Orbital and peri-orbital amyloidosis: a report of four cases.

Our objective was to describe our experience with orbital amyloidosis, and illustrate the different forms of presentation. This was a retrospective case series of four patients with biopsy-proven orbital amyloi- dosis, over the period from 2014 to 2016. We describe its diagnostic and clinical characteristics, management and systemic study. The series comprised three women and one man of mean age 52 ± 9.4 years. Affected sites were the lacrimal gland, tarsal conjunctiva, lacrimal sac and orbit. In three of the four patients, calcifications were observed. Three patients had associated ptosis. The patient with orbital involvement suffered an unusual vascular complication during surgery and systemic disease was detected. Management included debulking and complete resection of the lesion. In conclusion, orbital amyloidosis presents as a wide variety of forms. Its diagnosis is biopsy-based. Calcifications in biopsy specimens or images should raise suspicion of amyloidosis. It is important to always check for systemic amyloidosis.

Transcaruncular rectus tendon fixation to the orbit and frontalis flap for complete third nerve palsy.

PURPOSE: To describe our experience and outcomes managing complete third cranial nerve palsy. METHODS: This was a retrospective analysis of the clinical records of 7 consecutive patients treated at our centre for unilateral third nerve palsy over the period 2010-2016. We describe our surgical approach using a frontalis muscle flap to correct the eyelid ptosis associated with medial fixation of the rectus muscle tendon to the orbit to correct the horizontal deviation. RESULTS: The seven patients, four women and three men, were of mean age of 44 ± 19 years [18-75 years]. Follow up was 29 ± 31 months [5-82 months]. In the preoperative exam, exotropia in prism diopters (PD) was -70 ± -28 PD [-30 to -90 PD]. At the end of follow up, this was reduced to -11 ± -14 PD [0 to -30 PD]. Preoperative marginal reflex distance 1 (MRD1) was -4 ± 1 mm [-3 to -5 mm] and palpebral fissure height (PFH) was 0.5 ± 1 mm [0-2 mm]. Surgical undercorrection was the target in all patients due to the absent or poor Bell's phenomenon. At the end of follow up, MRD1 was 2.5 ± 0.5 mm [2-3 mm] and PFH was 7 ± 1 mm [6-8 mm]. Cosmetic and functional results were good in all patients. CONCLUSIONS: Medial fixation of the rectus muscle tendon to the orbit associated with a frontalis muscle flap is a valid option for the treatment of exotropia and ptosis in patients with third cranial nerve palsy.

Radiation maculopathy after epimacular brachytherapy for the treatment of subfoveal choroidal neovascularization secondary to age-related macular degeneration.

PURPOSE: To describe a case of radiation maculopathy after a 24-Gy single-fraction epimacular brachytherapy delivered concomitantly with ranibizumab for the treatment of age-related macular degeneration. METHODS: Case report. RESULTS: An 82-year-old man with neovascular age-related macular degeneration was treated with epiretinal brachytherapy and 2 intraocular injections of ranibizumab with initial good response. Nineteen months after initial treatment, visual acuity decreased and the patient was diagnosed of radiation maculopathy. CONCLUSION: Radiation-related ocular side effects such as maculopathy may become evident several years after the treatment. Thus, vascular abnormalities that may appear in the follow-up of these patients can easily be misdiagnosed as a complication of the previous choroidal neovascularization.

Rabdomioma cardiaco como manifestación de esclerosis tuberosa. Presentación de dos casos y revisión de la literatura / Cardiac rhabdomyoma as manifestation of tuberous sclerosis. Presentation of two cases and literature review

Los rabdomiomas cardiacos son tumores benignos dependientes de las fibras musculares miocárdicas, los cuales usualmente son múltiples, pero tienden a disminuir tanto en número como en tamaño con el crecimiento, con una regresión espontánea en 90% de los casos. Hacen parte de los tumores cardiacos primarios, los cuales son poco frecuentes, con una incidencia que varía entre 0,0017% y 0,28%. El más frecuente de los tumores cardiacos primarios es el rabdomioma. Se describe asociación con esclerosis tuberosa hasta en 72% de los casos, razón por la cual ésta debe buscarse ante el hallazgo de rabdomioma cardiaco.

Cardiac rhabdomyomas are benign tumors derived from cardiac muscle fibers. They are usually multiple, but tend to decrease both in number and size with growth, with spontaneous regression in 90% of cases. These lesions are part of the primary cardiac tumors, which are uncommon, and have a variable incidence between 0.0017 and 0.28%. The most common primary cardiac tumor is the rhabdomyoma. An association between rhabdomyoma and tuberous sclerosis has been described in up to 72% of cases. For this reason, a patient with cardiac rhabdomyoma should be investigated for tuberous sclerosis.

Ring melanoma of the ciliary body: clinical and ultrasound biomicroscopic characteristics.

BACKGROUND: Diagnosis of ring melanoma is clinically difficult since the mass can remain hidden with standard slit-lamp biomicroscopy. The aim of this study was to evaluate the utility of ultrasound biomicroscopy (UBM) as a diagnostic tool for ring melanoma of the ciliary body. METHODS: This was a retrospective study of 6 eyes of 6 patients at Princess Margaret Hospital, Toronto, Ont., with a diagnosis of ring melanoma of the ciliary body. The tumor extension was measured clinically, gonioscopically, ultrasonographically (by UBM), and pathologically. RESULTS: Since 2000, 6 cases of ring melanoma have been diagnosed at Princess Margaret Hospital: 2 women and 4 men, median age 57 years. Five patients presented as uncontrolled hyperchromic glaucoma (83%). Ciliary body involvement on slit-lamp examination ranged from 60 to 180 degrees. Ciliary body involvement as assessed by UBM ranged from 180 to 300 degrees. Tumor extension according to pathological examination ranged from 210 to 360 degrees. With these measures, the ring melanoma clinical criteria were not filled by 5 patients. INTERPRETATION: UBM is an important tool in determining the extent of ciliary body involvement and classifying these lesions.