The Surgical Treatment of Robin Sequence.

BACKGROUND

We present an outcomes analysis of the surgical treatment of Robin sequence including all infants and comorbid conditions treated by tongue-lip adhesion (TLA) or mandibular distraction osteogenesis (MDO).

METHODS

A 19-year single-institution, multisurgeon retrospective review of all syndromic and nonsyndromic neonates with Robin sequence treated with TLA (1994-2004) or MDO (2004-2013) was performed. Comorbid conditions were recorded in all patients. Outcomes include incidence of tracheostomy, improvement in obstructive breathing, and surgical complications. Need for repeat distraction and conversion from TLA to MDO were included as secondary end-points. Polysomnography data were recorded preoperatively at 1 month and 1 year as a measure of airway improvement.

RESULTS

Seventy-four MDO patients and 15 TLA patients during the study period met inclusion criteria. There was no significant difference in mean age at intervention (32.1 ± 29.0 vs 35.5 ± 32.1 days), birth weight (2.9 ± 0.7 vs 3.2 ± 0.6 kg), prematurity (23.0 vs 35.7%), or intrauterine growth restriction (31.1 vs 15.4%). Central nervous system anomalies (24.3% vs 0.0%; P < 0.04) and gastrostomy tubes (66.2% vs 33.3%; P < 0.03) were present more frequently in MDO patients versus TLA patients. Rates of other organ system anomalies were similar between the groups. The success rate was significantly higher in the MDO group (90.5% vs 60.0%; P < 0.008). Postoperative tracheostomies occurred in 8.1% of the MDO group and 33.3% of the TLA group (P < 0.02). Preoperative apnea-hyponea index was similar between the two groups (38.3 vs 38.1). The apnea-hyponea index was significantly improved in the MDO group at 1 month (4.0 vs 21.7; P < 0.002) and 1 year (5.7 vs 20.5; P < 0.005). Surgical complications were statistically less in the MDO group (20.3 vs 53.3%; P < 0.02).

CONCLUSIONS

In the heterogeneous population of Robin sequence, MDO demonstrates superior outcomes measures at 1 month and 1 year compared to TLA. Fewer complications occurred in the MDO group compared to the TLA.