Department of Pediatric Intensive Care and Anesthesia, Jichi Children's Medical Center Tochigi, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke-shi, Tochigi, 329-0498, Japan.
Department of Pediatric and Congenital Cardiovascular Surgery, Jichi Children's Medical Center Tochigi, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke-shi, Tochigi, 329-0498, Japan.
J Anesth. 2021 Apr;35(2):303-306. doi: 10.1007/s00540-020-02894-5. Epub 2021 Jan 15.
With the surgical improvement of congenital heart disease, Fontan operation has been applied to many complicated patients in recent years. This is the first report of a child with panhypopituitarism who underwent Fontan operation. A 5-year-old boy was scheduled for Fontan operation. He previously underwent Blalock-Taussig shunt and bidirectional Glenn operations for univentricular heart with double-outlet right ventricle and pulmonary atresia. He was receiving hydrocortisone and 1-desamino-8-D-arginine vasopressin (DDAVP) for panhypopituitarism secondary to removal of craniopharyngioma performed at the age of three years. Although urine output and serum sodium concentration were adequately controlled by adjustment of vasopressin infusion rate during surgery, massive pleural effusions and ascites developed postoperatively, which required several days for control by adjusting the dose of oral DDAVP and normalize the serum sodium level. Intraoperative management of Fontan operation for a patient with panhypopituitarism was controllable by appropriate hormone replacement. However, postoperative fluid management was complicated by the clinical features of panhypopituitarism and Fontan physiology.
随着先天性心脏病手术的进步，Fontan 手术近年来已应用于许多复杂的患者。这是首例报道的接受 Fontan 手术的全垂体功能减退症患儿。一名 5 岁男孩计划接受 Fontan 手术。他之前因右心室双出口和肺动脉闭锁行 Blalock-Taussig 分流术和双向 Glenn 手术。他因三岁时行颅咽管瘤切除术而继发全垂体功能减退症，目前正在接受氢化可的松和 1-脱氨-8-D-精氨酸加压素（DDAVP）治疗。尽管在手术期间通过调整加压素输注率来适当控制尿量和血清钠浓度，但术后仍出现大量胸腔积液和腹水，需要通过调整口服 DDAVP 的剂量和使血清钠水平正常化来数天才能控制。全垂体功能减退症患者的 Fontan 手术的术中管理可通过适当的激素替代来控制。然而，术后液体管理因全垂体功能减退症和 Fontan 生理学的临床特征而变得复杂。
