Richard Collin

The associations of floppy eyelid syndrome: a case control study.

OBJECTIVE To describe the demographic features of a large series of patients with floppy eyelid syndrome (FES) and to investigate the associations of the condition with keratoconus, obstructive sleep apnea-hypopnea syndrome (OSAHS), and a variety of upper and lower eyelid features. DESIGN Case control study. PARTICIPANTS The test group comprised 102 patients with FES. A control group of 102 patients were recruited from a diabetic retinopathy clinic and matched on a 1:1 basis on age, gender, and body mass index (BMI). METHODS A full medical and ophthalmic history was taken. Patients also underwent a full ocular examination, including an assessment of upper and lower lid laxity and upper lid levator function. Keratoconus grading was made using the Oculus Instruments Pentacam imaging system (Oculus Optikgerate GmbH, Wetzlar, Germany). Patients were screened for OSAHS using the Epworth daytime somnolence score. Matched statistical analysis of dichotomous data was made using Mantel-Haenszel methods for odds ratios and McNemars test. Analysis of continuous data was performed using a matched t test and tests for symmetry of larger tables were made using the McNemar-Bowker test. MAIN OUTCOME MEASURES The significance of association of FES with keratoconus, OSAHS, smoking history, medial and lateral canthal laxity of the upper and lower lids, levator function, lash ptosis, and dermatochalasis. RESULTS Significant associations were found between FES and OSAHS (P = 0.0008), keratoconus (P<0.0001), lash ptosis (P<0.0001), dermatochalasis (P = 0.02), upper lid medial canthal laxity (P = 0.02), upper lid distraction (P = 0.001), palpebral aperture (P = 0.004), and levator function (P = 0.005). CONCLUSIONS Floppy eyelid syndrome seems to be a condition strongly associated with OSAHS and keratoconus. As well as providing a platform for an etiologic hypothesis for the condition, these findings should also encourage clinicians to be aware of these associations and to direct further treatment. FINANCIAL DISCLOSURE(S) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Systemic anomalies in 77 patients with congenital anophthalmos or microphthalmos

Congenital anophthalmos and microphthalmos are rare conditions which can have associated pathology in the second eye and/or systemic anomalies. A retrospective review of 77 patients with congenital anophthalmos or microphthalmos seen at Moorfields Eye Hospital over a 13 year period was performed. A detailed description of the ocular and systemic anomalies present in our series of patients is given, and the current understanding of the pathogenesis of congenital anophthalmos and microphthalmos is reviewed.

Anterior lamellar repositioning and grey line split for upper lid entropion in ocular cicatricial pemphigoid

Purpose. Trichiasis in ocular cicatricial pemphigoid (OCP) is usually due to cicatricial entropion and is a major cause of ocular morbidity. Unfortunately in this disease, direct surgery on the conjunctiva often results in marked inflammation and cicatrisation. This paper assessed a procedure that corrects cicatricial entropion of the upper lid while avoiding surgery to the conjunctiva.Methods. A grey line upper lid split and a vertical anterior lamellar repositioning was performed on 16 lids of 11 patients with OCP.Results. Anatomical success was achieved in 72% of lids at 1 year and 61% had complete success with no lashes touching the globe. These outcomes were maintained up to 4 years. There were no perioperative complications. Two patients post-operatively developed severe conjunctival inflammation that required systemic immunosuppression. The causes of failure were primary surgical failure (n = 2), progression of cicatrisation secondary to surgically induced inflammation (n = 1), development of misdirected lashes (n = 1) and late recurrence at 7 months (n = 1). One patient developed peaking of the eyelid.Conclusions. This procedure has a good long-term outcome with minimal complications. Activation of severe conjunctival inflammation occurred in 13% of cases and this must be considered pre- and post-operatively.

Early Cure Rates with Narrow-Margin Slow-Mohs Surgery for Periocular Malignant Melanoma

BACKGROUND Staged excision with rush-processed paraffin-embedded tissue sections (Slow-Mohs) is an effective treatment for periocular melanoma. Although there is no consensus on initial margins of excision, narrower margins in the eyelids have the functionally and cosmetically important consequence of smaller postoperative wounds. OBJECTIVES To report early cure rates for periocular melanoma using Slow-Mohs surgery with en-face margin sectioning. METHODS Retrospective, multicenter, noncomparative case series. Slow-Mohs surgery in 14 patients with periocular melanoma from 2000 to 2006. RESULTS Fourteen patients underwent 14 Slow-Mohs procedures for eight lentigo maligna, one nodular, and one superficial spreading melanoma, and four lentigo maligna, 12 primary, and two recurrent tumors. The most common site was the lower eyelid (8/14, 57.1%). Breslow thickness ranged from 0.27 to 1.70 mm, with four cases less than 0.76 mm and one case greater than 1.5 mm. Five cases were a Clark level II or greater. Complete excision was achieved with one level (6 cases) or two or three levels (8 cases), with 2- to 3-mm margins at each level in all but one case. With median follow-up of 36 months, there were two local recurrences (2/14, 14.3%). CONCLUSION Slow-Mohs with en-face sections achieves similar early cure rates to previously published margin-controlled excision techniques. Narrow margins of excision can optimize tissue preservation without compromising outcome.

Split thickness buccal mucous membrane grafts and beta irradiation in the treatment of recurrent pterygium

BACKGROUND Pterygium is a common problem and after surgical removal may recur in up to 80% of cases, depending on the technique of primary excision. Recurrent pterygia can be aggressive and repeated excision may result in severe conjunctival scarring and shortening, resulting in insufficient conjunctiva to perform further grafting and lid surgery. When there is insufficient autologous conjunctiva, mucous membrane must be obtained from other sites. Full thickness buccal mucous membrane grafts have been described, but they may result in a beefy red appearance, with graft contraction and a poor tear film. METHOD The use of split thickness buccal mucous membrane grafts is described in three patients with recurrent pterygium, two in combination with lamellar keratoplasty. β Irradiation was used as adjuvant therapy in all cases. RESULTS In all three cases an acceptable cosmetic appearance was achieved, with no recurrence of the pterygium, and a good range of eye movements. CONCLUSIONS It is recommended that split thickness buccal mucosal grafts, combined with β irradiation, should be considered in complex cases of pterygium recurrence when there is insufficient autologous conjunctiva and conjunctival shortening with restricted eye movements.

Floppy eyelid syndrome: stretching the limits.

Floppy eyelid syndrome is a distressing condition that can cause significant morbidity and vision loss. Many systemic and ocular associations have been proposed, most notably keratoconus and obstructive sleep apnea-hypopnea syndrome. Although conservative treatments can sometimes be effective, a wide variety of surgical treatments to tighten the upper eyelid have been described. The underlying pathogenesis remains elusive, although progress has been made in the identification of extracellular matrix changes in the tarsal plate. This systematic review discusses the issues surrounding ambiguities in the definition of floppy eyelid syndrome as well as what is currently known about its clinical features, ocular and systemic associations, pathological changes, and proposed theories of pathogenesis. In addition a critical discussion of the proposed surgical treatments and their reported success rates and follow-up times is provided.

Lax eyelid syndrome or ‘progeria’ of eyelid tissues

PURPOSE The Lax Eyelid Syndrome was described by Van den Bosch and Lemij 1 as an uncommon disorder seen in non-obese elderly people and characterised by chronic ocular surface irritation symptoms and a “floppy upper eyelid”. The authors present some new features of the lax eyelid syndrome. METHODS The authors report five patients, belonging to a younger age group, who presented with premature laxity of all the eyelid tissues. This caused medial and lateral canthal dystopia and eyelid malposition including ptosis, entropion and ectropion. Initial surgical correction was often followed by recurrence after some time. CONCLUSIONS The authors highlight the differences between lax eyelid syndrome, cutis laxa, floppy eyelid syndrome and the blepharochalasis syndrome and suggest that lax eyelid syndrome can be thought of as “progeria” or premature ageing of the eyelid tissues to distinguish it clearly from these other conditions.

Changes in Fibroblast Mechanostat Set Point and Mechanosensitivity: An Adaptive Response to Mechanical Stress in Floppy Eyelid Syndrome

PURPOSE Floppy eyelid syndrome (FES) is an acquired hyperelasticity disorder affecting the upper eyelid. The tarsal plate becomes hyperelastic with a loss of intrinsic rigidity. As a result, the eyelid is subjected to cyclic mechanical stress. This condition was used as a model to investigate changes in dynamic fibroblast contractility in the context of chronic cyclic mechanical stress. METHODS Contractile efficiency was investigated in a free-floating, three-dimensional collagen matrix model. Intrinsic cellular force measurements and responses to changes in gel tension were explored using a tensioning culture force monitor (t-CFM). Gene expression differences between cell lines exhibiting differences in contractile phenotype were explored with a genome level microarray platform and RT-PCR. RESULTS FES tarsal plate fibroblasts (TFs) showed an increased contractile efficiency compared with the control, and t-CFM measurements confirmed a higher intrinsic cellular force at plateau levels. Cyclic stretch/relaxation experiments determined that TFs in FES maintained a functional tensional homeostasis response but with an altered sensitivity, operating around a higher mechanostat set point. Gene expression array and RT-PCR analysis identified V-CAM1 and PPP1R3C as being upregulated in FES TFs. CONCLUSIONS These changes may represent an adaptive response that allows tensional homeostasis to be maintained at the high levels of tissue stress experienced in FES. Gene expression studies point to a role for V-CAM1 and PPP1R3C in mediating changes in the dynamic range of mechanosensitivity of TFs. This work identifies FES as a useful model for the study of adaptive physiological responses to mechanical stress.

Merkel Cell Carcinoma of the Eyelid: Management and Prognosis

IMPORTANCE The literature on Merkel cell carcinoma (MCC) of the eyelid remains scarce, and there has yet to be a study using the most up-to-date TNM staging system for this rare but aggressive tumor. OBJECTIVE To analyze the TNM stage, management, and outcomes of patients with MCC of the eyelid. DESIGN, SETTING, AND PARTICIPANTS Retrospective case series of 21 patients from 5 tertiary referral centers in the United Kingdom and Australia with primary MCC of the eyelid presenting at a median age of 77 years, with median follow-up of 54 months. Tumors were staged according to the American Joint Committee on Cancer, 7th edition, TNM criteria for eyelid carcinoma and MCC. MAIN OUTCOMES AND MEASURES TNM stage, treatment modalities, and clinical outcome. RESULTS The eyelid carcinoma TNM stages were T2aN0M0 for 5 patients, T2bN0M0 for 7 patients, T3aN0M0 for 4 patients, T3bN0M0 for 3 patients, T2bN1M0 for 1 patient, and T3aN1M0 for 1 patient. The MCC TNM stages were T1N0M0 for 12 patients, T2N0M0 for 7 patients, T1N1M0 for 1 patient, and T2N1M0 for 1 patient. One patient had a sentinel lymph node biopsy, and 8 patients underwent head/neck imaging. Eighteen patients underwent a wide local excision, 12 with a paraffin section and 6 with a frozen section. Two patients underwent Mohs surgery, 1 of whom required an orbital exenteration. Twelve patients (57%) received adjuvant radiotherapy, and 2 patients received chemotherapy. The local recurrence rate was 10%, the regional nodal recurrence rate was 10%, and the distant metastatic recurrence rate was 19%. The lowest T category tumor metastasizing to both regional nodes and distant locations was a T2a (eyelid TNM)/T1 (Merkel TNM) tumor measuring 8 mm. Two patients with T3a (eyelid TNM)/T2 (Merkel TNM) tumors died of metastatic MCC. CONCLUSIONS AND RELEVANCE The majority of patients with MCC of the eyelid present with localized eyelid disease of T category T2 (eyelid TNM)/T1 (Merkel TNM). A wide local excision with margin control remains the mainstay of treatment, whereas the use of radiotherapy is institution specific. Tumors with a low T category are associated with regional nodal and distant metastatic disease. It may therefore be reasonable to consider a sentinel lymph node biopsy or strict regional lymph node surveillance for all MCCs of the eyelid, regardless of T category or size.

Long-term Outcomes of Surgical Approaches to the Treatment of Floppy Eyelid Syndrome

OBJECTIVE To identify and describe the different procedures used in the treatment of floppy eyelid syndrome (FES) at Moorfields Eye Hospital and to evaluate their effectiveness. DESIGN Cross-sectional study. PARTICIPANTS A total of 71 patients who had undergone surgery for FES over a 13-year period since 1995 at Moorfields Eye Hospital were recruited. Retrospective data from 7 patients were also included, providing data for 78 patients. METHODS Patients underwent a full ocular examination. A survival analysis was determined by plotting Kaplan-Meier curves for each type of procedure encountered. Comparison of survival trends was made using a log-rank test. The possible effects of bias arising from bilaterality of the condition were investigated using a sensitivity analysis and a Cox regression analysis allowing for clusters. Tests for surgeon bias were made using the Fisher exact test. MAIN OUTCOME MEASURES Recurrence of the condition. An assessment of recurrence was made clinically by 2 independent observers who were masked to the type of surgery the patient had undergone. RESULTS Four different forms of surgical treatment were encountered: (1) Full-thickness wedge excision (FTWE) (26 patients, 33 procedures); (2) Upper lid lateral tarsal strip (LTS) (31 patients, 43 procedures); (3) Medial canthal (MC) and lateral canthal (LC) plication (15 patients, 19 procedures); (4) Medial tarsal strip (6 patients, 6 procedures). A total of 44 of 101 procedures had failed. Superior long-term survival outcomes of both LC/MC plication (P = 0.003) and upper lid LTS (P = 0.001) procedures over FTWE was demonstrated. However, survival comparison between the LC/MC plication and LTS groups did not achieve significance (P = 0.37). No significant difference in outcome between surgeon groups of equivalent experience was demonstrated (P = 0.18). No bias arising from bilaterality of the condition was identified. CONCLUSIONS These data provide strong evidence of better survival outcomes in FES using the MC/LC plication and LTS procedures in comparison with the FTWE procedure. On the basis of experience from our unit, we recommend that the FTWE procedure be avoided as a form of treatment for FES in favor of the MC/LC plication, LTS, or medial tarsal strip procedure. FINANCIAL DISCLOSURE(S) The author(s) have no proprietary or commercial interest in any materials discussed in this article.