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Purpose: Spina bifida (SB) is the most common congenital cause of incontinence in childhood. This study attempts to determine the relationships between urinary/faecal incontinence, methods of management, and Health Related Quality of Life (HRQoL) in people with SB. Patients and method: A total of 460 patients (300 adults and 160 adolescents) from six centres in France have taken part in this cross-sectional study. Clinical outcome measures included walking ability, urinary/faecal continence, and medical management. HRQoL was assessed using the SF36 in adults and the VSP in adolescents and their parents. Univariate and multivariate analysis was used to determine the relationships between clinical parameters and HRQoL. Results: HRQoL were significantly lower than in the general population. Adult women had significantly lower scores than men, and adolescent females had significantly lower scores for psychological well being. We did not found strong relationship between incontinence and HRQoL in this population. Moreover patients surgically managed for urinary/fecal incontinence did not show significantly higher scores of HRQoL. Conclusion: Using generic HRQoL measures, urinary/faecal incontinence and their medical management may not play a determinant role in HRQoL of persons with SB. However many other factors affect HRQoL in these patients. A longitudinal study design is recommended to assess whether incontinence management is associated with improve HRQoL.

Hemolymphangioma is a benign malformation of the lymphatic vessels. Cervical localisation is the most frequent. Mediastinal localisation is rare, but may be life-threatening because of airway compression. We report on a four-month-old boy who presented, in a context of epidemics, with clinical signs of acute bronchiolitis requiring mechanical ventilation for several days. Chest X-rays showed an important mediastinal mass with airways compression. Diagnosis was evoked on CT-scan aspects and confirmed by histology. Surgery allowed complete resolution, but dysphonia and oropharyngeal disorders persisted for several months. This rare congenital malformation is reviewed.

Resume Le neuroblastome est la tumeur la plus frequente en periode neonatale. L'objectif de cette etude a ete d'analyser la presentation clinique, les modalites therapeutiques, et l'evolution des nouveau-nes ayant presente un neuroblastome localise (NBL). Population et methodes. – Cinquante-deux enfants traites selon des recommandations therapeutiques homogenes (protocoles NBL 90 et 94) entre 1990 et 1999 ont ete recenses. Resultats. – L'âge median au moment du diagnostic etait de 12 jours (0–28) et la decouverte a eu lieu des la periode antenatale chez 14 patients (27 %). Le siege de la tumeur etait abdominal (n = 40, dont 20 localisations surrenaliennes), thoracique (n = 8), pelvien (n = 3), et cervical (n = 1). Une amplification de l'oncogene N-myc a ete trouvee dans un cas sur les 40 evaluables. La taille de la tumeur etait inferieure a 5 cm dans 25 cas, comprise entre 5 et 10 cm dans 25 cas et superieure a 10 cm dans deux cas. Sept enfants avaient une tumeur en sablier, parmi lesquels cinq presentaient des deficits neurologiques. Un enfant est decede d'hemorragie suite a la ponction a visee diagnostique. Trente-sept patients ont ete operes d'emblee, parmi lesquels deux sont decedes de complications postoperatoires et trois ont eu une nephrectomie. Les 14 autres ont ete consideres comme inoperables et ont ete traites par chimiotherapie initiale et exerese secondaire chez 12. L'un d'entre eux est decede quelques jours apres le debut de la chimiotherapie. Au total, 48 enfants sont vivants en remission complete, dont quatre apres une evolutivite cutanee (3) ou medullaire (1). La survie globale est de 92 % avec un recul median de 46 mois (0–113 mois). Conclusion. – L'excellent pronostic des NBL justifie des indications operatoires prudentes, fondees sur des criteres anatomiques et radiologiques tres precis, en particulier chez le nouveau-ne. Une chimiotherapie adaptee au poids, realisee par une equipe experte, permet d'operer certains de ces patients dans des meilleures conditions.

Objectives: The purpose of this study focused on cervical neuroblastoma (NB) was to assess the prognosis, define the most suitable methods of investigation, and evaluate risk factors for complications following primary surgery. Methods: Between 1990 and 1999, we conducted two consecutive prospective multicentric studies (NBL90 and NBL94) on localized NB. Because the first study (1990–1994) found surgery-related morbidity and mortality, several surgical risk factors (i.e. adhesion to major vessels, size, friability, and dumb bell tumor) were defined and used prospectively as criteria of resectability in the second study (1994–1999). Results: Of 617 cases included in the two studies, 43 involved cervical NB including 17 cervicothoracic tumors. With a median follow-up of 4 years, overall survival and event-free survival rates were 91 and 81%, respectively with no significant difference between cervical or cervicothoracic NB. Seventeen patients were included in the second study; surgery was used as the first line treatment in 11. Full pre-operative work-up was performed in eight patients, demonstrating one or more risk factors in three. The remaining three patients underwent emergency surgery with no pre-operative work-up or only ultrasound: two developed serious complications. All three patients presenting documented risk factors developed post-operative complications versus only two of the eight patients who presented no risk factor ( n =5) or were inadequately evaluated ( n =3) ( P =0.06). None of the five patients in whom full work-up demonstrated no risk factor had post-operative complications ( P =0.02). Conclusions: Cervical neuroblastoma has a favorable prognosis. Surgery is the treatment of choice but there is a risk of complications. Appropriate pre-operative work-up is mandatory to evaluate resectability. The surgical risk factors defined for our second study seem to be significant predictors of post-operative complications.

Objectives To evaluate the outcome of implantation of the AMS 800 artificial urinary sphincter in children with neurogenic bladder. Patients and methods The records of 107 children (74 boys and 33 girls) treated for neurogenic urinary incontinence by implantation of the AMS 800 artificial sphincter between 1983 and 1993 were reviewed retrospectively. The underlying cause of incontinence in 92 patients was spina bifida, the mean age at implantation was 13.7 years and 30 patients had undergone previous bladder neck surgery. Implantation was carried out in combination with another surgical procedure in 24.3% of patients. The mean follow-up was 61 months (minimum 12). Results The mean operational life of the sphincter was 56 months. No deaths occurred but removal of the artificial sphincter was necessary in 20 patients (19%). Surgical revision was not required in 44 patients (41%) but at least one revision was performed in 63 and more than one in 42. There were 21 mechanical failures, 40 surgical complications and 39 cases involving changes in the dynamics of bladder function. Of the 87 children (81%) in whom the device is still in place, 72 are completely continent (83%), 10 are slightly wet, and five are incontinent. The overall success rate was 77%. Conclusions The results emphasize that for successful implantation of an artificial urinary sphincter in children, the pre-operative bladder capacity must be sufficient and previous surgery should not have been performed on the bladder neck, the site of choice for implantation in children. Sphincterotomy is not always necessary in paediatric patients. Children and parents should be informed of the high complication rate and the need for long-term follow-up.

Patients with spinal dysraphism may have severe constipation and faecal incontinence. The impact of antegrade colonic enema (ACE) in the management of patients with spina bifida (SB) is analysed. In a multicentre cross-sectional study, constipation, faecal incontinence and faecal management were described. Cases surgically treated were identified. Data were collected from 423 patients, of whom 230 did not use any manoeuvre or laxatives to assist evacuation. Conventional treatment was used in 193 patients, including digital extraction in 39%, retrograde enema in 21% and oral laxatives in 52%. For intractable constipation and overflow of faecal incontinence, 47 patients were treated with ACE, of whom 41 used the method at a mean time of interview of 4.1 +/- 1.9 years after ACE operation; six abandoned ACE for conventional management. With ACE, faecal continence was significantly improved compared with conventional management, and neither retrograde rectal enema nor digital extraction were required. The conduit was fashioned to the right colon in 32 cases and to the left colon in nine cases. This study provides information on a multicentre experience in bowel management in SB patients. Whatever the technique used, ACE has improved faecal status compared with conventional therapy.

Purpose Hydrocele seems to be the most frequent complication in children who undergo surgery for varicocele and the issue of the optimal management of hydrocele remains controversial. In this retrospective study we evaluated the incidence and management of hydrocele following surgical treatment of varicocele in children treated at 8 European centers of pediatric surgery. Materials and Methods In a 5-year period 278 children between 7 and 17 years old underwent surgical treatment for unilateral left varicocele, including 187 using video surgery and 91 via an open inguinal approach. Results At an average followup of 24 months (range 12 to 60) 34 children (12.2%) had a left hydrocele. Of the 278 children 14 (5%) were lost to followup. The hydrocele appeared between 1 week and 44 months (median 2 months) after surgery. Concerning hydrocele management 16 of 34 children (47%) were treated with scrotal puncture while under local anesthesia, which led to hydrocele regression after a median of 3 punctures (range 1 to 5), 12 (35.3%) underwent clinical observation since the hydrocele reduced spontaneously within a median of 12 months after its appearance and 6 (17.7%) were treated with open surgery. In 4 cases the hydrocele disappeared and in 2 it recurred after surgery and was successfully treated with punctures. Conclusions This study shows that the median incidence of hydrocele after varicocele surgery is about 12% but it seems higher after artery nonsparing vs sparing procedures (17.6% vs 4.3%). On the contrary, no difference was found when the procedure was performed using video surgery or with the open approach. Hydroceles generally develop a few months later but may also appear several years after the surgical repair of varicocele. Noninvasive procedures (scrotal punctures or clinical observation) seem to induce total hydrocele regression in more than 82% of cases. Children who undergo surgery for varicocele should undergo long-term followup to detect a possible hydrocele. In fact, the 5.4% of children lost to followup in our study may potentially have had a hydrocele. Surgery is not always successful for this condition, as shown in the 2 cases of recurrent hydrocele after surgical repair.

Purpose: The encouraging initial results of gastrocystoplasty led us to perform it for failed bladder exstrophy closure. We assess the functional outcome of the augmented bladder and evaluate complications related directly to use of the stomach in this specific group of children.Materials and Methods: We performed gastrocystoplasty in 22 children an average of 9.5 years old with a small, poorly compliant bladder after staged reconstruction of bladder exstrophy failed. Followup ranged from 6 months to 6 years (mean 3 years).Results: Complete urinary continence was achieved in 14 children (64%). Voiding via the urethra was possible in 13 patients (60%) but post-voiding residual urine was significant in 12. Bladder capacity increased from a mean of 77 to 270 ml. Bladder capacity decreased during followup in 3 children, requiring repeat augmentation. Six children had isolated dysuria and 2 had dysuria with hematuria. Perforation of the gastric patch and a bleeding gastric ulcer occurred in 1 patient e...

In a series of 150 patients with congenital urologic disorders diagnosed in utero and managed in the neonatal period from 1980 to 1985, 48 cases were ureteropelvic junction obstructions. One was a false positive, and 47 were documented pyelocaliceal distension and retention; 12 of them were bilateral. Five cases had a spontaneous resolution. Three had a nephrectomy performed (destroyed kidney). Fifty kidneys have been operated on (Anderson-Hynes dysmembered pyeloplasty). Ninety percent of the cases are reported as clinically, biologically, and radiologically fair. Six percent postoperative complications are reported. The authors pointed out the great interest in neonatal repair of this condition, using microsurgical techniques without stent or nephrostomy. A comparison is made of the overall results with an identical series of older patients operated on during the same period.

Curative surgery of Hirschsprung's disease (HD) was performed in 32 infants younger than 3 months of age from January 1, 1977, December 31, 1986. There were 24 males, seven of whom presented with total colonic aganglionosis. Preoperative relief of obstruction was carried out by only enemas in 25 patients (81%), with addition of total parenteral nutrition of 8 to 27 days in case of severe enterocolitis. Colostomy was never performed as a routine procedure. Preoperative morbidity occurred in three cases: one enterocolitis and two sigmoid performations that were successfully treated by colostomy. Weight at operation ranged from 3.3 to 6.0 kg (mean, 4.9 kg). Swenson's (25), Duhamel's (5), and Soave's procedures (2) were performed. There was no mortality. Follow-up varied from 2 to 10 years. Postoperative morbidity occurred in five patients (one anastomotic leak, two stenosis, one volvulus, and one recurrent enterocolitis). All other patients are considered as definitively cured. Comparison with data in the literature permits one to advocate primary corrective treatment of HD without decompression in infants less than 3 months of age.

According to major reports in the literature satisfactory cosmetic and genital results after correction of male epispadias have been achieved in only 50 per cent of the patients. Since 1976 we have been using the Duckett transverse island flap principle adapted to epispadiae anatomical conditions. The original technique was used in our initial 18 patients and the Asopa double flap technique was performed in the subsequent 14. Excellent results were obtained in both series. However, there was a high percentage of fistulas that required secondary repair. The techniques and results are described.

No single major procedure is able to achieve all necessary goals without problems in the treatment of anorectal malformations. The Mollard-Pena (MOPE) procedure adds the benefits of an anoplasty based on the original perineal flap described by Mollard, but slightly modified, and the precise recognition of all muscle structures as reported by Pena and De Vries. Cosmetic as well as functional early results are encouraging.