Case Report Anesthetic management of a myotonic dystrophy patient with paraganglionoma ☆ Ashwin Subramaniam MBBS, Master of Medicine, FRACP, FCICM (Adjunct Lecturer) a,b , Robert Grauer MBBS, FANZCA c , David Beilby MBBS, FANZCA c , Ravindranath Tiruvoipati MBBS, MS, MCh, FRCSEd, MSc, FCICM, EDIC (Associate Professor) a,b, ⁎ a Monash University, Clayton, Victoria, Australia b Frankston Hospital, Peninsula Health, Frankston, Victoria, Australia c Box Hill Hospital, Eastern Health, Box Hill, Victoria, Australia Received 12 January 2015; revised 10 March 2016; accepted 10 March 2016 Keywords: Myotonic dystrophy; dystrophia myotonica; pheochromocytoma; paraganglionoma; general anesthesia; hypertension Abstract Myotonic dystrophy (DM), though rare, can significantly complicate anesthesia due to muscular and extra-muscular involvement. When this condition is compounded by a pheochromocy- toma, anesthetizing such patients becomes extra challenging. We present a case report of a 61-year-old lady with congenital DM, with the whole gamut of associated features, was diagnosed with a noradrenaline secreting paraganglionoma following investigation of refractory hypertension. We anesthetized her for an open resection of the lesion. The conduct of anesthesia and recovery of this patient is described. Our experience suggests that anesthetizing these patients though challenging can be safely managed with relaxant general anesthesia and epidural analgesia with meticulous care pre, intra and post-surgical intervention. Crown Copyright © 2016 Published by Elsevier Inc. All rights reserved. 1. Introduction Much attention is required to manage myotonic dystrophy (DM) for anesthesia and surgery. DM is associated with prolonged duration of action of drugs used during anesthesia and may also cause dangerous interactions such as severe arrhythmia, significantly limiting the choice of anesthetic and adjuvant drugs. At the same time, management of blood pressure (BP) and heart rate (HR) during pheochromocytoma resection requires meticulous care as severe hypertension and tachycardia can result in catastrophic complications [1]. Although there are quite a few publications on the anesthetic management of individual conditions, we could only find one case report in the Japanese literature where the anesthetic management for the combination of DM and pheochromocytoma was described [2]. Differences in technique are summarized in Table 1. The conduct of anesthesia and recovery of this patient is described. 2. Case history A 61-year-old lady was diagnosed with pheochromocy- toma when investigated for uncontrolled hypertension over a ☆ Conflict of interest: None of the authors have commercial association or financial involvement that might pose a conflict of interest in connection with this article. ⁎ Corresponding author at: Department of Intensive Care Medicine, Frankston Hospital, Frankston, Victoria 3199, Australia. E-mail address: travindranath@hotmail.com (R. Tiruvoipati). http://dx.doi.org/10.1016/j.jclinane.2016.03.035 0952-8180/Crown Copyright © 2016 Published by Elsevier Inc. All rights reserved. Journal of Clinical Anesthesia (2016) 34, 21–28