CAPS original papersDiaphragmatic pacing for the treatment of congenital central alveolar hypoventilation syndrome
Section snippets
Materials and methods
The records of the 6 patients who are in the diaphragmatic pacing program at the Montreal Children's Hospital, Montreal, QC, Canada were reviewed. In addition to demographic details, associated anomalies were noted. Data regarding age at surgery, technical approach, and short- and long-term complications, including equipment failure and replacement, were recorded. All 6 patients had primary CCAHS diagnosed during the first few weeks of life. The assessment included neurologic evaluation and
Results
Of the 6 patients studied, there were 4 females and 2 males (Table 1). The age at insertion of the pacer was between 1.5 and 8 years (mean = 4 years) (Fig. 2).
Pacing was attempted 1 week after the surgical procedure in all patients. In none of the cases did primary nonfunction of a pacemaker occur.
After the initial recovery, patients start a phase of diaphragmatic conditioning, which involves progressive increments in the length of time the phrenic nerve is paced. Diaphragmatic conditioning is
Discussion
Congenital central alveolar hypoventilation syndrome is a rare respiratory disorder characterized by varying degrees of abnormal ventilation when the patient is awake and a completely insufficient response to hypoxia and hypercapnia during sleep, regardless of the absence of pulmonary or neuromuscular disease.
The cause of this disease results from failure of autonomic control of ventilation. The region of primary involvement is probably the central neurons of the medulla, especially the carbon
References (15)
- et al.
Hirschsprung's disease associated with Ondine's curse: report of three cases and review of the literature
J Pediatr Surg
(1994) - et al.
In pursuit (and discovery) of a genetic basis for congenital central hypoventilation syndrome
Resp Phys Neuro
(2005) - et al.
Diaphragmatic pacing in children with congenital central hypoventilation syndrome
Surgery
(1995) Congenital central hypoventilation syndrome: an update
Pediatr Pulmonal
(1998)The neurocristopathy
Hum Pathol
(1974)- et al.
Congenital central hypoventilation syndrome and Hirschsprung's disease
Arch Dis Child
(1998) - et al.
Central hypoventilation syndrome associated with ganglioneuroblastoma
Eur J Pediatr Surg
(1995)
Cited by (0)
Presented at the 39th Annual Meeting of the Canadian Association of Pediatric Surgeons, August 23-26, 2007, St John's Newfoundland, Canada.