Chamberland Mathieu, Poulin Marc-Antoine, Beaudoin Danielle
Hôpital de l'Enfant-Jésus, Québec (QC) G1J 1Z4 1401, 18 rue, Canada.
Case Rep Otolaryngol. 2021 Nov 19;2021:1315117. doi: 10.1155/2021/1315117. eCollection 2021.
Triple "A" syndrome (TAS) is a rare autosomal recessive disorder that presents in childhood with achalasia cardia, alacrima, ACTH-resistant adrenal insufficiency, with sensorimotor and autonomic polyneuropathy developing later in the course of the disease. . An adult white male affected by this syndrome underwent an uneventful total thyroidectomy for malignancy and suffered delayed bilateral recurrent laryngeal nerve palsy in the early postoperative hours. The palsy spontaneously resolved after a five-week course.
Given the rarity of this severe condition and the absence of surgical or medical causes identifiable, there is possibility that it is the neurological involvement caused by TAS that predisposed the patient to this adverse outcome, precipitated by standard manipulations during surgery.
“AAA”综合征(TAS)是一种罕见的常染色体隐性疾病,儿童期表现为贲门失弛缓症、无泪症、促肾上腺皮质激素抵抗性肾上腺功能不全,在疾病后期会出现感觉运动和自主神经多神经病。一名患有该综合征的成年白人男性因恶性肿瘤接受了顺利的全甲状腺切除术,并在术后早期出现延迟性双侧喉返神经麻痹。该麻痹在经过五周的病程后自行缓解。
鉴于这种严重疾病的罕见性以及无法确定手术或医学原因,有可能是TAS引起的神经受累使患者易出现这一不良结局,手术中的标准操作促使了该情况的发生。