Lelio Baldeschi

Consensus statement of the European Group on Graves' orbitopathy (EUGOGO) on management of GO

Summary of consensus a. All patients with GO should (Fig. 1):Be referred to specialist centers;Be encouraged to quit smoking;Receive prompt treatment in order to restore andmaintain euthyroidism.b. Patients with sight-threatening GO should be treatedwith i.v. GCs as the first-line treatment; if the responseis poor after 1–2 weeks, they should be submitted tourgent surgical decompression.c. The treatment of choice for moderate-to-severe GO isi.v. GCs (with or without OR) if the orbitopathy isactive;surgery(orbitaldecompression,squintsurgery,and/or eyelid surgery in this order) should beconsidered if the orbitopathy is inactive.d. In patients with mild GO, local measures and anexpectant strategy are sufficient in most cases, buttreatment may be justified if QoL is affectedsignificantly. In memoriam This document is dedicated to the memory of MarkPrummel (1956–2005), one of the founders ofEUGOGO, who greatly contributed to expanding ourunderstanding of clinical and therapeutic aspects of GO.

Consensus statement of the European group on Graves' orbitopathy (EUGOGO) on management of Graves' orbitopathy.

Luigi Bartalena, Lelio Baldeschi, Alison J. Dickinson, Anja Eckstein, Pat Kendall-Taylor, Claudio Marcocci, Maarten P. Mourits, Petros Perros, Kostas Boboridis, Antonella Boschi, Nicola Curro, Chantal Daumerie, George J. Kahaly, Gerasimos Krassas, Carol M. Lane, John H. Lazarus, Michele Marino, Marco Nardi, Christopher Neoh, Jacques Orgiazzi, Simon Pearce, Aldo Pinchera, Susanne Pitz, Mario Salvi, Paolo Sivelli, Matthias Stahl, Georg von Arx, and Wilmar M. Wiersinga

Surgical or medical decompression as a first-line treatment of optic neuropathy in Graves’ ophthalmopathy? A randomized controlled trial

Objective  Only a small percentage of Graves’ ophthalmopathy (GO) patients develop optic neuropathy with impending loss of visual acuity. Therapy with methylprednisolone pulses is the treatment of first choice in severe and active GO patients. When the effect is insufficient, patients are usually treated with surgical decompression. We investigated whether surgery could become the first‐line treatment, thus preventing treatment with steroids.

Clinical Features of Dysthyroid Optic Neuropathy: A European Group on Graves Orbitopathy (EUGOGO) Survey

Background: This study was performed to determine clinical features of dysthyroid optic neuropathy (DON) across Europe. Methods: Forty seven patients with DON presented to seven European centres during one year. Local protocols for thyroid status, ophthalmic examination and further investigation were used. Each eye was classified as having definite, equivocal, or no DON. Results: Graves’ hyperthyroidism occurred in the majority; 20% had received radioiodine. Of 94 eyes, 55 had definite and 17 equivocal DON. Median Clinical Activity Score was 4/7 but 25% scored 3 or less, indicating severe inflammation was not essential. Best corrected visual acuity was 6/9 (Snellen) or worse in 75% of DON eyes. Colour vision was reduced in 33 eyes, of which all but one had DON. Half of the DON eyes had normal optic disc appearance. In DON eyes proptosis was > 21 mm (significant) in 66% and visual fields abnormal in 71%. Orbital imaging showed apical muscle crowding in 88% of DON patients. Optic nerve stretch and fat prolapse were infrequently reported. Conclusion: Patients with DON may not have severe proptosis and orbital inflammation. Optic disc swelling, impaired colour vision and radiological evidence of apical optic nerve compression are the most useful clinical features in this series.

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.

A New and Validated CT-Based Method for the Calculation of Orbital Soft Tissue Volumes

PURPOSE There is no consensus as how to calculate orbital soft tissue volume based on CT or MRI scans. The authors sought to validate their technique and to assess the intraobserver and interobserver variability of their calculations of bony orbital volume (OV), orbital fat volume (FV), and extraocular muscle volume (MV) on CT scans of humans. METHODS The authors calculated these volumes with the use of a manual segmentation technique on CT scans with commercially available software. Two observers (one of them masked) calculated the orbital soft tissue volumes in a CT scan of a phantom constructed of dry skull, butter, and chicken muscle. These calculations were compared with previously taken standard volume measurements of these materials. Repetitive calculations on one CT scan by the same observer were compared. Soft tissue volumes taken from 10 orbital CT scans were calculated by two observers and compared. From the data acquired, intraobserver and interobserver variability was calculated. RESULTS Outcomes of these calculations using this software approximated the volumes of the phantom measured with standardized techniques. Accuracy of the phantom calculations between the two observers varied from +0.7% to -0.7% for FV and between -1.5% and -2.2% for MV. Mean differences between the repeated calculations were smaller than 5%. The intraclass correlation coefficient varied from 0.961 to 0.999. CONCLUSIONS Calculating orbital soft tissue volume using a manual segmentation technique for CT scans is a reliable and accurate tool.

Bony orbital development after early enucleation in humans

AIM To analyse the extent of bony orbital volume reduction after enucleation in humans. METHODS Volumetric studies on bony orbital volumes based on three dimensional reconstructions acquired from high resolution computed tomograph (CT) scans were performed in 29 patients with acquired anophthalmia and four patients before enucleation (controls). Eight patients (follow up 25–52 years) were enucleated in childhood aged between 0.4 and 8 years (group I), 21 in adulthood aged between 15 and 53 years. Fifteen of these patients (group IIa) had long standing anophthalmia (follow up 7–53 years), six patients (group IIb) were enucleated 9 months to 4 years before CT. RESULTS Bony orbital volumes were reduced in all patients with long standing anophthalmia. The median percentage reduction in enucleated orbits was 7.0% in group I, 3.8% in group IIa, and 1.9% in group IIb. In patients with long standing anophthalmia (I and IIa) the reductions were statistically significantly different (p <0.01) from zero. There was some evidence of a correlation between orbital volume reduction and age at enucleation (rho = 0.36, p = 0.09, Spearman rank correlation coefficient) and a statistically significant correlation between volume reduction and time interval since enucleation (rho = −0.5, p = 0.003). Clinically none of the patients showed significant facial asymmetry. CONCLUSIONS These data provide strong evidence that enucleation both in children and adults is associated with a reduction of bony orbital volume and that this decrease in volume is associated with increasing time. However, the reduction is smaller than generally assumed and does not cause obvious facial asymmetry. It is more related to the time interval since enucleation than the age at enucleation, which makes a mechanism of volume adaptation more likely than just retardation of growth.

Anterior suspended flaps: a modified approach for external dacryocystorhinostomy

BACKGROUND/AIMS External dacryocystorhinostomy (DCR) is a reliable but difficult surgical technique for the treatment of any obstruction of the lacrimal drainage system lying distal to the internal opening of the common canaliculus. In this prospective study, a simplified external DCR procedure and the results obtained on a series of 45 consecutive patients, in which traditional external DCR was indicated, are described. METHODS In this modified procedure only very large and mobile anterior flaps of the lacrimal sac and nasal mucosa are created. Thanks to the large size and the great motility the two flaps can be easily sutured. Two double armed 6/0 polyglycolic acid sutures are used to join the two flaps, to elevate them anteriorly in order to avoid adhesions with underlying tissues, and to approximate the deep planes of the wound. The mean operative time was measured. RESULTS At the end of follow up period (mean 17 months, range 14–24 months) all patients had no sign or symptoms of tearing and normal Jones I dye test. The mean operative time was 28.6 minutes (range 23–44 minutes) CONCLUSION We believe that our modified technique can be used to simplify and speed up traditional external DCR without decreasing its well known reliability.

Age and gender-specific reference values of orbital fat and muscle volumes in Caucasians

Aim To provide age and gender-specific reference values for orbital fat and muscle volumes (MV) in Caucasian adults. Patients and Methods Computed tomographic scans of 160 orbits from 52 men and 55 women, aged 20–80 years, not affected by orbital disease were evaluated. Orbital bony cavity volume (OV), fat volume (FV) and MV were calculated by a previously validated method using the software program Mimics. Ratios of FV to OV and of MV to OV were determined. Results OV, FV and MV were all significantly larger in men than in women (p<0.001), but FV/OV and MV/OV were similar in both sexes. OV and MV did not change with age, but FV increased with advancing age in both women (p<0.001) and men (p<0.001). Linear regression analysis with FV/OV and MV/OV ratios as dependent variables and age and gender as independent variables showed a significant correlation between age and FV/OV (r=0.52, p<0.0001) and age and MV/OV (r=−0.26, p=0.001). Conclusions Advancing age is associated with an increase of FV/OV and a minor decrease of MV/OV. Gender-specific differences in orbital FV and MV disappear once FV and MV are related to OV, by calculating the ratios FV/OV and MV/OV. Age-specific gender-neutral reference ranges (2.5 and 97.5 percentiles) of FV/OV and MV/OV are presented.

Brachytherapy as Part of the Multidisciplinary Treatment of Childhood Rhabdomyosarcomas of the Orbit

INTRODUCTION Rhabdomyosarcomas in the orbit form a major challenge in terms of cure without severe side effects in childhood cancer. Our specifically developed approach consists of applying brachytherapy to the tumor area using a mold. Analysis of its results for 20 patients was performed. METHODS AND MATERIALS Thirteen patients were referred for brachytherapy if complete remission was not reached after chemotherapy (Group I) and 7 in case of relapse (Group II). In total, 20 patients were treated between 1991 and 2007. Four were female and 16 male; their ages varied from 1.1 to 16.5 years, with an average of 8.5 years. After macroscopically radical tumor resection, molds with holes drilled to hold flexible catheters were placed into the orbit. The dose to the clinical target volume was 40-50 Gy. RESULTS Three patients of Group I and 1 patient of Group II developed local recurrence and underwent exenteration. The progression-free survival in Group I is 71.9% (95% CI 0.44-1.0), in Group II 85.7% (95% CI 0.60-1.0), the overall 5-year survival rate of the entire group is 92% (95% CI 0.76-1.0). During treatment, no serious side effects were observed. The late complications encountered in this series were cataract in 2 patients, 1 of whom also developed mild retinopathy. Two patients with ptosis needed surgical correction. No facial asymmetries or bone growth anomalies were observed. CONCLUSIONS This entire procedure of brachytherapy with a mold offers a tailor-made treatment for orbital rhabdomyosarcomas with only few signs of late toxicity.