Abstract
Aim: To report the alignment, visual outcome and surgical strategy for severe bilateral congenital esotropia caused by a congenital cranial dysinnervation disorder (CCDD). This was successfully treated with a combination of interventions that included alternate occlusion, botulinum toxin, and conventional and augmented transposition procedures. Methods: A girl presented at 2 months of age with a marked esotropia and severe bilateral impairment of abduction of both eyes, and an inability to fix with either eye in the primary position. Her investigation and treatment are described. Results: The patient initially underwent alternate daily occlusion to prevent the development of amblyopia, followed by simultaneous bilateral medial rectus (MR) botulinum toxin and large bilateral MR recessions based on pre-operative forced duction testing (FDT). This operation was performed at 1 year of age with the aim of releasing the tight MR and reducing the esotropia. After initial improvement, the impairment of abduction gradually increased over the next month. She subsequently underwent sequential inferior oblique myectomy and augmented transposition procedures, which enabled her to fix in the primary position with either eye and with a small-angle esotropia. Conclusion: Substantial improvement in the alignment of the eyes in a child with a complex congenital esotropia secondary to a CCDD is achievable.
Highlights
Congenital cranial dysinnervation disorders (CCDDs) are an uncommon, potentially visually disabling subgroup of strabismus conditions that ‘arise from aberrant innervation of the ocular and facial musculature’
This case study describes the successful outcome in a child with severe bilateral congenital impairment of abduction with a management strategy that may be considered for similar motility scenarios
The diagnosis of a CCDD was made through the signs of nystagmus movements on attempted elevation, preoperative forced duction testing (FDT) findings and the persistent but improved under-action of abduction after performing surgery to recess both medial recti
Summary
Congenital cranial dysinnervation disorders (CCDDs) are an uncommon, potentially visually disabling subgroup of strabismus conditions that ‘arise from aberrant innervation of the ocular and facial musculature’. Nhs.uk absence of one or more cranial nerves, synkinetic eye movements and restrictions or under-actions of one or more extraocular muscles,[2,3] with genetic abnormalities in many.[4] This group of strabismus disorders includes Duane’s retraction syndrome (DRS), monocular elevation deficiency (MED), Moebius syndrome, congenital fibrosis syndrome of the extraocular muscles (CFEOMs),[5] and some cases of congenital superior oblique palsy[6] and of Brown’s syndrome.[7] These alignment abnormalities can compromise visual acuity and the field of binocular single vision, and result in persistently poor ocular alignment and potential secondary psychosocial symptoms. This case study describes the successful outcome in a child with severe bilateral congenital impairment of abduction with a management strategy that may be considered for similar motility scenarios
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