Current Trends in Surgical Airway Management of Patients with Robin Sequence

Background & Purpose 

Robin sequence (RS) is the clinical triad of micrognathia, glossoptosis, and airway obstruction. Mortality rates range as high as 65%, though improved nutritional support and airway management may reduce this rate. In severe cases, surgical intervention may be indicated to relieve airway obstruction. Though the efficacy of certain surgical interventions (e.g. tracheostomy, tongue-lip adhesion – TLA, mandibular distraction osteogenesis – MDO) in improving patient outcomes is well established, algorithms dictating decision making and peri-operative protocols are poorly defined. To aid in establishment of distinct protocols among surgeons treating RS, we designed a survey to elucidate current practice trends.

Method/Description

            A 22-question survey was designed on SurveyMonkey (www.surveymonkey.com) and sent via e-mail to members of the American Cleft Palate-Craniofacial Association and International Society of Craniofacial Surgeons. Questions were related to surgeon experience in treating RS, and peri-operative protocols. Responses were collected for 8 weeks.

 

Results

            A total of 151 responses were collected. Most respondents were surgeons practicing in North America (82.8%), in a university hospital setting (81.5%), and had completed a fellowship in pediatric plastic surgery or craniofacial surgery (76.2%). Pre-operative protocols varied widely. While 78.8% of respondents performed direct laryngoscopy, only 49.7% routinely obtained pre-operative polysomnography. Minimum apnea hypopnea index (AHI) for surgical intervention ranged from <10 (21.4%) to >30 (6.8%). 74.2% reported MDO as their most common primary surgical modality, with 12.6% primarily utilizing TLA. Similarly, only 45.7% perform TLA. Surgeon experience influenced operative selection, with 80% of those in practice 0-5 years primarily utilizing MDO, compared to 56% in practice >15 years.

Among those performing MDO, there were variations in osteotomy selection (inverted L ramus–39.3%, angle–37.8%), distraction vector (horizontal–64.0%, oblique–22.1%), type of device (internal–80.0%, external–23.1%), and use of virtual surgical planning (yes/sometimes–50.0%, no–50.0%). 25.2% did not incorporate a latency phase. Daily activation length mostly ranged from 1.0mm (45.1%) to 2.0mm (32.3%), with most choosing an endpoint of class 3 occlusion (56.0%) or “as far as possible” (28.4%). There was no consensus on consolidation phase (4-6 weeks–20.0%, 6-8 weeks–31.3%, 8-10 weeks–19.1%, >10 weeks– 25.2%). Most respondents (90.3%) reported low rates (0-24% of patients) of required secondary intervention for apnea after distraction.

             

Conclusions

Surgical airway management in patients with RS varied widely. Clear trends were not identified in preoperative evaluation, type of surgical intervention, intraoperative or postoperative protocols, though MDO was utilized more than other modalities, particularly among younger surgeons. Further studies and collaborative efforts will help guide standards of care in the airway management of these patients.