Is Obstructive Apnea More Severe in Syndromic Than Nonsyndromic Patients With Robin Sequence?

Patients with syndromic Robin sequence (RS) are known to have poorer outcomes from operations to relieve obstructive apnea (OA) compared with those with nonsyndromic RS. The reason for this is unknown. The purpose of this study was to compare OA in unoperated syndromic and nonsyndromic patients with RS. We hypothesized that the syndromic group wouldhave more severe OA and that this could explain the inferior operative results.

This was a retrospective cohort study of patients with RS at Boston Children's Hospital from 1994-2018. Patients with at least 1 polysomnogram (PSG) before any airway operation were included. Patients were separated into syndromic and nonsyndromic groups. The apnea-hypopnea index (AHI) measured from PSGs was the primary outcome variable. Descriptive statistics and logistic regression were calculated, and statistical significance was set at P < .05.

The study sample included 79 patients: 44 in the syndromic group (56%) and 35 in the nonsyndromic group (44%). The mean age at PSG was 7.4 ± 10.2 months, and 56% were female patients. The mean AHIs for the syndromic and nonsyndromic groups were not statistically significantly different: 11.9 ± 20.6 and 12.5 ± 13.9, respectively (P = .839). Among patients who underwent both an airway operation (tongue-lip adhesion, mandibular distraction, or tracheostomy) and a postoperative PSG (n = 42), the mean postoperative AHI was 5.7 ± 15.7 for the syndromic group (74% decrease, n = 20) and 1.3 ± 2.2 for the nonsyndromic group (92% decrease, n = 22, P = .038).

In unoperated patients with RS, there is no significant difference in the severity of OA between syndromic and nonsyndromic patients. The inferior results seen after operations to relieve OA in syndromic patients may be a result of other factors related to the syndromic diagnosis rather than the level of preoperative OA.