Wu, Chris Y. M.D.; Stacey, Andrew W. M.D., M.S.; Kahana, Alon M.D., Ph.D.
Purpose: To compare the visual and exophthalmometry outcomes of simultaneous versus staged balanced decompression in patients with thyroid-related compressive optic neuropathy (CON).
Methods: All consecutive patients who underwent simultaneous or staged balanced decompressions for clinically diagnosed thyroid-related CON performed by 4 surgeons at the Kellogg Eye Center of the University of Michigan between 1999 and 2014 were included in the study. Demographic, medical, and surgical covariates were collected. Orbits were stratified by decompression technique. Primary outcomes were improvement in CON score (which quantifies visual dysfunction using logarithm of the minimum angle of resolution, color vision, and Humphrey visual field mean deviation), and improvement in proptosis. The authors performed univariate and descriptive statistics to identify baseline differences and covariates associated with the outcomes of interest; multivariate mixed linear regression models (to adjust for interorbit correlation) were then constructed with inclusion of potential confounders with p value ≤0.1.
Results: In total, 80 orbits of 53 patients were included in the study. Of the 80 orbits, 61% underwent simultaneous balanced decompression, and 39% underwent staged balanced decompression. Mean CON score reduction was 6.12 ± 9.7 and mean proptosis reduction was 5.63 ± 2.6 mm. Staged balanced decompression was significantly associated with greater CON score reduction (p = 0.038). However, staged (vs. simultaneous) decompression technique did not remain an independent predictor (p = 0.950) after multivariate analysis adjusted for confounders. For proptosis reduction, there were no statistically significant differences between simultaneous and staged balanced decompression on univariate (p = 0.122) or multivariate mixed linear regression models (p = 0.812).
Conclusion: Simultaneous and staged balanced decompression are equally efficacious in treating visual dysfunction and exophthalmos due to thyroid eye disease in patients with clinically diagnosed CON. Patient choice should be the primary consideration and care should be individualized. Further studies validating the CON score used in the study and comparative studies individualizing surgical treatment of thyroid eye disease are warranted
Greenberg, Erin L. M.D.; Cox, Kyle F. M.D.; Margo, Curtis E. M.D.; Leyngold, Ilya M. M.D.
A 74-year-old white man presented 32 years after being clinically diagnosed with uveal melanoma. On examination, a necrotic, fungating mass emanated from the left orbit (Fig. 1). An orbit CT scan showed a heterogenous lesion occupying the entire orbit and engulfing a phthisical globe (Fig. 2). Systemic work-up for metastatic disease was negative. Exenteration with split-thickness skin graft was performed. Histopathologic examination showed that the orbit was effaced by sheets of malignant melanocytes (HMB-45 and S-100 positive) and necrotic tissue (Fig. 3). The surgical margins were uninvolved. The patient remained disease free at a two-year follow up.
Ali, Mohammad Javed F.R.C.S.; Mishra, Dilip Kumar M.D.
Lacrimal sac wall granulomas are uncommon and secondary to chronic inflammation. A female, 60 years old presented with left-sided epiphora of 2 years duration and a rapidly growing, palpable, firm lesion below the medial canthus of 2 months duration, extending laterally towards the inferior orbital rim (Fig. 1A). The lesion was nontender and showed no regurgitation on pressure. Irrigation of the lacrimal passages was suggestive of a left acquired nasolacrimal duct obstruction. CT scan showed a mass lesion in the lacrimal sac region without any overlying bony erosions. An impression of possible lacrimal sac neoplasm was made and patient underwent an exploration and extirpation of the sac. Gross examination showed the lesion to arise from the lateral wall of the lacrimal sac (Fig. 1B). Whole mount section of the lesion showed the mass to arise from the lateral sac wall all along its entire extent (Fig. 1C). Histopathological examination revealed features suggestive of a chronic granuloma arising from the chronically inflamed and fibrosed sac wall (Fig. 1D). Taking the endemic geographical nature (South India) into account, detailed laboratory work up was performed to rule out granulomas of tubercular or fungal origin, which yielded negative results. Although uncommon, lacrimal sac granulomas should also be kept in the differential diagnosis of a rapidly growing lacrimal sac mass.
Grob, Seanna R. M.D., M.A.S.; Jakobiec, Frederick A. M.D., D.SC.; Rashid, Alia M.D.; MacIntosh, Peter M.D.; Kelly, Hillary M.D.; Fay, Aaron M.D.
A 13-year-old female presented with left unilateral proptosis, blurry vision, and diplopia. Clinical examination showed left sided visual acuity of 20/50, limited extraocular movement, 5-mm proptosis, and optic disc edema. CT and MRI displayed a large, intraconal, well-demarcated soft tissue mass with inferotemporal displacement of the optic nerve. The imaging appearance was unusual and diagnosis remained uncertain. Histopathologic analysis of the biopsy specimen confirmed the diagnosis of atypical syncytial meningioma. The tumor cells were positive for both androgen and progesterone receptors and the Ki67 stain was positive (proliferation index of 8%). The patient was treated with proton beam radiation therapy (total dose 50.4 GyE) that suppressed tumor growth and has preserved visual acuity to date (20/40). Differential diagnosis and approaches to therapy are explored.
Davies, Emma C. M.D.; Jakobiec, Frederick A. M.D., D.Sc.; Stagner, Anna M. M.D.; Iwamoto, Mami A. M.D.
A rapidly growing, large (horizontal diameter of 3.1 cm) eyebrow lesion in a nonagenarian patient was found on pathologic examination to demonstrate an admixture of islands of anucleated, washed out eosinophilic “ghost” cells with surrounding nucleated, small germinal basaloid cells. Further analysis disclosed adipophilin granular positivity in the necrotic zones, negative nuclear staining for androgen receptor and strong nuclear positivity for Ki67 in the basaloid cells (proliferation index of 50%). These findings are consistent with a highly mitotically active pilomatrixoma. The lesion recurred after initial resection but returned the same histopathologic features as the primary. Several clinical features were notably atypical for pilomatrixoma—specifically, the age of the patient, rapid lesion growth and recurrence, and clinical appearance and large size of the mass. The immunohistochemical findings can help to distinguish this tumor from other skin neoplasms, especially sebaceous carcinoma in an older individual.
Richardson, Marc A. M.D.; Lewis, Kyle T. M.D.
This is a case of a 44-year-old female with a history of Graves’ orbitopathy presented to the emergency department after waking from a nap with sudden onset of left facial and periorbital swelling, ecchymosis, and subconjunctival hemorrhage. A CT scan obtained in the emergency department revealed a left blowout fracture and enlarged extraocular muscles. The patient lives with her mother and both adamantly denied any trauma. The patient had sustained a spontaneous orbital fracture; a process reported but few times in the medical literature.
Compton, Christopher J. M.D.; Clark, Jeremy D. M.D.; Thompson, Matthew P. D.O.; Lee, Hui Bae H. M.D., F.A.C.S.; Nunery, William R. M.D., F.A.C.S.
A 13-month-old boy was presented with new onset proptosis of the right eye. CT scan and MRI showed an enhancing mass in the right superior orbit with local bone remodeling and erosion. A craniotomy was performed for biopsy and sub-total resection. Histopathology and immunohistochemistry confirmed the lesion to be nodular fasciitis. Nodular fasciitis lesions are classically found in the anterior ocular adnexa, especially in pediatric patients. This is the first reported case of nodular fasciitis arising in the posterior orbit of a child younger than 16.
Hussain, Rumana N. M.B.B.S., M.A. Oxon., F.R.C.Ophth.; Clark, Martin M.B.B.S., F.R.C.S.; Berry-Brincat, Antonella M.D., M.R.C.Ophth
A good functional and cosmetic result after midfacial reconstructive surgical procedures is of paramount importance. We describe the use of a Polyetheretherketone (PEEK) implant to reconstruct the midface area, after extensive mutilating surgery due to an infiltrative skin tumor. A 67-year-old male patient underwent multiple and extensive surgeries to the left cheek and lower lid because of a highly aggressive metatypical basal cell carcinoma. Complete resection of the recurrent tumor resulted in a cosmetically evident absent cheek contour and facial deformity. The PEEK implant was used to restore the bony cheek contour, with good aesthetic outcome and restoration of the facial symmetry. Preoperative planning with 3-dimensional CT scans allow for customization of the implant. PEEK implants have been scantily described in the periorbital region. The material has a very low reported morbidity and also has the advantage of improving intraoperative predictability and reducing surgical time in complex reconstructive procedures.
Tran, Ann Q. BS; Thuro, Bradley A. MD; Albert, Daniel M. MD; Lucarelli, Mark J. MD; Potter, Heather D. MD
A 6-year-old girl presented with a left nodular mass around the punctum. Previous debulking in a similar location at 10 weeks and 8 months of age confirmed fibrous hamartoma of infancy. Pathology at the initial surgery revealed benign-appearing fibroadipose tissue, vasculature, and smooth muscle. Pathologic examination from the third debulking illustrated less fibrous trabeculae extending into increased amounts of mature-appearing adipocytes with collections of immature-appearing fibrocytes. The lesion had slight differences in pathology compared with prior surgical specimens; however, was still consistent with fibrous hamartoma of infancy. No recurrence has been reported since the last surgery.
Jakobiec, Frederick A. M.D., D.Sc.; Rashid, Alia M.B.Ch.B.; Yoon, Michael K. M.D.
A 56-year-old man developed a 2 × 2 mm, yellow-orange painless, smooth-surfaced nodule in the middle third of his left upper eyelid. Microscopic evaluation disclosed a spindle cell tumor that was well circumscribed by a perineurium. The tumor cells manifested wavy, bland nuclei displaying comma-shaped and pointed ends. These cells were uniformly S100 positive. Immunohistochemical analysis further revealed an even dispersion of numerous dot-like neurofilaments diagnostic of an isolated intraneural neuroma. A neuroma differs from a schwannoma which fails to exhibit neurofilaments except in a peripherally located compressed nerve of origin. Simple excision is recommended.