Will the exophthalmos in a 13-year-old patient with Graves' disease and hyperthyroidism resolve with treatment?

Will Exophthalmos Resolve in a 13-Year-Old with Graves' Disease?

Exophthalmos in pediatric Graves' disease will likely improve but not completely resolve with treatment alone—approximately 70% of cases remain stable with a wait-and-see approach, while surgical intervention (thyroidectomy) offers the best chance for measurable regression of proptosis. 1, 2

Natural History and Expected Outcomes

• In most pediatric Graves' patients, eye disease is self-limiting and subclinical, though approximately one-third develop clinically relevant ophthalmopathy that can be disabling. 3

• Among 641 pediatric patients with Graves' ophthalmopathy, 70% of clinicians recommend a wait-and-see policy for moderate cases, with intervention reserved for worsening or persistently active disease despite achieving euthyroidism. 2

• Exophthalmos typically does not fully resolve with medical management alone—antithyroid drugs and achieving euthyroid status can alleviate symptoms to some extent but have limited impact on proptosis itself. 3, 4

Treatment Approach for This 13-Year-Old

Initial Medical Management

• Methimazole is the preferred antithyroid drug for controlling hyperthyroidism in patients with moderate-to-severe orbitopathy, as it does not worsen eye disease. 1, 5

• Mandatory smoking cessation counseling (if applicable to the patient or household) is essential, as smoking significantly worsens orbitopathy and increases prevalence in adolescents. 1, 6, 2

• Ocular lubricants are almost always required to combat exposure from eyelid retraction and proptosis. 1

Advanced Interventions for Proptosis Reduction

• Teprotumumab (IGF-1 receptor inhibitor) directly reduces proptosis and clinical activity scores in patients with active moderate-to-severe disease and represents the most effective medical therapy for reducing exophthalmos. 7, 1

• Total thyroidectomy offers measurable regression of exophthalmos—in a surgical series, 12 of 15 patients (80%) experienced regression at mean follow-up of 15.6 months, with mean reduction of 2.1 mm (range 1-5 mm), and no cases worsened postoperatively. 8

• Orbital decompression, high-dose steroids, or radiation should be considered for severe proptosis or optic neuropathy, though these are typically reserved for sight-threatening cases. 1

Treatment Selection Algorithm

For moderate exophthalmos without optic neuropathy:

1. Start methimazole to achieve euthyroidism 1, 5
2. Provide ocular lubricants and monitor for 6-12 months 1
3. If proptosis persists or worsens despite euthyroidism, consider teprotumumab 1
4. If medical therapy fails or recurrent hyperthyroidism occurs, thyroidectomy offers the best chance for proptosis regression 8

For severe or worsening exophthalmos:

1. Achieve euthyroidism with methimazole 5
2. Initiate teprotumumab if disease is active 1
3. Consider orbital decompression if sight-threatening features develop 1

Critical Monitoring Requirements

• Screen for optic neuropathy with visual acuity, color vision, visual fields, pupillary examination, and fundus examination at each visit. 7, 1, 6

• Measure exophthalmos by exophthalmometer at baseline and follow-up visits to objectively track progression or regression. 7

• Monitor thyroid function every 4-6 weeks initially, then every 2-3 months once stable to maintain euthyroidism, as both hyper- and hypothyroidism worsen ophthalmopathy. 1, 4

Critical Pitfalls to Avoid

• Avoid radioactive iodine (RAI) in patients with clinically evident ophthalmopathy, as it carries well-documented risk of worsening eye disease, particularly in adolescents. 5, 3, 4

• Do not perform strabismus surgery before orbital decompression if both are needed, as decompression unpredictably changes alignment. 1

• Two-thirds of respondents would intervene with steroids if eye disease worsens or remains active despite achieving euthyroidism—do not assume euthyroidism alone will resolve active ophthalmopathy. 2