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Transmesenteric hernias are extremely rare. A strangulated hernia through a mesenteric opening is a rare operative finding. Preoperative diagnosis still is difficult in spite of the imaging techniques currently available. The authors describe two cases of paediatric patients presenting with bowel obstruction resulting from a congenital mesenteric hernia. The first patient had a 3-cm wide congenital defect in the ileal mesentery through which the sigmoid colon had herniated. The second patient is a newborn infant who presented with symptoms and radiographic evidence of neonatal occlusion. At surgical exploration, a long segment of the small bowel had herniated in a defect in the ileal mesentery. A brief review of epidemiology and anatomy of transmesenteric hernias is included, along with a discussion of the difficulties in diagnosis and treatment of this condition.

Background: The purpose of the study is to present the author's experience with congenital bladder diverticula in seven pediatric patients at a developing world tertiary care center., Materials and Methods: Records of seven patients diagnosed and treated as congenital bladder diverticulum, from January 1998 to December 2009 were retrospectively reviewed for age, sex, clinical symptoms, investigative work-up, operative notes, and postoperative follow-up., Results: All patients were males. Age at presentation ranged from six months to six years (mean three years and six months). All were manifested postnatally by urinary tract infection in four cases, bladder retention in three cases and abdominal pain in two cases. Diagnosis was suggested by ultrasound and confirmed by voiding cystourethrography (VCUG) in all cases and urethrocystoscopy in three cases. Open surgical excision of diverticulum was done in all the patients associated with ureteral reimplantation in four patients with VCUG-documented high-grade vesicoureteral reflux (VUR). Average follow-up was four years; there is a resolution of symptoms and no diverticulum recurrence at the defined mean follow-up., Conclusion: Recurrent urinary tract infections and voiding dysfunction in pediatric population should always be evaluated for congenital bladder diverticulum. Investigations such as abdominal ultrasound, VCUG and nuclear renal scanning, form an important part of preoperative diagnostic work-up and postoperative follow up. Diverticulectomy with ureteral reimplantation in case of high-grade reflux, provides good results without recurrence.

Background: To analyze the spectrum of testicular tumors in prepubertal children and the therapeutic resultants in an unselected population., Materials and Methods: Our hospital database was analyzed for testicular tumors from January 1995 to December 2010 concerning clinical presentation, treatment and therapeutic results., Results: Eight patients were operated on because of testicular tumors. In six cases (75%) the tumor was benign: benign teratoma (four cases), epidermoid cyst (one case) and immature teratoma (one case). Two patients (25%) had a malignant tumour: yolk-sac tumour (two cases). All this children underwent surgery. Radical inguinal orchidectomy was performed in six cases and conservative surgery was performed in two cases. One patient has received adjuvant chemotherapy. Follow-up was uneventfully three years after primary surgery., Conclusion: In prepubertal children, most testicular tumours are benign. If tumour markers were negative testis-preserving surgery can be proposed, complete excision of the tumour should be ascertained. In the case of testicular teratoma, the possibility of contralateral tumour should be considered in the follow-up.

Objective: Our purpose was to review our experience with congenital diaphragmatic hernia emphasizing diagnosis, management, and outcome., Study Design: We conducted a retrospective review of all cases of babies with congenital diaphragmatic hernia diagnosed and treated in our centre from 1998 to 2010., Results: There were 28 congenital diaphragmatic hernia cases, 13 girls and 15 boys with a mean weight birth of 3 kg. Three patients (10, 6% of cases) died within a few hours after admission. In the remaining cases, surgery was performed after a stabilization period of 2 days. The diaphragmatic defect was sitting in the posterolateral left in 23 cases and right in 2 cases. Its dimensions were on average 4,5 cm, tow cases of agenesis of the cupola were seen and required the placement of gortex prosthesis. The remaining cases are treated by direct closure of defect. Postoperative course was marked by an early death in context of respiratory distress in six cases and later with sepsis in tow cases. The outcome was favourable in 17 cases (60, 7%), despite the occurrence of sepsis in four cases and evisceration in two cases., Conclusions: Congenital diaphragmatic hernia remains a serious disease with high mortality and morbidity despite advances in prenatal diagnosis and neonatal resuscitation.

Situs inversus is a challenge to the physician, both for the diagnostic and for the therapeutic. A combination of partial situs inversus, annular pancreas and polysplenia with bowel malrotation has been reported in a newborn who presented as duodenal obstruction. Situs inversus is rare especially without heart malformation. All the abnormalities in this combination can be explained on the basis of multiple organ malrotation. It also demonstrates the necessity of meticulous investigation and precise description of the anomaly as far as the management of this case is concerned.

Hydatid cysts are endemic in certain regions of the world and particulary in North Africa. They are usually located in the liver, lung, and spleen, though many uncommon locations have been reported. This is the first report of a child with primary pelvic hydatid disease causing a sciatic compression.

Purpose: Appendicitis is the most common abdominal inflammatory process in children which were sometimes followed by complications including intra-abdominal abscess. This later needs classically a surgical drainage. We evaluated the efficacy of antibiotic treatment and surgical drainage., Methods: Hospital records of children treated in our unit for intra-abdominal post appendectomy abscesses over a 6 years period were reviewed retrospectively., Results: This study investigates a series of 14 children from 2 to 13 years of age with one or many abscesses after appendectomy, treated between 2002 and 2007. Seven underwent surgery and the others were treated with triple antibiotherapy. The two groups were comparable. For the 7 patients who receive medical treatment alone, it was considered efficient in 6 cases (85%) with clinical, biological and radiological recovery of the abscess. There was one failure (14%). The duration of hospitalization from the day of diagnosis of intra-abdominal abscess was approximately 10.28 days (range 7 to 14 days). In the other group, the efficacy of treatment was considered satisfactory in all cases. The duration of hospitalization was about 13 days (range: 9 to 20)., Conclusion: Compared to surgical drainage, antibiotic management of intra-abdominal abscesses was a no invasive treatment with shorter hospitalization.

Triple A syndrome (Allgrove syndrome, MIM No. 231550) is a rare autosomal recessive disorder characterised by ACTH-resistant adrenal insufficiency, achalasia of the cardia, and alacrimia. The triple A gene has been previously mapped to chromosome 12q13 in a maximum interval of 6 cM between loci D12S1629 and D12S312. Using linkage analysis in 12 triple A families, mostly originating from North Africa, we confirm that the disease locus maps to the 12q13 region (Zmax = 10.89 at theta = 0 for D12S1604) and suggest that triple A is a genetically homogeneous disorder. Recombination events as well as homozygosity for polymorphic markers enabled us to reduce the genetic interval to a 3.9 cM region. Moreover, total linkage disequilibrium was found at the D12S1604 locus between a rare allele and the mutant chromosomes in North African patients. Analysis of markers at five contiguous loci showed that most of the triple A chromosomes are derived from a single founder chromosome. As all markers are located in a 0 cM genetic interval and only allele 5 at the D12S1604 locus was conserved in mutant chromosomes, we speculate that the triple A mutation is due to an ancient Arabian founder effect that occurred before migration to North Africa. Since we also found linkage disequilibrium at D12S1604 in two patients from Southern Europe (France and Spain), the founder effect might well extend to other Mediterranean countries. Taking advantage of a YAC contig encompassing the triple A minimal physical region, the triple A gene was mapped to a 1.7 Mb DNA fragment accessible to gene cloning.