Puri S, Yaddanapudi S, Menon P
Department of Anaesthesia and Intensive Care Postgraduate Institute of Medical Education and Research (PGIMER) Chandigarh India.
Department of Paediatric Surgery Postgraduate Institute of Medical Education and Research (PGIMER) Chandigarh India.
Anaesth Rep. 2022 Jun 17;10(1):e12172. doi: 10.1002/anr3.12172. eCollection 2022 Jan-Jun.
A nine-year-old girl diagnosed with ROHHAD-NET (rapid-onset obesity, hypothalamic dysfunction, hypoventilation, autonomic dysfunction and neuroendocrine tumour) syndrome was scheduled for excision of a large paravertebral ganglioneuroma under general anaesthesia. Her comorbidities included hypothyroidism, diabetes insipidus and autonomic dysfunction. Intra- and postoperative complications included intra-operative hypotension, long surgical time and prolonged postoperative ventilation. Complete weaning from ventilation was initially unsuccessful and she was ultimately discharged on domiciliary nasal BiPAP therapy. The peri-operative care of children with this syndrome is challenging due to the involvement of multiple organ systems. In this report, we describe how pre-operative optimisation, well-planned intra-operative management and intensive postoperative care are essential for a favourable outcome.
一名九岁女孩被诊断为ROHHAD-NET（快速发作性肥胖、下丘脑功能障碍、通气不足、自主神经功能障碍和神经内分泌肿瘤）综合征，计划在全身麻醉下切除一个大型椎旁神经节瘤。她的合并症包括甲状腺功能减退、尿崩症和自主神经功能障碍。术中和术后并发症包括术中低血压、手术时间长和术后通气时间延长。最初尝试完全脱机失败，最终她在家中接受鼻罩双水平气道正压通气（BiPAP）治疗后出院。由于该综合征涉及多个器官系统，对患有这种综合征的儿童进行围手术期护理具有挑战性。在本报告中，我们描述了术前优化、精心规划的术中管理和强化的术后护理对于取得良好结果至关重要。
