Association of N-acetylcysteine and glucagon during percutaneous cholangiography in the treatment of inspissated bile syndrome.

Inspissated bile syndrome (IBS) is a rare neonatal disease. In the majority of cases, it resolves spontaneously and treatment is conservative. Follow-up is recommended with close monitoring of laboratory tests. When IBS does not resolve spontaneously, a catheter can be inserted into the gallbladder for cholangiography, which allows irrigation and drainage. Despite this treatment, some biliary tract obstruction may persist. We report on the case of a 3-month-old infant whose continuous biliary obstruction caused by IBS was successfully managed by interventional radiology with the association of N-acetylcysteine and glucagon. Even as first-line agents, these would allow more rapid clearance of gallstones and prevent infectious complications of indwelling catheters as well as decrease the need for surgery.

Ovarian fibromatosis and sotos syndrome with a new genetic mutation.

BACKGROUND: Sotos syndrome is one the most common overgrowth conditions, after Beckwith-Wiedemann syndrome. As with other overgrowth syndromes, Sotos syndrome can be associated with an increased risk of tumors. CASE: We describe a young girl with Sotos syndrome and ovarian fibromatosis with a new mutation not reported before in the literature. SUMMARY AND CONCLUSION: Development of ovarian tumor in Sotos syndrome has been poorly documented. Ovarian fibromatosis is a very rare non neoplastic disease. Management is guided by the benignity of the lesion and consists of surgical excision of the fibroma.

[Open elbow dislocation with distal ischemia: a therapeutic emergency]. / Luxation ouverte du coude avec ischémie distale : une urgence thérapeutique.

We report the case of a 13-year-old boy with a traumatic elbow dislocation, open stage 2 (Cauchoix and Duparc), with distal ischemia. Exposure of the neurovascular humeral bundle through the wound accentutated the clinical emergency. This clinical observation consisted of elbow dislocation with all severity criteria: (i) opening of the skin, (ii) association with a fracture of the medial humeral epicondyle, and (iii) neurological deficit in the territory of the median nerve. The purpose of this report is to remind physicians that no investigation should delay surgery in elbow dislocation. Despite initial distal ischemia, no vascular exploration is required. Early reduction of the disloction is the key point of care.

Septic arthritis due to a Sneathia species most closely related to Sneathia sanguinegens.

Sneathia sanguinegens is an infrequent bacterium in clinical specimens. We describe a case of right elbow septic arthritis due to a Sneathia species most closely related to S. sanguinegens in a young immunocompetent woman. S. sanguinegens has never been implicated in osteoarticular infections.

[Strangulated umbilical hernia in children (Burkina Faso): differences with developed countries]. / Hernies ombilicales étranglées chez l'enfant au Burkina Faso: différences avec les pays développés.

Umbilical hernias occur frequently in children but complications are rarely reported. This study assesses the incidence of complicated umbilical hernias in our patients, evaluates data for risk factors, and shows dissimilarities with those encountered in developed countries. This study reports all children operated for complications due to strangulated umbilical hernia over a period of 3 years. On the whole, 162 children had umbilical hernias treated during this period. Thirty (18.5%) of these had complicated hernias. The average age of the complicated group was 3(1/2) years. Twenty-nine cases had a painful irreducible umbilical mass. Twenty-four children had bowel obstruction, while stercoral fistula occurred in one child. The average diameter of the hernia ranged between 1 and 1.5 cm. Five patients had ischemic intestine that required resection. One patient died. When active observation and follow-up after 1 year is difficult or not feasible when the wall defect diameter is 1.5 cm or less, and in suspicion of incarceration (unexplained abdominal pain, and irreducibility), umbilical hernia should be operated.

[Non vascularized toe phalangeal transfers for symbrachydactyly. Active range of motion without joint reconstruction]. / Transferts non vascularisés de phalanges d'orteils pour symbrachydactylie. Mobilités articulaires actives sans reconstruction articulaire.

Symbrachydactyly is a rare congenital malformation of the hand and its treatment is controversial. Non vascularized toe phalangeal transfers have been used for management of short digits for three children. Six phalanges have been harvested complete with their periosteum. No joint reconstruction has been performed and all children have undergone surgery at a young age. Four of six digits involved have an active range of motion (range 30 to 105 degrees ). All authors who have reported active range of motion of toe phalangeal transfers have performed joint reconstruction. Here, we report obtaining active range of motion of phalangeal transfers without necessity of joint reconstruction.

[A voluminous sacrococcygeal teratoma: prenatal diagnosis, in-utero treatment and obstetric management]. / Tératome sacrococcygien de la taille du faetus: surveillance anténatale, thérapeutique faetale in utero et prise en charge obstétricale.

Sacrococcygeal teratoma is the most common and benign fetal tumor. Fetuses with sacrococcygeal tumors that are predominantly solid and highly vascularized have a high risk of fatal issue. Hydrops and tumor hemorrhage are associated with a highest risk of fetal death. Management of these tumors includes ultrasound scan with Doppler and magnetic resonance imaging (MRI) is usually used for evaluation of its intrapelvic extension and relationship to the other structures. New in-utero treatments as vascular coagulation have been applied in fetuses with highly vascular teratomas but these techniques are still experimental and need more investigations. The management of delivery depends on associated anomalies, tumor vascularity and size.

Outcome of surgical management of concealed penis.

OBJECTIVE: To review the etiological factors, clinical presentations and outcome of our surgical technique to correct the anomaly of concealed penis. PATIENTS AND METHODS: Thirty consecutive patients were treated in 2000-2004. Mean age at operation was 6.7 years (range 13 months-15.4 years). They underwent penoplasty alone (22 patients) or penoplasty with liposuction of prominent prepubic fat pad (eight patients). The medical records of all the patients were retrospectively reviewed, and 23 patients were interviewed, examined clinically and questioned about their initial complaint and the results of our technique. Mean follow up was 28 months (range 6 months-5 years). RESULTS: The patients most commonly presented with cosmetic (60.0%), voiding (56.6%) and psychosocial (50.5%) concerns. Three underlying anatomic defects contributing to concealment were observed: fibrotic dartos fascia with poor skin attachment at the base of the penis (93%), prominent prepubic fat pad (40%) and postoperative phimosis leading to trapped penis (26%). Early postoperative complications occurred in 4 patients (13.3%) and all were treated conservatively and successfully. Fourteen patients (46.6%) underwent secondary minor day-case operations, the majority for excision of redundant subcoronal preputial collar. Clinical examination of 23 patients showed very good results in terms of general appearance and accessibility. The older boys were generally more satisfied with the results of surgery than the parents of younger children, whose main source of dissatisfaction was the final circumcised appearance of the penis. CONCLUSION: The surgical approach used to correct concealed penis alleviates the initial complaint and provides good cosmetic and functional results with greater satisfaction in older patients. Our technique had a low early complication rate but a significant number of patients needed a secondary procedure to improve the final cosmetic results.

Secondary localisation of an intra-thoracic benign mesenchymoma in the fossa poplitea: a rare paediatric case.

Thymomas are tumours that rarely occur in children, are almost invariably benign, and are usually discovered incidentally in the anterior mediastinum on chest X-rays. Whereas in adults these tumours are often associated with myasthenia gravis and other autoimmune diseases, this occurrence is very rare in the paediatric population. Multiple localisation and/or extra-thoracic recurrence of thymomas in children also appears to be exceptional with no reported cases in the English literature. We report one rare paediatric case.

[Early spontaneous ileal perforations in preterm infants: report of 4 cases]. / Perforations iléales précoces sans signes d'entérocolite ulcéronécrosante: à propos de quatre cas survenus chez des prématurés.

An ileal perforation occurred shortly after birth in 4 very premature newborns. Diagnosis was made on an abdominal distension with a pneumoperitoneum on X-ray. There were no biological, radiological nor histological signs of necrotizing enterocolitis. There were no digestive short- or long-term complications. According to the few authors who described this syndrome, there are some risk factors, but they were not clearly involved in our cases. Ileal perforation in the absence of signs of necrotizing enterocolitis is rarely reported but should be well known because of its good prognosis.

[Laparoscopic surgery for undescended testicles]. / Testicules impalpables. Place de la coelioscopie.

PURPOSE: Impalpable and undescended testis is a very common preoccupation for specialized pediatric surgery teams. The laparoscopy as the single most accurate modality for diagnosis and localization of impalpable undescended gonad is well established by all authors. However the therapeutic attitude and the technique are still a matter of debate. MATERIAL AND METHODS: A series of 122 boys presenting an impalpable and undescended testis was studied. All the children beneficiated of a primary laparoscopic exploration. During this exploration, when a testis was found, the clamping of the spermatic vessel was made and the final descent took place few months later. RESULTS: In our study the testis was found in 60 cases and the spermatic vessel's clamping assured the descent of a well vascularized testis in about 80% of cases. CONCLUSION: Clamping the spermatic vessel during the laparoscopic exploration assured, thanks to the development of a supply, a good vascularisation of the testis during its second step descent. Our retrospective study has showed the good reliability of laparoscopic surgery as primary treatment in front of impalpable undescended testicles in comparison to the one-step classical orchiopexy.

[Management of cleft lip and palate in university hospital of Rouen]. / Prise en charge des fentes faciales et palatines à la clinique chirurgicale du centre hospitalier universitaire de Rouen.

Cleft lips and cleft palates are managed in the department of Pediatric surgery in Rouen for the last 30 years. From the antenatal diagnosis, the parents got in touch with the surgeon who will coordinate this management. Around thirty new patients are treated every year. The chronology of the treatment is of "classic" manner. The cleft lip is repaired at about 3 weeks of age and the palatoplasty is performed after the age of 1 year. In view to maintain the intimacy of the consultation we did not institute multidisciplinary consultations. The other members of the interdisciplinary team will intervene during the follow up depending on the form of the cleft and the encountered problems. The information and the files circulate freely and are discussed together.

Epididymitis in children: is further investigation necessary after the first episode?

OBJECTIVE: The aim of this study was to evaluate the usefulness of investigating underlying urinary tract pathology after the first episode of acute epididymitis in children with no prior urological history, and also to assess the possible predictive factors of urological disease at the time of diagnosis. METHODS: Children with acute epididymitis were studied retrospectively over a period of 8 years. Diagnosis was made either using ultrasonography or surgical exploration. Renal ultrasonography and voiding cystourethrography were performed 1 month after epididymitis. RESULTS: These tests were carried out in 38 children who revealed 7 anomalies (18%). Only 1 patient received further surgery : endoscopic treatment of a ureterocele. We also studied 3 predictive factors: age <2 years; recurrence, and urine bacteria. None could be associated with the presence of urinary tract pathology. CONCLUSION: The authors suggest that, when there is no previous urological anomaly and absence of bacteriuria, routine screening for epididymitis should be carried out following the second episode.

[Intestinal volvulus after enterocystoplasty: report of three cases in children]. / Volvulus intestinal après entérocystoplastie: à propos de trois cas chez l'enfant.

Enterocystoplasty complications related to the presence of intestinal mucous presence in the bladder are well known and well reported in the literature. Mechanical problems in the intestinal tract due to using gut for bladder augmentation are not as well recognised. We analyzed three cases of children treated by ileocystoplasty who presented an intestinal volvulus in two cases and a cecal volvulus in one case around the vascular pedicle of the intestinal patch used for bladder augmentation.

Type 1-primary cutaneous meningioma of the scalp.

Type 1-primary cutaneous meningioma is a rare and often clinically unsuspected lesion of the scalp, forehead or paravertebral region which occurs at birth or during childhood. The pathogenesis of these lesions still remains uncertain. Several authors have emphasized that type 1-cutaneous meningiomas are not real tumors but sequestrated meningoceles or heterotopic meningeal nodules of the skin. Nevertheless, the search for an intracranial or intravertebral connection should be carried out. We describe the clinical and pathological features of a congenital type 1-meningioma of the vertex. No cranial defect or intracranial tumor was found. However, the cutaneous lesion was directly linked to a large cranial vein. The purpose of this study was to illustrate this rare lesion and to indicate the possible surgical risks and the pathological characteristics.

[US-guided percutaneous drainage of an infected epidermoid cyst of the spleen in a child]. / Drainage percutané sous échoguidage d'un kyste épidermoïde infecté de la rate chez un enfant.

Epidermoid cyst of the spleen is a rare entity (2.5% of all splenic cysts) and rarely becomes complicated by hemorrhage, rupture or infection. Classically, management consisted of total or partial splenectomy. We report the case of an 8-year-old boy presenting with a splenic abscess complicating an epidermoid cyst. Percutaneous drainage of the abscess was performed under sonographic guidance and completed by intravenous antibiotic therapy. Six weeks later, laparoscopic surgery was performed and the splenic parenchyma could be preserved.

Results of the Gil Vernet procedure in preventing contralateral reflux in unilateral ureteric reflux.

OBJECTIVE: To evaluate whether contralateral meatal advancement based on the technique described by Gil Vernet decreases the risk of postoperative contralateral reflux, which may occur after a unilateral reimplantation. PATIENTS AND METHODS: From January 1986 to 1997, 321 reimplantations were performed for unilateral vesico-ureteric reflux (VUR) using the Cohen procedure. In cases where the contralateral meatus was symmetrical or had a pathological appearance, preventive contralateral surgery with meatal advancement was performed. RESULTS: Ureteric reimplantation was exclusively performed unilaterally in 254 patients and in 67 a contralateral meatal advancement was performed. There were 29 cases of contralateral reflux at the 4-month follow-up. In nine patients contralateral reimplantation was necessary for persistent symptomatic VUR, the reflux resolved spontaneously in 14 and a radiological examination was necessary in six. Reflux also appeared on the Gil Vernet side in only 6% of patients; there were no clinical symptoms and the outcome was favourable. CONCLUSION: The advancement of the meatus using the Gil Vernet procedure is simple, with no surgical complications. We suggest that this technique constitutes a useful surgical alternative in the prevention of contralateral reflux.

[Extrarenal nodular nephrogenic blastema in the inguinal canal: report of two cases]. / Blastème néphrogène nodulaire extrarénal de siège inguinal: à propos de deux cas.

Two observations of extrarenal nephrogenic nodule in inguinal canal are reported. We underline the difficulties in differential diagnostic with true Wilms' tumor, and in sparing these pediatric cases from more aggressive treatment. The main morphological arguments in favor of non-tumoral nephrogenic nodular remnants are: the size of the lesion, the association with a congenital inguinal hernia suggesting a congenital abnormality, and the organoid organization of blastemic tissue surrounding the cavities lined with "hobnail" epithelium evoking a mesonephrogenic origin.