From the Department of Anesthesiology, University of Iowa, Iowa City, Iowa.
A A Pract. 2022 Jun 20;16(6):e01600. doi: 10.1213/XAA.0000000000001600. eCollection 2022 Jun 1.
Malan syndrome, also known as Sotos-like syndrome or Sotos syndrome 2, is an overgrowth disorder that results from point mutations of the nuclear factor I/X (NFIX) gene. The phenotype consists of macrocephaly, facial dysmorphisms, hypotonia, and developmental delay. While case reports in the anesthesiology literature regarding the care of patients with Sotos syndrome do not identify significant anesthetic problems, more knowledge concerning the care of patients with Malan syndrome is necessary. Anesthesia providers must be aware of this clinical phenotype and prepare for possible difficult intravenous access and difficult airway management.
Malan 综合征，又称 Sotos 样综合征或 Sotos 综合征 2，是一种由核因子 I/X（NFIX）基因突变引起的过度生长障碍。表型包括大头畸形、面部畸形、低张力和发育迟缓。尽管麻醉学文献中关于 Sotos 综合征患者护理的病例报告并未确定明显的麻醉问题，但需要更多关于 Malan 综合征患者护理的知识。麻醉提供者必须了解这种临床表型，并为可能出现的困难静脉通路和困难气道管理做好准备。
