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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.52 no.4 São Paulo Dec. 1994 

Pierre Robin sequence and obstructive sleep apnea


Sequência de Pierre Robin e apnéia do sono tipo obstrutivo



Rubens ReimãoI; Elio Giacomo PapaizII; Luiz Fernando PapaizIII

IDepartment of Neurology, Hospital das Clínicas, University of São Paulo Medical School and Sleep Disorders Center, Reimão Clinic, São Paulo
IIDepartment of Radiology, Dentistry School, University of São Francisco, Bragança Paulista, São Paulo
IIIFellow in Radiology, São Paulo Dentistry Association




The case of a 12-year-old female patient with Pierre Robin sequence is reported, in which reduction of the pharyngeal airway leads to obstructive sleep apnea syndrome (OSAS) and excessive daytime sleepiness. Radiological evaluation, computerized tomography and magnetic resonance image showed bilateral temporomandibular ankylosis. Cephalometric data evidenced marked reduction of the posterior airway space. Three all-night polysomnographic evaluations detected severe OSAS with decrease in oxygen saturation. The Multiple Sleep Latency Test (MSLT) perfomed on two separate days objectively quantified the excessive daytime sleepiness with short sleep latencies; stage REM was not present. Polysomnography, MSLT and thorough radiologic studies, in this case, made it possible to determine the severity of OSAS, the site of obstruction, and the associated malformations.

Key words: sleep apnea syndrome, apnea, Pierre Robin, sleep, polysomnography, cephalometrics.


É relatado o caso de paciente do sexo feminino de 12 anos de idade, com sequência de Pierre Robin, cuja redução da luz faríngea leva a apnéia do sono tipo obstrutivo e consequente sonolência excessiva diurna. Estudo radiológico articular, tomografia computadorizada e ressonância nuclear magnética evidenciam anquilose bilateral da articulação temporomandibular. Cefalometria mostra redução acentuada do espaço aéreo posterior. Polissonografia realizada durante três noites inteiras apresenta síndrome de apnéia do sono tipo obstrutivo, severa, acompanhada de redução da saturação de oxigénio. No Teste das Latências Múltiplas do Sono (TLMS) há objetivamente sonolência excessiva diurna, com descréscimo da latência do sono; não ocorre estágio REM. Avaliação polissonográfica, do TLMS e radiológica extensa permitem, neste caso, estagiar a severidade de acometimento e as malformações concomitantes e detectar o sítio de obstrução.

Palavras-chave: síndrome de apnéia do sono, apnéia, Pierre Robin, sono, polissonografia, cefalometria.



Texto completo disponível apenas em PDF.

Full text available only in PDF format.



Acknowledgments: We are indebt to Beatriz Lefèvre for psychological evaluation and support.



1. Adair NE, Mathews BL, Haponik EF. Techniques to assist selection of appropriate therapy for patients with obstructive sleep apnea. Head Neck Surg 1991, 2: 81-86.         [ Links ]

2. Augarten A, Sagy M, Yahav J, Barzylay Z. Management of upper airway obtruction in the Pierre Robin syndrome. Br J Oral Manxillofac Surg 1990, 28: 105-109.         [ Links ]

3. Bull MJ, Givan DC, Sandove AH, Bixler D, Hearn D. Improved outcome in Pierre Robin sequence: effect of multidisciplinary evaluation and management. Pediatrics 1990, 86: 294-301.         [ Links ]

4. Carskadon MA, Dement WC, Mitler MM, Roth T, Westbrook PR, Keenan S. Guidelines for the Multiple Sleep Latency Test (MSLT): a standard measure of sleepiness. Sleep 1986, 9: 519-524.         [ Links ]

5. Cozzi F, Pierro A. Glossoptosis-apnea syndrome in infancy. Pediatrics 1985, 75: 836-843.         [ Links ]

6. Debery-Borowicki B, Kukwa A, Banks R. Cephalometric analysis for diagnosis and treatment of obstructive sleep apnea. Laryngoscope 1988, 98: 226-234.         [ Links ]

7. Freed G, Pearman MA, Brown AS, Barot LR. Polysomnographic indications for surgical intervention in the Pierre Robin sequence: acute airway managment and follow-up studies after repair and take-down of tongue-lip adhesion. Cleft Palate J 1988, 25: 151-155.         [ Links ]

8. Gilhooly JT, Smith JD, Howell LL, Deschaine BL, Richey SL. Bedside polysomnography as an adjunct in the management of infants with Robin sequence. Plast Reconstr Surg 1993, 92: 23-27.         [ Links ]

9. Guilleminault C, Mondini S. Need for multi-diagnostic approaches before considering treatment in obstructive sleep apnea. Laryngsocope 1988, 98: 226-234.         [ Links ]

10. Guilleminault C, Riley R, Powel N. Sleep apnea and cephalometric measurements. Chest 1984, 86:793-794.         [ Links ]

11. Guilleminault C, Partinen M, Praud JP, Quera-Salva MA, Powel N, Riley R. Morphometric facial changes and obstructive sleep apnea in adolescents. J Pediatr 1989, 114: 997-999.         [ Links ]

12. Heaf DP, Helms PJ, Dinwiddie R, Matthew DJ. Nasopharyngeal airways in Pierre Robin sequence: effect of multidisciplinary evaluation and management. Pediatrics 1990, 86: 294-301.         [ Links ]

13. Jamieson A, Guilleminault C, Partinen M, Quera-Salva MA. Obstructive sleep apneic patients have craniomandibular abnormalities, Sleep 1986, 9: 469-477.         [ Links ]

14. Letica M, Adrich MS. Obstructive sleep apnea in Pierre Robin and related craniofacial syndromes. In: Association of Professional Sleep Societies Annual Meeting Abstracts. Phoenix, U.S.A.: Association of Professional Sleep Societies, 1992, p 171.(abstr).         [ Links ]

15. Ogborn MR, Pemberton PJ. Late development of airway obstruction in the Robin anomalad (Pierre Robin sequence) in the newborn. Aust Pediatr J 1985, 21: 199-200.         [ Links ]

16. Rechtschaffen A, Kales A. A Manual of standardized terminology, technique and scoring systems for sleep stages of human subjects. Los Angeles: Brain Information Service, University of Carlifornia, 1986.         [ Links ]

17. Reimão R. Contribuição ao estudo dos distúrbios do sono na infância. Tese. Faculdade de Medicina, Universidade de São Paulo, São Paulo, 1983.         [ Links ]

18. Reimão R. Teste das Latências Múltiplas do Sono: técnica, indicação e interpretação. Reun Neurofisiol Clin 1990, 5: 45-46.         [ Links ]

19. Reimão R, Diament A. Sono na infância. São Paulo: Sarvier, 1985.         [ Links ]

20. Riley RW, Guilleminault C, Herran J. Cephalometric analysis and flow volume loops in obstructive sleep apnea patients. Sleep 1983, 6: 304-317.         [ Links ]

21. Robin P. Glossoptosis due to atresia and hypotrophy of the mandibule. Am J Dis Child 1934, 48: 541-547.         [ Links ]

22. Series F, Pierre SS, Carrier G. Surgical correction of nasal obstruction in the treatment of mild sleep apnoea: importance of cephalometric in predicting outcome. Thorax 1993, 48: 360-363.         [ Links ]

23. Williams AJ, Williams MA. Walker CA, Bush PG. The Robin anomalad (Pierre Robin sequence): a follow-up study. Arch Dis Child 1981, 56: 663-668.         [ Links ]



Aceite: 29-abril-1994.

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