Orbital amyloid tumor associated with myasthenia gravis.

We present a case of a 53-year-old female with an 11 year history of myasthenia gravis (MG) with palpebral ptosis in the left eye which had become more marked over the previous year. Examination revealed a painless left orbital mass causing ptosis. The tumor was surgically removed and histopathology revealed deposits of a hyaline substance which when stained with Congo Red had an apple-green birefringence with polarized light, typical of amyloid. In the absence of amyloid deposits elsewhere in the body, amyloid tumor (AT) was diagnosed. No myelo- or lymphoproliferative syndromes, systemic involvement by amyloidosis or any autoimmune disease were found. The evolution and aesthetic results where satisfactory. Only two cases of orbital AT associated with MG have been described previously; however, in one of the cases, the symptoms of the AT had led to a false diagnosis of MG.

Impact of the ethmoid volume on endoscopic medial wall decompression outcomes in Graves' orbitopathy.

BACKGROUND: Endoscopic orbital decompression is a well-established technique for Graves orbitopathy treatment. AIMS: Measurement of the ethmoid volume and its correlation with proptosis reduction in patients undergoing endoscopic orbital decompression for Graves' orbitopathy. METHODS: We retrospectively reviewed clinical charts of 209 patients (319 orbits) operated by a surgeon at our institution between 1998 and 2019. RESULTS: Average age was 46.9 years. About 83.3% of the patients were female, 54.6% were smokers, 93.8% were euthyroid at the time of decompression, 3.8% were hypothyroid and 2.4% were hyperthyroid. About 77.5% of the patients underwent bilateral surgery, the remainder and unilateral. Subsequent stages included strabismus surgery in 22.5% patients, Müllerectomy 37.8%, with or without canthoplasty (14.8 vs. 23%). Mean proptosis reduction was 4.61 mm. Mean ethmoid volume was 5.57 mm3. Ethmoid volume had a moderate positive correlation with proptosis reduction (r s = 0.49, p < .001). Further investigation, statistically significant moderate correlation was found only in the small (SE) and big (BE) ethmoid groups. CONCLUSION: We advise evaluating ethmoid sinus pneumatization on computed tomography before decompression to estimate possible anatomical limitations. Additional wall decompression might be advocated in most severe cases.

Orbital cavernous haemangioma; profile and outcome of 76 patients managed surgically.

Background: Orbital cavernous haemangioma (OCH) is one of the most common primary orbital tumours in adults. They can encroach on intraorbital or adjacent structures and be considered 'anatomically' malignant. Aims: To report a series of patients that were all managed surgically, with possible specificities and propose predictors of treatment outcome. Methods: We realised a hospital-based retrospective case review including data from 76 patient files. Results: The mean age was 37.8 years. Unilateral proptosis was observed in all patients. Visual loss was found in 32 patients (42.1%). Findings on eye examination included lagophthalmos (76.3%), blepharoptosis (21.1%), complications of corneal exposure (19.6%), strabismus (13.2%) and fundoscopic abnormalities (60.5%). Abnormal fundoscopy was significantly associated with decreased visual acuity (VA) (p < .001). Small tumours were predominant (65.8%) and size was associated with VA (correlation coefficient r = -0.5, p < .001). Surgery was mainly by lateral orbitotomy (94.7%), with early post-operative complications in 18 patients (23.7%). Multivariable analysis showed that tumour size, preoperative VA and abnormal fundoscopy were significantly associated with postoperative VA. Conclusion: Clinical and radiological profiles are consistent. Tumour size, preoperative VA, and abnormal fundoscopy seem to be important factors that could influence outcome expectations. Surgical management is still predominantly by open approaches. Severe complications remain rare.

Silent sinus syndrome: combined sinus surgery and orbital reconstruction - report of 15 cases.

BACKGROUND: Silent sinus syndrome (SSS) is defined as spontaneous, painless enophthalmos, hypoglobus with orbital floor resorption and maxillary sinus collapse on the ipsilateral side. Different methods of orbital floor reconstruction have been proposed. AIMS/OBJECTIVES: The purpose was to analyse the results of combined endoscopic sinus surgery (ESS) and reconstruction using orbital floor implant of 15 patients with SSS and to present recent histological findings. MATERIALS AND METHODS: Retrospective case review of 15 patients with SSS treated in clinic between 2007 and 2017. RESULTS: Eleven women and four men presented with unilateral, spontaneous enophthalmos. Averaged duration of enophthalmos was 10.7 months. On affected side, mean enophthalmos was 2.6 mm and hypoglobus 2.7 mm. Computed tomography imaging (CT) imaging showed maxillary sinus opacification on the affected side in every case, and the orbital floor was displaced downwards in all cases. In total, 13 patients underwent simultaneous ESS and rebuilding of orbital floor with a titanium implant. Statistical analysis confirmed significant differences for pre- and postoperative measure of enophthalmos and hypoglobus. CONCLUSION AND SIGNIFICANCE: Implementation of titanium implants is the reliable method of reconstruction that allows good aesthetic result, shorter time of procedure with an excellent long-term outcome and satisfactory patient's tolerance.

Lacrimal gland pleomorphic adenoma: a review of 52 cases, 15-year experience.

BACKGROUND: Lacrimal gland (LG) tumours are rare neoplasms. Pleomorphic adenoma (PA) is the most common histologic variant, representing ∼20% of all LG tumours. PA tends to recur leading to great morbidity. AIMS: We carried out this study to share our experience and provide recent data on the clinical aspects, radiologic findings, management and outcome after treatment. METHODS: We realised a hospital-based retrospective case review including data collected from 52 patients, managed over 15 years. RESULTS: The mean age was 39.8 ± 2 years. Unilateral painless proptosis was the most constant sign (51.9%). CT-scan showed predominantly isodense lesions (96.2%), with regular borders (94.2%), measuring on average 2 cm. MRI showed isointense lesions on T1-weighted images in 96.2% of cases. No pre-operative biopsy was done. Surgical management was mainly external lateral orbitotomy (94.2%). There was no recurrence, considering a mean follow-up period of 12.6 years. Size of tumour at the time of diagnosis increased with age (r = +0.36, p = .01). CONCLUSIONS: Clinical and radiologic characteristics are consistent with literature. Older patients seem to present larger tumours. We believe that biopsy is not necessary if appropriate imaging is done. Complete, intact resection is generally sufficient to minimise the risk of recurrence.

Treatment of recurrent retinal detachment secondary to optic nerve coloboma with injection of autologous platelet concentrate.

A 14-year-old girl presented with a recurrent retinal detachment secondary to optic nerve coloboma in her left eye with a small retinal hole in the bed of the coloboma, confirmed by optical coherence tomography. The patient was treated by injection of 0.05 mL of autologous platelet concentrate into the coloboma along with vitrectomy and gas exchange. After 8 months, the retina was attached and optical coherence tomography revealed closure of the retinal hole. Considering our experience, autologous platelet injection may be considered a treatment option for recurrent retinal detachment secondary to optic nerve coloboma.