Atlanto axial instability due to neurofibromatosis: case report.

Neurofibromatosis is an autosomal dominant genetic disease, characterized by café au lait spots, neurofibromas and several bony anomalies. Deformities of the spine are the most frequent alterations. Involvement of the cervical spine has been studied less frequently. The case of a 16-year-old male patient affected by neurofibromatosis, with cervical pain without neurological symptoms is presented. X-rays, CT-scan and MRI demonstrated the presence of cervical kyphosis, occipitoaxial instability and atlantoaxial instability with subluxation. Posterior occipito-C2 fusion was performed with prior placement of a halo-vest. The outcome at four years was good with solid occipito axial fusion, moderate loss of cervical spine flexion and moderate-to-severe limitation of cervical spine rotation. The incidence and variety of alterations of the cervical spine in patients affected with neurofibromatosis is discussed, as well as the results obtained by the treatment.

[Pigmented villonodular synovitis of the wrist. Apropos of a case]. / Synovite villonodulaire hémopigmentée du poignet. A propos d'un cas.

The authors presented a case of pigmented villonodular synovitis of the wrist with extrinsic bony defects on opposing articular surfaces, seen after two recurrences following excision surgery. They performed a total synovectomy followed by curettage and autogenous bone grafting of the carpal and radiocubital bone lesions. Four years after the procedure, the patient-a woman aged 44-was symptom-free and there was no objectivable evidence of recurrence. Pigmented villonodular synovitis (PVNS) is an uncommon, benign, chronic process that causes synovial proliferation. Its etiology is unknown. The majority of patients is young to middle-aged adults. Especially the knee but other joints are involved including the hip, ankle, wrist, hand, and foot. Both soft tissue and bone abnormalities occur in PVNS. Magnetic resonance imaging (MRI) shows a heterogenous synovial mass with intermediate and low signal intensity areas on T1 and T2-weighted images, consistent with hemosiderin deposition. Computed tomography defines clearly the extrinsic bone erosions and excavations, which usually exhibit a sclerotic margin, produced by intraosseous invasion of the expanding synovia. After excision recurrence is common, leading eventually to arthrodesis, arthroplasty and even radiation therapy. The etiology of PVNS is controversial. First approach is conservative surgical treatment with total synovectomy and curettage of the osteolytic foyers. Bone grafts are employed for filling the sites of osteolysis. The sensitivity of MRI permits early detection of the process and may obviate the need for radical management options in PVNS.

Myxoma causing paralysis of the posterior interosseous nerve.

A case of posterior interosseous nerve paralysis at the level of the Frohse arcade, caused by an intramuscular myxoma, is reported. Review of the literature shows this to be an exceptional presentation of this tumor as a cause of a compressive peripheral neuropathy. Twelve months after surgical treatment, total neurological recovery was observed.