Department of Neurosurgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany.
International Center for Lysosomal Disorders (ICLD), University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Eur Spine J. 2022 Jul;31(7):1693-1699. doi: 10.1007/s00586-022-07168-0. Epub 2022 Mar 10.
Spinal abnormalities frequently occur in patients with mucopolysaccharidosis (MPS) types I, II, IV, and VI. The symptoms are manifold, which sometimes prolongs the diagnostic process and delays therapy. Spinal stenosis (SS) with spinal cord compression due to bone malformations and an accumulation of storage material in soft tissue are serious complications of MPS disease. Data on optimal perioperative therapeutic care of SS is limited.
A retrospective chart analysis of patients with MPS and SS for the time period 01/1998 to 03/2021 was performed. Demographics, clinical data, neurological status, diagnostic evaluations (radiography, MRI, electrophysiology), and treatment modalities were documented. The extent of the SS and spinal canal diameter were analyzed. A Cox regression analysis was performed to identify prognostic factors for neurological outcomes.
Out of 209 MPS patients, 15 were included in this study. The most dominant type of MPS was I (-H) (n = 7; 46.7%). Preoperative neurological deterioration was the most frequent indication for further diagnostics (n = 12; 80%). The surgical procedure of choice was dorsal instrumentation with microsurgical decompression (n = 14; 93.3%). A univariate Cox regression analysis showed MPS type I (-H) to be associated with favorable neurological outcomes.
Early detection of spinal stenosis is highly relevant in patients with MPS. Detailed neurological assessment during follow-up is crucial for timeous detection of patients at risk. The surgical intervention of choice is dorsal instrumentation with microsurgical decompression and resection of thickened intraspinal tissue. Patients with MPS type I (-H) demonstrated the best neurological course.
黏多糖贮积症（MPS）I、II、IV 和 VI 型患者常出现脊柱异常。其症状多种多样，这有时会延长诊断过程并延迟治疗。由于骨畸形和软组织中储存物质的积累导致的脊髓压迫引起的脊柱狭窄（SS）是 MPS 疾病的严重并发症。关于 SS 的最佳围手术期治疗护理的数据有限。
对 1998 年 1 月至 2021 年 3 月期间患有 MPS 和 SS 的患者进行回顾性图表分析。记录人口统计学、临床数据、神经状态、诊断评估（影像学、MRI、电生理学）和治疗方式。分析 SS 的程度和椎管直径。进行 Cox 回归分析以确定神经结局的预后因素。
在 209 名 MPS 患者中，有 15 名纳入本研究。最主要的 MPS 类型是 I（-H）（n=7；46.7%）。术前神经恶化是进一步诊断的最常见指征（n=12；80%）。首选的手术方法是背侧器械固定伴显微减压（n=14；93.3%）。单变量 Cox 回归分析显示，MPS 型 I（-H）与良好的神经结局相关。
在 MPS 患者中，早期发现脊柱狭窄非常重要。在随访期间进行详细的神经评估对于及时发现有风险的患者至关重要。首选的手术干预是背侧器械固定伴显微减压和增厚的椎管内组织切除。MPS 型 I（-H）患者的神经功能预后最佳。
