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Medicine (Baltimore). 2019 Apr;98(17):e15427. doi: 10.1097/MD.0000000000015427.

Massive abscess with prolonged respiratory failure due to newly diagnosed myotonic dystrophy: A case report.

Author information

1
Department of Emergency Medicine.
2
Division of Neurology, Department of Internal Medicine IV, Osaka Medical College, Osaka, Japan.

Abstract

RATIONALE:

Myotonic dystrophy is a progressive multisystem genetic heterogeneous disorder. General anesthesia with opioids increases the risk of prolonged postanesthetic respiratory recovery in myotonic dystrophy patients.

PATIENT CONCERNS:

A 20-year-old previously healthy woman was transferred to our emergency department for further workup of respiratory failure, and massive ascites with abscess caused by endometriosis. Hypercapnic respiratory failure persisted under intensive care unit (ICU) management, but finally improved after cessation of fentanyl as a sedative agent.

DIAGNOSIS:

Myotonic dystrophy type 1.

INTERVENTIONS:

Massive ascites with abscess was accordingly managed by drainage, antibiotics, and an antifungal agent. Myotonic dystrophy type 1 was confirmed after molecular genetic testing revealed a cytosine-thymine-guanine repeat length of 400 to 450 in the DMPK gene.

OUTCOMES:

The patient was discharged without complications on hospital day 69.

LESSONS:

Myotonic dystrophy should be considered when hypercapnic respiratory failure persists in sedated ICU patients. Opioids should not be used for perioperative management of patients with myotonic dystrophy.

PMID:
31027145
DOI:
10.1097/MD.0000000000015427
[Indexed for MEDLINE]
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