Histiocitosis de células de Langerhans con compromiso vertebral y de partes blandas: Caso clínico / Langerhans cell histiocytosis with vertebral involvement and soft tissue extension: Clinical case

La histiocitosis de células de Langerhans es una enfermedad heterogénea de etiología desconocida, que se caracteriza por la proliferación no controlada de histiocitos. Es poco frecuente y, si bien el compromiso óseo es común, la afectación vertebral es rara. Se presenta una niña de 4 años que consultó por dolor abdominal difuso de un mes de evolución, al que se agregó constipación y, posteriormente, debilidad en los miembros inferiores. El examen físico mostraba clonus e hiperreflexia en los miembros inferiores y la marcha era inestable. Se realizó una resonancia magnética, que mostró la vértebra dorsal 9 (D9) plana con tejido blando patológico en el espacio epidural y laterovertebral. Se realizó una cirugía descompresiva, artrodesis para fijar la columna y toma de biopsia, que confirmó el diagnóstico de histiocitosis de células de Langerhans. Recibió 6 meses de tratamiento con metilprednisona y vinblastina, de acuerdo con el protocolo LCH III, con excelente evolución y remisión completa. Conclusión. Frente a una imagen radiológica de vértebra plana o colapso vertebral, debe pensarse en histiocitosis de células de Langerhans como diagnóstico diferencial.

Langerhans cell histiocytosis is a heterogeneous disease of unknown etiology characterized by proliferation of Langerhans cells. It is a rare disease. Bone involvement is common but vertebral disease is rare. We present a 4 year old patient with abdominal pain and neurologic symptoms. Magnetic resonance showed vertebra plana in D9 with involvement of paravertebral soft tissues. The child underwent surgery for decompression and biopsy. Biopsy confirmed Langerhans cell histiocytosis. She was treated with vinblastine and prednisone during 6 months following LCH-III with complete recovery of neurologic symptoms. Conclusion. Langerhans cell histiocytosis is a differential diagnosis in a radiograph with vertebra plana or collapse of vertebral body.

Percutaneous Vertebroplasty Following Postural Reduction in Unstable Vertebra Plana; Is it a Contraindication?

OBJECTIVE: Severe vertebral body collapse (vertebra plana) is considered a contraindication to vertebroplasty by most authors. The purpose of this study is to determine the efficacy of vertebroplasty in treating severe compression fracture patients with osteoporosis. METHODS: 16 patients underwent 18 vertebroplasties following postural reduction for vertebra plana. The fractures were defined vertebrae that have collapsed to more than 75% of their original height. Imaging and clinical features were analyzed, including involved vertebrae level, vertebral height after postural reduction for 2 days, injected cement volume, clinical outcome and complications. RESULTS: Involved veretebra were located from level T7 to L4. Vertebral body collapse averaged 79% (range 12~25%) of the original height. After pillow reduction for 2 days, vertebral body height increased 35% of the original height (range 15~45%). The kyphotic wedge was 12 degrees before procedure and was decreased 7.0 degree after vertebroplasty. The mean injected cement volume was 3.8 ml (range 2.0~4.9 ml). After the procedure, surgical outcome was excellent in 8 (50%) of 16 patients, good in 7 (42%) and unchanged in one (8%). The mean pain score (VAS score) prior to vertebroplasty was 8.3 and it changed 3.2 after the procedure. Cement leakage to the adjacent disc (5 cases) and paravertebral soft tissues (4 cases) developed but there were no major complications. CONCLUSION: We propose that vertebra plana due to osteoporosis is not a contraindication to vertebroplasty. Vertebroplasty following postural reduction for severe compression fracture is safe and effective treatment.