Free tubularised vesical mucosa graft for congenital stenosis of the urethra in children.

BACKGROUND: Reconstruction of urethral strictures in children remains a challenge to the pediatric surgeon as these are often related to different rare congenital anomalies with various clinical presentations that endanger renal function and should be repaired in young children. Multiple techniques have been described for their repair. We aimed to determine whether the use of a free tubularised bladder mucosal graft associated to a prior tubeless vesicostomy was feasible and sure, as this technique of reconstruction using tubularised grafts has not been described yet in young children. RESULTS: Two newborn male patients were referred to our department. Both presented a congenital stenosis of the urethra as a part of a complex urethral malformation. Surgery involved prior tubeless vesicostomy, free bladder mucosal graft for urethral reconstruction, and vesicostomy closure for both children. Postoperative evolution was satisfying in both children and cystourethroscopy showed permeable urethra. Satisfying cosmetic and functional results have been obtained in the two cases. CONCLUSIONS: The prior vesicostomy prevents kidney damage in the context of complex genital and urinary malformations. Bladder mucosa's immunohistological properties are the most similar to those of the urethral tissue, and are appropriate for this type of correction, making our technique feasible and sure. LEVEL OF EVIDENCE: 5.

[Management of urachal remnants in children: Is surgical excision mandatory?]. / Prise en charge des résidus de l'ouraque au cours de l'enfance : l'exérèse chirurgicale est-elle obligatoire ?

INTRODUCTION: The classical management of urachal remants consists in surgical resection, in order to prevent infections and long term malignancies. However, some reports have recently spread a wait and see management. The aim of our study was to report the results of the surgical management in our center. MATERIAL AND METHODS: We conducted a retrospective, monocentric review of all patients managed for urachal remnants from January 2005 to December 2014. RESULTS: Thirty-five patients have been operated during the study period (18 girls and 17 boys). Mean age at surgery was 4,9±4,4 years old. Twenty-seven patients were referred due to symptoms whereas 8 were discovered incidentally (4 by ultrasound scan and 4 during laparoscopy). Among them, 10 were urachal cysts, 15 were urachus sinusa and 10 were patent urachus. Thirty were operated using an open approach and 5 using a laparoscopic approach. Mean length of stay was 3,8±1,7days (1-10) with a mean duration of bladder drainage of 2,5±1 days. No major complications occurred. No abnormal tissue was discovered at the histological analysis. CONCLUSION: Presentation of urachal remnants is variable but surgical outcomes remain excellent in our experience. When symptoms occur, the surgical decision is easy, but when the diagnosis is incidental, the decision is much more complicated. Official guidelines could ease the decision process and the management of urachal anomalies.

[Laparoscopic transposition of lower polar vessels for pyelo-ureteral junction obstruction: preliminary experience]. / Transposition des vaisseaux polaires pour syndrome de la jonction pyélo-urétérale : expérience préliminaire.

AIM OF THE STUDY: Lower pole vessels are a classical cause of PUJO even in children. The "gold standard" in the management of PUJ obstructions remains a dismembered pyeloplasty as described by Anderson and Hynes. However, some authors have developed an alternative procedure to this approach with encouraging results. The aim of our study was to evaluate our preliminary results concerning laparoscopic vascular hitch for crossing vessels. MATERIAL AND METHODS: We conducted a retrospective, monocentric study of all patients managed by this technique from January 2010 to December 2012. RESULTS: Eleven patients (7 boys, 4 girls) were managed by laparoscopy at a mean age of 10.7 years (5.4-17). They were referred to our center for clinical symptoms (intermittent pain 7, high blood pressure 1, UTI 1), antenatal diagnosis or accidental discovery. Obstruction was confirmed by MAG3 nephrogram and the presence of obstructive vessels by tomodensitometry or MRI. Mean operative time was 90.2minutes (48-184). Seven patients over 11 were strictly managed by laparoscopic transposition of lower pole vessels. Four required a classical video-assisted dismembered pyeloplasty due to a potential intraluminal stenosis. The latter were suspected by a distension test with furosemid in all four cases. Mean follow-up was 12.9±3 months. Nine patients over 11 were totally non symptomatic, whereas 2 still present mild intermittent pain. In all cases, ultrasound scans show an improvement of the pelvic dilatation. CONCLUSION: Laparoscopic transposition of lower pole vessels is a suitable and feasible alternative for the management of obstructive PUJ. Our preliminary experience emphasizes the need for a precise preoperative selection of patients along with a per operative evaluation of the obstruction. Further experience seems required to improve our criteria in this indication. LEVEL OF EVIDENCE: 5.

[Van der Woude syndrome: An unrecognised clinical entity]. / Le syndrome de Van der Woude : une entité clinique méconnue.

Van der Woude syndrome is known to be the first syndromic cause of oral cleft. Apart clefts the cardinal signs are lower lip pits and hypodontia. IRF6 gene mutations have been recently identified as potential cause in this syndrome which permits to better understand its phenotype heterogeneity. Based on a literature review, we tried to cover the different aspects of this syndrome with an emphasis on genetic counselling and surgical correction of lip pits.

Safety and feasability of ureteroscopy for pediatric stone, in children under 5 Years (SFUPA 5): A French multicentric study.

INTRODUCTION: Ureteroscopy (URS) can be proposed as first-line therapy for the management of pelvic stones from 10 to 20 mm and for lower ureteric stones in children. However, little is known about the success and the morbidity of URS in young children. Ureteroscopic treatment may present matters in young children because of the small size of the pediatric kidney and the small size of the collecting system. OBJECTIVE: To assess safety and efficacy of URS for the treatment of urinary stones in children aged of 5 years or less. STUDY DESIGN: After the institutional ethical board approval was obtained, we conducted a retrospective, analytic, multicentric study that included all URS performed between January 2016 and April 2022 in children aged of 5 years or less. In this non-comparative case series, anonymized pooled data were collected from 7 tertiary care centers of pediatric patients. Endpoints were the one-session SFR at 3 months and per and postoperatives complications. Descriptive statistics were applied to describe the cohort. RESULTS: Eighty-three patients were included. For them, 96 procedures were performed at the median age of 3.5 years (IQR: 0.8-5) and median weight of 14 Kg (6.3-23). Median stone size was 13 mm (4-45). There were 65 (67 %) renal stones treated with flexible URS, most of which were in the renal pelvis (30 %) and in the lower calix (33 %). A ureteral access sheath was used in 91 % procedures. Preoperative ureteral stent was placed in 52 (54 %) of patients. None of patients had ureteral dilatation. The single-session SFR was 67.4 % (56.3 and 89.2 % for flexible URS and semi-rigid URS respectively) and children require 1.4 procedures to achieve complete stone clearance. The overall complication rate was 18.7 %, most of them were minor (Clavien I-II). Intraoperative perirenal extravasation (Clavien IIIb) due to forniceal rupture was documented in 6.2 % of cases, related to an increased intrapelvic pressure (IPP) performed in a closed pelvicalyceal system. DISCUSSION: Pediatric urologists should be aware of forniceal rupture based on the presence of extravasation of contrast during endourological procedures especially when they have difficulties to reach lower caliceal stone in small patient. CONCLUSION: URS in patients aged of 5 years or less, is a complex minimally invasive procedure with reasonable efficacy and low morbidity. Intrarenal stones treated by RIRS in young children carries the risk of additional procedures to complete stone clearance.

Delayed diagnosis of neurological bladder following spinal cord injury without radiological abnormality.

Spinal cord injuries are a well-known cause of neurological bladder and a well-known complication of high-velocity trauma. They can, however, be difficult to diagnose in young children, in whom the absence of initial neurological symptoms and normality of the radiological workup can be falsely reassuring. Management of associated injuries can further delay diagnosis. We present 2 cases of spinal cord injuries without radiological abnormality revealed by delayed bladder retention.

[Pediatric anterior urethral injuries: time to take stock]. / Mise au point sur la prise en charge des traumatismes de l'urètre antérieur de l'enfant.

INTRODUCTION: Anterior urethral injuries are a rare entity in the pediatric age group. Data of the literature are limited to the injuries of the posterior urethra. The aim of this study was to take stock of the initial management of this disease, from our experience and data of the literature. PATIENTS AND METHODS: A literature review and a retrospective study were conducted. We used our department database to find all the patients treated for anterior urethral injuries at the Children's Hospital of Toulouse between 2000 and 2011. Data on patients with trauma of the anterior urethra were analysed. RESULTS: Among the 13 patients treated for urethral injuries, seven patients had trauma to the anterior urethra. The initial symptom was an acute retention of urine in three cases (43%) and urethral bleeding in six cases (85%). All patients with acute retention of urine had emergency management consisting in endoscopic realignment and urinary diversion by suprapubic catheter. Patients with hematuria were treated with paracetamol and non-steroidal anti-inflammatory. After a mean follow-up of 507 days (332-893), none had dysuria and no posttraumatic stenosis has been demonstrated in uroflowmetry. CONCLUSION: In our experience, the trauma of the anterior urethra of the child had a satisfactory development through appropriate management. Clinical and uroflometry follow-up is necessary.

[Is there an outcome difference between posterior urethral valves diagnosed prenatally and postnatally at the time of antenatal screening?]. / Impact de l'âge au diagnostic sur le devenir à long terme des patients opérés de valves de l'urètre postérieur.

PURPOSE: Posterior urethral valves (PUV) diagnosed during childhood have classically been associated with a better outcome than antenatally diagnosed PUV. The aim of our study was to compare long-term outcome of these two patients' groups. MATERIAL AND METHODS: We retrospectively reviewed the medical records of boys with PUV managed between 1990 and 2010. Patient demographics, clinical background, radiographic data (including prenatal ultrasonography data when available), renal and bladder functional outcomes, surgical procedures and urinary tract infections (UTI) were abstracted. Impaired renal function (IRF) was defined as glomerular filtration rate less than 90 mL/min/1.73 m(2) at last follow-up. RESULTS: We identified 69 patients with confirmed PUV. Thirty-eight were diagnosed prenatally (group 1) at 30.5 weeks of gestation and 31 had a delayed diagnosis (group 2) at a median age of 6.31 years. At diagnosis, 20 patients in group 1 had renal insufficiency versus two in group 2 (P<0.05). At the end of mean follow-up of 7.2 ± 0.5 years, in group 1, 26.3% developed IRF versus 6.3% in group 2 (mean follow-up 2.3 years). Mean age at last follow-up was 7.3 years in group 1 versus 8.3 in group 2 (P>0.05). In group 1, 27% had voiding dysfunction versus 30% in group 2 (NS). In group 1, 35% had UTI during follow-up versus 10% (P=0.01). CONCLUSION: During the follow-up, the patients with delayed diagnosis VUP have developed fewer complications related to the initial obstruction than the population who was detected antenatally and managed from the early hours of life. However, the rate of IRF and voiding disorders in our study, associated with the data of the literature, highlights the potential persistence and worsening of these conditions. That is why, whatever the age at diagnosis, VUP patients require a close monitoring.

[Proteus syndrome: Case report of bladder vascular malformation causing massive hematuria]. / Une malformation vasculaire vésicale rare dans le cadre du syndrome de Protée.

Proteus syndrome is a rare, sporadic disorder consisting of disproportionate overgrowth of multiple tissues, vascular malformations, and connective tissue or epidermal nevi. Due to mosaic pattern of distribution, the phenotypes are variable and diverse. Vascular malformations are part of the major criteria used to define and diagnose this syndrome. It can involve the gastrointestinal tract, spleen, or the urinary tract but bladder malformations are rare. We report here a case of bladder vascular malformation in a 12-year-old boy known to have Proteus syndrome and review the literature on bladder malformations or tumors in this syndrome.

[Distal and late ureteral obstruction: a rare complication following dextranomer/hyaluronic acid injection for vesicoureteral reflux in children]. / L'obstruction urétérale distale secondaire : une complication rare du traitement endoscopique par Deflux(®) du reflux vésico-urétéral de l'enfant.

Surgically relevant obstruction after dextranomer/hyaluronic acid injection (Dx/Ha, Deflux(®)) for the treatment of vesicoureteral reflux (VUR) is rare with a 0.6% incidence. It occurs usually during the early postoperative period. We report here the case of a 9-year-old boy with a history of VUR who was previously treated with Deflux(®) and was referred more than 2 years later with acute flank pain (as he already did 2 weeks after surgery with a spontaneous relief under medical treatment). Initial radiological investigations showed hydronephrosis caused by distal ureteral obstruction which required open surgery removal of the Dx/Ha and Cohen procedure. This is the second case of delayed symptomatic obstruction due to Dx/Ha reported in the literature.

[Antenatal diagnosis of a congenital granular cell tumor]. / Tumeur granuleuse gingivale congénitale de découverte anténatale.

INTRODUCTION: Neuman's tumors also called congenital epulis or congenital gingival granulomatous tumors are rare and benign oral cavity tumors. They are usually discovered at birth. CASE REPORT: A 32 year-old pregnant female patient was followed for gravid diabetes well controlled. A maxillary tumor in the fetus was revealed by the third trimester US. A fetal MRI was prescribed, after discussion with the antenatal diagnostic center, to determine the localization, size, and nature of the tumor. It revealed a 3 cm long tumor with cystic like areas not communicating with the encephalus. A caesarian section was performed 2 weeks before term. A simple tumoral excision was performed at 12 hours of age. Histological analysis confirmed the diagnosis. There was no local recurrence after 6 months of follow-up. DISCUSSION: A prenatal diagnosis of congenital gingival granulomatous tumor is rare. It is usually made at the third trimester ultrasound scan, rarely with fetal MRI. Nevertheless, prenatal diagnosis allows for a better postnatal management.

[Long-term outcome of patients with renal transplant and neural tube defect]. / Suivi au long terme des patients transplantés rénaux ayant un dysraphisme spinal.

PURPOSE: To evaluate the long term outcome of renal transplant in patients with a neural tube defect causing voiding dysfunctions. PATIENT AND METHODS: Between 1993 and 2010, 18 cadaveric renal transplants were performed in 16 patients (5 females and 11 males) older than 15 years with a neural tube defect and voiding dysfunction. RESULTS: The patients had dialysis since the mean age of 27.4 and have been transplanted at the mean age of 32.2. The survival rate of the first kidney transplant was 93.75% at 1 year and 63.3% at 5 and 10 years respectively. With a mean follow-up of 6.67 years, 11 out of 16 first transplants remained functional (68.75%) The median survival of the first transplants was 13.52 years. At the end of the follow-up, 13 out of 18 transplants were still functional (72.2%). The mean serum creatinine level was 123.9 micromol/l with a mean glomerular filtration rate estimated by the simplified MDRD formula of 67 ml/min for the 13 still functional transplants. Before transplantation, 66% of patients had a neuro-urologic assessment versus 100% thereafter. CONCLUSION: Renal transplantation in patients with neural tube defect is feasible without surgical particularities to those of other renal failure causes. These type of patients represented less than 1% of the followed cohort with an average graft survival rate of 63.3% at five and 10 years. The median survival time of the first graft was 13.52 years.

[About a fatal infectious myositis: case report]. / A propos d'un cas de myosite infectieuse fatale.

Soft tissues' infections involve fascia and musculature which permit to define different anatomic entities as necrotizing fasciitis or myositis. We describe here the case of an infectious myositis whose initial clinical approach did not allow to get the right diagnosis. The tomodensitometry showed typical lesions which induced an accurate management, but could not avoid the death of the patient because of major wounds and a poor general background.

[Management of unintentional pediatric female genital trauma]. / Prise en charge des traumatismes du périnée accidentels chez la jeune fille.

INTRODUCTION: Unintentional pediatric female genital trauma is frequent in the daily practice of emergency wards. However, scientific data are rare in the literature, leading to variability in their management. The aim of this study was to evaluate our practice in order to obtain epidemiological data and define clinical guidelines. MATERIAL AND METHODS: We conducted a retrospective study from March 2013 to January 2015 and identified all emergency visits for this pathology. Data were extracted from the patients' charts and a statistical analysis was performed. RESULTS: One hundred and eighteen patients were admitted during the study period, with an average age of 5.9years. Straddle injuries accounted for 73 % of the injury mechanisms. Most wounds involved the majora and minora labia. Sixty-five patients did not require stitches (55.9 %); 29 patients were examined with Meopa® but 43 % required a more precise surgical exploration in the OR, due to the lack of compliance. Forty-six patients were sutured in the OR. Associated lesions (undiagnosed in the emergency department) were diagnosed during surgical exploration in 13 patients (22 %) with two urethral wounds. Significant wound size differences were observed in 69 % of patients between the pre- and intraoperative assessments. CONCLUSION: Surgical exploration under general anesthesia should be proposed for all unintentional female genital trauma unless the patient is older than 8 and allows complete examination at the emergency department consultation.

[Conservative management of tracheal injuries in children: Clinical case and literature review]. / Prise en charge conservatrice d'un traumatisme trachéal : cas clinique et revue de la littérature.

Tracheal injuries are a rare but potentially lethal entity, most particularly in pediatrics. While standardized management allows quick therapeutic decisions in adults, based on the results of the initial bronchoscopy, the use of diagnostic exams remains unclear during childhood. We describe the case of a 6-year-old patient with a posterior tracheal wall injury due to a car accident, which was managed without tracheal endoscopy. Based on our experience and on a literature review, we suggest clarifying the management of tracheal injury in children, defining the place of computerized tomodensitometry and endoscopy in the decision process.

Male infants with hypospadias and/or cryptorchidism show a lower 2D/4D digit ratio than normal boys.

BACKGROUND: In humans the ratio of the index finger to the ring finger is sexually dimorphic, with the mean ratio being larger in women than in men. It has been suggested that this difference is related to prenatal androgen exposure. This has been further demonstrated in children with congenital adrenal hyperplasia. Normal development of the male external genitalia is linked to androgen-mediated events during gestation. We therefore wanted to determine if the 2D:4D digit ratio was normal in boys with cryptorchidism or hypospadias. METHODS: We prospectively enrolled all prepubertal patients seen in the outpatient clinic for cryptorchidism or hypospadias between September and December 2012. We then compared their 2D:4D digit ratio with two control groups made up of normal boys and normal girls. Interobserver and intraobserver variability was evaluated. RESULTS: We included 57 boys with hypospadias and/or cryptorchidism, 79 boys without genital abnormalities and 25 girls without genital abnormalities. The mean 2D:4D ratio for both hands was significantly different between the three groups, with the digit ratio for boys with genital anomalies being lower than for normal boys and normal girls (p<0.0001). CONCLUSIONS: It appears that boys with genital abnormalities (cryptorchidism and/or hypospadias) have a lower 2D:4D digit ratio than boys without genital anomalies.

[Thymoma in children: a rare and difficult diagnosis]. / Thymome de l'enfant : un diagnostic parfois difficile.

Thymoma is extremely rare within the pediatric age range, which could lead to delayed diagnosis. Based on the clinical case of a mediastinal tumor in an 8-year-old patient, we detail the key points in the management of this disease highlighted by our recent experience.

[Biliary peritonitis after traumatic rupture of a choledochal cyst]. / Péritonite biliaire par rupture traumatique d'un kyste du cholédoque.

Choledochal cysts are rare congenital malformations of the biliary tract. Traumatic rupture of a choledochal cyst can be misleading. An 11-year-old boy was admitted for peritonitis and intestinal occlusion after blunt abdominal trauma, evolving over 48 h. Laparotomy revealed bile ascites and a suspected duodenal perforation. After referral to our center, a CT scan showed a perforated choledochal cyst. Six months later, a complete excision of the cyst was successfully performed. This treatment is mandatory because of the risk of further complications such as lithiasis, pancreatitis, cholangitis, biliary cirrhosis, and malignant transformation (cholangiocarcinoma).