Pa. Cistulli et al., Relationship between craniofacial abnormalities and sleep-disordered breathing in Marfan's syndrome, CHEST, 120(5), 2001, pp. 1455-1460
Citations number
34
Categorie Soggetti
Cardiovascular & Respiratory Systems","Cardiovascular & Hematology Research
Objectives: To examine the prevalence and nature of craniofacial abnormalit
ies in patients with Marfan's syndrome and to investigate the relationship
between craniofacial abnormalities and obstructive sleep apnea (OSA) severi
ty in these patients.
Design: Cross-sectional.
Setting: Marfan's syndrome clinic in a tertiary teaching hospital.
Patients: Fifteen consecutive adult patients (7 men and 8 women; mean [+/-
SD] age, 34.8 +/- 13.2 years) who had Marfan's syndrome.
Measurements and results: Apneic status was determined from standard overni
ght polysomnography testing. Measurements from standardized lateral cephalo
metric radiographs were compared to normative data. Thirteen patients had O
SA, which was defined as an apnea/hypopnea index (AHI) of >5 episodes per h
our (mean AHI, 22 +/- 15 episodes per hour). A high prevalence of craniofac
ial abnormalities was found with significant gender differences for some of
the variables. Significant abnormalities for the entire group were bimaxil
lary retrusion, a reduced maxillary length, an increased total anterior fac
e height, a long lower anterior face height, an obtuse gonial angle, a stee
p mandibular plane, a reduced posterior nasal airway height, a reduced post
erior airway space, and an increased distance from the mandibular plane to
the hyoid bone. Univariate analysis revealed significant correlations among
the total anterior face height, the upper anterior and posterior face heig
hts, the mandibular length, and AHI. There was a significant correlation be
tween the rank of the number of cephalometric abnormalities per patient and
AHI in those patients with OSA.
Conclusions: Craniofacial abnormalities are common in patients with Marfan'
s syndrome. The relationship between some cephalometric parameters and apne
a severity suggests a potential role of craniofacial structure in the patho
genesis of OSA in these patients.