Three-dimensional morphological analysis of isolated metopic synostosis.
Morphological differences were quantified in three-dimensions among individuals with untreated isolated metopic synostosis and between those individuals and similar aged-matched normal dry skulls to test two hypotheses: first, that the dysmorphology is a self-correcting condition; and second, that a lack of vertical growth of the skull produces this dysmorphology. Three-dimensional (3D) coordinates were recorded for 22 craniofacial landmarks from CT scans of 15 metopic patients, ranging from 5- to 32-months-old, and of four normal dry skulls, ranging in age from 6- to 36-months-old. The patient population was diagnosed with isolated metopic synostosis at The Johns Hopkins Medical Institutions in Baltimore, Maryland or Children's Hospital in St. Louis, Missouri. Comparisons between the metopic age groups indicate that the trigonocephalic phenotype worsens through time. Between 5 and 14 months, the neurocranium displays an increase in vertical growth. This was followed by a lack of vertical growth between 14 and 32 months. The face displays a lack of vertical growth from 5 to 14 months and an increase in vertical growth after 14 months. Comparisons between the metopic age groups and the normal skulls indicate that the trigonocephalic head is taller superoinferiorly and longer anteroposteriorly. Relative to the normal phenotype, the inferior temporal region in the metopic phenotype is narrow. These findings enabled the rejection of both hypotheses and localized form differences between normal and metopic phenotypes. Based on these results, we suggest that the trigonocephalic phenotype worsens with age and the amount of vertical growth that produces the trigonocephalic phenotype varies throughout growth with respect to location within the skull and age.
Zumpano
M P
MP
The Johns Hopkins School of Medicine, Department of Cell Biology and Anatomy, Baltimore, Maryland 21205, USA. mzumpano@nycc.edu
Carson
B S
BS
Marsh
J L
JL
Vanderkolk
C A
CA
Richtsmeier
J T
JT
D000367:Age Factors; D016022:Case-Control Studies; D002675:Child, Preschool; D003398:Craniosynostoses / Q000473:pathology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008954:Models, Biological; D010641:Phenotype; D011856:Radiographic Image Enhancement; D012886:Skull / Q000033:anatomy & histology / Q000254:growth & development / Q000473:pathology; D014057:Tomography, X-Ray Computed