Department of Physical Medicine and Rehabilitation, Christian Medical College, Vellore, Tamil Nadu, India.
NeuroRehabilitation. 2013;33(1):121-6. doi: 10.3233/NRE-130935.
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%.
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.
We report two patients with Gorham's disease who had neurological deficits secondary to spinal cord involvement, and their rehabilitation perspectives.
Both patients achieved satisfactory ambulation and functional outcomes.
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.
脊柱 Gorham 病非常罕见，通常预后不良。迄今为止，文献中报道的病例很少。其特征是血管性血管肿块侵犯骨骼，无跳跃区，最终导致受累骨溶解。脊柱和/或内脏受累者发病率和死亡率高。神经并发症使死亡率增加到 33%，而合并乳糜胸时，死亡率增加到 50%以上。
描述脊柱 Gorham 病患者的临床病程以及在规划和实施有效的康复服务以进行管理方面所面临的挑战。
我们报告了两名因脊髓受累而出现神经功能缺损的脊柱 Gorham 病患者及其康复情况。
两名患者均实现了令人满意的步行和功能结局。
可能需要多次修订手术以确保脊柱稳定性。与这些患者一起工作时，必须时刻警惕脊柱稳定性以及可能出现的严重肺部并发症，并在必要时准备做出适当的管理决策。
