Graves' orbitopathy in a patient with adrenoleukodystrophy after bone marrow transplantation.

OBJECTIVE: For many years, the treatment of X-linked childhood cerebral adrenoleukodystrophy (XALD) consisted of hydrocortisone replacement and a mixture of short chain-fatty acids, known as 'Lorenzo's oil'. Recently, bone marrow transplantation (BMT) has also been used. CASE REPORT: We report the case of a patient affected by XALD who developed Graves' hyperthyroidism (GH) and Graves' orbitopathy (GO) after BMT and who we could follow-up for 6.5 years afterwards. EVIDENCE SYNTHESIS: A boy affected by XALD was treated at the age of 6 years, with a whole BMT from his sister. One year after BMT, the transplanted patient presented TSH at the lower normal value and 3 years later he developed thyrotoxicosis. After a further 2 years, the patient developed GO, which showed clinical evidence of reactivation 5 years after its onset as a consequence of an attempt to treat thyrotoxicosis by means of I(131) (300 MBq). Seven years after BMT, the donor showed alterations of thyroid autoimmunity and 1 year thereafter she developed GH. She never presented GO during a subsequent 5 year follow-up. CONCLUSIONS: This case illustrates that autoimmunity originating from a pre-symptomatic donor can be transferred into the host during allogeneic stem cell transplantation. In cases where autoimmune phenomena are recognized in the donor prior to donation, alternative donors or T-cell manipulation of the graft might be considered.

Corneal astigmatism in Graves' ophthalmopathy.

PURPOSE: To describe corneal astigmatism in patients under the age of 55 years with Graves' ophthalmopathy and to compare it to a control group. METHODS: Retrospective, nonrandomized comparative observational case series. A total of 109 patients with inactive Graves' ophthalmopathy and 109 age- and gender-matched control individuals without Graves' ophthalmopathy were examined with manual corneal keratometry. In the Graves' patients, 52% had a CT scan available for review, and 41% underwent orbital, strabismus, and/or eyelid surgery with a follow-up of keratometry over a mean period of 1.7 years after surgery. RESULTS: For the dioptres, there was a statistically significant difference between the Graves' and the control eyes at 3-3.25 dioptres in both eyes (t-test, right eyes, P=0.042; left eyes, P=0.041). For the meridians, the astigmatism was found to be greater at the meridians 95-100 degrees (t-test, P=0.044) and 105 degrees -110 degrees (t-test, P=0.017) in the right eyes only. In 10 (9%) Graves' patients, the astigmatism had caused newly acquired reduced visual acuity. Greater astigmatism in dioptres did not correlate with specific CT scan findings (chi(2)-test, df=2, P=0.187). Following orbital, strabismus, and eyelid surgery, the dioptres and meridians of the astigmatism did not change in 56 (68%) and 66 (81%) eyes, respectively. CONCLUSIONS: Graves' ophthalmopathy may be associated with greater with-the-rule corneal astigmatism, which, overall, is not influenced by orbital, strabismus, or eyelid surgery. The astigmatism may possibly be caused by soft-tissue fibrosis in the superolateral orbital region.

Torsional diplopia in Graves' orbitopathy. Three case reports.

Torsional diplopia may frequently accompany Graves' orbitopathy and is often not recognized. Some patients with manifest cyclotorsion do not complain of torsional diplopia because it is concealed by the large vertical and/or horizontal deviation. Torsional diplopia can occur spontaneously or appear after decompression or strabismus surgery. We discuss some interesting points arising from three cases. This will be followed by a discussion of the literature on the pathogenesis of cyclodeviation in Graves' orbitopathy.