From Bascom Palmer Eye Institute, Miami, Florida.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Kara M. Cavuoto, Bascom Palmer Eye Institute, 900 NW 17th Street, Attn: Kathy Corser, Room 242, Miami, FL 33136.
Congenital eyelid eversion is a rare condition that may occur in isolation or in association with systemic disorders. It is often treated conservatively; however, surgical intervention is required in some cases. We present a review of the literature and a case of bilateral upper eyelid eversion, which was addressed with three surgical procedures.
A 10-day-old African-American female infant presented to the emergency department with bilateral upper eyelid eversion that had been present since birth (Fig. 1). The patient was the 6 lb 1 oz product of an uncomplicated, full-term normal spontaneous vaginal delivery. Labor was not prolonged and mechanical intervention was not required. The family history was negative for ocular adnexal anomalies. The parents were able to reposition the eyelids. However, the eversion returned when the infant cried or grimaced.
Figure 1. Persistent Bilateral Upper Eyelid Eversion After Pressure Patching. Chemosis Had Resolved with the Patching and Lubrication.
On initial examination, bilateral upper eyelid eversion with chemosis and keratinization was prominent. The examiner was able to rotate the eyelids into proper position manually. Anterior lamellar shortage was not noted.
The patient was treated for 1.5 weeks with bilateral pressure patches that were placed for 24 hours at a time on three separate occasions. Lubrication was also instituted. Of note, the patient also demonstrated euryblepharon that was greater in the left eye than in the right eye. Both eyelid eversion and euryblepharon have a common association with Down syndrome. However, in this patient the lateral canthus was not lax nor was there an association with deficiency of the skin of the eyelids, both of which are common in euryblepharon.1
The conservative measures of patching and lubrication did not improve the eversion. Surgical intervention was then undertaken to improve eyelid position and reduce chemosis. At 26 days of life, the infant underwent bilateral upper eyelid fornix sutures and lateral temporary suture tarsorrhaphy with 6-0 chromic gut. At the conclusion of the procedure, the infant’s upper eyelids were in proper position and the visual axes were clear.
The patient returned for follow-up on postoperative day 9 with recurrence of the eversion medially in both upper eyelids. The parents reported that the eversion recurred on postoperative day 3. Superior lateral tarsal strip procedures with lateral suture tarsorrhaphy were performed on day of life 37. Two 9-mm wide tarsal segments were excised and submitted to pathology. Histologic examination showed mild chronic inflammatory cell infiltrate but no atypia. The patient returned on postoperative day 3 (day of life 40) after the second surgery with a mild amount of left upper eyelid eversion.
On day of life 51, she presented with recurrent upper eyelid eversion that was worse on the left side. On day of life 58, she underwent further eyelid surgery to anchor the tarsal plates to the anterior lamella. An eyelid crease incision and post-orbicularis dissection exposed the superior margin of the tarsal plate. The superior edge of the tarsal plate was engaged with three sets of double-armed 6-0 polyglactin sutures in a lamellar fashion along a vertical orientation. Each arm was then passed through the anterior lamella exiting 3.5 mm above the incision (Fig. 2). Lamellar sutures were taken to avoid corneal trauma. Each set of double-armed sutures was tied temporarily while medial and lateral suture tarsorrhaphies were performed. The central visual axes were not obstructed. Following completion of the tarsorrhaphies, the tarsal anchoring sutures were tied permanently to achieve the proper amount of tension and eyelid repositioning. The patient returned on day of life 65 with the upper eyelids in proper anatomic position. The medial tarsorrhaphies were no longer intact, but the parents reported that the eversion had not recurred.
Figure 2. Intraoperative Photograph on Day of Life 58 Demonstrating Tarsal Anchoring Sutures. The Sutures Were First Passed in a Vertical Lamellar Fashion Through the Superior Tarsal Plate. Each Arm Was Then Passed Through the Anterior Lamella to Exit 3.5 mm Above the Incision.
The patient was lost to follow-up for a period of time, but the parents returned with the child at 7 months of age (4 months after the third procedure). They reported that the eversion had not recurred; on examination, the patient’s upper eyelids were in proper position without lagophthalmos (Fig. 3). Eversion was not induced during crying.
Figure 3. Photograph Taken 4 Months After the Third Procedure Showing the Upper Eyelids in Proper Position.
Genetic analysis by fluorescent in-situ hybridization (FISH) was obtained during the course of treatment. A 46,XX,i(21)(q10) isochromosome of chromosome 21 due to an unbalanced Robertsonian translocation was identified. A diagnosis of Down syndrome was made. This isochromosome is postulated to occur in approximately 5% of children with Down syndrome and is paternal in origin.2,3
An echocardiogram was performed during the patient’s cardiac evaluation. It was essentially unremarkable except for a left aortic arch with aberrant right subclavian artery. A geneticist evaluated the patient and will continue to monitor for conditions associated with Down syndrome, including hypothyroidism. The child was also referred for an audiology evaluation.
Congenital eyelid eversion is a rare condition, with fewer than 100 cases reported in the literature since its first description by Adams in 1896.4 The condition is usually bilateral and most often involves the upper eyelids. Infants are often born with the eyelids everted, thus exposing the tarsal conjunctiva. The conjunctiva may become chemotic and obscure visualization of the globes.
Eyelid eversion can be defined as an externalization of the conjunctiva. The characteristics of this condition include transient eyelid laxity, horizontal eyelid excess, overlapping of the lower eyelid margin by the upper eyelid, and the ability to reposition the eyelid manually. The conjunctiva may become keratinized if eversion is prolonged.
Previous histologic examinations of the eyelids have not revealed any abnormalities. Eyelid eversion usually resolves without permanent sequelae after conservative management. Eversion must be distinguished from ectropion, which is defined as an out turning of the eyelid margin, because surgical intervention is almost always indicated in the latter condition.5–7
Multiple mechanisms have been theorized to cause congenital eyelid eversion. Some have postulated that passage through the birth canal may interrupt venous drainage from the eyelid and periorbital tissues.8 Another proposed birth-related etiology is facial trauma caused by uterine contractions.9 Contractions may distort and evert the eyelids. Many authors suggest the pressure exerted on the eyelids during delivery as the etiology.10,11 The conjunctival irritation may result in orbicularis spasm. This spasm hinders normal venous and lymphatic return and exacerbates the conjunctival chemosis. The chemosis in turn worsens the spasm and a cyclical, progressive pattern develops.6 Alternatively, Sellar et al. postulated that the affected children may have a horizontal excess of the upper eyelid.12 Loeffler and Hornblass agree that it is difficult to elucidate the exact mechanism of eversion, but suggest a laxity of the attachment between the anterior and posterior lamellae.13 However, the common postulated pathway is secondary orbicularis spasm and the cyclical pattern described above.
The majority of published cases involve complete eversion following uncomplicated births. There is a racial predilection for African-American children. Among white children, there is an increased incidence of Down syndrome. A notable finding among African-American children is the presence of relatively long upper eyelids in the horizontal dimension. Conversely, white patients have more varied anomalies including absent tarsal plates, absent meibomian glands, tetrastichiasis, and epicanthus.
The natural course of the eversion is spontaneous resolution within the first few weeks of life. However, intervention may be indicated if amblyopia is likely to occur from obscuration of the central visual axis. Conservative measures include aggressive lubrication, topical antibiotics, topical steroids, and moisture chambers to alleviate conjunctival irritation and edema. Tape may be placed on the skin to splint the eyelid into proper position. If unsuccessful, more invasive interventions may be warranted. Procedures described in the literature include intermarginal sutures, temporary suture tarsorrhaphy, wedge resection of lateral portions of upper and lower eyelids, tarsectomy with mullerectomy, skin grafts, excision of redundant conjunctiva, reconstruction of the relationship of the anterior and posterior lamellae with reconstruction of the fornices, and subconjunctival injection of hyaluronidase.5,13 Hyaluronidase is postulated to hasten reduction of conjunctival chemosis. Following treatment, most patients are able to achieve normal eyelid appearance and function.
Two of these previously described surgical procedures (suture tarsorrhaphy/fornix sutures and lateral tarsal strip) were performed in our patient in attempts to prevent eversion and shorten the relatively long horizontal length of the upper eyelids. Ultimately, she required the tarsal anchoring sutures described above to maintain proper upper eyelid position. The cycle of eversion and orbicularis spasm was successfully broken in our patient to facilitate resolution of the condition. These tarsal anchoring sutures may be considered as a primary or subsequent surgical method of repair for patients with more severe eversion whose condition persists despite conservative measures.
- Katowitz WR, Katowitz JA. Congenital and developmental eyelid abnormalities. Plast Reconstr Surg. 2009;124(1 suppl):93e–105e. doi:10.1097/PRS.0b013e3181aa2a9b [CrossRef]
- Gole L, Lian NB, Lian NP, Rauff M, Biswas A, Choolani M. Prenatal detection of Isochromosome 21 by QF-PCR: a comparison between FISH and traditional karyotyping. Fetal Diagn Ther. 2008;24:47–50. doi:10.1159/000132406 [CrossRef]
- Berend SA, Canun S, McCaskill C, Page SL, Shaffer LG. Molecular analysis of mosaicism for two different de novo acrocentric rearrangements demonstrates diversity in Robertsonian translocation formation. Am J Med Genet. 1998;80:252–259. doi:10.1002/(SICI)1096-8628(19981116)80:3<252::AID-AJMG14>3.0.CO;2-Q [CrossRef]
- Adams A. A case of double congenital ectropion. Med Fortnightly. 1896;9:136.
- Bentsi-Enchill KO. Congenital total eversion of the upper eyelids. Br J Ophthalmol. 1981;65:209–213. doi:10.1136/bjo.65.3.209 [CrossRef]
- Miller R, Martin F, Allen H. A case of congenital ectropion in Down’s syndrome. Austr N Z J Ophthalmol. 1988;16:119–125. doi:10.1111/j.1442-9071.1988.tb01260.x [CrossRef]
- Pico G. Congenital ectropion and distichiasis: etiologic and hereditary factors: a report of cases and review of the literature. Transactions. 1957;55:663–700.
- Stern E, Campbell C, Faulkner W. Conservative management of congenital eversion of the eyelids. Am J Ophthalmol. 1973;75:319–320.
- Erb A. Ein Fall von doppelseitigem congenitalem Ectropion des Oberlids. Cor Bl Schweiz Aerzte. 1909;39:733–734.
- Duke-Elder S. Total eversion of the upper eyelids. In: Duke-Elder S.System of Ophthalmology. London: Kimpton; 1964:866–867.
- Stillerman NL, Emanuel B, Padorr MP. Eversion of the eyelids in the newborn without an apparent cause. J Pediatr. 1966;69:656–658. doi:10.1016/S0022-3476(66)80058-6 [CrossRef]
- Sellar PW, Bryars JH, Archer DB. “Late presentation of congenital ectropion of the eyelids in a child with Down syndrome: a case report and review of the literature.”J Pediatr Ophthalmol Strabismus. 1992;29: 64–67.
- Loeffler M, Hornblass A. Surgical management of congenital upper-eyelid eversion. Ophthalmic Surg. 1990;21:435–437.