Torsion of a wandering spleen: a case report.

BACKGROUND: Wandering spleen is a rare entity in child. It is generated by laxity or failure of development of spleen ligaments causing the migration of the spleen from its normal anatomical site to another abdominal or pelvic location. It can be congenital or acquired. The most dangerous complication is the occurrence of torsion of the spleen around its pedicle. aim: Report of a new observation and present the diagnostic, treatment and outcome aspects of torsion of ectopic spleen. CASE: We report the case of a 17 years old girl who was admitted for an acute abdomen pain and fever. Clinical examination revealed generalized abdominal defence and an under umbilical mass which was very painful on palpation. Ultrasound and CT scans have visualized the pelvic mass, which measured four inches long axis. The spleen wasn't on its normal seat. The patient was operated urgently. Surgical exploration showed that the mass corresponded to a wandering spleen in pelvic position, necrotic and twisted around its pedicle. A splenectomy was performed with simple sequences. CONCLUSION: torsion of the wandering spleen can progress to total necrosis of the spleen mass. This complication is feared in any ectopic and painful spleen.

[Congenital pulmonary malformations: clinical, radiological and treatment features]. / Les malformations broncho-pulmonaires: aspects diagnostiques radiologiques et thérapeutiques.

BACKGROUND: Congenital broncho-pulmonary malformations (CBM) are rare, essentially presented by congenital lobar emphysema, bronchogenic cysts, pulmonary sequestrations and cystic adenomatoid malformations. The diagnosis can be in prenatal. In postnatal, symptoms are variable. Radiological investigations lead to diagnosis in all cases. AIM: To study the principal clinic, radiologic and therapeutic of the congenital broncho-pulmonary malformations through ten cases. METHODS: Retrospective study of 10 cases of congenital bronchopulmonary malformations diagnosed between 2003 and 2010 in our institution. RESULTS: The mean ages at the time of diagnosis is 2 months (4 days to 16 months). The sex ratio is 1. The symptoms consisted of recurrent pneumonia in 4 cases, respiratory distress in 2cases, bronchiolite in 2 cases and 2 cases of antenatal diagnosis. All patients have a chest X-ray, night patients have a chest computerized tomography and one patient has a bronchial endoscopy. Ten cases of BPM have been investigated: five congenitals lobar emphysema, tow pulmonary sequestrations, tow cystic adénomatoid malformation and one bronchogenic cyst. Eight patients required surgical treatment involving pneumonectomy (1 case), lobectomy (5 cases),segmentectomy (1 case) and in 1 case the pulmonary sequestration was treated by ligature of the anomalous artery with pulmonary resection. The histopathological examination confirmed the diagnosis in all cases. The postoperative period was uneventful in 8 cases with a mean of follow-up of 2 years (5 months to 5years). Tow patient died after surgical treatment. CONCLUSION: The diagnosis of BPM malformations can be clinical, confirmed by radiological investigations. The improvement in prenatal ultrasound diagnosis modified the management strategy. The treatment varies frome attitude conservatrice to pneumonectomy.

[Duodenal duplication revealed by acute pancreatitis. A case report]. / Duplication duodenale revelee par une Pancreatite aigue. A propos d'un cas.

BACKGROUND: Duodenum duplications are uncommon congenital anomalies. Most symptomatic cases are diagnosed in childhood and usually present with obstructive or bleeding symptoms. Acute pancreatitis is rarely attributed to these cysts. AIM: To report a new case of duodenum duplication revealed by acute pancreatitis. CASE REPORT: This 3 year old child presented with an acute pancreatitis. Abdominal ultrasonography and Computer tomography were performed showing a cystic mass depending of the 2nd duodenum. Diagnosis of duodenal duplication is made in laparotomy. A surgical resection of the duplication was performed respecting the papilla. Microscopic examination of the specimen confirmed the duodenal duplication. The patient was asymptomatic after the intervention. CONCLUSION: Duodenum duplications are uncommon congenital anomalies. Acute pancreatitis might be revealing presentation.

Beckwith-Wiedemann syndrome: Clinical, histopathological and molecular study of two Tunisian patients and review of literature.

BACKGROUND: Beckwith-Wiedemann syndrome (BWS) is a rare overgrowth syndrome characterized by congenital malformations and predisposition to embryonic tumors. Loss of methylation of imprinting center 2 (IC2) is the most frequent alteration and rarely associated with tumors compared to paternal uniparental disomy of chromosome 11 (UPD(11)pat) and gain of methylation of imprinting center 1. METHODS: Our study aimed to describe the clinical, histopathological and genetic characteristics of two patients and establish genotype-phenotype correlations. The clinical diagnosis was based on the criteria defined by the international expert consensus of BWS. Molecular study of 11p15.5 methylation status was assessed using methylation-specific-multiplex ligation probe amplification (MS-MLPA). RESULTS: Patients were aged 12 months and 3 months and fulfilled the clinical score of BWS. MS-MLPA showed molecular alterations consisting of loss of methylation in IC2 (IC2-LOM) at the maternal allele for one patient and a mosaic UPD(11)pat for the second patient in whom follow-up at 6months revealed adrenocortical carcinoma (ACC) with low grade of malignancy. Molecular subtypes guide the follow-up and tumor surveillance, our major concern. CONCLUSION: We have to take into account the psychological impact of a possible tumor whatever the underlying mechanism is. Nevertheless, the tumor risk remains high for UPD(11)pat. Our study extended the phenotype of BWS with absence of macrosomia in Tunisian patients, contrasting with literature, and added a supplementary case of ACC in the tumor spectrum of BWS patients with UPD(11)pat.

Alimentary tract duplications in children: report of 16 years' experience.

BACKGROUND: Alimentary tract duplications (ATDs) are a rare condition in children, characterised by a large pathogenic, clinical, and histological polymorphism. Surgical observation and pathologic evaluation of the resected specimens are the only way to confirm the diagnosis. In this study, we want to analyse the anatomical, clinical and therapeutic aspects of this entity. PATIENTS AND METHODS: A total of 12 cases of ATD were diagnosed over a 16-year period at paediatric surgery department. The diagnosis was evoked on clinical and radiological data. Histological study of the resected specimens confirmed the diagnosis in all cases. RESULTS: The mean age of patients at diagnosis was 41 months with a peak of incidence at the 1 st year of life (42%). Out of a total 12 cases, 10 were girls and 2 were boys. Abdominal pain and vomiting were the most frequent presenting features. Ultrasonography, tomodensitometry and magnetic resonance imaging were useful for diagnosis. ATDs were localised on the oesophagus in one case, the stomach in one case, the duodenum in four cases, the ileum in five cases, and the colon in one case. All these duplications were cystic, with three communicating duplications. All patients underwent surgery, and resection procedure was chosen according to duplication type and site. Histological study confirmed the diagnosis in all cases. CONCLUSION: ATDs are a rare condition in children. Diagnosis relies on histology, and treatment can only be by means of surgery. The outcome after surgery is generally favourable. Diagnosis and precocious surgery of ATDs can warn serious complications.

Peptic oesophageal stricture in children: management problems.

BACKGROUND: Peptic oesophageal stricture (PES) is a serious complication of gastroesophageal reflux disease (GERD) in childhood. The treatment of PES is still controversial, ranging from simple oesophageal dilations to resection/anastomosis of the stenotic portion of the oesophagus. In this study, we want to share our experience with 11 children with GERD and PES. PATIENTS AND METHODS: A retrospective review of clinical data obtained from children who underwent dilation and antireflux surgery for PES was performed. RESULTS: A total of 11 patients were diagnosed with PES. The clinical picture was dominated by dysphagia. Barium swallow showed hiatal hernia in nine cases (82%). Oesophageal strictures were located most commonly in the lower third of the oesophagus (91%). Three Children (27%) with PES had a neurologic impairment and patients had a mean duration of symptoms of 20 months (range, 3 month to 6.2 years) before intervention. Children received a median of four dilations (range, 1-21 dilations) for PES. Time to first dilation from age of diagnosis was a mean of 4.5 months (range, 2-14 months). Antireflux surgery was performed in all patients. Post-operatively, seven patients required repeat oesophageal dilation. Patients were followed with serial dilation for a median of 6 years (range, 1-9 years) and only one patient has a continued requirement of oesophageal dilation for PES. CONCLUSION: GERD complicated by PES is an important condition affecting a significant number of children. Early and effective treatment of both stricture and GERD is required to improve the prognosis of this serious condition.