Ocular Findings in Pontine Tegmental Cap Dysplasia. Issue 12 (December 2020)

• Record Type: Journal Article
• Authors: Mehner, Lauren C.; Jung, Jennifer L.; Maloney, John A.; McCourt, Emily A.
• Is Part Of: Cornea. Volume 39:Issue 12(2020)
• Publication Date: 2020-12
• Subjects: pontine tegmental cap dysplasia -- neurotrophic cornea -- pediatric cornea; Cornea -- Periodicals; Cornea -- Periodicals; Cornée -- Périodiques; 617.719
• DOI: 10.1097/ICO.0000000000002361
• Languages: English
• ISSNs: 0277-3740

Abstract

Abstract : Purpose: To describe the ocular complications experienced by patients with pontine tegmental cap dysplasia (PTCD) and the management strategies used to care for these children. Methods: Subjects with PTCD were recruited through social media advertisement and completed a survey gathering information on potential ocular problems related to the patient's PTCD disease and any current or previous treatments. Results: Twenty-two patients or guardians completed the survey. Neurotrophic cornea was the most common ocular diagnosis (82%), followed by facial palsy (59%), dry eye syndrome (59%), and blepharitis (55%). Other diagnoses included cortical visual impairment (27%), strabismus (27%), amblyopia (18%), and nystagmus (18%). Common treatment modalities included lubricating eye drops (59%) or ointment (50%), contact lenses (14%), punctal plugs (27%), glasses (45%), and patching (18%). The most common surgical interventions were temporary or permanent tarsorrhaphy (64%) and amniotic membrane grafts (23%). In total, 68% of families reported self-injury to eyes and 91% reported the child to be primarily a visual learner. Conclusions: PTCD is a newly described, very rare disorder with a variety of vision-threatening ocular manifestations. It is essential that the ophthalmologist be aware of the potential for neurotrophic cornea because timely treatment could prevent corneal scarring, perforation, and blindness. Abstract : Supplemental Digital Content is Available in the Text.