Maxillofacial Plastic and Reconstructive Surgery

Korean Association of Maxillofacial Plastic and Reconstructive Surgeons (대한악안면성형재건외과학회)
권호 표지

Sequential Management of Pierre Robin Sequence: Case Report

상기도 폐쇄를 보이는 Pierre Robin Sequence 환아의 순차적 치료: 증례보고

  • Kim, Bae-Kyung (Department of Oral and Maxillofacial Surgery, College of Dentistry, Kyung Hee University,) ;
  • Kwon, Yong-Dae (Department of Oral and Maxillofacial Surgery, College of Dentistry, Kyung Hee University,) ;
  • Ryu, Sun-Youl (Department of Oral and Maxillofacial Surgery, College of Dentistry, Chonnam National University) ;
  • Choi, Yong-Ha (Department of Oral and Maxillofacial Surgery, College of Dentistry, Kyung Hee University,) ;
  • Ohe, Joo-Young (Department of Oral and Maxillofacial Surgery, College of Dentistry, Kyung Hee University,) ;
  • Suh, Joon-Ho (Department of Oral and Maxillofacial Surgery, College of Dentistry, Kyung Hee University,) ;
  • Ko, Su-Jin (Department of Dentistry, Division of Orthodontics, Eulji General Hospital, Eulji University School of Medicine)
  • 김배경 (경희대학교 치의학전문대학원 구강악안면외과학교실) ;
  • 권용대 (경희대학교 치의학전문대학원 구강악안면외과학교실) ;
  • 유선열 (전남대학교 치과대학 구강악안면외과학교실) ;
  • 최용하 (경희대학교 치의학전문대학원 구강악안면외과학교실) ;
  • 오주영 (경희대학교 치의학전문대학원 구강악안면외과학교실) ;
  • 서준호 (경희대학교 치의학전문대학원 구강악안면외과학교실) ;
  • 고수진 (을지대학교 의과대학 을지병원 치과교정학교실)
  • Received : 2011.04.05
  • Accepted : 2011.05.09
  • Published : 2011.05.31
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Abstract

Pierre Robin Sequence (PRS) is known as an anomaly consisting of respiratory obstruction with glossoptosis, micrognathia and cleft palate in a newborn. The etiology of PRS is not known, but several factors may be involved simultaneously. Mortality rate of PRS is about 5~30% and the treatment method is divided into both conventional treatments and surgical interventions. If the respiratory obstruction is not resolved by the conventional method, surgical treatment, such as subperiosteal release of the floor of the mouth, tongue-lip adhesion, tracheostomy, distraction osteogenesis may be needed. This study reports a case of PRS in a newborn male at 20 days, with dyspnea and feeding difficulties. Clinical examination showed micrognathia with glossoptosis and cleft palate as the typical PRS triad. We tried surgical intervention with subperiosteal release of the floor of the mouth and tongue-lip adhesion and surgery was successful. At $19^{th}$ months, we also repaired the incomplete cleft palate successfully using 2-Flap palatoplasty.

Keywords

References

  1. Oh YJ, Rotaru Horatiu, Park YW, Kwon KJ, Kim SG, Kim MK. Combination therapy using glossopexy and radiofrequency therapy in Pierre Robin Sequence. J Korean Assoc Maxillofac Plast Reconstr Surg 2010;32:242-5.
  2. Delorme RP, Larocque Y, Caouette-Laberge L. Innovative surgical approach for the Pierre Robin anomalad: subperiosteal release of the floor of the mouth musculature. Plast Reconstr Surg 1989;83:960-4; discussion 965-6. https://doi.org/10.1097/00006534-198906000-00004
  3. Parsons RW, Smith DJ. A modified tongue-lip adhesion for Pierre Robin anomalad. Cleft Palate J 1980;17:144-7.
  4. 홍창의. 소아과학. In: 안효섭, editor. 홍창의 소아과학 제9판. 서울: 대한교과서주식회사; 2007. p. 1230-1.
  5. Champion R. Treatment of cleft palate associated with micrognathia. Br J Plast Surg 1956;8:283-90.
  6. Pasyayan HM, Lewis MB. Clinical experience with the Robin sequence. Cleft Palate J 1984;21:270-6.
  7. Hoffman W. Outcome of tongue-lip plication in patients with severe Pierre Robin sequence. J Craniofac Surg 2003;14:602-8. https://doi.org/10.1097/00001665-200309000-00002
  8. Kirschner RE, Low DW, Randall P, et al. Surgical airway management in Pierre Robin sequence: is there a role for tongue-lip adhesion? Cleft Palate Craniofac J 2003;40:13-8. https://doi.org/10.1597/1545-1569(2003)040<0013:SAMIPR>2.0.CO;2
  9. Denny A, Amm C. New technique for airway correction in neonates with severe Pierre Robin sequence. J Pediatr 2005;147:97-101 https://doi.org/10.1016/j.jpeds.2005.02.018
  10. Schaefer RB, Stadler JA 3rd, Gosain AK. To distract or not to distract: an algorithm for airway management in isolated Pierre Robin sequence. Plast Reconstr Surg 2004;113:1113-25 https://doi.org/10.1097/01.PRS.0000110323.50084.21
  11. Caouette-Laberge L, Bayet B, Larocque Y. The Pierre Robin sequence: review of 125 cases and evolution of treatment modalities. Plast Reconstr Surg 1994;93:934-42.
  12. Lehman JA, Fishman JR, Neiman GS. Treatment of cleft palate associated with Robin sequence: appraisal of risk factors. Cleft Palate Craniofac J 1995;32:25-9. https://doi.org/10.1597/1545-1569(1995)032<0025:TOCPAW>2.3.CO;2
  13. Myer CM 3rd, Reed JM, Cotton RT, Willging JP, Shott SR. Airway management in Pierre Robin sequence. Otolaryngol Head Neck Surg 1998;118:630-5.
  14. Cruz MJ, Kerschner JE, Beste DJ, Conley SF. Pierre Robin sequences: secondary respiratory difficulties and intrinsic feeding abnormalities. Laryngoscope 1999;109:1632-6. https://doi.org/10.1097/00005537-199910000-00016
  15. Hawkins DB, Williams EH. Tracheostomy in infants and young children. Laryngoscope 1976;86:331-40. https://doi.org/10.1288/00005537-197603000-00002
  16. Denny AD, Amm CA, Schaefer RB. Outcomes of tongue-lip adhesion for neonatal respiratory distress caused by Pierre Robin sequence. J Craniofac Surg 2004;15:819-23. https://doi.org/10.1097/00001665-200409000-00023
  17. Spring MA, Mount DL. Pediatric feeding disorder and growth decline following mandibular distraction osteogenesis. Plast Reconstr Surg 2006;118:476-82. https://doi.org/10.1097/01.prs.0000227740.48021.c3
  18. Ryu SY, Lee YU, Seo IY. Surgical treatment of airway obstruction in infants with Pierre Robin sequence. J Korean Oral Maxillofac Surg 2004;30:237-45.