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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

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

http://dx.doi.org/10.1590/S0004-282X1994000400017 

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

 

 


SUMMARY

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.


RESUMO

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.

 

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Aceite: 29-abril-1994.

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