Merkel Cell Carcinoma Metastases To Caruncle With Orbital Extension: Report and Literature Review.

Merkel cell carcinoma (MCC) is an uncommon and aggressive skin cancer of neuroendocrine origin. The tumor usually presents with a locoregional spread and most frequently metastasizes to the skin, liver, bone, lung, and brain. Despite the orbit being a relatively common site of metastases, it has rarely been reported in patients with MCC. The authors present a case of biopsy-proven orbital metastatic MCC in an 86-year-old male who presented with a rapidly enlarging right caruncle/subconjunctival mass with orbital extension and a history of forearm MCC excision 3 years prior. There are only 3 reported cases of distant metastatic MCC to the orbit, all presenting as a mass originating from extraocular muscles; and no cases of caruncle involvement.

Pilomatrixoma of the Periorbital Region: A Retrospective Review.

PURPOSE: Pilomatrixoma is a benign cutaneous adnexal tumor that differentiates toward hair follicle cells. It infrequently occurs in the periorbital region. In the periorbital area, it is often located in the upper eyelid and eyebrow region. Periorbital pilomatrixoma is often misdiagnosed clinically and is less common than other benign skin tumors. In this retrospective review, we evaluate the demographic, clinical, and histopathological data of patients with periorbital pilomatrixoma, treated from 2010 to 2023. METHODS: A retrospective analysis of periorbital pilomatrixoma cases treated during a period of 13 years was performed. Deidentified data in relation to the age at surgical excision, gender, location, size, histopathological features, treatment, and recurrence were collected. All histological slides were reviewed by an attending histopathologist. RESULTS: A total of 77 cases of periorbital pilomatrixoma were diagnosed by histopathological examination and treated from 2010 to 2023. A slight female preponderance (56%) was noted, and the majority of cases (52%) were presented in the first 2 decades of life. The left periorbital region was involved in 45.5% of cases, and 54.5% in the right. The upper lid was the most affected site. Two cases of recurrence were reported. No malignant transformation was noted. CONCLUSIONS: Pilomatrixoma is an uncommon benign periorbital tumor, commonly misdiagnosed clinically. Ophthalmologists and surgeons should suspect pilomatrixoma in the periorbital region, especially in children or young adults. Complete surgical excision is curative, and recurrence is rare.

Paediatric Orbital Juvenile Xanthogranuloma: A Case Series and Review of the Literature.

PURPOSE: Juvenile xanthogranuloma (JXG) is a subtype of histiocytosis characterised histologically by foamy non-Langerhan cells with Touton giant cells. It typically manifests as a single self-limiting cutaneous nodule in the paediatric population. Orbital JXG is extremely rare, and its clinical course and management are not well understood or defined. Herein we present 3 cases of orbital JXG and provide a detailed literature review. METHODS: Review of 3 cases with orbital JXG and literature review of all published cases. RESULTS: Three presented cases demonstrate the heterogeneous clinical course of orbital JXG. Although centred around the use of steroids, there is neither robust evidence nor consensus on its management. The wider JXG literature is currently concentrated around the classification of JXG with respect to histiocytosis, especially the exclusion of extracutaneous JXG as separate diseases. This separation is based on clinical, histopathological, and molecular findings. It is unclear where orbital JXG best fits in this emerging classification of JXG. CONCLUSION: Our review of the cases and literature on orbital JXG show that it may manifest with variable clinical course and its molecular pathogenic mechanism may be different to that of the cutaneous JXG.

Corneal Neurotization for Neurotrophic Keratopathy: A Multicentre Experience.

PURPOSE: Corneal neurotization is an emerging technique that offers potential for visual rehabilitation in neurotrophic keratopathy. This study reports on a multicenter experience and outcomes for both direct and indirect methods of corneal neurotization. METHODS: Retrospective case series. Sixteen patients with neurotrophic keratopathy who underwent corneal neurotization across 5 centers in Australia and Israel were identified for inclusion. Corneal neurotization was performed via direct neurotization from the ipsilateral or contralateral supraorbital/supratrochlear nerve or by the use of an interpositional sural nerve graft. Change in corneal sensitivity (measured in millimeters by the Cochet-Bonnet aesthesiometer), visual acuity, and corneal health. RESULTS: Over a mean follow-up period of 31.3 months (range: 3 months-8 years), mean corneal sensitivity improved from 3.6 mm (range: 0-25 mm) to 25.3 mm (range: 0-57 mm). Visual acuity improved on average from 20/380 to 20/260. Twelve of 16 patients (75.0%) improved in at least 2 out of the 3 main outcome measures. Nine patients (56.3%) showed an improvement in visual acuity; 13 (81.3%) showed an improvement in average corneal sensitivity; and 11 (68.8%) showed an improvement in corneal health. There were no intraoperative or postoperative complications. CONCLUSIONS: Corneal neurotization is an emerging surgical treatment option for the management of neurotrophic keratopathy. With appropriate case selection, outcomes are favorable and complication rates are low, for a condition that is otherwise challenging to manage. Patients with severe neurotrophic keratopathy should be considered for this surgical treatment option.

Orbital necrobiotic xanthogranuloma with necrotizing scleritis and absent cutaneous manifestations.

Necrobiotic xanthogranuloma (NXG) is a rare systemic disease, that commonly manifests with orbital and ocular adnexal involvement, presenting with periocular yellow papules and plaques that may ulcerate. Periorbital skin lesions are a hallmark of the disease, and in their absence the diagnosis may be delayed, preventing prompt systemic evaluation and appropriate treatment of this condition. We report a unique case of a 58-year-old female patient with NXG that presented with severe bilateral necrotizing scleritis, left orbital mass and no cutaneous manifestations of NXG. This case highlights the importance of considering NXG, despite absence of skin lesions, when other ophthalmological manifestations are present. Recognition of NXG is crucial due to the high lifelong risk of developing hematological malignancy.

Orbital apex syndrome in herpes zoster ophthalmicus clinical features, treatment and outcomes: a case series and literature review.

Orbital Apex Syndrome (OAS) complicating Herpes Zoster Ophthalmicus (HZO) is associated with significant visual impairment. We present four patients with HZO OAS, to highlight clinical features and outcomes in order to promote earlier recognition and management of this potentially sight-threatening complication. CT and MRI imaging findings included expansion and enhancement of extraocular muscles and intraconal fat and involvement of the orbital apex and cavernous sinus. All patients received systemic steroid and antiviral therapy, but a standardised dosage and duration of treatment remains to be defined. Final visual acuity and extraocular motility outcomes were variable.

Giant molluscum contagiosum with preseptal cellulitis.

Molluscum contagiosum (MC) is a benign cutaneous viral infection commonly affecting children, sexually active adults, immunocompromised individuals, and patients on immunosuppressive therapy. Giant periorbital MC lesions with preseptal cellulitis are rare. We present 2 such pediatric cases. Case 1 was a 9-month-old boy with an enlarging left upper eyelid mass and preseptal cellulitis. MC was confirmed on histopathology, and surgical excision was curative. Case 2 was an 18-month-old girl with a right lower eyelid ulcerating mass and preseptal cellulitis. She was managed with antibiotics and surgical excision.