Intestinal duplication revealed by posterior reversible encephalopathy syndrome / 소아과

We report a unique case of intestinal duplication detected on posterior reversible encephalopathy syndrome (PRES) in a 13-year-old girl. She was admitted to the pediatric Emergency Department because of generalized seizures. Radiological assessment revealed a large, well-defined, thick-walled cystic lesion in the mid abdomen, suggestive of duplication cyst associated to a PRES. Exploration confirmed the diagnosis of ileal duplication cyst, and the mass was resected. The postoperative course was uneventful. Both hypertension and neurological dysfunction resolved after the mass resection. A followup brain magnetic resonance imaging was performed 9 months later and showed complete resolution of the cerebellar changes. Although extrinsic compression of the retroperitoneal structures has not been reported in the literature as a complication of duplication cyst, we strongly believe that this is the most logical and plausible hypothesis that would explain the pathogenesis of PRES in our patient.

Unusual malignant neoplasms of ovary in children: two cases report / 소아과

Sex cord tumors with annular tubules are known to originate from the sex cord of embryonic gonads that synthesize Sertoli cells, Leydig cells, granulosa cells, and theca cells of the ovarian stroma, while ovarian small cell carcinoma of the hypercalcemic type is a type of neuroendocrine tumor. Both these tumors are uncommon, potentially malignant neoplasms in children. We report the case of a sex cord tumor with annular tubules in an 11-year-old girl and a case of small cell carcinoma of the hypercalcemic type in a 10-year-old girl. We also discuss the prognosis and management of these tumors.

Presternal giant epidermal cyst: about 2 cases

Epidermal cysts are a common benign lesion, occurring often above the shoulder and within the face and the scalp. Presternal site and huge volume are quite rare, especially in children. The authors report on two paediatric cases, discuss etiopathogenesis and diagnostic difficulties. A 2,5 and 3-year-old boys were admitted for voluminous subcutaneous tumour of the chest. The lesions had appeared since neonatal period with rapid growth after a minimal trauma for the last year. The physical examination found a great presternal cystic mass of 70 mm in diameter which is painless round and mobile. Ultrasonography and magnetic resonance imaging, showed a subcutaneous cystic mass with no mediastinal involvement or other localization in both. Complete surgical excision including the overlying skin with direct cutaneous closure was performed in both cases. The histological examinations confirmed the diagnosis of epidermal cyst. Postoperative courses were uneventful with no recurrence during respectively 18 months and 5 years follow-up periods. Giant presternal epidermal cyst is uncommon and can raise diagnostic problems. Imaging investigations facilitate accurate diagnosis. Because of malignant degeneration, surgical excision should be mandatory

Xanthogranulomatous pyelonephritis in childhood: diagnosis difficulties and success of conservative treatment

Xanthogranulomatous pyelonephritis is an uncommon form of chronic pyelonephritis rarely seen in children. Preoperative diagnosis is difficult and management is still obscure. The authors report on a focal pseudotumoral case and discuss therapeutic modalities. A 2-year-old boy was admitted for a 2-weeks history of fever and right flank pain. Physical examination revealed a right lumbar mass. White blood cell count was 12.800/mm3 and C reactive protein elevated [50 mg/1]. The urine culture was negative. Ultrasonography and computerized tomography demonstrated a focal multilocular cystic mass of the lower polar of right kidney, measuring 80 millimeters, with peripheral enhancement and perirenal involvement. At surgical exploration, a huge abscess of the lower polar of the kidney with dense adhesions to adjacent structures were noted. Excision of the collection and local drainage were performed. Pathologic examination of biopsy specimen confirmed the diagnosis of Xanthogranulomatous pyelonephritis. Intraoperative culture was positive for Escherichia coli. Successful treatment of the lesion was achieved with adjunctive antibiotic therapy. The postoperative course was uneventful over a follow-up period of 2 years. Xanthogranulomatous pyelonephritis should be considered in the differential diagnosis of a renal mass. A first-line conservative treatment must be strongly recommended in pediatric focal cases

[Interventional endoscopy in the gastrointestinal tract in children]

Interventional endoscopic procedures involving the gastrointestinal [GI] tract in children are very common. Over the last several years the number of procedures in this area has steadily increased. To study indications and results of GI interventional endoscopies performed in a Pediatric GI unit. Retrospective study over a period of 10 years [1999- 2008]. Data were collected from endoscopic files. All procedures were performed endoscopically under general anesthesia. 185 interventional endoscopic procedures were done during this period in 96 patients [58 boys] median age: 4.9 +/- 4.7 years [1 month-13 years]. Indications were as follows: esophageal stenosis [n= 46; 47.9%], rectal polyp resection [n=21; 19.8%], esophageal varices ligation [n=19; 19.7%], foreign body extractions [n= 8; 8.3%] and percutaneous endoscopic gastrostomy [n= 2; 2.1%]. One hundred and thirteen esophageal dilations [61.1% of procedures] were performed in 46 patients: caustic stenosis [n=22; 22.9%], peptic stenosis [n=13; 13.5%] and anastomotic stenosis [n= 11; 11.4%]. The main indication of interventional GI endoscopy in this series was oesophageal stenosis followed by rectal polyp resection, esophageal varices ligation and foreign body extractions

[Pyelonephritis xanthogranulomatous in childhood: case report and litterature review]

Xanthogranulomatous pyelonephritis [XGP] is a specific form of chronic inflammatory kidney disease rarely seen in children. The Symptoms are often vague and non-specific the aim of this paper is to return the particularities of imaging features in xanthogranulomatous pyelonephritis. insisting on differential diagnosis with renal tumors, especially in case of no renal stone or tract obstruction evidence, We report a case of xanthogranulomatous pyelonephritis in a 2-year-old boy involving the lower renal pole which demonstrates the diagnostic difficulties encountered in this disease Xanthogranulomatous pyelonephritis is a rare condition in children and should be included in the differential diagnosis of a child presenting a renal mass

[Cholestasis in infant: a study of the children's hospital of Tunis]

Cholestasis in infant constitutes an heterogeneous group of disease; diagnosis and management are often difficult. The Aim of the study is to describe clinical, paraclinical characteristics and outcome of infants hospitalized for cholestasis in children's Hospital of Tunis. A retrospective study of 94 infants with cholestasis was conducted. Patients were hospitalized in four departments of paediatrics of our hospital between January 1995 and December 2005. Cholestasis complicating severe sepsis and visceral leishmaniasis were excluded. Incidence of cholestasis was 8.5 cases/year which represented 0.72% of the hospitalizations. Sex ratio was 1.08 and mean age at diagnosis was 105 days [extremes: 1 day- 24 months]. Biliary atresia was the most common cause of extra hepatic cholestasis [13.8%]. Normal A GT cholestasis [11.7%], benign neonatal cholestasis [11.7%] and bile duct hypoplasia [9.5%] represented the most common aetiologies of intra hepatic cholestasis. Aetiology remained unknown in 12.7% of cases. Only three infants with biliary atresia had Kasai operation. After a mean follow-up of 6 years, 18% of patients had portal hypertension, 14.8% had hepatic failure and mortality rate was 14.8%. Cholestasis of unknown aetiologies are frequent in our hospital. Poor prognosis, in our study, is due to delay to diagnosis and difficulties in medical and surgical management

Clinical characteristics and outcome of cystic fibrosis: report of 16 cases

The aim of this report is to determine clinical characteristics and outcome of Cystic Fibrosis [CF]. Cases of CF managed at Infantile Medecine A Department in Children's Hospital of Tunis during 13 years [1994 -2006] were reviewed. 16 children had CF. They were 8 males and 8 females. 13 patients were consanguineous and four had similar familial cases. The mean age at diagnosis was 19 months [10 days, 13 years]. 3/4 of patients were symptomatic within the first trimester of life. Revealing symptoms were: obstructive bronchopathy associated to chronic diarrhea [n = 6], oedema-anemia-hypotrophy-hypoprotidemia syndrome [n = 3], meconium ileus [n = 4], bronchiectasis [n = 2] and chronic diarrhea [n = l]. The diagnosis was confirmed by sweat test and genotypic data. The F508 del was the most frequent mutation [54%]. Clinical outcome was characterized by the occurrence of respiratory and nutritional complications: acute respiratory failure [n = 6], chronic respiratory failure [n = 3], chronic pseudomonas aeruginosa infection [n = 6] at a medium age of 3.8 years, recurrent haemoptysis [n = 2], pleural effusion [n = 2], a malnutrition [n = 10] and diabetes associated to puberty delay in one patient. Seven patients died at mean age of 4.4 years [6 months, 17.3 years]. Among surviving patients, six had no compromised nutritional status or lung function. Prenatal diagnosis was performed in three families. CF is characterized by earliest onset and severity of symptoms. Therapeutic insufficiency is the main cause of precocious complications and poor prognosis in our series