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An unusual cause of diaphragm pacer failure in congenital central hypoventilation syndrome
- Source :
- J Clin Sleep Med
- Publication Year :
- 2022
- Publisher :
- American Academy of Sleep Medicine (AASM), 2022.
-
Abstract
- Congenital central hypoventilation syndrome is a rare genetic disorder affecting ventilatory response to hypercapnia and/or hypoxemia. We describe a case of diaphragm pacing (DP) failure in a 38-year-old woman with congenital central hypoventilation syndrome who used DP as ventilatory support only during sleep for 24 years. Diagnostic evaluation began with examination of external DP equipment, but adjustment did not elicit adequate diaphragm contractions. Clinical evaluation and transtelephonic monitoring showed absent function of the right pacer and diminished function of the left pacer. The patient had surgical exploration of her internal DP components. The operation revealed that the right pacer receiver had significant circumferential calcium accumulation. After replacement of the receivers in subcutaneous pockets closer to the skin surface, robust diaphragm contractions bilaterally occurred with stimulation. This case suggests DP failure can result from development of calcification and increased distance from the skin surface to the receivers due to weight gain. CITATION: Kwon A, Lodge M, McComb JG, et al. An unusual cause of diaphragm pacer failure in congenital central hypoventilation syndrome. J Clin Sleep Med. 2022;18(3):949–952.
- Subjects :
- Pulmonary and Respiratory Medicine
medicine.medical_specialty
business.industry
Case Reports
Congenital central hypoventilation syndrome
Diagnostic evaluation
medicine.disease
Diaphragm (structural system)
Hypoxemia
Diaphragm pacing
Neurology
Internal medicine
medicine
Cardiology
Neurology (clinical)
medicine.symptom
Complication
business
Hypercapnia
Calcification
Subjects
Details
- ISSN :
- 15509397 and 15509389
- Volume :
- 18
- Database :
- OpenAIRE
- Journal :
- Journal of Clinical Sleep Medicine
- Accession number :
- edsair.doi.dedup.....07a54507a37b2440397eac1bb9266481