Polysomnography and Spina Bifida: assessing viability of home sleep study
Summary
Sleep related breathing disorders (SRBD), including central and obstructive apnea, are known to be more common in children with spina bifida (SB) and Chiari II malformation (CM-2).1-4 Diagnosis can be challenging and costly with in-house polysomnography (PSG). Though there is data to suggest an increased incidence of SRBD in the SB population, there is not much data about sleep quality in children with CM-2 and SB. In our clinical experience, we note that these children have disturbed sleep, which may be related in part to disordered breathing or low ferritin levels or brainstem abnormalities. Poor sleep in this population has been theorized to contribute to sudden death in young adult patients with spina bifida, and more commonly contributes to cognitive dysfunction. 5 Management of SRBD in SB population requires a multidisciplinary team, including Physical Medicine & Rehabilitation, Otolaryngology, Pulmonlogy, and Neurosurgery. We hypothesize that with appropriate systematic and early management of SRBD in SB, we will decrease morbidity and mortality as well as establish parameters for early intervention.
Our first aim is to provide standardized care to all children diagnosed with SB at Arkansas Children’s. Under this protocol, we will use a questionnaire-based evaluation of sleep quality, a sleep study (home and formal polysomnography) and formal pulmonary and otolaryngology evaluations. We expect to consequently identify sleep disturbances in a timelier manner. Secondarily, we aim to determine the efficacy of home sleep studies in diagnosing sleep disorders in this population, which will provide an opportunity to innovate and engineer home based PSG to better meet the needs of developmentally delayed children who may require them throughout the course of their lives.
The Spinal Cord Disorders Program serves approximately 300 patients throughout the state of Arkansas. Records of these patients will be reviewed to determine if they meet inclusion criteria. During routine clinic visit, subject’s upper airway will be assessed by ENT using a flexible scope. A polysomnogram will be scheduled, along with a home sleep study within 1 month of completion of formal PSG. No surgical interventions (other than urgent interventions) will be completed in this 30-day interval. During their routine Spinal Cord Disorders visit, questionnaires regarding sleep quality and sleep related health quality will be obtained. Once these studies are performed, depending on the findings, clinical management of the diagnoses will be pursued as per current standards of practice. If any surgical intervention is undertaken, the patient will undergo repeat PSG as indicated to evaluate for residual abnormalities.
All children (0-21 years) diagnosed with CM-2 or SB will be included in this protocol. We will maintain a database of all these patients and may perform a retrospective/ prospective chart review to contribute towards quality improvement in the management of these patients. The database will include all information including age, race, sex, height, weight, symptoms, MRI brain results, questionnaire scores, sleep study results, ENT clinic findings, swallowing study results, surgeries performed, dates of all procedures, sleep clinic visits if performed, and interventions at the clinic visits.
Keywords:
- Spina Bifida
- Polysomnography
- Abnormal sleep
- Chiari Malformation