Treatment of children with Pierre Robin Syndrome

Abstract

Introduction. Incidence of Pierre Robin sequence (PRS) according to different studies varies from 1:8500 to 1:30000 newborns. Swallowing dysfunction may lead to aspiration of food, saliva and gastric content, resulting in severe aspirational pneumonia. Obstructive sleep apnea (OSA) may be a life threatening condition for children with PRS and may cause death by asphyxia. Average mortality rate for PRS according to different studies is approximately 16 % and varies depending on several factors: number of patients in studies, severity of the condition, diagnostic methodologies and presence of associated syndromes in maxillofacial region. Mortality rate in severe PSR (especially associated PSR) is 41,4 %. Aside from high mortality, OSA contributes to the development of cardiovascular disease including pulmonary hypertension, which progresses more rapidly than in adults. OSA exacerbates existing comorbidities; in the absence of timely treatment sleep apnea leads to learning impairment and behavioral problems, developmental delay, failure to thrive. Methods. 80 children with PSR aged between 4 days and 1 year have undergone compression-distraction osteosynthesis (CDO). 48,8 % (39) of children were diagnosed with severe degree of OSAS, 35 % (28) had moderate degree, 16,3 % (13) had mild degree. Pretreatment diagnostics included standard set of examinations and specialist consultations. Special diagnostics included CT of head and neck, fiberoptic nasopharyngoscopy, examination by ENT specialist for the assessment of the degree of upper airways obstruction and exclusion of pathologies and anomalies of nasal cavity, pharynx, larynx and trachea, which also may be the cause for reduced airflow and upper airway obstruction (intubated children haven’t undergone these examinations). Photographs were taken and videos of clinical manifestations of airway obstruction during sleep were recorded. Blood gas tests were regularly taken; blood oxygenation levels were measured with a pulse oximeter. Polysomnography was performed, which allows for accurate and comprehensive evaluation of severity of OSA based on multiple parameters. Extra-cortical and external compression-distraction devices (CONMET, KLS Martin, Synthes) were used. Results. Good outcome was achieved in 90 % (72), satisfactory in 6,3 % (5) and negative in 3,8 % (3) of cases.