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Complete tubular colonic duplication (CTCD) is exceedingly rare. The association of CTCD with an anorectal malformation is unusual. This malformation may be found unexpectedly at laparotomy. We present 3 cases of surgically proven neonate CTCD discovered at laparotomy for anal atresia. We reviewed the mode of clinical presentation, the imaging, and laparotomy findings. Our series illustrates that this rare disease presents perinatally in association with anal atresia, with or without other associated anomalies. more...

Resume Introduction et objectifs Etudier les aspects diagnostiques et evolutifs des mega-ureteres primitifs de decouverte antenatale et analyser les resultats de la prise en charge medicochirurgicale postnatale. Patients et methodes Nous avons fait une analyse retrospective de 12 patients nes entre 1998 et 2009 avec un mega-uretere primitif decouvert en antenatal entre 20 et 32 semaines d’amenorrhee (SA). Un bilan radiologique et isotopique postnatal de l’appareil urinaire a ete realise a tous les patients qui ont ete mis sous traitement antiseptique urinaire et suivis depuis la naissance. Resultats Il s’agissait de neuf garcons et trois filles, tous nes a terme avec une trophicite normale. L’âge moyen de diagnostic antenatal de l’hydronephrose etait de 26 SA. L’âge moyen de diagnostic postnatal etait de dix mois. Le mega-uretere etait unilateral gauche dans cinq cas, droit dans deux cas et bilateral dans cinq cas, soit 17 unites renales atteintes dont huit mega-ureteres non obstructifs non refluants, trois refluants, cinq obstructifs et un obstructif et refluant. Le mega-uretere etait associe a un syndrome de la jonction pyelo-ureterale obstructif du cote controlateral dans un cas et a une duplicite ureterale avec un renicule polaire superieur du meme cote dans un autre cas. La fonction renale etait inferieure a 20 % dans quatre unites renales. La duree moyenne de suivi des patients etait de quatre ans avec des extremes de dix mois et 11 ans. L’evolution etait marquee par des infections urinaires dans sept cas dont quatre avaient un mega-uretere non obstructif non refluant. Le recours a la chirurgie etait justifie chez six patients dont deux pour mega-uretere non obstructif non refluant complique d’infections urinaires recidivantes, trois pour mega-uretere obstructif avec alteration severe de la fonction renale et un pour mega-uretere refluant bilateral grade V. L’âge moyen du traitement chirurgical etait de 22,6 mois avec des extremes de six mois et sept ans. Conclusion La moitie de nos patients ayant un mega-uretere primitif de decouverte antenatale a necessite un traitement chirurgical. Les infections urinaires recidivantes, l’alteration de la fonction renale inferieure a 20 % et l’importance de la dilatation de l’uretere avec reflux vesico-ureteral de haut grade etaient les facteurs predictifs pour la chirurgie. more...

Background: Although the association of urinary lithiasis and urinary tract malformation is not rare, their management poses challenges. The aim of this study was to evaluate the relationship between urolithiasis and malformations of the urinary system. There were 34 patients (19 males and 15 females) with a mean age of 4.8 years (range, 2 months to 14 years). All patients had urinary lithiasis with a urinary tract malformation. Abdominal pain was the most frequent clinical symptom (38%). Urinary infection was found in 7 patients (21%) and macroscopic haematuria was present in 10 patients (29%). The most frequent urinary tract malformations were megaureter (8 cases), uretero-pelvic junction obstruction (7 cases) and vesico-ureteric refl ux (8 cases), but its malformative origin could not be confi rmed. Treatment consisted of lithiasis extraction in 32 cases associated with specific treatment of the uropathy in 27 cases. Postoperative outcome was uneventful in all cases. In fact, urinary lithiasis and urinary tract malformation association is not rare. Indeed, 9-34% of urinary lithiasis are noted to be associated with urinary tract malformation. Positive diagnosis relies specifi cally on kidney ultrasound, intravenous urography, and urethrocystography. Treatment depends on the type of urinary tract malformation, localisation and size of the urinary lithiasis. Conclusion: In conclusion, urinary lithiasis and urinary tract malformation association is a frequent eventuality. Surgical intervention is the usual mode of treatment.Key words: Children, treatment, urinary lithiasis, urinary tract infection, urinary tract malformation more...

Resume L’uretre surnumeraire, dit aussi duplication uretrale est une malformation rare interessant essentiellement le garcon. Elle se definit par la juxtaposition de deux ou plusieurs canaux a structure musculaire avec un revetement muqueux de type excreto-urinaire. Divers aspects peuvent se voir en fonction du siege de l’uretre en exces et de son caractere complet ou incomplet. Dans ce present travail, nous nous sommes interesses a un aspect particulier de cette malformation, qui est la forme epispade. more...

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 Mullerian 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 Mullerian 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 Mullerian 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. more...

Polysplenia syndrome is a rare malformation characterized by the association of multiple rates and other congenital anomalies dominated by cardiac, vascular, intestinal and bile malformations. We report the observation of a patient operated in the neonatal period (3 days) for an upper intestinal obstruction with situs inversus. Surgical exploration noted the presence of multiple rates, a preduodenal vein, a biliary atresia and a duodenal atresia. The surgical procedures performed were a latero-lateral duodeno-duodenostomy and hepatoportoenterostomy of KASAI with simple immediate and delayed outcomes. The follow up was of 23 years. We recall the epidemiological characteristics of this malformative association and we discuss the role played by the prognosis of polysplenia syndrome in the evolution of biliary atresia. The diagnosis and treatment of biliary atresia are always urgent to increase the chances of success of the Kasai, and the chances of prolonged survival with native liver. However, almost all long-term survivors (even anicteric) have biliary cirrhosis, which requires lifelong follow up. more...

Objectif Etudier les aspects epidemiologiques, anatomopathologiques, cliniques et evolutifs des teratomes sacrococcygiens (TSC) chez l’enfant dans le sud et le centre tunisiens.

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. more...

But: La perforation spontanee de la voie biliaire extra-hepatique (SPBD) est rare chez l'enfant. La pathogenie, les modalites diagnostiques et les options therapeutiques sont revues et discutees. Methode: Les auteurs reportent deux nouveaux cas de SPBD chez deux nouveau-nes garcons, âges respectivement de 27 et 21 jours. Resultats: Les deux nouveau-nes se presentaient avec une jaunisse cholestatique. L'ultra-sonographie abdominale montrait une masse extra-hepatique. La laparotomie exploratrice montrait que la perforation etait localisee sur le canal cystique dans le premier cas et sur le choledoque (CBD) pour le second. Le niveau de la perforation etait repere et les deux patients subissaient un simple drainage biliaire externe. L'evolution post operatoire etait simple pour les deux patients avec un suivi de 2 a 4 ans. Conclusion: SPBD est rare mais est une cause importante de jaunisse chirurgicale chez l'enfant. La pathogenie de SPBD est inconnue et multifactorielle et le diagnostic souvent problematique. Le traitement chirurgical est toujours necessaire et une approche conservative est normalement recommandee. Le pronostic est bon apres un traitement chirurgical precoce. more...

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. Identifi ed 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.Key words: Epidermolysis bullosa, pyloric atresia, surgery more...

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. more...

Background: Wound infection is the most frequent complication in burn patients. There is a lack of guidelines on the use of systemic antibiotics in children to prevent this complication. Patients and Methods: A prospective study is carried out on 80 patients to evaluate the role of antibiotic prophylaxis in the control of infections. Results: The mean age was 34 months (9 months to 8 years). There was a male predominance with sex ratio of 1.66. The mean burn surface size burn was 26.5% with total burn surface area ranging from 5% to 33%, respectively. According to American Burn Association 37% (30/80) were severe burns with second and third degree burns >10% of the total surface body area in children aged more...

Congenital bilobar emphysema is reported to be extremely rare. We describe 3 cases, and we review the diagnosis problems and treatment methods of this childhood respiratory tract pathologic condition. more...

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. more...

Gastric perforation in neonates is an uncommon condition. It could be spontaneous but a contributing cause should be sought.To review our experience of treating 8 neonates with gastric perforation over the past 20 years.We reviewed the records of all newborns admitted to our hospital between 1990 and 2010 with regard to gender, age at admission, contributing factors, associated anomalies, site of perforation, type of operation, and outcome.Of the eight neonates, five were female and three male. The average weight was 2130 g. Four babies were premature. Three infants were ventilated for respiratory difficulty. Five patients had associated anomalies. Perforation occurred in the lesser curvature in 4, at the greater curvature in 3, and at the anterior antrum surface in 1. All patients were treated with gastrorrhaphy. Four neonates required additional gastrostomy. Mortality was 75% (6 infants).Active perinatal management, early treatment of primary pathologies, and protection of the stomach against distension in neonates at risk are essential in the management of neonatal gastric perforation. more...

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. more...

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. 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. 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. 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. more...

Background/Purpose Multiple echinococcosis (ME) is a severe disease in childhood inaccessible to an initial radical surgical treatment. The aim of this study was to evaluate the efficacy of Albendazole in ME and to discuss the role of surgery in this pathology. Methods Eleven patients were included in a prospective study between 1996 and 2004. ME was defined by the presence of 10 or more cysts in the same organ. Albendazole was given as 10 mg/kg daily continuously. Treatment outcome was defined as cure, improvement, stabilization or deterioration. Surgery was discussed after 1 year of treatment. Results Our patients totalized 296 cysts located essentially on the liver (178 cysts) and the lungs (78 cysts). With exclusive Albendazole therapy, 57.7% pulmonary cysts and 96% peritoneal cysts were considered as cured. This rate was only 31.5% in hepatic localization. After surgical therapy, 67.4% of hepatic cysts were cured. No productive biliary fistula was observed. Two patients were operated laparoscpically. The total treatment duration ranged between 1 and 5 years. Parasitologic examination of operated cysts showed that 30% of them were viable even after 3 years of treatment. Conclusion Albendazole has proven a strong efficacy in pulmonary and peritoneal localizations. A complementary surgical treatment is often necessary in hepatic localizations and it is facilitated with previous Albenazole therapy. Combination of ABZ and surgery seems to have encouraging results and must be applied for those patients. The high rate of viable cysts after medical therapy is problematic and must incite to develop new antihelminthic agents. more...

Purpose The aim of this work is to discuss the pathogenesis of the segmental dilatation of the intestine (SDI) and to review its clinical presentation and the ways to confirm the diagnosis. Methods Eight cases of pathologically proven SDI from 1987 to 2003 were reviewed and discussed. There were 7 newborns and a 1-year-old boy. Results Our patients are 5 boys and 3 girls. In all cases, the diagnosis was not suspected before surgery. Two patients presented with a low neonatal bowel obstruction. Six patients were operated for omphalocele, which was the most frequent associated malformation. The SDI involved the ileum in all patients. The treatment consisted on a resection of the dilated segment with an end-to-end anastomosis. Histological examination demonstrated the presence of ganglion cells in all cases. The muscular layer was hypertrophied in two cases and very thin in one case. A heterotopic gastric mucosa was observed in one case. No anomalies were observed in 5 cases. The postoperative course was uneventful in 6 cases with a mean follow-up of 5 years. Conclusions Segmental intestinal dilatation is an exceptional pathology with an unknown etiology and a misleading clinical presentation. Several theories were proposed to explain this malformation; however, most authors are rather inclined to an embryological theory incriminating an extrinsic intrauterine intestinal compression. Most cases are neonatal discoveries. The clinical polymorphism and the lack of specificity of radiological investigations explain the difficulties to have a preoperative diagnosis. However, this difficulty is compensated by the favorable evolution after the resection of the dilated segment. more...

Xanthogranulomatous pyelonephritis (XGP) is an unusual and rare form of chronic renal suppuration in children, which appear to have a multifactorial pathogenesis. It is usually diffuse, but can remain localized to one part of the kidney, resulting in a misleading pseudoneoplastic appearance raising a problem of differential diagnosis with the other renal masses, particularly Wilms tumour. The authors present a retrospective analysis of three cases of XGP observed in the Monastir department of paediatric surgery. The preoperative diagnosis was pyonephrosis with perirenal abscess in two cases and Wilms tumour in the third case. The diagnosis of XGP was established on histological examination of the operative specimens. The aim of this study is to emphasize the diagnostic difficulties of this disease that, in localized forms, often lead to inappropriate treatment and to establish a treatment regimen for this rare disease. more...

Desmoplastic small round cell tumor (DSRCT) correspond to a recent clinicopathological entity, individualized in 1989 into the group of tumors with small round cells. This pathology puts ethiopathogenic, diagnostic, therapeutic and prognosis problems. Indeed, the ethiopathogenic is still unknown, diagnosis is asserted only by immuno-histochimic and cytogenetic study because of the big number of differential diagnoses and the anatomopathologic polymorphism. Its treatment is not well codified and its outcome remains dark in spite of therapeutic progress. The objective of this work is to report a personal observation of a DSRCT and to proceed to a review of the literature to clarify the epidemiological, clinical, paraclinical and therapeutic aspects of this rare tumor. more...

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. more...