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Exophthalmos (Proptosis)
Published in K. Gupta, P. Carmichael, A. Zumla, 100 Short Cases for the MRCP, 2020
K. Gupta, P. Carmichael, A. Zumla
Exophthalmos or proptosis is characterized by a staring expression with prominence of the eyeballs and retraction of the upper eyelids. Hyperthyroidism is a common cause of exophthalmos. Although in most patients with hyperthyroidism the condition is bilateral, in the early stages, exophthalmos may be more marked in one eye. Exophthalmos may also develop in an eye of a patient previously treated for hyperthyroidism. Beside lid retraction, lid lag and wide palpebral fissures are important physical signs that usually result because of excessive sympathetic stimulation. Infiltrative ophthalmopathy occurs due to what is thought to be autoimmune disease. Retro-orbital fat, connective tissue, and the muscles are all involved.
Organ-specific autoimmune diseases
Published in Gabriel Virella, Medical Immunology, 2019
Gabriel Virella, George C. Tsokos
One of the clinical hallmarks of Graves’ disease is exophthalmos (protrusion of the eyeball). Exophthalmos can be unilateral or bilateral and may be associated with proptosis, conjunctivitis, and/or periorbital edema. Exophthalmos is secondary to retro-orbital accumulation of fibroblasts, adipocytes, and muscle cells, all of which exhibit TSH receptor antigen to which TSI binds and alters their function.
Endocrinology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Mehul Dattani, Catherine Peters
Eye signs of Graves disease are variable. Severe Graves ophthalmopathy is much less common in children than in adults. Malignant exophthalmos is virtually unknown. Features in childhood include lid retraction, lid lag, exophthalmos, proptosis (Fig. 13.44), ophthalmoplegia, chemosis of the conjunctiva, pain, swelling and irritation. Graves dermopathy, whereby there is accumulation of mucopolysaccharides in skin and subcutaneous tissues, is rare in children.
Extraocular muscle enlargement in retinoencephalofacial angiomatosis
Published in Orbit, 2020
Alisha Kamboj, Andrea A. Tooley, Kyle J. Godfrey, Mary D. Maher, Hermann D. Schubert, Michael Kazim
The differential diagnosis of EOM enlargement includes thyroid eye disease and other inflammatory, vascular, and neoplastic conditions. Arteriovenous communications of the orbit may secondarily enlarge the EOMs through infiltration, bleeding, and pressure-related effects.6 Nevertheless, this is the first report, to the authors’ knowledge, of retinoencephalofacial angiomatosis presenting with proptosis in the setting of EOM enlargement. In this case, anomalous vessels were noted throughout the left orbit and temporal fossa soft tissues, and may have contributed to EOM enlargement. These vessels infiltrated the intraconal fat to form a large vascular malformation. It is likely that the same vascular infiltration into the EOMs is responsible for their enlargement. While impaired venous outflow through the malformation may also have contributed to the muscle enlargement, one would expect that the same venous outflow obstruction would produce dilation of the superior ophthalmic vein, not seen in this patient. In addition, the vessels penetrated and enlarged the superficial and deep bellies of the temporalis muscle and permeated the subcutaneous fat to form a cutaneous vascular excrescence. For this patient, surgery to reduce exophthalmos was not recommended due to risk of hemorrhage and diplopia. Instead, ongoing clinical observation was advised to monitor the degree of proptosis. Orbital bone decompression may be considered if progressive proptosis leads to diplopia, optic neuropathy, and exposure keratopathy.
Thyroid eye disease presenting with superior rectus/levator complex enlargement
Published in Orbit, 2020
Yao Wang, Pradeep Mettu, Talmage Broadbent, Phillip Radke, Kevin Firl, J. Banks Shepherd, Steven M. Couch, Angeline Nguyen, Amanda D. Henderson, Timothy McCulley, Collin M. McClelland, Ali Mokhtarzadeh, Michael S. Lee, James A. Garrity, Andrew R. Harrison
We identified TED patients presenting with enlarged superior rectus/levator complex enlargement at four academic institutions. The clinical presentation of these patients differed from that of the previously-described cohort of all TED patients from Olmsted County, Minnesota.1 Of our nineteen study patients, 63.2% had hyperthyroidism, compared to 90% in the incidence cohort, and 26.3% were euthyroid, compared to 6% in the incidence cohort. All of our patients presented with exophthalmos compared to 62% in the incidence cohort. 63% of our patients presented with upper eyelid retraction, versus 71% in the incidence cohort. Only 17% of patients in the incidence cohort suffered from diplopia, versus 63% in our TED population (all but two had vertical misalignment). Even though a quarter of our patients were euthyroid, it is important to note that average TSI was elevated at 3.7 and TRAb was 2.5IU/L. Although TSI initially was normal in one patient (Figure 1), it was elevated several months later upon recheck. This finding suggests there is a lag time to positive seroconversion, and consideration should be given to rechecking antibody titers if they are initially normal.
Lacrimal gland pleomorphic adenoma: a review of 52 cases, 15-year experience
Published in Acta Oto-Laryngologica, 2019
Pedro Clarós, Emmanuel Choffor-Nchinda, Marta Lopez-Fortuny, Aleksandra Zofia Sobolewska, Andres Clarós
Exophthalmos was defined as a proptosis greater than 18 mm from the lateral orbital rim to the corneal apex on Hertel’s exophthalmometer, or a difference between both eyes greater than 2 mm. Raised intraocular pressure was considered when the intraocular pressure was greater than 22 mmHg or >2 mmHg difference between the two eyes. Tumour sizes were grouped into small tumours, measuring strictly less than 1.5 cm, medium-sized tumours between 1.5cm to 3cm inclusive and large tumours strictly greater than 3 cm. These measurements were CT-scan based, taking into account the axial length of the lesions. Duration of symptoms, defined as time of evolution of symptoms prior to consultation, was grouped as follows: <10 months, 10–19 months, 20–29 months and ≥30 months.