Occipital dermoid cyst associated with dermal sinus complicated with meningitis: A case report.

BACKGROUND: Occipital dermal sinus, usually associated with dermoid cyst, is a rare entity; it results from the persistence of an abnormal embryonal communication between the skin and the intradural space. Its main complication is intracranial infection. CASE DESCRIPTION: This 2-year-old girl was hospitalized for meningitis. Neuroradiological studies revealed a cystic mass of the posterior fossa communicating with the skin and hydrocephalus. The diagnosis of dermoid cyst associated with dermal sinus was established at surgery. The patient was treated with radical excision of both the occipital cyst and the dermal sinus associated with systemic antibiotic therapy. She had a good outcome. CONCLUSION: Posterior fossa dermoid cyst should be considered in all children with chronic occipital skin lesion, especially a dermal sinus. We emphasize the importance of early neurosurgical treatment of dermoid cysts to prevent the development of severe complications.

Unusual intraatrial thrombus in a neonate with coarctation of the aorta.

Left atrial thrombus in neonates is uncommon. We describe a newborn with coarctation of the aorta, in whom a thrombus confined to the left appendage was discovered on the first day of life and was thought to be a myxoma.

[Hereditary hemorrhagic telangiectasia. Report of a pediatric case]. / La maladie de Rendu-Osler-Weber : à propos d'une nouvelle observation pédiatrique.

Hereditary hemorrhagic telangiectasia, or Rendu-Osler-Weber syndrome, is an autosomal dominant multiorgan disorder. This multisystemic vascular dysplasia is determined by a mutation of one of two main genes, endoglin (ENG) or HHT1, or ACVRL1 or HHT2. These mutations induce vascular disorders that cause recurrent epistaxis and eventually multiple telangiectasia and arteriovenous visceral malformations. We report the case of a 7-year-old girl who developed severe hypoxemia due to multiple pulmonary arteriovenous malformations.

[Aicardi syndrome associated with severe congenital ptosis]. / Un syndrome d'Aicardi associé à un ptosis congénital sévère.

Aicardi syndrome is a rare neurodevelopmental disorder characterized by corpus callosum agenesis, chorioretinal lacunae and early-onset infantile spasms. We report a particular case of Aicardi syndrome characterized by the association of the classical triad of severe bilateral ptosis, pontocerebellar hypoplasia, and perisylvian polymicrogyria in a girl born to non-consanguineous parents, but whose mother suffered from idiopathic generalized epilepsy.

[MR imaging features of tuberculosis of the sellar region]. / Aspects IRM de la tuberculose de la région sellaire.

PURPOSE: Tuberculosis of the sellar region, especially the pituitary gland, is rare. The purpose of this article is to demonstrate through a review of five clinical cases the value of imaging, especially MR imaging, in the evaluation of this pathology. PATIENTS AND METHODS: CT and MRI of the brain were obtained in all cases along with a chest radiograph. RESULTS: Four patterns were detected on MRI: pituitary tuberculoma mimicking adenoma; pituitary abscess, extending to the cavernous sinus in one case and associated with infundibulum thickening in another; hypophysitis with suprasellar extension in association with tuberculous meningoencephalitis; and infundibular thickening associated with tuberculous meningoencephalitis. Diagnosis was based on biopsy in two cases and combination of imaging and clinical data in three cases. Outcome was favorable with anti-tuberculosis drugs. CONCLUSION: Irrespective of the imaging features, a history of travel to an endemic region combined to other findings such as infundibular thickening should raise concern for the possibility of tuberculosis even in the absence of signs of systemic infection.

[Recurrent inflammatory myofibroblastic tumor with renal, retroperitoneal and lymph node involvement]. / Tumeur myofibroblastique inflammatoire récurrente avec triple localisation, rénale, rétropéritonéale et ganglionnaire.

Inflammatory myofibroblastic tumors are uncommon and benign tumors with unknown aetiology. First reported in the lungs, the inflammatory myofibroblastic tumors have been observed in other locations, especially in the abdomen and the pelvis. We report a 14-year-old adolescent female, who presented sequentially an inflammatory pseudotumor of lymph node, the left kidney and the retroperitoneum. Extrapulmonary inflammatory myofibroblastic tumors are mesenchymal solid tumors. They are frequently circumscribed and confined to a single organ. The recurrence of some inflammatory myofibroblastic tumors and their expression of chromosomal abnormalities found in some types of lymphoma suggest that some of these lesions constitute a true neoplastic process.

[Frantz's tumor: anatomoclinical study of six Tunisian cases]. / La tumeur de Frantz: etude anatomoclinique de six cas tunisiens.

Solid and pseudopapillary tumour (Frantz's tumour) is a rare low-grade neoplasm of the pancreas. We report six new cases. Our objective is to specify clinical and pathological characteristics of this rare neoplasm and to discuss its histogenesis. A retrospective review was considered on six Tunisan patients who had solid and pseudopapillary tumor of the pancreas. A review of medical registries and morphological analysis with immunohistochemical study were carried out in all cases. Four patients were female and two patients were male with a median age of 27,5 years (range: 14 - 68 years). Abdominal pain was the most common initial symptoms (5 cases/6). Abdominal computed tomography and/or ultrasonography was used in all the cases. The tumour was in the tail of the pancreas in 4 patients and in the body of the pancreas in one patient; one tumor involved all the pancreas. The median diameter of the tumour was 16,8 cm (range: 8 - 35 cm). Three tumours had an extrapancreatic extension. All patients underwent surgical resection. No adjuvant therapy was recommended. The mean follow up period was 24 months (range: 5 - 78 months). Only one patient died during the surgery. Except for this patient, none experienced tumor recurrence or tumor-related mortality during the follow up period. Solid and pseudopapillary tumour of the pancreas is an uncommon neoplasm which shows distinct clinicopathologically characteristics. Despite diverse studies, its histogenesis remains undetermined. This tumor should be distinguished from other pancreatic neoplasms because its prognosis is excellent after surgical resection.

[Central neurocytoma: three case reports]. / Neurocytome central: à propos de trois observations.

Central neurocytoma is classically recognized as an intraventricular benign brain tumour. Extraventricular localisation is rarer. We report two intraventricular neurocytoma and another bulbar extraventricular neurocytoma. Histologically, central neurocytoma presents remarkable likeness characteristics to oligodendroglioma, but immunohistochemical study distinguishes this tumour. Imaging appearances (CT, MRI) raise the diagnosis and immunohistochemical study confirm it. The purpose of our work is to assess the value of imaging (CT, MRI) in the diagnosis of central neurocytoma.

[Trans-ethmoid encephaloceles]. / Les céphalocèles transethmoïdales.

We report two cases of ethmoidal cephaloceles. Ethmoidal cephaloceles are very rare and most commonly present with recurrent episodes of meningitis or sometimes as a nasal mass. Diagnosis is made at MR and CT. Such imaging studies should be obtained in patients with recurrent meningitis or patients with polypoid nasal lesions prior to biopsy.

[Value of imaging in the assessment of malignant fibrous histiocytoma of the soft tissues]. / Place de l'imagerie dans le bilan des histiocytomes fibreux malins des parties molles.

Malignant fibrous histiocytoma (MFH) is a rare and potentially highly malignant sarcoma. The authors report 6 cases of MFH in various sites: two in the chest wall, one in the pelvis, two in the gluteal zones and one on the scalp. Ultrasonography and computed tomography were the main imaging methods used in the assessment of the structure and extension of the tumor. A poor prognosis was noted in four cases: death within a few months in the two thoracic sites, recurrence in the pelvic and scalp lesions, radical surgery allowed recovery in two cases. A review of the literature showed that MRI and CT are complementary in the initial staging and follow-up of these patients.