable undescended testis (50 versus 15%, p = 0.02). In the bilateral group patients with 1 or more nonpalpable testes were also nearly 3-fold as likely to have an intersex condition than those with bilateral palpable undescended gonads (47 versus 16%, p = 0.07). Meatal position was graded as anterior in 33% of cases, mid in 25% and posterior in 41% with the more posterior location conferring a significantly greater likelihood of an intersex condition (anterior 2 of 26, mid 1 of 20 and posterior 21 of 33). CONCLUSIONS: Gonadal palpability is an important predictor of an intersex state in unilateral and bilateral cases of cryptorchidism with hypospadias. Patients with an undescended testis that cannot be palpated are significantly more likely to have an intersex condition than those in whom the undescended testis may be palpated on physical examination. The severity of hypospadias likewise has a strong positive correlation with an intersex state.