Black bone MRI in unisutural craniosynostosis patients.

In unisutural craniosynostosis, a single suture of the skull vault is closed
before birth, and normal growth of the skull and brain is restricted. This
particularly involves the sagittal suture, the metopic suture and the coronal
suture. Besides an abnormal skull shape, additional related problems are
encountered. Children with sagittal synostosis are reported to have a higher
prevalence of speech/language impairment, while metopic synostosis puts
children at risk for disturbed behaviour, particularly attention deficiency
hyperactivity disorder (ADHD) and autism, and amblyopia (Nguyen 2014). Coronal
synostosis is specifically related to visual disturbances, such as strabismus
and amblyopia. These associated problems can only be detected at an older age,
when screening of speech/language, behaviour, and vision can be performed
reliably. Screening is routinely done at the ages of 2, 4 and 6 years of age as
part of the clinical protocol. It is unknown why these children have a
synostosis-specific higher risk on associated problems, and whether or not this
can be predicted early on. Early detection of neurocognitive and visual
deficiencies would enable early management and improved counselling of the
parents.

Specifically for metopic synostosis there is a debate on whether or not to
perform surgery for the moderate phenotype (Anolik et al., 2016; Birgfeld et
al., 2013). The earlier the fusion of the suture occurs during embryogenesis,
the more severe the phenotype is. Treatment of the severe presentation of
trigonocephaly consist of surgical correction of the frontal bones and
supra-orbital rims, for which there is consensus on surgical indication. There
is however no consensus whether or not to perform surgery for the intermediate
form, because the benefits regarding neurocognitive functions are unknown.

Primary Objective:
To validate the predictive value of MRI data for neurocognitive deficits and
visual functioning by relating these data from newborns with unisutural
craniosynostosis to the psychological and ophthalmologic test results, taken
routinely at the ages of 2, 4 and 6 years.

Secondary Objective: To test ASL as an objective criterium for a surgical
indication by comparing cerebral blood flow in trigonocephaly with norm data
and by relating it to the severity of the phenotype.

An observational pilot study of a cohort with longitudinal follow-up. The study
will take around 2 years of inclusion for BB MRI. The number of referred
patients per year is 40 for sagittal synostosis, 15 for metopic synostosis, and
5 for unicoronal synostosis, and will thus have a total of 100 potential
patients for inclusion. The final results of the study will take 7 years to
have the final psychological test at age 6 included. No extra visits to the
hospital will be required for the participants, as we can combine the MRI
procedure with other routinely scheduled visits. Within out center, we have
age-specific control data for both DTI and ASL.

The benefit of participation is the avoidance of radiation that is associated
with a 3D-CT scan of the head. Another potential advantage could be the early
detection of intrinsic brain disorders that are associated with neurocognitive
impairments, enabling earlier treatment by psychologists and ophthalmologist.
The risk lies in having anesthesia for a period of 45 minutes. The associated
risks for anesthesia are very low, based on our own experience within the
Sophia Children*s Hospital; in patients with syndromic craniosynostosis a brain
MRI is taking with the use of anesthesia according to protocol, as a high
prevalence of brain anomalies are present in this group, and no complications
have been encountered.