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.
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.