Visual outcomes in children with syndromic craniosynostosis: a review of 165 cases

Hinds, Anne-Marie; Thompson, Dorothy; Rufai, Sohaib R; Weston, Kelly; Schwiebert, Kemmy; Panteli, Vasiliki; James, Greg; Bowman, Richard

doi:10.1038/s41433-021-01458-5

Cited by 11 publications

(21 citation statements)

References 18 publications

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“…Out of all ONH parameters, Patel et al 14 found that nasal and temporal RNFLs demonstrated the lowest interexaminer ICCs (0.77 and 0.68, respectively). Patel et al 13 also found that success rates dipped in toddlerhood and then increased with age, consistent with our findings.…”

Section: Research In Contextsupporting

confidence: 93%

“…1). Although our craniofacial unit has utilized other ophthalmological investigations in children with craniosynostosis, 13 we had not yet utilized handheld OCT in these patients prior to this study. Handheld OCT has been utilized to describe the normal development of the optic nerve 14 and fovea 15 in children, with excellent feasibility.…”

Section: Neurosurgical Intervention Involves Expanding Thementioning

confidence: 99%

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Feasibility and Repeatability of Handheld Optical Coherence Tomography in Children With Craniosynostosis

Rufai

Bowman

Bunce

et al. 2021

Trans. Vis. Sci. Tech.

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To determine whether handheld optical coherence tomography (OCT) is feasible and repeatable in children with craniosynostosis.Methods: This was a prospective cross-sectional study. Children with syndromic and non-syndromic craniosynostosis 0 to 18 years of age were recruited between February 13, 2020, and October 1, 2020. Main outcome measures included feasibility (patient recruitment and handheld OCT success rates) and repeatability, which were assessed using intraclass correlation coefficients (ICCs) where repeated images of the optic nerve head (ONH) within the same visit were available. ONH parameters used for repeatability analysis included cup depth, width, and area; disc width; rim height; retinal thickness; retinal nerve fiber layer thickness; and Bruch's membrane opening minimum rim width.Results: Fifty children were approached, and all 50 (100%) were successfully recruited. Median age was 51.1 months (range, 1.9-156.9; interquartile range, 37.0-74.2), and 33 of the children (66%) were male. At least one ONH image was obtained in 43 children (86%), and bilateral ONH imaging was successful in 38 children (76%). Factors boosting the likelihood of success included good understanding and cooperation of the child and parent/guardian and availability of an assistant. Repeatability analysis was performed in 20 children, demonstrating good repeatability (ICC range, 0.77-0.99; the majority exceeded 0.90). OCT correctly identified two cases of intracranial hypertension, one of which was undetected by prior fundoscopy. Conclusions:Handheld OCT is feasible and repeatable in children with syndromic and non-syndromic forms of craniosynostosis.Translational Relevance: Our handheld OCT approach could be used for the clinical surveillance of children with craniosynostosis.

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Section: Research In Contextsupporting

confidence: 93%

Section: Neurosurgical Intervention Involves Expanding Thementioning

confidence: 99%

Feasibility and Repeatability of Handheld Optical Coherence Tomography in Children With Craniosynostosis

Rufai

Bowman

Bunce

et al. 2021

Trans. Vis. Sci. Tech.

Self Cite

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“…In addition, one should keep in mind that fixed exotropia in a young child can be related to various organic and neurological causes (i.e., 3rd nerve palsy, hydrocephalus, craniosynostosis, Prader-Willi syndrome, etc.) [ 13 , 14 , 15 , 16 ]. Naturally, a thorough ophthalmological evaluation was performed when the child was first evaluated.…”

Section: Discussionmentioning

confidence: 99%

Subtle Combined Hamartoma of the Retina and Retinal Pigment Epithelium Causing Recurrent Exodeviation

Abramowicz

Delvaulx

Fave

et al. 2022

Case Rep Ophthalmol

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A 3-year-old girl presented with recurrent exotropia following primary strabismus surgery. Careful fundus examination of the left eye revealed loss of the foveal reflex and presence of a subtle grayish mass with overlying white fluff. Optical coherence tomography through the lesion revealed disorganization of inner and outer retinal layers with accompanying epiretinal gliosis. Together, these findings were suggestive of combined hamartoma of the retina and retinal pigment epithelium (CHRRPE). No syndromic association was found. CHRRPE is a rare retinal tumor that usually presents with visual loss, strabismus, or follows an asymptomatic course. Retinal tumors must be kept in mind whenever loss of foveal reflex occurs concurrently with strabismus.

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“… 53 In children with craniosynostosis monitored at GOSH, clinical assessments and VEPs are currently performed according to an ophthalmological surveillance protocol. 54 …”

Section: Methodsmentioning

confidence: 99%

“…Wherever possible, VEP assessments will be performed and graded according to a modified criterion created by Thompson et al 53 In children with craniosynostosis monitored at GOSH, clinical assessments and VEPs are currently performed according to an ophthalmological surveillance protocol. 54…”

Section: Secondary Outcome Data Assessmentmentioning

confidence: 99%

Recognition of intracranial hypertension using handheld optical coherence tomography in children (RIO Study): a diagnostic accuracy study protocol

et al. 2022

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IntroductionPaediatric intracranial hypertension (IH) is a rare but serious condition that can pose deleterious effects on the brain and vision. Estimating intracranial pressure (ICP) in children is difficult. Gold standard direct ICP measurement is invasive and carries risk. It is impractical to routinely perform direct ICP measurements over time for all children at risk of IH. This study proposes to assess the diagnostic accuracy of handheld optical coherence tomography (OCT), a non-invasive ocular imaging method, to detect IH in children.Methods and analysisThis is a prospective study evaluating the diagnostic accuracy of handheld OCT for IH in at risk children. Inclusion criteria include clinical and/or genetic diagnosis of craniosynostosis, idiopathic intracranial hypertension, space occupying lesion or other conditions association with IH and age 0–18 years old. Exclusion criteria include patients older than 18 years of age and/or absence of condition placing the child at risk of IH. The primary outcome measures are handheld OCT and 48-hour ICP assessments, which will be used for diagnostic accuracy testing (sensitivity, specificity, positive predictive value, negative predictive value and accuracy). Main secondary outcome measures include visual acuity, fundoscopic examination, contrast sensitivity, visual field testing and visual evoked potentials, wherever possible.Ethics and disseminationEthical approval was granted for this study by the East Midlands Nottingham 2 Research Ethics committee (UOL0348/IRAS 105137). Our findings will be disseminated through presentation at relevant meetings, peer-reviewed publication and via the popular media.Trial registration numberISRCTN52858719

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Visual outcomes in children with syndromic craniosynostosis: a review of 165 cases

Cited by 11 publications

References 18 publications

Feasibility and Repeatability of Handheld Optical Coherence Tomography in Children With Craniosynostosis

Feasibility and Repeatability of Handheld Optical Coherence Tomography in Children With Craniosynostosis

Subtle Combined Hamartoma of the Retina and Retinal Pigment Epithelium Causing Recurrent Exodeviation

Recognition of intracranial hypertension using handheld optical coherence tomography in children (RIO Study): a diagnostic accuracy study protocol

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