Rare retro peritoneal tumor in infants.

Lipoblastoma is a rare mesenchymal tumor, occuring in less than three-year-old infants in 90% of the cases and in 40% of the cases in chidren aged less than one year. It can be present at birth. It is a benign tumor, with defined margins, that can be locally aggressive, without metastases. We report the case of retroperitoneal lipoblastomatosis, with a neonatal diagnosis made by ultrasonographic and CT features.

Self extrusion on an ingested foreign body: a case report.

Foreign body ingestion is a frequent issue in paediatrician's practice. Foreign bodies often pass the gastro-intestinal tract spontaneously but can sometimes generate complications (1% of the cases). The migration of ingested foreign bodies is rare, but their spontaneous extrusion through the skin is even rarer and was previously described only in the neck. We report an unusual case of a spontaneous extrusion of an ingested foreign body through the skin of the lower abdomen. Observation: A 2 year-old boy, presented with a 2cm inflammatory swelling of the hypogastric region. Laboratory analysis showed hyperleukocytosis (16 7770 /mm3) and high C reactive protein level at 12mg/L. Ultrasonography and computed tomography allowed us to diagnose a parietal foreign body extruding through the skin and to eliminate associated complication (perforation, vascular fistula…). The foreign body was extracted by a surgical incision. This observation is very rare but it is also uncommon because of the nature of the ingested foreign body which was a wooden piece. Its ingestion was explained by a paediatric mental disorder.

Prognostic factors in children with extracranial malignant germ cell tumors: a monocentric pediatric Tunisian study.

Background Extracranial Germ cell tumors (GCT) are a rare and a heterogeneous group of pediatric cancers but highly curable. Aim We aimed to review management, outcome and prognostic factors that influence overall survival (OS) in a pediatric Tunisian oncologic unit. Methods We retrospectively evaluated between January 1998 and December 2012, 33 patients affected by extracranial germ cell tumors and treated according to TGM95 protocol established by the SFOPin a pediatric Tunisian oncologic unit. Results Patients had a mean age of 57 months (ranges: 1 day-13 years). There were 19 girls and 14 boys. Primary sites included 12 sacrococcygeal, 11 ovarian, 6 testicular, 3retro peritoneal and 1 mediastinal site. After a mean follow up of 26.1 months (ranges: 0-96 months), OSat 2 years and 5 years were respectively 82% and 75%. Event-free survival were respectively 79% at 2 years and 74% at 5 years. Various prognostic factors have been studied according to Kaplan-Meier. Univariate analyses identified significant factors which influence strongly OS: the stage (p=0.04), the completeness of surgery (p<0.001) and the relapse (p = 0, 0001). A multivariate study showed that only the quality of resection and the clinical stage remained strong significant prognostic factors (p=0,021) for 5-year OS. Conclusion Disease stage, completeness of surgery and relapse have been established as the most powerful prognostic parameter in our analysis. The improvement of survival of patients affected by extracranial germ cell tumors in Tunisia is a real achievement mainly due to the success of salvage treatments.

Multi slice computed tomography approach in the assessment of supracondylar humeral fractures in children.

The purpose of this study was to assess the impact of Multi Slice Computed Tomography (MSCT) on the understanding of the spatial displacement of supracondylar humeral (SCH) fractures, their classification and their management. A prospective study was conducted on 63 children with SCH fractures Gartland II or Lagrange 2 and 3, over a period of 30 months. The patients were 42 boys and 21 girls, aged between 3 and 14. All patients were imaged using conventional radiography. Thirty-two patients underwent MSCT and 3-dimensional reconstructions. According to the Lagrange classification system, 16 patients had type 2 fractures and 47 had type 3 fractures. In type 2, the posterior cortices of both medial and lateral columns were bent on CT (n = 6). In type 3, CT-scan made it possible to distinguish two subgroups. In the first subgroup (n = 12) there was fracture of both anterior and posterior cortices of the lateral column; however, the posterior cortical surface of the medial column was preserved. In the second subgroup (n = 14), there was no cortical surface contact in the medial column, but the continuity of the posterior cortical surface of the lateral column was preserved. Based on a new concept of column stability, the use of CT-scan has allowed for a better understanding of supracondylar fractures in children.

Childhood soft tissue chondroma. Two cases report.

BACKGROUND: Soft tissue chondroma is a rare benign tumour, which is generally seen in adult. It consists of islands of heterotopic cartilaginous tissue and most localised on the hands and the feet. The hypothesis that microtrauma is involved in the aetiology of this condition has yet to find any factual support. AIM: To report two paediatric cases of soft tissue chondroma. CASES REPORT: The first is a soft tissue chondroma of the posterior aspect of the left axilla in a 3-year-old boy. CT scans showed a spherical fatty density soft tissue mass without evident calcifications, attaching the infraspinous muscle. The second patient is a 9-year-old girl presented with a right auricular finger soft tissue chondroma. Radiographs showed several punctuated calcifications with adjacent bone scalloping. MRI revealed a lobulated soft tissue mass attaching the flexor tendons. The tumours were entirely removed. Histological examination showed cartilaginous tissue in both cases. At follow-up, the patients had good functions without evidence of recurrence. CONCLUSION: Simple excision should suffice to treat soft tissue chondroma but care should be taken to make the excision complete if recurrence are to be avoided.

[Granulocytic sarcoma of the maxillary sinus: a pediatric case report]. / Sarcome granulocytaire du sinus maxillaire. À propos d'un cas pédiatrique.

BACKGROUND: Granulocytic sarcoma is a rare malignant extramedullary neoplasm of myeloid precursor cells, occuring before or after onset of leukaemia. Involvement of the head and neck region is rare, generally concerning the orbit. AIM: To illustrate imaging findings of granulocytic sarcoma in an unusual location; maxillary sinus. CASE: We report a case of maxillary sinus granulocytic sarcoma in a 13-month old boy revealed by facial nerve palsy, ptosis and jugal swelling, without any evidence of haematological disorders. The patient underwent computed tomography and magnetic resonance imaging exam which demonstrated a non specific maxillary sinus mass with destruction of the orbit floor and the alveolar bone. Diagnosis was obtained after bone biopsy allowing histological and immunohistochemical studies. CONCLUSION: Granulocytic sarcoma is a serious condition because of the associated hematologic disorders. In a sinus or orbit location, imaging features are non specific. Unless hematologic history is present, diagnosis is difficult and an immuno histo chemical study is required.

Magnetic resonance evaluation of acetabular residual dysplasia in developmental dysplasia of the hip: a preliminary study of 27 patients.

Thirty-one hips in 27 young girls, treated for developmental dysplasia of the hip in the authors' institute since 2003, showed persistent radiographic evidence of residual acetabular dysplasia. These hips were registered as candidates for pelvic osteotomy. A prospective study was conducted and these hips were evaluated by magnetic resonance imaging (MRI); the average age of the patients was 5 years. MRI measurement of acetabular angle and acetabular head index in 2 different landmarks (bone and cartilage) was performed. The results were correlated with plain radiographic film evolution. MRI studies revealed sufficient cartilaginous acetabular coverage in 27 hips, cartilaginous acetabular dysplasia in 2 hips, and short acetabulum in 2 others. The 27 hips with thick cartilage of the acetabular roof were subsequently followed up by plain radiographs. The average follow-up period was 2.1 years. The authors observed a spontaneous progressive ossification of the cartilaginous acetabular roof in all the 27 cases. In 4 cases, the correction of the acetabular angle was complete. They concluded that MRI promotes more accurate selection of patients for pelvic osteotomy and aids in the choice of the most appropriate type of osteotomy. Clinical imaging examples are presented and need to be further evaluated.

[Management of blunt duodenal and pancreatic injuries in children (about a series of 8 cases)]. / Prise en charge des traumatismes fermés du bloc duodéno- pancréatique chez l'enfant (a propos d'une série de 8 cas).

AIM: The aim of this retrospective study is to report eight cases of blunt duodeno-pancreatic trauma in infants, emphasizing on the role of imaging in acute assessment of the lesions and in further management. METHODS: We reported eight cases of duodeno-pancreatic injuries between 2006 and 2008, 5 boys and 3 girls with an age ranging from 3 to 12 years (median age: 7 years). Trauma circumstances were: car accident (n=2), domestic injury (n=5) and bicycle's fall injury (n=1). All patients underwent abdominal ultrasonography and CT scan in the initial evaluation and during the follow-up. RESULTS: Imaging showed the following pancreatic lesions: 3 corporeal fractures, 2 caudal fractures and one between the corporeal and the caudal portions. Four pancreatic haematomas were found. The associated lesions were duodenal, splenic, hepatic and renal. Two isolated duodenal haematomas were found. Two patients improved spontaneously, the six others developed complications: 4 acute pancreatitis, two infections, 3 pseudocysts and one retroperitoneal collection. Management was chirurgical in one case, medical in two cases, endoscopic in 2 cases and three percutaneaous drainages were performed. CONCLUSION: Blunt duodeno-pancreatic injuries in children have to be evaluated by an early imaging modality, in order to perform acute assessment of the lesions. Primary conservative treatment is advocated while clinic, biologic and imaging follow-up is required to detect complications, which management can be endoscopic, percutaneous or surgical.

Imaging of benign lipomatous tumours of the limbs in children.

BACKGROUND: Lipomatous tumours of the limbs in children are rare, and lipoblastoma is the most common soft tissue tumour. Most of them have typical imaging features, but their clinical presentation and their management may vary, depending on the exact histological subtype. The aim of our study is to illustrate the main clinical, radiological and histological features of the different benign lipomatous tumours in children. METHODS: Review of the literature. RESULTS: It is about a descriptive study of paediatric cases of benign lipomatous tumours of limb in children. CONCLUSION: The imaging findings are helpful and can provide essential components for the diagnosis.

Pyelonephritis xanthogranulomatous in childhood: case report and literature review.

BACKGROUND: Xanthogranulomatous pyelonephritis (XGP) is a specific form of chronic inflammatory kidney disease rarely seen in children. The Symptoms are often vague and non-specific AIM: 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. CASE: 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 CONCLUSION: Xanthogranulomatous pyelonephritis is a rare condition in children and should be included in the differential diagnosis of a child presenting a renal mass.

[Congenital hepatic fibrosis in children. Report of 9 cases and review of the literature]. / Fibrose hepatique congenitale chez l'enfant. A propos de 9 cas et revue de la litterature.

Congenital hepatic fibrosis is a recessive autosomic disease with two major risks: gastrointestinal haemorrhage caused by portal hypertension and cholangitis related to bacterial infection of dilated intrahepatic bile ducts.. The aim of our study is to define epidemiological features, the presenting symptoms, the diagnosis, the evolution and the management of this disease. Between January 1990 and December 2000, we reported the cases of nine children with this disease at children hospital of Tunis. Three were male and six female. The mild age was three years and six months. Consanguinity was present in five cases and similar cases were found in six cases. The FHC was revealed by portal hypertension in five cases, angiocholitis in one case and by portal hypertension and angiocholitis in three cases. Liver biopsy was done in seven children. Ultrasound examination of the liver and kidney revealed caroli syndrome in five cases and polykystose renal in two cases The intravenous pyelography was performed in four cases showing precalicial canalicular ectasia in four cases. Eosophageal endoscopy had shown oesophageal varices in six patients. The follow up had shown that three patients had gastrointestinal bleeding, three had angiocholitis. One patient died with multivisceral failure. The treatment of acute bleeding has needed blood transfusion in four cases. Primary prevention of bleeding was done by endoscopic sclerosis alone in one case and associated to betablokers in two cases. Secondary prevention of varices bleeding was done by sclerotherapic in two cases, by beta blokers alone in one case and by betablokers associated to elastic ligation of oesophageal varices in one case.

[Primary T-cell lymphoma of the pancreas in children]. / Lymphome T primaire du pancreas chez l'enfant.

Involvement of the gastrointestinal tract is frequently reported among the extranodal sites of non-Hodgkin's lymphoma, but primary lymphoma of the pancreas is very rare. We report the case of a 11-year-old boy explored for epigastric pain with fever leading to the diagnosis of high-grade primary non Hodgkin's T-cell lymphoma, originating from pancreas. This pancreatic lesion is documented by sonography, CT and MRI. This patient died quickly with sever infection.

[Hypertrophic osteoarthropathy revealing a malignant thymoma in a child]. / Osteoarthropathie hypertrophiante revelant un thymome malin chez un enfant. Apport de l'imagerie.

Association of hypertrophic osteoarthropathy and malignant thymoma is very rare in children. Authors report a case of a 14 year old girl followed for rheumatic fever since she was 8 years old. She was explored for arthralgia. The exam showed hippocratic fingers, soft tissues hypertrophy of lower limbs and dyspnoea. Plain radiography had demonstrated symmetrical ossifying periostisis in tubular bones of lower limbs evocating hypertrophic osteoarthropathy diagnosis. Chest radiography has demonstrated bilobate anterior mediastinal mass. Ultrasonography and MRI revealed a voluminous anterior mediastinal mass with cystic and fleshy components. Thymic adenocarcinoma diagnosis was made by surgical biopsy.

[First seizure in children. Exploration strategy]. / Première crise convulsive de l'enfant. Stratégie d'exploration.

We have carried out a retrospective study on 51 children aged between 2 years 3 months and 13 years in order to determine the findings and to define the indications of neuroimaging modalities in children with a first unprovoked seizure. Children who had neuroimaging studies were divided into two groups based on the results of neuroimaging normal or abnormal. We have compared the two groups according to each clinical and electroencephalographic parameters studied. Neuroimaging abnormalities were found in 47% of cases. According to our results and literature review, we concluded that the imaging should be done if we have at least one of the criteria: an abnormal neurological examination after the seizure and focal slowing waves on the electroencephalogram.