Rachel Kalmann

Outcome of orbital decompression for disfiguring proptosis in patients with Graves' orbitopathy using various surgical procedures

Aim: To compare the outcome of various surgical approaches of orbital decompression in patients with Graves’ orbitopathy (GO) receiving surgery for disfiguring proptosis. Method: Data forms and questionnaires from consecutive, euthyroid patients with inactive GO who had undergone orbital decompression for disfiguring proptosis in 11 European centres were analysed. Results: Eighteen different (combinations of) approaches were used, the swinging eyelid approach being the most popular followed by the coronal and transconjunctival approaches. The average proptosis reduction for all decompressions was 5.0 (SD 2.1) mm. After three-wall decompression the proptosis reduction was significantly greater than after two-wall decompression. Additional fat removal resulted in greater proptosis reduction. Complications were rare, the most frequent being worsening of motility, occurring more frequently after coronal decompression. The average change in quality of life (QOL) in the appearance arm of the GO-QOL questionnaire was 20.5 (SD 24.8) points. Conclusions: In Europe, a wide range of surgical approaches is used to reduce disfiguring proptosis in patients with GO. The extent of proptosis reduction depends on the number of walls removed and whether or not fat is removed. Serious complications are infrequent. Worsening of ocular motility is still a major complication, but was rare in this series after the swinging eyelid approach.

Prevalence and management of elevated intraocular pressure in patients with Graves' orbitopathy.

AIMS To investigate the prevalence and to discuss the necessity of treating elevated intraocular pressures (IOP) in patients with Graves’ orbitopathy (GO). In addition, to study the effects of orbital decompression and extraocular muscle surgery on IOP. METHODS The records of consecutive patients with GO referred in a 5 year period were studied and those selected, in which glaucoma medication had been prescribed, or a diagnosis of primary open angle glaucoma (POAG) or of ocular hypertension (⩾22 mm Hg) (OH) had been made. The necessity of treating these patients with glaucoma medication was questioned and the effects of corticosteroids, orbital decompression, and extraocular muscle surgery on the IOP were evaluated. RESULTS Of 482 patients with GO, 23 (4.8%) met the inclusion criteria. Four patients (0.8%) had POAG, four had elevated IOPs and visual field defects consistent with dysthyroid optic neuropathy, and 15 (3.1%) had only elevated IOPs. Five patients with OH showed a permanent drop of IOP after orbital decompression, two had a marked decrease of their IOP after recession of the inferior rectus muscle. CONCLUSIONS POAG has the same prevalence in the general Dutch population as in the GO subgroup. The combination of elevated IOPs and visual field defects in GO patients may be attributed to other mechanisms than obstructed aqueous outflow in the trabecular meshwork and should be treated accordingly. Orbital decompression and extraocular muscle surgery may lower the IOP in patients with GO.

Diabetes mellitus; a risk factor in patients with Graves' orbitopathy

AIMS To assess the prevalence of dysthyroid optic neuropathy (DON) in patients with diabetes mellitus (DM) and Graves’ orbitopathy (GO) and to investigate the complications of surgery for GO in these patients. METHODS The records of 482 consecutive patients with GO referred in a 5 year period were studied. Those patients who also had DM were selected for further study. The prevalence of insulin dependent diabetes mellitus (IDDM) and non-insulin dependent diabetes mellitus (NIDDM) was registered, as well as the prevalence and course of DON. In the patients who underwent surgery for GO the postoperative complications were recorded. RESULTS Out of 482 patients with GO, 15 (3.1%) also had DM. Eight (1.7%) had IDDM, 7 (1.4%) had NIDDM. Five patients (33.3%) three with IDDM and two with NIDDM developed DON with 50% improvement of visual acuity after treatment, whereas in the whole population of 482 GO patients 19 had DON (3.9%), showing 69.4% improvement of vision after treatment. 10 patients with GO and DM were operated for GO; in one of them an optic atrophy developed as a result of a postoperative haemorrhage directly after a three wall orbital decompression by coronal approach. No other postoperative complications occurred. CONCLUSIONS The prevalence of IDDM in patients with GO is higher than in the normal population. DON occurs much more frequently in patients with GO and DM than in the total group of GO patients and seems to have a worse visual prognosis.

Treatment of severe idiopathic orbital inflammation with intravenous methylprednisolone

Background Prednisone pulse therapy is used to treat active non-infectious orbital inflammatory disease to attain faster clinical improvement and to shorten the duration of prednisone treatment. This study addresses the use of intravenous methylprednisolone (IVMP) pulse therapy, in addition to oral prednisone (OP), in the treatment of severe idiopathic orbital inflammation (IOI). Methods This was a multicentre retrospective cohort study. Patients with severe IOI treated with IVMP pulse and OP therapy (IVMP+OP) were compared with patients with IOI who were treated only with OP. Main outcome measures were duration of prednisone treatment, symptom-free outcome and complications. Results Between 2000 and 2007, 12 patients with severe IOI were treated with IVMP+OP and 15 patients were treated with OP only. The median treatment duration was 160 (range 34–680) days in the IVMP+OP group and 110 (range 27–730) days in the OP-only group. In patients who had severe IOI, 73% in the IVMP+OP group and 87% in the OP-only group were symptom-free after treatment. No patients developed complications related to prednisone therapy. Conclusion In our study there was no advantage of treating patients with severe IOI with IVMP+OP in terms of shortened treatment duration, lower cumulative dose or decrease in persistent symptoms. We suggest that the indication of IVMP in the treatment of severe IOI is limited to speeding symptom relief and recovery from optic nerve dysfunction.

Methylprednisolone pulse therapy for patients with dysthyroid optic neuropathy

PURPOSE 1) To test the short- and long-term efficacy of Methylprednisolone Pulse Therapy (MPT) for patients with Dysthyroid Optic Neuropathy (DON). 2) To assess possible side-effects of MPT. 3) To assess whether a delay in decompressive surgery would influence the visual outcome. STUDY DESIGN Retrospective, uncontrolled. METHOD The records of 62 consecutive patients with DON, who received MPT between 1994 and 2000, were evaluated with special attention for pre- and post treatment ophthalmic and orthoptic parameters, Clinical Activity Score (CAS), visual fields and visual evoked potentials, and treatment complications. DON-patients were hospitalized and received four times 500 mg methylprednisolone intravenously. Immediately afterwards they were treated with oral prednisone (maximal dose 60 mg) and/or orbital irradiation (10 times 2 Gy). Evaluation was done one day after the last bolus (T1) and when the orbitopathy had been stable for at least six months (T2). RESULTS Visual acuity, proptosis, elevation and CAS all improved significantly at T1, whereas the lid aperture did not change. At T2, 24 out 62 (39%) DON-patients were stable with normal vision. The other 38 (61%) had undergone orbital decompression because of persistent or recurrent DON within one week to six months after MPT. Final visual acuity in the whole group (121 eyes) was less than 0.1 in 3 eyes, between 0.1 and 0.5 in 17 and more than 0.5 in 101 eyes. Treatment complications were: pylorus ulcer (n = 1), increased osteoporosis (n = 1), abcess formation (n = 1), and increase in insulin dosage (n = 1). CONCLUSIONS 1) MPT is efficious as emergency treatment. 2) One-third of DON-patients are spared decompressive surgery. 3) MPT causes only rare and no serious side-effects. 4) The final visual outcome after MPT and orbital decompression is comparable to the results of immediate decompression.

Evaluation of classification systems for nonspecific idiopathic orbital inflammation.

Purpose: To systematically analyze existing classification systems for idiopathic orbital inflammation (IOI) and propose and test a new best practice classification system. Methods: A systematic literature search was conducted to find all studies that described and applied a classification system for IOI. Classification categories used in more than two studies were extracted, and criteria for these categories were defined using common descriptors. Using patient data, these newly defined classification systems were evaluated. Reliability was tested by inter- and intrarater agreement of two raters and distinction tested by evaluating clinical differences among classification categories. Feasibility, face validity, and content validity were qualitatively tested. Results: The most frequently encountered IOI classification systems were based on onset (acute, chronic), histopathology (classic, granulomatous, sclerosing), or localization (diffuse, extraocular muscle, lacrimal gland, sclera, optic nerve). Systems based on histopathology and localization showed good reliability (κ values range 0.74–0.89), were easy to apply (feasibility), and described the biologic process (face validity). Because of static sampling, histopathology-based systems had moderate content validity and moderate distinction between classification categories. Being a static measure, localization had moderate content validity, but good distinction. It was found that content validity was improved by combining histopathology and localization into a two-dimensional classification system. Conclusions: This combined histopathology and localization-based classification system provides a repeatable, easy to use, plausible, and complete classification system that can be used to further advance the research of IOI.

The Role of Biopsy in Diagnosing Patients Suspected of Idiopathic Orbital Inflammation

Purpose: To provide a framework when to biopsy patients suspected of idiopathic orbital inflammation (IOI). Materials and Methods: One-hundred seventeen patients were selected with signs of orbital inflammation in whom after history and physical examination, no definite diagnosis was made. In this cohort, the role of biopsy in making a diagnosis was evaluated and compared to a therapeutic trial of corticosteroids. Results: In 67 of 117 patients, a diagnosis was evident after imaging and laboratory testing. In 50 remaining patients, a decision had to be made to biopsy or administer a therapeutic trial of corticosteroids. Thirty-nine patients underwent biopsy as the next step. Nine patients underwent a therapeutic trial of corticosteroids of which one was subsequently subjected to biopsy. Two patients spontaneously resolved. No patients with a malignancy were inadvertently treated with corticosteroids. One patient experienced a biopsy related complication but did not lose vision. Conclusions: This study suggests using a therapeutic trial of corticosteroids only in patients with low suspicion of malignancy with muscular and apical mass localizations, or with optic-nerve compression. This framework was demonstrated to be safe in not delaying diagnosis of malignancies and efficient in providing a rapid diagnosis.

Idiopathic Orbital Inflammation and Graves Ophthalmopathy

Idiopathic orbital inflammation (IOI) is a poorly understood disease entity in which an orbital inflammatory process is found with, by definition, no identifiable local or systemic cause. Graves ophthalmopathy (GO) is often mentioned as a disease to exclude in the diagnosis of IOI. In the Orbital Clinic of the University Medical Center Utrecht, we have encountered 4 patients in whom diagnoses of both IOI and GO were made at different times. In this case series, we describe the clinical and diagnostic features of these patients, show that both IOI and GO can occur at different times in the same patient, and demonstrate the ways the diseases can be differentiated.

Identification of infectious entities in idiopathic orbital inflammation biopsies

Idiopathic orbital inflammation (IOI) is a poorly understood disease that can cause symptoms of pain, proptosis and diplopia. The condition is considered of non-infectious origin and a diagnosis is only made after exclusion of a neoplasm, primary infection and systemic disorders. However, a recent history of upper respiratory tract infection prior to development of IOI is not uncommon.1 It has been postulated that IOI is caused by an auto-immune response that develops after an infectious trigger.2 We investigated whether IOI may have an infectious origin by using molecular techniques to identify genetic material of a panel of viruses and bacteria in tissue biopsies of patients with IOI and controls. After ethical review board approval, 48 orbital biopsy specimens were collected at orbital clinics in Amsterdam, Maastricht, Rotterdam …

Reflections in the mirror – Idiopathic intracranial hypertension and non‐specific orbital inflammation in identical twins

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