关键词: Ehlers–Danlos atlantoaxial instability collagenopathy congenital connective tissue surgery

来  源:   DOI:10.3389/fvets.2023.1234995   PDF(Pubmed)

Abstract:
Ehlers-Danlos syndrome is a rare, heritable connective tissue disorder characterized by soft, hyperextensible skin, joint hypermobility, and tissue fragility, the severity of which can range from mild to severe. A 9-month-old male entire miniature Dachshund was presented following peracute tetraparesis. Neurological examination was suggestive of intracranial vestibular disease or high cervical myelopathy. MRI revealed atlantoaxial instability and subluxation, resulting in marked spinal cord compression at C1-C2, which was surgically stabilized. On discharge from the hospital, skin fragility was noted as the result of skin tearing during tape removal. A piece of full-thickness antebrachial skin was submitted for histopathology which showed changes consistent with Ehlers-Danlos syndrome. This case report describes the first case of atlantoaxial instability and subluxation in a dog as the result of a confirmed underlying collagenopathy.
摘要:
Ehlers-Danlos综合征是一种罕见的,遗传性结缔组织疾病的特点是软,过度伸展的皮肤,关节过度活动,组织脆弱,其严重程度可从轻度到重度。急性四瘫后出现了一个9个月大的雄性完整的小型腊肠。神经系统检查考虑颅内前庭疾病或高颈脊髓病。MRI显示寰枢椎不稳定和半脱位,导致C1-C2处明显的脊髓压迫,手术稳定。出院后,皮肤脆性是胶带去除过程中皮肤撕裂的结果。提交了一块全层前臂皮肤进行组织病理学检查,其变化与Ehlers-Danlos综合征一致。该病例报告描述了狗的第一例寰枢椎不稳定和半脱位,这是确诊的潜在胶原病的结果。
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