Reactivation of Graves' Orbitopathy
Thyroid-related orbitopathy (TRO) or Graves' orbitopathy can present with proptosis and compressive optic neuropathy in severe cases. Although medical therapy, including corticosteroids and radiation therapy, are effective in select scenarios, bony orbital decompression is the surgical treatment of choice for stable proptosis or compressive optic neuropathy that is poorly responsive to immunosuppression. In the majority of cases, an adequate decompression should be long-lasting, because it is usually performed on patients with inactive disease. In the current study, however, the authors presented a potentially new phenomenon -- reactivation of Graves' orbitopathy after decompression.
Baldeschi L, Lupetti A, Vu P, Wakelkamp IM, Prummel MF, Wiersinga WM
Ophthalmology. 2007;114:1395-1402
Three female patients were presented who had received decompression for stable exophthalmos secondary to Graves' disease. Average duration of disease activity at time of decompression was 2.37 years (range, 0.5-6 years). Thyroid function was also stable for an average of 15.83 years (range, 2.5-9 years). In all 3 cases, a decompression was performed that was deemed initially adequate. Three weeks into the postoperative period, increased periorbital edema and worsening for restrictive myopathy developed in all patients. They were all were diagnosed with delayed decompression-related activation (DDRR); one received oral glucocorticoids, a second received orbital radiation, and the third oral and IV glucocorticoids followed by radiation. All had resolution of disease with stability lasting at least 7.53 years on average (range, 5.7-10.6 years).
TRO is an autoimmune inflammatory disease in which surgical interventions are usually offered after the inflammatory component appears quiescent. However, guidelines for establishing clinical stability are based on subjective observations; if clinical measurements and examinations are stable for 6 months, a patient's disease is usually considered quiet. This study demonstrated that inflammation can be retriggered by surgery. This occurrence, however, is quite rare; the incidence of 3 cases out of 239 cases performed during this time period equals an incidence of 1.3% for DDRR. Moreover, further analysis of the 3 cases presented revealed that 2 of the 3 had stability of eye disease for only half a year, and both these cases were smokers. Given that all studies regarding TRO have demonstrated worse disease in smokers, it is feasible to suggest that these 2 patients were not really stable and that their surgery should have been delayed longer.
Abstract
Supported by an independent educational grant from Genentech
Introduction
Thyroid-related orbitopathy (TRO) or Graves' orbitopathy can present with proptosis and compressive optic neuropathy in severe cases. Although medical therapy, including corticosteroids and radiation therapy, are effective in select scenarios, bony orbital decompression is the surgical treatment of choice for stable proptosis or compressive optic neuropathy that is poorly responsive to immunosuppression. In the majority of cases, an adequate decompression should be long-lasting, because it is usually performed on patients with inactive disease. In the current study, however, the authors presented a potentially new phenomenon -- reactivation of Graves' orbitopathy after decompression.
Reactivation of Graves' Orbitopathy After Rehabilitative Orbital Decompression
Baldeschi L, Lupetti A, Vu P, Wakelkamp IM, Prummel MF, Wiersinga WM
Ophthalmology. 2007;114:1395-1402
Summary
Three female patients were presented who had received decompression for stable exophthalmos secondary to Graves' disease. Average duration of disease activity at time of decompression was 2.37 years (range, 0.5-6 years). Thyroid function was also stable for an average of 15.83 years (range, 2.5-9 years). In all 3 cases, a decompression was performed that was deemed initially adequate. Three weeks into the postoperative period, increased periorbital edema and worsening for restrictive myopathy developed in all patients. They were all were diagnosed with delayed decompression-related activation (DDRR); one received oral glucocorticoids, a second received orbital radiation, and the third oral and IV glucocorticoids followed by radiation. All had resolution of disease with stability lasting at least 7.53 years on average (range, 5.7-10.6 years).
Comment
TRO is an autoimmune inflammatory disease in which surgical interventions are usually offered after the inflammatory component appears quiescent. However, guidelines for establishing clinical stability are based on subjective observations; if clinical measurements and examinations are stable for 6 months, a patient's disease is usually considered quiet. This study demonstrated that inflammation can be retriggered by surgery. This occurrence, however, is quite rare; the incidence of 3 cases out of 239 cases performed during this time period equals an incidence of 1.3% for DDRR. Moreover, further analysis of the 3 cases presented revealed that 2 of the 3 had stability of eye disease for only half a year, and both these cases were smokers. Given that all studies regarding TRO have demonstrated worse disease in smokers, it is feasible to suggest that these 2 patients were not really stable and that their surgery should have been delayed longer.
Abstract
Supported by an independent educational grant from Genentech
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