|Year : 2022 | Volume
| Issue : 3 | Page : 749-751
Blepharoptosis as a presenting sign of Hashimoto's thyroiditis in a young girl: A case report and review of literature
Manju Meena1, Arcot S Karthikeyan2
1 Oculoplastics and Ocular Oncology Services, Senior Consultant and International Faculty, CMER (Shenzhen) Dennis Lam Eye Hospital, Chengongmiao, Futian District, Shenzhen, China
2 Pediatric Ophthalmology, Strabismus and Ocular Genetics, Senior Consultant and International Faculty, CMER (Shenzhen) Dennis Lam Eye Hospital, Chengongmiao, Futian District, Shenzhen, China
|Date of Submission||25-Jan-2022|
|Date of Acceptance||08-Feb-2022|
|Date of Web Publication||16-Jul-2022|
Dr. Manju Meena
Oculoplastics and Ocular Oncology Services, Senior Consultant and International Faculty, CMER (Shenzhen) Dennis Lam Eye Hospital, 1-2/F, Shengtang Building, No 1 Tairan 9th Road, Chengongmiao, Futian District, Shenzhen
Source of Support: None, Conflict of Interest: None
Thyroid eye disease (TED) refers to the ocular symptoms and signs caused by an autoimmune pathology affecting the thyroid gland and the orbit. It is most commonly seen in patients with Graves' disease and rarely with Hashimoto's thyroiditis (HT). Upper eyelid retraction is the most consistent eyelid sign. Blepharoptosis in TED has been reported sparsely and is mostly associated with myasthenia gravis. Here, we report a case of blepharoptosis as a presenting sign of HT in a young girl.
Keywords: Autoimmune diseases, blepharoptosis, Hashimoto's thyroiditis, thyroid eye disease
|How to cite this article:|
Meena M, Karthikeyan AS. Blepharoptosis as a presenting sign of Hashimoto's thyroiditis in a young girl: A case report and review of literature. Indian J Ophthalmol Case Rep 2022;2:749-51
|How to cite this URL:|
Meena M, Karthikeyan AS. Blepharoptosis as a presenting sign of Hashimoto's thyroiditis in a young girl: A case report and review of literature. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 11];2:749-51. Available from: https://www.ijoreports.in/text.asp?2022/2/3/749/351124
Thyroid eye disease (TED) is characterized by immune-mediated inflammation of the orbital tissues. Severe orbitopathy often occurs in patients with Graves' disease but is rare in Hashimoto's thyroiditis (HT). Mild upper eyelid retraction has been reported to be a common finding in HT. However, blepharoptosis with orbitopathy as a presenting feature of HT has never been reported. We report a rare presentation of HT in a young Chinese girl presenting with blepharoptosis and TED.
| Case Report|| |
A 13-year-old Chinese girl presented with a chief complaint of drooping of the left upper eyelid for past 2 years. The ptosis was slowly progressive, and she denied any history of diurnal variation and trauma. Her birth and developmental history was uneventful. There was no significant systemic history. Her old medical record revealed negative repetitive nerve stimulation test (RNS), muscle biopsy, and neostigmine tests. Family history revealed her mother to have congenital ptosis.
On ophthalmological examination, her best-corrected visual acuity (BCVA) was 20/20 OU. She had unilateral severe ptosis involving OS. MRD 1 was 5 mm in OD and 0 mm in OS [Figure 1]. The levator muscle function was 15 mm in OD and 9 mm in OS. There was lower lid retraction in OU. There was reduced palpebral fissure height in downgaze OS. Her anterior segment examination was normal. Posterior segment evaluation was normal in OS. Color vision testing was normal OU. Exophthalmos measured by Hertel's exophthalmometer was 17 mm OD and 19 mm OS. Her intraocular pressure (IOP) was 15 mm Hg OD and 17 mm Hg OS in primary gaze and increased to 21 mm Hg in OS in upgaze.
|Figure 1: (a) A 13-year-old girl presented with severe drooping of the left upper eyelid for 2 years. On examination, she had 5 mm of left blepharoptosis with a clinical activity score of 2. Also note the lower eyelid retraction in OU with inferior scleral show OS > OD. There is no eyelid edema or conjunctival congestion. There is mild right upper eyelid retraction. (b) Worm's eye view showing left-sided proptosis.|
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Ocular motility examination revealed a small left hypotropia. She had a limitation of elevation OS. Forced duction test (FDT) confirmed limitation of elevation and indicated a tight left inferior rectus muscle. She had diplopia in upgaze and downgaze. Ice test and fatigue test were negative.
Her Clinical Activity Score (CAS) was 2 (one point each for motility restriction and proptosis). The laboratory parameters revealed her euthyroid status with high anti-thyroid peroxidase antibodies (Anti-TPO-Ab) of 834 U/mL (normal value: <60 U/mL) and anti-thyroglobulin antibodies were 90 U/mL (normal value: <60 U/mL). The thyroid receptor antibodies (TRab) were 0.30 IU/L (normal value: <1.75 IU/L). MRI of orbits revealed mild proptosis OS. There was enlargement of the left inferior rectus and medial rectus muscle bellies sparing the tendons, consistent with the findings of TED. The rest of the extraocular muscles appeared normal [Figure 2]. The patient was advised to undergo fine-needle aspiration cytology (FNAC) of the thyroid gland, but the mother refused.
|Figure 2: (a) The MRI scan of the left orbit showing enlargement of the belly of inferior rectus muscle seen in the sagittal plane. (b) There was a mild proptosis on the left side, and mild thickening of bilateral medial rectus muscle bellies sparing the tendons can be seen in the axial plane.|
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The diagnosis of possible HT associated with TED was considered. She was referred to a pediatric endocrinologist. She was advised periodic follow-up to monitor her TED activity and strabismus.
| Discussion|| |
TED is commonly seen in patients with hyperthyroidism but can also be seen in euthyroid and hypothyroid status. In patients with suspected TED, if the TRAb is negative, the differential diagnosis should include carotid-cavernous fistula, sphenoid meningioma, orbital lymphoma, idiopathic orbital myositis, and IgG4-related disease. In our patient, there was no history of trauma and the MRI scan showed no evidence of any orbital tumor or carotid cavernous fistula (CCF). She had no pain on eye movement, which ruled out orbital myositis or lymphoma. Ocular adnexal IgG4-related disease is often manifested as enlargement of lacrimal glands; extraocular muscle involvement, though reported, is rare. Graves' and HT represent opposite sides of the same disease spectrum. In Graves' disease, the TSH-receptor antigen is shared by the thyroid gland as well by the orbital tissues, thus explaining the extra-thyroidal manifestation of the disease. However, the TSH-receptor hypothesis cannot apply to HT as antibodies against the TSH- receptors are generally not found in these patients.
The clinical manifestations of TED are mild in the pediatric age group as compared to adults. HT is usually not associated with overt ophthalmopathy, but extraocular muscle involvement has been reported in the literature. Tjiang et al. reported the presence of upper eyelid retraction and mild inflammatory signs in patients with HT. They reported the overall prevalence of eye signs in one-third of patients; upper eyelid retraction was the main feature. Kan et al. conducted a comparative study between HT patients (n = 110) and healthy subjects. They reported that the eye signs were present in 22.7% of patients with HT. Chengqu et al. reported a case of HT-associated ophthalmopathy that masqueraded as a unilateral double elevator palsy. Kirmizibekmez et al. reported a case of Hashitoxicosis in an adolescent girl who presented with features of Graves' disease and later diagnosed with HT. Yoshihara et al. reported two cases of HT presented with severe orbitopathy. In HT, because the eye muscle damage cannot be due to TSH receptor antibodies, other pathogenic mechanism might be responsible for the orbitopathy.
Our patient presented with severe blepharoptosis along with lower eyelid retraction and mild exophthalmos, which were consistent with TED. Her laboratory data was conclusive of HT. Blepharoptosis in patients with HT has never been reported in the literature. The unique features in our case include the pediatric age group, blepharoptosis, vertical strabismus, and a laboratory diagnosis of HT. The possible etiology in our case could have been levator aponeurosis disinsertion and inflammation. The presence of inferior rectus muscle enlargement could result in upgaze restriction and lower lid retraction. To the best of our knowledge, this is the first case of HT who presented with blepharoptosis in conjunction with TED in the pediatric age group.
| Conclusion|| |
HT may present at an early age with unusual presentation such as blepharoptosis, which can pose a diagnostic conundrum, often resulting in delayed diagnosis and inappropriate intervention. Thus, awareness of this atypical presentation of TED is important.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]