Michelle E. Wilson, MD, Sarah Thornton, BS, Ann P. Murchison, MD, MPH, Jurij R. Bilyk, MD
A 53-year-old man with hyperthyroidism presented with asymmetric proptosis and diplopia. Thyroid-stimulating immunoglobulin was elevated, suggesting active thyroid eye disease. Imaging of the orbits revealed enlargement of the extraocular muscles, including irregular enlargement of the left lateral rectus muscle. Biopsy of the lateral rectus muscle demonstrated infiltration of the muscle with Bcl-2 positive B lymphocytes consistent with chronic lymphocytic leukemia (CLL). Evaluation for systemic lymphoproliferative disease was negative. The patient was treated with orbital radiotherapy at specific dosages for both TED and CLL. He responded well to therapy with a reduction in proptosis and diplopia and no evidence of recurrent CLL.
Nicholas H. Andrew, MBBS, Sarah E. Coupland, MBBS, PhD, FRCPath (Professor), Adnan Pirbhai, MD, FRSC, Dinesh Selva, MBBS (Hons), FRACS, FRANZCO (Professor)
Lymphoid hyperplasia (LH) is a benign lymphoproliferative disorder that, in a minority of cases, may be associated with concurrent or metachronous non-Hodgkin lymphoma. LH cases are further subdivided into “reactive” and “atypical” categories based on the presence or absence of unequivocal malignant features. With improving molecular diagnostic technologies, “reactive” LH is by far the most common category of LH, with atypical LH accounting for only a small minority of specimens. Similarly, lesions previously diagnosed as LH are now being revised as low-grade B-cell non-Hodgkin lymphoma or diagnosed as newly described benign conditions such as IgG4-related disease. Additional differential diagnoses include specific and nonspecific orbital inflammations, infiltrative processes, and depositions. Hence, there are emerging changes in the patterns and proportions of entities that fall within the spectrum of lymphoproliferative disorders of the orbit and ocular adnexa. Reactive LH and low-grade malignant lymphoproliferative disorders in the orbit and ocular adnexa are clinically and radiologically indistinguishable from each other, requiring tissue biopsy in all cases. The prognosis of ocular adnexal LH is generally favorable, but the small risk of non-Hodgkin lymphoma mandates follow-up for at least 5 years. We summarize the current state of knowledge on LH occurring in the orbit and ocular adnexa.
Petrina Tan, FRCS (Ed), Raman Malhotra, FRCOphth
The incidence of glaucoma worldwide is increasing. Often, these patients may also be referred to oculoplastic surgeons for either age-related problems or issues secondary to medical or surgical glaucoma treatment. Eyelid malpositions (ptosis or lid retraction) may occur after glaucoma filtration surgery or the use of prostaglandin analogues. We review the incidence and mechanisms of lid malpositions found in the literature. Surgical correction for these malpositions also requires careful consideration and should be tailored to the individual patient to avoid undesirable surgical outcomes. For instance, overcorrection of ptosis may result in bleb exposure and the risk of blebitis or endophthalmitis. As use of topical prostaglandin analogues increases, we summarize the reports of prostaglandin analogue-associated periocular changes and its underlying etiologic mechanisms. Cessation of these medications may be required in some patients. Where topical treatment is to be continued, eyelid surgery has to be individualized and approached conservatively. It is imperative that patients with glaucoma and oculoplastic concerns be considered as a distinct entity to avoid unfavorable outcomes.
Franziska Bucher, MD, Julia Fricke, MD, Antje Neugebauer, MD, Claus Cursiefen, MD, Ludwig M. Heindl, MD
Parry-Romberg syndrome is a rare disease characterized by slowly progressive atrophy affecting facial subcutaneous tissues, including the underlying muscles and osteocartilaginous structures. Various periocular, ocular, and neuro-ophthalmological manifestations have been described in Parry-Romberg syndrome. The most common periocular disorders include enophthalmos, eyelid, and orbit alterations. The most frequent ocular disorders include corneal and retinal changes, and the most common neuro-ophthalmological disorders involve optic nerve, ocular motor and pupillary dysfunction. Besides the characteristic facial abnormalities, systemic manifestations may occur, including neurologic, dermatologic, cardiac, endocrine, infectious, orthodontic, and maxillofacial disorders. So far, mainly brief case reports describe these ophthalmological findings. Therefore, we summarize the ocular, periocular, and neuro-ophthalmological findings in detail, describe diagnostic modalities, and outline therapeutic options.