Amanda C. Maltry

Chronic anophthalmic socket pain treated by implant removal and dermis fat graft

Aims To report the outcome of orbital implant removal and dermis fat graft (DFG) implantation in patients with chronic anophthalmic socket pain (ASP), in whom all detectable causes of pain had been ruled out and medical management had failed. Methods Retrospective, multicentre case series. A review of all cases undergoing orbital implant replacement with DFG between 2007 and 2013 was conducted at the University of Iowa Hospitals and Clinics (UIHC), USA, and St. Erik Eye Hospital, Sweden. Inclusion criteria included (1) chronic ASP >2 years and unresponsive to treatment, (2) absence of pathological or structural cause for pain established by socket examination and orbital imaging, and (3) minimum 12-month post-surgical follow-up. Results Six cases with chronic ASP were identified, four were post-enucleation and two were eviscerated at an average age of 45 years. The incidence of chronic ASP among enucleations at UIHC over a 6-year period was 0.7%. Indications for enucleation and evisceration included tumours and glaucoma. Intractable ASP had been present for an average of 11 years and persisted despite medical management. All patients were free of pain within 3 months of implant removal and DFG placement and remained pain free at an average 24 months following surgery. Conclusions Orbital implant replacement with DFG was effective at relieving chronic ASP, and pain resolution was sustained in all cases. This surgical intervention may be a useful management option for patients in whom all detectable causes of chronic pain have been excluded and have failed medical pain management.

Immunopathology and histopathology of conjunctival biopsies in patients with presumed idiopathic punctal stenosis

Background/aims Numerous processes have been implicated as causes of punctal stenosis. Recent studies have highlighted inflammation in punctoplasty specimens in patients with punctal stenosis. Conjunctival biopsy has been suggested as a means to determine underlying aetiologies, although little is known regarding what conjunctival pathologies are associated with punctal stenosis. Our objective is to examine the pathological and immunological findings in conjunctival biopsy specimens in patients with presumed idiopathic punctal stenosis. Methods A retrospective chart review was performed at a single institution over a 5 year period of patients with presumed idiopathic punctal stenosis who underwent conjunctival biopsy for histopathological and/or direct immunofluorescence (DIF) examination. Patients with known aetiologies of punctal stenosis were excluded. Results 23 patients met inclusion criteria. Conjunctival biopsies (n=36) from all 23 patients underwent histological examination. 35 specimens (97.2%) showed lymphocytic infiltrates. Two patients (8.7%) had findings suggestive of sarcoidosis. Conjunctival biopsies from 18 of the 23 patients were also evaluated by DIF. Nine patients (50.0%) had fibrinogen characteristics suggestive of lichen planus. There were no complications related to the conjunctival biopsy procedures. Conclusions Conjunctiva appears to be an excellent proxy in evaluating patients with presumed idiopathic punctal stenosis. The biopsy is safe and provides ample tissue. Clinically, abnormal tissue should be targeted, if available. 11 of the 23 patients in this study demonstrated findings consistent with underlying immunological disorders, suggesting that conjunctival biopsy may play a role in identifying underlying aetiologies of punctal stenosis. Knowledge of these underlying conditions impacted treatments for several patients.

A Unique Ocular Presentation of Extragenital Lichen Sclerosus.

Lichen sclerosus is a chronic, debilitating, and recurring disease that is most commonly seen affecting the anogenital region. Extragenital locations of lichen sclerosus has been well documented, frequently seen in the shoulders, neck, trunk, breasts, and arms, however, infrequently of the face. Specifically, extragenital lichen sclerosus has been reported in several cases to be involving the infraorbital region, but to our knowledge it has never been found affecting the adnexa of the eye. To our understanding, this is the first documented report of a patient with extragenital lichen sclerosus of an eyelid.

Chronic inflammatory granulomata after use of GenTeal gel during strabismus surgery

Subconjunctival granulomata developed in 2 patients after use of GenTeal gel to lubricate the cornea during strabismus repair. We report the clinical findings and histopathology of the subconjunctival nodules. These lesions are similar to those previously reported following use of GenTeal gel in retinal surgery and use of similar large polymers in nonocular surgeries. Copious irrigation should be considered when conjunctival wounds are exposed to GenTeal gel, and alternative corneal wetting agents should be considered for use during strabismus surgery.

Periorbital necrobiotic xanthogranuloma successfully treated with intravenous immunoglobulin

Background: Necrobiotic xanthogranuloma (NXG) is a rare non-Langerhans histiocytosis with cutaneous manifestations, most commonly of the periorbital skin, and is often associated with hematologic disorders such as monoclonal gammopathy. Treatment of NXG is notoriously difficult, and fraught with recurrence and progression. Case Presentation: The authors describe a case of NXG with periorbital involvement in a patient with a complex autoimmune and hematologic medical history. The biopsy of this rare lesion prompted subsequent evaluation for an underlying disorder, which led to the diagnosis of multiple myeloma. Her NXG lesions demonstrated remarkable clinical improvement after treatment with intravenous immunoglobulin (IVIG). Conclusions: This case demonstrates the ophthalmologist’s critical role in the diagnosis and management of NXG, as early detection cannot only prevent ophthalmic consequences such as ocular perforation and blindness, but also prompt further investigation that may reveal an underlying disorder or systemic involvement, including hematologic malignancy as in this case. NXG has been effectively treated with IVIG in a handful of reported cases. To the author’s knowledge, this is the third case of periorbital NXG successfully treated with IVIG, and the first in the ophthalmic literature.

Waiting to deliver a final diagnosis

We report a patient with left eye positional transient visual obscurations, diplopia, a left afferent pupillary defect, and optic disk edema-all occurring during pregnancy. Noncontrast magnetic resonance imaging revealed an orbital mass encasing the optic nerve and extending through the superior orbital fissure into the cavernous sinus. An magnetic resonance imaging with gadolinium after parturition and strongly positive somatostatin receptor scintigraphy suggested the diagnosis of meningioma. Biopsy confirmed the diagnosis, and she was treated with CyberKnife stereotactic radiosurgery. In cases of atypical presentation, somatostatin receptor scintigraphy can help distinguish optic nerve sheath meningioma from alternative orbital masses.

Malignant Orbital Melanoma Associated with Oculodermal Melanocytosis

Originally received: October 21, 2016. Final revision: November 30, 2016. Accepted: December 12, 2016. Available online: February 10, 2017. Manuscript no. 2016-608. 1 Department of Optometry & Vision Sciences, The University of Melbourne, Melbourne, Australia. 2 Department of Computing and Information Systems, The University of Melbourne, Melbourne, Australia. 3 Visual Neuroscience Group, School of Psychology, University of Nottingham, Nottingham, United Kingdom. 4 Beijing Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Key Laboratory of Ophthalmology and Visual Sciences, Beijing, China. 5 Department of Ophthalmology, Medical Faculty Mannheim of the Ruprecht-Karls, University of Heidelberg, Heidelberg, Germany. Financial Disclosure(s): The author(s) have made the following disclosure(s): A.M.M.: Financial support e Heidelberg Engineering, Heidelberg, Germany; Haag-Streit Diagnostics, Koniz, Switzerland; CentreVue, Padova, Italy. J.B.J.: Advisory board e Mundipharma Co; Patents e Biocompatibles UK Ltd., Franham, United Kingdom; University of Heidelberg, Heidelberg, Germany. A.T.: Financial support e Heidelberg Engineering, Heidelberg, Germany; Haag-Streit AG; CentreVue SpA. Supported by the Australian Research Council Linkage Project (no.: 130100055; industry partner, Heidelberg Engineering, GmBH, Heidelberg, Germany). The funding organization had no role in the design or conduct of this research. Author Contributions: Conception and design: McKendrick, Turpin Analysis and interpretation: McKendrick, Denniss, Turpin Data collection: McKendrick, Wang, Jonas, Turpin Obtained funding: none Overall responsibility: McKendrick, Denniss, Wang, Jonas, Turpin