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Craniofacial

Anomalies

Name:

Dolichocephaly

Description:

Dolichocephaly is a long keel-shaped skull with prominent forehead and occiput. It may be associated with craniosynostosis of the sagittal suture; it may be commonly seen in the premature infant and is more frequently a normal variant. It is more common in preterm fetuses in the breech rather than cephalic presentation and in fetuses with oligohydramnios of long duration. Dolichocephalic fetuses with preterm premature rupture of membranes appear to be twice as likely to suffer respiratory distress syndrome than normocephalic infants. Individuals with dolichocephaly have a decreased cephalic index of 60 to 70 (CI = head width/head length x 100; normal is 70 to 80). The skull develops from mesenchyme, with the portion surrounding the brain termed the neurocranium. The neurocranium consists of a cartilaginous portion (chondrocranium) and a membranous portion. The chondrocranium is the cartilaginous base of the skull, which develops by fusion of several cartilages. The membranous neurocranium ossifies to form the cranial vault, the bones of which are separated by connective tissue membranes known as sutures. Six fibrous areas, the fontanelles, are also present. The five major sutures are the frontal, or metopic, sagittal, coronal, lambdoid and squamosal. The sutures allow for moulding to occur during the delivery process, and enlargement to accommodate normal brain growth. When the cranial bones fuse prematurely there is decreased growth potential. Bone deposition occurs abnormally, with the majority being directed away from the fused bony plate. In turn, perimeter sutures adjacent to the prematurely fused suture compensate in growth to a greater extent than sutures distant to the fused area. The primary cause of synostosis may be a defect at the cranial base at the site of initial attachment of the dura; other studies suggest a vascular defect causing deficient osteoclasia. Secondary craniosynostosis may occur because of brain abnormalities such as microcephaly or holoprosencephaly, or may be due to fetal constraint. Craniosynostosis has been reported to be present in 0.5 to 3.4 per 1000 live births. Sagittal synostosis accounts for 50%; coronal 22%; metopic 6%; and other or multiple 23%. Unilateral coronal synostosis is as common as bilateral. A predominance of males is noted, particularly for sagittal involvement. Craniosynostosis often causes alteration in head shape, ridging over the sutures, and/or fontanelle closure. Different terms have been used to describe the head shape alterations due to craniosynostosis, with resultant shape dependent upon the suture(s) involved.

Diagnosis:

Because sutures represent the boundaries of different cranial plates that are curvilinear, clear visualisation of overall structural detail often is difficult. The diagnosis of craniosynostosis is generally made on the basis of abnormal cephalic index (CI), coupled with other features of unusual head shape which include increased vertical diameter of the skull, frontal bossing/ prominent forehead, flat face, proptosis/shallow orbits, hypoplastic nasal bridge, and abnormal eye measurements. These differences are best appreciated if the face is evaluated in all three planes - sagittal, coronal and transverse. The cephalic index (CI) is obtained using a transverse or axial image at the maximum dimensions of the head. Biparietal and fronto-occipital diameters are measured, in both cases from the outer margin of the calvarium to the opposing outer margin. CI = BPD/FOD x 100. Mean CI is 78.3%; SD is 4.4%. Values below 70 are interpreted to be indicative of dolichocephaly (or scaphocephaly), while values above 86 indicate brachycephaly. Three-dimensional visualisation based on volumetric ultrasonographic image data offers the possibility of revealing the structural coherence of the sutures and fontanelles in the fetal skull more clearly. This technique allows imaging of coronal, lambdoidal and squamosal sutures, anterior, posterior, mastoid and sphenoid fontanelles. Identification of craniosynostosis should lead to a detailed search for associated anomalies which, in particular, affect the limbs - short limbs, polydactyly and syndactyly.

Differential Diagnosis:

Minor degrees of change in skull shape are part of the variation seen in the general population, and are not necessarily associated with craniosynostosis. This is particularly true of brachycephaly and dolichocephaly.

Sonographic Features:

CI less than 70 Unusual head shape

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Associated Syndromes


3M
Adrenoleukodystrophy
Bloom
Bowen-Conradi
Cranioectodermal dysplasia
Currarino: severe congenital contractural arachnodactyly
FG
Galactosialidosis
German
Glutaric aciduria, type II
Hurler
Jones
Lin-Getting
Marfan
Marshall-Smith
Mevalonic aciduria
Sanfilippo
Setleis
Sialidosis, type 2
Sotos
Tricho-dento-osseous type I

References


Prenatal visualisation of cranial sutures and fontanelles with three-dimensional ultrasonography
Pretorius DH, Nelson TR
J Ultrasound Med 13: 871-876

Fetal facial defects: associated malformations and chromosomal abnormalities
Nicolaides KH, Salveson DR, Snijders RJM, Gosden CM
Fetal Diagn Ther 8: 1-9

Estimating fetal age: effect of head shape on BPD
Hadlock FP, Deter Rl, Carpenter RJ, Park SK
Am J Roentgenol 137: 83-5

Craniosynostosis update 1987
Cohen MM Jr
Am J Med Genet Suppl 4: 99-148

In: Human Malformations and Related Anomalies
Toriello H
Oxford University Press, New York, p 589-602

Dolichocephaly and oligohydramnios in preterm premature rupture of the membranes.
Levine D; Kilpatrick S; Damato N; Callen PW
J Ultrasound Med May;15(5):375-9

The differential diagnosis of abnormal head shapes: separating craniosynostosis from positional deformities and normal variants.
Huang MH; Mouradian WE; Cohen SR; Gruss JS
Cleft Palate Craniofac J May;35(3):204-11