Mkochi Vincent L, Mkandawire Nyengo
Assistant lecturer and Postgraduate Trainee in Trauma and Orthopaedics, College of Medicine, University of Malawi.
Consultant Trauma and Orthopaedic Surgeon; Dean of Faculty of Medicine, College of Medicine, University of Malawi.
Malawi Med J. 2018 Jun;30(2):127-131. doi: 10.4314/mmj.v30i2.13.
Grisel syndrome is a non-traumatic rotary subluxation of C1 on C2 (atlantoaxial subluxation). It is a rare condition predominantly described in paediatric population with previous history of upper respiratory infections orotolaryngeal procedures. The diagnosis is established by the association of clinical and radiologic findings4,6.We report a case of 15-year old boy with Grisel syndrome accompanying multifocal sepsis (pericarditis, septic polyarthritis, pneumonia and pharangytis ), treated surgically - occipital cervical fusion using struts of iliac crest tri-cortical graft wired to the occiput and C3 and C4.
To describe a case of non-traumatic atlantoaxial rotatory subluxation (Grisel syndrome) and to review clinical and radiologic aspects, pathophysiology, and treatment of this rare condition, which if left untreated can lead to catastrophic outcomes. Authors are trying to demonstrate the significance of early intervention for improved outcomes, and that physicians should examine patients with multifocal sepsis thoroughly so as not to miss foci of infection and have high index of suspicion of the syndrome in patients that present with neck swelling, and develop torticollis and neurological deficit.
This is a case presentation of a 15-year-old boy who presented with an acute history of neck swelling, shortness of breath, orthopnoea, pericardial effusion, right hip and knee swelling. He later developed torticollis and left sided hemiplegia. On admission, he was septicemic. There was no history of head or neck trauma. Cervical spine radiograph showed increased atlantodental index (ADI) and prevertebral soft tissue swelling. Consent was sought from guardian and patient to use images and case notes for submission for publication.
The patient was treated with analgesia, drainage of pericardial effusion and intravenous antibiotics. The right knee sepsis was noted on admission and drained on the day pericardial drain was inserted, while the right hip was missed - only to be noted 59 days later with limb length discrepancy. The C1/C2 subluxation was noted 17 days after admission. Reduction was done soon after diagnosis and maintained with a halo-thoracic immobilizer. A week later, he underwent posterior occipital cervical fusion. The hemiplegia resolved fully.
Multifocal staphylococcal septicemia can present with unusual foci of infection, which may be missed especially if the patient is very sick. Grisel syndrome with an associated left hemiplegia and septic arthritis of the right hip were diagnosed late in this case. Grisel syndrome patients might end up with catastrophic outcomes if left untreated or mis-diagnosed, but if prompt treatment is initiated, full functional recovery is achievable.
格里斯尔综合征是C1相对于C2的非创伤性旋转半脱位（寰枢椎半脱位）。这是一种罕见病症，主要见于有上呼吸道感染或口咽手术史的儿科患者。诊断通过临床和影像学检查结果来确定。我们报告一例15岁男孩患格里斯尔综合征并伴有多灶性败血症（心包炎、脓毒性多关节炎、肺炎和咽炎），接受了手术治疗——采用带三面皮质的髂嵴骨块支撑物进行枕颈融合术，将支撑物用金属丝固定于枕骨以及C3和C4椎体。
描述一例非创伤性寰枢椎旋转半脱位（格里斯尔综合征）病例，并回顾这种罕见病症的临床和影像学表现、病理生理学及治疗方法。若不治疗，该病症可能导致灾难性后果。作者试图证明早期干预对改善预后的重要性，以及医生应全面检查多灶性败血症患者，以免漏诊感染病灶，对于出现颈部肿胀、斜颈及神经功能缺损的患者应高度怀疑该综合征。
这是一例15岁男孩的病例报告，其有颈部肿胀、呼吸急促、端坐呼吸、心包积液、右髋和膝关节肿胀的急性病史。随后出现斜颈和左侧偏瘫。入院时，他处于败血症状态。无头部或颈部外伤史。颈椎X线片显示寰齿间距（ADI）增大及椎前软组织肿胀。已征得监护人及患者同意使用图像和病例记录用于提交发表。
患者接受了镇痛、心包积液引流及静脉使用抗生素治疗。入院时发现右膝败血症，并在插入心包引流管当天进行了引流，而右髋部感染被漏诊——直到59天后出现肢体长度差异才被发现。入院17天后发现C1/C2半脱位。诊断后不久进行了复位，并使用头胸固定器维持固定。一周后，他接受了后路枕颈融合术。偏瘫完全恢复。
多灶性葡萄球菌败血症可能表现为不寻常的感染病灶，尤其是在患者病情严重时可能被漏诊。该病例中格里斯尔综合征伴发左侧偏瘫及右髋脓毒性关节炎诊断较晚。格里斯尔综合征患者若不治疗或误诊，最终可能导致灾难性后果，但如果及时治疗，可实现完全功能恢复。
