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.

Combination of clonidine sedation and spontaneous breathing-pressure support upon acute respiratory distress syndrome: a feasibility study in four patients.

INTRODUCTION: As alpha-2 agonists preserve ventilator drive, patients presenting with acute respiratory distress syndrome (ARDS, Pa02/FiO2 < 200) were managed using sedation with an alpha-2 agonist, clonidine, combined to spontaneous ventilation (SV) + pressure support ventilation (PS). METHODS: Sedation was provided by an alpha-2 agonist, clonidine 1-2 microg x kg(-1( x h(-1), without bolus administration, and supplemented with a neuroleptic, loxapine, if needed. Four patients presenting with ARDS were managed with pressure support ventilation (PS = 8 cm H20,rarely 10-12 cm H20) and high PEEP (10-20 cm H20). Energy requirements were minimized, if appropriate, with hypothermia caused by extra-renal replacement therapy or intentional hypothermia (35-36 degrees C). Repeated echocardiographic examinations revealed no right ventricular failure. RESULTS: Recovery of ARDS, i.e. sustained increase of P/F > 200 for > 24 h, was observed, over 2-5 days. CONCLUSION: Use of an alpha-2 agonist as first-line sedative agent led to absence of respiratory depression and spontaneous ventilation. Upon ARDS, the lowered intrathoracic pressure observed with SV+PSV allowed one to recruit alveoli with high levels of PEEP, without impairing right ventricle function.

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).

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.

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.

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.

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.

[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".

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.

[Phimosis: medical treatment or circumcision?]. / Décalottage ou circoncision: quelle indication dans le phimosis?

Phimosis remains a major question for mothers and induces a high number of consultations. However, large epidemiologic series have shown that congenital phimosis may disappear spontaneously in around 80% of cases with development and should'nt need any treatment before the age of five or six years old. Theses elements go against sociocultural habits which induce many circumcisions before the age of six. Corticoid treatment of phimosis seems to be very efficient to facilitate the disparition of phimosis. The aim of this literature review is to evaluate medical, socioeconomical and therapeutics aspects of phimosis. Based on these data, we recommend never to treat phimosis before the age of one year old, to wait as long as possible for spontaneous evolution of phimosis until the age of five years old, and, in case of non evolution of congenital phimosis or pathologic phimosis to use as first step treatment corticoid topics rather than surgical treatment.

[Cystic intrathoracic hemolymphangioma: a rare differential diagnosis of acute bronchiolitis in an infant]. / Hémolymphangiome kystique intrathoracique: un diagnostic différentiel rare de bronchiolite aiguë chez un nourrisson.

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

Genetic homogeneity of the urofacial (Ochoa) syndrome confirmed in a new French family.

The urofacial syndrome (UFS) or Ochoa syndrome has been reported as a rare autosomal recessive disorder comprising a uropathy and facial abnormalities. The gene was mapped on chromosome region 10q23-q24. We report the first European cases of UFS. Haplotype analyses in our French family were compared with those previously described in patients from Columbia and America (literature data). The results are compatible with the same localization of the critical region and favor the hypothesis of genetic homogeneity.

Sacrococcygeal teratoma with an intradural and extramedullary extension in a neonate: case report.

OBJECTIVE AND IMPORTANCE: The intradural extension of a sacrococcygeal teratoma is rare and has been reported previously in only two cases. We present a case of a sacrococcygeal teratoma with an intradural and extramedullary extension. CLINICAL PRESENTATION: The patient was a full-term female infant, born with a sacral mass. Ultrasonography, computed tomography, and magnetic resonance imaging showed an intraspinal extension. INTERVENTION: The operation confirmed the extension of the mass up to the T4 level, and total tumor removal was achieved. The pathological examination showed a mature teratoma. CONCLUSION: The postoperative course was uneventful, and at the 3-year follow-up examination, the patient had experienced normal growth without deficit in the lower limbs but had a neurogenic bladder. Magnetic resonance imaging disclosed no tumor recurrence. This case represents the third case in the literature of an intradural extension of a sacrococcygeal teratoma and the first one with an extramedullary component so extensive.

An experimental model of gastroschisis using fetoendoscopy: preliminary results and technical considerations.

BACKGROUND: The aim of this study was to show the feasibility of an experimental model of gastroschisis using fetoendoscopic surgery on sheep fetuses, and also to perform amnioinfusion until delivery using an in-dwelling intraamniotic catheter. METHODS: We analyzed the data from 18 pregnant ewes having 26 fetuses, which underwent surgery at 80 days of gestation (full term, 145 days). The fetendo technique was used to create a gastroschisis in 15 fetuses. The fetal abdominal wall was opened on the left side of the cord using scissors. The omentum and the intestinal loops were eviscerated using atraumatic forceps. Eleven fetuses were used as a control group. Twenty-one fetuses underwent amnioinfusion; a simple exteriorized catheter was used in seven cases and an intraamniotic catheter with an implantable port was used in the other 14. All ewes and fetuses were killed at the end of the experiment by an intravenous injection of pentotal; thereafter, the fetuses underwent necropsy. RESULTS: Twelve fetuses died and 14 survived (53.8%); seven of the 15 that underwent gastroschisis survived (46.7%). An amniotic infection occurred in nine fetuses (34.6%); of these, six died and three that were administered antibiotics survived. CONCLUSIONS: Our experience shows that this experimental model of gastroschisis is feasible and reproducible, and that a repeated amnioinfusion can be performed with an in-dwelling catheter in pregnant ewes. The use of an implantable port is safer than a simple exteriorized catheter.

Ureteropelvic junction obstructions: prenatal diagnosis and neonatal surgery in 47 cases.

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

Combined approach in the treatment of anorectal malformations: the MOPE procedure.

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

Management of pediatric laryngotracheal stenosis.

In the past 6 years, 40 children underwent surgery for laryngotracheal stenosis; 32 by the external approach and 8 by endoscopic CO2 laser. Twenty-seven children (67%) were less than 5 years old at the time of treatment and 80% of the stenoses (n = 32) corresponded to an etiology that is secondary to endotracheal intubation and/or tracheotomy. By grading the stenoses according to the amount of narrowing of the lumen, the authors emphasize the interest of conservative treatment (endoscopic for grade I [less than 70%, n = 8], and treatment by external surgical methods for grade II [70% to 90%, n = 13], grade III [90% to 99%, n = 14], and grade IV [total obstruction, n = 5]). At this time, the most commonly used technique is laryngotracheoplasty with costal cartilage interposition. In this series, 88% of the patients were successfully decannulated. As for the treatment of stenosis in infants, the authors describe their recent experience of laryngotracheofissure in 7 patients as an alternative to either tracheotomy in cases of difficult extubation or laryngotracheoplasty when the child is underweight.

Management of Hirschsprung's disease: curative surgery before 3 months of age.

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

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.