Abstract
Pierre Robin sequence (PRS) may lead to life-threatening respiratory and feeding disorders. With the aim to analyse the association of the severities of retrognathia and glossoptosis with those of respiratory and feeding disorders, we retrospectively studied a series of 50 infants with retrognathia, glossoptosis, cleft palate, and airway obstruction. The patients were managed from birth to at least 6 years of age by a single pediatric team at the Armand Trousseau Hospital in Paris within a 12 years period (2000–2012). Retrognathia and glossoptosis were graded in the neonatal period according to a specific clinical examination. Ventilation assistance was required for 32/50 (64%) patients, and enteral feeding for 41/50 (82%). The grades of retrognathia and glossoptosis and the severity of respiratory disorders did not differ between patients with isolated PRS and syndromic PRS. Severe respiratory disorders were more common and long-lasting feeding (>12 months) was more frequently required in patients with syndromic PRS compared with isolated PRS (42 vs. 13%, p = 0.04 and 42 vs. 4%, p < 0.01 respectively). Using univariate analysis, neurological impairments and laryngomalacia were associated with severe respiratory disorders [Odds ratio (OR) 5.0, 95% CI 1.3–19.6; and OR 14.6, 95% CI 1.3–161.4; p < 0.05] as well as with long-lasting feeding (>12 months) disorders (OR 18.6, 95% CI 3.9–89.2 and OR 20.4, 95% CI 3,4–122.8; p < 10−2). Syndromic SPR status was also associated with severe respiratory disorders (OR 4.9, 95% CI 1–32.5; p < 0.05). Using multivariate analysis, only syndromic PRS status was predictive for severe respiratory disorders (adjusted OR 8, 95% CI 1.47–44.57; p < 0.05); and only neurological impairments remained a significant risk for long lasting feeding disorders (>12 months) (adjusted OR 21.72, 95% CI 3.4–138.63; p < 10−2). The grades of retrognathia and glossoptosis were not predictive factors for the severity of respiratory and feeding disorders.Conclusion: In children with PRS, the severity of clinical conditions may not correlate with anatomic variables but rather with laryngeal abnormalities, neurological impairement and syndromic PRS status.
Highlights
Pierre Robin sequence (PRS), originally described as associating retrognathia, glossoptosis and neonatal upper airway obstruction, has been secondarily considered a malformation sequence arising from limited mandibular growth and a vertical tongue position, leading to a U-shaped cleft palate [1, 2]
We retrospectively studied a series of 50 infants with PRS who were managed homogeneously in a single center, and found no significant association between the grades of anatomic variables and the severity of respiratory and feeding disorders
Our series includes an equal number of isolated Pierre Robin sequence (iPRS) and syndromic Pierre Robin sequence (sPRS), while most published series reported high rates of non-isolated PRS, under the names syndromic, associated, or PRS-plus [3,4,5,6, 12, 14,15,16,17,18,19]
Summary
Pierre Robin sequence (PRS), originally described as associating retrognathia, glossoptosis and neonatal upper airway obstruction, has been secondarily considered a malformation sequence arising from limited mandibular growth and a vertical tongue position, leading to a U-shaped cleft palate [1, 2]. Mortality rate for children with PRS has significantly decreased, it remains substantial, up to 16.6% according to latest reports [7, 8]. Predicting the severity of respiratory and digestive disorders in newborn infants with PRS could help to reduce both morbidity and mortality rates and to choose the most suitable treatment. Conservative treatments include prone position, palate plates, nasopharyngeal tube, and non-invasive ventilation; and surgical treatments include mandibular distraction osteogenesis (MDO), tongue-lip adhesion (TLA), and tracheostomy [9]. Even if a direct causal link between anatomic features and functional disorders may be reasonably suspected, this has not been yet demonstrated
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