Chest
Clinical InvestigationsSLEEPRelationship Between Craniofacial Abnormalities and Sleep-Disordered Breathing in Marfan’s Syndrome
Section snippets
Patients
Fifteen consecutive adult patients (7 men and 8 women) with Marfan’s syndrome presenting for routine follow-up at a specialist Marfan’s syndrome clinic of a university teaching hospital were studied. All patients fulfilled the diagnostic criteria for Marfan’s syndrome.4 Informed consent was obtained from all patients, and the study was approved by the ethics committee of our institution.
Sleep Studies
All of the patients underwent standard nocturnal polysomnography testing, as previously described.6 Sleep
Results
All patients were white and had the typical clinical features of Marfan’s syndrome (ie, they were generally young, tall, and thin; Table 1). Men were significantly taller than women (188 ± 3 vs 175 ± 2 cm; p < 0.01) and had a greater neck circumference (37.0 ± 1.0 vs 34.0 ± 0.5 cm; p < 0.05). Thirteen of the 15 patients had OSA, which was defined as an AHI more than five episodes per hour. The severity of apnea in these patients was mild to moderate, with a mean AHI of 22 ± 15 episodes per hour
Discussion
This cephalometric study of patients with Marfan’s syndrome provides evidence that such patients have a high prevalence of craniofacial abnormalities. The finding of a relationship between indexes of apnea severity and various cephalometric measurements suggests that these structural abnormalities are likely to play a role in predisposing these individuals to OSA.
Various craniofacial abnormalities have been described in patients with Marfan’s syndrome, although the literature on this topic has
Acknowledgment
The authors gratefully acknowledge the assistance of the staff of the Sleep Disorders Center and Dr. Richmond Jeremy from the Marfan Clinic at Royal Prince Alfred Hospital. We thank Dr. Anthony Pistolese for his analysis of the cephalometric radiographs.
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Supported by a National Health and Medical Research Council of Australia Scholarship and the Australian Lung Foundation/Sensormedics Fellowship in Sleep Disorders (PAC).