Burkitt lymphoma in a child with Bloom syndrome.

BACKGROUND: Bloom syndrome is a rare disease characterized by chromosomal instability and increased risk of developing lymphoma. OBSERVATION: We report on a case of Bloom syndrome in a 5-year-old boy with Burkitt lymphoma. The diagnosis was suspected by growth retardation, repeated respiratory infections, facial telangiectasia, and a low immunoglobulin level, then confirmed cytogenetically by sister chromatid exchanges. Chemotherapy was poorly tolerated, which required reducing the doses. Unfortunately, it was not sufficient to control the neoplasm and the patient died 14 months after diagnosis. CONCLUSIONS: Cancers in Bloom syndrome are a challenge since the potentially life-threatening side effects of the chemotherapy may require modifications in standard treatment such as dose reduction, which can compromise the tumor prognosis.

Dysplasia epiphysealis hemimelica: a report of four cases.

UNLABELLED: Dysplasia epiphysealis hemimelica (DEH), also known as Trevor's disease, is a rare congenital skeletal developmental disorder in childhood. It is characterized by an asymmetric osteocartilaginous overgrowth arising from epiphyses or epiphyseal equivalents. Lesions have hemimelic topography, usually occur in the lower limbs, most commonly in the ankle and the knee and affect either the medial or lateral part of one epiphysis. OBJECTIVE: The purpose of this study is to describe the imaging features of DEH by reporting four cases. MATERIALS AND METHODS: We present four cases of DEH in one female and three males aged between 7 and 15 years. Lower limb is involved in all cases and patients suffer from pain, limited function and deformity. Radiographs and CT findings were reviewed. All patients were treated by surgical excision. RESULTS: Plain X-ray revealed in all cases an irregular ossification arising from the affected epiphysis. The CT scan revealed an irregular fragmented osteocartilaginous mass involved from the epiphysis, with enlargement of epiphyses and intra-articular extension. CONCLUSION: The early diagnosis and treatment of DEH is necessary in preventing articular function, CT assists in defining the anatomic relationship between the mass and its parent epiphysis and in evaluating the condition of the articular cartilage and soft tissue. The distinct clinical and radiographic features should enable to differentiate the osteochondroma and the DHE.

[Orbital subperiosteal hematoma in child with sickle cell thalassemia. A case report]. / Hématome sous-périosté orbitaire chez un enfant drépano-thalassémique.

Sickle cell anemia is the most common hemoglobinopathy worldwide; its musculoskeletal complications consist more often of medullary infarctions involving long bones. Orbital infarction is uncommon. We report on a case of orbital subperiosteal hematoma in a 9-year-old male with a medical history of sickle cell thalassemia.

[MRI exploration for the evaluation of orbital implant biocolonization in children enucleated for retinoblastoma]. / Apport de l'IRM dans la surveillance de la biocolonisation des implants intra-orbitaires chez des enfants énucléés pour rétinoblastome.

PURPOSE: To evaluate the contribution of MRI exploration for the evaluation of orbital prothesis biocolonization. PATIENTS AND METHODS: We studied ten eyes of ten children who underwent enucleation for retinoblastoma and synthetic hydroxyapatite orbital implantation. Each patient was examined by MRI imaging with gadolinium within 2 months to 1 year. RESULTS: After gadolinium administration, five implants showed an enhancement. Nodular enhancement around the implant was noted in one patient with a recurrence of retinoblastoma, and brain metastases were shown in two cases. None of the orbital implants was rejected. CONCLUSION: Fibrovascular colonization reduces the risk of orbital implant migration. Magnetic resonance imaging is safe and effective in detecting extrusion or tolerance of the orbital implant.

[Intrabronchial migratory cereal-ear, an unusual foreign body inhalation]. / Epis de graminée intrabronchiques migrateurs, un corps étranger hors du commun.

Bronchial foreign body inhalation is a frequent and potentially serious accident in children. It can provoke long-term complications such as bronchiectasis and recurrent pulmonary infections. The foreign body is generally blocked in a bronchus and induces obstructive emphysema with superinfection. We report 2 cases of bronchial inhalation of a migrating cereal-ear, eliminated by parietal fistulization, in 9- and 11-year-old boys.

[Pachydermoperiostosis: a report of two cases]. / Pachydermopériostose: à propos de deux cas.

Pachydermoperiostosis or primary hypertrophic osteoarthropathy is a benign genetically determined disease, manifested by finger clubbing, hypertrophic skin changes and periosteal bone formation. It is rarely reported and was for a long time misdiagnosed and confused with secondary hypertrophic osteoarthropathy. We present the imaging features in two cases and review the literature.