Payne Russell, Sieg Emily, Fox Edward, Harbaugh Kimberly, Rizk Elias
Department of Neurosurgery, Penn State Milton S. Hershey Medical Center, 30 Hope Drive, Building B, Suite 1200, Hershey, PA, 17033, USA.
Department of Orthopedics, Pennsylvania State Milton S. Hershey Medical Center, Hershey, PA, USA.
Childs Nerv Syst. 2016 Dec;32(12):2453-2458. doi: 10.1007/s00381-016-3166-3. Epub 2016 Jul 21.
Multiple hereditary exostoses (MHE) is a rare autosomal dominant condition that results in the growth of cartilage-capped prominences that often cause nerve compression and injury. Many patients suffer from continued and debilitating chronic pain which leads some to advocate avoiding surgical intervention in patients with multiple hereditary exostoses. We present a review of the literature as well as a case series at our institution in order to evaluate the role of surgery in multiple hereditary exostoses.
We searched the literature for reports of patients with multiple hereditary exostoses undergoing surgery for nerve compression. We then reviewed the recent experience at our institution which revealed two patients with multiple hereditary exostoses.
Our literature search revealed that there have been several case series and retrospective analyses in the literature that assess the benefit of surgery in the case of nerve compression caused by exostoses. The majority of these reports are of solitary exostoses. Few reports expand on the role of surgery in patients with multiple hereditary exostoses suffering from nerve compressions secondary to bony overgrowth. A recent review of the experience at our institution revealed two patients with multiple hereditary exostoses who together underwent a total of four surgeries for treatment of peripheral nerve compression resulting in pain or weakness. Postoperative evaluation revealed improvement in pain and/or motor strength following each operation.
Based on our experience and literature review, we advocate that nerve compression in selected individuals with multiple hereditary exostoses that results in neurological injury should be considered for nerve decompression and resection of the offending exostosis.
多发性遗传性骨软骨瘤(MHE)是一种罕见的常染色体显性疾病,会导致软骨帽状突出物生长,常引起神经受压和损伤。许多患者遭受持续且使人衰弱的慢性疼痛,这使得一些人主张避免对多发性遗传性骨软骨瘤患者进行手术干预。我们对文献进行综述,并展示我们机构的一系列病例,以评估手术在多发性遗传性骨软骨瘤中的作用。
我们在文献中搜索多发性遗传性骨软骨瘤患者因神经受压而接受手术的报告。然后回顾了我们机构最近的经验,发现了两名多发性遗传性骨软骨瘤患者。
我们的文献检索发现,文献中有几个病例系列和回顾性分析评估了手术在骨软骨瘤引起神经受压情况下的益处。这些报告大多是关于孤立性骨软骨瘤的。很少有报告阐述手术在患有因骨过度生长继发神经受压的多发性遗传性骨软骨瘤患者中的作用。最近对我们机构经验的回顾发现两名多发性遗传性骨软骨瘤患者共接受了四次手术,以治疗导致疼痛或无力的周围神经受压。术后评估显示每次手术后疼痛和/或运动力量均有改善。
基于我们的经验和文献综述,我们主张对于因多发性遗传性骨软骨瘤导致神经损伤的特定个体,若存在神经受压,应考虑进行神经减压和切除致病的骨软骨瘤。