Torsion of a wandering spleen a case report

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. Report of a new observation and present the diagnostic, treatment and outcome aspects of torsion of ectopic spleen. 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. 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]

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. To study the principal clinic, radiologic and therapeutic of the congenital broncho-pulmonary malformations through ten cases. Retrospective study of 10 cases of congenital bronchopulmonary malformations diagnosed between 2003 and 2010 in our institution. The mean ages at the time of diagnosis is 2months [4 days to 16months]. The sex ratio is 1. The symptoms consisted of recurrent pneumonia in 4cases, respiratory distress in 2cases, bronchiolite in 2 cases and 2cases 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 ad‚nomatoid malformation and one bronchogenic cyst. Eight patients required surgical treatment involving pneumonectomy [1case], 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. 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

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. To report a new case of duodenum duplication revealed by acute pancreatitis. This 3 year old child presented with an acute pancreatitis. Abdominal ultrasonography and Computer tomography were performed showing a cystic mass depending of the 2[nd] 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. Duodenum duplications are uncommon congenital anomalies. Acute pancreatitis might be revealing presentation

[Congenital Hyperinsulinism: review of 12 Tunisian cases]

Congenital hyperinsulinism in infancy [CHI] is a heterogeneous disorder with respect to genetics and response to therapy. Data on CHI are sporadic in North African population. To characterize the clinical features and outcome of 12 Tunisian patients with CHI. data of patients diagnosed with CHI during the period 1989-2007 were retrospectively analyzed. Diagnosis was considered whenever hyperinsulinemia 10 UI/ml was concomitant to hypoglycemia<3mmol/l and/or high insulin to glucose ratio>0.3 and/or positif glucagon test. Transient causes of hypoglycemia, adrenal and growth hormone deficiency were excluded. There were nine infants diagnosed at a median age of 17 months and three newborns. Permanent hyperammoniemia, found in one patient, guided to leucine-sensitive hyperinsulinism. Seven patients presented with seizures, two with psychomotor delay and one with recurrent malaises. Among 42 assays of plasmatic insulin, when in hypoglycemia, 40% only were 10 U/ml. Three patients resisted to diazoxide and underwent subtotal pancreatectomy complicated by diabetes mellitus in two cases and persistent hypoglycemia in one patient. Histological examination concluded to diffuse hyperplasia of pancreatic cells. Diazoxide was discontinued in four out the eight responders patients. Four patients died, seven patients developed variable degrees of mental retardation and five suffered from epilepsy. Early onset forms were, as reported in the literature, mostly resistant to medical therapy. The high proportion of neurological sequelae is related to diagnosis delay or to a late surgery. We focus on the importance of a precocious diagnosis and aggressive treatment of hypoglycemia

[Posterior uretral valves, about a series of 44 cases]

To explore the physical symptoms and radiological findings for the diagnosis of posterior urethral valves [PUV], to point of the therapeutic snags depending on gravity and delay of lesions. We reviewed the records of 44 patients with PUV. Mean age: 2 years 1/2 [range 1 day-13 years]. More than 75% of patients were less than 2 years old. In utero diagnosis was made in 8 patients [14%]. After birth, the diagnosis was based on urological signs [as frequent as diagnosis is made belatedly] and extra urological signs. Cystourethrography [CUG] showed posterior urethral dilation in all cases. Ultrasonography [US] showed abnormalities in 30 cases and intravenous urography [IVU], made in 21 cases, was normal in appearance in 7 of them. At diagnosis, it was a renal failure in 36 patients and 8 of them had a terminal renal deficiency. Urethroscopy made in 40 patients, showed PUV in all cases. There were 29 types I valves, 3 type II valves, 5 type III and 3 unclassifiables cases. Treatment was endoscopic in 41 cases and by lamination in the 3 others. Mean follow up is 9 years [range16months-19 years] and 19 patients have terminal renal failure from which 3 are deceased. PUV are dangerous obstructive uropathies in boy whom end at terminal renal failure in more than 33% of cases. Precocious diagnosis and early ablation of PUV are able to limit the complications inherent to this pathology

[Sternal clefit, a rare congenital anomaly. Report of the first Tunisian case]

Sternal cleft is an uncommon visually dramatic congenital anomaly of the chest wall. It is resulting of failure of the two lateral mesodermal sternal bars fusion by the eight weeks of gestation. Superior defects are the commonest forms, usually isolated. Clinical and surgical aspects of stemal cleft are presented. The advantages of early surgery in the neonatal period are developed. We report the first Tunisian case of a superior sternal cleft associated to haemangioma in a newborn boy. Scanning shows non-appearance of manubrium at the upper part of sternum. Sternal bars showing a U-shaped incomplete sternal cleft. Surgical repair consisted of reconstructing a new sternum from sternal bars and resection of haemangioma. The patient had good aesthetic and functional results. The appearance of a child with its heart bulging through its chest wall is very disturbing to parents. Early surgery is most easy and most comforting