Radiotherapy for the treatment of thyroid eye disease-a prospective comparison: Is orbital radiotherapy a suitable alternative to steroids?

BACKGROUND AND AIMS: To evaluate the efficacy of orbital radiotherapy (OR) for the treatment of thyroid eye disease (TED). METHODS: Thirty-five consecutive patients with active TED with contraindications to steroid therapy received a course of OR. Bilateral retrobulbar irradiation was performed with a total dose of 20 Gy. 7-points clinical activity score (7-CAS), ocular motility, visual acuity (VA), exophthalmos and eyelid retraction were prospectively evaluated at 3, 6 and 12 months and compared with baseline data. RESULTS: There was a statistically significant improvement in 7-CAS at 3, 6 and 12 months post-treatment (p < 0.05). Ocular motility disturbances improved at 6 and 12 months (p < 0.05). Visual acuity remained stable; there was no significant change in exophthalmos (mean 24 mm, SD 3 mm) or eyelid retraction (marginal reflex distance mean 6 mm, SD 1.5 mm) during the follow-up period. No side effects were registered. CONCLUSIONS: This study suggests that OR might be effective in reducing 7-CAS and ocular motility disturbances. No significant improvement in proptosis or eyelid retraction should be expected from this treatment. OR might be considered a suitable alternative treatment in TED for patients who cannot tolerate steroids.

Semiautomatic regional segmentation to measure orbital fat volumes in thyroid-associated ophthalmopathy. A validation study.

This study was designed to validate a novel semi-automated segmentation method to measure regional intra-orbital fat tissue volume in Graves' ophthalmopathy. Twenty-four orbits from 12 patients with Graves' ophthalmopathy, 24 orbits from 12 controls, ten orbits from five MRI study simulations and two orbits from a digital model were used. Following manual region of interest definition of the orbital volumes performed by two operators with different levels of expertise, an automated procedure calculated intra-orbital fat tissue volumes (global and regional, with automated definition of four quadrants). In patients with Graves' disease, clinical activity score and degree of exophthalmos were measured and correlated with intra-orbital fat volumes. Operator performance was evaluated and statistical analysis of the measurements was performed. Accurate intra-orbital fat volume measurements were obtained with coefficients of variation below 5%. The mean operator difference in total fat volume measurements was 0.56%. Patients had significantly higher intra-orbital fat volumes than controls (p<0.001 using Student's t test). Fat volumes and clinical score were significantly correlated (p<0.001). The semi-automated method described here can provide accurate, reproducible intra-orbital fat measurements with low inter-operator variation and good correlation with clinical data.

Spheno-orbital meningiomas: surgical approaches and outcome according to the intraorbital tumor extent.

OBJECT: This study proposes a topographical classification of spheno-orbital meningiomas. Its aim was to define whether the different intraorbital localizations require different surgical approaches and have different recurrence rates and outcomes. MATERIAL AND METHODS: Sixty patients with spheno-orbital meningiomas operated upon between 1983 and 2003 were reviewed. Four types were identified according to the extent of intraorbital tumor invasion: I: lateral or superolateral (15 cases); II: medial and inferomedial (8 cases); III: orbital apex (25 cases); IV: diffuse (12 cases). Three surgical approaches were used: lateral orbitotomy (15 cases with lateral or superolateral tumors), supraorbital-pterional approach (42 cases, including all 8 inferomedial cases, all 25 orbital apex cases, and 9 of 12 diffuse tumors), and a fronto-temporal-orbitozygomatic approach (only 3 cases with diffuse meningiomas and large-scale tumor invasion in the infratemporal fossa and cavernous sinus). RESULTS: Tumor removal was complete (Simpson grades I and II) in 40 cases, and incomplete in 20 (33.3%). There were two postoperative deaths (3.3%). A sufficient clinical follow-up was obtained in 52 cases. The clinical outcome was excellent in 26 patients (50%), good in 16 (30.8%), moderate in 6 (11.5%), and poor in 4 (7.7%). Twenty-two of 52 patients (42.3%) had tumor recurrence; however, 44 (84.6%) achieved tumor control after surgery alone through two or more operations. The recurrence rate was correlated with the Simpson grade of resection and the intraorbital tumor location. Significantly higher rates of recurrence were recorded for the orbital apex type (50%) and diffuse forms (60%), than for the inferomedial (28.5%) and superolateral forms (23%). CONCLUSIONS: Spheno-orbital meningiomas may be classified according to the location and extent of the intraorbital tumor invasion. The different localizations may require different surgical approaches, with different chances of complete removal. The location and extent of the intraorbital tumor results in different recurrence rates, lower for superolateral and inferomedial forms than for orbital apex and diffuse forms.

Unilateral exophthalmos associated with ipsilateral mucosal turbinate hypertrophy: benign exophthalmos syndrome (BES). A description of a new clinical condition.

PURPOSE: To report the clinical and radiologic characteristics of a group of patients who experienced unilateral exophthalmos associated with ipsilateral mucosal turbinate hypertrophy. The clinical features of these patients are presented and a hypothesis proposed to explain this condition for which the authors introduce the term benign exophthalmos syndrome (BES). METHODS: Retrospective, noncomparative case series. PARTICIPANTS: Four patients experienced slow progressive unilateral exophthalmos associated with ipsilateral mucosal turbinate hypertrophy, with no evidence of orbital mass or extraocular muscles involvement. INTERVENTION: Main Outcome Measures. Symptomatic outcome and measurement of the degree of relative exophthalmos. RESULTS: The onset of exophthalmos was associated with clinical and radiologic features that resemble BES. In all patients, radiologic examination demonstrated an ipsilateral mucosal turbinate hypertrophy and not the presence of orbital disease or expanding lesions of paranasal sinus. After daily intranasal spray of steroid, in three of the four cases the globe returned to within 1 mm of exophthalmometry of the contralateral eye. CONCLUSIONS: The relationship between the feature of paranasal sinus disease and the development of ipsilateral exophthalmos has been described in the literature. The four cases described herein appear peculiar for the slow progressive onset of the exophthalmos, without inflammatory and mass effect signs. This condition associated in all cases with ipsilateral hypertrophy of the nasal mucosa provides a guide to a hypothetical mechanism for BES. According to these hypothesis, the therapy should be devoted to the nasal disease more than the orbital.

Ophthalmic complications after surgery for nasal and sinus polyposis.

PURPOSE: To describe ophthalmic complications after nasal and sinus surgery. METHODS: Four cases with orbital complications were retrospectively selected from among more than 2000 cases of orbital pathologies. RESULTS: Motility disturbances due to extraocular muscle injury occurred in two patients after intranasal ethmoidectomy and in one patient after a Caldwell-Luc procedure. In the fourth case an orbital apex syndrome was noted after intranasal ethmoidectomies. CONCLUSIONS: Ophthalmic complications may occur after nasal and sinus surgery, even using an endoscopic procedure. Successful handling of these complications could be reached by on their early recognition and treatment.

Orbital venous-lymphatic malformations (lymphangiomas) mimicking cavernous hemangiomas.

PURPOSE: To illustrate that orbital venous-lymphatic malformations (lymphangiomas) may rarely simulate cavernous hemangiomas. METHODS: Retrospective case review. RESULTS: Five patients were identified from a series of 85 patients with venous-lymphatic malformations. The age range was 21 to 69 years, and all cases presented with a history of slowly progressive or long-standing proptosis. Computerized tomography revealed relatively homogeneous intraconal masses that were well defined anteriorly. Two of the cases had expansion of the orbit, and one had focal calcification. The three who had magnetic resonance imaging showed heterogeneous contrast enhancement. The preoperative diagnosis in every case was cavernous hemangioma, and intraoperatively the lesions resembled cavernous hemangiomas. However, posterior dissection was difficult in all patients because of dense adhesions and, in one case, led to a central retinal artery occlusion. The histology was characteristic of orbital venous-lymphatic malformations in all five cases. CONCLUSIONS: Deep orbital venous-lymphatic malformations presenting in adulthood may be rarely confused with cavernous hemangiomas. In doubtful cases, significant intralesional heterogeneity, best seen on magnetic resonance imaging, and focal calcification may help distinguish the two entities. This differentiation is important, because dissection of venous-lymphatic malformations is fraught with more complications than surgical excision of a cavernous hemangioma.

Traumatic displacement of the globe into the ethmoid sinus.

PURPOSE: To describe displacement of the globe into the ethmoid sinus after an orbital trauma. METHOD: Case report. A 58-year-old man sustained trauma of the left eye and orbit, which resulted in displacement of the globe into the ethmoid sinus. One day after injury, surgery was performed to restore the intact globe into position within the orbit. RESULTS: After operation, the globe held in its anatomical site, and 10 months after surgery the visual acuity was 20/100, slight pallor of the optic disk was present, and there was no limitation of the horizontal and vertical ductions. CONCLUSIONS: To our knowledge, this is the first case of traumatic displacement of the globe into the ethmoid sinus with satisfactory restoration of normal globe position and preservation of vision.

Orbital dermis-fat graft using periumbilical tissue.

Dermis-fat grafts are currently used in orbital reconstruction in a variety of procedures. The most frequent harvesting site is the gluteal area. However, we encountered some patients with anophthalmic socket who wished to avoid a visible scar on the buttock. In this article, we describe the effort to offer the patient an alternative donor site. Of the last 36 patients with anophthalmic socket who needed a dermal fat implant, 11 wished to avoid a visible scar on the buttock. To satisfy their requests we have endeavored to harvest the dermis graft from the periumbilical area. The rate of absorption, the motility, and the satisfaction of the patients were used as outcome measures and were analyzed carefully. Of 11 patients, 4 were women and 7 were men. The ages of these patients ranged from 24 to 56. The maximum follow-up was 137 months and the minimum 22 months, with a mean follow-up of 79 months. Some degree of absorption of the graft developed in one patient who had a severe absorption and required further operation. Of 11 cases, there were 7 with excellent motility, 3 with good motility, and 1 not evaluated. The motility was measured with the final prosthesis. The results for all patients were satisfactory. The periumbilical area has sufficient concentration of subdermal fat, and it is a relatively hair-free region as the lateral quadrant of the buttock. This area is a suitable alternative donor site of dermal fat implant for anophthalmic socket, especially in young women.

Orbital dirofilariasis.

PURPOSE: To warn the ophthalmologist to consider orbital dirofilariasis in the differential diagnosis of inflammatory masses of the orbit. PATIENTS: Two cases with non-specific swelling and pain of the orbit who had a initial diagnosis of "inflammatory pseudotumor of the orbit". RESULTS: These are two new cases of orbital dirofilariasis. CONCLUSION: In the ophthalmic literature, the highest incidence of ocular dirofilariasis is reported in Italy. Since both these patients had no history of travel in endemic areas of Dirofilaria, this parasitic infection is presumably not rare in Italy and is probably underdiagnosed.

Dermoid cysts: 16-year survey.

A total of 145 patients with orbital dermoid cysts examined at the Orbital Clinic, Institute of Ophthalmology, School of Medicine, University of Naples "Federico II" over a period of 16 years were reviewed. The orbital cysts were classified as exophytic and endophytic, according to their site of attachment in relation to the orbital rims. This classification can explain the different natural history of these lesions. The exophytic cysts growing externally are discovered in childhood, whereas the endophytic ones are discovered later in life when they produce bone damage, with or without invasion of the adjacent structures.