Gorham's disease of the spine

Abstract

BACKGROUND: Gorham's disease of the spine is very rare and usually associated with poor prognosis. Till date very few cases have been reported in the literature. It is characterized by osseous invasion by angiomatous vascular mass without skip areas, eventually causing lysis of affected bone. Morbidity and mortality are high in those with spinal and/or visceral involvement. Neurological complications increase the mortality to 33% whereas with chylothorax, mortality increases to more than 50%. OBJECTIVES: To describe the clinical course and the challenges in planning and implementing effective rehabilitation services for management of patients with Gorham's disease of the spine. METHODS: We report two patients with Gorham's disease who had neurological deficits secondary to spinal cord involvement, and their rehabilitation perspectives. RESULTS: Both patients achieved satisfactory ambulation and functional outcomes. CONCLUSION: Multiple revision surgeries may be required to ensure spinal stability. When working with these patients, one must remain vigilant about spinal stability as well as about possible serious pulmonary complications, and be prepared to make appropriate management decisions when necessary.

Gorham’s Disease of Cervicothoracic Spine

“…Multiple revision surgeries were performed for this patient to ensure spinal stability. The second patient was a 15-year-old boy presented with progressive spinal deformity since eight years ago, back pain for three months, complete weakness of lower limbs, and bowel-bladder incontinence [4]. This patient was also managed surgically.…”

“…In case of an unstable spine or severe kyphosis, surgical decompression and stabilization are the available options, although not encouraging, mainly due to the underlying osteoclastic process of the vertebra. Moreover, multiple revision surgeries may be required to achieve spinal stability [3,4]. Accordingly, the treatment of Gorham's disease of the spine has become a challenge for spine surgeons, as well as its diagnosis.…”

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“…We carried out a revision of all clinical cases present in English language literature (Medline Pubmed) regarding pleural effusion secondary to GSS. Pleural effusion is a very rare presentation in GSS, indeed we founded only 51 clinical cases reported 3 ones were excluded from this work because the pleural effusion was presumably secondary to the surgical procedure [42,47,48]. Thus this review considers 48 case reports from literature in addition to ours (table 1).…”

“…Clinical presentation n. % Dyspnea 4,5,[8][9][10][11][12][13]16,18,20,21,23,24,[26][27][28][31][32][33]35,37,[41][42][43]45 31 72.1 Chest pain 4,9,10,12,22,23,29,31,34 13 30.2 Other pain 14,30,32,38,39,42 6 14.0 Fever 27,30,32 4 9.3 Asymptomatic pleural effusion 7,25,38 3 7.0…”

“…Types of pleural effusion n. % Chylous 4,7,8,10,13,14,[40][41][42][43][44][45][46][47] 42 85.7 Serohematic 9,11,12 5 10.2 Trasudate 39 1 2.0 Bloody 5 1 2.0 Side n. % Bilateral 5,[7][8][9]10,13,14,19,25,26,30,33,37,38,40,43 20 40.8 Right 8,11,12,18,21-23,27,28,32,35,38,40- Mean follow-up time (yrs ± SD) 2.0 ± 1.87…”