Khan Shah Alam, Sehat Khosro, Calthorpe Denis
Department of Orthopaedic Surgery, Derbyshire Royal Infirmary, Derby, United Kingdom.
Spine (Phila Pa 1976). 2003 Aug 15;28(16):E313-5. doi: 10.1097/01.BRS.0000084663.17968.3A.
A case of cervical cord compression in an elderly lady with Hurler's syndrome is reported.
To report the occurrence of cervical cord compression in a 56-year-old patient of Hurler's syndrome, making her the oldest patient with Hurler's. The case report also highlights the appropriate management that could be given to this group of difficult patients.
The literature on Hurler's syndrome is reviewed in terms of long-term survival and surgical management of spinal compression. No report in the literature describes cervical spine decompression in Hurler's syndrome in a patient this old.
Clinical and radiologic features of Hurler's induced cervical cord compression are described. Pathologic changes of Hurler's myleopathy are also reported.
The patient underwent cervical spine decompression and tolerated the procedure well. The patient was able to mobilize free of the wheel chair and showed significant neurologic recovery.
The case report highlights the success of surgery in Hurler's induced cervical cord compression. It also adds to the literature the oldest patient of Hurler's syndrome to undergo any form of spinal surgery.We present here a 59-year-old lady with Hurler's syndrome, who underwent a successful cervical spine decompression and stabilization for cervical myelopathy. At 59 years of age, she was the oldest case of Hurler's to undergo spinal surgery. The case highlights the importance of undertaking major spinal surgeries in these difficult inherited disorders with reasonable success, as against common belief.
报告一例患有Hurler综合征的老年女性发生颈髓压迫的病例。
报告一名56岁Hurler综合征患者发生颈髓压迫的情况,使其成为患有Hurler综合征的最年长患者。该病例报告还强调了对此类疑难患者可采取的适当治疗方法。
就Hurler综合征的长期生存情况及脊柱压迫的手术治疗对相关文献进行了综述。文献中尚无关于如此高龄的Hurler综合征患者颈椎减压的报告。
描述了Hurler综合征所致颈髓压迫的临床和影像学特征。还报告了Hurler脊髓病的病理变化。
患者接受了颈椎减压手术,手术耐受性良好。患者能够独立活动,无需轮椅,并显示出明显的神经功能恢复。
该病例报告突出了手术治疗Hurler综合征所致颈髓压迫的成功。它还为文献增添了接受任何形式脊柱手术的最年长Hurler综合征患者。我们在此介绍一名59岁患有Hurler综合征的女性,她因颈椎脊髓病成功接受了颈椎减压和固定手术。59岁的她是接受脊柱手术的最年长Hurler综合征患者。该病例突出了在这些疑难遗传性疾病中进行大型脊柱手术并取得合理成功的重要性,这与普遍看法相反。