An unusual cause of diaphragm pacer failure in congenital central hypoventilation syndrome.
J Clin Sleep Med
; 18(3): 949-952, 2022 Mar 01.
Article
en En
| MEDLINE
| ID: mdl-34846295
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.
Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Terapia por Estimulación Eléctrica
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Apnea Central del Sueño
Tipo de estudio:
Diagnostic_studies
Límite:
Adult
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Female
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Humans
Idioma:
En
Revista:
J Clin Sleep Med
Año:
2022
Tipo del documento:
Article