Management and prognosis of Merkel cell carcinoma of the eyelid.

OBJECTIVE: To evaluate the clinical presentation, treatment, and long-term follow-up of eyelid Merkel cell carcinoma. DESIGN: Retrospective noncomparative interventional case series. PARTICIPANTS: Fourteen patients with primary eyelid Merkel cell carcinoma. METHODS: Cases of Merkel cell carcinoma for which long-term follow-up was available were solicited from members of the American Society of Ophthalmic Plastic and Reconstructive Surgery through an on-line e-mail/news group. MAIN OUTCOME MEASURES: Follow-up period, treatment history, presence and type of recurrence, and mortality. RESULTS: Average follow-up was 33.4 months. Of the 14 cases identified, only 2 patients (14%) received prophylactic therapy beyond wide surgical excision. Three patients (21%) had recurrences, none of whom initially received prophylactic therapy (i.e., radiation therapy, lymph node dissection, and/or chemotherapy) beyond wide surgical excision. One patient (7%) died of metastatic Merkel cell carcinoma. CONCLUSIONS: Merkel cell carcinoma is a rare skin malignancy that occasionally affects the eyelid, with the potential for regional and distant metastasis. Consideration should be given to the use of prophylactic adjunctive therapies beyond wide surgical excision while simultaneously considering the morbidity of these therapies.

Benign approach to a malignant orbital tumor: metastatic renal cell carcinoma.

A well-circumscribed, pseudoencapsulated benign-appearing lesion in the retrobulbar orbit of a 50-year-old man was found to be a metastatic renal cell carcinoma. The patient had a nephrectomy for renal cell carcinoma 15 years before, and had developed four other isolated metastases over the preceding 8 years. He had a further distant metastasis 1 year later, but is living and well 4 years after excision of the orbital tumor. Metastatic renal cell carcinoma of the orbit may have very benign characteristics, both clinically and radiographically, and can often be managed effectively by local excision with prolonged survival.

Effects of eyelid protractor excision for the treatment of benign essential blepharospasm.

Data were analyzed from 37 patients with essential blepharospasm who had undergone upper eyelid protractor excision with brow suspension, and who had a median follow-up of 40 months (range, 12 to 83.5 months) after surgery. Of the 37 patients, 15 (40.5%) had sufficient relief of their spasms at their last follow-up; 22 patients (59.5%) had functionally significant recurrent or residual spasms after upper eyelid surgery. Of these 22 patients, 12 (55%) had recurrences more than six months after surgery, and four patients (18%) had recurrences more than one year after surgery. Patients with Meige syndrome were no more likely to experience recurrent spasms after upper eyelid surgery than patients who lacked a lower facial spasm component. Recurrence of symptoms was primarily the result of lower eyelid spasms in 11 patients (50%), upper eyelid spasms in one patient (5%), and all eyelids in six patients (27%). The location of recurrent spasms could not be ascertained in four patients (18%), with whom we had only telephone contact. Seven of 12 patients (58%) undergoing secondary procedures had relief from their spasms at the end of this study. Excluding those patients who chose to decline further surgery, 22 of 27 patients (81.5%) received satisfactory relief of symptoms after upper eyelid and, if needed, lower eyelid and repeat upper eyelid protractor excision. These long-term results are comparable to those reported previously for differential section of the facial nerve. Because the side effects of eyelid protractor excision are generally more acceptable, we recommend that when surgery is determined to be necessary for essential blepharospasm, upper eyelid protractor excision is the preferred surgical approach.

Efficacy of the primary dye test.

A primary dye test was performed on the right lacrimal system of 25 normal subjects by three physicians of differing levels of experience in performing the test. The frequency of positive primary dye tests for each of the three examiners was higher than previously reported. The most experienced examiner had the highest percentage (100%) of positive primary dye tests, and the least experienced examiner had the lowest percentage (80%) of positive tests. However, variation between the most experienced examiner and the other two was due, at least in part, to a difference in fluorescein instillation. Therefore, the effect of experience could not be ascertained. The most experienced examiner used four moistened fluorescein strips medially and each used a 10-minute interval after dye instillation before looking for evidence of dye passage through the nasolacrimal duct. Twenty-one (84%) of the subjects had a positive primary dye test on initial blowing of the nose. This indicates that even inexperienced examiners can expect to detect a high percentage of functioning lacrimal drainage system with the method of the experienced examiner.

Malakoplakia of the eyelid. Clinical, histopathologic, and ultrastructural characteristics.

A 56-year-old man had a slowly growing, elevated mass excised over the right medial canthus of the right eye. Results of histopathologic examination disclosed a dermal infiltrate of histiocytes ("von Hansemann histiocytes") containing the characteristic laminated, basophilic inclusions (Michaelis-Gutmann [M-G] bodies). Results of histochemical studies showed that the M-G bodies stained positively for calcium, iron, and mucopolysaccharides. Results of electron microscopic studies showed that the histiocytes contained numerous membrane-bound lysosomal bodies of variable size and density. Some histiocytes demonstrated coccobacilli, some of which were degenerated and incorporated into large phagolysosomes. The latter provided the nidus for the deposition of inorganic elements (calcium and iron) and mucopolysaccharides to form the M-G bodies which displayed concentric calcific rings with radiating spicules of hydroxyapatite crystals. The pathogenesis of malakoplakia is discussed. The case reported herein represents the third one involving the ocular adnexa. Only ten cases of cutaneous malakoplakia have been reported previously.

Ocular and adnexal complications of unilateral orbital advancement for plagiocephaly.

Strabismus, ptosis, lateral canthal dystopia, nasolacrimal obstruction, and cranial nerve palsy were noted preoperatively in 32%, 21%, 14%, 12%, and 9% of 34 patients, respectively, undergoing ophthalmologic evaluation prior to unilateral orbital advancement for plagiocephaly. Thirty-two percent of the patients had normal preoperative ocular and adnexal examination results. Ptosis, strabismus, and amblyopia were frequently acquired postoperative abnormalities in 29%, 18%, and 18% of the patients, respectively. Forty-four percent of the patients had no new abnormalities following craniofacial surgery.

Intralesional triamcinolone for cutaneous palpebral sarcoidosis.

Cosmetically disfiguring bilateral palpebral sarcoid dermatitis in a 32-year-old woman was treated with intradermal triamcinolone. There was rapid and complete resolution of lesions and there was no evidence of recurrence or hypopigmentation at a one-year follow-up examination. Thus, intradermal corticosteroid therapy can be useful for skin lesions in patients with sarcoidosis who do not require systemic therapy.