Anesthetic management of a myotonic dystrophy patient with paraganglionoma

Ashwin Subramaniam; Robert Grauer; David Beilby; Ravindranath Tiruvoipati

doi:10.1016/j.jclinane.2016.03.035

Anesthetic management of a myotonic dystrophy patient with paraganglionoma

J Clin Anesth. 2016 Nov:34:21-8. doi: 10.1016/j.jclinane.2016.03.035. Epub 2016 May 2.

Authors

Ashwin Subramaniam¹, Robert Grauer², David Beilby², Ravindranath Tiruvoipati³

Affiliations

¹ Monash University, Clayton, Victoria, Australia; Frankston Hospital, Peninsula Health, Frankston, Victoria, Australia.
² Box Hill Hospital, Eastern Health, Box Hill, Victoria, Australia.
³ Monash University, Clayton, Victoria, Australia; Frankston Hospital, Peninsula Health, Frankston, Victoria, Australia. Electronic address: travindranath@hotmail.com.

PMID: 27687340
DOI: 10.1016/j.jclinane.2016.03.035

Abstract

Myotonic dystrophy (DM), though rare, can significantly complicate anesthesia due to muscular and extra-muscular involvement. When this condition is compounded by a pheochromocytoma, 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.

Keywords: Myotonic dystrophy; dystrophia myotonica; general anesthesia; hypertension; paraganglionoma; pheochromocytoma.

Publication types

Case Reports

MeSH terms

Adrenal Gland Neoplasms / complications
Adrenal Gland Neoplasms / diagnosis
Adrenal Gland Neoplasms / surgery*
Adrenergic alpha-Antagonists / therapeutic use
Adrenergic beta-Antagonists / therapeutic use
Anesthesia, Epidural
Anesthesia, General / methods*
Anesthetics, Intravenous / administration & dosage
Anesthetics, Local / administration & dosage
Catecholamines / blood
Catecholamines / urine
Coronary Vasospasm / drug therapy*
Coronary Vasospasm / etiology
Coronary Vasospasm / urine
Female
Humans
Hypertension / drug therapy*
Hypertension / etiology
Hypertension / urine
Middle Aged
Myotonic Dystrophy / complications*
Paraganglioma / complications
Paraganglioma / diagnosis
Paraganglioma / surgery*
Perioperative Care*
Phenoxybenzamine / therapeutic use
Pheochromocytoma / complications
Pheochromocytoma / diagnosis
Pheochromocytoma / surgery*
Propranolol / therapeutic use
Tomography, X-Ray Computed

Substances

Adrenergic alpha-Antagonists
Adrenergic beta-Antagonists
Anesthetics, Intravenous
Anesthetics, Local
Catecholamines
Phenoxybenzamine
Propranolol

Supplementary concepts

Hypertension Resistant to Conventional Therapy