Testicular tumors in patients with exstrophy-epispadias complex.

PURPOSE: Due to separated pubic bone and patent processus vaginalis, males with exstrophy-epispadias complex often present with inguinal hernia during infancy. Since most of these testicles are operatively repositioned, testicular development is assumed to be normal. However, there is a paucity of knowledge about long-term testicular development in males with exstrophy-epispadias complex. We identified males with sonographic intratesticular abnormalities or testicular tumor in exstrophy-epispadias complex. MATERIALS AND METHODS: Since 2003, a Germany wide cross-sectional followup study has been permanently offered to men with exstrophy-epispadias complex, focusing on andrological issues. A total of 22 men with exstrophy-epispadias complex presented to our clinical service for andrological evaluation, including testicular ultrasound. RESULTS: Sonography showed testicular and epididymal pathology in more than 50% of patients, with intratesticular abnormality in 23%, most commonly testicular microlithiasis (9%). Three patients underwent testicular biopsy. Histopathological evaluation revealed 1 case of testicular intraepithelial neoplasia and 2 benign testicular stromal tumors (1 Sertoli cell tumor and 1 Leydig cell tumor). Followup visits at 10, 28 and 68 months were uneventful. CONCLUSIONS: The observation of comorbid testicular tumor in males with exstrophy-epispadias complex should prompt a preventive health examination after puberty, which gives these patients the opportunity for further appropriate diagnostics and treatment if necessary. Biopsy is recommended for sonographically detected intratesticular lesions. Organ sparing procedures are worth considering, especially when stromal tumors with favorable outcome are discovered. However, current oncologic principles must be strictly followed. Although the etiology and true incidence of testicular tumors in exstrophy-epispadias complex are still unclear, our findings highlight the importance of long-term followup in patients with exstrophy-epispadias complex.

Pregnancy management in women within the bladder-exstrophy-epispadias complex (BEEC) after continent urinary diversion.

BACKGROUND: Management of operative delivery in pregnant women after reconstruction of the bladder-exstrophy-epispadias complex (BEEC) using bowel segments remains a challenge. PATIENTS AND METHODS: We report urological history, pregnancy and delivery course of two BEEC patients after previous abdominal bowel surgeries. One had an ileocecal pouch after previously failed reconstruction, and the other had an ileum augmentation and a catheterizable Mitrofanoff stoma after functional reconstruction of the exstrophic bladder. RESULTS: Frequent bacteriuria and hydronephrosis warranted low-dose prophylaxis throughout pregnancy in one female, bilateral mild upper tract dilatation sonographic monitoring in both patients. Both were successfully delivered by cesarean section. No complications or clinical and sonographic signs for prolapse occurred. However, our operative experience revealed the importance of the abdominal incision type after different reconstructed reservoirs. CONCLUSION: Though care should be intense in pregnant BEEC individuals, patients should not be discouraged to have own children. To facilitate successful pregnancy outcome operative delivery should be done as a interdisciplinary team work and emergency situations should be avoided by meticulous planning and counseling of the BEEC patients.

Pelvic-floor imaging using three-dimensional ultrasonography and magnetic resonance imaging in the long term follow-up of the bladder-exstrophy-epispadias complex.

OBJECTIVE: To investigate whether established pelvic floor variables can be used to predict the risk of uterine prolapse after surgery and to compare two different operative strategies, pelvic adaptation with functional reconstruction and an open pelvis with urinary diversion, as a congenital bony pelvis and pelvic floor defect predisposes females with bladder-exstrophy-epispadias complex (BEEC) to uterine prolapse. PATIENTS, SUBJECTS AND METHODS: We conducted a cross-sectional study using perineal three-dimensional ultrasonography (3D-US) and magnetic resonance imaging (MRI) to describe pelvic floor anatomy in 19 women with BEEC (mean age 27.3 years) and five controls, with the outcome evaluated by a semi-structured interview. The analysis of 3D-US and MRI was conducted by two independent investigators. RESULTS: Of the 19 women with BEEC, 13 initially had a functional bladder reconstruction, six a urinary diversion with removal of the exstrophic bladder, and 12 women had closure of the pelvis either by traction bandage or osteotomy, and in the other seven the symphysis was not approximated. Four patients had a complete and one a mild uterine prolapse. After a mean follow-up of 24 years, the mean symphyseal diastasis was 4.5 cm after symphyseal approximation, 9.0 cm without symphyseal approximation in BEEC and 0.49 cm in controls. The mean levator hiatus was 4.9 cm on 3D-US and 4.1 cm on MRI after symphyseal approximation, 5.9 and 7.6 cm without symphyseal approximation and 4.2 and 3.2 cm in controls, respectively. The respective mean levator angle was 86.6 degrees and 87.3 degrees after symphyseal approximation, 104.1 degrees and 101.3 degrees without and 71.3 degrees and 45.5 in controls. Prolapse was statistically significantly more common in patients with no symphyseal approximation. CONCLUSION: This is the first study showing that perineal 3D-US is useful for pelvic floor imaging in BEEC. Established pelvic floor variables might be useful for predicting the risk of pelvic organ prolapse in BEEC.

Long-term follow-up of male patients after reconstruction of the bladder-exstrophy-epispadias complex: psychosocial status, continence, renal and genital function.

OBJECTIVE: There is a paucity of knowledge about long-term outcome issues in the bladder-exstrophy-epispadias complex (BEEC). Adult male BEEC patients were investigated in respect of bladder and renal function, fertility, genital function and psychosocial facts. PATIENTS AND METHODS: In a cross-sectional study, 17 adult male BEEC patients (mean age 23.4 years) from a single centre were evaluated with a questionnaire, renal and bladder ultrasound, blood tests, hormonal profile and semen analysis. RESULTS: Phenotypically one patient had complete epispadias and 16 had classical bladder exstrophy. Five patients underwent a one-stage functional reconstruction as a primary and 12 as a redo procedure. After a mean follow-up of 19.4 years, 15 bladders were preserved with 12 voiding per urethram and 3 performing intermittent catheterization; 2 were secondarily diverted. Significant residual urine was present in 10; kidneys were normal in 14 patients. Sixteen patients proved ejaculations, 3 had normospermia, 7 oligoasthenospermia and 6 azospermia. In patients with only one single bladder neck procedure normospermia was statistically significant. CONCLUSION: After functional BEEC reconstruction, long-term bladder function is preserved with mostly normal renal function. The number of bladder neck attempts has a significant influence on andrologic outcome. Detailed analysis may detect multifactorial pathogenesis from the impaired sperm quality in the BEEC.

Safety and tolerability of adjuvant topical tacrolimus treatment in boys with lichen sclerosus: a prospective phase 2 study.

BACKGROUND: Management of lichen sclerosus (LS) in boys is still controversial. Although in most cases only the prepuce is affected, meatal and urethral involvement may require major surgical reconstruction with substantial morbidity. OBJECTIVE: Because the frequency of such complicated courses is still unclear, an adjuvant postoperative treatment is highly desirable. Therefore, we addressed safety and tolerability of tacrolimus 0.1% ointment in the postoperative period. DESIGN, SETTING, AND PARTICIPANTS: Among 222 penile surgeries, in 25 cases LS was confirmed histologically and 20 of those patients participated in the adjuvant treatment study. Moreover, 18 patients of the same cohort showed a lichenoid inflammatory reaction pattern suggestive of early but not fully established LS. INTERVENTIONS AND MEASUREMENTS: Subsequent to the operation and after explicit information about off-label use, parents applied tacrolimus 0.1% ointment twice daily to the glans and the meatus for 3 wk in cases of proven LS. The 18 patients with possible early LS were followed up only without any treatment. Clinical follow-up was performed up to 13 mo (median). RESULTS AND LIMITATIONS: All 20 LS patients completed the topical treatment without any relevant side-effects. Two relapses occurred in the treatment group and were clinically cured with an additional 3-wk cycle of topical tacrolimus 0.1% ointment. None of the 18 early LS cases progressed to full-scale LS. CONCLUSIONS: This is the first study showing that tacrolimus 0.1% ointment applied immediately after surgery of fully established LS is a tolerable and most probably safe adjuvant novel treatment option. Because the therapy led to disease control in all treated individuals for >1 yr (median), this study establishes the groundwork for future trials with expanded treatment and follow-up periods to verify the true clinical benefit of tacrolimus in patients after LS surgery. Lichenoid tissue reactions suggestive of early LS seem to require no adjuvant treatment.

Genital and reproductive function in males after functional reconstruction of the exstrophy-epispadias complex--long-term results.

OBJECTIVES: Genital and reproductive function have a strong effect on the quality of life of adolescent and adult male patients with the exstrophy-epispadias complex (EEC). According to the limited available data, early exstrophy reconstruction, as well as recurrent infectious or operative trauma, have been responsible for the low fertility rates. METHODS: We evaluated 21 adult male patients with EEC. Of the 21 patients, 17 had undergone single-stage reconstruction, 1 had undergone a staged approach, 2 had primarily undergone urinary diversion, and 1 had only undergone external genital reconstruction of epispadias. All were evaluated with a semistructured questionnaire, clinical examination, ultrasonography, and hormonal and semen analyses. RESULTS: All patients reported erections, and 19 were sure about ejaculation. Of the 21 patients, 18 patients proved antegrade and 1 retrograde ejaculation; 2 patients were not able to retrieve their specimen. Fifteen patients had a regular testicular size, and four had unilateral and two bilateral small testes. Four had irregular intratesticular ultrasound findings. Of these 4 patients, 1 had a testicular intraepithelial neoplasia that was treated with radiotherapy. The hormonal analysis findings were normal for 17 patients and 4 had elevated follicle-stimulating hormone levels. The semen analysis showed normozoospermia in 3, asthenozoospermia in 5, oligo-asthenozoospermia in 6, and azoospermia in 5 patients. The seminal parameters included fructose 1441.8 microg/mL (normal 1200-4500), zinc 43.3 microg/mL (normal 70-250), and alpha-glucosidase 19.13 mU/mL (normal >20). CONCLUSIONS: Single-stage reconstruction with consequent placement of the colliculus seminalis in the posterior urethra results in normal ejaculation in 94.1% of patients with EEC. Because of the severely impaired sperm quality and hormonal findings, patients with EEC should be offered adequate diagnostic and treatment options.