Syndromic Multisuture Craniosynostosis With Associated Anterior Segment Dysgenesis, Optic Nerve Hypoplasia, and Congenital Glaucoma

Schultz, Kelly P.; Wiggins, Claire J.; Streff, Haley; Shah, Veeral S.; Buchanan, Edward P.

doi:10.1177/1055665618820481

Cited by 8 publications

(7 citation statements)

References 12 publications

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“…9 Less common etiologies of visual impairment, such as optic nerve hypoplasia and congenital glaucoma, have also been documented in association with craniosynostosis. 10 The role of the ophthalmologist in the care of patients with craniosynostosis is, therefore, critical and involves prompt detection of elevated ICP, expeditious diagnosis and treatment of amblyopia in early childhood, and specific management for other ocular manifestations that may be present. This review aims to explore currently recognized neuro-ophthalmological symptoms of craniosynostosis and discuss evaluation and management considerations for the pediatric ophthalmologist seeking to maximize the preservation of vision.…”

Section: Introductionmentioning

confidence: 99%

Neuro-Ophthalmological Manifestations of Craniosynostosis: Current Perspectives

Duan¹,

Skoch²,

Pan³

et al. 2021

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Craniosynostosis, a premature fusion of cranial sutures that can be isolated or syndromic, is a congenital defect with a broad, multisystem clinical spectrum. The visual pathway is prone to derangements in patients with craniosynostosis, particularly in syndromic cases, and there is a risk for permanent vision loss when ocular disease complications are not identified and properly treated early in life. Extensive advancements have been made in our understanding of the etiologies underlying vision loss in craniosynostosis over the last 20 years. Children with craniosynostosis are susceptible to interruptions in visual input arising from strabismus, refractive errors, and corneal damage; any of these aberrations can result in understimulation of the visual cortex during childhood neurodevelopment and permanent amblyopia. Elevated intracranial pressure resulting from abnormal cranial shape or volume can lead to papilledema and, ultimately, optic atrophy and vision loss. A pediatric ophthalmologist is a crucial component of the multidisciplinary care team that should be involved in the care of craniosynostosis patients and consistent ophthalmologic follow-up can help minimize the risk to vision posed by such entities as papilledema and amblyopia. This article aims to review the current understanding of neuro-ophthalmological manifestations in craniosynostosis and explore diagnostic and management considerations for the ophthalmologist taking care of these patients.

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Section: Introductionmentioning

confidence: 99%

Neuro-Ophthalmological Manifestations of Craniosynostosis: Current Perspectives

Duan¹,

Skoch²,

Pan³

et al. 2021

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“…4,13 Furthermore, late intervention can result in chronically elevated ICP, which can contribute to visual impairment. 7,8 The surgery itself is also more complex and poses further operative risks such as increased blood loss. 13 Our patient additionally presented with cochlear implants requiring that we utilize bipolar cautery due to the presumed risk that monopolar cauterization may damage the device and that the heat generated by it may injure auditory neurons.…”

Section: Discussionmentioning

confidence: 99%

“…Delayed operative management in syndromic patients not only can impact brain development resulting in intellectual disability, but can also affect overall cosmesis due to progression of deformity of the cranial base 4,13 . Furthermore, late intervention can result in chronically elevated ICP, which can contribute to visual impairment 7,8 . The surgery itself is also more complex and poses further operative risks such as increased blood loss 13 .…”

Section: Discussionmentioning

confidence: 99%

“…[3][4][5] Patients also frequently present with characteristic extra-cranial manifestations including midface anomalies, orbital involvement (ie exophthalmos, hypertelorism, vision loss), and limb defects. 4,6,7 Chiari malformations and hydrocephalus are often linked to syndromic cases, increasing the medical complexity of these patients. 8 Consequently, a multidisciplinary approach is necessary to manage these patients.…”

mentioning

confidence: 99%

“…Syndromic cases are more likely to present with multi-suture involvement contributing to craniofacial dysmorphisms, elevated intracranial pressure (ICP), and restricted brain growth resulting in intellectual disability 3–5 . Patients also frequently present with characteristic extra-cranial manifestations including midface anomalies, orbital involvement (ie exophthalmos, hypertelorism, vision loss), and limb defects 4,6,7 . Chiari malformations and hydrocephalus are often linked to syndromic cases, increasing the medical complexity of these patients 8 .…”

mentioning

confidence: 99%

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Late Presenting Multi-Suture Craniosynostosis

Dibbs

Beh

Donoho

et al. 2021

Journal of Craniofacial Surgery

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Skeletal expansion via craniofacial distraction osteogenesis technique in syndromic craniosynostosis: impact on ophthalmic parameters

Kai,

Khaliddin,

Hassan

et al. 2024

Int Ophthalmol

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BackgroundThis study aims to compare the changes in ophthalmic parameters among syndromic craniosynostosis patients who underwent craniofacial skeletal expansion procedures via distraction osteogenesis (DO). MethodA retrospective study was conducted involving syndromic craniosynostosis patients who underwent surgical expansion via the DO technique from the year 2012 to March 2022. Changes in six parameters which consist of visual acuity, refractive error, optic disc health, intraocular pressure, degree of proptosis and orbital volume were measured objectively pre and post-surgery. For categorical parameters, the Chi-square cross-tab test was done. Paired sample T-test was used for normally distributed variables. Wilcoxon signed-rank test was used for non-normally distributed data. ResultsVisual impairment was present in 21.4% of eyes before surgery and increased to 28.5% post-surgery. Three patients had changes of refractive error postsurgery with one developed hypermetropia, another developed anisometropia and the last had improvement to no refractive error. Two patients had optic disc swelling which was resolved post-surgery. Intraocular pressure changes were inconsistent post-surgery. All patients achieved a signi cant reduction in the degree of proptosis post-surgery. Orbital volume calculation using computed tomography (CT) scans shows a signi cant increase in volume post-surgery for all patients. ConclusionOur study shows a signi cant increase in orbital volume post-surgery with a reduction in the degree of proptosis. Optic disc and nerve health improved after the surgery. Changes in terms of visual acuity, refractive error and IOP were inconsistent after the surgical intervention.

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Syndromic Multisuture Craniosynostosis With Associated Anterior Segment Dysgenesis, Optic Nerve Hypoplasia, and Congenital Glaucoma

Cited by 8 publications

References 12 publications

Neuro-Ophthalmological Manifestations of Craniosynostosis: Current Perspectives

Neuro-Ophthalmological Manifestations of Craniosynostosis: Current Perspectives

Late Presenting Multi-Suture Craniosynostosis

Skeletal expansion via craniofacial distraction osteogenesis technique in syndromic craniosynostosis: impact on ophthalmic parameters

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