患有科芬-洛里综合征患者颈椎黄韧带焦磷酸钙晶体沉积行椎板切除术后八年随访：一例报告
Eight years of follow-up after laminectomy of calcium pyrophosphate crystal deposition in the cervical yellow ligament of patient with Coffin-Lowry syndrome: A case report.
作者信息
Morino Tadao, Ogata Tadanori, Horiuchi Hideki, Yamaoka Shintaro, Fukuda Mitsumasa, Miura Hiromasa
机构信息
Spine Center, Ehime University School of Medicine Department of Pediatrics, Ehime University School of Medicine Department of Orthopedic Surgery, Ehime University School of Medicine, Shitsukawa, Tohon City, Ehime, Japan.
出版信息
Medicine (Baltimore). 2016 Aug;95(31):e4468. doi: 10.1097/MD.0000000000004468.
BACKGROUND
We report 8 years of follow-up after decompression to treat cervical myelopathy in a patient with Coffin-Lowry syndrome (CLS). CLS is a rare X-linked semidominant syndrome associated with growth and psychomotor retardation, general hypotonia, and skeletal abnormalities. In this patient, the spinal cord was compressed by calcium pyrophosphate crystal deposition in the cervical yellow ligament (YL). To date, only 1 report has described clinical features after surgery for calcified cervical YL in CLS.
METHODS
A 15-year-old male with tetraplegia secondary to compression of the cervical spinal cord induced by a hypoplastic posterior arch of C1 and calcification of the YL from C2 to C7 was treated surgically with laminectomy from C1 to C7. The patient's history, clinical examination, imaging findings, and treatment are reported. The patient was incapable of speech because of mental retardation, so he could not describe his symptoms. Gait disturbance worsened over the 2 months before admission to our hospital. At admission, the patient could not move his extremities, and tendon reflexes of the upper and lower extremities were significantly increased. Computed tomography of the cervical spine showed YL calcification from C2 to C7. Magnetic resonance imaging showed consecutive compression of the cervical spinal cord. We diagnosed quadriplegia secondary to cervical cord damage and performed emergency surgery.
RESULTS
During C1-C7 laminectomy, YL calcification in C2-C7 was observed. The calcification was confirmed as calcium pyrophosphate by crystal analysis. Quadriplegia gradually resolved, and almost disappeared by 2 weeks after the operation. Cervical hyperlordosis was observed in radiographs starting from 1 month after the operation, but it has not progressed and is not associated with any symptoms.
CONCLUSIONS
The efficacy of decompression continued, and no postoperative complications have occurred during at least 8 years of follow-up.
背景
我们报告了1例患有科芬-洛里综合征（CLS）的患者在减压治疗颈椎脊髓病后8年的随访情况。CLS是一种罕见的X连锁半显性综合征，与生长发育和精神运动发育迟缓、全身肌张力减退及骨骼异常有关。在该患者中，脊髓被颈椎黄韧带（YL）中的焦磷酸钙晶体沉积压迫。迄今为止，仅有1篇报告描述了CLS患者钙化颈椎YL手术后的临床特征。
方法
一名15岁男性，因C1后弓发育不全及C2至C7的YL钙化导致颈椎脊髓受压继发四肢瘫痪，接受了C1至C7椎板切除术。报告了患者的病史、临床检查、影像学检查结果及治疗情况。该患者因智力发育迟缓无法言语，所以无法描述自身症状。在入院前2个月，步态障碍加重。入院时，患者四肢无法活动，上下肢腱反射明显亢进。颈椎计算机断层扫描显示C2至C7的YL钙化。磁共振成像显示颈椎脊髓连续受压。我们诊断为颈椎脊髓损伤继发四肢瘫痪并进行了急诊手术。
结果
在C1 - C7椎板切除术中，观察到C2 - C7的YL钙化。通过晶体分析证实钙化物质为焦磷酸钙。四肢瘫痪逐渐缓解，术后2周时几乎消失。术后1个月开始的X线片显示颈椎前凸，但未进展且未出现任何症状。
结论
减压治疗效果持续存在，在至少8年的随访期间未发生术后并发症。
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