[Reconstruction of bladder exstrophy: retrospective study of 57 patients with evaluation of criteria in favor of the acquisition of continence]. / Reconstruction de l'exstrophie vésicale: étude rétrospective de 57 patients avec évaluation des critères favorisant l'acquisition de la continence.

In order to evaluate the results of staged surgical reconstruction in terms of urinary continence, spontaneous voiding and preservation of the upper urinary tract, we reviewed the charts of 42 boys and 15 girls treated at Hôpital Saint-Joseph for bladder exstrophy between 1965 and 1995. All patients underwent staged repair, associating bladder closure, bladder neck plasty, then genitoplasty, including bladder augmentation in 7 cases (12%). Criteria for good outcome in terms of continence are defined and factors influencing outcomes are reviewed. A total of 38 patients (67%) achieved good or acceptable urinary continence (22 or 39% and 16 or 28%, respectively). Outcome was poor in 19 patients, including 13 (23%) who underwent secondary urinary diversion. Bladder stones, which developed in 13 patients (23%), were the commonest long-term complication of bladder exstrophy reconstruction. Ten patients who underwent pelvic osteotomies ultimately had better continence and 9 are dry. Bladder neck reconstruction was performed at a later age overall (mean 10 years). Repeat bladder neck reconstruction was generally associated with poor results. The technique of bladder neck reconstruction did not appear to influence outcome. This retrospective study confirms that a carefully planned surgical reconstruction for bladder exstrophy can lead to satisfactory long-term urinary continence in most patients. Factors contributing to successful results include early bladder closure, pelvic osteotomy, adequate bladder neck reconstruction with bladder neck suspension in girls, and a motivated child and family. Alternatives to surgical reconstruction should be discussed. Ultimate predictors of outcome in bladder exstrophy repair are difficult to ascertain.

Bladder exstrophy: evaluation of factors leading to continence with spontaneous voiding after staged reconstruction.

PURPOSE: We performed a long-term retrospective review of patients with bladder exstrophy to evaluate the results of staged surgical reconstruction in regard to urinary continence, spontaneous voiding and preservation of the upper urinary tract. MATERIALS AND METHODS: We reviewed the charts of 42 boys and 15 girls treated at Hôpital St. Joseph for bladder exstrophy between 1965 and 1995. All patients underwent staged repair, including bladder augmentation in 7 (12%) and secondary urinary diversion in 13 (23%). Criteria for good outcome in terms of continence are defined and factors influencing outcomes are reviewed. RESULTS: A total of 38 patients (67%) achieved good or acceptable urinary continence (22 or 39% and 16 or 28%, respectively). Outcome was poor in 19 patients, including 13 (23%) who underwent secondary urinary diversion. Bladder stones, which developed in 13 patients (23%), were the most common long-term complication of bladder exstrophy reconstruction. Ten patients who underwent pelvic osteotomies ultimately had better continence and 9 are dry. Bladder neck reconstruction was performed at a later age overall (mean 10 years). Repeat bladder neck reconstruction was generally associated with poor results. The technique of bladder neck reconstruction did not appear to influence outcome. CONCLUSIONS: A carefully planned surgical reconstruction for bladder exstrophy can lead to satisfactory long-term urinary continence in most patients. Factors contributing to successful results include early bladder closure, pelvic osteotomy, adequate bladder neck reconstruction with bladder neck suspension in girls, and a motivated child and family. Alternatives to surgical reconstruction should be discussed. Ultimate predictors of outcome in bladder exstrophy repair are difficult to ascertain.

[Varicocele in children and adolescents. Review of a series of 58 cases]. / Varicocèle de l'enfant et de l'adolescent. Mise au point à propos d'une série de cinquante-huit cas.

5 to 25% of male adolescents present with a varicocele; only those cases inducing serious discomfort or affecting the development of the testis require surgical treatment. Nevertheless, the results of surgery are disappointing, with a high recurrence rate. Based on a better knowledge of testicular venous drainage, it appears that conventional high ligation of the spermatic vein (s) might not be sufficient to cure varicocele. Fifty-eight patients were operated upon using four different procedures. Only complete, proximal ligation of all of the visibly dilated veins seems to prevent recurrences with a follow-up of at least one year. However, out of eleven cases treated with this original procedure, one patient, already operated upon twice without success, developed secondary atrophy.

[Varicocele in childhood and adolescence. Evaluation apropos of a series of 58 cases]. / Varicocèle de l'enfant et de l'adolescent. Mise au point à propos d'une série de cinquante-huit cas.

5 to 25% of the male adolescents present with a varicocele; only those cases inducing serious discomfort or affecting the development of the testis require surgical treatment. Nevertheless the results of surgery are disappointing with a high recurrence rate. Based on a better knowledge of the testicular venous drainage, it appears that the conventional high ligation of the spermatic vein(s) might not be sufficient to cure the varicocele, fifty-eight patients were operated upon using four different procedures. Only a complete and proximal ligation of all of the visibly dilated veins seems to prevent recurrences with a follow-up of at least one year. However, out of eleven cases treated with this original procedure, one patient, already operated upon twice without success, developed secondary atrophy.

Spontaneous hematoma of the kidney simulating a nephroblastoma. Report of two cases.

The International Society of Pediatric Oncology (SIOP) 6 clinical trial for Wilms' tumor (WT) includes preoperative chemotherapy for all nonmetastatic patients. Approximately 50% of patients treated in this manner can be classed stage 1 after surgery. This strategy requires a positive diagnosis in the absence of histologic data. Diagnostic errors are very rare: Only 1.5% of the first 856 cases entered in the SIOP 6 trial were found to be benign. This article reports two very similar cases of spontaneous hematoma of the kidney that were initially misdiagnosed as hemorrhagic WT. Unusual severe acute anemia was a feature of both cases. Even the most modern state-of-the-art imaging techniques were unable to rule out the possibility of a tumor subjacent to a voluminous renal hematoma. The patients' courses during preoperative chemotherapy were of no formal diagnostic value, and the correct diagnosis was not made until surgery.

[Transureteroureterostomy. Results in a series of 42 children and young adults]. / Transurétérourétérostomie. Résultats d'une série de quarante-deux enfants et adultes jeunes.

Forty two TUU have been performed from 1974 to 1986 in children and young adults: the youngest was 10 years, the oldest 26. The mean follow-up has been more than 5 years: 23 bladder exstrophies, 12 neurogenic bladders, 5 mega-ureters and 2 retroperitoneal tumors. TUU were performed after failure of uretero-cysto-neostomies (8/42) ou uretero-sigmoid reimplantation (19/42). The technique with details about drainage (16/42) is described. In 5 cases, complications appeared early: 3 fistulae, 2 sweelings of the anastomosis. tests done 3 months after the operation did not show any other complication in relation with the TUU. Nevertheless one reflux and 3 stenoses of the end of the recipient ureter caused dilatation of two upper urinary tracts. During the long term follow-up, it was necessary to perform 2 nephrectomies of the donor ureter and 5 operations on the end of the recipient ureter. These complications were independant of the TUU. The final result of TUU was excellent.

[Ureteral reimplantation using the psoas bladder hitch. Apropos of 42 cases]. / Réimplantation urétérale avec fixation de la vessie au psoas (vessie psoïque). A propos de 42 observations.

42 cases of ureterocystoneostomy with psoas hitch were performed: 22 boys, 20 girls. The majority of the cases were done between ages 4 and 6. Initially 8 cases presented with reflux, 9 with megaureter and reflux, 20 with megaureter without reflux, 5 cases of ureterocele with duplication. 2 cases were done as a primary procedure, 4 cases as a part of undiversion, 36 after failure of a reimplantation either for reflux (13) or for stenosis (23). The technique is described: four cases were done bilaterally. Another procedure accompanied the psoas hitch: 20 ureteral modelage, 3 transuretero-ureterostomies. Follow up varied from 1 to 13 years with good results in 27 cases, fair results in 10 and poor results in 5.

[The valves of the posterior urethra]. / Valves de l'urètre postérieur.

75 cases of posterior urethra valves have been seen and treated from 1952 to 1984. The average follow-up is 11 years. The clinical symptomatology is described and the different treatments are analyzed from a critical point of view. A the present time the progress made by pediatric intensive care, minimization of endoscopes, better understanding of lesions, indicate that the only treatment has to be the valves. A urinary diversion is to be considered only if the life of the boy is in danger. It is rare to have to reimplant the ureters. 3 boys died. From the cases with long follow-up, 4 have high blood pressure, 16 have renal insufficiency, of these one had a successful renal transplant, 6 are dialyzed. The problems of urinary continence and fertility are discussed.

[Polyp of the posterior urethra. Apropos of 6 cases]. / Polype de l'urètre postérieur. A propos de 6 cas.

Polyp of posterior urethra is a rare cause of urinary obstruction. Attached by a pedicle to the superior part of the veru montanum, such a polyp is mobile and can move in the bulbar urethra, causing urinary retention. If can also bleed provoking either hematuria or urethrorrhagia. Urinary infection can be present. Vesico-urethral ultrasound examination can show the polyp. But the diagnosis is made on a voiding cystourethrogram either at the end of an intravenous pyelogram or after suprapubic puncture of the bladder. Cystourethroscopy can also diagnose the lesion, and can treat it by electrocoagulation of the pedicle. Surgery by suprapubic incision and cystostomy is another method of treatment. It is perhaps less dangerous that the endoscopic treatment because the pedicle is very close to the ejaculatory ducts. Pathology of polyp of posterior urethra is variable but benign. The most frequent lesion is a fibrous polyp specially in children. Recurrency of such a lesion is very exceptional.

[Vulvar cysts disclosed in a neonate. Apropos of 6 cases]. / Kystes vulvaires à révélation néonatale. A propos de six cas.

The authors report six cases of vulvar cysts in female neonates. Careful examination of the vulva normally leads to correct diagnosis of this rare lesion, but a few additional examinations may be helpful. Simple puncture without anesthesia is generally sufficient for a successful treatment of this benign and isolated tumor. The authors go on to discuss the embryological, histological and histochemical aspects, and review the literature.

[Pyelo-ureteral duplication and vesico-ureteral reflux]. / Duplicité pyélo-urétérale et reflux vésico-urétéral.

106 children (76 girls and 30 boys) were treated for vesico-ureteric reflux in double upper urinary tracts. The results of different treatment protocols are discussed. Medical treatment is rarely successful. Partial or total nephrectomy is sometimes necessary because of very poor renal function. Cohen's technique for the "en bloc" ureterocystoneostomy of the two ureters seems to provide the best conservative treatment. But the results, which are good as far as the reflux is concerned, are often unsatisfactory because of the scarring of the renal parenchyma, especially as regards the inferior pole, and because of persistent urinary infection.

[Treatment of megaureter in children by Cohen's operation combined with ureteral reconstruction. 81 reconstructed and reimplanted ureters]. / Traitement du méga-uretère de l'enfant par l'opération de cohen associée à un modelage de l'uretère. 81 uretères modelés et réimplantés.

From January 1973 to June 1983, 69 children have been operated on: remodeling of a primary mega-ureter and reimplantation of the tailored ureter with the Cohen technique. 81 ureters have been treated this way. 68 children have been followed and the results of this operation are evaluated on 80 ureters. There are 65 satisfactory results, 5 mediocre and 10 bad (5 stenosis, 5 reflux); another operation has been done for these cases: 3 nephrectomy, 7 conservative treatments have been successful.

[A series of 34 cases of prune belly syndrome in children]. / Etude d'une série de trente-quatre cas d'aplasie de la musculature abdominale chez l'enfant.

The congenital triad of abdominal muscle deficiency, urinary tract anomalies and cryptorchidism is often called prune-belly syndrome because of the wrinkled abdominal skin of these neonates. Other associated anomalies may be noted, such as cardiovascular, gastrointestinal or orthopedic disorders. The incidence is about 1 in 40 000 births. Prune-belly is in fact a spectrum of diseases falling into three main groups with very different prognoses and treatments. In group 1, the severe urinary and pulmonary abnormalities usually lead to stillbirth or death in infancy, and need no surgical management. In group 2, the mean survival rate is high (83%), and surgery may be required in some cases, mainly for urinary diversion. In group 3, the disorders are very mild, and the children are often called "pseudo-prunes". The etiology is unknown, but the syndrome appears to be due to arrested mesenchymal development between the sixth and tenth weeks of gestation. In groups 2 and 3, the main problems are to ensure sterility of the urine by close supervision, and to prevent the severe accidents which may be caused by retrograde radiological exploration. A more conservative approach to the syndrome appears to improve the prognosis, and the spontaneous improvement of big megaureters without infectious complications would seem to favor this line or argument.

Congenital curvature of the penis without hypospadias.

A series of 18 cases of congenital curvature of the penis without hypospadias is reviewed. Two types of curvature are differentiated. One type (12 cases) without any other anomaly of the penis is treated by a single-stage operation without involving the urethra. In the second type (6 cases), the curvature is associated with hypoplasia of the urethra on a more or less long portion, and it has to be treated as a concealed hypospadias.

Simplified treatment of ectopic ureterocele in 35 children.

From 1970 to 1977, 35 patients with ectopic ureterocele underwent a simplified treatment which consisted of the removal of the upper part of the kidney and its dilated ureter, but with no attention to the intravesical swelling. The procedure is straightforward and can be performed on very young children in poor physical condition. It can be used in all cases of ectopic ureterocele whether the contralateral upper urinary tract is normally refluxing or dilated. Of 33 cases with a reasonably long follow-up the results were good in 26 cases. In 19 cases the infection disappeared immediately and the ureterocele collapsed. If the lower ureter was dilated there was an improvement of its diameter in 13 of 14 cases. If the ureter was refluxing, the reflux disappeared in most patients. In only 7 patients did the reflux persist, necessitating a reoperation to reimplant the ureter and remove the ureterocele. In seven cases the results were not satisfactory: one case of dilatation in the superior contralateral urinary tract, two cases of persistence of reflux in the lower ureter and 4 cases in which the urinary infection persisted.