Metopic suture in fetuses with Apert syndrome at 22-27 weeks of gestation.
OBJECTIVE
To examine the possible association of skull deformity and the development of the cranial sutures in fetuses with Apert syndrome.


METHODS
Three-dimensional (3D) ultrasound was used to examine the metopic and coronal sutures in seven fetuses with Apert syndrome at 22-27 weeks of gestation. The gap between the frontal bones in the transverse plane of the head at the level of the cavum septi pellucidi was measured and compared to findings in 120 anatomically normal fetuses undergoing routine ultrasound examination at 16-32 weeks.


RESULTS
In the normal group, the gap between the frontal bones in the metopic suture at the level of the cavum septi pellucidi, decreased significantly with gestation from a mean of 2.2 mm (5th and 95th centiles: 1.5 mm and 2.9 mm) at 16 weeks to 0.9 mm (5th and 95th centiles: 0.3 mm and 1.6 mm) at 32 weeks. In the seven cases with Apert syndrome, two-dimensional ultrasound examination demonstrated the characteristic features of frontal bossing, depressed nasal bridge and bilateral syndactyly. On 3D examination there was complete closure of the coronal suture and a wide gap in the metopic suture (15-23 mm).


CONCLUSIONS
In normal fetuses, cranial bones are believed to grow in response to the centrifugal pressure from the expanding brain and proximity of the dura to the suture is critical in maintaining its patency. In Apert syndrome, the frontal bossing may be a mere consequence of a genetically predetermined premature closure of the coronal suture. Alternatively, there is a genetically predetermined deformation of the brain, which in turn, through differential stretch of the dura in the temporal and frontal regions, causes premature closure of the coronal suture and impaired closure of the metopic suture.
Faro
C
C
Harris Birthright Research Centre for Fetal Medicine, King's College Hospital Medical School, London, UK.
Chaoui
R
R
Center for Prenatal Diagnosis and Human Genetics, Berlin, Germany.
Wegrzyn
P
P
Harris Birthright Research Centre for Fetal Medicine, King's College Hospital Medical School, London, UK.
Levaillant
J M
JM
Department of Obstetrics and Gynecology, Centre Hospitalier Intercommunal, Créteil, France.
Benoit
B
B
Maternite, Hopital Princesse Grace, Monaco.
Nicolaides
K H
KH
Harris Birthright Research Centre for Fetal Medicine, King's College Hospital Medical School, London, UK.
D000168:Acrocephalosyndactylia / Q000000981:diagnostic imaging / Q000196:embryology*; D000293:Adolescent; D000328:Adult; D003393:Cranial Sutures / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000196:embryology*; D005260:Female; D005865:Gestational Age; D006801:Humans; D008423:Maternal Age; D011247:Pregnancy; D012886:Skull / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000196:embryology; D016216:Ultrasonography, Prenatal / Q000379:methods