Congenital Entropion With Intact Lower Eyelid Retractor Insertion George B. Bartley, M.D., Jeffrey A. Nerad, M.D., Robert C. Kersten, M.D., and Leo J. Maguire, M.D.
Congenital lower eyelid entropion is generally considered to result from improper development of the retractor aponeurosis insertion to the inferior portion of the tarsal plate. We treated three patients with this uncommon disorder. At operation, aponeurotic defects were anticipated and specifically sought, but in each case the lower eyelid retractors were inserted normally. In two patients, entropion was relieved by surgical disinsertion and then advancement of the retractors. In the third patient, who also had multiple concomitant facial and systemic developmental anomalies, improvement in the lower eyelid malpositions required a combination of procedures. The intraoperative findings in our patients demonstrate that disinsertion of the lower eyelid retractors is not a universal etiologic mechanism in congenital entropion.
T RUE CONGENITAL lower eyelid entropion is uncommon; at one eye clinic only a single case was observed among 160,000 patients over a 25-year period.' Epiblepharon, in contrast, is common, especially in Japanese children, 24% of whom have the condition at the age of 1 year.! Differentiation between the two disorders is important. Marked corneal irritation is infrequent with epiblepharon, which typically Accepted for publication July 3, 1991. From the Department of Ophthalmology (Drs. Bartley and Maguire), Mayo Clinic and Mayo Foundation, Rochester, Minnesota; Department of Ophthalmology, University of Iowa, Iowa City, Iowa (Dr. Nerad); and Department of Ophthalmology, University of Cincinnati Hospital, Cincinnati, Ohio (Dr. Kersten). This study was supported by an unrestricted grant from Research to Prevent Blindness, Inc. Reprint requests to George B. Bartley, M.D., Department of Ophthalmology, Mayo Clinic, 200 First St. S.W., Rochester, MN 55905.
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resolves spontaneously as the child's face develops. Congenital entropion, however, often requires prompt surgical intervention to prevent potentially blinding corneal scarring. The putative etiologic mechanism for congenital lower eyelid entropion is improper development of the retractor aponeurosis insertion. 3•4 We treated three patients with this uncommon eyelid malposition. At operation, aponeurotic defects were anticipated and specifically sought, but in each case the lower eyelid retractors were inserted normally.
Case Reports Case 1 A male infant was born after a normal 40week gestation; the vaginal delivery was uncomplicated. Matter from both eyes was observed on the first postpartum day, and erythro-
Fig. 1 (Bartley and associates). Case 1. Congenital entropion of left lower eyelid with a secondary corneal ulcer in a 3-week-old male infant.
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Fig. 2 (Bartley and associates). Case 1. The orbital septum (held by forceps) has been dissected from the underlying capsulopalpebral fascia. The lower eyelid retractors were intact. The arrow identifies preaponeurotic fat.
mycin ointment was prescribed by a pediatrician. Inflammation of the right eye quickly resolved, but discharge from the left eye persisted. A white opacity was apparent on the left cornea at 2 weeks of age, and the child was referred to an ophthalmologist. A corneal ulcer was diagnosed, scrapings were taken for cul-
Fig. 4 (Bartley and associates). Case 1. A minimal ectropion of the eyelid margin was desired to facilitate healing of the corneal ulcer.
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Fig. 3 (Bartley and associates). Case 1. The lower eyelid retractors were surgically disinserted, resected, and reattached to the inferior tarsal edge.
ture (which ultimately were negative), and hourly administration of tobramycin drops and polymyxin B-bacitracin ointment was prescribed. The patient was subsequently examined at 3 weeks of age by one of us (L.].M.), who confirmed the diagnosis of a corneal ulcer and observed that the left lower eyelid was rotated inward against the globe. Later that same day, the eyelid was operated on by one of us (G.B. B.), and the cornea was examined and cultured (Fig. 1). The skin was incised in a faint eyelid crease near the inferior edge of the tarsus. The orbicularis was divided horizontally,
Fig. 5 (Bartley and associates). Case 1. Residual corneal scar and satisfactory eyelid position four months after surgery.
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Fig. 6 (Bartley and associates). Case 2. Congenital bilateral lower eyelid entropion in a 13-month-old girl.
and the orbital septum was dissected from the lower eyelid retractors. An aponeurotic disinsertion was sought, but the insertion of the retractors appeared normal (Fig. 2). Plication of the retractors to the inferior edge of the tarsus with a silk suture caused the eyelid to rotate moderately away from the globe, so the suture was removed. The retractors were surgically disinserted and a small distal portion was resected. The aponeurosis was reattached to the inferior tarsal border with three interrupted 5-0 chromic sutures (Fig. 3). A minimal overcorrection (slight eversion) of the eyelid margin (Fig. 4) was desired to ensure that the cornea would not be abraded, and the ultimate eyelid position was hoped to be normal once the chromic sutures absorbed. After surgery, fortified gentamicin and van-
Fig. 8 (Bartley and associates). Case 2. The lower eyelid retractors were surgically disinserted, resected, and reattached to the inferior tarsal edge.
Fig. 7 (Bartley and associates). Case 2. Abnormally abundant fat that was excised. The lower eyelid retractors were intact.
comycin eye drops were prescribed, and the corneal ulcer healed rapidly. A small corneal scar persisted, but it was inferior to the visual axis. The lower eyelid was in normal position four months after surgery (Fig. 5), and no complications developed during a follow-up of ten months. Case 2 Bilateral lower eyelid entropion was observed by one of us (I.A.N.) in a 10-week-old female infant with a 6-p and 13-q balance translocation. In addition to the eyelid malpositions, the child had congenital glaucoma, nasolacrimal drainage abnormalities, persistent hyaloid vessels, cleft palate, club feet, and lacked a corpus callosum. The corneas tolerated the entropion satisfactorily until the age of 13 months (Fig. 6),
Fig. 9 (Bartley and associates). Case 3. Congenital bilateral lower eyelid entropion in a 2-week-old male infant. The lower eyelid retractors were intact.
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Fig. 11 (Bartley and associates). Case 3. Normal eyelid positions three months after surgery. no complications had developed during a follow-up of one year.
Discussion Fig. 10 (Bartley and associates). Case 3. The lower eyelid retractors (held by forceps) were surgically disinserted, resected, and reattached to the inferior tarsal edge. when operation was required. The lower eyelid retractors were carefully inspected and were intact, but there was a greater than normal amount of fat in the eyelids (Fig. 7), possibly caused by a defect in the orbital septum. The fat was excised, and the retractors were surgically disinserted (Fig. 8), resected slightly, and reattached to the inferior tarsal edge. The entropion was improved, but persistent corneal irritation necessitated two additional operations (another bilateral retractor advancement and tarsal strip procedure on the left lower eyelid; canthoplasty on the right lower eyelid and tarsotomy and marginal rotation on the left lower eyelid) to achieve a satisfactory eyelid position. Although the right lower eyelid was slightly rotated inward against the globe at the most recent examination (two years after surgery), the corneas were clear without staining.
Case 3 A 2-week-old male infant with a history of bilateral conjunctivitis since birth was found by one of us (R.C.K.) to have true entropion of both lower eyelids (Fig. 9). The inferior corneal epithelium of each eye stained with fluorescein, but there was no ulceration. The lower eyelids were operated on through a subciliary incision; the retractor aponeurosis was bilaterally intact. The retractors were surgically disinserted, resected 3 to 4 mm, and secured to the inferior tarsal border (Fig. 10). Additionally, a small strip of preseptal orbicularis muscle was excised. The entropion was relieved (Fig. 11), and
The etiologic mechanisms for upper and lower eyelid entropion are distinct. Inversion of the upper eyelid usually is associated with an inchoate or deformed tarsal plate; in its extreme form, this anomaly is referred to as the tarsal kink syndrorne.v" Of the various theories that have been proposed to explain the cause of congenital lower eyelid entropion, only two have been favored. In 1907, Leblond" attributed inversion of the eyelid to hypertrophy and overaction of the marginal orbicularis fibers, a view that was reasserted by von Herrenschwand'<" in 1916 and 1917 and by Fox" in his 1956 review of the topic. Leblond's etiologic mechanism was generally accepted until 1983, when Tse, Anderson, and Fratkin" demonstrated histologically that the orbicularis fibers near the eyelid margin were not hypertrophied. The authors postulated from their findings in three patients that disinsertion of the retractor aponeurosis from its normal attachments to the inferior portion of the tarsal plate is the most likely origin of congenital lower eyelid entropion. A similar mechanism was hypothesized by Quickert, Wilkes, and Dryden" in an independent report two months later, and since then it has been considered the primary cause of this uncommon congenital eyelid malposition. The intraoperative findings in our three patients demonstrated that disinsertion of the lower eyelid retractors is not a universal etiologic mechanism in true congenital entropion. No new anatomic reason for the eyelid malpositions was apparent, however, by either clinical examination or surgical exploration. In Case 2, the abnormally abundant orbital fat within the anterior portion of the eyelid may have exerted a mechanical effect on the eyelid margin. Cases
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1 and 3 exhibited spasm of the pretarsal orbicularis in that the eyelid margins could be everted to a normal position, but they would immediately rotate inward against the globe with a blink. We agree with Tse, Anderson, and Fratkin" that overaction or apparent hypertrophy of the pretarsal orbicularis may be secondary to ocular irritation and is not the underlying mechanism for the entropion. The successful surgical results achieved in Cases 1 and 3 by resecting and advancing the aponeurosis might be interpreted as tacit evidence for a retractor defect. A similar operation is commonly used for involutional entropion, however, even though disinsertion of the lower eyelid retractors is not always clinically apparent and has not been demonstrated histopatho-
logically."
The anatomic relationship of the lower eyelid retractors to the tarsal plate in congenital entropion would best be studied with cross sections of a full-thickness wedge resection, as was done by Hawes and Dortzbach" in their study of adults. Such specimens, unfortunately, are usually not obtained as an incidental consequence of congenital entropion repair because horizontal laxity is not typical. We hope that this study will stimulate others to obtain tissue for histopathologic analysis in patients with this uncommon disorder, if clinically appropriate. ACKNOWLEDGMENT
Niloofar Z. Gharagozloo, M.D., translated reference 12 and Helmut Buettner, M.D., translated references 13 and 14.
References 1. Czukrasz, 1.: Congenital entropion due to epiblepharon. Br. J. Ophthalmol. 34:318, 1950.
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2. Noda, S., Hayasaka, S., and Setogawa, T.: Epiblepharon with inverted eyelashes in Japanese children. I. Incidence and symptoms. Br. J. Ophthalmol. 73:126, 1989. 3. Tse, D. T., Anderson, R. L., and Fratkin, J. D.: Aponeurosis disinsertion in congenital entropion. Arch. Ophthalmol. 101:436, 1983. 4. Quickert, M. H., Wilkes, T. D. 1., and Dryden, R. M.: Nonincisional correction of epiblepharon and congenital entropion. Arch. Ophthalmol. 101:778, 1983. 5. Callahan, A.: Reconstructive Surgery of the Eyelids and Ocular Adnexa. Birmingham, Alabama, Aesculapius Publishing Company, 1966, pp. 37-40. 6. Hiles, D. A., and Wilder, L. W.: Congenital entropion of the upper lids. J. Pediatr. Ophthalmol. 6:157,1969. 7. Biglan, A. W., and Buerger, G. F.: Congenital horizontal tarsal kink. Am. J. Ophthalmol. 89:522, 1980. 8. Zak, T. A.: Congenital primary upper eyelid entropion. J. Pediatr. Ophthalmol. Strabismus 21:69, 1984. 9. McCarthy, R. W.: Lamellar tarsoplasty. A new technique for correction of horizontal tarsal kink. Ophthalmic Surg. 15:859, 1984. 10. Bosniak. S., Hornblass, A., and Smith, B.: Reexamining the tarsal kink syndrome. Considerations of its etiology and treatment. Ophthalmic Surg. 16:437,1985. 11. Price, N. c.. and Collin, J. R. 0.: Congenital horizontal tarsal kink. A simple surgical correction. Br. J. Ophthalmol. 71:204, 1987. 12. Leblond, E.: Etiologie de I'entropion congenital. Arch. Ophtalmol. 27:782, 1907. 13. von Herrenschwand, F.: Entropium palpebrarum congenitum. Klin. Monatsbl. Augenheilkd. 56:509,1916. 14. von Herrenschwand, F.: Ueber Entropium congenitum und Epiblepharon. Klin. Monatsbl. Augenheilkd. 58:385,1917. 15. Fox, S. A.: Primary congenital entropion. Arch. Ophthalmol. 56:839, 1956. 16. Hawes, M. J., and Dortzbach, R. K.: The microscopic anatomy of the lower eyelid retractors. Arch. Ophthalmol. 100:1313, 1982.