Abstract
Patients with congenital central hypoventilation syndrome (CCHS) develop alveolar hypoventilation resulting from a failure of central ventilatory control. Late-onset CCHS (LO-CCHS), which may be precipitated by severe respiratory infection or exposure to sedatives or general anesthesia, presents after the neonatal period. Since CCHS patients require lifelong mechanical-assisted ventilation, in western countries, diaphragm pacing is used to provide adequate alveolar ventilation and oxygenation during rest and daily activities. The main anesthesia-related concern regarding CCHS is postoperative respiratory failure or apnea, and anesthetic agents should be minimized to avoid further respiratory depression after surgery. A 5-year-old girl with LO-CCHS was referred to our hospital for implantation of a phrenic nerve stimulator for diaphragm pacing. Respiratory infection triggered the need for permanent nocturnal ventilator support at age 3 years and tracheotomy was performed at age 4 years. Repeated self-dislodgement of the ventilator tube led to hypoxic ischemic encephalopathy. The patient was thought to require mechanical ventilation under minimum sedation and pain management during the early postoperative period. The co-administration of dexmedetomidine and morphine provided effective conscious sedation with protection of the surgical site and without adverse events. She was discharged from the intensive care unit with a home ventilator at 3 days post-operation.
Highlights
Congenital central hypoventilation syndrome (CCHS) is characterized by the lack of adequate autonomic control of respiration with decreased sensitivity to hypercapnia and hypoxia in the absence of neuromuscular or lung disease or an identifiable brainstem lesion [1]
In the latest Japanese survey, 22 of 37 CCHS patients survived without further disability, 8 survived with disability, and 7 died [2]
There are no data on the prevalence of late-onset CCHS (LO-CCHS), which refers to patients who are diagnosed with CCHS after 28 days of age [3]
Summary
Congenital central hypoventilation syndrome (CCHS) is characterized by the lack of adequate autonomic control of respiration with decreased sensitivity to hypercapnia and hypoxia in the absence of neuromuscular or lung disease or an identifiable brainstem lesion [1]. Pre-operative course A 5-year-old, 17-kg girl, who had been diagnosed elsewhere as LO-CCHS and who had required nocturnal PPV through a tracheostomy since the age of 4 years, was referred to our hospital for implantation of a phrenic nerve stimulator for diaphragm pacing. She was born at 40-week gestation and weighed 3170 g without fetal distress. Tidal volume of 120 mL and a respiratory rate of 14 breaths/min was adopted to achieve an EtCO2 level over 45 mmHg. The operative procedure was based on a previous report of diaphragm pacing by stimulation of the phrenic nerve with a spinal cord stimulator in patients with central hypoventilation syndrome [8]. Because the cost of the device is not reimbursed by the health care insurance system in Japan, all medical expenses were covered by the patient’s family
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