Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Sleep Apnea 
Treatment: Durable Medical Equipment (respiratory assist device E04701 x 1, E0562 x 1) 
The insurer denied the Durable Medical Equipment (respiratory assist device E04701 x 1, E0562 x 1).
The denial is overturned. 

The patient is a female child with medical history significant for macrocephaly and megalencephaly with increased extra-axial fluid spaces and communicating hydrocephalus status post VP (Ventriculo-Peritoneal) shunt placement, history of obstructive sleep apnea status post adenoidectomy and tonsillectomy, now resolved following treatment with Zyrtec and Flonase. She is followed closely by Sleep Medicine for ongoing issues. 

The patient underwent a follow-up sleep study revealing significant central sleep apnea that had been present in some form previously, but was basically unmasked upon resolution of the obstructive sleep apnea. The study demonstrated 37 central respiratory events, yielding a central apnea index of 5/16 events per hour, occurring mostly during REM (rapid eye movement) sleep, associated with oxygen desaturation into the 80s. Her limb movement arousal index was also elevated at 6.8 events per hour, with a periodic limb movement index elevated at 10.8 events per hour. Sleep efficiency was low at 76.4% (percent). 

Based on this information, a trial of BiPAP (bi-level positive airway pressure) was recommended, with mask transition prior to dosing titration. Initial settings were anticipated empirically with pressures 8/4 and rate of 12. The overriding concern at this time is that the presence of central sleep apnea is related to megalencephaly and may be an indicator of Chiari malformation that would ultimately require surgical decompression. Radiographic evaluation has been recommended. 

Yes, the requested health service is medically necessary. 

Central sleep apnea is rare in children after one year of age when brain maturation, particularly of the premature infant population, has occurred, with the exception of children with the very rare disorder central hypoventilation syndrome or Ondine's curse. It is not clear why this particular child has central sleep apnea, but there is concern that it may be related to her megalencephaly and may be an indicator of possible Chiari malformation. The literature does include case studies of children with myelomeningocele and/or Chiari malformation in conjunction with central sleep apnea. 

Under discussion in this case is the appropriate management strategy. In general, continuous positive airway pressure (CPAP), in which continuous positive pressure is applied via an appliance such as a face mask during both inhalation and exhalation phases, is used for obstructive sleep apnea to effectively tent the airway open. In the case of central sleep apnea, where there may be periods of apnea, bi-level positive airway pressure (BiPAP) with a back-up rate is the treatment of choice because of the variable inhalation and exhalation pressures and the back-up rate that can engage should apneic pauses become excessive. The literature supports use of BiPAP (bi-level positive airway pressure) in children with central sleep apnea.