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Floppy Eyelid Syndrome and Blepharochalasis
Floppy Eyelid Syndrome and Blepharochalasis Robert Goldberg, M.D., Stuart Seiff, M.D., Joan McFarland, M.D., Kenneth Simons, M.D., and Norman Shorr, M.D. 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 pa~ tients surgical horizontal eyelid shortening led to improvement in symptoms and findings. A SYNDROME of chronic papillary conjunctivitis in overweight men with easily everted, floppy upper eyelids was first described in 1981 by Culbertson and Ostler.' These authors postulated eyelid eversion during sleep, with corneal-pillow contact, as the cause of the keratoconjunctivitis; Parunovic" doubted this mechanism and Schwartz, Gelender, and Forster" suggested that the mechanism of injury might be related to a poor interface between the loose eyelid and the underlying bulbar conjunctiva. The cause of tarsal laxity in the floppy eyelid syndrome is not known. There is no association in the reported cases with systemic diseases of collagen or elastic tissue, except for one case of hyperglycinemia.! which is not classically considered to involve connective tissue.' A frequent association with obesity is observed, and in one case weight loss was accompanied by relief of syrnptoms.! However, the reason for this association is obscure. Histopathologic investigations':' have shown chronic conjunctivitis with scarring and variable inflammation of the underlying tarsus. The
Accepted for publication June 16, 1986. From the Jules Stein Eye Institute, UCLA School of Medicine, Los Angeles, California. Reprint requests to Norman Shorr, M.D., 435 N. Roxbury Dr., Beverly Hills, CA 90210.
376
appearance of the tarsal collagen has been normal in all reported cases. Conservative treatment, including lubrication and patching, has met with variable suecess. l . 3,6,7 Early reports of surgical treatment with eyelid horizontal shortening procedures have been more encouraging.v" Blepharochalasis was described as early as 1807 according to Alvis," but Fuchs" is credited with naming the syndrome and providing a unifying description in the literature. Blepharochalasis is characterized by recurrent episodes of painless nonpitting edema of the upper eyelids, generally beginning around the time of puberty. The lower eyelids are occasionally affected and unilateral cases have been reported." The episodic edema eventually results in permanent eyelid changes characterized by redundant thinning of the skin, discoloration, telangiectasis, and atrophic relaxation of the eyelid support structures. Levator aponeurosis relaxation or dehiscence is frequent in the atrophic stage, leading to blepharoptosis. Prolapse of orbital fat and lacrimal tissue can occur; Stieren" recognized the prolapse as a consequence of atrophic changes in the orbital septum, refuting the earlier concept that this "ptosis adiposa" represented a separate form of the disease. Recent reports have drawn attention to pseudoepicanthal fold formation" and acquired blepharophimosis" as additional manifestations of the atrophic changes that characterize the late stages of blepharochalasis. Although early investigators suspected a female sex preference, Alvis" found equal numbers of males and females in his tabulation of reported cases. Recent case reports also suggest an equal sex incidence (Table). Familial cases of blepharochalasis have been reported, showing autosomal dominant inherirance.P'" An association with thickened double upper lip and variably with goiter (the Laffer-Ascher syndrome) has been described.F'" There have been scattered reports of blepharochalasis occurring in individuals with systemic disease, including a recent case," but no unifying pattern has emerged.
©AMERICAN JOURNAL OF OPHTHALMOLOGY
102:376-381,
SEPTEMBER,
1986
Vol. 102, No.3
Floppy Eyelid Syndrome and Blepharochalasis
TABLE SEX DISTRIBUTION IN CASES OF BLEPHAROCHALASIS REPORTED SINCE 1970
377
one case of blepharochalasis lead us to suggest an end-stage and possibly a primary relationship between these two conditions.
SEX DISTRIBUTION INVESTIGATORS
YEAR
MALE
FEMALE
Kett esy29 Steiglitz and Crawford 27
1970 1974 1978 1979 1979 1984 1984 1985
2 1 0 3 0 0 1
0 2 1 1 1 1 0 ?*
MOthlendyck and Hundeiker 23 Collin and associates 13 Brazin, Stern, and Johnson" Finney and 'Peterson3O Ghose, Balwant, and Yogeshwar24 Custer, Tenzyl, and Kowalcyzk'"
?*
'Not specified.
Histopathologic investigations of eyelid tissue have shown thinning of the skin and variable degrees of disorganization of elastic tissue. 9,l1 ,12,14,16,19,21,25,26 Increased vascularity is uniformly noted, and chronic inflammation is generally observed. The results of medical therapy have been disappointing. Many treatments, including antihistamines and corticosteroids, have been tried through the years but none have been effective. A surgical approach, involving excision of excess skin along with horizontal shortening and blepharoptosis repair, is generally recommended. Several investigators pointed out the need to postpone surgery until the disease is quiescent. 13,25,27 Overcorrection of blepharoptosis is not infrequent. 14,27 The clinical and pathologic similarities between two cases of floppy eyelid syndrome and
Case Reports Case 1 A 56-year-old man had had recurrent eyelid swelling during his teen-age years. He had undergone blepharoptosis repair several times in early adulthood, with only temporary effect. In 1984 he had bilateral blepharoptosis, atrophic upper and lower eyelids bilaterally, and prolapsing lacrimal glands that were worse on the left side (Fig. I, left). He complained of irritative ocular symptoms. Visual acuity was 20/25 in both eyes. Slit-lamp examination disclosed poor-quality tear film and papillary conjunctivitis. Schirmer testing showed 30 mm of wetting bilaterally. The patient was thought to have quiescent blepharochalasis. On initial surgical approach, dehiscence of the levator aponeurosis and lateral canthal tendon attenuation were found bilaterally. The levator dehiscence was repaired, but residual blepharoptosis required subsequent reinforcement with full-thickness upper eyelid resections bilaterally. The patient also underwent horizontal shortening of the upper and lower eyelids with canthal resuspension. Postoperatively, his irritative ocular symptoms and corneal surface abnormalities were noted to improve (Fig. I, right). Histopathologic analysis of resected eyelid tissue showed chronic inflammation of the tarsal conjunctiva; routine and elastin stains
Fig. 1 (Goldberg and associates). Case 1. Left, Preoperatively there is laxity of eyelids and supporting structures, with blepharoptosis and lacrimal gland prolapse. Right, Postoperatively.
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AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 2 (Goldberg and associates). Case 1. Conjunctiva showing chronic inflammatory cell infiltrate with normal underlying tarsal collagen (hematoxylin and eosin, x 40).
showed no abnormalities of tarsal collagen or elastic tissue (Fig. 2). Case 2 A markedly overweight 39-year-old man with diabetes and hypertension had a IS-year history of irritative ocular symptoms. He slept on his right side and was unaware of nocturnal eyelid eversion. There was no history or symptoms of connective tissue or metabolic disorders, and his family history was unremarkable. He had been treated with lubricants, patching, antibiotics, and topical corticosteroids without improvement. Best corrected visual acuity was R.E.: 20/40 and L.E.: 20/80. On examination, his upper eyelids were floppy and easily everted with gentle upward traction (Fig. 3, left). He had bilateral papillary
Fig. 3 (Goldberg and associates). Case Right, Postoperatively.
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September, 1986
conjunctivitis. The corneas showed global punctate flurrescein staining and surface irregularity. We found pannus formation and deep vascularization as well as stromal scarring; the findings were more advanced in the left eye (Fig. 4). Schirmer strips showed 20 mm of wetting in the right eye and 25 mm in the left eye. Cytologic, bacterial, and chlamydia I cultures were negative, as were serologic tests for syphilis. Floppy eyelid syndrome was diagnosed. The patient elected to undergo bilateral upper eyelid horizontal shortenings. Pentagonal 5-mm full-thickness resections were taken from the lateral third of both upper eyelids, followed by layered closure. Postoperatively, the patient's eyelids showed improved tone and no longer everted with gentle upward traction (Fig. 3, right). The patient reported symptomatic improvement, and his corneal findings stabilized with improvement of the surface irregularities. Histopathologic analysis of resected eyelid tissue disclosed chronic conjunctival inflammation with normal tarsal architecture (Fig. 5). Electron microscopy showed normal ultrastructure of the tarsal collagen. Case 3 A 50-year-old man had a one-year history of irritative ocular symptoms along with complaints of nocturnal eyelid eversion on the right side. His symptoms were unresponsive to lubricants, nocturnal taping of the eyelids, and topical antibiotics. His medical history was unremarkable. Corrected visual acuity was R.E.: 20120 and 1. E.: 20/80 (this eye had long-
Left, Preoperatively upper eyelids are floppy and easily everted.
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Floppy Eyelid Syndrome and Blepharochalasis
Fig. 4 (Goldberg and associates). Case 2. Cornea shows stromal vascularization as well as marked surface irregularities.
standing amblyopia). His eyelids showed loss of elasticity, and were easily everted on gentle upward traction (Fig. 6), and he showed bilateral eyebrow and eyelid ptosis and lacrimal gland prolapse. Slit-lamp examination disclosed papillary conjunctivitis, with early vascular pannus formation superiorly, worse on the right side. A pterygium was noted on the right. Bacterial and chlamydial cultures were negative and a serologic test for syphilis was nonreactive. The patient underwent right upper eyelid horizontal shortening, with full-thickness pentagonal block resection followed by formation and resuspension of a lateral tarsal tongue (Fig. 7). The lower eyelid was shortened horizontally via lateral canthal resuspension. Postoperatively, the patient's eyelids no longer everted with gentle upward traction. His irritative symptoms improved, and there was stabilization of the corneal findings with resolution of the papillary conjunctivitis. Histopathologic evaluation of full-thickness resected eyelid tissue disclosed chronic conjunctival inflammation, with normal tarsal collagen on routine as well as elastin stains.
379
Fig. 5 (Goldberg and associates). Case 2. Tarsal conjunctiva showing chronic inflammatory cell infiltrate with normal underlying tarsal collagen (hematoxylin and eosin, x 40). pointed out, the corneal staining patterns in floppy eyelid syndrome typically involve the entire cornea rather than displaying the inferior pattern associated with nocturnal lagophthalmos. Also, there is not a one-to-one correspondence between the side with keratoconjunctivitis and the preferred side for sleeping. In Case 2, for example, the patient slept only on his right side but he had bilateral corneal changes that were worse on the left side. Also, many reported cases did not include nocturnal eyelid eversion. Perhaps a more probable explanation is poor contact between the lax upper eyelid and the globe, accounting for the corneal changes in the floppy eyelid syndrome, as suggested by Schwartz, Gelender, and Forster." This mechanism might also account for the ocular surface
Discussion The pathogenesis of the keratoconjunctivitis observed in the floppy eyelid syndrome is probably multifactorial, but clinical evidence for the importance of eyelid eversion during sleep is not overwhelming. As Parunovic-
Fig. 6 (Goldberg and associates). Case 3. Preoperatively upper eyelids are floppy and easily everted and there is generalized laxity of eyelid support structures.
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AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 7 (Goldberg and associates). Case 3. Surgical photographs. Top, Full-thickness pentagonal wedge excision of lateral eyelid tissue. Bottom, Layered closure with formation and resuspension of tarsal tongue.
changes in blepharochalasis. The eyelid depends on firm, even contact with the globe to provide a proper wiping effect upon the tear film. It is postulated that this mechanism is rendered ineffective by the lax eyelid, resulting in an inadequate tear film, poor corneal wetting, and subsequent corneal and conjunctival compromise. This mechanism explains why surgical eyelid tightening is successful in cases recalcitrant to conservative treatment. It also provides an explanation for the findings of Liu and Stasior" that lower eyelid length showed a positive correlation with symptoms of ocular irritation. Floppy eyelid syndrome and blepharochalasis in these three cases shared several clinical and pathologic features. Although only the patient with blepharochalasis reported recurrent episodes of eyelid edema, all three had similar findings in regard to eyelid laxity and loss of elasticity. All three patients had upper
eyelids that were easily everted, or floppy. All showed papillary conjunctivitis, poor-quality tear film, and keratitis; ocular surface abnormalities are a diagnostic component of floppy eyelid syndrome but have not previously been mentioned in association with blepharochalasis. The resected eyelid tissue showed similar nonspecific histopathologic changes in all three cases. The literature, also, suggests a possible relationship between floppy eyelid syndrome and blepharochalasis. Tapaszto, Liszkay, and Vass" include a photograph of a patient with blepharochalasis whose upper eyelid everted in a configuration typical of floppy eyelid syndrome. Additionally, several case reports of floppy eyelid syndrome mentioned dermatochalasis of the upper eyelids.P and recurrent swelling was noted in one case." Thus, floppy eyelid syndrome and blepharochalasis share many important clinical characteristics. Ocular surface abnormalities with keratoconjunctivitis can be present in both syndromes, and may be secondary to disruption of the normal firm interface between the eyelid and the globe. Surgical horizontal shortening appears to treat the keratoconjunctivitis effectively by correcting the eyelid laxity and restoring the normal mechanism of the eyelids. It is our conclusion that these two syndromes in their final stages are clinically very similar and structurally and histopathologically identical. The eyelids and supporting structures, when subjected to chronic significant inflammation, follow a course of generalized atrophy and attenuation of muscles, tendons, and ligaments. We propose that late-stage blepharochalasis and advanced floppy eyelid syndrome definitely represent the results of a "final common pathway" process. Possibly these two syndromes are inflammatory processes that are part of a single spectrum of disease. Both syndromes respond to the same surgical management. A careful clinical history is paramount in the diagnosis of these disorders.
References 1. Culbertson, W. W., and Ostler, H. B.: The floppy eyelid syndrome. Am. J. Ophthalmol. 92:568, 1981. 2. Parunovic, A.: Floppy eyelid syndrome. Br. J. Ophthalmol. 67:264, 1983. 3. Schwartz, 1. K., Gelender, H., and Forster,
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Floppy Eyelid Syndrome and Blepharochalasis
R. K.: Chronic conjunctivitis associated with "floppy eyelids." Arch. Ophthalmol. 101:1884, 1983. 4. Gerner, E. W., and Hughes, S. M.: Floppy eyelid with hyperglycinemia. Am. J. Ophthalmol. 98:614, 1984. 5. Stanbury, J.: Metabolic Basis of Inherited Disease, 5th ed. New York, McGraw-Hill, 1983, pp. 561-565. 6. Moore, M. B., Harrington, J., and McCulley, J. P.: Floppy eyelid syndrome. Management including surgery. Ophthalmology 93:184, 1986. 7. Huber-Spitzy, V., Grabner, G., and Stur, M.: Das Syndrom des schlaffen Oberlides (Floppy Eyelid-Syndrom). Klin. Monatsbl. Augenheilkd. 185:289, 1984. 8. Dutton, J. J.: Surgical management of floppy eyelid syndrome. Am. J. Ophthalmol. 99:557, 1985. 9. Alvis, B. Y.: Blepharochalasis. Report of a case. Am. J. Ophthalmol. 18:238, 1935. 10. Fuchs, E.: Uber Blepharochalasis (Erschlaffungder Lidhaut). Wien. Klin. Wochenschr. 9:1,09, 1896. 11. Brazin, S., Stern, L., and Johnson, W.: Unilateral blepharochalasis. Arch. Dermatol. 115:479, 1979. 12. Stieren, E.: Blepharochalasis. Report of two cases. Ann. Ophthalmol. 23:625, 1914. 13. Collin, J. R. 0., Beard, c., Stern, W. H., and Schoengarth, D.: Blepharochalasis. Br. J. Ophthalmol. 63:542, 1979. 14. Custer, P. L., Tenzel, R. R., and Kowalczyk, A. P.: Blepharochalasis syndrome. Am. J. Ophthalmol. 99:424, 1985. 15. Panneton, P.: Le blephatochalazis-i-a propos de 51 cas dans une merne famille. Arch. Ophtalmol. 53:729, 1936. 16. Schulze, F.: Beitrag zur hereditaren Blepharochalasis. Klin. Monatsbl. Augenheilkd. 147.863, 1965.
381
17. Ascher, K. W.: Blepharochalasis mit Struma und Schleimhautduplicatur der Oberlippe. Klin. Monatsbl. Augenheilkd. 65:86, 1920. 18. Eisenstodt, L. W.: Blepharochalasis with double upper lip. Am. J. Ophthalmol. 32:128, 1949. 19. Segal, P., and [ablonska, S.: Le syndrome D' Ascher. Ann. Oculist. 194:511, 1961. W. J., Stepniak, R., and 20. Orlowski, Zwierzchowski, R.: Le syndrome de Laffer-Ascher etude clinique et pathogenique. Ann. Oculist. 196:362, 1963. 21. Tapaszto, 1., Liszkay, L., and Vass, Z.: Some data on the pathogenesis of blepharochalasis. Acta Ophthalmol. 41:167, 1963. 22. Malbran, J.: Einseitige Liihmung der Hebemuskeln und das Laffer-Ascher-Syndrom. Klin. Monatsbl. Augenheilkd. 155:597, 1969. 23. Miihlendyck, H., and Hundeiker, M.: Blepharochalasis (Fuchs) und Laffer-Ascher-Syndrom. Hautarzt 29:474, 1978. 24. Ghose, S., Balwant, R. K., and Yogeshwar, D.: Blepharochalasis with multiple systemic involvement. Br. J. Ophthalmol. 68:529, 1984. 25. Weidler, W. B.: Blepharochalasis. Report of two cases with the microscopic evaluation. J.A.M.A. 61:1128, 1913. 26. Verhoeff, F. H., and Friedenwald, J. S.: Blepharochalasis. Arch. Ophthalmol. 51:554, 1922. 27. Stieglitz, L. N., and Crawford, J. S.: Blepharochalasis. Am. J. Ophthalmol. 77:100, 1974. 28. Liu, D., and Stasior, O. G.: Lower eyelid laxity and ocular symptoms. Am. J. Ophthalmol. 95:545, 1983. 29. Kettesy, A.: Blepharochalasis. Foramen, Ursachen, Operation. Klin. Monatsbl. Augenheilkd. 156:318, 1970. 30. Finney, J., and Peterson, H.: Blepharochalasis after a bee sting. Plast. Reconstr. Surg. 73:830, 1984.
Accepted for publication June 16, 1986. From the Jules Stein Eye Institute, UCLA School of Medicine, Los Angeles, California. Reprint requests to Norman Shorr, M.D., 435 N. Roxbury Dr., Beverly Hills, CA 90210.
376
appearance of the tarsal collagen has been normal in all reported cases. Conservative treatment, including lubrication and patching, has met with variable suecess. l . 3,6,7 Early reports of surgical treatment with eyelid horizontal shortening procedures have been more encouraging.v" Blepharochalasis was described as early as 1807 according to Alvis," but Fuchs" is credited with naming the syndrome and providing a unifying description in the literature. Blepharochalasis is characterized by recurrent episodes of painless nonpitting edema of the upper eyelids, generally beginning around the time of puberty. The lower eyelids are occasionally affected and unilateral cases have been reported." The episodic edema eventually results in permanent eyelid changes characterized by redundant thinning of the skin, discoloration, telangiectasis, and atrophic relaxation of the eyelid support structures. Levator aponeurosis relaxation or dehiscence is frequent in the atrophic stage, leading to blepharoptosis. Prolapse of orbital fat and lacrimal tissue can occur; Stieren" recognized the prolapse as a consequence of atrophic changes in the orbital septum, refuting the earlier concept that this "ptosis adiposa" represented a separate form of the disease. Recent reports have drawn attention to pseudoepicanthal fold formation" and acquired blepharophimosis" as additional manifestations of the atrophic changes that characterize the late stages of blepharochalasis. Although early investigators suspected a female sex preference, Alvis" found equal numbers of males and females in his tabulation of reported cases. Recent case reports also suggest an equal sex incidence (Table). Familial cases of blepharochalasis have been reported, showing autosomal dominant inherirance.P'" An association with thickened double upper lip and variably with goiter (the Laffer-Ascher syndrome) has been described.F'" There have been scattered reports of blepharochalasis occurring in individuals with systemic disease, including a recent case," but no unifying pattern has emerged.
©AMERICAN JOURNAL OF OPHTHALMOLOGY
102:376-381,
SEPTEMBER,
1986
Vol. 102, No.3
Floppy Eyelid Syndrome and Blepharochalasis
TABLE SEX DISTRIBUTION IN CASES OF BLEPHAROCHALASIS REPORTED SINCE 1970
377
one case of blepharochalasis lead us to suggest an end-stage and possibly a primary relationship between these two conditions.
SEX DISTRIBUTION INVESTIGATORS
YEAR
MALE
FEMALE
Kett esy29 Steiglitz and Crawford 27
1970 1974 1978 1979 1979 1984 1984 1985
2 1 0 3 0 0 1
0 2 1 1 1 1 0 ?*
MOthlendyck and Hundeiker 23 Collin and associates 13 Brazin, Stern, and Johnson" Finney and 'Peterson3O Ghose, Balwant, and Yogeshwar24 Custer, Tenzyl, and Kowalcyzk'"
?*
'Not specified.
Histopathologic investigations of eyelid tissue have shown thinning of the skin and variable degrees of disorganization of elastic tissue. 9,l1 ,12,14,16,19,21,25,26 Increased vascularity is uniformly noted, and chronic inflammation is generally observed. The results of medical therapy have been disappointing. Many treatments, including antihistamines and corticosteroids, have been tried through the years but none have been effective. A surgical approach, involving excision of excess skin along with horizontal shortening and blepharoptosis repair, is generally recommended. Several investigators pointed out the need to postpone surgery until the disease is quiescent. 13,25,27 Overcorrection of blepharoptosis is not infrequent. 14,27 The clinical and pathologic similarities between two cases of floppy eyelid syndrome and
Case Reports Case 1 A 56-year-old man had had recurrent eyelid swelling during his teen-age years. He had undergone blepharoptosis repair several times in early adulthood, with only temporary effect. In 1984 he had bilateral blepharoptosis, atrophic upper and lower eyelids bilaterally, and prolapsing lacrimal glands that were worse on the left side (Fig. I, left). He complained of irritative ocular symptoms. Visual acuity was 20/25 in both eyes. Slit-lamp examination disclosed poor-quality tear film and papillary conjunctivitis. Schirmer testing showed 30 mm of wetting bilaterally. The patient was thought to have quiescent blepharochalasis. On initial surgical approach, dehiscence of the levator aponeurosis and lateral canthal tendon attenuation were found bilaterally. The levator dehiscence was repaired, but residual blepharoptosis required subsequent reinforcement with full-thickness upper eyelid resections bilaterally. The patient also underwent horizontal shortening of the upper and lower eyelids with canthal resuspension. Postoperatively, his irritative ocular symptoms and corneal surface abnormalities were noted to improve (Fig. I, right). Histopathologic analysis of resected eyelid tissue showed chronic inflammation of the tarsal conjunctiva; routine and elastin stains
Fig. 1 (Goldberg and associates). Case 1. Left, Preoperatively there is laxity of eyelids and supporting structures, with blepharoptosis and lacrimal gland prolapse. Right, Postoperatively.
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AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 2 (Goldberg and associates). Case 1. Conjunctiva showing chronic inflammatory cell infiltrate with normal underlying tarsal collagen (hematoxylin and eosin, x 40).
showed no abnormalities of tarsal collagen or elastic tissue (Fig. 2). Case 2 A markedly overweight 39-year-old man with diabetes and hypertension had a IS-year history of irritative ocular symptoms. He slept on his right side and was unaware of nocturnal eyelid eversion. There was no history or symptoms of connective tissue or metabolic disorders, and his family history was unremarkable. He had been treated with lubricants, patching, antibiotics, and topical corticosteroids without improvement. Best corrected visual acuity was R.E.: 20/40 and L.E.: 20/80. On examination, his upper eyelids were floppy and easily everted with gentle upward traction (Fig. 3, left). He had bilateral papillary
Fig. 3 (Goldberg and associates). Case Right, Postoperatively.
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September, 1986
conjunctivitis. The corneas showed global punctate flurrescein staining and surface irregularity. We found pannus formation and deep vascularization as well as stromal scarring; the findings were more advanced in the left eye (Fig. 4). Schirmer strips showed 20 mm of wetting in the right eye and 25 mm in the left eye. Cytologic, bacterial, and chlamydia I cultures were negative, as were serologic tests for syphilis. Floppy eyelid syndrome was diagnosed. The patient elected to undergo bilateral upper eyelid horizontal shortenings. Pentagonal 5-mm full-thickness resections were taken from the lateral third of both upper eyelids, followed by layered closure. Postoperatively, the patient's eyelids showed improved tone and no longer everted with gentle upward traction (Fig. 3, right). The patient reported symptomatic improvement, and his corneal findings stabilized with improvement of the surface irregularities. Histopathologic analysis of resected eyelid tissue disclosed chronic conjunctival inflammation with normal tarsal architecture (Fig. 5). Electron microscopy showed normal ultrastructure of the tarsal collagen. Case 3 A 50-year-old man had a one-year history of irritative ocular symptoms along with complaints of nocturnal eyelid eversion on the right side. His symptoms were unresponsive to lubricants, nocturnal taping of the eyelids, and topical antibiotics. His medical history was unremarkable. Corrected visual acuity was R.E.: 20120 and 1. E.: 20/80 (this eye had long-
Left, Preoperatively upper eyelids are floppy and easily everted.
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Floppy Eyelid Syndrome and Blepharochalasis
Fig. 4 (Goldberg and associates). Case 2. Cornea shows stromal vascularization as well as marked surface irregularities.
standing amblyopia). His eyelids showed loss of elasticity, and were easily everted on gentle upward traction (Fig. 6), and he showed bilateral eyebrow and eyelid ptosis and lacrimal gland prolapse. Slit-lamp examination disclosed papillary conjunctivitis, with early vascular pannus formation superiorly, worse on the right side. A pterygium was noted on the right. Bacterial and chlamydial cultures were negative and a serologic test for syphilis was nonreactive. The patient underwent right upper eyelid horizontal shortening, with full-thickness pentagonal block resection followed by formation and resuspension of a lateral tarsal tongue (Fig. 7). The lower eyelid was shortened horizontally via lateral canthal resuspension. Postoperatively, the patient's eyelids no longer everted with gentle upward traction. His irritative symptoms improved, and there was stabilization of the corneal findings with resolution of the papillary conjunctivitis. Histopathologic evaluation of full-thickness resected eyelid tissue disclosed chronic conjunctival inflammation, with normal tarsal collagen on routine as well as elastin stains.
379
Fig. 5 (Goldberg and associates). Case 2. Tarsal conjunctiva showing chronic inflammatory cell infiltrate with normal underlying tarsal collagen (hematoxylin and eosin, x 40). pointed out, the corneal staining patterns in floppy eyelid syndrome typically involve the entire cornea rather than displaying the inferior pattern associated with nocturnal lagophthalmos. Also, there is not a one-to-one correspondence between the side with keratoconjunctivitis and the preferred side for sleeping. In Case 2, for example, the patient slept only on his right side but he had bilateral corneal changes that were worse on the left side. Also, many reported cases did not include nocturnal eyelid eversion. Perhaps a more probable explanation is poor contact between the lax upper eyelid and the globe, accounting for the corneal changes in the floppy eyelid syndrome, as suggested by Schwartz, Gelender, and Forster." This mechanism might also account for the ocular surface
Discussion The pathogenesis of the keratoconjunctivitis observed in the floppy eyelid syndrome is probably multifactorial, but clinical evidence for the importance of eyelid eversion during sleep is not overwhelming. As Parunovic-
Fig. 6 (Goldberg and associates). Case 3. Preoperatively upper eyelids are floppy and easily everted and there is generalized laxity of eyelid support structures.
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AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 7 (Goldberg and associates). Case 3. Surgical photographs. Top, Full-thickness pentagonal wedge excision of lateral eyelid tissue. Bottom, Layered closure with formation and resuspension of tarsal tongue.
changes in blepharochalasis. The eyelid depends on firm, even contact with the globe to provide a proper wiping effect upon the tear film. It is postulated that this mechanism is rendered ineffective by the lax eyelid, resulting in an inadequate tear film, poor corneal wetting, and subsequent corneal and conjunctival compromise. This mechanism explains why surgical eyelid tightening is successful in cases recalcitrant to conservative treatment. It also provides an explanation for the findings of Liu and Stasior" that lower eyelid length showed a positive correlation with symptoms of ocular irritation. Floppy eyelid syndrome and blepharochalasis in these three cases shared several clinical and pathologic features. Although only the patient with blepharochalasis reported recurrent episodes of eyelid edema, all three had similar findings in regard to eyelid laxity and loss of elasticity. All three patients had upper
eyelids that were easily everted, or floppy. All showed papillary conjunctivitis, poor-quality tear film, and keratitis; ocular surface abnormalities are a diagnostic component of floppy eyelid syndrome but have not previously been mentioned in association with blepharochalasis. The resected eyelid tissue showed similar nonspecific histopathologic changes in all three cases. The literature, also, suggests a possible relationship between floppy eyelid syndrome and blepharochalasis. Tapaszto, Liszkay, and Vass" include a photograph of a patient with blepharochalasis whose upper eyelid everted in a configuration typical of floppy eyelid syndrome. Additionally, several case reports of floppy eyelid syndrome mentioned dermatochalasis of the upper eyelids.P and recurrent swelling was noted in one case." Thus, floppy eyelid syndrome and blepharochalasis share many important clinical characteristics. Ocular surface abnormalities with keratoconjunctivitis can be present in both syndromes, and may be secondary to disruption of the normal firm interface between the eyelid and the globe. Surgical horizontal shortening appears to treat the keratoconjunctivitis effectively by correcting the eyelid laxity and restoring the normal mechanism of the eyelids. It is our conclusion that these two syndromes in their final stages are clinically very similar and structurally and histopathologically identical. The eyelids and supporting structures, when subjected to chronic significant inflammation, follow a course of generalized atrophy and attenuation of muscles, tendons, and ligaments. We propose that late-stage blepharochalasis and advanced floppy eyelid syndrome definitely represent the results of a "final common pathway" process. Possibly these two syndromes are inflammatory processes that are part of a single spectrum of disease. Both syndromes respond to the same surgical management. A careful clinical history is paramount in the diagnosis of these disorders.
References 1. Culbertson, W. W., and Ostler, H. B.: The floppy eyelid syndrome. Am. J. Ophthalmol. 92:568, 1981. 2. Parunovic, A.: Floppy eyelid syndrome. Br. J. Ophthalmol. 67:264, 1983. 3. Schwartz, 1. K., Gelender, H., and Forster,
Vol. 102, No.3
Floppy Eyelid Syndrome and Blepharochalasis
R. K.: Chronic conjunctivitis associated with "floppy eyelids." Arch. Ophthalmol. 101:1884, 1983. 4. Gerner, E. W., and Hughes, S. M.: Floppy eyelid with hyperglycinemia. Am. J. Ophthalmol. 98:614, 1984. 5. Stanbury, J.: Metabolic Basis of Inherited Disease, 5th ed. New York, McGraw-Hill, 1983, pp. 561-565. 6. Moore, M. B., Harrington, J., and McCulley, J. P.: Floppy eyelid syndrome. Management including surgery. Ophthalmology 93:184, 1986. 7. Huber-Spitzy, V., Grabner, G., and Stur, M.: Das Syndrom des schlaffen Oberlides (Floppy Eyelid-Syndrom). Klin. Monatsbl. Augenheilkd. 185:289, 1984. 8. Dutton, J. J.: Surgical management of floppy eyelid syndrome. Am. J. Ophthalmol. 99:557, 1985. 9. Alvis, B. Y.: Blepharochalasis. Report of a case. Am. J. Ophthalmol. 18:238, 1935. 10. Fuchs, E.: Uber Blepharochalasis (Erschlaffungder Lidhaut). Wien. Klin. Wochenschr. 9:1,09, 1896. 11. Brazin, S., Stern, L., and Johnson, W.: Unilateral blepharochalasis. Arch. Dermatol. 115:479, 1979. 12. Stieren, E.: Blepharochalasis. Report of two cases. Ann. Ophthalmol. 23:625, 1914. 13. Collin, J. R. 0., Beard, c., Stern, W. H., and Schoengarth, D.: Blepharochalasis. Br. J. Ophthalmol. 63:542, 1979. 14. Custer, P. L., Tenzel, R. R., and Kowalczyk, A. P.: Blepharochalasis syndrome. Am. J. Ophthalmol. 99:424, 1985. 15. Panneton, P.: Le blephatochalazis-i-a propos de 51 cas dans une merne famille. Arch. Ophtalmol. 53:729, 1936. 16. Schulze, F.: Beitrag zur hereditaren Blepharochalasis. Klin. Monatsbl. Augenheilkd. 147.863, 1965.
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17. Ascher, K. W.: Blepharochalasis mit Struma und Schleimhautduplicatur der Oberlippe. Klin. Monatsbl. Augenheilkd. 65:86, 1920. 18. Eisenstodt, L. W.: Blepharochalasis with double upper lip. Am. J. Ophthalmol. 32:128, 1949. 19. Segal, P., and [ablonska, S.: Le syndrome D' Ascher. Ann. Oculist. 194:511, 1961. W. J., Stepniak, R., and 20. Orlowski, Zwierzchowski, R.: Le syndrome de Laffer-Ascher etude clinique et pathogenique. Ann. Oculist. 196:362, 1963. 21. Tapaszto, 1., Liszkay, L., and Vass, Z.: Some data on the pathogenesis of blepharochalasis. Acta Ophthalmol. 41:167, 1963. 22. Malbran, J.: Einseitige Liihmung der Hebemuskeln und das Laffer-Ascher-Syndrom. Klin. Monatsbl. Augenheilkd. 155:597, 1969. 23. Miihlendyck, H., and Hundeiker, M.: Blepharochalasis (Fuchs) und Laffer-Ascher-Syndrom. Hautarzt 29:474, 1978. 24. Ghose, S., Balwant, R. K., and Yogeshwar, D.: Blepharochalasis with multiple systemic involvement. Br. J. Ophthalmol. 68:529, 1984. 25. Weidler, W. B.: Blepharochalasis. Report of two cases with the microscopic evaluation. J.A.M.A. 61:1128, 1913. 26. Verhoeff, F. H., and Friedenwald, J. S.: Blepharochalasis. Arch. Ophthalmol. 51:554, 1922. 27. Stieglitz, L. N., and Crawford, J. S.: Blepharochalasis. Am. J. Ophthalmol. 77:100, 1974. 28. Liu, D., and Stasior, O. G.: Lower eyelid laxity and ocular symptoms. Am. J. Ophthalmol. 95:545, 1983. 29. Kettesy, A.: Blepharochalasis. Foramen, Ursachen, Operation. Klin. Monatsbl. Augenheilkd. 156:318, 1970. 30. Finney, J., and Peterson, H.: Blepharochalasis after a bee sting. Plast. Reconstr. Surg. 73:830, 1984.