Tongue Lip Adhesion Outcomes and Mechanism of Obstruction in Robin Sequence

PURPOSE: Increasingly, data shows favorable outcomes with regard to surgical airway management with the less-invasive Tongue-Lip Adhesion (TLA), and unfavorable long-term sequelae of neonatal distraction, raising questions of its necessity.  This purpose of this study is to show pre- and post-op polysomnography data in children treated with tongue-lip adhesion, with and without oxygen supplementation, that demonstrate dramatically improved airway function over the first six months of life.  This series describes an approach that can obviate in most cases the need for mandibular distraction, even in severe and syndromic forms of Robin sequence.

 

METHODS: All patients meeting clinical criteria for Pierre Robin Sequence (PRS) and who had undergone tongue-lip adhesion with pre- and post-op sleep studies were reviewed. The clinical series included 17 subjects (13 non-syndromic, 4 with Stickler syndrome). All but one were managed either by TLA alone or in combination with positioning and/or supplemental oxygen; the 17^th (Stickler syndrome) subsequently underwent mandibular distraction; an additional nonsyndromic patient underwent mandibular distraction prior to palatoplasty.  None underwent tracheostomy.  Most received gastrostomy to insure optimal nutrition.

 

RESULTS: Of the 13 nonsyndromic patients managed with TLA as definitive treatment, the mean preop apnea-hypopnea index (AHI) was 37.5 (range 21.0-71.8); the mean initial postop AHI improved to 7.7 (range 0.9-31.0).  Of the four syndromic patients, the mean preop AHI was 65.5 and improved to 23.1 postop, with one failing TLA and subsequently treated with mandibular distraction and a post-distraction AHI of 1.0.  By approximately six months of age the AHI among both syndromic and nonsyndromic patients had improved further to a mean of 1.8 (range 0-5.3) with the addition of oxygen in patients with initial postop AHI>10. Of those treated with oxygen in addition to TLA, all showed improvement in AHI, and all were weaned prior to palatoplasty.  Overall growth was essentially normal.  “Catch-up” mandibular AP growth was clinically variable, with followup varying from ten months to 11 years.

 

CONCLUSION: TLA provides a less-invasive and reliable treatment option for the majority of cases of PRS, including syndromic forms; mandibular distraction remains an essential surgical option but is necessary only in select cases. Furthermore, the clinical successes and failures of this protocol provide new insight in the pathophysiology of the airway problem in Robin sequence, and substantiates the theory that in most cases of newborn retrognathia functional recovery will occur with time and growth.