Kais Maazoun

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.

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

Spontaneous or idiopathic perforation of the extra hepatic bile duct in neonates and infants is a rare disease but is an important cause of jaundice, requiring early corrective surgery. The exact cause remains unclear and the diagnosis is not always easy. We reviewed the records of two cases of spontaneous perforation of the bile duct (SPBD) treated at the Department of Paediatric Surgery at Monastir Medical School Hospital, Tunisia, from 1994 to 2002.

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.

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.

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.

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.

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.

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.

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.

La Polyorchidie : A propos de 2 cas

Resume La polyorchidie est une anomalie uro-genitale rare. Nous rapportons deux observations de deux enfants âges respectivement de 3 et 5 ans, ayant une ectopie testiculaire gauche. L’exploration chirurgicale a mis en evidence deux testicules a gauche avec un troisieme testicule droit en place. Un abaissement des deux testicules dans l’hemi-scrotum gauche a ete realise. A travers ces deux observations, les auteurs insistent sur les particularites therapeutiques de la polyorchidie.