[Treatment of scoliosis and scoliokyphosis in Recklinghausen neurofibromatosis].

Spine deformities are a frequent symptom of neurofibromatosis Recklinghausen. Especially NF1 shows next to numerous alterations in the skeleton in some cases massive scoliosis and kyphosis. There are different theories for the development of the spine deformities, one of them is that specific alterations of the vertebra are caused by an elevated intraspinal pressure on the osteoporotic bone. A classification from a clinical point of view discriminates 3 types of severity. Type 1 shows instead of the in x-rays inconspicuous findings neurofibromatosistypical alterations in other diagnostic procedures (e.g. MRI). Extreme variations like short curved scoliokyphosis with massive destruction and severe spine imbalance are described as type 3. Operative treatment is dependent on the severity of the deformity. Intraspinal tumors have to be removed. Because of the elevated neurological risk the proceeding has to be very careful, sometimes there is a temporary Halo-extension necessary. Anterior substance defects are filled with bone or cages. The posterior instrumentation (in most of the cases a 2-rod-stabilization) is performed by transpedicular screws. Frequently there is a concave chest wall plastic (CTP) indicated. To prevent neurological complications early surgical procedure is sometimes necessary. Complications can be reduced by careful proceeding, exact preoperative diagnostic and classification. But next to operative experience a qualified anaesthesiological and intensive care units are absolutely necessary.