To the question of the genesis of respiratory disorders in sleep in children with syndromic craniosynostosis: a comparative analysis of the data of polygraphic research and computer morphometry

SUMMARY. Respiratory disorders during sleep in children with syndromic craniosynostosis: a comparative analysis of polygraphic data and computer morphometry.

Syndromic craniosynostosis (SCS) is a group of craniofacial malformations that includes the Cruson, Apert, Pfeiffer syndromes, and others, which are accompanied by premature fusion of cranial sutures or other craniofacial anomalies, including hypoplasia of the maxilla, cleft lip or palate, stenosis or choanal atresia, Chiari‑1 malformation, hydrocephalus, tracheobronchial dysplasia.

PURPOSE  of this work was to carry out a comparative analysis of the results of polysomnographic studies and morphometric data of patients with SCS to determine possible morphological predictors of the severity of respiratory disorders.

MATERIALS AND METHODS. A retrospective study was carried out in 43 patients (24 girls (56 %) and 19 boys (44 %) aged 40.55 ± 32.55 (3.6–135.9) months with various forms of SCS, who underwent instrumental assessment of respiratory function in the period from 2013 to 2021. All patients underwent examination and treatment in the conditions of the Federal Institution “National Medical Research Center of Neurosurgery named after Academician N.N. Burdenko. Syndromic diseases were represented by the following forms: 16 cases — Crouzon syndrome (23.2 %), Apert’s syndrome — 11 (25.6 %), Pfeiffer syndrome — 10 (23.3 %), and unspecified syndromes — 6 (13.9 %). Polysomnography (PSG) was performed in 32 patients (74.4 %), in 18 cases. (41.7 %) — computer monitoring pulse oximetry (CMP), among them in 7 cases. (16.3 %) both PSG and CMP were performed.

RESULTS. A moderate significant positive correlation (r = 0.4 p <0.01) was revealed between the values of the minimum saturation and the width of the choanas, i. e. the greater the choanal width, the higher the minimum saturation level. In addition, there was a negative correlation between the degree of obstructive sleep apnea syndrome (OSAS) and choanal height (r = 0.4 p <0.01). A negative correlation was found between the minimum saturation and the degree of dystopia of the cerebellar amygdala (r = 0.6), between the degree of dystopia of the cerebellar tonsils and the OSAS degree (r = 0.6). For patients with moderate and severe OSAS, a CT pattern of “interruption” of the air column at the choanal level is typical, as detected by 3D CT of reconstruction of the upper airways in 73 % cases, versus 16 % cases. (p <0.05) in patients with mild OSAS.

CONCLUSION. 1. Patients with syndromic forms of craniosynostosis are characterized by respiratory disorders, among which obstructive sleep apnea syndrome prevails. 2. Computer monitoring pulse oximetry during night sleep can serve as a method for screening respiratory disorders in patients with syndromic craniosynostosis. If signs of respiratory disorders are detected, patients need to conduct a polysomnographic study, which allows assessing the severity of violations and also clarifying their nature. 3. Despite the obvious relationship of morphological abnormalities detected in patients with syndromic craniosynostosis and respiratory disorders, only a correlation was found between the level of minimal saturation and choanal width. Respiratory disorders are determined by the summation of many factors of morphological abnormalities detected in this group of children. For a full clinical assessment of a patient, it is necessary, in addition to modern imaging diagnostics, to conduct a modern functional examination.

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