Lymphangiomatosis of the spine: two cases requiring surgical intervention

Abstract

Study design: Two cases of lymphangiomatosis of the spine are presented.

Objectives: To report two cases of lymphangiomatosis of the spine requiring surgical intervention and to review the literature.

Summary of background information: 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.

Methods: Two cases of lymphangiomatosis causing neural compression and instability at the cervicothoracic junction are presented. Both patients underwent surgical decompression and stabilization.

Results: One patient died, whereas the other regained full function and activity.

Conclusions: Surgery is indicated when lymphangiomatosis causes neural compression and instability of the spine. Surgical outcome is strongly influenced by extraskeletal involvement.