PDF(Pubmed)
Abstract：
Severe congenital neutropenia (SCN), commonly known as the Kostmann syndrome, is a rare and complex set of disorders defined by a lack of neutrophil maturation in the bone marrow, leading to life-threatening complications. This case report discusses a young adult patient scheduled for elective laparoscopic cholecystectomy. The patient presented with skin lesions which are a common scenario of Kostmann syndrome, but along with that, our patient posed challenges of short neck, limited neck extension, and gynecomastia. These additional conditions dramatically increased the challenges for anesthesiologists to address the anticipated difficult airway. The anticipated difficult airway challenges were handled by following the protocols of difficult airway guidelines 2022.
摘要：
严重的先天性中性粒细胞减少症（SCN），通常被称为Kostmann综合征,是一组罕见且复杂的疾病，定义为骨髓中中性粒细胞缺乏成熟，导致危及生命的并发症.此病例报告讨论了一名计划进行选择性腹腔镜胆囊切除术的年轻成年患者。患者出现皮肤病变，这是Kostmann综合征的常见情况，但与此同时,我们的病人提出了脖子短的挑战，有限的颈部延伸，和男性乳房发育症。这些额外的条件大大增加了麻醉医师解决预期的困难气道的挑战。通过遵循困难气道指南2022的方案来处理预期的困难气道挑战。
