Lymphangiomatosis of the spine: two cases requiring surgical intervention.

Abstract

Two cases of lymphangiomatosis of the spine are presented. To report two cases of lymphangiomatosis of the spine requiring surgical intervention and to review the literature. Lymphangiomatosis is a rare childhood disease characterized by abnormal lymph tissue at multiple sites. Skeletal and visceral involvement are both common. Prognosis depends on the extent of extraskeletal disease. Two cases of lymphangiomatosis causing neural compression and instability at the cervicothoracic junction are presented. Both patients underwent surgical decompression and stabilization. One patient died, whereas the other regained full function and activity. Surgery is indicated when lymphangiomatosis causes neural compression and instability of the spine. Surgical outcome is strongly influenced by extraskeletal involvement.