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.

Ureterocele containing a stone in a duplex system.

The formation of calcified stone in the ureterocele, a frequent event in adults, is very uncommon in a pediatric age. We present a case of a ureterocele containing a calcified stone in an 8-year-old girl. The diagnosis was made with radiological investigations (intravenous pyelogram, ultrasonography and, eventually, voiding cystourethrogram). Meatostomy and calculus extraction, or open surgery in more complicated cases, are the treatments of choice.

[Gastric heterotopia: clinical and histological study of 12 cases]. / Hétérotopie gastrique: étude anatomo-clinique de 12 cas

BACKGROUND: Gastric heterotopia is a rare congenital lesion, described everywhere in the body, but involves predominantly the digestive tract. Diagnosis is based on histologic examination and requires the presence of gastric mucosa, especially fundic. This diagnosis is usually easy, but sometimes it can be misinterpreted as gastric metapalsia . This latter is an acquired and frequent lesion of the gastrointestinal tract. AIMS: To determine the relationship between this affection and the other digestive malformations, to describe the clinical characteristics and the evolution of this lesion and to discuss the differential diagnosis particularly the gastric metaplasia. METHODS: Twelve cases of gastric heterotopia were diagnosed over a 12-year period at Habib Thameur Hospital. Clinical data was obtained and all the slides were reviewed. RESULTS: Nine cases were found in Meckel's diverticulum (75%), one case in intestinal duplication, one case in the esophagus and another in the gallbladder. The mean age of patients at diagnosis was 16 years with a peak of incidence at the first decade of life. Intestinal obstruction and digestive bleeding were the most presenting features. Heterotopic gastric mucosa complicates other congenital anomalies such as common mesentery, vestigial polyp of the liver, appendiceal agenesis and heterotopic pancreas. Differential diagnosis consists in gastric metaplasia was found extensively in a patient with Crohn's disease associated with Meckel's diverticulum. CONCLUSION: Gastric heterotopia is frequently associated with congenital anomalies especially with Meckel's diverticulum and digestive duplication. Diagnosis relies on histology, mainly on finding heterotopic fundic glands in normal organizational structure. Sometimes, differentiating between gastric heterotopia and gastric metaplasia requires clinical confrontation.

Congenital hyperinsulinism: review of 12 Tunisian cases.

BACKGROUND: 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. AIM: To characterize the clinical features and outcome of 12 Tunisian patients with CHI. METHODS: data of patients diagnosed with CHI during the period 1989-2007 were retrospectively analyzed. Diagnosis was considered whenever hyperinsulinemia ≥ 10µ 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. RESULTS: 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 ≥ 10µU/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. CONCLUSION: 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.

[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.

Sternal cleft, a rare congenital anomaly. Report of the first Tunisian case.

BACKGROUND: 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. AIM: Clinical and surgical aspects of sternal cleft are presented. The advantages of early surgery in the neonatal period are developed. CASE REPORT: 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. CONCLUSION: 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.

[Angiomatoid fibrous histiocytoma: rare tumor of intermediate malignancy]. / Histiocytome fibreux angiomatoide: une tumeur rare a malignite intermediaire.

Angiomatoid fibrous histiocytoma is a rare tumour affecting young adults. Unlike conventional malignant fibrous histiocytoma, its extension is only local thus giving a good prognosis. We report the cases of a 9 year-old girl and a 16 year-old boy presenting respectively, with an axillary tumour 5 cm of diameter and a paravertebral subcutaneous tumour 1.5 cm of diameter. In both cases, the diagnosis was not initially suspected. The treatment consisted in surgical resection.