Abstract:
To report an instructive case involving destructive spondylitis and synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome, presenting with torticollis and postoperative dysphagia without hoarseness, attributed to hidden myotonic dystrophy (DM). A 51-year-old male patient with a cervical deformity, who was previously managed conservatively for a metastatic tumor, underwent reconstruction surgery and subsequently experienced postoperative dysphagia. The presence of destructive spondylitis with torticollis, warranting prompt assessment to prevent paralysis, adds complexity to the delayed identification of DM. Given the rarity of DM, peculiar neurological symptoms and other systemic comorbidities did not lead to a preoperative diagnosis without prior knowledge. The patient\'s dysphagia induced respiratory arrest and required reintubation. Challenges in extubation and ventilator weaning arose due to hypercapnia. Superimposed COVID-19 infection elongated the duration of intubation. Extubation failed due to aspiration pneumonia and required a tracheotomy. Despite laryngeal elevation and preservation of the relaxation of the oesophageal entrance, the sensation and movement of the tracheopharynx were disturbed. The patient exhibited an oropharyngeal propulsive disorder, predominantly indicative of motor neuron disease. The patient\'s mother stated that his brother had been hospitalized for a long time after abdominal surgery. Finally, the patient was diagnosed with DM, which is known to cause post-anesthetic dysphagia. Recognizing the existence of severe destructive cervical spondylitis associated with SAPHO is crucial. Although DM is not very common, it is not classified as extremely rare. Therefore, surgeons should be mindful of the potential risks associated with general anesthesia in patients with DM. The complexity of preoperative conditions may hinder an accurate diagnosis. Recognizing and establishing preoperative expectations can assist surgeons in preventing complications, even if complex spinal surgery is required for patients with DM.
摘要:
报告一例涉及破坏性脊柱炎和滑膜炎-痤疮-脓疱病-骨肥大-骨炎(SAPHO)综合征的指导性病例,表现为斜颈和术后吞咽困难,但没有声音嘶哑,归因于隐性肌强直性营养不良(DM)。一名患有颈椎畸形的51岁男性患者,以前曾保守治疗转移性肿瘤,接受了重建手术,随后经历了术后吞咽困难。伴有斜颈的破坏性脊柱炎,保证及时评估以防止瘫痪,增加了DM的延迟识别的复杂性。鉴于DM的稀有性,特殊的神经系统症状和其他系统合并症并没有导致在没有事先知识的情况下进行术前诊断.患者吞咽困难导致呼吸停止,需要重新插管。由于高碳酸血症,拔管和撤机的挑战出现了。叠加的COVID-19感染延长了插管的持续时间。由于吸入性肺炎,拔管失败,需要进行气管切开术。尽管喉部抬高并保留了食道入口的松弛,气管咽部的感觉和运动受到干扰。患者表现为口咽推进障碍,主要指示运动神经元疾病。病人的母亲说,他的兄弟在腹部手术后已经住院很长时间了。最后,病人被诊断为DM,已知会导致麻醉后吞咽困难。认识到与SAPHO相关的严重破坏性颈椎病的存在至关重要。虽然DM不是很常见,它没有被归类为极其罕见。因此,外科医生应注意DM患者全身麻醉的潜在风险.术前条件的复杂性可能会阻碍准确的诊断。认识和建立术前期望可以帮助外科医生预防并发症。即使DM患者需要进行复杂的脊柱手术。