Primary orbital melanoma: a case series and literature review.

Primary orbital melanoma (POM) is a very rare condition. We report further four cases and review all previously reported cases. We present a multicentre retrospective review of patients with POM. Clinical, radiological, surgical, histological, and follow-up data is presented. Four patients with POM were identified between 2000 and 2013. All presented with proptosis and diplopia without reduced vision. Two had known pre-existing blue cell naevi. All were stage T1N0M0. All underwent exenteration with adjuvant radiotherapy. All are disease free at follow-up durations of 24-151 months. The present three cases and review of all cases in the literature suggest a higher likelihood of disease-free survival from primary exenteration (7/8 disease-free survival, 1/8 death from metastatic disease) than wide local excision (7/16 disease-free survival, 9 recurrence or metastasis of whom 4 died). Adjuvant radiotherapy may additionally improve outcomes.

Eyebrow reconstruction.

This is a systematic review of eyebrow reconstruction options, using the PubMed database, as well as dermatology and plastic surgery texts. Eyebrow reconstruction options in various clinical scenarios (small, large, medial, lateral, and total defects) are presented. The goals of eyebrow reconstruction are to provide structural, functional, and aesthetic restoration. A good understanding of various eyebrow reconstruction techniques is essential for plastic, dermatologic, and oculoplastic surgeons.

T-Cell Large Granular Lymphocyte Leukemia in the Lower Eyelid.

The authors describe a case of T-cell large granular lymphocytic leukemia nodular lesion of the eyelid. To their knowledge, this has not been reported previously to occur in the eyelids. They have also reviewed previous literature reports on similar skin lesions in areas elsewhere.

Malignant hair follicle tumors of the periorbital region: A review of literature and suggestion of a management guideline.

Malignant hair follicle tumors are rare skin adnexal malignancies that have a predilection for the head and neck region. They can be categorized into a number of different subtypes. Histologically, they are distinct from their benign counterpart. To the best of our knowledge, there is no extensive review of these malignancies, especially in the periorbital region. We aim to provide a literature review and a guideline for management of these malignant tumors in the periorbital region. Database from Medline, PubMed, Embase, and Google Scholar were consulted. A total of 16 cases from the literature on hair follicle malignancies in the periorbital region were included in this review. The clinical presentations, diagnostic patterns, investigations used, and best management approach of these tumors are discussed. The American Joint Committee on Cancer (AJCC) 7(th) edition carcinoma of the eyelid staging system was used to describe their behaviors. We recommend wide excision surgery and a close follow-up for these tumors. Tumors presenting with a late stage require work-up for distant metastasis and consideration for exenteration procedures. The role of radiotherapy and chemotherapy in this context is still uncertain.

Management of periorbital basal cell carcinoma with orbital invasion.

Basal cell carcinoma (BCC) is the most common eyelid malignancy; however, orbital invasion by periocular BCC is rare, and management remains challenging. Established risk factors for orbital invasion by BCC include male gender, advanced age, medial canthal location, previous recurrences, large tumor size, aggressive histologic subtype and perineural invasion. Management requires a multidisciplinary approach with orbital exenteration remaining the treatment of choice. Globe-sparing treatment may be appropriate in selected patients and radiotherapy and chemotherapy are often used as adjuvant therapies for advanced or inoperable cases, although the evidence remains limited. We aim to summarize the presentation and treatment of BCC with orbital invasion to better guide the management of this complex condition.

Unifocal Langerhans cell histiocytosis of the superior oblique muscle.

Orbital Langerhans cell histiocytosis (LCH) without bone involvement is rare. Isolated involvement of an extraocular muscle without bone change, to the best of the authors' knowledge, has not been previously reported. They describe a unique case of unifocal LCH of the superior oblique muscle with no bone involvement. A 16-year-old girl presented with a 2-week history of diplopia and headache. CT and MRI showed an enlarged right superior oblique muscle with no associated bone involvement. Biopsy with partial debulking revealed LCH and staging confirmed unifocal disease. The lesion underwent complete radiologic resolution following surgery. There was no recurrence after 16 months follow up.

Frequency of simultaneous nasal procedures in endoscopic dacryocystorhinostomy.

PURPOSE: To assess the frequency of simultaneous nasal procedures in powered endoscopic dacryocystorhinostomy performed by oculoplastic surgeons. DESIGN OF STUDY: Retrospective, multicenter study. METHODS: Demographic, clinical, and surgical data of consecutive endoscopic dacryocystorhinostomy cases at 3 oculoplastic centers, over periods of 6, 4, and 2.2 years, respectively, were reviewed. The rates of simultaneous nasal procedures (septoplasty, turbinectomy, and polypectomy) were studied. Complication rates in the patients who had simultaneous endonasal procedures were analyzed. RESULTS: Five hundred seventy-six cases (mean age: 63.2 years [16.2-94 years], women: 67.3%). Of the total cohort of patients, 14.1% required a simultaneous endonasal procedure during endoscopic dacryocystorhinostomy, 11.9% (range among surgeons: 5.2%-15%) required septoplasty, 1.5% required middle turbinate surgery, and 0.34% required polypectomy. Of the 81 patients with concomitant procedures, 1 had postoperative epistaxis, and 1 had an asymptomatic septal adhesion. The anatomical and functional success rates for the entire cohort were 95.6% and 87.8%, respectively. CONCLUSIONS: A significant proportion of patients undergoing endoscopic dacryocystorhinostomy may require concomitant endonasal procedures. Hence, endonasal lacrimal surgeons using techniques that aim to marsupialize the entire lacrimal sac may benefit from expertize in the management of concomitant nasal pathologic study.

LESCs: Lateralizing Eyelid Sleep Compression Study.

PURPOSE: To study the hypothesis that in normal patients, changes in eyelid elasticity may occur asymmetrically and in relation to the side on which the individual sleeps. DESIGN: Prospective, consecutive, single-center study within a large, tertiary-referral ophthalmology department within a university hospital. METHODS: This prospective study was carried out consecutively on 262 normal patients. The 3 inclusion criteria were 1) age≥55 years, 2) absence of facial nerve palsy, and 3) absence of eyelid trauma or surgery. Immediately before the ocular plastic surgeon assessed the patient, each patient was questioned in a separate consulting room by the attending orthoptist as to his or her customary side of sleeping. After detailed explanation, the "history-masked" ocular plastic surgeon then assessed the patient's upper eyelid laxity, the main outcome measurement. This was performed by asking the seated patient to look down and then gently grasping the upper eyelids close to the eyelid margin, just medial to the lateral commissure. The ocular plastic surgeon, with thumbs pronated, simultaneously distracted both upper eyelids superiorly, laterally, and anteriorly. The measured separation of the upper eyelid from the globe conjunctiva was obtained using calipers. Eyelid laxity grading was designated as grade 1: 0 to 1.9 mm; grade 2: 2.0 to 3.9 mm; grade 3: 4 to 9 mm; and grade 4: floppy. RESULTS: Two hundred sixty-two patients (58% females) who consecutively satisfied the selection criteria were evaluated, of which 70.22% (183/262) patients had significantly greater laxity of the upper eyelid that corresponded to the side on which they customarily slept. An unpaired t test used to compare the eyelid laxity between the sleeping side and nonsleeping side was statistically significant (p<0.001). CONCLUSION: Normal patients demonstrate a correlation between the side on which they historically or customarily sleep and the laxity of their ipsilateral upper eyelid.

Successful "medical" orbital decompression with adjunctive rituximab for severe visual loss in IgG4-related orbital inflammatory disease with orbital myositis.

IgG4-related disease (IgG4-RD) is an inflammatory condition of unknown etiology that can cause tumefactive lesions in a number of tissues and organs, including the orbit and ocular adnexa. Diagnostic criteria for IgG4-RD, including pathology and clinical features and pathology, have been recently proposed. This study presents the first case of unilateral acute visual loss secondary to IgG4-related orbital inflammatory disease with orbital myositis that was complicated by severe compressive optic neuropathy. After initial treatment with pulsed intravenous methylprednisolone, followed by rituximab and radiotherapy, there was a marked improvement in orbital inflammation and clinical and radiological improvement in the compressive optic neuropathy. After 9 months of follow up, the orbital inflammatory disease remained in remission.

Idiopathic inflammatory orbital myositis presenting with vision loss.

We present a first case of 58-year-old man with vision loss in a biopsy-proven idiopathic inflammatory orbital tendon sparing myositis. Tests for thyroid autoantibodies were negative at the initial presentation and at 10-month follow-up period. The diagnosis was confirmed on histopathological examination and was also supported by avid sarcolemmal staining for MHC-1 and MHC-2.

Apocrine adenocarcinoma of the eyelid: case series and review.

PURPOSE: To report 2 patients with apocrine adenocarcinoma of the eyelid and review the literature. DESIGN: Noncomparative, retrospective case series and review. PARTICIPANTS: Clinical data of 2 patients with histopathologic diagnoses of apocrine adenocarcinoma of the eyelid were obtained from medical records. The histopathology and immunohistochemistry data for the 2 cases were studied. A retrospective review was performed on all reported cases of eyelid apocrine carcinoma. MAIN OUTCOME MEASURES: Clinical features, histopathology, immunohistochemistry, and clinical course following treatment are reported. The results of a retrospective review of clinical presentations, local and systemic spread, treatment method, and prognosis from published case reports on eyelid apocrine adenocarcinoma are discussed. RESULTS: One patient had orbital invasion at presentation. This patient had radiotherapy alone and had no systemic or local recurrence at 2 years. One patient underwent a 4-mm margin wide surgical excision. This patient had no local or systemic recurrences at 1 year. A review of the literature suggests that the prognosis of adnexal apocrine tumors is indeterminate and variable. Most patients have successful outcomes following local surgical excision. Radiotherapy has been used as an adjunctive treatment for local metastatic disease. CONCLUSIONS: Apocrine adenocarcinoma is a rare adnexal tumor of the eyelid, which may present with orbital invasion.

Natural history of primary paediatric optic nerve sheath meningioma: case series and review.

PURPOSE: To study the natural history, clinical and radiological characteristics of primary paediatric optic nerve sheath meningioma (PPONSM). METHODS: Retrospective study of eight paediatric patients who were treated between 1994 and 2016 at the University Hospital Zurich, Switzerland and the Royal Adelaide Hospital, Australia. Clinical records and imaging studies were reviewed. RESULTS: The mean age at presentation was 11 years (range: 6-17 years). There were six female patients and two male patients. 2/8 patients had associated neurofibromatosis type 2. Patients were followed up for 71-297 months (mean 156±70 months). 6/8 patients were observed through the course of their disease and 2/8 patients were treated with radiotherapy. 2/8 patients who were observed had minimal change in vision and did not experience tumour growth after long-term follow-up. CONCLUSIONS: This is the largest PPONSM case series with long-term data on patients treated conservatively. We highlight that a small subset of these tumours are indolent and can be managed using observation alone.

The phenotype of limbal epithelial stem cells.

PURPOSE: The purpose of this study was to identify phenotypic markers of human limbal stem cells in fetal and adult corneas. METHODS: RNA from microscopically dissected superficial limbal and central fetal (18 weeks) corneas was amplified and used to generate P(32)-labeled, reverse-transcribed antisense RNA that was linearly amplified and hybridized to a focused stem cell cDNA microarray. Differential gene expression of fetal limbus was compared with the expression of central cornea. Microarray differential expression experiments were performed on P63-expressing primary cultured limbal epithelial cells (passage 1; Pa1) and primary cells passaged 5 times (Pa5). Semiquantitative RT-PCR assay and immunohistochemistry were performed on fetal and adult corneas and cultured primary limbal epithelial cells, to confirm the results of the microarray experiments. Slow-cycling (pulsed bromodeoxyuridine label-retaining) limbal epithelium in corneal organ culture was studied for the expression of four selected upregulated limbal genes. RESULTS: Of the 266 genes tested, 33 were differentially overexpressed (more than twofold) in the fetal limbus (compared with central cornea) and primary cultured limbal epithelium compared with primary cells after 5 passages. Cytokeratin 15 (CK15) and cytokeratin 14 (CK14) are expressed in limbal basal epithelium and P-cadherin (CDH3) and Wnt-4 expression was restricted to basal and immediate parabasal limbal epithelium of both the adult and fetal corneas). Bromodeoxyuridine label retaining epithelium in corneal organ culture (slow-cycling cells) expressed the four selected limbal upregulated genes. CONCLUSIONS: For the first time, a focused stem cell pathway microarray analysis has been performed on fetal cornea and cultured limbal explant epithelium. CK15, CK14, CDH3, and Wnt-4 are expressed in the basal limbal epithelial cells.

Periocular necrotising fasciitis: a multicentre case series.

INTRODUCTION: Necrotising fasciitis (NF) is a severe infection of deep subcutaneous soft tissues with high morbidity and mortality. Periocular necrotising fasciitis (PONF) is a very rare condition with many unanswered questions about the presentation and management. We present a retrospective case series of patients with PONF from three centres in Australia and two in the UK to investigate the clinical and microbiological characteristics and outcomes and report on patients treated with antibiotics alone. RESULTS: Twenty-nine patients (20 men; 69%) with PONF were identified and followed up for between 2 months and 10 years (median 57, mean 52.6 months) between 1990 and 2013. Conditions associated with chronic immunocompromise were present in 16/29 (55%). Twenty-one (75%) recalled minor periocular trauma or an infected lesion, two having been assaulted by the same assailant. Systemic shock occurred in 6/29 (21%) patients and 1 died. Group A, ß-haemolytic Streptococcus was the most common bacterium identified (25/29, 86%). Intravenous antibiotics were used in all patients, and up to five tissue debridements were required to control the disease in 23/29 (74%); reconstructive surgery was required in 12/29 (41%) patients. One patient died from the disease and visual loss occurred in four eyes of four patients (14%). CONCLUSIONS: PONF has a better prognosis than disease elsewhere in the body, but is still associated with significant risk of visual loss and a small risk of death. Intravenous antibiotic treatment with cautious observation may be reasonable in selected patients with a low threshold for debridement.