[McCune-Albright syndrome: a case report and literature review].

McCune-Albright syndrome is an inherited disease characterized by the association of fibrous dystrophy of bone, café-au-lait skin spots and precocious puberty revealing endocrine hyperactivity. Genetically, this disease is due to a mutation of the Gs protein responsible for activation of adenylate cyclase with excessive production of cAMP. The particular morphology of café-au-lait spots should suggest early diagnosis.

Congenital syndactyly: a retrospective study of 18 cases at the Department of Orthopaedic Surgery and Traumatology of the Habib Bourguiba University Hospital, Sfax, Tunisia.

Congenital syndactylies are frequent congenital malformations of the hand. They can be an isolated finding or they can be found in association with other polymalformative syndromes. Several surgical techniques used to treat them have been described in the literature.

A Review of Ewing Sarcoma Treatment: Is it Still a Subject of Debate?

Background: The Ewing sarcoma (ES) represents 10 to 15% malignant bone tumors and 40 to 45% pediatric malignant bone tumors. The aim of this review is to clarify the therapeutic results and prognostic factors of this entity.

Methods: A systematic review of the literature was performed.

Fibrodysplasia ossificans progressiva: diagnosis and surgical management.

Fibrodysplasia (or Myositis) Ossificans Progressiva (FOP) is a rare genetic disease with variable expression, characterized by the association of congenital anomalies of the toes and fingers and progressive appearance of ectopic bone within the skeletal muscles, often following a trauma or an infection. FOP initially affects the nape and thoracic paravertebral muscles. With age, there is a progression of ossifications to other muscular groups following a proximodistal and cranio-caudal extension.

[Orthopaedic manifestations of Von Recklinghausen's neurofibromatosis].

Objective: Von Recklinghausen's neurofibromatosis is a dominant autosomic genetic disease characterized by different clinical manifestations. The goal of this work was to study its orthopaedic manifestations and to show the characteristics of their management.

Method: A retrospective study was carried out on 15 patients having a Von Recklinghausen's neurofibromatosis.

[Ewing sarcoma osseous and extraosseous : a clinicopathologic study of 29 cases].

Background: Ewing's sarcoma (ES) is a rare tumour accounting for 10% of primary malignant bone tumours in children and 3% of all childhood malignancies. ES belongs to a group of small round cell tumours.

Aim: In this review, we will describe the main clinicopathological features of this rare tumour and discuss its prognosis.

Adenoid cystic carcinoma of the parotid with facial nerve invasion.

Background: Adenoid cystic carcinoma comprises approximately 10% of all epithelial salivary neoplasms and most frequently involves the parotid. Perineural invasion is a common feature but intraneural invasion and spread is less common.

Aim: The authors report a case of parotid adenoid cystic carcinoma with invasion and spread via the facial nerve with an emphasis on radiologic features.

A rare Wilkins group II fracture of the proximal radial metaphysis.

In this study, we report a rare fracture of the proximal radial metaphysis in an 11-year-old child, involving an anterior displacement of the proximal radial metaphysis instead of a displacement of the epiphysis, which preserved its normal connections with the capitellum. This fracture was successfully treated by closed reduction and wire fixation. The reduction maneuver attempted to reduce the proximal radial metaphysis under the epiphysis instead of doing the opposite.

Neuroimaging findings in children with infantile spasms.

Objective: The aim of the study was to document the neuroimaging findings of children with infantile spasms (IS) seen over a 3-year period.

Methods: All children below the age of 4 years who presented to the Pediatric Department at the Northern Area Armed Forces Hospital, Hafr Al-Batin, Kingdom of Saudi Arabia from January 1, 1998 to December 31, 2000 with a history of seizures, atypical movements, psychomotor delay, flexor, extensor spasms or both were included in the study. Relevant birth, developmental and family history as well as information on the pattern of fits were documented.

Petro-cavernous chondroma. CT and MRI features.

The authors report an unusual location of a benign chondroma of the petro-sphenoidal synchondrosis extending into the cavernous sinus. Computerized tomography and magnetic resonance image features were characteristic of chondroid tumor. However, pathologic verification is mandatory in order to distinguish chondroma from chondroblastoma or chondroid chordoma.

Magnetic resonance imaging and Neuro-Behcet Disease.

Behcet disease is a rare multi-system immune related vasculitis which may involve the central nervous system, usually during the course of the illness. Magnetic resonance imaging is currently the modality of choice for the detection of the parenchymal lesions in their different phases, as well as the dural sinus venous thrombosis.