Early Surgical Management of Infants with Robin Sequence in a Large Multi-center Sample

Abstract

OBJECTIVE: Studies of infants with Robin sequence (RS), the triad of micrognathia, glossoptosis and upper airway obstruction, are limited by the rarity of the condition and heterogeneity of the sample that results from phenotypic and diagnostic variability. Most knowledge of this condition is sourced from small, single-institution samples and is inherently biased. The purpose of this study is to characterize early surgical management of infants with RS in a large multi-institution sample. METHODS: This is a cross-sectional study initiated as part of the Children’s Hospital Neonatal Consortium (CHNC) micrognathia working group and includes infants with micrognathia admitted to CHNC centers from 2010-2020. Data was abstracted from the Children’s Hospital Neonatal Database (CHND), which captures clinical information for infants admitted to neonatal intensive care units at each of the 34 CHNC centers. Predictor variables included demographic data, birth characteristics, cleft and syndrome status, and operative details. Outcome variables included length of stay, death, feeding and respiratory support at hospital discharge, and need for secondary airway operations. Trends in surgical management between centers and over time were also evaluated. A p-value<0.05 was considered statistically significant for all analyses. RESULTS: Of the 248,010 patients included in CHND during the study period, 3256 (1.3%) had a diagnosis of micrognathia and 1294 (39.7%) of these had an operation to correct upper airway obstruction during infancy. For those that had an operation, mean age and weight at operation were 34.9±33.6 days and 3515.4±712.8 grams, respectively. A syndromic diagnosis was made in 152 (1.2%) prior to hospital discharge, with Stickler (n=71, 46.7%) and Treacher Collins Syndromes (n=28, 18.4%) most common. Operations included: mandibular distraction osteogenesis (MDO), n=856 (66%); tracheostomy, n=331 (26%); and tongue-lip adhesion (TLA), n=107 (8%). Analysis revealed several notable associations: Compared to MDO and TLA, initial tracheostomy was performed more commonly for male (51.0% vs 58.0%, p=0.03) infants without a cleft palate (35.3% vs 63.1%, p<0.001), and/or with Treacher Collins syndrome (0.4% vs 7.3%, p<0.001). Length-of-stay was shorter for MDO compared to TLA (57.9 +/- 40 vs. 69.0 +/- 40 days, p=0.007), and shorter for TLA compared to tracheostomy (69.0 +/- 40.0 vs. 110.4 +/- 71.0 days, p<0.001). Exclusive oral feeding was achieved by NICU discharge more frequently after MDO than TLA (35.4% vs 15.0%, p<0.001) and least commonly after tracheostomy (3.6%). The rate of gastrostomy tube placement was highest after tracheostomy (76.4%) and was and lowest after MDO (TLA=54.7%, MDO=28.5%, p<0.001). Infants that had MDO were less likely to require a second airway operation than those with TLA (3.5% vs 17.8%, p<0.001). Mortality was low for all operations (0.5%). CONCLUSION: In this cohort, the largest dataset of infants with RS ever reported, MDO was associated with shorter hospital stay, improved oral feeding, and lower rates for gastrostomy tube placement and secondary airway operations compared to TLA and tracheostomy. Prospective multi-center studies are necessary to further support these conclusions.