Geetha Sindhu, Verma Neeta, Singam Amol
Anaesthesiology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND.
Cureus. 2024 Jan 2;16(1):e51504. doi: 10.7759/cureus.51504. eCollection 2024 Jan.
Ectodermal dysplasia, a heterogeneous group of rare genetic disorders, is characterized by the aberrant development of ectodermal structures, leading to various clinical anomalies. This case report presents a unique and challenging case of a 33-year-old male with ectodermal dysplasia who underwent Le Fort III advancement and implant rehabilitation surgery to address severe craniofacial and dental deficiencies. This case, characterized by facial dysmorphism, craniofacial anomalies, and the absence of a nasal bone, highlights the complexity of surgical planning required to address these diverse clinical features. The crucial element of this report is the innovative approach to airway management through trans mylohyoid/submental intubation, which successfully navigated the patient's aberrant anatomy. Multidisciplinary collaboration played a pivotal role in achieving a holistic and patient-centered approach. By sharing this case, we aim to provide insights into the nuances of managing complex patients with ectodermal dysplasia, emphasizing the importance of individualized care, innovative techniques, and interdisciplinary teamwork to optimize patient outcomes and contribute to advancing medical knowledge.
外胚层发育不全是一组罕见的遗传性疾病,具有异质性,其特征是外胚层结构发育异常,导致各种临床异常。本病例报告介绍了一名33岁患有外胚层发育不全的男性的独特且具有挑战性的病例,该患者接受了Le Fort III前徙术和种植体修复手术,以解决严重的颅面和牙齿缺陷。该病例的特征为面部畸形、颅面异常以及鼻骨缺失,突出了应对这些多样临床特征所需手术规划的复杂性。本报告的关键要素是通过经下颌舌骨肌/颏下插管进行气道管理的创新方法,该方法成功应对了患者异常的解剖结构。多学科协作在实现全面且以患者为中心的治疗方法中发挥了关键作用。通过分享此病例,我们旨在深入了解管理患有外胚层发育不全的复杂患者的细微差别,强调个性化护理、创新技术和跨学科团队合作对于优化患者治疗效果以及推动医学知识进步的重要性。