Keratoconus associated with floppy eyelid syndrome.

Floppy eyelid syndrome is a recently described entity, which characteristically involves overweight individuals. The characteristic findings are an upper lid that may be readily everted, tarsal laxity, and diffuse papillary conjunctival changes. The cause of floppy eyelid syndrome is believed to be a mechanical disorder due to the eversion of the lids while sleeping. The cause of keratoconus remains uncertain. There are strong proponents to a mechanical etiology for this disease. The authors report five cases of floppy eyelid syndrome with concomitant keratoconus. One patient with bilateral keratoconus had bilateral symmetric floppy eyelid syndrome. The other four patients had asymmetric keratoconus and floppy eyelid syndrome. In all four patients, the keratoconus was significantly worse in the eye with the more severe case of floppy eyelid syndrome. In addition, these four patients all gave a history of sleeping with their head facing predominantly on the side with the floppy eyelid syndrome and keratoconus. Two patients with keratoconus and floppy eyelid syndrome were able to undergo successful contact lens rehabilitation of their keratoconus after treatment of the floppy eyelid syndrome.     

Floppy eyelid syndrome: a diagnostic dilemma.

BACKGROUND: Floppy eyelid syndrome may be the underlining cause of papillary keratoconjunctivitis. Patients initially report a nonspecific irritation, redness, or a foreign body sensation. METHODS: As a result of its vague presentation, floppy eyelid syndrome is frequently misdiagnosed. A careful slit-lamp evaluation and a complete history aids in the diagnosis. CONCLUSIONS: The most distinctive feature of floppy eye syndrome is a pliant upper tarsus that is easily everted without excess manipulation. Histopathology has attributed the laxity of the lid to a decreased amount of elastin within the tarsus. Treatment includes prevention of the upper lid from everting during sleep or surgical procedures such as horizontal eyelid shortening. Our case report illustrates a patient with a classic case of floppy eyelid syndrome.     

Eyelid retractor surgery as an adjunct to cicatricial ectropion repair

Purpose : To evaluate the effectiveness of eyelid retractor repair in cicatricial ectropion of the lower eyelid. Methods : The study design was a prospective case series. One hundred and twenty eight eyelids were operated on in 100 consecutive patients with cicatricial ectropion. All patients underwent lower eyelid retractor repair via a conjunctival approach combined with skin replacement to the anterior lamella with or without a horizontal lid tightening procedure. When only medial ectropion was present, a medial‐based transpositional skin flap was used to repair the anterior lamella (26 eyelids). The remaining eyelids with ectropion involving all or most of the eyelid underwent upper‐to‐lower eyelid lateral‐based transpositional skin flap repair (92 eyelids), or full thickness free skin grafting (10 eyelids). Horizontal lid tightening was performed by lateral canthoplasty in 123 eyelids. Results : Relief of cicatricial ectropion symptoms was reported in 90% of patients overall. A normal punctum position was achieved in 70% of eyelids, overall, and was highest (88%) with a medial‐based transpositional skin flap. Conclusions : Eyelid retractor repair combined with skin replacement and horizontal lid shortening is an effective procedure for cicatricial ectropion.     

Medial upper eyelid shortening to correct medial eyelid laxity in floppy eyelid syndrome: a new surgical approach

PURPOSE: To describe and present the results of a new surgical technique for patients with floppy eyelid syndrome, based on the medial upper eyelid stretching encountered in this condition. METHODS: A case series of 24 patients with floppy eyelid syndrome who where found to have symptomatic predominately medial upper eyelid laxity was analyzed. The history, clinical features, histopathology, and outcome were reviewed after patients underwent medial upper eyelid shortening with or without upper eyelid skin reduction as the first surgical procedure. RESULTS: Of the 24 patients, 18 were men (75%) with a mean age at referral of 56 years, having ocular discomfort and conjunctival irritation/papillary conjunctivitis as the main complaints at presentation. Obesity was present in 96% of cases, with lower eyelid laxity/ectropion (50%) and upper eyelid eyelash ptosis (29%) in conjunction with the upper eyelid laxity. The affected side was related to sleeping habits or recurrent mechanical eyelid trauma. Histologic studies showed a nonspecific inflammatory cell infiltrate and loss of elastin with loose dermal connective tissue. After surgery, complete relief of ocular symptoms and good functional and cosmetic results were present in all cases after 18 months of follow-up. CONCLUSIONS: This new surgical approach is based on the presence of predominately medial upper laxity in patients with floppy eyelid syndrome. The excision of this stretched area stabilized the upper eyelid in an anatomic fashion, providing a good and stable long-term result. The possible mechanisms involved in the medial upper eyelid stretching are discussed.     

Surgical management of floppy eyelid syndrome

Four patients (five eyes) with the classic findings of floppy eyelid syndrome all had chronic irritative symptoms, with papillary conjunctivitis, and a soft, rubbery, floppy, and easily everted upper eyelid. All affected eyes were treated surgically by a full-thickness eyelid shortening procedure which produced immediate relief of symptoms. Histopathologic study of the resected eyelids showed only an inflammatory infiltrate in the conjunctiva, but failed to identify specific cause for the lax tarsus.     

Utility of meibography in the evaluation of meibomian glands morphology in normal and diseased eyelids

PurposeTo study the utility of meibography for the morphology of meibomian glands in normal eyelids and in various eyelid diseases.DesignA cross-sectional study.MethodsA newly designed transilluminator, fitting both the upper along with lower eyelids, and an infrared camera were used to obtain video clips of the meibomian glands for 60 asymptomatic subjects with normal eyelid margin. Parameters studied included, ocular surface, Schirmer test, and tear breakup time (TBUT). The meibomian glands of patients with meibomian glands’ abnormality secondary to infectious, inflammatory, malignant, congenital, or post-radiation therapy disease related etiologies were compared with normal patients. Still pictures were extracted from the video clips to evaluate the meibomian glands for gland dropout and gross morphological changes.ResultsIn normal subjects, meibomian glands appeared to be thinner and longer in the upper eye lids than in the lower eye lids. Gland dropout occured with increased age, more in the lower eye lid and in females. Excessive gland drop out (> 75%) was seen in patients with history of trachoma, Stevens Johnson syndrome, severe blepharitis, and post-radiation for orbital tumors. Variable gland drop out was noticed in patients with floppy eyelid syndrome, and blepharitis. In patients with congenital distichiasis, partial or complete gland drop out at the part of the eyelid margins affected by distichiasis was noticed.ConclusionsThe newly designed transilluminator permitted the examination of both upper and lower eye lid meibomian glands with minimal discomfort. Evaluating the anatomical changes involving meibomian glands with meibography may help increase our understanding of the meibomian gland-related diseases, monitor the effects of treatment, and provide helpful information for patient education.     

Surgical management of seventh nerve paralysis and floppy eyelid syndrome

A seven-stage approach to the management of the paretic eyelid complex has been described. These stages include supportive care, planning and execution of general facial reanimation, lower eyelid and canthal resuspension or support, passive upper eyelid animation, dynamic lid animation, and soft tissue repositioning. A final stage, the epilogue, is described for the treatment of the synkinesis and hypertonicity that often develop. Recent developments in these areas are discussed. Floppy eyelid syndrome, first described by Culbertson and Ostler, is a syndrome of chronic papillary conjunctivitis in overweight patients with easily everted eyelids. The syndrome has now been associated with a variety of other conditions and findings. Surgical management with horizontal shortening of the floppy eyelids remains the mainstay of therapy.     

Floppy eyelid syndrome and blepharochalasis

Floppy eyelid syndrome and blepharochalasis may represent a spectrum of one underlying disease. Two patients with floppy eyelid syndrome and one with blepharochalasis shared important clinical similarities. All three patients displayed eyelid laxity associated with papillary conjunctivitis and ocular surface abnormalities. Histopathologic findings were similar in the three cases, characterized by chronic conjunctival inflammation with normal tarsal collagen. In all three patients surgical horizontal eyelid shortening led to improvement in symptoms and findings.     

Floppy eyelid syndrome. Management including surgery

Six overweight patients, five men and the first woman to be reported, are described with floppy eyelid syndrome. Five patients had bilateral ocular involvement. Only two patients were free of other ocular surface problems. Concurrent diagnoses included blepharitis, keratoconjunctivitis sicca, lagophthalmos, medicamentosal conjunctivitis, molluscum contagiosum, and masquerade syndrome. Five patients responded to appropriate therapy, which included shielding the eyes during sleep. Three patients underwent a horizontal lid tightening procedure, which resulted in permanent correction of their floppy eyelids and resolution of their ocular signs and symptoms.     

Sticky eyelid syndrome

PURPOSE: To report on patients seen with an unusual condition affecting the eyelids. The syndrome manifests as a temporary adhesion which forms between the upper and lower eyelid associated with laxity of the lower lid retractors. This results in a peculiar closure of the lids. DESIGN: Retrospective case reports. METHODS: Charts of affected patients were reviewed for their clinical histories, examination findings, external photographs, and the results of treatment. RESULTS: Ten consecutive patients with the associated findings were reviewed. All cases were unilateral. Seven patients were Asian and three were Caucasian. Nine patients were symptomatic; of these, all were treated conservatively except for one who requested surgery. Two cases are described and photographs are shown. CONCLUSION: Lower eyelid retractor laxity combined with a temporary adhesion between the upper and lower lid results in the clinical findings of Sticky Eyelid Syndrome.     

Acquired lax eyelid syndrome: an unrecognized cause of the chronically irritated eye

PURPOSE: To describe the demographics, symptoms, and findings of acquired lax eyelid syndrome and to assess the efficacy of the 4-eyelid tarsal strip-periosteal flap technique to treat horizontally lax upper and lower eyelids. METHODS: A retrospective, case-series review of 80 patients (320 eyelids) evaluated from January 2000 to April 2004 for lax upper and lower eyelids causing chronic irritation was performed. Ten patients with diagnosed floppy eyelid syndrome or obstructive sleep apnea were excluded. Height and weight of all patients were recorded to calculate body mass index. Lateral tarsal strip fixation of all 4 eyelids to periosteal flaps based inside the orbital rim was performed to achieve horizontal tightening. Postoperative follow-up ranged up to 52 months. Preoperative/postoperative symptoms and examination findings of upper and lower eyelid distraction, keratopathy, and conjunctival inflammation were compared. RESULTS: The most common presenting symptoms were epiphora (85.7%) and irritation (80%). The most common examination findings were upper and lower eyelid horizontal laxity and palpebral conjunctival injection (100% patients). Thirteen of 70 patients (18.6%) were obese, based on body mass index; 26 of 70 patients (37.1%) were mildly overweight; 29 of 70 patients (41.4%) were normal weight; and 2 of 70 patients (2.9%) were underweight. After surgery, 91% of patients had improved or resolved symptoms and signs; 2.5% of dehiscences occurred with the use of the 4-eyelid technique. Gradual continued improvement was observed for up to 1 year. CONCLUSIONS: Evaluation of patients presenting with chronically irritated eyes should include distraction of both the upper and lower eyelids and examination for conjunctival inflammation. Acquired lax eyelid syndrome is similar to floppy eyelid syndrome; however, 43.3% of patients were normal weight or underweight. The 4-eyelid tarsal strip-periosteal flap fixation is a rapid and effective technique for correction of this syndrome.     

An unusual case of floppy eyelid syndrome

A 51-year-old obese man with a history of nocturnal upper eyelid eversion secondary to floppy eyelids presented with a Staphylococcus aureus corneal ulcer. No papillary conjunctivitis was present. The corneal ulcer was treated successfully. This case demonstrates the importance of ruling out spontaneous nocturnal lid eversion as a cause for unexplained ocular surface pathology and emphasizes the fact that the floppy eyelid syndrome may present in the absence of classic papillary conjunctivitis.     

Reversal of floppy eyelid syndrome with treatment of obstructive sleep apnoea

Purpose : To describe the beneficial effects of treatment of obstructive sleep apnoea on the symptoms and signs of floppy eyelid syndrome. Method : A case of sleep apnoea with associated floppy eyelid syndrome is reported. Results : A 32‐year‐old man presented with left floppy eyelid syndrome and a known diagnosis of obstructive sleep apnoea. He underwent treatment for 4 years with continuous positive airways pressure by mask during sleep and the symptoms and signs of his floppy eyelid syndrome disappeared. Conclusion : Treatment of obstructive sleep apnoea may reverse the changes of floppy eyelid syndrome.     

Operation of the floppy eyelid. Symptomatic cases require surgical eyelid stabilization

BACKGROUND: Floppy Eyelid is a rare condition causing chronic papillary conjunctivitis and chronic corneal disorders (superficial punctate keratitis, epithelial and stromal ulcers). It is characterized by an extremely enlarged and "floppy" upper eyelid which can be easily everted by slight elevation. Usually obese men are affected who use to sleep face down either on the right or on the left side. Pushing the eyelid against the pillow, the lid is intermittently everted at sleep. This lagophthalmus with rubbing of the exposed eye and lid structures causes all pathologic disorders. Shielding the eye at night may help temporarily. PATIENTS: We performed surgery on 7 men with symptomatic floppy eyelid in the age of 42 to 61 years. The patients had been symptomatic between 1 month and 4 years prior to the definite diagnosis. Follow-up time has been 7 months to 4.5 years. RESULTS: In all cases surgery achieved improvement. CONCLUSIONS: Cases of unclear conjunctival or corneal damage and inflammation should led consider Floppy Eyelid as a possible cause. The typical clinical findings make diagnosis easy. As complete stopping of eyelid-rubbing by changing the patient's sleeping habits is mostly not easily possible, for acute therapy of threatening damages to cornea and conjunctiva a surgical shortening of the lid is necessary which stabilizes the lid in order to avoid nightly spontaneous eversion for a long time.     

Full thickness eyelid transsection (blepharotomy) for upper eyelid lengthening in lid retraction associated with Graves' disease

AIM: To report on a modified technique for upper eyelid lengthening in lid retraction associated with Graves' disease. METHODS: A prospective consecutive interventional case series. 41 patients, 38 women and three men, with a mean age of 55 years (range 32-75 years) were included. Full transsection of the upper eyelid with optional transsection of the lateral horn was performed in 60 lids. A central bridge of conjunctiva was left intact in the pupillary axis. Before and after surgery, the skin crease, palpebral aperture, and scleral show were measured and the contour of the lid margin was assessed. The overall result was graded as "perfect," "acceptable," and "failure." RESULTS: The mean period review was 6 months (range 2-36 months). Preoperatively, upper eyelid retraction varied from 1 mm to 7 mm; mean scleral show was 2 mm in 45 lids, the palpebral aperture was in the desired height (SD 1 mm) in 53 of 60 lids (88%) and was reduced by 3 mm (mean), the skin crease remained unchanged (SD 1 mm) in 44 of 60 lids (73%). The result was considered "perfect" in 43 and "acceptable" in 14 of 60 lids after one or two surgical approaches. In three lids, the surgery had failed. CONCLUSION: In contrast with other surgical techniques the surgical approach presented here is easy to perform and still leads to very satisfying and predictable postoperative results.     

Floppy eyelid syndrome

PURPOSE OF REVIEW: Although floppy eyelid syndrome causes significant ocular symptoms and morbidity, the condition is often underdiagnosed. This review will highlight diagnostic features of the condition, emphasizing recent advances in the understanding of its pathophysiology. Current therapeutic strategies and surgical techniques are discussed. RECENT FINDINGS: Current concepts regarding the underlying pathophysiology of floppy eyelid syndrome revolve around upregulation of elastin degrading enzymes and mechanical factors. Together, these forces cause instability of the eyelid scaffold, resulting in eyelid malposition and ocular symptoms. Newer surgical treatments aim to preserve tarsus to improve eyelid stability and position. SUMMARY: Floppy eyelid syndrome--an underdiagnosed condition--produces significant ocular morbidity. Symptoms range from occasional redness and irritation to corneal ulcer. Diagnosis is based on ocular signs, including easy or spontaneous eversion of the upper eyelids in conjunction with conjunctivitis and keratitis. The condition, associated with body mass index and obstructive sleep apnea, should be suspected in any obese patient with a chronic red and tearing eye. Treatment consists of supportive measures such as ocular lubrication, eyelid taping or a shield, and surgery to address horizontal laxity and redundant eyelid tissues.     

Pachydermoperiostosis: A Rare Cause of Marked Blepharoptosis and Floppy Eyelid Syndrome

Abstract

A 34-year-old African-American man was referred for eyelid swelling and ocular discomfort. He was found to have floppy hypertrophic eyelids and marked bilateral mechanical ptosis that was present since childhood. Systemic examination was significant for furrows on his forehead and scalp, coarse facial features, and enlarged hands and feet with clubbing of the fingers and toes. Radiographic imaging of the long bones demonstrated periostosis, and MRI of the head revealed a pituitary macroadenoma. Pituitary and thyroid hormone levels were normal. The patient was diagnosed with pachydermoperiostosis and a non-secreting pituitary macroadenoma. Bilateral upper lid tightening via wedge resection was followed by bilateral external levator advancement ptosis repair in a staged manner. The patient achieved symptom relief and improved lid position postoperatively.     

EYELID RECONSTRUCTION USING A MONOPEDICLE FLAP AND BUCCAL MUCOSA: REPORT OF 15 CASES

A method is described for eyelid reconstruction to close a large defect involving the lid margin using a myocutaneous flap over a free buccal mucosal graft. The procedure was performed on 15 patients over a three-year period. Thirteen patients had loss of lower lid tissue alone. One included the lateral canthus and one over 50% of both upper and lower lids. Results were good in all patients except the one with extensive reconstruction of both lids. Advantages over other techniques of lid reconstruction include ease of surgery, adaptability of the technique to any eyelid site, good tissue match and the need for only a one-stage procedure.     

Pyoderma gangrenosum of the ocular adnexa: a rare condition with characteristic clinical appearances

PURPOSE: To describe the clinical characteristics of periocular pyoderma gangrenosum and to highlight features that may encourage early diagnosis of this extremely rare condition. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS AND METHODS: Four patients with periocular pyoderma gangrenosum were treated in the Orbital Unit at Moorfields Eye Hospital over the course of a decade. MAIN OUTCOME MEASURES: Presenting clinical features, therapy, and outcome. RESULTS: Four patients (female) between the ages of 56 and 75 years (mean, 65 years; median, 64 years) sought treatment for slowly evolving, painful, unilateral blue-gray swellings of the pretarsal tissues of the lower (1 case), upper (1 case), or both eyelids (2 cases). The swelling progressed to frank tissue necrosis and loss of full-thickness eyelid, with patchy sparing of the lid margin or lashes; in some cases, there was a very distinctive preservation of the pretarsal marginal artery across full-thickness eyelid defects. The lid loss characteristically involved the lateral one third of the lower eyelid (3 of 4 lids), the central part of the upper eyelid (3 of 4 lids) and, in one case, extending into the postseptal tissues in the inferotemporal quadrant of the orbit. In 3 patients, the pyoderma, often associated with a positive serum rheumatoid factor (three of three cases where measured), responded well to systemic immunosuppression and eyelid repair was undertaken during the quiescent phase. In a single patient, relapsing disease led to loss of the eye as a result of involvement of the globe and deep orbital tissues. CONCLUSIONS: Although an extremely rare condition, periocular pyoderma gangrenosum has a typical clinical appearance, and early recognition and immunosuppression will reduce the ocular morbidity.     

Lid lengthening by sclera interposition for eyelid retraction in Graves'' ophthalmopathy.

The efficacy of scleral grafts for eyelid lengthening in patients with thyroid related upper and/or lower lid retraction was evaluated in 62 consecutive patients with Graves' ophthalmopathy who underwent lid surgery in the last 3.5 years. Seventy-eight upper and 30 lower lids were lengthened by scleral interposition. A good or acceptable result was achieved in 50% of all operated upper lids after one procedure. This percentage increased to 75% after a second and to 77% after a third procedure. Persistent temporal retraction and nasal overcorrection were the major complications. In lower lid lengthening the success percentage was 90% after one operation. We conclude that scleral grafting for upper eyelid lengthening has no distinct advantage in comparison with other lengthening techniques. Scleral implants to lengthen lower lids are very effective.