Journal of pediatric ophthalmology and strabismus · 2025
Synergistic Convergence in Horizontal Gaze Palsy With Progressive Scoliosis: A Case Report.
СтатьяКлинический случайНазвание и аннотация: Перевод в очереди
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Elhilali Hala, Shoala Tarek Saad, Ahmed Habeba Mostafa, Farag Christina S
Аннотация
This interventional case report documents the underlying genetic mutation, secondary muscle changes, and the result of surgical intervention in a patient presenting with synergistic convergence and esotropia. A 16-year-old patient underwent full ophthalmic examination, radiological imaging, and genetic testing. Diagnosis of horizontal gaze palsy with progressive scoliosis was confirmed, suggesting central miswiring as a cause of the synergistic convergence. Whole exome sequencing gene test revealed homozygous ROBO3 gene mutation (p.Leu888Arg) at a mutational hot spot with a GERP score of 4.88. Several computational analyses predicted the variant to be damaging. Bilateral medial rectus recessions of 7 and 8 mm were performed. Both medial rectus muscles were found tight, but not atrophic. Orthotropia and temporary resolution of synergistic convergence were documented. This case study reports a new mutation in the ROBO3 gene, and it is one of the few cases of central synergistic convergence that underwent surgical intervention and documenting secondary muscle changes.
Предметные рубрики MeSH
AdolescentHumansConvergence, OcularDNA Mutational AnalysisMutationOcular Motility DisordersOculomotor MusclesOphthalmologic Surgical ProceduresOphthalmoplegia, Chronic Progressive ExternalReceptors, ImmunologicScoliosisRoundabout ProteinsReceptors, Cell SurfaceCongenital Cranial Dysinnervation Disorders