Ibrahim Ahmed G, Crockard H Alan
Victor Horsley Department of Neurosurgery, The National Hospital for Neurology and Neurosurgery, London, United Kingdom.
J Neurosurg Spine. 2007 Dec;7(6):594-600. doi: 10.3171/SPI-07/12/594.
Basilar impression (BI) secondary to osteogenesis imperfecta (OI) is a rare but debilitating condition that is often progressive unless it is halted. More recently, ventral decompression surgery has been advocated for this condition. This study is a retrospective review of the 21-year experience of ventral decompression surgery and dorsal occipitocervical fixation in patients with BI secondary to OI and is the largest patient series reported to date.
Twenty patients treated between 1982 and 2003 by the senior author at the authors' institution were included in this study. All patients underwent ventral decompression surgery followed by dorsal craniocervical stabilization. Patients were followed up for a median of 10 years.
There were no intraoperative or perioperative deaths. Postoperatively, 16 of 20 (80%) patients showed objective improvement or maintained their good preoperative level of function. After surgery, of the 15 patients admitted with Karnofsky Performance Scale (KPS) scores of 70% or less, 11 improved, two remained unchanged, one patient's condition deteriorated, and one patient died of an unrelated cause. Of five patients admitted with a KPS score of 80% or greater, no patient's condition deteriorated in the short- and midterm period, but one patient had recurrence 15 years after surgery. At the end of follow-up, 25% of the patients had recurrence of brainstem compression symptoms or had died, and 15% showed no improvement after surgery. All of the remaining patients (60%) had sustained a long-term benefit from surgery.
Aggressive ventral decompression surgery and dorsal stabilization for patients with BI secondary to OI can not only halt disease progression but can also produce a good and sustainable long-term functional outcome, even in those patients who present as severely symptomatic. Patients who presented early with minor symptoms had good long-term outcomes.
成骨不全（OI）继发的颅底凹陷症（BI）是一种罕见但使人衰弱的疾病，若不加以阻止，通常会进展。最近，有人主张对这种疾病进行前路减压手术。本研究是对OI继发BI患者进行前路减压手术和后路枕颈固定术21年经验的回顾性分析，是迄今为止报道的最大患者系列。
本研究纳入了1982年至2003年间由资深作者在其所在机构治疗的20例患者。所有患者均接受了前路减压手术，随后进行了后路颅颈稳定术。对患者进行了中位时间为10年的随访。
术中及围手术期均无死亡病例。术后，20例患者中有16例（80%）显示出客观改善或维持了术前良好的功能水平。手术后，15例入院时卡氏功能状态评分（KPS）为70%或更低的患者中，11例病情改善，2例保持不变，1例病情恶化，1例患者死于无关原因。5例入院时KPS评分80%或更高的患者中，短期和中期均无病情恶化，但1例患者术后15年复发。随访结束时，25%的患者出现脑干压迫症状复发或死亡，15%的患者术后无改善。其余所有患者（60%）均从手术中获得了长期益处。
对OI继发BI的患者积极进行前路减压手术和后路稳定术，不仅可以阻止疾病进展，而且即使对那些症状严重的患者也能产生良好且可持续的长期功能结果。早期出现轻微症状的患者长期预后良好。
