Craniofacial Dysmorphology in Infants With Non-Syndromic Unilateral Coronal Craniosynostosis

Bellaire, Christopher P; Devarajan, Alex; Napoli, James G.; Rutland, John W; Liu, Helen; Jacob, Laya; Mandelbaum, Max; Sayegh, Farah; Margulies, Ilana G.; Goel, Pedram; Delman, Bradley N.; Urata, Mark M.; Taub, Peter J.

doi:10.1097/scs.0000000000008464

Cited by 3 publications

(3 citation statements)

References 29 publications

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“…The present study leverages existing advanced shape analysis imaging methods to quantify foraminal and orbital volumes to add to the literature on anatomical morphometrics in UCS. 22,23 The study demonstrates significant asymmetries in a variety of orbital structures among infants with UCS. The data indicate that the orbits on synostotic sides were narrower and had less volume overall than the contralateral side, which is consistent with current literature.…”

Section: Discussionmentioning

confidence: 77%

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Orbital Foraminal Morphometrics in Nonsyndromic Unilateral Coronal Craniosynostosis

et al. 2023

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Nonsyndromic unilateral coronal craniosynostosis (UCS) is a rare congenital disorder that results from premature fusion of either coronal suture. The result is growth restriction across the suture, between the ipsilateral frontal and parietal bones, leading to bony dysmorphogenesis affecting the calvarium, orbit, and skull base. Prior studies have reported associations between UCS and visual abnormalities. The present study utilizes a novel geometric morphometric analysis to compare dimensions of orbital foramina on synostotic versus nonsynostotic sides in patients with UCS. Computed tomography head scans of pediatric UCS patients were converted into 3-dimensional mesh models. Anatomical borders of left and right orbital structures were plotted by a single trained team member. Dimensions between synostotic and nonsynostotic sides were measured and compared. Medical records were examined to determine prevalence of visual abnormalities in this patient cohort. Visual abnormalities were reported in 22 of the 27 UCS patients (77.8%). Astigmatism (66.7%), anisometropic amblyopia (44.4%), and motor nerve palsies (33.3%) represented the 3 most prevalent ophthalmologic abnormalities. Orbits on synostotic sides were 11.3% narrower (P < 0.001) with 21.2% less volume (P = 0.028) than orbits on nonsynostotic sides. However, average widths, circumferences, and areas were similar between synostotic and nonsynostotic sides upon comparison of supraorbital foramina, infraorbital foramina, optic foramina, and foramina ovalia. Therefore, previously proposed compression or distortion of vital neurovascular structures within bony orbital foramina does not seem to be a likely etiology of visual abnormalities in UCS patients. Future studies will examine the role of ocular and/or neuro-ophthalmologic pathology in this disease process.

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Section: Discussionmentioning

confidence: 77%

“…Prior studies have not evaluated the morphometrics of important orbital foramina structures in patients with UCS. The present study leverages existing advanced shape analysis imaging methods to quantify foraminal and orbital volumes to add to the literature on anatomical morphometrics in UCS 22,23 …”

Section: Discussionmentioning

confidence: 99%

Orbital Foraminal Morphometrics in Nonsyndromic Unilateral Coronal Craniosynostosis

et al. 2023

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“…Unilateral coronal synostosis (UCS) not only results in cosmetic malformation with ipsilateral flattening of the frontal and parietal bones, temporal retrusion with elevation and recession of the supraorbital rim (also known as “harlequin” eye deformity) ( 1 ). Of note, single-suture craniosynostosis may also result in a significant volumetric reduction of the skull cavity on the affected side with consequent functional impact due to potential increases in intracranial pressure (ICP) in about 15–20% of children ( 2 ).…”

Section: Introductionmentioning

confidence: 99%

Bilateral fronto-orbital advancement combined with cranial vault release using a free-floating bone flap technique for nonsyndromic unilateral coronal synostosis

Liang,

Chen,

Yang

et al. 2023

Transl Pediatr

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Background The goals of operative treatment for unilateral coronal synostosis (UCS) are to improve appearance and allow unrestricted brain growth. However, for severe unilateral premature closure of the coronal suture, existing methods do not address the compression of the brain or expand the volume of the skull cavity. We report our retrospective experience with bilateral fronto-orbital advancement combined with cranial vault release using a free-floating bone flap (CVR + FFBF) technique and the resulting changes in the anterior cranial vault asymmetry index (ACVAI) and intracranial volume. Methods Twenty patients with UCS who underwent bilateral fronto-orbital advancement combined with CVR + FFBF technique from April 2014 to May 2019 were included. Surgical efficacy was evaluated by the ACVAI and intracranial volume before the operation, 1 week after the operation, and at the last follow-up (average 19.8 months; range, 12 to 40 months). The measurement data are presented as the mean ± standard deviation and were statistically analyzed by t -test. Results The ACVAI was 9.07%±3.55% before the operation, 3.56%±3.42% 1 week after the operation, and 3.13%±2.41% at the last follow-up. The ACVAI 1 week after the operation was significantly lower than that before the operation ( t =4.827, P<0.001). There was no significant difference between the ACVAI 1 week after the operation and at the last follow-up ( t =0.660, P=0.517). The intracranial volume was 1,027.85±112.25 mL in patients before the operation and 1,131.92±161.71 mL in the normal control group, which was a statistically significant difference ( t =2.364, P=0.023). The intracranial volume significantly increased 1 week after surgery: 1,081.62±111.10 mL ( t =8.703, P<0.001), and this trend continued at the last follow-up (1,386.90±119.30 mL) similarly to the normal control group (1,438.22±89.28 mL). At the last follow-up, there was no significant difference between the two groups ( t =1.540, P=0.132). Conclusions For the treatment of UCS, bilateral fronto-orbital advancement combined with CVR + FFBF technique offers functional and cosmetic outcomes in terms of intracranial volume expansion and fronto-orbital symmetry.

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Variations in orbital morphology, globe:orbit volume relation, and ophthalmological outcome in unicoronal synostosis

Lif,

Ntoula,

Larsson

et al. 2024

JPHS

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Nonsyndromic unicoronal synostosis is associated with variability of severity in orbital morphology and ophthalmological manifestations. The relation between the two is not fully understood, nor how surgical treatment with fronto-orbital advancement and remodelling (FOAR) changes the relation. The aim of this study was to elucidate associations between ophthalmological manifestations and variations in orbital morphology and globe:orbit volume ratios preoperatively and at long-term follow-up after surgery. Twelve children referred to Uppsala Craniofacial Center who underwent computed tomography and standardized ophthalmological examinations regarding strabismus, spherical equivalent, astigmatism, anisometropia, and subnormal vision preoperatively and at 3 years of age were included. Orbits and globes were segmented. Principal component analysis elucidated morphological variation, and symmetry between orbital pairs was measured as the Dice similarity coefficient and globe:orbit volume ratios were calculated. The defined orbital shape variations were correlated with strabismus, refractive error, and subnormal vision. Different shape variations were associated with strabismus pre- and postoperatively and ipsi- and contralateral astigmatism. Greater improvement in orbital symmetry after surgery was associated with improvement in astigmatic anisometropia and new onset strabismus at follow-up. A small globe:orbit volume ratio was associated with preoperative strabismus, while the opposite was seen at follow-up. Different mechanisms seem to cause strabismus pre- and postoperatively, and FOAR might not sufficiently correct orbital morphology.

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Craniofacial Dysmorphology in Infants With Non-Syndromic Unilateral Coronal Craniosynostosis

Cited by 3 publications

References 29 publications

Orbital Foraminal Morphometrics in Nonsyndromic Unilateral Coronal Craniosynostosis

Orbital Foraminal Morphometrics in Nonsyndromic Unilateral Coronal Craniosynostosis

Bilateral fronto-orbital advancement combined with cranial vault release using a free-floating bone flap technique for nonsyndromic unilateral coronal synostosis

Variations in orbital morphology, globe:orbit volume relation, and ophthalmological outcome in unicoronal synostosis

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