J.M. Guys

Surgical management of duplex ureteroceles

We reviewed the cases of 95 children with duplex ureteroceles treated in this department over an 18-year period. There were 101 ureteroceles (6 bilateral). Diagnosis and treatment were analyzed. Special attention was paid to newborns screened in utero. We always strove to preserve functional renal tissue whenever possible. In keeping with this goal, three surgical techniques were used: (1) upper pole heminephrectomy; (2) ureterocele excision, bladder neck reconstruction, and ureter reimplantation with or without cutaneous ureterostomy of the upper pole ureter; and (3) endoscopic ureterocele incision. Follow-up studies using x-ray and radionuclide imaging demonstrated satisfactory renal function in 86.6% of patients. These findings support a conservative approach to ureteroceles using endoscopic ureterocele incision as the primary treatment. Lower urinary tract reconstruction may be associated in cases involving urinary tract infection, obstruction or incontinence. Upper pole heminephrectomy should be performed only after functional evaluation following ureterocele incision or cutaneous ureterostomy.

Laparoscopic Partial Splenectomy: Indications and results of a multicenter retrospective study

IntroductionPartial splenectomy (PS) in children is a surgical option in haematological diseases and focal splenic tumours. The aim of this study was to describe the feasibility and the results of laparoscopic partial splenectomy in children in these two indications by a multicentric retrospective study.MethodsThe authors reviewed the files of all children who underwent laparoscopic PS between March 2002 and September 2006 in two paediatric surgical centers. The data of 11 children were collected and included clinical presentation, age, gender, radiographic examinations, surgical procedure, need for blood transfusion and early complications.ResultsFrom March 2002 to September 2006, laparoscopic PS had been performed on 11 children (6 boys, 5 girls) aged 23 months to 11 years (mean 7, 9). Four children had splenic focal tumours and seven had haematological diseases: six hereditary spherocytosis (HS) and one hemoglobinosis E. During the surgical procedure for haematological diseases 75–80% of the splenic tissue was removed. When PS was performed for focal splenic tumours, the splenic remnant was around 70%. No preoperative complications occurred (no bleeding, no diaphragmatic injury). Neither preoperative nor conversion was necessary. One postoperative complication occurred (left pleural effusion) but required no further treatment. The mean hospital stay was 7.7 days (range from 3 days to 10 days). No infectious postoperative complications occurred; the mean follow up was 21.1 months (range 3–52 months).ConclusionLaparoscopic partial splenectomy is feasible and safe in children with hypersplenism or focal splenic tumours. Partial splenectomy is a good way to prevent postsplenectomy infections by preservation of the immune role of spleen in children with haematological diseases. This technique performed for focal splenic tumours allows the surgeon to choose the size of the splenic remnant.

Tracheobronchial ruptures from blunt thoracic trauma in children

BACKGROUND/PURPOSE Tracheobronchial ruptures in blunt thoracic trauma in children are rare. The aim of this study was to suggest the means of an early diagnosis and a conservative management as often as possible. METHODS Sixteen cases of tracheobronchial ruptures by blunt thoracic trauma were observed over 26 years in 9 regional pediatric centers. RESULTS There were 12 boys and 4 girls, from ages 1 hour to 17 years. Nine children presented with associated lesions. Fibroscopy established the following diagnosis: 8 tracheal wounds and 8 bronchial wounds. Six children were operated on within 18 hours (on average) after installation of a thoracic drainage. Two lobectomies, 3 ideal tracheal sutures, and 1 bronchial suture were performed. Seven children were treated exclusively by thoracic drainage. Two of them were intubated through the lesion, leading to a transitory endoprothesis accompanied or not by an external thoracic drainage. One infant recovered spontaneously. There were no deaths in this series. Two recurrent postoperative nerve injuries were noted, one of which was a transitory spontaneously resolutive scar bud and one a granuloma treated by laser. Three times, a stenosis occurred after a conservative management. Two were operated on. CONCLUSIONS Tracheobronchial ruptures in children are rare. An early fibroscopy holds an important place in the approach of this pathology. Treatment is variable, based on thoracic lesions, their tolerance by the child, and associated lesions. Surgery is not the only therapy because conservative treatment by simple thoracic drainage or lesion intubation has proved effective.

ENDOSCOPIC TREATMENT OF URINARY INCONTINENCE: LONG-TERM EVALUATION OF THE RESULTS

PURPOSE We report on the use of polydimethylsiloxane for endoscopic treatment of urinary incontinence in children with neurogenic bladder and discuss our results to determine optimal criteria for patient selection. MATERIALS AND METHODS A total of 44 children (19 males) have been treated endoscopically for urinary incontinence since 1995. Etiology was spina bifida in 35 cases. Previous surgery had been performed on 24 patients, including bladder neck reconstruction with (17) or without bladder augmentation. Mean patient age at injection was 13 years (range 7 to 17). A single transurethral injection was given in 23 cases, 2 in 17 and 3 or more in 4. Mean volume at each injection was 3.5 cc and for each patient the total volume injected was 5.7 cc. Mean delay between 2 injections was 6 months (range 3 to 15). RESULTS Followup ranged from 6 to 53 months (median 28). Of the patients 15 (34%) are dry (continent greater than 4 hours, no urinary pad during the day), 11 (25%) are improved (continent 2 to 3 hours, occasional pad) and 18 had poor results. In the entire series only gender and preoperative hyperactivity influenced the results, as the best results were achieved in females with a stable bladder (44% of girls versus 21% of boys were cured). Good results persisted at 12-month followup in patients treated with only 1 injection (until 36 months for older patients) and after the last of 2 injections. Of the patients treated with 3 or more injections 1 was dry at 12-month followup and treatment failed in 3. CONCLUSIONS Injection of polydimethylsiloxane at the bladder neck achieved continence in 34% of neurogenic bladder cases. Results were better in girls with a stable bladder. Results deteriorated in the first 12 months of followup. No more than 3 injections are advised if a satisfactory result is not achieved.

Appendicovesicostomy: an alternative urinary diversion in the child.

On the basis of 10 observations, the authors report their experience of the continent appendicovesicostomy (Mitrofanoff technique). Indications, techniques and results are discussed. The authors also present a technique of transureteral continent cystostomy.

RE: USE OF POLYDIMETHYLSILOXANE FOR ENDOSCOPIC TREATMENT OF NEUROGENIC URINARY INCONTINENCE IN CHILDREN

PURPOSE We report on the injection of polydimethylsiloxane for endoscopic treatment of urinary incontinence in children with neurogenic bladder and determine the optimal criteria for patient selection. MATERIALS AND METHODS We have treated 17 boys and 16 girls since 1995. The etiology of incontinence was spina bifida in 24 cases. Previous surgery was performed in 18 patients, including bladder neck reconstruction in 15 and bladder augmentation in 9. Mean patient age at injection was 13 years (range 7 to 17). We administered 1, 2 and 3 injections in 21, 11 and 1 patients, respectively. Mean volume at each injection was 3.2 cc. Mean interval between injections was 6 months (range 3 to 15). In all cases injection was done transurethrally. RESULTS Followup ranged from 6 to 41 months (median 16). A total of 11 patients (33.3%) are dry (continence for greater than 4 hours and no urinary pad use during the day) and 8 (24.2%) are improved (continence for 2 to 3 hours and minimal pad use). Results are poor in 14 cases. Overall previous bladder neck surgery or preoperative detrusor hyperactivity did not influence results. Good results were mainly associated with female gender (47.4% of girls versus 10.5% of boys achieved cure). CONCLUSIONS Injection of polydimethylsiloxane at the bladder neck resulted in continence in 33% of neurogenic bladder cases. Better results occurred in girls and injection did not compromise other surgical procedures. Polydimethylsiloxane seems more suitable than bovine collagen due to potential problems with biological product use.

A Novel Technique for Reconstruction of the Abdominal Wall in the Prune Belly Syndrome

There is currently widespread enthusiasm for abdominal wall reconstruction in patients with the prune belly syndrome. We have devised an operation that appears to offer some advantages over those proposed by Ehrlich and Randolph. The technique preserves the umbilicus, and thickens and strengthens the anterior abdominal wall. By narrowing the waist, it also produces a better cosmetic appearance. After full thickness resection of a varying amount of skin from the central abdomen, the anterior wall is sutured in double-breasted fashion, thus, preserving all vascularization and the umbilicus. Since 1969 we have successfully performed this procedure on 9 prune belly patients including 1 girl. The results were excellent in terms of duration and cosmetic appearance.

Endoscopic Injection With Polydimethylsiloxane for the Treatment of Pediatric Urinary Incontinence in the Neurogenic Bladder: Long-Term Results

PURPOSE We report the long-term results of endoscopic injection of PDMS in correcting urinary incontinence in children with neurogenic bladder. MATERIALS AND METHODS We performed a single center, retrospective study of patients receiving endoscopic injections of PDMS. All procedures were performed as outpatient surgery using endoscopic guidance. Procedure tolerance was assessed at 15 days, and efficacy was evaluated at 3 months, 6 months and annually thereafter. Success was defined as periods of dryness between bladder voiding of more than 4 hours during the daytime without the need to wear pads. Improvement was defined as periods of dryness greater than 2 and less than 4 hours with occasional protection. RESULTS A total of 49 children (21 boys and 28 girls) have received 1 or more injections of PDMS since 1995. Etiology was spina bifida in 41 patients (84%), with surgery (enterocystoplasty and/or bladder neck reconstruction) performed previously in 27 patients. Mean patient age was 14 years (standard deviation 4.8). Mean volume of PDMS per treatment was 3.6 ml. At the end of the 6-year mean followup 16 patients (33%) were continent and 7 (14%) were improved. Continence was unchanged in the remaining 26 patients (53%). After a significant deterioration of the results the outcome remained almost unchanged from 18 months of followup. Bladder neck surgery, bladder hyperactivity if medically controlled and gender have no influence statistically on the long-term results. CONCLUSIONS Injection of PDMS for incontinence of neurogenic origin is a reliable technique that achieves long-term continence in almost a third of the patients. This procedure can be used either as first line treatment or in addition to other surgical techniques. The initial success of the procedure seems predictive of success in the long term, and results are stable after 18 months of followup.

Is Hypospadias Associated with Prenatal Exposure to Endocrine Disruptors? A French Collaborative Controlled Study of a Cohort of 300 Consecutive Children Without Genetic Defect

BACKGROUND Numerous studies have focused on the association between endocrine-disrupting chemicals (EDCs) and hypospadias. Phenotype variability, the absence of representative comparison groups and concomitant genetic testing prevent any definitive conclusions. OBJECTIVE To identify the role of occupational and environmental exposures to EDCs in nongenetic isolated hypospadias. DESIGN, SETTING, AND PARTICIPANTS A total of 408 consecutive children with isolated hypospadias and 302 normal boys were prospectively included (2009-2014) in a multi-institutional study in the south of France, the area of the country with the highest prevalence of hypospadias surgery. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS In patients without AR, SRD5A2, and MAMLD1 mutations, parental occupational and professional exposures to EDCs were evaluated based on European questionnaire QLK4-1999-01422 and a validated job-exposure matrix for EDCs. Environmental exposure was estimated using the zip code, the type of surrounding hazards, and distance from these hazards. Multivariate analysis was performed. RESULTS Fetal exposure to EDCs around the window of genital differentiation was more frequent in the case of hypospadias (40.00% vs 17.55%, odds ratio 3.13, 95% confidence interval 2.11-4.65). The substances were paints/solvents/adhesives (16.0%), detergents (11.0%), pesticides (9.0%), cosmetics (5.6%), and industrial chemicals (4.0%). Jobs with exposure were more frequent in mothers of hypospadiac boys (19.73% vs 10.26%, p=0.0019), especially cleaners, hairdressers, beauticians, and laboratory workers. Paternal job exposure was more frequent in the cases of hypospadias (40.13% vs 27.48%, p=0.02). Industrial areas, incinerators, and waste areas were more frequent within a 3-km radius for mothers of hypospadiac boys (13.29% vs. 6.64%, p<0.00005). Association of occupational and environmental exposures increases this risk. CONCLUSIONS This multicenter prospective controlled study with a homogeneous cohort of hypospadiac boys without genetic defects strongly suggests that EDCs are a risk factor for hypospadias through occupational and environmental exposure during fetal life. The association of various types of exposures may increase this risk. PATIENT SUMMARY Our multi-institutional study showed that parental professional, occupational, and environmental exposures to chemical products increase the risk of hypospadias in children.

Value of Electrophysiological Assessment After Surgical Treatment of Spinal Dysraphism

PURPOSE This study was conducted in children treated surgically for spinal dysraphism between 1994 and 2000 to determine the value of electrophysiological parameters for followup in comparison with clinical, radiological and urodynamic findings. MATERIALS AND METHODS Electrophysiological examination was performed based on electromyographic detection of perineal muscle activity with evaluation of the sacral reflexes and measurement of lower limb and perineal evoked potentials. Clinical and radiological assessment consisted of physical examination, kidney ultrasonography and retrograde cystouretrography. Urodynamic assessment included determination of urethral pressure profile, bladder activity, compliance, voiding pressure and sphincter synergy. A total of 45 children were included in the study. In 28 cases electrophysiological assessment was performed routinely in 20 children (group 1) before and after (6 months and annually thereafter) surgical treatment (mean followup 30 months) and in 8 children (group 2) only after surgical treatment (mean followup 65 months). Electrophysiological findings were correlated with the urodynamic and clinical findings. Sensitivity, specificity and positive and negative predictive values were calculated. In 17 children (group 3) electrophysiological assessment was performed only once either before or after surgery. RESULTS Electrophysiological assessment with combined electromyographic detection and perineal evoked potentials was highly sensitive in predicting urodynamic impairment, particularly vesico-sphincter dyssynergia (sensitivity 100%) or bladder dysmotility (sensitivity 86%). Specificity of perineal evoked potentials for detecting urodynamic dysfunction was 90%. Electromyographic detection and perineal evoked potentials also demonstrated satisfactory sensitivity in confirming urological impairment (sensitivity 79%, negative predictive value 90%). CONCLUSIONS Electromyographic detection and perineal evoked potentials are valuable for assessment of patients after surgical treatment of spinal dysraphism. Because these techniques are minimally invasive and highly reproducible, they are suitable for baseline assessment in most cases (tethered cord, lipoma, syringomyelic cavities). Urodynamic assessment should be reserved for patients at high risk for secondary neurogenic bladder dysfunction such as myelomeningocele or sacral agenesis.