Development of sleep-related breathing disorders at 8 years of age in a myelomeningocele patient

Abstract

  Myelomeningocele (MMC) is the most severe form of spina bifida that presents a variety of symptoms based on the spinal cord lesions of MMC, hydrocephalus and Chiari II malformation. Management of respiratory complications is important for prolonging the life expectancy of patients with MMC during childhood. Recent studies indicate a high prevalence of sleep-related breathing disorders (SRBD) in MMC patients. We encountered an 8-year-old girl with MMC located in the thoracic to sacral spine, who underwent neurosurgery at 6 and 7 years of age for treatment of Chiari II malformation. We under-recognized SRBD in MMC and did not perform routine respiratory evaluation. Respiratory evaluation was finally performed because of choking secondary to difficulty in expectoration and oxygen desaturation at 8 years of age. An interview and the results of a sleep questionnaire were insufficient to detect SRBD. A portable monitor showed loss of breathing effort during apnea, 3% oxygen desaturation index (ODI) of 70.9 and apnea hypopnea index (AHI) of 12, indicating that the patient had central sleep apnea syndrome. Non-invasive positive pressure ventilation therapy resulted in a 3%ODI of 1 and AHI of 1. Her respiratory status was stable thereafter. Routine evaluation of SRBD is important in the health management of MMC. Although there are comprehensive guidelines regarding SRBD in the care of people with spina bifida overseas, there is no such guideline in Japan. For adequate health care management, comprehensive guidelines for MMC are required in Japan as well.