Neurophysiological brainstem investigations in isolated Pierre Robin sequence
Introduction
Pierre Robin Sequence (PRS) is characterized by glossoptosis, cleft palate, and retrognathia. Its origin remains obscure and is probably not single. PRS can be isolated, or associated with developmental defects. Careful evaluation and management of respiratory and feeding problems has led to an improvement in both morbidity and mortality [1], [2]. Neurophysiological investigations form part of the clinical evaluation. Here we report the results of a retrospective study on a series of newborns with an isolated form of PRS in whom polysomnography (PSG), electromyography (EMG), blink reflexes (BRs), and brainstem auditory evoked responses (BAERs) were recorded during the first two months. These results will form the basis for discussion of two main points: the contribution of neurophysiological examinations to therapeutic management, and the pathophysiology of respiratory and feeding disorders.
Section snippets
Patients
Patients were 25 full-term newborns with isolated PRS without any cardiac, skeletal, ocular or other disorder. They were hospitalized in the neonatal or maxillofacial surgery unit of our hospital. According to the severity of clinical feeding difficulties and respiratory disorders they were divided into three groups: (I) normal bottle-feeding and normal respiration, or minor difficulties: slow sucking, transient neonatal respiratory distress (7 cases); (II) dysphagia requiring tube feeding,
Polysomnography
The total duration of recording was 150.48±31.68 min and the duration of sleep 126.56±32.02 min. In all cases, the EEG showed the absence of any pathology and a maturation that corresponded to age, with a normal electrical and clinical organization of sleep stages. The number and duration of central respiratory pauses were normal for age in the 25 patients.
Obstructive or mixed apneas (OMA) were observed in 23/24 patients (the 25th having undergone tracheotomy). The baby who did not exhibit any
Discussion
These results observed in our series of newborns with an isolated form of PRS raise the question of the contribution of neurophysiological examinations to the evaluation and management of the condition, on one hand, and to the understanding of the pathophysiology of PRS, on the other.
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