Will the right Robin patient rise, please? Definitions and criteria during management of Robin sequence patients in the Netherlands and Belgium

J Craniomaxillofac Surg. 2015 Jan;43(1):92-6. doi: 10.1016/j.jcms.2014.10.015. Epub 2014 Nov 5.

Abstract

Background: Robin Sequence (RS) is characterized by micrognathia and upper airway obstruction (UAO), with or without cleft palate, causing respiratory and feeding problems. Management options are: positioning; nasopharyngeal airway (NPA); tongue-lip adhesion (TLA); mandibular distraction (MDO); and tracheostomy. Controversy exists in literature regarding RS definition and management. Here we describe definitions, management strategies and criteria in opting for management strategies, used by Dutch and Belgian cleft teams.

Methods: A specifically designed questionnaire was sent to members of all 16 Dutch and Belgian cleft teams.

Results: 14 cleft teams returned 35 questionnaires. All used micrognathia as definition criterion, 93.4% cleft palate, 51.5%glossoptosis and 45.7% UAO. Six different RS definitions were used; even within a single team >1 definition was used. All teams used different management strategies: all used positioning, 10 NPA, 6 TLA, 7 MDO, 8 tracheostomy, 5 refer patients with invasive treatment indication. Criteria in opting management modalities were: O2-saturation (89.3%), clinical presentation (86.2%), growth and feeding problems (69.0%), polysomnography (62.1%), and differed within teams.

Conclusion: The Dutch and Belgian cleft teams use variable RS definitions, different management modalities and criteria in choosing management strategies. A single, strict definition and evidence-based management guidelines should be formulated for optimal patient care.

Keywords: Cleft palate; Definition; Diagnostic criteria; Micrognathia; Robin sequence; Treatment.

MeSH terms

  • Airway Obstruction / diagnosis
  • Belgium
  • Cleft Palate / diagnosis
  • Eating / physiology
  • Glossoptosis / diagnosis
  • Growth Disorders / prevention & control
  • Humans
  • Intubation, Intratracheal
  • Lip / surgery
  • Mandible / surgery
  • Micrognathism / diagnosis
  • Netherlands
  • Osteogenesis, Distraction / statistics & numerical data
  • Oxygen / blood
  • Patient Care Team
  • Patient Positioning
  • Pierre Robin Syndrome / diagnosis*
  • Pierre Robin Syndrome / therapy
  • Polysomnography / statistics & numerical data
  • Tongue / surgery
  • Tracheostomy / statistics & numerical data

Substances

  • Oxygen