Allogenic dehydrated human pericardium patch graft (Tutoplast): A novel use for reconstruction in orbital implant exposure.

PURPOSE: Exposure of orbital implant post enucleation or evisceration remains one of the common complications irrespective of the type of implant used. Dermis fat graft and temporalis fascia have been used to repair the implant exposure in anophthalmic sockets. Tutoplast® pericardium, gamma sterilised dehydrated human pericardium, has been used as a scleral patch graft for glaucoma drainage device exposure and scleral thinning post squint surgery. We report the novel use of Tutoplast® patch graft to repair orbital implant exposure in this case series. METHODS: The case notes of three patients who received Tutoplast® pericardium patch graft to repair implant exposure were reviewed. Data regarding presenting symptoms, implant type, time to exposure from primary surgery, post-operative complications and length of follow up post Tutoplast® pericardium patch graft were collected. RESULTS: Three patients presented with implant exposure following evisceration. The presenting symptoms were recurrent infection, discharging socket and discomfort in all three patients. One patient had a Medpore implant, two had silicone implants. Total follow-up ranged from 9 to 22 months. In all three cases, there was relief from symptoms and the graft had incorporated fully into the surrounding orbital tissue with no recurrent exposure noted during the follow-up period. CONCLUSION: Tutoplast® pericardium has demonstrated a good safety profile and is a suitable material to use as a patch graft for orbital implant exposure.

Periocular cutaneous sarcoid: case series and review of the literature.

OBJECTIVES: To illustrate the varying clinical presentations of cutaneous sarcoidosis affecting the periocular region, which may masquerade as other clinical entities such as basal cell carcinoma or seborrheic dermatitis. Furthermore, the authors present an unusual observation of lupus pernio involving the adnexal region with the rare presence of perineural granulomas on histology following incisional biopsy. METHODS: We report a consecutive series of four cases with lesions involving the eyelids with varying clinical appearances. All four patients presented to our adnexal service undergoing incisional diagnostic biopsy. Histology following biopsy subsequently resulted in further investigation and management of both local cutaneous lesions and systemic sarcoidosis. RESULTS: Three of our four cases had evidence of pulmonary involvement on chest X-ray. Over an 18-month period, one of two patients responded to intralesional triamcinolone and subsequently to oral methotrexate (15 mg/week). Two patients were observed with their periocular lesions remaining stable without therapy. CONCLUSIONS: All four patients presented to the adnexal service with lesions of varying morphology and were diagnosed with sarcoidosis following incisional biopsy highlighting the vital role of oculoplastic surgeons in diagnosing this multisystem inflammatory disease. We describe our experience of intralesional triamcinolone, oral methotrexate and watchful observation in the management of such lesions.

Squamous cell carcinoma in the anophthalmic socket: a series of four cases with HPV-16 profiling.

PURPOSE: To present the clinical and histological features of squamous cell carcinoma (SCC) in the anophthalmic socket in four adult patients, and to determine the presence of human papillomavirus infection (HPV). METHODS: Retrospective case series of four adult patients with SCC of the anophthalmic socket. P16 immunohistochemistry and HPV testing was carried out in all cases. The authors report clinical findings, histopathology, management and outcomes for all four patients with conjunctival SCC. Previously reported cases of conjunctival SCC in anophthalmic sockets were reviewed. RESULTS: Four adult patients presented with eyelid lumps, discharge or change in prosthesis fit. Common examination findings included papillomatous changes, eyelid masses and epithelial changes. Three out of the four cases (75%) were positive for p16 by immunohistochemistry and the same cases positive for HPV-16 DNA. All patients received cryotherapy, topical or intralesional chemotherapy. Two patients proceeded to exenteration for control of invasive disease. CONCLUSIONS: To the authors' knowledge, this is the largest series of SCC in the anophthalmic socket with comprehensive annotation of HPV status. Although socket conjunctiva is protected from environmental radiation, there is still a risk of neoplastic transformation in this tissue, thus patient education and regular checking of sockets by ophthalmologists should be undertaken as a preventative measure. The potential role of HPV in these tumours warrants further investigation.

Nonophthalmic symptoms secondary to ocular torticollis from severe blepharoptosis: an underappreciated but treatable condition.

The purpose of this case report is to describe reversible systemic symptoms associated with ocular torticollis due to blepharoptosis. A retrospective chart review identified patients with symptomatic ocular torticollis due to bilateral congenital or acquired blepharoptosis who underwent surgical correction (levator resection or frontalis sling). Preoperative and postoperative assessment of systemic symptoms in adults and developmental milestones are reported. The authors present 4 cases, 1 adult and 3 young children, all with severe bilateral upper eyelid ptosis. In the adult patient, it caused debilitating back pain that required orthopedic evaluation but was completely relieved with ptosis repair surgery. In the toddlers, subjective developmental motor delay was rapidly reversed following ptosis repair surgery. The authors suggest that primary care physicians, and spine and neuromuscular specialists, consider the possibility of ocular torticollis due to eyelid ptosis in the evaluation of patients with unexplained back and/or neck pain or with gross motor developmental delay. The authors propose that symptomatic ocular torticollis is an indication for ptosis repair.

Xanthogranulomatous variant of immunoglobulin G4 sclerosing disease presenting as ptosis, proptosis and eyelid skin plaques.

A 70-year-old male was referred to the oculoplastic clinic with left-sided ptosis and floppy eyelids. During follow-up, bilateral upper lid xanthelasma developed with worsening ptosis and proptosis, which was worse on the left side. A left orbital biopsy showed xanthogranulomatous inflammation of the orbit. The patient was treated with a variety of immune modulator regimes but due to a variety of side-effects, treatment was discontinued. The left orbit was surgically debulked twice and histology revealed xanthogranulomatous inflammation, with the additional features of sclerosis, lymphoid aggregates and a prominent population of plasma cells. Around 80% of the plasma cells expressed immunoglobulin G4 (IgG4). This case report reveals an association between xanthogranulomatous inflammation of the orbit and a prominent population of IgG4-positive plasma cells. We propose that the overall disease is a novel variant of IgG4 sclerosing disease of the orbit and suggest that cases of histologically proven xanthogranulomatous inflammation should be stained for IgG4 if there is an accompanying plasma cell population.

Thyroid-associated orbitopathy: Current insights into the pathophysiology, immunology and management.

Half the patients suffering from Graves' disease develop thyroid-associated orbitopathy (TAO). The severity of TAO varies considerably with a mild form characterized by dry eyes and discomfort to the severe form with sight-threatening exposure keratopathy and optic neuropathy. The pathogenesis and immunologic mechanisms underlying Graves' disease and TAO is unknown, however, advances toward this understanding have indicated a prominent role of orbital fibroblasts, T cells and B cells. These advances have led to novel strategies for clinical treatment using immunomodulatory modalities. Initial results included use of infliximab and etanercept (anti-TNF agents), but currently there is an increasing interest in anti-B cell (Rituximab) therapy. Rituximab has shown promising results in progressive, sight-threatening TAO. It has also shown encouraging results in halting or slowing the disease process in patients unresponsive to corticosteroids. The primary advantage of these immunomodulatory agents is based upon targeting the molecular mediators of the disease and avoiding the potential side effects of non-specific therapies.