Biliary atresia: experience of a nord africain center.

BACKGROUND: Biliary atresia (BA) is a progressive inflammatory destructive process of the bile ducts occurring in about one of every 20.000 live births. If left untreated, biliary atresia can lead to liver failure. AIM: This is the first study on biliary atresia from Africa. The Aim of our study is to describe the clinical and prognostic aspects of biliary atresia in a Tunisian medical centre, where integrated medico-surgical management of children with liver diseases is lacking and liver transplant is not available. METHODS: Patients who were diagnosed with BA and underwent portoenterostomy between January 1985 and December 2010 at a tertiary regional hospital in Tunisia were included in this analysis. RESULTS: 74 patients were diagnosed with BA. The patients included 34 boys (45.9%) and 40 girls (54.1%). All patients received Kasai operation as the primary treatment and the median patient age at Kasai operation was 60 days (range 3-180 days). The median followup time for the patients was 72 months (range 2 months-23 years 6 months). Out of the 74 patients who received Kasai operation, 49 patients are being followed regularly in the outpatient clinic. Eight patients died immediately after Kasai operation by either hepatocellular decompensation or by cholangitis. Seventeen patients were lost to follow-up. Fifteen out of 49 patients who underwent portoenterostomy for BA are alive at median six years following Kasai intervention. Ten patients out of the 49 who are being followed regularly were Jaundice free. Two patients had portal hypertension. All these patients had survived. Five patients survived with signs of liver failure in four cases. Two of them had received a liver transplant abroad. Survival with the native liver was 6.7 % at 5 years with Kasai operation alone. CONCLUSION: BA still has a very severe prognosis in Tunisia Reducing the age at Kasai operation remains the most important target to reduce the need for LT in infancy and childhood. Centralised care will help to build surgical expertise.

[Gastric volvulus in children: five case reports]. / À propos de 5 cas de volvulus gastrique chez l'enfant.

UNLABELLED: Gastric volvulus is an abnormal rotation of all or a part of the stomach around one of its axes. It is a rare cause of intestinal obstruction in children. This anomaly can be primary, due to abnormalities of the gastric ligaments, or secondary to other congenital malformations. We report on the cases of five children treated between January 1994 and December 2011, four boys and one girl, with a medium age of 7 months. Diagnosis was based on clinical features, particularly in the upper gastrointestinal Rx contrast study, which confirmed the diagnosis. Four out of the five children underwent laparoscopic surgery with fixation of the stomach. A diaphragmatic hernia was associated in one case. Antireflux surgery was performed in three cases, and a diaphragmatic defect was closed in one case. The follow-up was uneventful after a medium period of 7 years. CONCLUSION: good knowledge of this anomaly is the guarantee of early diagnosis and optimum treatment to ameliorate the prognosis.

[Cervical teratoma in a child]. / Tératome cervical chez l'enfant.

Teratomas are unusual tumors derived from all 3 germs cells layers: endoderm, mesoderm, and ectoderm, with varying proportions. The cervical area is exceptionally affected. We report 4 cases of cervical teratoma. The clinically and radiologically suggested diagnosis was confirmed by histology. We describe herein the main clinical, radiological, and histological aspects and outcomes of this disease. Despite its most often benign histologic nature, cervical teratoma may threaten newborn infants' life due to airway compression. A multidisciplinary approach to the disease starting at delivery is required to improve the prognosis.

[Isolated congenital tracheoesophageal fistula]. / Fistules oesotrachéales congénitales isolées.

INTRODUCTION: Isolated tracheoesophageal fistula without esophageal atresia is a rare congenital malformation. Its etiology is obscure. Diagnosis is difficult but must be made early. PURPOSE: To study the clinical, radiological, and evolutionary sights of this malformation. PATIENTS AND METHODS: We report 4 cases of tracheoesophageal fistula, collected in the department of pediatric surgery of Monastir Hospital and in the neonatology unit of Sousse Hospital during the period between January 2001 and December 2010. RESULTS: The clinical picture consisted in a coughing bout and cyanosis after each feeding. Thoracic and abdominal imaging showed aspiration pneumonia, atelectasis, and gas within the colon. Gastrointestinal opacification demonstrated the fistula in 2 cases. Tracheoscopy visualized the tracheoesophageal fistula in the other 2 cases. Treatment was surgical and consisted in the section-ligation of the tracheoesophageal fistula with pleural interposition in all cases. The course was simple in two cases with a 3-year and 3.5-year follow up, respectively, but the infants died in the other 2 cases. CONCLUSION: Although a rare malformation, tracheoesophageal fistula should be suggested as a diagnosis when respiratory symptoms occur during feeding starting during the neonatal period.

[Esophageal strictures in children with epidermolysis bullosa]. / Sténoses œsophagiennes de l'épidermolyse bulleuse.

UNLABELLED: Esophageal stricture is a rare but often severe complication of recessive dystrophic epidermolysis bullosa in children. The purpose of the study was to review this digestive complication with emphasis on diagnostic modalities and therapeutic management. PATIENTS AND METHODS: This was a retrospective study of two pediatric cases of esophageal stenosis that occurred during generalized recessive dystrophic epidermolysis bullosa of the Hallopeau-Siemens type. RESULTS: The 2 patients were aged 8 years 8 months and 11 years 5 months, respectively. Dysphagia was of early onset, before the age of 10 years in both cases. Esophageal opacification led to the diagnosis of esophageal stenosis located in the upper 1/3 of the esophagus in 1 case and at the junction between the middle and the lower 1/3 of the esophagus in the other case. None of the 2 patients received medical treatment, and pneumatic dilation was the treatment method that was advocated. Esophageal endoscopy showed the stenosis and helped guide the positioning of the balloon catheter. These patients underwent 2 and 3 sessions of dilation, respectively, at intervals of 2 months and 1 year. Balloon dilation has allowed the patients to have a more comfortable life with decreased dysphagia and a substantial improvement in nutritional status. However, this improvement was transient (1 patient had symptomatic recurrence of stenosis after 3 years), which shows that monitoring of the patients and the resumption of dilatation sessions may be necessary. CONCLUSION: Esophageal strictures in dystrophic epidermolysis bullosa of the Hallopeau-Simens type are severe and difficult to support. Pneumatic dilatation is the treatment of choice for the fragile esophagus. It gives satisfactory results and can be repeated without significant risk.

[Thoracic congenital ectopic right kidney with diaphragmatic hernia: a rare developmental anomaly]. / Rein droit intrathoracique et hernie diaphragmatique congénitale : une association rare chez le nouveau-né

Ectopic intrathoracic kidney is a very rare congenital anomaly, which is often asymptomatic and discovered incidentally on chest radiography. Diagnosis of this ectopia in the neonatal period is extremely rare. We report the case of a female infant admitted for mild respiratory distress at birth in relation with maternofetal infection in whom a right posterior opacity on chest x-ray was diagnosed as intrathoracic kidney by sonography and chest CT scan. Diaphragmatic hernia could not be confirmed at this stage. At 2 months, the presence of air blebs on chest x-ray performed for viral bronchiolitis confirmed an associated right Bochdalek hernia. The infant, although asymptomatic, underwent surgery. The intrathoracic kidney was reduced into the abdominal cavity at the time of surgery. The postoperative course was uneventful and renal function was normal.

[Management and follow-up of antenatally diagnosed primary megaureters]. / Prise en charge et évolution des méga-uretères primitifs de découverte anténatale.

INTRODUCTION AND OBJECTIVES: To study the diagnostic and outcome aspects of antenatally diagnosed primary megaureters and analyse the results of the postnatal medicochirurgical management. PATIENTS AND METHODS: The authors report a retrospective analysis of 12 patients been born between 1998 and 2009 with primary megaureter antenatally diagnosed between 20 and 32weeks of gestation. A postnatal radiological assessment comprising urinary tract ultrasound, DTPA or MAG3 renal scintigraphy, retrograde cystography, DMSA renal scintigraphy and intravenous urography was performed. All the patients were put under urinary antiseptic treatment and followed since the birth. RESULTS: This study comprised nine boys and three girls, all been born at term with normal trophicity. Mean age of antenatal diagnosis of hydronephrosis was 26weeks of gestation. Mean age of definitive postnatal diagnosis was ten months. Left megaureter was seen in five children, right in two cases and five patients had bilateral megaureter comprising a total of 17 renal units of which eight units were non-obstructed/non-refluxing, three units were refluxing, five units were obstructed and one unit was obstructed and refluxing. Megaureter was associated to controlateral ureteropelvic junction obstruction syndrome in one patient and to ipsilateral ureteral duplicity with a superior polar kidney in another case. Renal function was less than 20% in four renal units. The mean follow-up was four years (range : 10months to 11years). Outcome was marked by urinary infections in seven cases of which four patients had non-obstructed/non-refluxing megaureter. Six patients required surgical correction, two for non-obstructed/non-refluxing megaureter complicated with urinary recurring infections, three for obstructed megaureter with severe renal functional deterioration and one patient for bilateral refluxing megaureter grade V. Two other patients required surgical treatment, one for controlateral ureteropelvic junction obstruction syndrome and the other for ipsilateral ureteral duplicity with destroyed superior polar kidney. Mean age at surgery was 22,6months (range : 6 months to 7 years). CONCLUSION: Half of our patients with antenatal diagnosis of primary megaureter required surgical correction. Urinary recurring infections, renal functional deterioration less than 20% and importance of ureteral dilatation with vesico-ureteric reflux grade V were predictive factors for surgery.

[Epispadiac urethral duplication in boys]. / L'urètre surnuméraire épispade chez le garçon.

Urethral duplication, also called supernumerary urethra, is a rare malformation essentially affecting the boys. It is defined by juxtaposition of two or more muscular channels with urinary tract mucosal lining. Various features may be observed depending on the site of the extra urethra and its incomplete or complete nature. In this study, the authors examined a particular aspect of this malformation, the epispadiac form.

Spontaneous perforation of the extrahepatic bile duct in infancy: report of two cases and literature review.

BACKGROUND: Spontaneous perforation of the bile duct (SPBD) is a rare disease in infancy. The pathogenesis, diagnostic modalities and treatment options for this condition are reviewed and discussed. METHODS: The authors report 2 new observations of SPBD in 2 male newborns aged respectively 27 and 21 days. RESULTS: The 2 newborns presented with cholestatic jaundice. Abdominal sonography showed an extrahepatic mass. Exploratory laparotomy revealed that the perforation was located in the cystic duct in the first case and in the common bile duct (CBD) in the second. The site of perforation was repaired and both patients underwent simple external biliary drainage. The postoperative course was uneventful for both patients with follow-up ranging from 2 to 4 years. CONCLUSIONS: SPBD is a rare, but important cause of surgical jaundice in infants. The pathogenesis of SPBD is unknown and multifactorial and diagnosis is often problematic. Surgical management is always required and a conservative approach is usually recommended. The prognosis is good with early surgical management.

[Intussusception owing to pathologic lead points in children: report of 27 cases]. / Les causes inhabituelles d'invagination intestinale aiguë: à propos de 27 cas.

OBJECTIVE: Intussusception owing to pathologic lead points is a challenging condition for pediatric surgeons. The aim of this study was to review the particularities of clinical presentation, the place of morphologic investigation in depicting the presence of an organic lesion and the management of secondary intussusception. PATIENTS AND METHODS: The authors report a series of 27 patients treated from 1986 to 2004, for secondary intussusception. RESULTS: Nineteen boys and 8 girls, aged from 45 days to 11 years (mean age: 40 months) presented with secondary intussusception: Meckel's diverticulum (13 cases); lymphoma (8 cases); intestinal duplication (3 cases); heterotopic pancreas (2 cases); intestinal polyp (1 case). All patients were operated upon after failure of hydrostatic reduction. An intestinal resection with an end to end anastomosis was done for 26 patients. The biopsy of a large abdominal mass after an easy reduction of the intussusception was performed in 1 case. Chemotherapy was started at the sixth postoperative day for the 8 children having lymphoma. Two of them died during therapy. For the 25 others, the postoperative course was uneventful with a mean follow-up of 4 years. COMMENTARY: The improvement of the management and the prognosis of secondary intussusception requires an early diagnosis. Morphologic examination must not be limited to the diagnostic of intussusception but must aim at searching a lead point. The reduction of this particular form is based exclusively on surgery.

[Morgagni hernia in infancy: report of 7 cases]. / Les hernies rétrocostoxiphoïdiennes chez l'enfant: à propos de 7 cas.

BACKGROUND: Morgagni or retrosternal hernia is a rare entity. It's representing almost 4% of all types of congenital diaphragmatic hernias in children. POPULATION AND METHODS: The data concerning 7 infants with Morgagni hernias (MH) have been retrospectively reviewed by the authors. RESULTS: Their ages at presentation ranged from 7 months to 11 years. There were 4 males and 3 females. The majority of patients had repeated chest infections. The diagnosis was made on chest radiograph and barium enema. All patients were operated through the abdomen (5 upper midline, 2 laparoscopic approach). The hernia sacs were excised and the defects repaired in all patients. DISCUSSION: Clinical awareness, early diagnosis and surgical treatment especially with laparoscopic correction, are important factors. CONCLUSION: Surgical correction is needed for asymptomatic MH in children to obviate the risk of complications and because the treatment is easy.

[Congenital esophageal stenosis due to tracheobronchial remnants: report of 2 cases and literature review]. / Sténose congénitale de l'oesophage par hétérotopie trachéobronchique: à propos de 2 cas et revue de littérature.

Congenital esophageal stenosis due to tracheobronchial remnants is a rare malformation whose diagnosis may be difficult. It is characterised by the abnormal presence of congenital tissue of tracheal origin in the esophageal wall, which is responsible for the narrowing of the esophagus. We report 2 cases whose treatment was surgical after failure of esophageal dilations. The presence of tracheal-bronchial tissue was confirmed by histological examination of the operative piece. Outcome was favourable and the final result was excellent. Recently, endoscopic ultrasonography has been proved useful in the diagnosis of congenital esophageal stenosis due to tracheobronchial remnants by showing the presence of cartilage, which explains the failure of dilation. The high rate of perforation in these cases is due to brutal fragmentation of the cartilaginous rings. Surgical resection of esophageal stenosis with the tracheobronchial tissue appears the only treatment susceptible to completely suppress the stenosis and its consequences.

[Omental lipoblastoma: a case report]. / Lipoblastome omental: à propos d'un cas.

Lipoblastoma is a rare benign neoplasm of foetal fat tissue. To our knowledge, less than ten cases of omental lipoblastoma have been reported in the literature. We present a new case of omental lipoblastoma in a 3-month old girl. The role of CT in the diagnosis and surgery planning of this tumor will be discussed.

[Malignant renal tumors before one year of age. Experience of a North African pediatric surgery service]. / Tumeurs rénales malignes avant l'âge d'un an. Expérience d'un service de chirurgie pédiatrique nord Africain.

UNLABELLED: The malignant tumours of the kidney are not very frequent during the first year of life and pose diagnostic and therapeutic problems. The aim of this work is to make an analysis of the epidemiologic, clinical and anatomo-pathological characteristics of these tumours during the first year of life and a development on the therapeutic methods and their results. MATERIAL AND METHODS: [corrected] This is a retrospective study of 8 observations of malignant tumours of the kidney whose first symptomatology appeared during the first year of life. RESULTS: The malignant tumours of the kidney observed before the one year age constituted 18% of the tumours of the kidney in the child. A female prevalence was noted with a sex-ratio of 0.6. The assessment of extension found cutaneous (one case), ganglionic (one case) and pulmonary (two cases) metastases. First chemotherapy was prescribed to five patients. The surgery consisted on a widened nephrectomy in seven cases and a tumorectomy for an infant presenting a nephroblastomatosis. The tumour corresponded to a nephroblastoma in seven cases and a rhabdoide tumour in the last case. Mortality was high (50%) caused by the toxicity of chemotherapy in three cases and an advanced stage of cancer in one case. CONCLUSION: Renal tumoral pathology occurring in infants less than one year of age poses true etiologic and therapeutic problems. The high frequency of the nephroblastoma and the absence of benign tumour in our series encourage us to evocate more often the malignant renal tumours and to practice per cutaneous biopsies in case of diagnostic doubt.

[Urethral duplication in boys: report of 4 cases]. / La duplication urétrale chez le garçon: à propos de 4 cas.

Urethral duplication is a rare anomaly, affecting mainly boys. The clinical presentation varies because of the different anatomical patterns of this abnormality. We report 4 cases of urethral duplication in children. An epispadiac duplication was present in 1 case, hypospadiac duplication in 1 case, Y-duplication in 1 case and a congenital prepubic sinus in the last case. The pre-operative probe was based on urethrocystography and fistulography. The surgical management included excision of the duplicated urethra in 3 cases. Transurethral incision of the intra-urethral septum was performed in the case of hypospadiac duplication. The characteristics of male urethral duplication are reviewed.

[Fetal rhabomyomatous nephroblastoma. Report of 2 cases and review of the literature]. / Le néphroblastome rhabdomyomateux foetal. A propos de deux cas et revue de la littérature.

OBJECTIVE: Fetal rhabdomyomatous nephroblastoma is a particular and very rare histologic variety of nephroblastoma. The aim of this work is to study the principal clinic, therapeutic and evolutive characteristics of the fetal rhabdomyomatous nephroblastoma through two personal cases and a review of the literature. PATIENTS AND METHODS: This is a retrospective study of two observations of fetal rhabdomyomatous nephroblastoma treated in the pediatric surgery departement of Monastir (Tunisia) among 47 cases of nephroblastoma. The diagnosis was confirmed in the two cases by the histologic examination. RESULTS: The two patients were a six and a sixteen months old boy and girl. They were admitted for a voluminous mass occupying the left half-abdomen. The radiologic and biologic explorations load, in the two cases, to the diagnosis of left nephroblastoma. After a first chemotherapy that did not induce a reduction of the tumoral volume, a widened left nephrectomy was performed for the two patients. The histologic examination of the two pieces of nephrectomy concluded to a fetal rhabdomyomatous nephroblastoma with existence in the second case of an extension of the lesions to the renal pelvis and ureter in the form of a pseudo-botryoïde tumor. The tumor was classified stage I in the first case and stage II N0 in the second. The treatment was completed by an adapted post operative chemotherapy according to the SIOP 9 protocol. The two patients are currently in complete remission with an overview of six years and half. CONCLUSION: The fetal rhabdomyomatous nephroblastoma is a special histologic form of nephroblastoma that is characterized by the paucity of pulmonary metastasis, the absence of response to chemotherapy and the possibility of tumoral extension in the renal pelvis and ureter. His prognosis is similar to the classical nephroblastoma.

[Pneumatic dilatation of caustic esophageal stenosis in children. Report of five cases]. / Dilatation pneumatique des sténoses caustiques de l'oesophage chez l'enfant. A propos de cinq cas.

The ingestion of caustic substances is a frequent accident in the child. The treatment of caustic strictures of the esophagus, which constitute the most frightening complication, varies from conservative treatment to esophageal replacement. We report the preliminary results of a prospective work established since July 1998 and aimed at evaluating the role of pneumatic dilatation in the treatment of these injuries. STUDIED POPULATION: The series included five boys with an average age of six years. Two children were neurologically handicapped. RESULTS: The caustic product was soda (three cases), a dishwasher solution (one case) and bleach (one case). Dysphagia was constant and concerned the semi-fluids in four cases. The stricture was extended over an average length of 4 cm. The total number of dilatations was 36, with an average of seven sessions/patient. No complication was noted. The result was good in four cases. The only failure was noted in a child who presented a gastroesophageal reflux secondary to the scarring phenomena. CONCLUSION: Pneumatic dilatation constitute a therapeutic mean whose results are attested. The best treatment remains, however, preventive: it is the setting out of reach of the child the dangerous products.