ISCFS 2019 Abstract Supplement
Metopic Synostosis
Introduction: The Craniofacial Collaboration UK (the CC-
UK) is a Clinical Psychology-led developmental screening
pathway for patients from the 4 Highly Specialised Cranio-
facial Centres in the UK (HSCC). The current paper is the
first comparison across suture types, examining the results
for 3 and 5-year-old children with sagittal (SS) and me-
topic (MS) synostosis. This is the first step towards under-
standing differences in the presentation of these different
diagnostic groups, with the aim of facilitating evidence-led
targeting of resources to greatest need.
Methods: Parents of 3 and 5-year-old children were asked
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to complete questionnaires regarding both developmental
and behavioural aspects: the Ages and Stages Questionnaire
(ASQ) and the Strengths and Difficulties Questionnaire
(SDQ). Questionnaires are given to families in advance of
attendance at one of the HSCC. As both questionnaires are
widely used standardised tools, results can be compared
with normative data.
Children were excluded from the analysis if they had not
previously undergone primary corrective surgery, or had a
known diagnosis with a genetic or other condition (such as
epilepsy) which might be expected to impact their devel-
opmental progress.
Results: Response rate ranged from 51% for the SDQ in
3-year olds with SS to 81% for the ASQ at 3. ASQ data
are reported for 375 children and SDQ data for 275. Com-
parison with normative data indicated no major deficits or
delays for the majority of children with both SS and MS
groups at 3 and 5 years. However, there was a consistent
difference in results between the two diagnostic groups at
both age groups, with lower mean parental ratings across
all domains of the ASQ and greater numbers of concerns
related to behavioural and social functioning for both age
groups for children with MS on the SDQ.
Conclusion: Whilst the majority of children in both diag-
nostic groups fell within the range that would be expected
for their age, children with MS were rated by their parents
as performing less well on both developmental and behav-
ioural measures across all domains. This is an important
indication that there may be differences in performance
related to diagnostic group. Despite attempts to exclude
children with known conditions, it is important to consider
the higher rate of chromosomal abnormalities and other
conditions, such as in vitro exposure to medication, that are
present in MS and could have an impact on development.
S8A-03 SESSION 8A: METOPIC
SYNOSTOSIS
NEUROPSYCHOLOGICAL,
BEHAVIOURAL AND PSYCHOLOGICAL
FUNCTIONING OF CHILDREN AND
Session 8A:
ADULTS WITH UNOPERATED METOPIC
SYNOSTOSIS
Osborn A.1,*, Roberts R.1, Mathias J.1,
Flapper W.2, Anderson P.2
1
School of Psychology, The University of
Adelaide, Adelaide, 2The Australian Cra-
niofacial Unit, Women’s and Children’s
Hospital, North Adelaide, Australia
Introduction: Research examining the cognitive, behav-
ioural and psychological outcomes of people with unop-
erated metopic synostosis (MS) is limited, but a recent
meta-analysis has reported that this group functioned
more poorly in each domain when compared to normative
samples or healthy controls. The current study extends on
this by assessing multiple cognitive (general cognition, ver-
bal & visuo-spatial abilities, attention, working memory,
information processing, executive functions), behavioural
(behaviour problems) and psychological (depression, anxi-
ety, satisfaction with appearance) outcomes in children and
adults who were diagnosed with MS, but did not undergo
corrective surgery.
Methods: Age-appropriate tests were selected to assess cog-
nition (Wechsler Preschool & Primary Scale of Intelligence,
Wechsler Intelligence Scale for Children, Wechsler Adult
Intelligence Scale or Wechsler Abbreviated Scale of Intel-
ligence; Developmental Neuropsychological Assessment
or Delis-Kaplan Executive Function System). Behavioural
and psychological outcomes were assessed using self-report
measures (Behaviour Rating Inventory of Executive Func-
tion; Strengths and Difficulties Questionnaire; Depression,
Anxiety & Stress Scale; Revised Child Anxiety & Depres-
sion Scale; Satisfaction with Appearance Scale). All persons
diagnosed with MS and treated conservatively at the Aus-
tralian Craniofacial Unit were approached, with NMS = 38
agreeing to participate (aged 2–29 years). Healthy age- and
sex-matched controls were additionally recruited from the
general community (Ncontrols = 27, recruitment ongoing), with
socio-economic status being used as a control variable.
Results: Preliminary analyses indicate that those with un-
operated MS are functioning at significantly lower levels
than their healthy peers in multiple cognitive domains (gen-
eral cognition, verbal & visuo-spatial abilities, executive
functioning), with these differences equating to medium to
large effect sizes. However, working memory and process-
ing speed appear to be unaffected. Persons with unoperated
MS also experience slightly more behavioural problems
(significant but small effects), but are not more depressed
or anxious, and no less satisfied with their appearance.
79

Conclusion: All patients with unoperated MS should be
monitored on an ongoing basis in order to ensure that they
receive appropriate support services to assess and manage
the cognitive problems that they may experience.
S8A-04 SESSION 8A: METOPIC
SYNOSTOSIS
CLOMIPHENE CITRATE AND
ENCLOMIPHENE HYDROCHLORIDE
EXPOSURE IS ASSOCIATED
WITH INTERFRONTAL SUTURE
ABNORMALITIES IN ZEBRAFISH (DANIO
RERIO)
Franz-Odendaal T.1, Bezuhly M.2,*
1
Department of Biology, Mount Saint
Vincent University, 2Division of Plastic
Surgery, Dalhousie University, Halifax,
Canada
Introduction: Craniofacial centres have observed a dra-
matic increase in the incidence of metopic craniosynostosis
over the last two decades. The underlying reason for this
rise in incidence is unknown, but some researchers have
speculated that pharmacological exposure in uteromay be
at play. Large population-based registries have pointed a
potential link between the use of fertility drug use and the
incidence of craniosynostosis. Here, we hypothesize that
clomiphene citrate and/or enclomiphene hydrochloride, the
active compounds in common fertility drugs in use over the
last two decades, could be cause metopic craniosynostosis.
Methods: Zebrafish (Danio rerio) are a valuable model
to study human disease and offer a number of advantages
over mammalian models. Zebrafish larvae were treated
with clomiphene citrate or enclomiphene hydrochloride
for five days at key points during calvariae development
(6.5 mm SL and 5.5 mm SL). Larval fish were then trans-
ferred to regular rearing water and raised until adulthood.
The zebrafish skeleton was analysed using a whole-mount
skeletal stain. Control fish were treated with the solvent or
were untreated.
Results: More than half (58%) of the treated fish exhib-
ited a delay in overall body growth compared to controls.
Furthermore, this delay was more striking in the fish that
were treated slightly later compared to earlier treatments.
Treatments with each chemical at 5.5mm SL affected cal-
variae development, particularly the interfrontal suture. The
overall head shape of these fish was broader caudally than
control fish.
80
PRS Global Open • 2019
Conclusion: The active components of common fertility
drugs appear to affect development of the anterior region
of the zebrafish skull, in particular the interfrontal suture.
Further research is required to explore these findings in
depth and to uncover the signaling mechanism involved.
S8A-05 SESSION 8A: METOPIC
SYNOSTOSIS
SCHOOL-AGE AESTHETIC OUTCOMES
IN CHILDREN WITH METOPIC
CRANIOSYNOSTOSIS AFTER
ENDOSCOPIC OR OPEN TREATMENT
Ha A. Y. S.1, Nguyen D.1, Skolnick G.1,
Naidoo S.1, Smyth M.2, Patel K.1,*
1
Plastic Surgery, 2Neurosurgery, Wash-
ington University in St. Louis, St. Louis,
United States
Introduction: One-year postoperative morphologic out-
comes after endoscopic treatment of metopic craniosyn-
ostosis have been shown to be equivalent to those after
fronto-orbital advancement. The authors hypothesized that
endoscopic and open repair would yield equivalent mor-
phologic outcomes at 5 years post-operative.
Methods: This study was a retrospective chart review of 15
consecutive non-syndromic patients with isolated metopic
craniosynostosis treated endoscopically with post-operative
helmeting. A gender-matched cohort of 17 patients treated
by fronto-orbital advancement was included for compari-
son. Primary morphologic outcomes were frontal width,
interfrontal divergence angle, the presence of lateral fron-
tal retrusion, and the Whitaker classification. Periopera-
tive variables included estimated blood loss, rates of blood
transfusion, length of stay, and operating time.
Results: There were no statistically significant differences
in frontal width and interfrontal divergence angle between
the endoscopic and open groups at 5 years post-operative.
However, the endoscopic group had significantly more pa-
tients classified as Whitaker Class I and significantly fewer
patients with lateral frontal retrusion compared to the open
group. The endoscopic group were younger at the time of
surgery and had improved perioperative outcomes related
to operating room time, hospital stay and blood loss. Both
groups had low complication and re-operation rates.
Conclusion: In our cohort of school-age children with
isolated metopic craniosynostosis, qualitative morphologic
outcomes following endoscopic repair were superior to