Abe Tatsuya, Seino Yutaka, Imai Hidekazu
Department of Anesthesiology, Niigata University Medical and Dental Hospital, Niigata, JPN.
Cureus. 2024 Aug 28;16(8):e68059. doi: 10.7759/cureus.68059. eCollection 2024 Aug.
Osteogenesis imperfecta (OI) is a congenital disease characterized by fractures and progressive bone deformities due to systemic bone fragility. A 12-month-old male infant diagnosed with OI type II, the most severe type, was scheduled for a tracheostomy. The patient presented with thoracic hypoplasia, which was treated with a high-flow nasal cannula, and a large skull owing to hydrocephalus with the head fixed in a left anteversion position. We encountered difficulties in mask ventilation during the rapid induction of general anesthesia. Oxygen saturation dropped temporarily, but the patient's condition stabilized after intubation. The tracheostomy was performed as scheduled and was completed without any complications. Difficulty in mask ventilation with low thoracic compliance due to thoracic hypoplasia, combined with air-induced gastric dilatation and upper airway obstruction, may have contributed to the ventilation failure. Most OI type II patients have large skulls owing to hydrocephalus and thoracic hypoplasia. Since no iatrogenic fractures related to airway management were observed in this or past cases, bone fragility may not be concerning to the extent that airway management becomes compromised. If patients present with poor oxygenation due to thoracic hypoplasia, the possibility of difficulty with mask ventilation due to low compliance should be considered in airway management.
成骨不全症(OI)是一种先天性疾病,其特征是由于全身性骨脆性导致骨折和进行性骨骼畸形。一名12个月大的男婴被诊断为II型OI,这是最严重的类型,计划进行气管造口术。该患者出现胸廓发育不全,采用高流量鼻导管进行治疗,并且由于脑积水导致头颅较大,头部固定在左前位。在全身麻醉快速诱导期间,我们在面罩通气方面遇到了困难。氧饱和度暂时下降,但患者在插管后病情稳定。气管造口术按计划进行,并且顺利完成,没有任何并发症。由于胸廓发育不全导致胸廓顺应性低,面罩通气困难,再加上气体引起的胃扩张和上呼吸道梗阻,可能导致了通气失败。大多数II型OI患者由于脑积水和胸廓发育不全而头颅较大。由于在本病例或以往病例中均未观察到与气道管理相关的医源性骨折,因此骨脆性可能不会严重到影响气道管理的程度。如果患者因胸廓发育不全而出现氧合不良,在气道管理中应考虑因顺应性低而导致面罩通气困难的可能性。