Cyanotic Breath-Holding Spell: A Life-Threatening Complication after Radical Resection of a Cervicomedullary Ganglioglioma

Abstract

Cyanotic breath-holding spell is a benign and self-limiting disease of young children but occasionally associated with sudden, unexpected death. The authors report a rare case in a 2-year-old girl with a severe form that started after radical resection of a cervicomedullary ganglioglioma. She was admitted to our hospital because of delayed and unstable gait. Since magnetic resonance imaging showed a cervicomedullary tumor, she underwent a radical resection and histology showed the tumor to be a ganglioglioma. Postoperatively, the function of the lower cranial nerves and cerebellum deteriorated and hemiparesis on the left became apparent, but she returned to the preoperative state in a few months. In addition, mild sleep apnea (Ondine curse) and severe cyanotic breath-holding spells occurred. The former responded to medication but the latter failed and continued several times per day with a rapid onset and progression of hypoxemia, loss of consciousness, sweating and opisthotonos. Five months after the operation, the patient returned home with a portable oxygen saturation monitor equipped with an alarm. This case indicates that cyanotic breath-holding spell, as well as sleep apnea, is critical during the early postoperative period. This is the first report observing that such spells may occur as a complication of radical resection of a cervicomedullary tumor.

Value of anti–modified citrullinated vimentin and third‐generation anti–cyclic citrullinated peptide compared with second‐generation anti–cyclic citrullinated peptide and rheumatoid factor in predicting disease outcome in undifferentiated arthritis and rheumatoid arthritis

“…MRI scans were obtained in 259 patients (64%). Data on 396 patients from the literature were obtained from 142 reports 1, 5‐145. Patients were followed until death or from 12 months to 408 months (median follow‐up, 36 months) for those who reportedly were alive at the last follow‐up.…”

Respiratory Health

“…There were recurring severe cyanotic breath-holding spells that continued several times per day with rapid onset and progression of hypoxemia, loss of consciousness, sweating, severe generalized and prolonged muscle contraction. In the beginning those spells were hard to control with medications, but 5 months after the surgery a portable monitor of oxygen saturation, equipped with an alarm in case of critical hypoxemia level helped the patient to recover to the extent that she could leave the hospital (Fujisawa et al 2005). Similar set of symptoms, also referred to as Ondine's curse, was described in an adult female patient admitted to the hospital for diagnostic of relapsing acute severe central hypoventilation, coexisting with trigeminal and glossopharyngeal neuralgia.…”

Sudden death in Lesch‐Nyhan disease

“…A related explanation involves centrally mediated expiratory apnea, also known as cyanotic breath-holding spells. 19,20 A mortality of 15.7% was reported in a study of 51 infants and young children with cyanotic breath-holding. 21 Impaired interactions between brainstem control of respiration and central sympathetic activity have been implicated in this disorder.…”