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Blepharochalasis
BLEPHAROCHALASIS L.
N.
STIEGLITZ, M.D.,
AND J.
S.
CRAWFORD,
M.D.
Toronto, Canada Blepharochalasis, a condition rarely seen by most ophthalmologists, is characterized by permanent changes in the eyelids after re current and unpredictable attacks of edema. T h e skin of the eyelids becomes baggy, wrin kled, sometimes thin, and laced with an in creased number of blood vessels. T h e eyelids may droop. Blepharochalasis is typically bi lateral, and mostly it is the upper eyelids that are involved. In more severe cases, the lower eyelids may also be affected. Herniation of fat may occasionally occur if the orbital sep tum is weakened. T h e cause of blepharochalasis is unknown. Usually it occurs before the age of 20. 1 Be cause many cases begin at puberty, an endo crine cause has been suggested. 2 Nontoxic enlargement of the thyroid occurs occasion ally.1'3"5 In a few patients edema of the up per lip has also occurred at the same time as edema of the eyelids. 4 " 7 N o evidence of al lergy occurred in any of the cases reported. Blepharochalasis is rarely inherited. 8 Fuchs suggested the name blepharochala sis, relaxed eyelid, in 1896. 9 Blepharochala sis must not be confused with dermatochalasis, a common condition found in older per sons in which the eyelids become baggy due to senile atrophy of the skin, or with recurFrom the Department of Ophthalmology, The Hospital for Sick Children, Toronto, Ontario. Reprint requests to J. S. Crawford, M.D., The Hospital for Sick Children, Toronto M5G 1X8, Ontario, Canada.
rent edema of the eyelid, which leaves no permanent change. T h e treatment for blepharochalasis is sur gical, as spontaneous recovery is unknown. T h e redundant eyelid skin and, if necessary, prolapsed fat is excised. In more severe cases, where blepharoptosis has been a prob lem, the levator muscle may be resected. T h e surgical correction of this cosmetic defect can be particularly difficult. C A S E REPORTS
Case 1—A 13-year-old girl had bilateral upper eyelid swelling of one year's duration, intermittent at first, but after four months of recurrent attacks, the eyelids were constantly baggy. The skin of the eyelids was wrinkled and slightly red (Fig. 1, left). Neither she nor her family had any history of al lergy. Results of routine laboratory tests, proteinbound iodine level, and serum-protein electrophoresis were normal. A complement fixation test for Trichinella was negative, and skin testing by the al lergy department also failed to show a cause for the swelling. There was no enlargement of the thyroid. Antihistamines had not relieved the attacks, and prednisone, 5 mg four times a day, reduced the swelling only slightly during the attacks. She underwent bilateral excision of redundant upper eyelid skin at age 13J4, and after this her ap pearance improved. However, the attacks of edema continued with further damage to the tissues (Fig. 1, right). Within a year the eyelids were again baggy, necessitating further surgery. Bilateral skin excisions were again performed, but the long-term result is not yet known. Case 2—A 12-year-old boy had a history of re current attacks of bilateral acute eyelid swelling that began at age 9. The attacks lasted three or four days and occurred as frequently as every two weeks during the winter. They were less frequent
Fig. 1 (Stieglitz and Crawford). Case 1. Left, Blepharochalasis in 13-year-old girl. The upper eyelids have become wrinkled and baggy after one year of recurrent attacks of eyelid edema. Right, Acute attack of eyelid edema, right eye. She had had previous surgery for blepharochalasis. 100
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Fig. 2 (Stieglitz and Crawford). Case 2. Left, At 12 years of age, showing baggy eyelids after three years of recurring eyelid edema. Right, Appearance three years after surgical correction for blepharochalasis. The skin of the upper eyelids is loose and there is a left blepharoptosis. in the summer. During an attack, the swelling was so great his eyes would be closed. Permanent changes in the eyelids occurred within a year after the onset of the first attack. The eyelids became baggy, and the number of blood vessels increased giving the upper eyelids a red appearance (Fig. 2, left). No cause was found, and medical treatment, which included antihistamines and 10 mg of prednisone three times a day during the attacks, was inef fective. His appearance improved with removal of an el lipse of skin from both upper eyelids, but the at tacks of edema continued, the first within a month of surgery, and further changes in the eyelids followed. Now, three years later, a serious cosmetic problem persists; the skin of the eyelids is again loose and the vessels are even more prominent. H e also has a left blepharoptosis (Fig. 2, right). The
attacks of edema have continued although they hav'e become less frequent recently. Case 3—By the time she was 10 years old, this girl already had severe changes in the eyelids and orbital tissues after recurrent attacks of edema. The skin of the eyelids was thin, baggy, wrinkled, and dark, and she had blepharoptosis with reduced levator muscle action. In addition, she had apparent atrophy of orbital fat, which gave her eyes a sunken appearance (Fig. 3, top left). Her first at tack at age 3^2 years followed a tonsillectomy by one day. The attacks were as frequent as every week in the beginning but became less frequent later on. No cause was found. Results of routine labora tory tests, protein-bound iodine, serum-protein electrophoresis, and gamma-globulin levels were all within normal limits. Trichinella complement fixa-
Fig. 3 (Stieglitz and Crawford). Case 3. Top left, Permanent changes in right upper eyelid after recurrent attacks of edema. The skin is thin, dark, and wrinkled, and the eyelid droops. There is also apparent atrophy of orbital fat giving the eye a sunken appearance. Top right, Postoperative picture showing overcorrection of blepharoptosis with ble pharochalasis. Bottom left, Appearance of patient after numerous surgical procedures for correction of blepharochalasis. The left upper eyelid was low ered to remedy overcorrected blepharoptosis. The skin of the eyelids is dark and wrinkled, and further atrophy of the eyelids and orbital tissues has oc curred due to the continuance of attacks of edema.
102
AMERICAN JOURNAL OF OPHTHALMOLOGY
J A N U A R Y , 1974
TABLE C A S E 3 : SURGICAL PROCEDURES FOR CORRECTION OF BLEPHAROCHALASIS
Age, yr 10 10i 18 19
Result
Procedure Left levator muscle resection, 14 mm (through Myotomies, left levator muscle Repeat myotomies, left levator muscle Right levator muscle resection, 4 mm (through Left medial to lateral levator-tarsus graft (for aspect of left upper eyelid) Right upper eyelid skin excision Right levator muscle resection, 8 mm (through
tion test and allergic skin testing were both nega tive. A biopsy taken from the left lower eyelid at age 6 was normal and showed no eosinophilia. She underwent a number of surgical procedures in an attempt to improve her appearance (Table). An overcorrection of her left blepharoptosis re quired three further procedures to achieve a satis factory result, and three procedures were required to correct her right blepharoptosis and to remove excess skin (Fig. 3, top right). In spite of this amount of surgery, she still has a serious problem with further atrophy of the eyelids and orbital tis sues. The skin of the eyelids has become darker, and the vessels are more prominent (Fig. 3, bottom
left).
DISCUSSION
Blepharochalasis remains a disease of un known cause despite the fact that its first de scription appeared in the last century. Be cause it is so rare, there is little information available regarding treatment. Eisenstodt6 reported a case in which excision of excess eyelid skin was performed, but further dam age to the eyelids occurred with the continu ance of attacks of eyelid edema. In all three patients reported in this paper, the continu ance of the disease has made it difficult to obtain good results from surgery. Because the frequency of attacks appears to decrease with age, surgery should perhaps be post poned until after a long quiescent period in the natural course of the disease. Case 3 had special problems because of the difficulty in correcting her blepharoptosis. Overcorrec tion of blepharoptosis can easily occur in blepharochalasis and may be partly due to loss of orbicularis muscle function after the attacks of edema. Blepharoptosis surgery should be left until the episodes of swelling
skin)
Overcorrection
conjunctiva) overcorrection of lateral
Undercorrection
skin)
have stopped, and then a levator muscle re section of about 10 mm through conjunctiva or a modified Fasanella-Servat procedure should be performed.10 SUMMARY
Three patients had blepharochalasis, a rare cosmetic problem. No cause was found in any of the cases. The results of surgical cor rection were unsatisfactory because the dis ease continued to progress. The frequency of attacks decreased with age, and results may be better if surgery is postponed until after a long quiescent period in the natural course of the disease. REFERENCES 1. Alvis, B. Y . : Blepharochalasis. Report of a case. Am. J. Ophthalmol. 18:238, 1935. 2. Weidler, W . B . : Blepharochalasis. J.A.M.A. 61:1128, 1913. 3. Benedict, W. L . : Blepharochalsis. Report of three cases. J.A.M.A. 87:1735, 1926. 4. Ascher, K. W . : Blepharochalasis and struma plus hypertrophy of lip glands. Klin. Wochenschr. 1:2287, 1922. 5. Papanayotou, P . H., and Hatziotis, J. C : Ascher's syndrome. Report of a case. Oral Surg. 35:467, 1973. 6. Eisenstodt, L. W . : Blepharochalasis with dou ble upper lip. Am. J. Ophthalmol. 32:128, 1949. 7. Tapaszto, I., Liszkay, L., and Vass, Z.: Some data on the pathogenesis of blepharochalasis. Acta Ophthalmol. 41:167, 1963. 8. Duke-Elder, S.: The ocular adnexa. In Text book of Ophthalmology, vol. 5. St. Louis, C. V. Mosby, 1952, p. 5001. 9. Fuchs, E . : Ueber Blepharochalasis. Wien. Klin. Wochenschr. 9:109, 1896. 10. Crawford, J. S.: Repair of blepharoptosis with a modification of the Fasanella-Servat opera tion. Can. J. Ophthalmol. 8:19, 1973.
N.
STIEGLITZ, M.D.,
AND J.
S.
CRAWFORD,
M.D.
Toronto, Canada Blepharochalasis, a condition rarely seen by most ophthalmologists, is characterized by permanent changes in the eyelids after re current and unpredictable attacks of edema. T h e skin of the eyelids becomes baggy, wrin kled, sometimes thin, and laced with an in creased number of blood vessels. T h e eyelids may droop. Blepharochalasis is typically bi lateral, and mostly it is the upper eyelids that are involved. In more severe cases, the lower eyelids may also be affected. Herniation of fat may occasionally occur if the orbital sep tum is weakened. T h e cause of blepharochalasis is unknown. Usually it occurs before the age of 20. 1 Be cause many cases begin at puberty, an endo crine cause has been suggested. 2 Nontoxic enlargement of the thyroid occurs occasion ally.1'3"5 In a few patients edema of the up per lip has also occurred at the same time as edema of the eyelids. 4 " 7 N o evidence of al lergy occurred in any of the cases reported. Blepharochalasis is rarely inherited. 8 Fuchs suggested the name blepharochala sis, relaxed eyelid, in 1896. 9 Blepharochala sis must not be confused with dermatochalasis, a common condition found in older per sons in which the eyelids become baggy due to senile atrophy of the skin, or with recurFrom the Department of Ophthalmology, The Hospital for Sick Children, Toronto, Ontario. Reprint requests to J. S. Crawford, M.D., The Hospital for Sick Children, Toronto M5G 1X8, Ontario, Canada.
rent edema of the eyelid, which leaves no permanent change. T h e treatment for blepharochalasis is sur gical, as spontaneous recovery is unknown. T h e redundant eyelid skin and, if necessary, prolapsed fat is excised. In more severe cases, where blepharoptosis has been a prob lem, the levator muscle may be resected. T h e surgical correction of this cosmetic defect can be particularly difficult. C A S E REPORTS
Case 1—A 13-year-old girl had bilateral upper eyelid swelling of one year's duration, intermittent at first, but after four months of recurrent attacks, the eyelids were constantly baggy. The skin of the eyelids was wrinkled and slightly red (Fig. 1, left). Neither she nor her family had any history of al lergy. Results of routine laboratory tests, proteinbound iodine level, and serum-protein electrophoresis were normal. A complement fixation test for Trichinella was negative, and skin testing by the al lergy department also failed to show a cause for the swelling. There was no enlargement of the thyroid. Antihistamines had not relieved the attacks, and prednisone, 5 mg four times a day, reduced the swelling only slightly during the attacks. She underwent bilateral excision of redundant upper eyelid skin at age 13J4, and after this her ap pearance improved. However, the attacks of edema continued with further damage to the tissues (Fig. 1, right). Within a year the eyelids were again baggy, necessitating further surgery. Bilateral skin excisions were again performed, but the long-term result is not yet known. Case 2—A 12-year-old boy had a history of re current attacks of bilateral acute eyelid swelling that began at age 9. The attacks lasted three or four days and occurred as frequently as every two weeks during the winter. They were less frequent
Fig. 1 (Stieglitz and Crawford). Case 1. Left, Blepharochalasis in 13-year-old girl. The upper eyelids have become wrinkled and baggy after one year of recurrent attacks of eyelid edema. Right, Acute attack of eyelid edema, right eye. She had had previous surgery for blepharochalasis. 100
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101
Fig. 2 (Stieglitz and Crawford). Case 2. Left, At 12 years of age, showing baggy eyelids after three years of recurring eyelid edema. Right, Appearance three years after surgical correction for blepharochalasis. The skin of the upper eyelids is loose and there is a left blepharoptosis. in the summer. During an attack, the swelling was so great his eyes would be closed. Permanent changes in the eyelids occurred within a year after the onset of the first attack. The eyelids became baggy, and the number of blood vessels increased giving the upper eyelids a red appearance (Fig. 2, left). No cause was found, and medical treatment, which included antihistamines and 10 mg of prednisone three times a day during the attacks, was inef fective. His appearance improved with removal of an el lipse of skin from both upper eyelids, but the at tacks of edema continued, the first within a month of surgery, and further changes in the eyelids followed. Now, three years later, a serious cosmetic problem persists; the skin of the eyelids is again loose and the vessels are even more prominent. H e also has a left blepharoptosis (Fig. 2, right). The
attacks of edema have continued although they hav'e become less frequent recently. Case 3—By the time she was 10 years old, this girl already had severe changes in the eyelids and orbital tissues after recurrent attacks of edema. The skin of the eyelids was thin, baggy, wrinkled, and dark, and she had blepharoptosis with reduced levator muscle action. In addition, she had apparent atrophy of orbital fat, which gave her eyes a sunken appearance (Fig. 3, top left). Her first at tack at age 3^2 years followed a tonsillectomy by one day. The attacks were as frequent as every week in the beginning but became less frequent later on. No cause was found. Results of routine labora tory tests, protein-bound iodine, serum-protein electrophoresis, and gamma-globulin levels were all within normal limits. Trichinella complement fixa-
Fig. 3 (Stieglitz and Crawford). Case 3. Top left, Permanent changes in right upper eyelid after recurrent attacks of edema. The skin is thin, dark, and wrinkled, and the eyelid droops. There is also apparent atrophy of orbital fat giving the eye a sunken appearance. Top right, Postoperative picture showing overcorrection of blepharoptosis with ble pharochalasis. Bottom left, Appearance of patient after numerous surgical procedures for correction of blepharochalasis. The left upper eyelid was low ered to remedy overcorrected blepharoptosis. The skin of the eyelids is dark and wrinkled, and further atrophy of the eyelids and orbital tissues has oc curred due to the continuance of attacks of edema.
102
AMERICAN JOURNAL OF OPHTHALMOLOGY
J A N U A R Y , 1974
TABLE C A S E 3 : SURGICAL PROCEDURES FOR CORRECTION OF BLEPHAROCHALASIS
Age, yr 10 10i 18 19
Result
Procedure Left levator muscle resection, 14 mm (through Myotomies, left levator muscle Repeat myotomies, left levator muscle Right levator muscle resection, 4 mm (through Left medial to lateral levator-tarsus graft (for aspect of left upper eyelid) Right upper eyelid skin excision Right levator muscle resection, 8 mm (through
tion test and allergic skin testing were both nega tive. A biopsy taken from the left lower eyelid at age 6 was normal and showed no eosinophilia. She underwent a number of surgical procedures in an attempt to improve her appearance (Table). An overcorrection of her left blepharoptosis re quired three further procedures to achieve a satis factory result, and three procedures were required to correct her right blepharoptosis and to remove excess skin (Fig. 3, top right). In spite of this amount of surgery, she still has a serious problem with further atrophy of the eyelids and orbital tis sues. The skin of the eyelids has become darker, and the vessels are more prominent (Fig. 3, bottom
left).
DISCUSSION
Blepharochalasis remains a disease of un known cause despite the fact that its first de scription appeared in the last century. Be cause it is so rare, there is little information available regarding treatment. Eisenstodt6 reported a case in which excision of excess eyelid skin was performed, but further dam age to the eyelids occurred with the continu ance of attacks of eyelid edema. In all three patients reported in this paper, the continu ance of the disease has made it difficult to obtain good results from surgery. Because the frequency of attacks appears to decrease with age, surgery should perhaps be post poned until after a long quiescent period in the natural course of the disease. Case 3 had special problems because of the difficulty in correcting her blepharoptosis. Overcorrec tion of blepharoptosis can easily occur in blepharochalasis and may be partly due to loss of orbicularis muscle function after the attacks of edema. Blepharoptosis surgery should be left until the episodes of swelling
skin)
Overcorrection
conjunctiva) overcorrection of lateral
Undercorrection
skin)
have stopped, and then a levator muscle re section of about 10 mm through conjunctiva or a modified Fasanella-Servat procedure should be performed.10 SUMMARY
Three patients had blepharochalasis, a rare cosmetic problem. No cause was found in any of the cases. The results of surgical cor rection were unsatisfactory because the dis ease continued to progress. The frequency of attacks decreased with age, and results may be better if surgery is postponed until after a long quiescent period in the natural course of the disease. REFERENCES 1. Alvis, B. Y . : Blepharochalasis. Report of a case. Am. J. Ophthalmol. 18:238, 1935. 2. Weidler, W . B . : Blepharochalasis. J.A.M.A. 61:1128, 1913. 3. Benedict, W. L . : Blepharochalsis. Report of three cases. J.A.M.A. 87:1735, 1926. 4. Ascher, K. W . : Blepharochalasis and struma plus hypertrophy of lip glands. Klin. Wochenschr. 1:2287, 1922. 5. Papanayotou, P . H., and Hatziotis, J. C : Ascher's syndrome. Report of a case. Oral Surg. 35:467, 1973. 6. Eisenstodt, L. W . : Blepharochalasis with dou ble upper lip. Am. J. Ophthalmol. 32:128, 1949. 7. Tapaszto, I., Liszkay, L., and Vass, Z.: Some data on the pathogenesis of blepharochalasis. Acta Ophthalmol. 41:167, 1963. 8. Duke-Elder, S.: The ocular adnexa. In Text book of Ophthalmology, vol. 5. St. Louis, C. V. Mosby, 1952, p. 5001. 9. Fuchs, E . : Ueber Blepharochalasis. Wien. Klin. Wochenschr. 9:109, 1896. 10. Crawford, J. S.: Repair of blepharoptosis with a modification of the Fasanella-Servat opera tion. Can. J. Ophthalmol. 8:19, 1973.