Long-Term Functional, Cosmetic and Sexual Outcomes of Hypospadias Correction Performed in Childhood.

OBJECTIVE: Hypospadias surgery, especially when performed early in life, may have a significant impact on the urinary and sexual functions in an adult. Because the literature is still limited, this paper assesses long-term functional, cosmetic and sexual results of hypospadias repair performed in childhood. PATIENTS AND METHODS: The study includes 275 patients older than 12 years treated for a hypospadias by an Onlay, Mathieu, Duplay, or Duckett's technique between January 1990 and December 2000. Flowmetry results were retrospectively obtained from patients' charts. The Paediatric Penile Perception Score (PPPS), the Hypospadias Objective Scoring Evaluation (HOSE) and the IIEF-5 score (when older than 16 years old) questionnaires were used to assess cosmetic and sexual results. The PPPS is designed to assess both penile self-perception with regard to meatus, glans, skin and general appearance. The HOSE is a five-point scoring system designed to allow an objective appraisal of the outcome of hypospadias repair, based on evaluating meatal location, meatal shape, urinary stream, straightness of erection, and the presence and complexity of any complicating urethral fistula. RESULTS: Qmax were within age-adjusted references, independent of the surgical technique, with median (range) Qmax of 18.8 ml/s (range 3-45, n = 136). Patients expressed a high satisfaction for every single item of the penile perception scale (PPPS), with mean values between 2 (satisfied) and 3 (very satisfied). Eighty-two percent were satisfied or very satisfied of the overall evaluation of penile appearance. Eighty-one percent of patients had a normal erectile function (IIEF-5 >22; n = 35/43). CONCLUSIONS: Taking into account the limitation of a small number of patients resulting from a low 21% questionnaire's response rate, the results of this study align with previous reports from the literature and confirms that hypospadias repair using standard techniques results in acceptable functional, cosmetic and sexual outcomes. This study highlights the need of developing a set of standard approved outcomes assessments tools for evaluating the long-term impact of hypospadias repair performed in infancy.

[Multiple bladder diverticula caused by occipital horn syndrome]. / Diverticulose vésicale liée à un syndrome de la corne occipitale.

We report on the case of a child who presented with recurrent, multiple, and voluminous bladder diverticula. Bladder diverticula are defined as a herniation of the mucosa through the bladder muscle or the detrusor. Causes are numerous and diverticula can be classified into primary congenital diverticula (para-ureteral - or Hutch diverticula - and posterolateral diverticula); secondary diverticula (resulting from chronic mechanical obstruction or from neurological disease; and diverticula secondary to connective tissue or muscle fragility. The latter is seen in disease entities such as prune belly syndrome, Ehlers-Danlos syndrome, cutis laxa syndrome, OHS (occipital horn syndrome), Menkes disease, and Williams-Beuren syndrome. In this patient, the cause of these diverticula was OHS, a genetic, recessive X-chromosome-linked syndrome, responsible for abnormal tissue caused by a disorder in copper metabolism. This case reminds us of the importance of pushing the diagnostic workup when presented with multiple and/or large bladder diverticula, and in particular to search for rare malformation syndromes after exclusion of an obstacle.

Influence of ureteral status on kidney washout during technetium-99m-DTPA diuresis renography in children.

To assess the influence of the ureter on renal washout during 99mTc-DTPA diuresis renography, ureteral images were reviewed in 42 children (median age: 5 mo) referred for hydronephrosis. Sixty-minute acquisitions were obtained in hydrated patients under bladder drainage. Furosemide was injected at 30 min. An abnormal ureter was defined as an intense and continuous image of greater than 10 min. A washout index was determined on renal (KT1/2) and ureteral (UT1/2) curves. Curve patterns corresponding to normal (type I), obstructive (II) and nonobstructive (III) cases were described. Compared with the x-ray data, diuresis renography was highly sensitive (91%) and specific (98%) for detecting any abnormality. Despite an obstructive KT1/2 (greater than 20 min), no patient with an abnormal ureter underwent therapy at the ureteropelvic junction. After surgery at the lower level, hydronephrosis regressed. Our data indicate that abnormal ureter findings at diuresis renography have to be recognized before planning therapy for children with hydronephrosis.

Urothelial lesions in Chinese-herb nephropathy.

Rapidly progressive renal fibrosis after a slimming regimen including Chinese herbs containing aristolochic acid (AA) has been identified as Chinese-herb nephropathy (CHN). We reported urothelial atypia in three patients with CHN, with the subsequent development in one patient of overt transitional cell carcinoma (TCC). Therefore, it was decided to remove the native kidneys, as well as the ureters, in all patients with CHN. Nineteen kidneys and ureters removed during and/or after renal transplantation from 10 patients were studied to assess critically urothelial lesions and to characterize the cellular expression of p53, a tumor-suppressor gene overexpressed in several types of malignancies. Multifocal high-grade flat TCC in situ (carcinoma in situ; CiS) was observed, mainly in the upper urinary tract, in four patients, a prevalence of 40%. In one of those patients, a superficially invasive flat TCC of the right upper ureter, as well as two additional foci of noninvasive papillary TCC, were found in the right pelvis and left lower ureter, respectively. This patient also presented recurrent noninvasive papillary TCC of the bladder. Furthermore, in all cases, multifocal, overall moderate atypia was found in the medullary collecting ducts, pelvis, and ureter. All CiS and papillary TCC, as well as urothelial atypia, overexpressed p53. These results show that the intake of Chinese herbs containing AA has a dramatic carcinogenic effect. Carcinogenesis is associated with the overexpression of p53, which suggests a role for a p53 gene mutation. The relationship of this mutation with the reported presence of AA DNA adducts in the kidney remains to be explored.

Visual sexual stimulation plethysmography: complementary test to nocturnal penile plethysmography.

Diurnal penile plethysmography under visual sexual stimulation (VSS) and nocturnal penile tumescence (NPT) plethysmography have been performed in 5 healthy volunteers and 195 consecutive men complaining of impotence. Our study demonstrates that VSS plethysmography may compensate at least partially for the shortcomings of classic NPT plethysmography, by allowing direct clinical estimation of "erectile performance." Twenty percent of the patients had full erection under VSS. These patients do not need further testing. VSS is a noninvasive and useful "first-line" investigation in erectile dysfunction.

Laparoscopic nephrolithotomy: the value of intracorporeal sonography and color Doppler.

Laparoscopic nephrolithotomy was used as an alternative to open surgery in a patient who had failed extracorporeal shock-wave lithotripsy and whose anteriorly located stone-bearing calix precluded percutaneous extraction. Endocavitary ultrasonography and color Doppler render the procedure safe and effective; localization of the stone, selection of an optimal nephrotomy site away from large vessels and where cortical thickness is minimal, and control of fragment clearance are greatly facilitated.

Undetectable prostate-specific antigen in aging men.

Undetectable prostate-specific antigen was found in three aging men despite the absence of any prostatic surgery or exogenous hormonal deprivation. Clinical and elementary hormonal workup revealed the presence of secondary hypogonadism. This finding confirms the hormonal dependency of this prostatic marker and may, in some cases, explain the discrepancy between prostatic volume and the value of serum prostate-specific antigen.

Free to total prostate-specific antigen (PSA) ratio improves the discrimination between prostate cancer and benign prostatic hyperplasia (BPH) in the diagnostic gray zone of 1.8 to 10 ng/mL total PSA.

OBJECTIVES: Improved discrimination between prostate cancer (PC) and benign prostatic hyperplasia (BPH) is clearly needed. Our aim in this study was to evaluate whether the free to total prostate-specific antigen (PSA) ratio would be useful in the gray zone of 1.8-10 ng/mL total PSA range. METHODS: In a consecutive series of 435 clinic patients referred for prostate evaluation, 308 had a total PSA < 10 ng/mL (92 had PC and 216 BPH). Free and total PSA were measured, and the free to total PSA ratio calculated. RESULTS: Total PSA values were significantly different between the two groups. For the 200 patients with a total PSA < 6 ng/mL, no significant difference in total PSA values were seen (P = 0.411), whereas free to total PSA ratios remained statistically different (P < 0.001). Receiver operating characteristic (ROC) curve analysis comparing the performances of total PSA over the ratio of free to total PSA showed a clear advantage for the ratio at all sensitivity levels. CONCLUSIONS: These data demonstrate that in a significant number (n = 308) of prostatic patients in the diagnostic gray zone of 1.8-10 ng/mL total PSA, the routine use of free to total PSA might be advantageous in discriminating between cancer and benign hyperplasia. This advantage remained for total PSA < 4 ng/mL. Further study is warranted to confirm these findings in an unselected population.

Efficacy of laser prostatectomy at 2-years' follow-up.

A total of 32 patients with symptomatic benign prostatic hyperplasia were treated with the right-angle firing neodymium:YAG laser fiber at a 60 W power setting. All these patients represented a high operative risk (ASA score > or = 3) and were excluded by the anesthesiologist for classic surgical treatment. Twenty-six patients were followed at least 24 months; the six remaining patients died of intercurrent disease. Efficacy of treatment was assessed at 3, 6, 12, 18, and 24 months postoperatively by International Prostatic Symptom Scores, peak urinary flow rates, prostatic volume, and radiographic or endoscopic imaging of the prostatic urethra. The mean preoperative symptom score was 25, the mean peak urinary flow rate 7.6 mL/sec, and mean prostatic volume 31 cc. At 2 years postoperatively, the mean symptom score was 5, the mean peak urinary flow rate was 19.3 mL/sec, and the mean prostatic volume was 18 cc. In two elderly patients, in spite of a significant improvement of symptomatology and peak flow rate, a surprisingly obstructed prostatic urethra was found endoscopically.

Transurethral ultrasound-guided laser-induced prostatectomy: a critical evaluation.

We evaluated 38 patients with a follow-up of 30 months after transurethral ultrasound-guided laser-induced prostatectomy (TULIP) for benign prostatic hyperplasia. The mean symptom score decreased by 54%, and peak urinary flow increased by 112%. For the entire series, 43.6% of the patients had an improved symptom score and 41% better urinary flow, but only 28.2% had improvement in both. Six patients (16%) required reoperation, two underwent a radical prostatectomy, and one patient presented total urinary incontinence. Also, 19% presented postoperative impotence, and 47% presented retrograde ejaculation. Although one third of the patients are improved with the TULIP procedure, the rate of complications is significantly higher than for TURP, which remains the most effective treatment of obstructive BPH.

Vessels around the ureteropelvic junction: significance and imaging by conventional radiology.

We studied the influence of vessels crossing the ureteropelvic junction and of the grade of hydronephrosis in 86 patients who underwent endoureteropyelotomy. With a follow-up ranging from 1 to 12 years, the presence of crossing vessels had a significant negative influence on the outcome and recurrence rate of the procedure. The success rate was 82% when no vessels were present but only 33% when crossing vessels were documented. In 13 of 15 failures (87%), a crossing vessel was found at open pyeloplasty. The degree of hydronephrosis was a less potent detrimental factor, with an 81% success rate in low-grade and 54% in high-grade hydronephrosis. The best results (90% success) were obtained in patients with no crossing vessels and low-grade hydronephrosis and the worst (30% success) in those with high-grade hydronephrosis in the presence of crossing vessels. Selection of patients according to those prognostic factors is recommended. Conventional intravenous urographic imaging techniques proved insufficient, and only angiography provided diagnostic accuracy; less-invasive methods are desirable.

Diverticulum formation after circular myotomy for esophageal atresia.

Out of our last 70 cases of esophageal atresia, a circular myotomy of the upper pouch (Livaditis technique) was required in six patients to achieve an end-to-end anastomosis: all had a lower tracheoesophageal fistula. They were operated on between 15 and 96 hours after birth, without any preoperative attempt of elongation of the upper pouch. Follow-up ranged from 14 to 77 months. Three patients had an uneventful postoperative course and late outcome, despite a moderate ballooning at the myotomy site in one. Three babies developed a mild stricture which responded easily to a few dilatations. Asymptomatic ballooning at the myotomy site was observed in one baby; the other two developed a diverticulum responsible for acute respiratory distress in one case and severe dysphagia in the other. both required esophageal replacement. These observations call attention to the fact that patients treated by myotomy should be followed very carefully early and late, especially if an anastomotic stricture develops or in case of associated tracheomalacia.

[Initial diagnosis of anorectal malformations]. / Le diagnostic initial des malformations ano-rectales.

An essential prerequisite for the correct surgical approach of the congenital anorectal anomalies is a precise diagnostic work-up demonstrating the level of the atresia and the nature of possibly associated anomalies. To be acquainted with basic embryology allows a better understanding of this pathology. In this study the value of clinical and radiological parameters has been analysed in sixty cases. An accurate diagnosis was established in one half of the patients only by clinical examination. In the other half, the X-ray with the child inverted according to Wangensteen and Rice proved to be frequently unreliable in contrast with opacification technics. In boys, retrograde urethrography (personal modification) demonstrated the fistula and its location in two-thirds of the cases with a doubtful clinical examination; similarly vaginal and rectal opacification led to a correct diagnosis in one half of the girls. During these investigations with a contrast medium, attention should be focused on the puborectalis sling in contraction and relaxation and its relationship with the terminal part of the bowel. In the remaining cases a colostomy or dilatation of the fistula during the neonatal period allowed the deferral of the definitive diagnosis.

[Anomalies of the urogenital system associated with anorectal malformations]. / Anomalies du système urogénital associées aux malformations anorectales.

Urinary anomalies associated with anorectal malformations were found in more than one half of 61 consecutive cases treated during the last ten years. This well known high frequency stresses the necessity of systematic investigations in order to diagnose these associated anomalies early in the life. The high incidence of associated anomalies (40%) found in the group of translevator deformities makes careful clinical examination and complete radiological investigation mandatory in all cases. Vesico-ureteral reflux (14 cases), renal agenesis (6 cases), multicystic kidneys (2 cases), urogenital sinus (3 cases) and hypospadias (6 cases) were the most significant findings. A normal aspect of renal ultrasonography at birth should allow to postpone intravenous pyelography which can be tricky in the neonatal period.

[Radio-urodynamic study of the lower urinary tract]. / L'étude radio-urodynamique de l'appareil urinaire inférieur.

Radio-urodynamic examination of the lower urinary tract is the best method to study the complex functional problems involving those structures. It will very often provide a correct diagnostic orientation in a single session. The main indications are (1) urinary incontinence when symptoms are unclear, or recurring after surgical correction, (2) neurogenic bladder dysfunction, (3) incipient obstructive uropathy and (4) when it is necessary to ascertain that micturition is normal. The necessary investment in personnel, time and material is considerable, and the indications are therefore to be limited to complex problems and to certain centers that have a special clinical and research interest in urodynamics. It is nevertheless highly justified by the improvement in therapeutic results engendered by a correct and precise diagnosis.

[Reversal of urinary diversion after radical surgery for rhabdomyosarcoma of the bladder]. / Dé-dérivation urinaire après chirurgie radicale pour rhabdomyosarcome de la vessie.

The authors report a case of reversal of urinary diversion in a 14 year old boy who had presented, three years previously with an embryonal rhabdomyosarcoma of the base of the bladder. The initial treatment had consisted of preoperative and postoperative chemotherapy combined with radical cystoprostatectomy with urinary diversion via a colonic conduit. The principle of the technique of reversal of urinary diversion are discussed in the light of this unusual case.

[Ureteral lithiasis. Development of the therapeutic strategy and the place of endoscopic treatment]. / Lithiases urétérales. Evolution de la stratégie thérapeutique et place du traitement endoscopique.

In order to evaluate the impact of endourological techniques and extracorporeal lithotripsy on the management of ureteral calculi, we reviewed the charts of 838 patients requiring hospitalization for this pathology from 1982 till 1987. 314 patients were managed conservatively and 524 required intervention therapy: 52 with blind endoscopic techniques, 93 with open surgery, 122 with retrograde ureteroscopy, 168 with percutaneous extraction and 92 with ESWL. Evolution of the therapeutic strategy is discussed and our present approach to ureteral stone management is outlined.

[Urinary continence after radical prostatectomy: importance of the vesico-urethral anastomosis. Objective evaluation]. / Continence urinaire après prostatectomie radicale: importance de l'anastomose vésico-urétrale. Evaluation objective.

Urinary continence after radical prostatectomy is frequently evaluated subjectively. The authors describe their technique of vesicourethral anastomosis and report their method of objective evaluation of postoperative urinary continence. At the 6th postoperative week, 15% of patients presented with severe incontinence. This percentage fell to 3.3% by the 16th postoperative week.

Secondary retroperitoneal teratoma.

Retroperitonal teratomas are rare. We report on a case of a retroperitoneal secondary localisation of a gonadal teratoma in a patient who had developed primary testicular teratoma 12 years previously. The retroperitoneal mass was detected with an abdominal CT requested for the management of a non-specific abdominal pain. CT and MRI examinations showed cystic retroperitoneal masses combined with calcifications and peripheral enhancement. Review of the literature is presented, including the common differential diagnoses to be considered.