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.

Huge Non-parasitic Mesothelial Splenic Cyst in a Child: A Case Report and Literature Review.

BACKGROUND: Splenic cysts are one of the relatively rare conditions in pediatric surgery practice. Primary non-parasitic splenic cysts are even more scarce. CASE PRESENTATION: A 13-years-old female patient presented with chronic left hypochondrial pain of 2 months duration. Abdominal ultrasonography and computed tomography revealed huge 18 cm × 14 cm × 10 cm splenic cyst. Deroofing of the cyst was done which was complicated by secondary infection. Subsequently, the patient was re-operated on and partial splenectomy done with good outcome at 6 months follow up. CONCLUSION: Partial splenectomy is the best management strategy for huge non-parasitic splenic cysts in children. There is also less recurrence rate of splenic cysts with preservation of splenic functions.

Contribution of laparoscopy in the abdominal tuberculosis diagnosis: retrospective study of about 11 cases.

OBJECTIVE: Abdominal tuberculosis is one of the most frequent extra-pulmonary localizations. Its diagnosis is difficult and may lead to a delayed prescription of specific treatment. This study is aimed at stressing the role of laparoscopy associated with a biopsy in the diagnostic confirmation of abdominal tuberculosis particularly in doubtful cases. METHODS: The diagnostic features of 11 cases hospitalized for abdominal tuberculosis in the Paediatric Surgery Department of Fattouma Bourguiba Hospital in Monastir for a 6-year period (2001-2006), were evaluated retrospectively. The diagnosis of abdominal tuberculosis was substantiated histopathologically by laparoscopy in all cases. The epidemiological and clinical characteristics along with the laboratory, radiological and histological data were studied. RESULTS: Eleven cases of abdominal tuberculosis with a mean age of 5.6 years were diagnosed. It was peritoneal tuberculosis in all cases and associated with intestinal localization in one case. A conversion to laparotomy was practiced in three patients: appendicular plastron in one case, pseudo-tumor aspect of an intestinal loop in another case and because of their pathological aspect appendicectomy and caecum biopsy in the third. The diagnosis was confirmed histologically by biopsies in nine cases and on excision pieces in the other two cases. All patients had an uneventful course with an antituberculosis treatment. CONCLUSION: Abdominal tuberculosis is still frequent in Tunisia. Because of its non-specific clinical presentation and the limited means of investigation, a laparoscopy with biopsy should be practiced as first line diagnostic tool in case of doubtful abdominal tuberculosis. The earlier the diagnosis is established and an adapted antituberculosis treatment is started, the better the prognosis is.

To determine validity of ultrasound in predicting acute appendicitis among children keeping histopathology as gold standard.

BACKGROUND: To determined the accuracy of ultrasound in diagnosis of acute appendicitis in children keeping histopathology as gold standard. METHODS: A prospective evaluations of all ultrasound for appendicitis from January 1, 2014, to June 15, 2017, was conducted at our hospital. A diagnostic protocol was implemented to reduce radiation exposure employing US as the initial imaging modality followed by CT for non-diagnostic US studies in patients with an equivocal clinical presentation. The imaging, operative findings, and pathology of 223 patients (females 80, males 143, age less than 14years) with diagnosed appendicitis were collected. The sensitivity, specificity, predictive value, and negative appendectomy rate were also analyzed. All those patients which had subjected to surgery were included to evaluate the true result of ultrasound in diagnosis of appendicitis. RESULTS: Of the 223 pediatric appendectomies performed in this time period, a total of 192 (86%) were diagnosed by ultrasound. The histopathology of 8 was normal (3.6%), CT done in 11 and three was normal. The negative appendectomy rate was 3.6%. US were the sole imaging modality in all patients. CONCLUSIONS: In the diagnosis of acute appendicitis in children, ultrasound is useful and accurate mode, which results in a significant decrease in negative appendectomies with no increase in the number of CT scans. This has important implications in the reduction of childhood radiation exposure. STUDY DESIGN: cross sectional validation.

Ovarian inflammatory myofibroblastic tumor in children.

Inflammatory myofibroblastic tumors or inflammatory pseudotumors are uncommon solid tumors that are highly vascularized, have an intermediate prognosis, and are associated with local recurrence, and rare metastasis. Inflammatory myofibroblastic tumors (IMT) most often are seen in the lung of young adults, but there have been reports of these tumors occurring in children, in various sites. The authors report a case of an ovarian IMT arising in a six-year-old girl (third case reported in the literature), treated by a right adnexectomy with uneventful postoperative course, and discuss recent reports.

[Polyorchidism: report of 2 cases and review of the literature]. / La polyorchidie: a propos de 2 cas.

Polyorchidism is a rare urogenital abnormality. The authors report two cases in children aged 3 years and 5 years with left ectopic testis. Surgical exploration demonstrated two testes on the left side with a third testis on the right. Descent of the two testes into the left hemi-scrotum was performed. The authors review the management of polyorchidism in the light of these two cases.

[Treatment of urinary stones in infants]. / Traitement de la lithiase urinaire du nourrisson.

Up until the beginning of the 1990s, surgery was the only available modality for the treatment of renal and ureteric stones in infants. From 1990 onwards, two new minimally invasive techniques were developed, leading to a real revolution in the management of these patients. The first and most spectacular breakthrough was extension of the application of ESWL to this age-group and the second breakthrough was ureteroscopy following the enormous technological progress in the manufacture of endoscopes. This technique is increasingly useful when lithotripsy is impossible or insufficient. Surgery is still indicated in the case of contraindications to minimally invasive methods or after failure of these methods. It is the method of choice for bladder stones. Medical treatment or watchful waiting have valuable indications, at the cost of regular clinical and radiological follow-up of patients. The choice of treatment modality depends on the size of the stone, its site, its composition, the anatomy of the urinary tract and the availability of the various techniques.

[Nephroblastoma and Wilms tumor: report of 2 cases]. / Néphroblastomatose et néphroblastome: à propos de 2 cas.

Nephroblastomatosis is a rare disease, considered to be a precursor of Wilms tumour. When it is isolated, recommended treatment consists of chemotherapy followed by surveillance. In the presence of associated Wilms tumour, treatment consists of preoperative chemotherapy followed by partial nephrectomy (when possible), followed by postoperative chemotherapy and surveillance.

Soave transanal one-stage endorectal pull-through in the treatment of Hirschsprung's disease of the child above two-year-old: a report of 20 cases.

BACKGROUND: The definitive treatment of Hirschsprung's disease is the removal of the aganglionic bowel by a pull-through surgery. In most cases, this surgery is performed in infancy or in the neonatal period as presentation in older children and adulthood is uncommon. MATERIALS AND METHODS: It is a retrospective study of 20 patients above two-year-old who underwent a transanal Soave one-stage endorectal pull-through procedure for Hirschsprung's disease between January 2002 and December 2010. RESULTS: Twenty patients were recruited in this study. Fourteen were males and six were females. Patient ages ranged from 2 to 14 years (median age: five years and three months). All patients presented with persistent constipation and abdominal distension. Two of them had an intestinal obstruction that required colostomy. Ten patients (50%) had a recto-sigmoid Hirschsprung's disease. All patients were operated on using a Soave one-stage endorectal pull-through procedure. The laparoscopy was necessary during the pull-through in three cases. The average duration of the intervention was 240 minutes. That represents almost the double of the duration of the same procedure in newborns and infants in our department (130 minutes). Early postoperative complications included one case of anastomosis leakage and one case of intussusception. Late postoperative complications were perineum irritation in five cases (25%), anal stenosis in four cases (20%) and enterocolitis in one case (5%). None of our patients developed fecal incontinence. Soiling was reported in four cases (20%). There was no death. CONCLUSION: Soave transanal one-stage endorectal pull-through is safely feasible in children of more than two years of age. Laparoscopy may be necessary whenever there are difficulties in the pull-through.

Pyloric atresia: A report of ten patients.

Pyloric atresia (PA) is uncommon. It occurs in 1:100000 live births. Neonates usually present soon after birth with copious non-bilious vomiting. The treatment is surgical and its prognosis is poor, especially, when it is associated with epidermolysis bullosa (EB). The aim of this study was to evaluate the clinical presentation, diagnosis, operative management, post-operative courses, and outcome in infant with PA, based in our cases and literature review. Charts of 10 patients who underwent surgery for PA in the department of paediatric surgery in a Teaching Hospital in Tunisia (Monastir) between 1990 and 2012 were reviewed. Data were analysed for demographic, clinical, therapeutic, and prognostic characteristics. The average of age at presentation was 2 days and there were six males and four females. The main presenting symptoms were non-bilious vomiting in 90% of cases. Abdominal X-ray showed gastric dilatation with an absence of gas in the rest of the intestinal tract in 90%, and a pneumoperitoneum in one. The surgical approach was laparotomy in all cases. Gastric perforation was observed in one patient and was completely repaired. The distribution of the anatomic variations was type A in nine cases and type B in one. Five patients underwent excision of the diaphragm and Heineke-Mikulicz pyloroplasty and gastroduodenostomy in the other five cases. Identified associated anomalies were Down's syndrome in one and EB in 2 (20%), one family has three affected sibling. Post-operative mortality rate was 70%. No standard surgical approach can be adopted a better management of PA or the associated anomalies depends on an early diagnosis and the availability of neonatal intensive care unit.

Recurrent intussusception in children and infants.

BACKGROUND: Recurrent intussusceptions in child and infants are problematic and there are controversies about its management. The aim of this study is to determine the details of the clinical diagnosis of recurrent intussusception and to determine the aetiology of recurrent intussusceptions. PATIENTS AND METHODS: It's a retrospective study of 28 cases of recurrent intussusception treated in the paediatric surgery department of Monastir (Tunisia) between January 1998 and December 2011. RESULTS: During the study period, 505 patients were treated for 544 episodes of intussusception; there were 39 episodes of recurrent intussusceptions in 28 patients; the rate of patients with recurrence was 5.5%. With comparison to the initial episode, clinical features were similar to the recurrent episode, except bloody stool that was absent in the recurrent group (P = 0,016). Only one patient had a pathologic local point. CONCLUSION: In recurrent intussusception, patients are less symptomatic and consult quickly. Systematic surgical exploration is not needed as recurrent intussusceptions are easily reduced by air or hydrostatic enema and are not associated with a high rate of pathologic leading points.

Video-assisted surgery in the management of hydatid renal cyst in children.

Renal hydatid disease is rare in children. Open surgery is the traditional method of treatment, but minimally invasive techniques are being increasingly used. Herein, we report our experience with laparoscopic management of renal hydatid cyst in four children via a transperitoneal approach in three cases and a retroperitoneoscopy in one. We conclude that transperitoneal laparoscopy can be offered for the management of hydatid renal cyst associated with other intraperitoneal localizations, whereas the retroperitoneoscopy is limited for the treatment of isolated hydatid renal cysts.

Congenital pancreatic cyst with Ivemark II syndrome: a rare case.

An infant with congenital pancreatic cyst with Ivemark II syndrome is reported because it is a rare association. The infant had associated situs inversus, asplenia, and complex congenital heart disease. The pancreatic cyst was successfully managed by cystoduodenostomy because of connection to the biliary tract. The infant succumbed as a result of heart failure at age 2 months. Prognosis depends on the presence of life-threatening malformations.

Lipoblastoma: a rare lesion in the differential diagnosis of childhood mediastinal tumors.

Lipoblastoma is a rare, benign, fatty tissue tumor that occurs in childhood. The location of this tumor in the mediastinum and extension to the chest wall is uncommon. We describe a 12-month-old male infant with a mediastinal lipoblastoma discovered because of a chest wall swelling. Computed tomography showed the deep component and fatty content of the lesion suggestive of the diagnosis. Total excision of the mass was carried out. The histologic examination of the lesion confirmed the diagnosis of lipoblastoma. Mediastinal lipoblastoma should be considered among the possible diagnoses of a fatty mediastinal tumor in childhood.

Transverse testicular ectopia: a three-case report and review of the literature.

OBJECTIVES: To describe the embryologic, clinical, and therapeutic features of transverse testicular ectopia (TTE) and to review the literature on similar cases. METHODS: Three cases of TTE were discovered accidentally during surgery for inguinal hernia and undescended testes; a persistent Müllerian duct syndrome (PMDS) was associated in 1 case. We also performed a literature search for other reports of TTE. RESULTS: A conservative surgical approach was chosen: transseptal orchiopexy was preferred in 2 cases and the Müllerian remnant was preserved in the first case. CONCLUSIONS: TTE is a rare anomaly that usually presents as a unilateral cryptorchidism and a contralateral descendent testis with an associated hernia. In suspected cases, laparoscopy and ultrasonographic evaluation may be helpful in diagnosing this condition before surgery. Surgery, transseptal orchiopexy, is highly recommended to manage TTE especially when vasa deferentia are fused. In case of PMDS, the authors preferred preserving Müllerian remnants because of the risk of vas deferens and testicular blood supply damage. Recently, laparoscopy is useful for both diagnosing and management of TTE and associated anomalies.

Laparoscopic treatment of hydatid cyst of the liver in children. A report on 34 cases.

PURPOSE: This study evaluated the safety and efficiency of laparoscopically treated liver cysts in children. METHODS: From September 2001 to July 2004, 34 patients underwent laparoscopic treatment of hydatid cysts of the liver. All patients had chest x-ray, abdominal sonography, and hydatid serology. The different stages of the procedure were the same as in open surgery: puncture, aspiration, injection of scolicidal agent, reaspiration, removal of proligerous membrane, and resection of the dome. RESULTS: The patients' mean average age was 7 years and 7 months (range, 3-14 years). The number of cysts ranged from 1 to 10 with a diameter of 40 to 150 mm (mean diameter, 65.5 mm). One case had a mesenteric associated hydatid cyst, another splenic hydatid cyst. The average length of hospital stay was 5 days (range, 4-14 days). No per- or postoperative complications were reported. At 12 to 45 months follow-up, no recurrence has been reported. CONCLUSION: Laparoscopy represents an excellent approach for the treatment of hydatid cyst of the liver in children.