INTRODUCTION
In 1923, Pierre Robin, a French stomatologist, described the combination of micrognathia, glossoptosis, and respiratory distress, with or without cleft palate. Infants with the most severe manifestations of this sequence have life-threatening respiratory compromise that impacts their ability to feed. Tracheostomy is the preferred treatment for refractory respiratory failure, but there is significant morbidity and a cumulative mortality risk associated with this option. Recent studies support emergent mandibular distraction, however the associated morbidity is substantial and it is unclear how this maneuver affects long-term mandibular growth. Additionally, distraction may not prevent tracheostomy.
Tongue-lip adhesion has long been advocated as primary treatment for infants with severe Robin sequence. This method surgically positions the tongue anteriorly and opens the orophanynx. Early cases documenting repair dehiscence and reports recommending rigorous preoperative screening with nasendoscopy have tempered enthusiasm for this technique.
PURPOSE
This study evaluates the effectiveness of tongue-lip adhesion for managing airway compromise in infants with the most severe manifestations of Robin sequence (nonsyndromic and syndromic) without regard to rigid preoperative selection criteria.
MATERIALS AND METHODS
We retrospectively reviewed 24 consecutive patients managed at our center with tongue-lip adhesion for refractory respiratory compromise associated with Robin sequence between 1994 and 2001. All patients had severe, sustained desaturations not alleviated by positioning, oxygen supplementation, or pressure assistance (CPAP). Additionally, all patients had early failure to thrive and required nasogastic feedings since birth. Tracheostomy had been considered in each case. Modified tongue-lip adhesion, as described by Armagasso, was done in each instance. The end point of the study was takedown of the tongue-lip adhesion during repair of the cleft palate (success) or tracheostomy/other treatment (failure).
RESULTS
13 patients were non-syndromic and 11 were syndromic (7 Stickler, 1 distal arthrogryposis type 2, 1 Freeman-Sheldon, 1 Borgesson-Fearson-Lehman, 1 DeLange). 19 patients required a gastric tube for feeding. Tongue –lip adhesion was performed at a mean age of 24 days (range 4-61). 19 patients were successfully managed with tongue-lip adhesion and had no further episodes of respiratory distress. Additionally, all 19 patients were able to begin oral feedings shortly after repair. All have had the adhesion released during repair of the palatal cleft, usually at 9 months. There were five failures. Four patients went on to have tracheostomy. Two patients were premature (birth weights 2200 grams and 990 grams) and had early dehiscence of the repair ascribed to poor nutrition. Another patient had severe GERD and required tracheostomy to prevent aspiration. The last patient failed extubation several times and tracheostomy was recommended to permit earlier extubation. One patient with tracheal stenosis had continued intermittent obstruction after tongue-lip adhesion and underwent mandibular distraction at 6 months of age, however, this was not successful and the child is being considered for tracheostomy.
CONCLUSIONS
Tongue-lip adhesion remains an effective first-line strategy for patients with refractory respiratory compromise associated with Robin sequence. The procedure has relatively low morbidity compared to tracheostomy or mandibular distraction and does not preclude these other treatment options. It should be considered as primary intervention for infants with severe Robin sequence.
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