Second Primary Neoplasms in Patients With Sebaceous Carcinoma of the Eyelid: A Population-based Study, 2000 to 2016.

PURPOSE: To investigate the risk of second primary neoplasms (SPNs) after primary sebaceous carcinoma of the eyelid (SCE). METHODS: Data on patients diagnosed with primary SCE as their first malignancy were extracted from the Surveillance, Epidemiology, and End Results database from 2000 to 2016. Observed-to-expected ratios of SPNs were calculated to estimate standardized incidence ratios (SIRs). Patients were compared with a reference population (RP) matched for age, gender, and race. RESULTS: Five hundred fifty-nine patients with primary SCE were identified, 16% of whom developed SPNs. SCE patients displayed a 61% increased risk of developing SPNs compared with the RP (p < 0.001). Overall, the risk of SPNs of the lungs (SIR = 1.82; p < 0.05), pancreas (SIR = 2.94; p < 0.05), salivary glands (SIR = 41.65; p < 0.001), and skin (SIR = 8.33; p < 0.05) was elevated. Only non-Hispanic Whites were at an increased risk (SIR = 1.51; p < 0.05). Patients 40-54 years old at the time of diagnosis were at the highest risk of developing SPNs compared with the RP (SIR = 3.15; p < 0.05). Women with SCE experienced an increased risk of breast cancer (SIR = 3.6; p < 0.05) and chronic lymphocytic leukemia (SIR = 8.8; p < 0.01). CONCLUSION: SCE patients are more likely to develop SPNs of the lungs, pancreas, salivary gland, and skin than the RP. Forty to fifty-four years old Caucasian patients are at the highest risk. Women are at an increased risk of developing breast malignancies and chronic lymphocytic leukemia. Clinicians should be cognizant of these risks when managing SCE patients.

Patency of the lacrimal drainage system in patients with a peripunctal tumour.

Purpose: To examine the patency or secondary obstruction of the lacrimal drainage system in patients with a peripunctal tumour.Methods: This retrospective, observational, and comparative study included 10 patients with a peripunctal tumour. Lacrimal probing and syringing in all patients and dacryoendoscopic examinations in 5 patients were performed to check for patency of the lacrimal drainage system. Tear meniscus height (TMH) was measured bilaterally in the upper and lower eyelids using anterior segment optical coherence tomography and compared in relation to the affected side using one-way ANOVA.Results: All patients did not complain of epiphora. Probing gave a hard stop and irrigation fluid passed into the nose. A patent punctum/canaliculus was also confirmed by dacryoendoscopy in all of the 5 patients. TMH was not significantly different among the sides (P = .900).Conclusions: This study shows patency of the lacrimal drainage system in patients with a peripunctal tumour and no significant difference in TMH among the sides, resulting in absence of epiphora in all patients.

Periocular infantile hemangiomas: Characteristics, ocular sequelae, and outcomes.

OBJECTIVES: To identify clinical factors associated with complications of periocular infantile hemangioma (IH) and monitor improvement in complication rates post-treatment. METHODS: Retrospective cohort study. Eighty-nine patients diagnosed with periocular IH at a pediatric dermatology clinic of a tertiary care center between 2001 and 2013 were included with parental approval. Parents were interviewed by telephone between July and September of 2015, then again in January 2018 to inquire about ophthalmologic follow-up. Electronic medical records were reviewed from January 2001 through January 2018. RESULTS: Sixty percent of patients demonstrated ocular sequelae, including astigmatism (33%), visual axis obstruction (29%), nasolacrimal duct obstruction (7%), ptosis (4%), amblyopia (3%), and strabismus (1%). Compared with superficial IH, deep and mixed IH had higher odds, 3.4 (P = 0.025) and 3.8 (P = 0.034), respectively, of developing ocular sequelae. All patients with astigmatism prior to involution of IH received systemic therapy, with a significant post-treatment decrease in the proportion of patients with astigmatism (40% to 18%, P = 0.027). Three-quarters of patients experienced complete IH involution by time of enrollment in kindergarten. Fifty-one (57.3%) patients received formal ophthalmologic evaluation confirmed through chart review or phone interview, with average follow-up duration of 51.2 months (range: 1.9, 99.3). CONCLUSION: Deep and mixed IH were more likely to demonstrate ocular complications than superficial IH. Rate of astigmatism decreased with systemic therapy. Our study suggests that patients with periocular IH have a lower rate of amblyopia now compared with the prepropranolol era and emphasizes the importance of early treatment of periocular IH to prevent permanent visual sequelae.

Angiosarcoma of the Eyelid With Superimposed Enterobacter Infection.

Angiosarcoma is a rare, aggressive, malignant endothelial neoplasm with a variable clinical presentation. The authors describe a case of angiosarcoma involving the eyelid that was complicated by a superimposed Enterobacter infection. Following positive cultures for E. aerogenes and multiple biopsies suspicious but not definitive for angiosarcoma, a final biopsy was consistent with angiosarcoma.

Choroidal ganglioneuroma in a patient with orbitopalpebral neurofibromatosis.

Ganglioneuroma is a rare, benign tumor originating from the sympathetic ganglion cells. Choroidal ganglioneuroma in association with neurofibromatosis type 1 (NF-1) has been reported in a few cases. This study describes a 21-year-old woman with orbitopalpebral NF, who underwent evisceration for pseudophakic bullous keratopathy, intractable glaucoma, and absolute painful eyes and who was found to have choroidal ganglioneuroma and retrobulbar plexiform neurofibroma on histologic examination. This case, together with the previously described cases, suggests that eyes with choroidal ganglioneuromas may have some common clinical and prognostic characteristics, although this condition cannot be diagnosed preoperatively. In patients with NF-1 who may undergo eye removal surgery, the possibility of choroidal tumors should be kept in mind.

Extensive facial and orbital infantile hemangiomas associated with high intraocular pressure.

High intraocular pressure is a rare ophthalmic condition associated with infantile hemangiomas that involves the orbit, eyelid, or both. Here, we describe a patient with extensive facial and orbital infantile hemangiomas associated with high intraocular pressure in the affected eye. The prompt management of this challenging condition is essential.

Levator-Muller's muscle recession with tarsorrhaphy: a technique for corneal protection in cases with cicatricial upper eyelid retraction.

AIMS: Cicatricial upper eyelid retraction with exposure keratopathy and impending corneal perforation requires prompt intervention. Standard procedures such as isolated levator recession, botulinum toxin, and lid weights will only induce a partial ptosis. Conventional tarsorrhaphy, though ideal to achieve complete closure, is likely to result in dehiscence in these cases. We describe a one-stage technique of levator and Muller's muscle recession combined with a tarsorrhaphy used in four patients with an impending corneal perforation due to cicatricial lagophthalmos. METHODS: This is an interventional, non-comparative retrospective case series of four patients who had undergone tarsorrhaphy in combination with levator recession. RESULTS: In all four cases, it was not possible to mechanically close the eyelids preoperatively due to cicatricial lid retraction involving the middle lamella. The aetiology for lagophthalmos was varied: (Case 1) bilateral sclerosing metastatic breast cancer involving the lids; (Case 2) severe anterior and middle lamella shortening due to actinic changes; (Case 3) middle and posterior lamella shortening due to glaucoma treatment and multiple surgery (Case 4) due to traumatic facial scarring and seventh nerve palsy. In all cases, the corneal thinning and epithelial defects resolved completely following surgery. In one case, we were able to partially reopen the tarsorrhaphy for further corneal surgery. DISCUSSION: We describe a safe, effective and reversible surgical procedure for managing cases with cicatricial upper eyelid retraction, which would otherwise lead to serious corneal complications.

Uncorrectable ptosis: primary cutaneous signet-ring cell carcinoma.

Primary cutaneous signet-ring cell carcinoma (PCSRCC) is a rare but aggressive tumor. Our case highlights a 60-year-old man who presented with eyelid ptosis, for which he underwent multiple surgical procedures over a 3-year period prior to referral to our clinic. These procedures were complicated by scarring, delayed healing, and poor cosmetic outcome. In addition, the patient was noted to develop progressive enophthalmos. These concerning signs led to a CT scan and subsequent eyelid biopsy, which revealed a diagnosis of PCSRCC. Further management has involved an MRI and orbitotomy with biopsy revealing widespread extension of the carcinoma. Exenteration was performed to reduce the likelihood of metastasis. There are few documented case reports of PCSRCC of the eyelid in the literature. Of the 33 published cases of PCSRCC, 27 cases involve the eyelids and the other 6 cases involve the axilla. The unique clinical features of this case will be discussed, in particular the presentation as ptosis, an otherwise commonplace complaint in the oculoplastics clinic. The surgical course and histopathologic findings will be presented. The literature regarding PCSRCC will be reviewed including demographics, management, and prognosis. Although rare, PCSRCC follows an aggressive course with characteristically delayed diagnosis. Early identification and treatment likely offer a better prognosis. Thus, description of the clinical presentation of this rare tumor may aid in recognition and earlier treatment.

HPV-Associated Squamous Cell Carcinoma of the Eyelid: Diagnostic Utility of p16 Immunohistochemistry and mRNA In Situ Hybridization.

BACKGROUND: High-risk (HR) Human papillomavirus (HPV) has been implicated in pathogenesis of squamous cell carcinomas (SCC) at several sites with mucocutaneous junctions, including the head and neck. SCC is the second most common eyelid malignancy. However, its association with transcriptionally active HR-HPV has not been adequately studied. METHODS: Two index cases of eyelid HPV-associated SCC are described in detail. A retrospective cohort of eyelid SCC was examined for p16 immunoexpression. Cases demonstrating p16 positivity or equivocal staining were subjected to high-risk HPV mRNA in situ hybridization (ISH). Quantitative real-time PCR (qPCR) was performed in mRNA ISH-positive cases for HPV genotyping. RESULTS: The two index patients were older adult females, with upper eyelid tumours. On histology, both tumours were non-keratinizing SCC with trabecular and nested architecture reminiscent of oropharyngeal HPV-associated non-keratinizing SCC, prompting p16 immunohistochemistry, which was positive. HR-HPV mRNA ISH was positive, and qPCR detected HPV16 in both cases. Three of 20 (15%) archival cases showed p16 immunopositivity and two (10%) showed equivocal staining. However, mRNA ISH was negative. All cases showing p16 immunostaining and lacking HR-HPV were keratinizing SCCs. Thus, 9% of all eyelid SCC examined demonstrated HR-HPV. CONCLUSION: The prevalence of HR-HPV in eyelid SCC is low in Indian patients. HPV-associated SCC may mimic commoner eyelid carcinomas as it lacks overt keratinization. In basaloid-appearing eyelid carcinomas, p16 immunopositivity should be followed by reflex HR-HPV mRNA ISH, as p16 immunohistochemistry alone has low specificity. The prognostic role, if any, of HPV association needs further evaluation.

Current concepts in the management of periocular infantile (capillary) hemangioma.

PURPOSE OF REVIEW: To review and evaluate the medical literature on new treatments for periocular infantile (capillary) hemangioma. Recent studies have shown a promising new therapy for infantile hemangioma using nonselective ß-blockers, including oral propranolol and topical timolol. RECENT FINDINGS: Conventional treatments for infantile hemangioma include the use of corticosteroids, laser, surgery, and immunomodulator therapy. Recently, systemic and topical ß-blockers have been used to successfully treat infantile hemangioma. The drugs' mechanism of action remains uncertain, but plausible theories include vasoconstriction, modulation of pro-survival signal transduction pathways, and endothelial cell apoptosis. Whereas no life-threatening adverse events from ß-blocker treatment have been described, there have been reports of bradycardia, hypotension, bronchospasm, hypoglycemia, and electrolyte disturbances resulting from systemic use of propranolol to treat infantile hemangioma. Sleep and gastrointestinal disturbances have also been frequently reported. Topical timolol application for localized, superficial tumors may confer similar efficacy as oral propranolol while reducing systemic effects. SUMMARY: Despite the recent explosion of interest surrounding this novel treatment, current treatment and protocol-monitoring recommendations are based largely on the experience of individual centers. Several randomized controlled studies are currently underway, the results of which will guide future standard-of-care treatment for infantile hemangioma.

Pathogenesis of involutional ectropion and entropion: the involvement of matrix metalloproteinases in elastic fiber degradation.

PURPOSE: To determine the elastic fiber content and ultrastructure as well as the expression of elastin-degrading enzymes in biopsy specimens from patients with involutional ectropion and entropion. MATERIALS AND METHODS: Twenty consecutive patients with involutional ectropion (group 1) and twenty consecutive patients with entropion (group 2) were matched with twenty control patients (basal cell carcinoma) regarding age and gender. Full-thickness eyelid resections performed in study and control patients were examined by light and transmission electron microscopy, computer-assisted measurements, and immunohistochemistry using antibodies against matrix metalloproteinase (MMP)-2, MMP- 7, and MMP-9. The Kruskal-Wallis test and the Pearson chi-square test were performed. RESULTS: Histopathologic analysis of the surgical specimens from patients with involutional ectropion and entropion showed a significant loss of elastic fibers in the eyelid skin, the pretarsal orbicularis oculi muscle, the perimeibomian tarsal stroma, and the intermeibomian tarsal stroma (P < 0.001). Residual elastic fibers revealed an abnormal ultrastructure. Immunohistochemistry demonstrated a significant overexpression of MMP- 2, MMP-7, and MMP-9 in the eyelid skin, the pretarsal orbicularis oculi muscle, the perimeibomian tarsal stroma, the intermeibomian tarsal stroma, and the conjunctiva in groups 1 and 2 compared to controls (P < 0.001). CONCLUSIONS: The present findings indicate that upregulation of elastolytic enzymes contributes to elastic fibre degradation in patients with involutional ectropion and entropion.

Management of facial disfigurement in orbitotemporal neurofibromatosis.

A 19-year-old woman with type I neurofibromatosis requested reconstruction of her severe facial disfigurement caused by the plexiform neurofibroma of the right upper eyelid. Previously, she had glaucoma surgery for buphthalmus and enucleation with dermis fat transplantation. She was unable to wear prosthesis in the last year. Transverse palpebral resection of the lesion, lateral canthal reconstruction, and frontal sling ptosis surgery were performed at the same session. Postoperatively, the cosmetic appearance of the patient was markedly improved. No complication or progression was observed during 2 years follow-up. We believe that an individual-based surgical plan may give acceptable results in these challenging cases.

Sebaceous carcinoma of upper eyelid.

A middle aged lady was surgically treated repeatedly elsewhere for growth on right upper lid before presentation to this department. On examination she was found to have nodulo-ulcerative non-tender growth, about 40 x 20 mm in size involving the lateral three-fourth of the lid. There was associated mild conjunctivitis and palpable pre-auricular lymph node. Lid growth was excised followed by lid reconstruction. Pre-auricular lymph node was also removed. Histopathology report of the tissue revealed it to be the palpebral sebaceous carcinoma, while lymph node showed reactive hyperplasia.

Eyelid edema as a first sign of lymphoma.

Chronic eyelid edema may be a symptom of different disease. The most common are autoimmune diseases such as orbital pseudotumor, vasculitis, sarcoidosis, or impaired vascular or lymphatic drainage. Rarely has it been reported as the sole manifestation of the lymphoma. Eyelid lymphoma is a special clinical entity in the spectrum of hematological malignancies. Here we present our clinical experience with eyelids lymphomas. First case is a 76-year-old female patient with bilateral edema of upper eyelid non-responding to anti-inflammatory therapy. Histological examination diagnosed mantle cells lymphoma. In the second case, 58-year-old patient was diagnosed with solitary unilateral tumor of the lower eyelid, where primary biopsy was ordered and diagnosis of MALT lymphoma was established after histological examination. In both cases, it was not solitary eyelid tumor, but systemic disease with multiple lymphadenopathy and bone marrow infiltration were found in follow-up examinations. Subsequently, patients care was given to the hemato-oncologist.

Impact of the type of anterior lamellar reconstruction on the success of modified Hughes procedure.

PURPOSE: To determine the long-term functional and cosmetic outcomes in patients who underwent modified Hughes procedure with different types of anterior lamellar reconstruction for lower eyelid defects. METHODS: This study included 58 patients who had undergone a modified Hughes flap for reconstruction of lower eyelids after tumor excision within a 10-year period. Data regarding patient demographics, size of eyelid defect, tumor pathology, surgical techniques, functional and cosmetic outcomes, and complications were recorded. Postoperative complications were evaluated according to the type of anterior lamella reconstruction (i.e., advancement flap or free skin graft). Multivariate logistic regression analysis was performed to identify risk factors affecting the success of the procedure. RESULTS: The average size of the lower eyelid defect was 22 ± 6.3 mm (range: 11-30 mm). The anterior lamella was reconstructed with advancement flaps and full-thickness skin grafts in 36 (58.6%) and 24 (41.4%) patients, respectively. Mean follow-up time was 23.6 ± 11.9 months. Postoperative complications included trichiasis (three patients; 5.2%), ectropion (two patients; 3.0%), flap necrosis (one patient; 1.7%), flap dehiscence (one patient; 1.7%), infection (one patient; 1.7%), and eyelid margin erythema (one patient; 1.7%). The rates of complication and secondary surgery were similar among the different types of anterior lamellar reconstruction (p=768 and p=0.139, respectively). Success of the modified Hughes procedure was not significantly affected by any of the identified risk factors (p>0.05). Functional and cosmetic outcomes were 96.6% and 94.8%, respectively. CONCLUSION: Modified Hughes procedure is a safe and effective option for the reconstruction of small and large defects of the lower eyelid, regardless of the type of anterior lamella reconstruction (i.e., advancement flap or skin graft).

Intracranial extension of meibomian gland carcinoma with pagetoid changes.

A 41-year-old man presented with exophthalmos and loss of visual acuity. Neuroradiological studies showed a large mass extending from the intraorbital region to the frontal lobe. In addition, it also involved the middle cranial and infratemporal fossae with accompanying skull destruction. The tumor was almost totally removed. The histological diagnosis was sebaceous carcinoma with pagetoid changes. Despite surgery and local irradiation, intracranial metastases were recognized one year later, and the patient underwent total tumor removal and whole-brain irradiation. Although multiple lung metastases were detected one year after the second operation, three years post-surgery he remains free of intracranial tumor recurrence. Sebaceous carcinoma of meibomian gland origin with pagetoid changes is a distinct, highly aggressive clinical entity. Early diagnosis and appropriate treatment are essential to improve the prognosis of patients with meibomian gland carcinoma with intracranial extension.