Andrea Papp MD, PhD, FEBO
Graves’ disease is an uncommon condition in children, occurring in about 0.02% of the paediatric population. Thyroid eye disease is even more rare and the symptoms are generally much milder than in adults and typically respond well to local treatment and control of the disturbed thyroid function, according to Andrea Papp MD, PhD, FEBO.
“Although the course of paediatric Graves’ orbitopathy was mild to moderate in most of our patients, these young patients still require careful follow-up as severe, sight-threatening orbitopathy requiring immunosuppression may occur at a young age in a rare subset of patients,” she told delegates attending the World Society of Paediatric Ophthalmology and Strabismus (WSPOS) Subspecialty Day at the 36th Congress of the ESCRS in Vienna.
Graves’ disease, also known as toxic diffuse goiter, is an autoimmune disease that affects the thyroid and is the most common cause of hyperthyroidism. Dr Papp’s retrospective case series studied the demographics, risk factors, clinical features and treatment options of children and adolescent patients with Graves’ diseases presenting at the thyroid eye clinic of the University Eye Hospital in Vienna, Austria over a period of five years.
A total of 48 patients were identified with Graves’ orbitopathy, all bilateral cases with a female to male ratio of about 3 to 1. The mean age at presentation was 12 years with a range from 2 years to 18 years. In terms of ethnicity, 40 patients (83%) were Caucasian, four (8%) were Asian, three (6%) were African and one (2%) was of Arabic descent.
Risk factors identified in the cohort included a positive family history in eight cases (17%), active smoking in 16 children (33%) and passive smoking in eight (17%). Additional autoimmune disorders were detected in three patients (6%), she said.
In terms of disease activity and clinical features, mild disease was diagnosed in 49%, moderate in 40% and severe paediatric orbitopathy in 11%. The principal clinical features included exophthalmos in 77%, lower eyelid retraction in 75%, upper eyelid retraction in 52%, conjunctival injection with sicca in 48%, ocular pain in 44%, lid oedema and/or erythema in 38% and acquired epiblepharon in 4%. Ocular motility involvement occurred in 38%, with limitation of abduction in 27% and elevation deficit in 21%. Symptomatic optic neuropathy was detected in 6% of patients, she added.
The mainstay of therapy is lubricating eye drops and ointments, which is effective in most cases, noted Dr Papp. Systemic control of disturbed thyroid function was indicated in three-quarters of patients and 23% received combination therapy with beta blockers. Five patients, however, required intravenous methylprednisolone pulse therapy that resulted in prompt clinical improvement without considerable side-effects, she concluded.
Andrea Papp: andrea.papp@meduniwien.ac.at
