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Published on: February 15, 2022
Prostaglandin-Associated Periorbitopathy in Children and Young Adults with Glaucoma
Jane S Kim1, Sonya Blizzard1, Julie A Woodward1
1Department of Ophthalmology, Duke University Medical Center, Durham, North Carolina.
Insights
Long-term unilateral prostaglandin analogue (PGA) use in children with glaucoma can cause mild to moderate eyelid and eye changes, known as prostaglandin-associated periorbitopathy (PAP). Patients should be informed about these potential side effects, especially with monocular therapy.
Area of Science:
- Ophthalmology
- Pediatric Glaucoma
- Ocular Adnexa
Background:
- Prostaglandin analogues (PGAs) are commonly used to treat glaucoma.
- Unilateral PGA treatment can lead to asymmetry in ocular and periocular features.
- Childhood glaucoma requires long-term management, often involving unilateral PGA therapy.
Purpose of the Study:
- To evaluate palpebral and orbital changes from long-term unilateral prostaglandin analogue (PGA) exposure in pediatric glaucoma patients.
- To identify specific features of prostaglandin-associated periorbitopathy (PAP) in this cohort.
- To assess the reliability of expert graders in identifying PGA-treated eyes.
Main Methods:
- Prospective cross-sectional cohort study of 29 patients with childhood glaucoma on unilateral PGAs for ≥12 months.
- Standardized clinical photographs were assessed by 3 masked expert graders.
- Graders identified PGA-treated eyes and graded ocular, palpebral, and periorbital features, with interrater reliability assessed using Fleiss kappa.
Main Results:
- Long-term unilateral PGA use (median 31.7 months) resulted in features of prostaglandin-associated periorbitopathy (PAP).
- Most frequent changes included eyelash trichomegaly/hypertrichosis (76%), high upper eyelid crease (69%), upper eyelid ptosis (52%), and superior sulcus hollowing (52%).
- Changes were mostly mild, though 20-30% showed moderate eyelid/eyelash alterations; interrater reliability was excellent (κ=0.815).
Conclusions:
- Mild-to-moderate prostaglandin-associated periorbitopathy (PAP) can occur in children and young adults with long-term unilateral PGA exposure.
- Educating patients and families about potential PAP is crucial, particularly when initiating monocular PGA therapy.
- These findings highlight the importance of monitoring for and understanding the cosmetic and functional implications of long-term PGA use in pediatric glaucoma.
Objective:
To evaluate for relative palpebral and orbital changes after long-term unilateral exposure to prostaglandin analogues (PGAs) in patients with childhood glaucoma.
Design:
Prospective cross-sectional cohort study.
Participants:
A total of 29 patients with history of childhood glaucoma, who were treated unilaterally with PGAs for at least 12 months.
Methods:
Based on 4 standardized clinical photographs (en face with eyes open, right and left side views with eyes open, and en face with eyes closed), 3 masked expert graders each independently selected the eye they perceived to have received unilateral PGA treatment by physical appearance alone and graded the following features relative to the other eye: (1) ocular (e.g., conjunctival hyperemia, iris heterochromia, and buphthalmos), (2) palpebral (e.g., eyelash trichomegaly, eyelash hypertrichosis, eyelid erythema, eyelid edema, eyelid hyperpigmentation, high upper eyelid crease, upper eyelid ptosis, upper and/or lower eyelid retraction, and eyelid skin atrophy with presence of telangiectasias), and (3) periorbital (e.g., superior sulcus hollowing, proptosis, enophthalmos, hypoglobus, and hyperglobus). An interrater reliability analysis was performed using the Fleiss kappa (κ) statistic to determine consistency among raters.
Main Outcome Measures:
Frequencies of each feature of prostaglandin-associated periorbitopathy (PAP); group consensus; interrater reliability of selected PGA-treatment laterality.
Results:
Median unilateral PGA exposure time was 31.7 months (interquartile range: 18.8-44.3 months). Eyelash trichomegaly and hypertrichosis (n = 22, 76%), high upper eyelid crease (n = 20, 69%), upper eyelid ptosis (n = 14, 52%), and superior sulcus hollowing (n = 15, 52%) were the most frequently observed features of PAP in PGA-treated eyes compared with untreated fellow eyes. Most of these changes were mild, but 20% to 30% of patients exhibited moderate eyelash and/or eyelid changes. One patient had severe PAP after long-term unilateral PGA exposure. Group consensus with correctly selected laterality was achieved in all patients. The inter-rater reliability was excellent (κ = 0.815, P < 0.001, 95% confidence interval [0.605, 1.000]).
Conclusions:
Mild-to-moderate changes in the ocular adnexa can develop in children and young adults with long-term PGA exposure. Patients and their families should be educated on the possibility of PAP, especially when initiating monocular PGA therapy.
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