Endoscopic injection of bulking agent around the ejaculatory ducts at the verumontanum for recurrent paediatric epididymitis.

INTRODUCTION: Paediatric recurrent epididymitis is frequently observed in several urogenital conditions, and may result in deterioration of long-term fertility. The management of recurrent epididymitis is still a therapeutic challenge for paediatric urologists, and as yet there is no consensus for treatment. OBJECTIVE: To present a minimally invasive endoscopic approach for the treatment of recurrent epididymitis (more than three episodes). PATIENTS AND METHODS: Eleven boys were referred with a history of recurrent epididymitis in a context of urogenital malformations. All children underwent endoscopic transurethral injection. Dextranomer/hyaluronic acid was injected around the ejaculatory ducts at the verumontanum (Summary Fig.). The medical records and outcomes of the patients were retrospectively reviewed. RESULTS: Of the 11 boys, two (18%) had a history of bladder exstrophy, three (27%) anorectal malformation, two (18%) peno-scrotal hypospadias, two (18%) posterior urethral valves, one (9%) seminal vesicle cyst, and one (9%) urethral stricture. The median age at injection was 3.75 years (range 8 months-14.7 years). Endoscopic injection effectively prevented recurrence in eight patients (73%) with a mean follow-up of 3 years (range 6 months-8.8 years). The mean injected volume was 0.7 ml/session. No perioperative complications were recorded. Vas clipping was performed in three patients after unsuccessful injections. DISCUSSION: The current discussion for management of recurrent epididymitis is mainly based on vas clipping. Endoscopic injection in the verumontanum could offer several potential advantages over vas clipping; moreover, it is easy to perform for an urologist who usually uses endourological approaches. It is believed that only Kajbafzadeh et al. have reported their experience with endoscopic injection in the verumontanum in seven patients with structural anomalies, and they had a 42% success rate. Similarly, the current study did not observe perioperative or postoperative complications. CONCLUSION: In this series, endoscopic injection of the verumontanum was considered to be a safe and effective treatment in almost 73% of children with recurrent epididymitis. It did not result in perioperative complications and not contraindicate a subsequent surgical procedure such as vas clipping.

Postural therapy for renal stones in children: A Rolling Stones procedure.

INTRODUCTION: Despite many advances, the management of renal stones - especially lower caliceal stones (LCS) - remains a challenge. The gravity-dependent location of the lower calices hinders the spontaneous clearance of fragments, which can be a nidus for future growth and symptomatic recurrence. Currently, there is no standard adjunctive therapy to facilitate fragment passage. OBJECTIVES: To report the safety and effectiveness of mechanical percussion diuresis and inversion (PDI) therapy for eliminating renal stones in children. PATIENTS AND METHODS: Since November 2013, children with residual fragments (after shock wave lithotripsy or flexible ureteroscopy) or native symptomatic renal stones were prospectively included in a protocol of four PDI sessions. After giving written consent, the children drank 10 ml/kg of water 30 min before therapy. They then laid in a prone Trendelenburg position on a couch angled at 45° and received continuous 10-min mechanical percussion applied over the affected flank by a physiotherapist (Figure summary). Tolerance stone burden reduction and stone clearance were documented with ultrasound 4 weeks after the last session. RESULTS: Seventeen participants, with a median age of 10.8 years (range 18 months to 18 years), received 82 PDI sessions performed over 22 months. The median stone diameter was 5 mm (range 3-9). All children tolerated the PDI therapy well. Over a median follow-up of 11 months (range 3-18), no significant adverse effects were noted. The overall stone-free rate was 65%. Four of the six patients with residual fragment passed their fragments. The patients who did not become stone free by PDI experienced a decrease in fragment size of 57% (range 34-71). The observance rate was 100%. DISCUSSION: Many studies have demonstrated that the gravity-dependent position of the lower calyces appears to be an important factor limiting the clearance of LCS. Positioning patients with a degree of inversion in order to put the collecting system beyond the horizontal plane affected the LCS through gravitational force. Complications were rare. PDI appeared to save costs and have similar success rates as shock wave lithotripsy for native small renal stones in children. CONCLUSION: PDI is safe and effective for facilitating gravity-dependent drainage of renal stones and provides an opportunity to treat children in a quick, non-invasive, economic, painless, non-radiative and diverting fashion. This therapy is a valuable alternative in the pattern of stone management. In case of persistent fragments, it is recommend that the number of sessions be increased to six.

Contribution of the foetal uro-MRI in the prenatal diagnosis of uronephropathies.

PURPOSE: To study the complementary diagnostic value and role in the perinatal management of foetal MRI in the prenatal diagnosis of abnormalities of the urinary tract. PATIENTS AND METHODS: Retrospective monocentric study from November 2002 to June 2011 of foetuses benefiting from an MRI after ultrasound diagnosis of uronephropathy abnormalities. Ultrasound and MRI data were compared with postnatal radiological and/or surgical data or with the foetopathology. The MRI analysis focused on the diagnostic concordance with the ultrasound, the complementary diagnostic contribution and/or a change in perinatal care. RESULTS: Of the 154 MRI examined, a follow-up was obtained for 108 cases. The indications for MRI were classified into six groups: suspected renal agenesis (n = 20, 18.5%), posterior urethral valve (n = 20, 18.5%), reflux or megaureter (n = 14, 13%), uretropelvic junction syndrome (n = 24, 22.5%), enlarged kidneys (n = 7, 6.5%) and others (n = 23, 21%). The information supplied by ultrasound was confirmed by MRI in 72 patients (67%). MRI provided additional data for 36 patients (33%) and changed the perinatal care for 16 patients (15%). CONCLUSION: Foetal uro-MRI is a useful complementary tool in the prenatal diagnosis of some uropathy abnormalities.

Oesophagogastric reconnection is possible.

INTRODUCTION: The first oesophagogastric dissociation was described by Bianchi in 1997 for the treatment of severe gastro-oesophageal reflux (GOR) in neurological patients to avoid serious respiratory complications. The dissociation leads to malabsorption and growth problems. We describe the first two cases of oesophagogastric reconnection long after lung growth. PATIENTS: Case no. 1 was a 12.7-year-old child with a history of type I oesophageal atresia. He required oesophagocoloplasty and gastric dissociation by the Bianchi procedure at the age of 3 months. He has oesophagogastric reconnection at 11 years old because he had a several macrocytic anaemia. Case no. 2 was also a 12-year-old boy, with an unlabelled multiple malformation syndrome with type I oesophageal atresia. He had an oesophagocoloplasty with gastric dissociation at the age of 3 months to protect his lungs. The reconnection was proposed at 10 years old because he developed multiple nutritional deficiencies and growth retardation. RESULTS: The two cases have no clinical symptom of reflux and their post-operative digestive contrasts are normal. Reconnection appears possible with or without preserving the Roux-en-Y loop. It improves absorption and corrects any vitamin deficiencies, allows the reconnected stomach to be monitored in terms of reflux and malignancy and enables enteral nutrition supplementation to be stopped. The possibility of reconnection after the Bianchi procedure therefore extends its indications, mostly in the case of oesophageal atresia complicated by severe GOR with pulmonary repercussions or tracheoesophageal cleft.

[Urinary dribbling in girls: which investigations in 2012?]. / Écoulement urinaire chez la fille: quelle imagerie en 2012?

Continuous urinary leakage, despite normal deliberate voiding, must suggest the diagnosis of ectopic ureter, more specifically in girls. Ectopic ureter is usually associated with duplex kidney and complete ureteral duplication. The strategy of investigations has changed over the past few years, due to MRI development in the analysis of urinary tract malformations. We report the case of a 4-year-old girl who presented with these symptoms and had a suspicion of left duplex kidney on the prenatal ultrasonography (US). Two US examinations during the first months of life were compatible with a left duplex kidney without any complication. Another US at the age of 4 years was reported as normal. We completed the investigation with MR urography, which revealed a left duplex kidney with a poorly functioning dysplastic upper pole, and the orifice of the ureter of this upper pole inserting in the vagina. The surgical treatment, realized by celioscopy, was partial nephrectomy of the upper pole, removing most of the ectopic ureter. US is the first exam to investigate urinary tract malformations. However, duplex kidney with small dysplastic pole and ectopic insertion of a nondilated ureter may be difficult to see, and a normal US should never end the investigations. Intravenous urography and renal scintigraphy used to be the reference complementary exams, but are now replaced by MR urography. Without using ionizing radiation, MR urography can visualize duplex kidney and ectopic ureteral insertion with high resolution, and evaluates renal function of each kidney and each pole. These morphological and functional data are essential to determine the surgical treatment.

Neurogenic bladder in children: basic principles, new therapeutic trends.

Diagnosis of neurogenic bladder is straightforward in children with myelomeningocele. However, recognition is more difficult in patients with occult dysraphism or central nervous system disorders since clinico-anatomical correlations are poor. Careful clinical examination and urodynamic exploration are mandatory for diagnosis and follow-up. Even if urinary leak is the first symptom, the main goal of the pediatric surgeon must be to preserve the upper urinary tract. The ideal protection strategy consists of ensuring that micturition is voluntary and complete and that the bladder capacity is sufficient with adequate compliance and sphincter outlet resistances. Balancing these functions requires a combination of medical and surgical treatment. A variety of techniques can be used depending on gender and age of the patient and social environment. In most cases, intermittent bladder catheterization is necessary to obtain complete evacuation of the bladder. Bladder capacity can be increased by anticholinergic drugs, injection of botulinum toxin into the bladder, and augmentation cystoplasty. Augmentation of bladder outlet resistances requires endoscopic injection of bulking agents, surgical bladder neck reconstruction and urethral lengthening, bladder neck suspension, and artificial urinary sphincter. In difficult cases, continent cystostomy with closure of the bladder neck can achieve definitive continence. At the beginning endoscopic treatment combining anti reflux procedure, injection of the bladder neck and botulinum toxin can be considered as a "total endoscopic management" and should be our first line. Other techniques are under evaluation. Sacral neuro-modulation has given promising results. Artificial tissue engineering will probably be used in the next future. Management of neurogenic bladder is not limited to urological considerations. Orthopedic, digestive, and sexual problems must also be taken into account in order to obtain an "acceptable quality of life".

Polymorphisms of MAMLD1 gene in hypospadias.

PURPOSE: Mastermind-like domain containing 1 (MAMLD1) is a causative gene for the fetal development of male external genitalia. Almost 10% of patients with both severe and non-severe hypospadias exhibit mutations of MAMLD1. The aim of this work was to determine whether polymorphisms of MAMLD1 are a genetic risk factor for hypospadias. MATERIAL AND METHODS: This study included 150 hypospadias with a range of severities and 150 controls. Direct sequencing of the MAMLD1 coding exons and their flanking splice sites was performed. In silico secondary and tertiary structure prediction and accessibility of changed amino acids were evaluated using JPred, Netsurf and PHYRE software. Functional studies of the transactivation of haplotypes on Hes3 promoter were performed in vitro using cDNAs of missense variants of MAMLD1. RESULTS: The p.P286S polymorphism was identified in 17/150 patients and 12/150 controls (11.3% vs. 8.0%, p = 0.32). The p.N589S polymorphism was identified in 22/150 patients and 12/150 controls (14.6% vs. 8.0%, p = 0.068). The double polymorphism (S-S haplotype) was present in 16/150 patients and 6/150 controls (10.6% vs. 4.0%, p = 0.044, OR = 2.87, CI from 1.09 to 7.55). The association of polymorphisms consistently revealed a modification in the structure prediction or amino acid accessibility in all three in silico models. The P286S, N589S and P286S + N589S proteins did not exhibit reduced transactivating activity on Hes3 promoter. CONCLUSION: Polymorphisms of MAMLD1 gene are frequent in patients with hypospadias. Although no change in transactivation was noted on Hes3 promoter, the in silico studies and the significantly increased incidence of the S-S haplotype in hypospadiac patients raise the hypothesis of a particular susceptibility conferred by these variants.

Pulmonary sequestration of the left upper lobe associated with a bronchogenic cyst: case report of an exceptional association.

Intralobar pulmonary sequestration (ILPS) and bronchogenic cyst are rare congenital diseases. We present the first case of the association between an ILPS located in the upper lobe and a bronchogenic cyst. This association has been discovered antenatally in a girl. The cystic lesion has been shown by a fetal MRI and confirmed by a CT scan at 3 weeks of life. She underwent a left upper lobectomy by thoracoscopy at the age of 5 months. Postoperative course was uneventfull. This case confirms the congenital origin of pulmonary sequestration and shows the interest of an early surgery.

Frantz's tumor: is mutilating surgery always justified in young patients?

BACKGROUND: Solid pseudopapillary tumor (Frantz's tumor) of the pancreas is a rare lesion. It is of low-grade malignancy but can cause extensive local invasion. The aim of this study was to assess the outcome of Frantz's tumors after incomplete resection. METHODS: We contacted all authors who published case reports describing incomplete resection of Frantz's tumor between 1985 and 2008 to request follow-up information. RESULTS: Follow-up information was obtained for 11 out 18 patients who underwent incomplete resection. Estimated median survival rate was 5.7 years (69.5 months). CONCLUSION: Since Frantz's tumor typically develops mainly in children and young women, a 5.7 year survival rate is unacceptable. Thus complete resection of locally invasive solid-pseudopapillary tumor of the pancreas is always justified, even at the price of difficult, mutilating surgery.

Association of three different congenital malformations in a same pulmonary lobe in a 5-year-old girl.

We report the case of a 5-year-old girl with persistent chest X-ray abnormalities following an episode of pneumonia who has a complex congenital pulmonary malformation comprising of a congenital pulmonary airway malformation, an intralobar sequestration and two bronchogenic cysts, all present within the same lobe. The observation suggests a common embryological origin of these malformations.

Laparoscopic nephrectomy of a cross-fused ectopic kidney in a child with hypertension.

OBJECTIVE: We report a case of laparoscopic nephrectomy of a cross-fused ectopic kidney in a 4-year-old girl with renal hypertension and Fanconi anemia. MATERIALS AND METHODS: We performed a transperitoneal laparoscopy. Ectopic kidney resection was done after dissection of the pathological kidney and after clamping vessels, using an ultrasonic device. Hospitalization time was 4 days. RESULTS: At 6 months, blood pressure was normalized and the patient showed an adequate growth curve. CONCLUSION: The transperitoneal route is very effective when a nephrectomy is necessary. It offers perfect exposure with limited risk of complications.

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.

Intestinal loop lengthening: early treatment of vanishing bowel.

Bianchi's procedure experience for short bowel syndrome in children is positive. This technique is generally performed after the first year of life. Here the authors propose a case of gastroschisis with prenatal spontaneous closure of abdominal defect and vanishing gut presenting as intestinal atresia, absence of ileo-cecal valve, and residual short intestinal dilatation, treated by early gut lengthening (ILP).

Abdominal lymphangiomas in children: interest of the laparoscopic approach.

BACKGROUND: Lymphangiomas are rare benign lesions of the lymphatic system. The most common symptoms are abdominal tumor or "acute abdomen" in children. The treatment of choice is complete surgical resection, but the recurrence rate with incomplete resection is high, and laparotomy exposes the patient to adhesions. The authors report their experience with the lymphangioma laparoscopic approach. METHODS: This retrospective study examined 15 consecutive operations for lymphangiomas in children, ages 5 months to 14 years, treated during the 5-year period from 1999 to 2004. RESULTS: Six patients were treated using the primary laparotomy approach, and nine patients underwent the laparoscopic procedure, six successfully. Three conversions were necessary (1 case requiring partial colectomy, 1 retroperitoneal case with adherence on the aorta and vena cava, 1 case with partial volvulus). Morbidity included two cases of acute occlusion caused by adhesions after laparotomy. There was no recurrence of lymphangioma during a mean follow-up period of 35 months. CONCLUSION: The laparoscopy procedure could be used successfully for abdominal lymphangioma, even in an emergency. When the laparoscopic resection is impossible, laparotomy or sclerotherapy can be discussed.

Quality of life and continence in patients with spina bifida.

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 improved HRQoL.

[Neurogenic bladder in children: basic principles in diagnosis and treatment]. / Vessies neurologiques de l'enfant: approche diagnostique et thérapeutique.

The diagnosis of neurogenic bladder can be easy in myelomeningocele and much more difficult in occult dysraphia or medical etiologies. Careful clinical examinations and urodynamic investigations are mandatory for the diagnosis and the follow up of affected patients. Clinico-anatomical correlations are poor. If urinary leak is the first apparent symptom, preservation of the upper urinary tract is the main goal of the surgeon. If natural history of the neurogenic bladder is destruction of the detrusor and paralysis of the trigona, obstructive uropathy is the main physiological concern. Urinary leak must be integrated in the global context of the bladder function in order to determine urinary incontinence type. Ideal micturition is voluntary, must be complete, and needs the synergistic action of a reservoir with a good capacity, a normal compliance, and adequate sphincter outlet resistances. Continence is obtained by balancing these functions, and associating medical treatment and surgery is necessary. Bladder intermittent catheterization is the clue to obtain in most of the cases complete evacuation of the bladder and protection of the upper urinary tract. Increasing bladder capacity is achieved more often by augmentation cystoplasty (colon, ileus, stomach and ureter can be used). Autoplasty at the beginning, artificial tissue engineering will be the future. Augmentation of the bladder outlet resistances need surgical reconstruction (young dees, Pipi-salles procedures...) or uretral and bladder neck suspensions, artificial urinary sphincters, endoscopic injections of bulking agents. All these techniques can be proposed and combined according to the patient's gender, age and social environment. Continent cystostomy allows obtaining continence in difficult cases and after unsuccessful surgery of the bladder neck. Other techniques are under evaluation and sacral neuromodulation give at the moment some promising results. Managing neurogenic bladder must not be considered only in urological terms: orthopedic troubles, digestive and sexual disorders must not be forgotten in order to obtain at least an "acceptable social life".

Duodenal duplications. Clinical characteristics, embryological hypotheses, histological findings, treatment.

BACKGROUND: The aim of this study was to analyse different clinical aspects and embryologic hypotheses of duodenal duplications. METHODS: Duodenal duplications occurring since 1995 were recorded. The age of the children at the time of diagnosis, the sex, location of the duplication, type of mucosa, clinical signs, associated lesions, and the type of surgical intervention were defined. RESULTS: We identified 5 patients (3 girls and 2 boys) who presented with histological or intraoperative findings of duodenal duplication. Their ages ranged from 4 days to 9 years, with the exception of two prenatal diagnoses. Three children were symptomatic: high intestinal obstruction (1 case), digestive bleeding (2 cases). In 1 case we found a palpable abdominal mass and 1 case was completely asymptomatic (prenatal detection). The abnormality was located on the duodenal concavity, originating from the third part in 2 cases and from the second part in 3 cases. All cases were non communicating types, 4 of which were cystic duplications and 1 was a tube-like variety. The epithelial lining was duodenal mucosa in all patients, but gastric heterotopies were identified in 2 cases. We performed two complete resections and 3 intraduodenal derivations. The outcome was uneventful in 5 cases with an average follow-up of 2 years. CONCLUSION: Duodenal duplications are rare malformations with several anatomical varieties. The preferred treatment for duodenal duplications is complete removal when the location allows it without endangering nearby anatomical structures.

A multicenter evaluation of urinary incontinence management and outcome in spina bifida.

PURPOSE: We describe urinary continence management and outcome in patients with spina bifida to identify the procedures that are most successful. MATERIALS AND METHODS: In a multicenter retrospective cohort study medical charts were studied. At the same time in a cross-sectional survey sociodemographic characteristics, orthopedic features and urinary continence were described based on the frequency of leakage from the viewpoint of patients or close relatives using a Likert scale of 5 items, namely 1-leakage permanent to 5-leakage never. RESULTS: A total of 421 patients were included, of whom 191 (45%) had been medically treated with a normal voiding pattern according to the patient viewpoint in 21%, clean intermittent catheterization in 61% and no specific bladder emptying method in 18%. The mean leakage score +/- SD was 2.74 +/- 1.55. On the other hand, 230 patients (55%) were surgically treated. Except for 23 patients who underwent noncontinent urinary diversion 207 were considered for treatment and continence description. The mean leakage score was 3.45 +/- 1.60. An artificial urinary sphincter in male and females, and a sling or Kropp technique in females were satisfactory when bladder enlargement was not required. In cases of bladder augmentation without continent diversion an artificial urinary sphincter in males and a bladder neck sling or cinch, Kropp and Young-Dees procedures in females have provided the best results. In cases of bladder enlargement with continent urinary diversion bladder neck closure or a wrap have provided the best results whatever the patient sex. CONCLUSIONS: Many factors may influence the choice of a technique, such as patient sex, bladder characteristics or orthopedic conditions. However, since to our knowledge no randomized, controlled study has been yet performed, definitive conclusions on the best way to achieve urinary continence in patients with spina bifida cannot be established.

A multicentre study of the management of disorders of defecation in patients with spina bifida.

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.