Breathing pacemakers: state-of-the-art

Forty patients, 25 males and15females, and 2-69 years [mean 26 years] with respiratory paralysis due to high cervical cord lesions, brain stem lesions, central hypoventilation as well as chronic hiccup, received phrenic nerve stimulators for pacing of the diaphragm. At a mean follow-uptime of 65 months [range 1 months to 12 years] 18 patients are entirely independent of mechanical respirators pacing round-the-clock, whereas 5 quadriplegics ventilate with pacers at different daytime intervals and use conventional ventilators during the night. Eight patient, including the 2 hiccup cases, have stopped pacing and 9 additional cases have died. Recent improvements of the pacemaker system with contractions. The implantation of breathing pacemakers has pronged life and improves the quality of life in the majority of the patients

Sleep apnea with intestinal aganglionosis: report of three cases; therapeutic considerations

Hirschsprung's disease can be associated with other congenital abnormalities, some of which are neural in origin. A rare association is with congenital failure of automatic control of respiration-central hypoventilation syndrome, sleep apnoea or Ondine's curse. Patients with this combination tend to have a short life expectancy. Diaphragmatic pacing by electrophrenic stimulation has proven useful in the management of patients with central hypoventilation. Three children, two females and one male, with this combination are described. The male child, who had total intestinal aganglionosis, died at the age of one month despite an ileostomy and nocturnal mechanical ventilation. The two females had aganglionosis more typical of Hirschsprung's disease, one requiring colostomy. At the ages of two and six years, respectively, phrenic nerve stimulators were implanted. Both girls remained independent of nocturnal mechanical ventilation two and three years after the commencement of diaphragm pacing. In patients with Ondine's curse and Hirschsprung's disease, in whom the aganglionosis can be effectively managed, diaphragm pacing may lead to independence from mechanical ventilation and prolonged life with an acceptable quality