Abstract
Pierre Robin Sendromu (PRS) 1902 yılında tanımlanmış olup damak yarığı, mandibuler retrognati ve/veya mikrognati ve glossopitozis şeklinde üç komponentten oluşmaktadır. Asfiksiye bağlı mortalite oranının % 30 olarak belirlendiği sendromda bir diğer problem de beslenme bozukluğudur. Bu nedenle hayatın ilk günlerinde beslenmenin ve solunumun rahat bir şekilde gerçekleştirilmesini sağlamak amacıyla müdahale gerekebilir. PRS’lu olgularda, hava yolu açıklığını sağlamak için literatürde bildirilmiş olan pozisyon verme, nazofarengeal tüp kullanımı, entübasyon ve cerrahi tedavi gibi yaklaşımlar mevcuttur. Hayatın ilk günlerinde uygulanan bu yaklaşımlar bebek ve aile için travmatik olabilmektedir. Bu yazıda 1 günlük iken beslenememe ve solunum problemleri ile başvuran ve ortodontik aparey kullanımı ile sorunsuz olarak taburcu edilen PRS’lu bir olgu sunulmuştur.
References
- Wittenborn W, Panchal J, Marsh JL, Sekar KC, Gurley J. Neonatal distraction surgery for micrognathia reduces obstructive apnea and the need for tracheotomy. J Craniofacial Surg 2004;15:623- 30.
- Turner L, Jacobsen C, Humenczuk M, Singhal VK, Moore D, Bell H. The effects of lactation education and a prosthetic obturator appliance on feeding efficiency in infants with cleft lip and palate. Cleft Palate Craniofac J 2001;38:519-24.
- Tunçbilek G, Özgür F, Balcı S. 1229 yarık dudak ve damak hastasında görülen ek malformasyon ve sendromlar. Çocuk Sağlığı ve Hastalıkları Dergisi 2004;47:172-6.
- Evans KN, Sie KC, Hopper RA, Glass RP, Hing AV, Cunningham ML. Robin sequence: From diagnosis to development of an effective management plan. Pediatrics 2011;127:936–48.
- Poets CF, Bacher M. Treatment of upper airway obstruction and feeding problems in Robin-like phenotype. J Pediatr 2011;159:887– 92.
- Ludwig B, Glasl B, Sader R, Schopf P. conservative orthodontic primary care of four newborns with the Pierre-Robin sequence triad: Case report. J Orofac Orthop 2007;68:56-61.
- Vatlach S, Maas C, Poets CF. Birth prevalence and initial treatment of Robin sequence in Germany: A prospective epidemiologic study. Orphanet J Rare Dis 2014;9:9.
- Hotz MM, Gnoinski WM, Nussbaumer H, Kistler E. Early maxillary orthopedics in CLP cases: Guidelines for surgery. Cleft Palate J 1978;15:405-11.
- Schaefer RB, Stadler JA, 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.
- Anderson KD, Cole A, Chuo CB, Slator R. Home management of upper airway obstruction in Pierre Robin sequence using a nasopharyngeal airway. Cleft Palate Craniofac J 2007;44:269-73.
Abstract
The Pierre-Robin Syndrome (PRS) has been defined in 1902 and consists of three components: cleft palate, mandibular retrognathia and/or micrognathia, and glossoptosis. The mortality rate due to asphyxia is 30% in the syndrome. Another common problem is malnutrition. Intervention may therefore be required to provide a comfortable route for feeding and breathing in the first days of life. Some previously reported approaches are positioning, nasopharyngeal tube usage, intubation and surgical treatment to provide airway patency. However these approaches may be traumatic for the baby and family in the early days of life. In this paper, a 1-day-old PRS case who presented with nutritional and respiratory problems and was later discharged without any problems by the use of an orthodontic palate plaque is presented
References
- Wittenborn W, Panchal J, Marsh JL, Sekar KC, Gurley J. Neonatal distraction surgery for micrognathia reduces obstructive apnea and the need for tracheotomy. J Craniofacial Surg 2004;15:623- 30.
- Turner L, Jacobsen C, Humenczuk M, Singhal VK, Moore D, Bell H. The effects of lactation education and a prosthetic obturator appliance on feeding efficiency in infants with cleft lip and palate. Cleft Palate Craniofac J 2001;38:519-24.
- Tunçbilek G, Özgür F, Balcı S. 1229 yarık dudak ve damak hastasında görülen ek malformasyon ve sendromlar. Çocuk Sağlığı ve Hastalıkları Dergisi 2004;47:172-6.
- Evans KN, Sie KC, Hopper RA, Glass RP, Hing AV, Cunningham ML. Robin sequence: From diagnosis to development of an effective management plan. Pediatrics 2011;127:936–48.
- Poets CF, Bacher M. Treatment of upper airway obstruction and feeding problems in Robin-like phenotype. J Pediatr 2011;159:887– 92.
- Ludwig B, Glasl B, Sader R, Schopf P. conservative orthodontic primary care of four newborns with the Pierre-Robin sequence triad: Case report. J Orofac Orthop 2007;68:56-61.
- Vatlach S, Maas C, Poets CF. Birth prevalence and initial treatment of Robin sequence in Germany: A prospective epidemiologic study. Orphanet J Rare Dis 2014;9:9.
- Hotz MM, Gnoinski WM, Nussbaumer H, Kistler E. Early maxillary orthopedics in CLP cases: Guidelines for surgery. Cleft Palate J 1978;15:405-11.
- Schaefer RB, Stadler JA, 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.
- Anderson KD, Cole A, Chuo CB, Slator R. Home management of upper airway obstruction in Pierre Robin sequence using a nasopharyngeal airway. Cleft Palate Craniofac J 2007;44:269-73.
Details
| Other ID | JA84PB73GR |
|---|---|
| Journal Section | Case Report |
| Authors | |
| Publication Date | December 1, 2017 |
| Submission Date | December 1, 2017 |
| Published in Issue | Year 2017 Volume: 11 Issue: 4 |
The publication language of Turkish Journal of Pediatric Disease is English.
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