Severity of Retrognathia and Glossoptosis Does Not Predict Respiratory and Feeding Disorders in Pierre Robin Sequence

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.

Quantification of Mandibular Morphology in Pierre Robin Sequence to Optimize Mandibular Distraction Osteogenesis

“…This conclusion aligns with the current pathophysiologic understanding of upper airway obstruction in PRS. Since the publication of a seminal paper by Sher in 1992, it has been understood that although micrognathia-associated glossoptosis underlies the airway obstruction in all patients with PRS to some extent, clinical airway obstruction is determined by a number of causes, including neurologic dysfunction, rate of neuromuscular maturation, and laryngomalacia (Randall et al, 1965; Sher, 1992; Morice et al, 2018). Additionally, Runyan et al (2018) published a retrospective analysis of 171 patients with PRS and found that the most significant factor predictive of surgical intervention was a high obstructive index on preintervention sleep study; the authors noted no differences in micrognathia between patients managed conservatively and those managed surgically ( P = 1.000) (Runyan et al, 2018).…”

“…Additionally, Runyan et al (2018) published a retrospective analysis of 171 patients with PRS and found that the most significant factor predictive of surgical intervention was a high obstructive index on preintervention sleep study; the authors noted no differences in micrognathia between patients managed conservatively and those managed surgically ( P = 1.000) (Runyan et al, 2018). Morice et al (2018) have also reported that grades of retrognathia and glossoptosis are not predictive of respiratory and feeding disorder severity in both patients with syndromic and nonsyndromic PRS (Morice et al, 2018).…”

Study on the Effect of Bilateral Mandible Distraction Osteogenesis About the Nutrition Status of Infants With Pierre-Robin Sequence

“…However, some scholars believe that nutritional changes should be studied independently with airway changes, because breathing and swallowing difficulties in PRS patients may not only be related to anatomical variation (such as micrognathia and glossoptosis etc. ), but also to abnormal laryngeal development, neurological disorders, and various other associated syndromes (27). For this part of the patients, high-calorie feeding should be applied as soon as possible (28), instead of waiting for the relief of airway symptoms to bring an improvement in nutrition status; the nutritional risk of PRS may not be accompanied by dyspnea, since the symptoms can appear immediately after birth, or may be delayed (29).…”

Anatomical analysis of auricle and mentum in Turkish population

“…[13] Pierre Robin sendromu gibi genetik hastalıklara eşlik edebilir. [14] Temporomandibüler eklem rahatsızlıkları, yeme ve çiğneme bozuklukları ve görsel bozukluklara yol açabilir. Özellikle yan açıdan görsel görünüm bozukluklarına neden olabileceği gibi rinoplastinin yanı sıra yapılan mentoplasti ameliyatlarının sık nedenidir.…”