Pediatrik Nöroloji Bilim Dalı İzleminde Olan ve Polisomnografi İncelemesi Yapılan Hastaların Retrospektif Olarak Değerlendirilmesi

Abstract

Cebeci K., Retrospective Evaluation of Polysomnographic Features in Children with Chronic Neurological Disorders and Multiple Disabilities. Hacettepe University Faculty of Medicine, Thesis in Pediatrics, Ankara-2020. Sleep-related breathing disorders have numerous negative outcomes on physiological, behavioral and neurocognitive development even in healthy children. On the other hand, there is an increased risk of sleep-related breathing disorders in patients who have neurological disabilities with medical complexity. The aim of our study was to evaluate characteristics of patients with a follow-up at Hacettepe University İhsan Doğramacı Children’s Hospital Department of Pediatric Neurology who had been through inpatient polysomnography (PSG) studies to document a) spectrum of sleep-related breathing disorders, b) characteristics of sleep parameters, c) determinations of risk factors, and d) the yield of PSG on patient management. Between 1 May 2015- 31 October 2019, a total of 137 patients were included. Mean age was 8 years (1,5 month-18,5 years), and boy: girl ratio was 1:1. According to the primary disease, the population was evaluated in 8 subgroups; 41 patients (30%) neuromuscular diseases, 32 patients (23%) syndromes, 16 patients ( 12%) cerebral palsy, 14 patients (10%) chromosomal diseases, 12 patients (9%) epilepsy, 9 patients (6%) others, 8 patients (5%) central nervous system malformations, 5 patients (4%) neurodevelopmental diseases. A total of 215 PSG studies were done; 77 patients had one, 138 patients had more than one PSG evaluation. Forty-three (64%) patients had signs and symptoms related to sleep-related disorder before PSG study. Most common symptoms were noted as snoring/ open mouth sleeping (n=43) and respiratory pause/ frequent awaking during night (n=33). Sleep apnea was present in 83% (n=110) of patients, the most of them being severe sleep apnea (54%, n=59). Patients mostly belong to syndromes (26%, n=29) and neuromuscular diseases (26%, n=29) groups. Comparison of PSG parameters revealed that, sleep efficiency (median: 52%, min: 25.1%, max: 93,9%) and desaturation index (median: 1,55, min: 0, max: 95) medians were the lowest in cerebral palsy group; apnea-hypopnea index (median: 12,4, min: 0,2, max: 62,1) median was the highest in syndromes; central apnea (median: 16, min: 1, max: 235) median was the highest in neurodevelopmental diseases group. Patients on respiratory support device (oxygen, CPAP, BIPAP or invazive mechanical ventilation support via tracheostomy) before PSG were 15% (n=21) of all patients. On the other hand, 54 patients (39%) required respiratory support for the first time after PSG study. Although mean age and distribution of gender were similar in these patients, the patients who had a change in respiratory management were diagnosed earlier (p<0.001). Children with cerebral palsy, central nervous system malformations and neurodevelopmental diseases required more respiratory support compared to other groups (p=0.01). Patients referred from the Department of Ear-Nose and Throat and Pediatric Inpatient Services mostly required respiratory support device (p=0.001). In this context, only 46.7% of patients who required respiratory support were referred from Pediatric Neurology outpatient clinic. All patients who had hypercarbia/ hypoventilation had been introduced respiratory support device and there were no patients on respiratory support device who presented with daytime sleepiness (p=0.003). There was no statistically significant difference in terms of accompanying scoliosis, chest deformity, respiratory function tests, primary disease activity, presence of craniofacial dysmorphic features, to be on physical therapy and rehabilitation, bedridden, or followed by nutrition and dietetics (p<0.05). Nevertheless, respiratory support devices were also used in patients with scoliosis (56.8% vs 43.2%), who were bedridden (69.2% vs %30.8) and had craniofacial dysmorphic features (54% vs 46%) after the PSG study(p>0.05). Despite limitations and retrospective nature of the study, inpatient PSG studies provide invaluable information to guide patient management in children with complex medical and neurological disorders. Awareness of sleep-related disorders should be increased at the Pediatric Neurology clinics.