Lymphangiomatosis of the spine: two cases requiring surgical intervention.

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.