Malignancy after Augmentation Enterocystoplasty: A Nationwide Study of Natural History, Prognosis and Oncogene Panel Analysis.

PURPOSE: We report the natural history and prognosis of tumors after augmentation enterocystoplasty, with a molecular analysis using an oncogene panel to search for potential targeted therapies. MATERIALS AND METHODS: This multicenter, nationwide, retrospective study included 16 patients. A panel of 21 clinically relevant oncogenes was tested on archival tumor specimens using next-generation sequencing. Survival rate was the main clinical outcome and sequences were compared to the reference genome for the genetic outcome. RESULTS: Augmentation enterocystoplasties were performed mainly for congenital neurogenic bladder and bladder exstrophy at a median patient age of 17 years (range 4 months to 45 years). Most of the malignancies were diagnosed because of clinical manifestations, with a median latency period of 20 years. Adenocarcinomas were mainly found after gastrocystoplasty, whereas urothelial cell carcinomas were typically found after colocystoplasty. Of the 16 patients 13 were diagnosed at an advanced stage of the disease (positive lymph nodes in 7, distant metastases in 6). The overall 1-year survival rate was 56%. Only 3 patients remained disease-free at a median followup of 70 months. Of the 9 tumors with analyzable DNA 4 were wild-type and 5 harbored missense mutations (KIT-p.Pro573Ser, PDGFRA-p.Glu587Lys, KRAS-p.Gly12Asp, ERBB4p.Arg484Lys, CTNNB1-p.Ser37Phe and p.Ser47Asn). CONCLUSIONS: Malignancy after augmentation enterocystoplasty is diagnosed late with frequent metastases and a very low 1-year survival rate. More than half the tested samples harbored missense mutations in oncogenes accessible to targeted therapies. An international collaboration to enlarge the genetic panel analysis of these tumors may offer new therapeutic hope to patients.

Surgery for no palpable testis before the age of one year: a risk for the testis?

INTRODUCTION: Surgery for undescended testis is now commonly recommended before the age of one year. However, the risk of testicular atrophy or miss location after surgery at a young age has not been clearly evaluated. OBJECTIVE: The objective of this study is to evaluate the rate of testicular atrophy after surgery for non-palpable testis before the age of one year. MATERIALS: Fifty-five patients operated between 2005 and 2014 for non-palpable testes were reviewed for clinical and ultrasound (US) evaluation. Median follow-up after surgery was of 68.5 months (range 26-130 months). The median age at surgery was of months (5-12 months). Eight patients (14.5%) had bilateral non-palpable testis; thus, 63 testes were evaluated. At surgery, 38 (60%) testes were located in the high inguinal canal; 25 (40%), in the abdominal cavity. Orchiopexy was performed with preservation of the testicular vessels for 58 testes. Fowler-Stephens (FS) procedure was performed for 5 testes. Testicular location was clinically evaluated, and testicular volume was measured using a standard sonogram technique in our pediatric radiology department. Ratio comparing the volume of the descended testis to the spontaneously scrotal located testis was calculated in unilateral forms. RESULTS: After surgery, testes had scrotal location in 62 cases and inguinal location in one case. Seven cases of atrophy were confirmed after US control (11%), more frequently (odds ratio, OR 11.68 [1.9-72.5]) in abdominal testis (24%) than in inguinal testis (2.6%). Atrophy testicular was more frequent with FS technique (OR 7.1 [1.3-40.1]), but the population was weak (N = 5). Median volume ratio for unilateral form was 0.88 [0-1.8]; 14 patients presented a ratio greater than 1. DISCUSSION: The influence of the young age at surgery and the risk of post operative testicular atrophy had not been clearly evaluated. The term of 'no palpable testis' supports an heterogeneous group mixing abdominal and extra-abdominal testis sharing a uniform clinical presentation. Our rate of atrophy in the group of abdominal testes (24%) and inguinal testes (2.6%) is similar to the literature, which concerns older patients. The long-term sonogram assessment demonstrated a good development of the testis after surgery, especially in inguinal cases. CONCLUSION: Surgery for no palpable testis before the age of one year does not lead to a superior risk of testicular atrophy compared with surgery at an older age and allows a good development of the testis.

[Experience of the single scrotal approach for recurrent undescended testis after primary orchiopexy or inguinal surgery]. / Expérience de l'abord scrotal unique dans les abaissements testiculaires après chirurgie inguinale ou échec d'orchidopexie préalable.

OBJECTIVE: To report our experience of the unique scrotal incision for the redo cases of orchiopexy after previous inguinal surgery or orchiopexy for undescended testis with a special attention regarding the place of the single scrotal approach. PATIENTS AND METHODS: Thirty-six patients operated between January 2003 and September 2015 in our surgical unit for secondary orchiopexy after previous inguinal surgery or orchiopexy for undescended testis (UDT) were included in a retrospective study. The secondary surgical procedure was initiated by a scrotal incision (Bianchi). In cases of difficulty by the scrotal incision an inguinal approach by the prior skin inguinal incision was performed. Patients were divided in two groups a group 1 of 10 patients with reascending testis following inguinal hernia repair and a group 2 of 26 patients with reascending testis after previous orchiopexy for UDT. A good result was defined as testis in scrotal position without evidence of atrophy. RESULTS: All the patients of group 1 were treated by a unique scrotal incision. In group 2, seven patients required a complementary inguinal approach. One hematoma complicated a scrotal procedure leading to a testicular atrophy. A good result was achieved in 35/36 patients (97%). CONCLUSION: Single scrotal incision is an efficient and easy way to perform secondary orchiopexy after hernia repair. After surgery for UDT, as for primary cases, it cannot be an exclusive approach, higher testis need a combined or an inguinal approach. LEVEL OF EVIDENCE: 5.

[Evaluation of a teaching tool for learning the surgical technique of inguinal orchidopexy]. / Évaluation d'un outil pédagogique pour l'apprentissage de la technique chirurgicale d'abaissement testiculaire.

INTRODUCTION: Practical training of the surgery resident is based on the companionship currently hampered in particular by the increase of the number of residents in training. We created a teaching tool to promote learning and validation of a technique of classic urologic surgery, inguinal orchidopexy. The objective is to evaluate the applicability and the relevance of this tool. MATERIAL AND METHODS: The tool is a technical evaluation sheet made from reference documentation. The trainers evaluated the residents at 3 times of the semester (hetero-evaluation at 0, 3 and 6 months). Residents evaluated themselves monthly on the same items. RESULTS: Three trainers and 6 residents in surgery participated in the study between May and November 2013. The initial evaluation confirmed that the theoretical knowledge was acquired prior to the practical learning. The level of residents was very uneven at the beginning of the study but not at the end of the semester. The monthly evaluations gave a progressive and significant increase of notes. The notes of the intermediate and final hetero-evaluations rose gradually and they were always superior to those previous self-assessments (P<0.05). The tool was considered simple and useful for the participants. CONCLUSION: This tool is applicable and relevant to the technical teaching of inguinal orchidopexy in this population. A larger study would be helpful to confirm it. This type of tool could be applied to the simple and common surgery techniques to enrich the educational tools used in the training. LEVEL OF EVIDENCE: 4.

[The surgical management of undescended testis between 6 and 12 months: A difficult message to convey]. / Prise en charge chirurgicale des testicules non descendus entre 6 et 12 mois : un message long à transmettre.

PURPOSE: The undescended testis (UT) is the most common congenital malformation of the genital apparatus in male infants. The main objective of the management of these patients is to reduce the risk of infertility and cancer. Since these risks increase with age of operation, learned societies have recommended earlier intervention. Following the publication in 2007 of the Nordic consensus that called for an intervention between 6 and 12 months of age, we issued this message to medical students in the Lower Normandy region of France. The aim of this study was to evaluate the implementation of this teaching through the evolution of the number of children operated between 6 and 12 months for a congenital UT in our center. MATERIAL AND METHODS: Between 2005 and 2014, we evaluated the yearly percentage of children operated for UT between 6 and 12 months compared to the percentage of children operated on between 12 and 24 months. A statistical study using the Chi(2) test was used to compare the 2005-2008 and 2009-2012 periods. RESULTS: The percentage of children operated on between 6 and 12 months of age increased from 13.7 % between 2005 and 2008 to 17.2 % between 2009 and 2014. From 2012 on, the number of children operated on between 6 and 12 months was consistently higher than the number of children operated on between 12 and 24 months. The proportion of children operated beyond 2 years remained stable over the study period. CONCLUSION: Since we implemented teaching of early surgery for children with UT, we have observed a gradual increase in the number of children operated between 6 and 12 months of age. Nevertheless, there are still many children operated between 12 and 24 months and beyond.

[Outpatient pediatric urology in France: A still under developed practice. Results of a survey of the French Section of pediatric Urology (SFUP)]. / L'urologie pédiatrique ambulatoire en France: une pratique encore trop peu développée. Résultats d'une enquête de la section française d'urologie pédiatrique (SFUP).

PURPOSE: Outpatient surgery is a very well adapted practice to the pediatric population. Our goal was to evaluate its actual development in the field of the pediatric urology. MATERIALS AND METHODS: A questionnaire was addressed to all the French pediatric surgery centers in which at least one surgeon was member of the French Section of Pediatric Urology. The questionnaire concerned the organization and the outpatient urology procedures performed during the years 2011 and 2012. RESULTS: Thirty of the 34 approached centers returned the questionnaire. The total number of patients concerned in this study was of 33,166. The practices were very variable among centers: the inferior limit of age for anesthesia ranged from 2 to 12 months and type of surgery was also heterogeneous. Foreskin surgery, orchiopexy for undescended testis, inguinal hernia, and endoscopic treatment of vesico-ureteral reflux were the only procedures performed for more than 60% of cases as outpatient surgery. Outpatient surgery is not well developed in hypospadias repair. Only five centers raised 60% of ambulatory procedures for distal cases. No serious complication related to the outpatient approach was reported. CONCLUSION: This survey demonstrated very heterogeneous practices among French pediatric urologic centers. According to the French guidelines about outpatient pediatric surgery, this type of care could be more developed if anesthesiologists and surgeons accepted to evolve in their practice.

Sacral neuromodulation in children with urinary and fecal incontinence: a multicenter, open label, randomized, crossover study.

PURPOSE: The clinical benefit of sacral neuromodulation is unclear due to the paucity of randomized trial data. The purpose of this study was to evaluate sacral neuromodulation for management of urinary and fecal incontinence in a pediatric population. MATERIALS AND METHODS: This multicenter, open label, randomized, crossover study included children older than 5 years. After trial stimulation of the S3 root a neuromodulator (InterStim) was implanted on the S3 foramen. Clinical examinations, voiding and bowel diaries, and urodynamic and manometric evaluations were performed at the beginning (t1) and end (t2) of the first period, and at the beginning (t3) and end (t4) of the second period. RESULTS: A total of 33 patients (24 boys) with a mean +/- SD age of 12.22 +/- 5.09 years were randomized. Etiologies were mainly of neurological origin. Incontinence was mixed urinary and fecal in 19 cases, urinary only in 9 and fecal only in 5. Cystometric bladder capacity increased during sacral neuromodulation (delta +24.27 ml vs -37.45 ml, p = 0.01). There was no significant change in other urodynamic or manometric parameters. Overall positive response rate was more than 75% for urinary (81%) and bowel (78%) function. Crossover analysis indicated that sacral neuromodulation is more effective than conservative treatment for both types of incontinence (p = 0.001). CONCLUSIONS: In a pediatric population sacral neuromodulation is effective for bladder and bowel dysfunction and should be considered before irreversible surgery.

[Acquired non hypertrophic pyloric stenosis in children]. / Sténose acquise non hypertrophique du pylore chez l'enfant.

UNLABELLED: Pediatric non hypertrophic pyloric stenosis (NHPS) are uncommon. Their causes and treatments are debated. MATERIAL AND METHOD: Retrospective review of all cases of NHPS from 3 pediatric surgery services during the period 1984-2002. RESULTS: Six children, aged 17 months to 15 years, underwent surgery for NHPS. Clinical symptoms, food vomiting and loss of weight, were present for several weeks before the diagnosis of NHPS was made. The diagnosis was peptic stenosis in 3 cases and has not been established in 3 cases. Search for Helicobacter pylori was negative in all cases. Failure of specific medical treatment and endoscopic dilatations led to pyloric resection in 3 cases and pyloroplasty in 3 cases. Post operative course was uneventful with normal oral feeding and normalisation of weight status. Histologic data were aspecific. No recurrence was observed. DISCUSSION: We discuss the origin of the pyloric stenosis, regarding clinical, operative and pathological data: were the stenosis the cause or consequence of peptic ulcer? Peptic disease is always advocated, but difficult to prove and may be excessively incriminated. Late symptomatic congenital and acquired idiopathic pyloric stenosis should be recalled. In all cases of proved pyloric stenosis, after failure of medical and endoscopic treatment, a simple surgical procedure (pyloroplasty) associated with medical treatment seems to be effective. CONCLUSION: The diagnosis of NHPS should be suspected in a child with food vomiting and loss of weight if his age is not concordant with hypertrophic pyloric stenosis. Upper gastro-intestinal series and endoscopy are diagnostic. The precise cause of the stenosis is more difficult to asses. When the medical treatment fails, a pyloroplasty is usually curative.

[A rare retroperitoneal tumor: abdomino-scrotal hydrocele. Two case reports]. / Une tumeur rétropéritonéale rare: l'hydrocèle abdominoscrotale. A propos de deux cas.

UNLABELLED: Abdominoscrotal hydrocele is a very rare condition, different from the majority of cases of hydroceles related to the persistence of the processus vaginalis. The anomaly consists of a large scrotal hydrocele which communicates in an hour-glass fashion with a large abdominal component through the inguinal canal. We report two cases observed in infants. CASE REPORTS: 1. A nine-month-old infant was referred because of a large bilateral hydrocele. On clinical examination on the right side in the lower quadrant of the abdomen was found a mass which communicated with the right scrotal pouch. Diagnosis of abdominoscrotal hydrocele was established by ultrasonography. During the surgical exploration the right testis was found to be dysmorphic, fusiform and ectopic in the inguinal canal. Surgical treatment comprised hydrocelectomy and right orchidectomy. 2. A six-month-old infant presented with a large right hydrocele in communication with an abdominal pouch located in the right lower quadrant, in association with a lymphoedema of the right limb. Diagnosis of abdominoscrotal hydrocele was confirmed by ultrasonography. During the surgical procedure the pouch was opened and everted. The testis was dysmorphic as in the first case, in normal scrotal position and was preserved. COMMENTS: Abdominoscrotal hydrocele is a rare condition reported in adult population and also in the infant. The exact mechanism by which it develops is unknown. The diagnosis can be suspected on clinical examination if an abdominal mass in a lower quadrant is palpable just above the inguinoscrotal pouch. The diagnosis relies on ultrasonography. Complications sometimes occur due to the pressure on adjacent structures (ureters, iliac vessels). Testicular dysmorphism has been reported in some patients. In any case, as spontaneous resolution of abdominoscrotal hydrocele has never been reported, surgical treatment is indicated.

[Congenital rectovestibular fistula. An unusual diagnosis that should not be ignored]. / Fistule rectovestibulaire congénitale. Un diagnostic exceptionnel à ne pas méconnaître.

UNLABELLED: Rectovestibular fistula without anorectal malformation is a rare anomaly. OBSERVATION: A six-week-old baby was hospitalized for a greater labium abscess. Stools leakage was secondary noted in the vagina and in abscess area. Clinical examination under anesthesia concluded to a congenital rectovestibular fistula with secondary drainage in the greater labium. Surgical treatment without colostomy led to recovery. COMMENTS: Congenital recto-vestibular fistula is a difficult diagnosis. It should be evoked when vaginal fecal leakage occurs in the absence of anorectal malformation or perineal injury. Fistula location is often confirmed by clinical examination under anesthesia. Treatment is surgical and allowed recovery without any functional damage.

Retroperineoscopic excision of a single blind ectopic ureter in childhood.

Retroperitoneoscopy is gradually gaining in importance in pediatric urology, especially for renal and adrenal diseases. Direct retroperitoneal pelvic access seems interesting in children when low urinary tract malformations are concerned. We report a case of a 12-year-old boy with a blind ectopic ureter managed by pelvic retroperitoneoscopy. We describe the surgical technique, focusing on the difficulty in the pediatric population. Nevertheless, retroperitoneoscopy is an excellent alternate way to manage such ureteral malformations.

[A rare case of epigastric heteropagus twinning]. / Un cas rare de jumeaux hétéropages épigastriques.

Epigastric heteropagus is a very rare form of conjoined twins. It results from an ischemic atrophy of the body structure of the monozygotic conjoined twins at an early gestational age. Diagnosis is made by prenatal echography which must look for congenital heart disease associated in 28% of epigastric heteropagus. Cesarean section is indicated to prevent for mechanical dystocia. The autosite component of epigastric heteropagus can successfully be treated with early minor surgery. Autosite twin survival is good. Discussion with the family is important to avoid needless terminations. We report a rare prenatal diagnosis of epigastric parasitic twinning in which the parasite had pelvis with lower limbs. At birth, the autosite had omphalocele containing only bowel loops from the parasite.

[Laparoscopic excision of an unusual appendiceal duplication]. / Exérèse laparoscopique d'une duplication appendiculaire atypique.

Duplications of the alimentary tract are rare congenital anomalies and vermiform appendix duplication is exceptional. The aim of this study was to report a case of cystic appendiceal duplication in a 4 years old child, unusual in its anatomic type, its clinical presentation and its way of management.

[Anterior hypospadias: Duplay or Mathieu?]. / Hypospadias antérieur: Duplay ou Mathieu?

OBJECTIVE: The Mathieu urethroplasty is considered to be the reference operation for the treatment of anterior hypospadias. The vertical incision of the urethral groove, described by Snodgrass, has extended the field of indications for urethroplasty by tubularisation of the urethral plate (Duplay principle), by allowing it to be applied in almost every case of anterior hypospadias, The objective of this study was to compare results of Mathieu urethroplasty and Duplay urethroplasty for the treatment of anterior hypospadias. MATERIALS AND METHODS: 50 Mathieu urethroplasties and 50 Duplay urethroplasties were performed by the same operator over the same period (1996 to 1999). All urethral sutures were performed by inverted running sutures. Urine drainage was ensured by Foley catheter for 48 hours. For Duplay operations, the urethral plate was incised longitudinally in 17 out of 50 cases. Foreskin reconstruction was performed 41 out of 100 cases. RESULTS: The follow-up is 6 months to 4 years. Three children (6%) in each group had to be reoperated because of urethral complications (fistula, meatal stricture, urethroplasty dehiscence). CONCLUSION: Our results confirm those published in the literature. Duplay and Mathieu urethroplasties provide equivalent results in terms of the urethra. The appearance of the urinary meatus appears to be more satisfactory after Duplay urethroplasty. The Snodgrass modification allows tubularisation of the urethral plate to be performed even when it is narrow. Cover of the urethroplasty by a foreskin subcutaneous connective tissue pedicled flap reduces the fistula rate of Duplay urethroplasty to the same value as that observed after Mathieu urethroplasty, although it classically used to be higher.

[Mathieu's urethroplasty in surgery for hypospadia postoperative complications]. / L'urétroplastie de Mathieu dans la chirurgie des complications postopératoires de l'hypospadias.

UNLABELLED: The aim of the study was to report the results of meatal based flap urethroplasty called Mathieu urethroplasty, in failed preliminary surgery for hypospadias. PATIENTS AND METHODS: Fourteen boys aged three to 14 years underwent surgery using a meatal-based flap urethroplasty for the correction of prior hypospadia repair complications. The secondary procedure was performed for glandular or coronal urethrocutaneous fistula in ten cases, completely failed urethroplasty in three cases, and for severe meatal retraction in one case. The surgical technique was the same as that used for the primary Mathieu procedures. RESULTS: Only one meatal retraction occurred; the other 13 cases had satisfactory procedure of hypospadias repair failures. Despite the scar skin used for secondary meatal based flap, good results can be achieved specially in two favorite indications: juxta coronal urethrocutaneous fistula and meatal retraction.

[Late revealing of congenital diaphragmatic hernia: diagnostic difficulties]. / Révélation tardive de la hernie diaphragmatique congénitale: difficultés du diagnostic.

Two patients, aged four and six months, with congenital diaphragmatic hernias, and who presented outside of the neonatal period, are reported. Clinical presentation was a progressive respiratory failure, and radiographic findings showed a consolidation of the left lung, associated with a pneumothorax in one and an ascites in the other. One had previous normal chest X-rays. All cases with herniated bowel showed connecting bowel segments passing through the diaphragmatic defect. All were repaired via an abdominal approach. Delay in presentation of congenital diaphragmatic hernias is not uncommon. Long-term prognosis is favourable and postoperative morbidity is minimal, despite late presentation and undesirable time delay between first sign and surgery.

Does the endoscopic incision of ureteroceles reduce the indications for partial nephrectomy?

OBJECTIVE: To determine whether the endoscopic incision of ureteroceles reduces the indications for partial nephrectomy. PATIENTS AND METHODS: Between 1987 and 1996, endoscopic incision was used as the first-line treatment of 18 children (13 boys, five girls, aged 8 days to 6 months) with a duplex-system ureterocele diagnosed antenatally (15) or in the first weeks of life during the course of a urinary infection (three). Of the 19 ureteroceles (one bilateral), four were intravesical and 15 ectopic, according to the American Academy of Paediatrics classification. Vesico-ureteric reflux into the inferior pole of the kidney was present in 10 children, seven of whom had an ectopic ureterocele. A functioning upper pole was detected by intravenous pyelography (IVP) in half the intravesical and in a third of the ectopic ureteroceles. RESULTS: Endoscopic incision resulted in decompression and reduction of dilatation in 16 cases; three with inferior pole reflux resolved on control cystography, whilst in seven with an ectopic ureterocele, reflux into the upper urinary tract was induced by endoscopic incision. In three children with an ectopic ureterocele, renal function had improved at 3 months, as assessed by IVP. Endoscopic incision was the only treatment for half the intravesical and six of 15 ectopic ureteroceles. Overall, nephrectomy was required in four of 18 patients (three partial nephrectomies for persistent dilatation and one total nephrectomy). Five nonfunctioning, undilated upper poles with no reflux were left in place. Nine vesico-ureteric reimplantations for persistent or induced reflux were carried out using the Cohen technique. CONCLUSION: Endoscopic incision can allow the deferral of nephrectomy, facilitate lower urinary tract reconstruction and reduce the indications for partial nephrectomy, if it is accepted that a nonfunctioning, undilated renal pole with no reflux can safely be left in place.