Ectropion
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Ectropion is an outward turning of the eyelid margin. Patients may experience symptoms due to ocular exposure and inadequate lubrication. Definitive management is surgical. Medical management is temporizing but can improve symptoms while waiting for surgery.[1][2]
Disease Entity
- ICD-9 374.10 Ectropion, unspecified
- ICD-10 H02.109 Unspecified ectropion of unspecified eye, unspecified eyelid
Disease
Ectropion is an outward turning of the eyelid margin. It primarily involves the lower lid. Upper eyelid eversion can occur in Floppy Eyelid Syndrome.[1]
Etiology
There are four main types of ectropion: involutional, cicatricial, mechanical, and paralytic.[1]
- Involutional ectropion is caused by increased horizontal laxity of the lower eyelid and disinsertion of the lower eyelid retractors.
- Cicatricial ectropion is caused by shortening of the anterior lamella, which is comprised of the skin and orbicularis muscle.
- Paralytic ectropion is caused by decreased orbicularis muscle tone supporting the lower eyelid.
- Additionally, mechanical ectropion can occur when a mass, such as a tumor, displaces the lower eyelid margin.
Congenital ectropion can occur rarely, and may be seen in association with other congenital defects such as blepharophimosis syndrome or euryblepharon.[1]
Congenital upper eyelid eversion (neonatal)
Congenital upper eyelid eversion is a rare neonatal eyelid malposition characterized by complete eversion of the upper eyelid margin, typically with marked conjunctival chemosis. It usually presents at birth or shortly thereafter and is most often bilateral.
Older literature described congenital upper eyelid eversion as “double congenital ectropion”.[3]
The exact cause remains unknown. Several mechanisms have been proposed:
- Anatomical
- Birth-related factors
- Birth canal-related trauma sustained during delivery
- Vicious cycle mechanism
- Once eversion occurs, orbicularis spasm may act as a sphincter creating a cycle of conjunctival compression and edema secondary to venous stasis, perpetuating eyelid eversion.[6]
- Iatrogenic/postsurgical
- Might occur as a postoperative complication following oculoplastic procedures for congenital ptosis.[7]
It is most often bilateral; however unilateral presentations have been reported although exceptionally rare.[8][9][10]
Some reports describe the condition more frequently in Black infants, particularly males.[11]
Trisomy 21 (Down syndrome) demonstrates a significant association, with affected cases presenting at a later stage and often requiring surgical intervention.[5]
Lamellar ichthyosis (collodion baby) is associated with severe bilateral ectropion.[12]
Risk Factors
- Age (gravity, loss of elasticity)
- Eyelid rubbing
- Repeated eyelid pulling (ex. contact-lens use)
- Floppy eyelid syndrome
- Long-term use of eye drops
- Skin conditions which involve the eyelid
- Trauma
- Prior Eyelid Surgery
General Pathology
Ectropion can be classified as:
- Involutional (most common)
- Paralytic
- Cicatricial
- Mechanical
- Congenital (very rare)
Pathophysiology
In involutional ectropion, the tarsoligamentous sling supporting the eyelid by attachment to the orbital rim via the medial and lateral canthal tendons becomes lax. In paralytic ectropion, orbicularis muscle tone is weak or absent due to facial nerve palsy. In cicatricial ectropion, the anterior or middle lamellae are shortened due to scarring. Midfacial hypoplasia, where the inferior orbital rim is located relatively posterior to the eyeball, results in both decreased lower eyelid support and increased propensity for lower eyelid retraction.
History
In addition to abnormal position of the lid, patients experience tearing, irritation/grittiness/foreign body sensation, red eye, and mucoid discharge. Symptoms are caused by ocular exposure, inadequate closure and lubrication. Inquire about habitual eye rubbing, which may accompany symptoms of itch from ocular allergy or ocular surface disease.
Physical Examination
- Facial architecture:
Examine the bony architecture of the lower orbital rim and midface position. Patients with hypoplastic midface, also known as hemiproptosis, will have an inferior orbital rim located posteriorly relative to the globe. - Facial nerve palsy:
Inspect the face and test facial muscle strength to assess for paralysis. - Eyelid laxity:
To test for horizontal laxity, place a thumb beneath the lateral canthus and push the eyelid laterally and superiorly. If the lid margin does not roll back into position, suspect a cicatricial component. In involutional cases, the ectropion typically disappears with this maneuver. The eyelid distraction test is done by pulling the lid away from the globe. Normal lid distraction is between 2-3 mm. If it is more than 5mm, there is substantial laxity. In cases of cicatricial ectropion, the eyelid malposition will often become accentuated by asking the patient to look upwards and to open his or her mouth at the same time; the maneuver places the anterior lamella on maximum stretch. - Eyelid pathology:
Examine the eyelid margin under magnification to look for signs of chronic blepharitis, palpebral conjunctival hypertrophy and keratinization, conjunctival scarring, and to rule out suspicious changes such as loss of lashes (madarosis), ulceration, or infiltration. - Punctal ectropion:
Assess the position of the lower punctum which may rotate away with medial laxity and no longer make contact with the ocular surface and tear lake. - Ocular surface:
Examine the cornea for epithelial changes secondary to exposure. - Neonates (congenital upper eyelid eversion):
Diagnosis is established clinically during the physical examination of the newborn, noting the characteristic appearance of the upper eyelid eversion. A comprehensive eye examination is required as well. Prompt management is recommended to prevent exposure-related corneal complications. Corneal integrity should be assessed and monitored, especially if chemosis prevents adequate eyelid coverage.
Differential diagnosis
- Eyelid malignancy
- Eyelid retraction secondary to proptosis (e.g. thyroid eye disease), excessive tissue removal with lower blepharoplasty, or inferior rectus recession without disinsertion of the lower lid retractors.
- Floppy Eyelid Syndrome
- Lamellar Ichthyosis
- Facial Nerve Palsy
Management
Definitive management is surgical. Medical management is temporizing but can improve symptoms while awaiting surgery.[2]
Deferral of surgery should be considered in 2 groups of patients. Ectropion induced by long-term use of eye drops such as dorzolamide and brimonidine may resolve with discontinuation, if feasible.[13] In patients with inflammatory skin conditions involving the eyelid, improvement or reversal of ectropion has been reported with improved control of inflammation.[14]
Medical therapy
- Lubrication of the ocular surface
- Horizontal taping of the eyelid
Surgery
- Lower eyelid laxity: the lower eyelid is horizontally tightened by a lateral tarsal strip or similar procedure.
- Lower eyelid retractor disinsertion: the Jones procedure reattaches retractors to the tarsus.
- Punctal ectropion: the medial spindle procedure reapposes the everted punctum.
- Cicatricial ectropion often requires lengthening of the anterior lamella by a skin graft.
- Paralytic ectropion requires horizontal tightening and correction of punctal ectropion. With facial nerve paralysis, corneal exposure and brow ptosis may also need to be addressed.
- Mechanical ectropion from facial ptosis may often require surgical elevation of the mid face (such as a suborbicularis oculi fat lift), or a face lift in conjunction with lower lid tightening.
- In some cases of heavy facial tissues or recurrence, periosteal fixation may not be successful and therefore require additional fixation using bone plates or bone tunnels to which the lower lid can be suspended.[15]
Congenital upper eyelid eversion (neonatal)
The goal of management is to protect the exposed conjunctiva, prevent desiccation, reduce chemosis, prevent amblyopia and allow spontaneous lid reversion. Conservative management is usually successful and should be first-line treatment unless significant complications are present.
Conservative management
- 5% hypertonic normal saline:
- Gauze pads soaked in hypertonic saline applied 2-4 times daily
- Mechanism: osmotic dehydration of chemotic conjunctiva[16]
- Commonly used to reduce chemosis
- Topical antibiotics:
- Prevent secondary bacterial infection
- Ointment preferred because it prevents desiccation of exposed conjunctiva[17]
- Topical lubricants:
- Protect exposed conjunctiva
- Topical corticosteroid (adjunct, selected cases):
- Short-term topical corticosteroids have been reported as part of conservative management in cases with marked chemosis/inflammation.[18]
- Use judiciously in neonates with close follow-up.
- Eye padding, taping:
- Light pressure patching to maintain lid position with topical antibiotic
- Sterile cotton pads, replaced daily
- Expected course: 2-3 weeks, with initial improvement in 2-3 days and partial lid reversion by day 5-7 in reported cases.[9]
- Caution: Manipulation/repositioning of the everted lid in neonates should be gentle, with awareness of potential autonomic (oculocardiac/oculorespiratory) reflex effects; respiratory arrest has been reported during lid manipulation.[19]
Surgical treatment
The strongest indication is failure of conservative management, chronicity, corneal complications, and parental/social factors requiring rapid resolution. Options include:
- Full thickness skin grafts: for anterior lamella lengthening, especially in cases with anterior lamella deficiency.[20]
- Temporary tarsorrhaphy
- Fornix sutures: placement of sutures in the upper eyelid fornix, excision of the tarsal segments, and anchoring of the tarsal plates to the anterior lamella.[8]
Complications
Ectropion surgery is considered safe and effective. Recurrence does occur occasionally after several years requiring a repeat surgery.
Like any eyelid surgery there is always a possibility of local post-operative bleeding or infection but these are generally minor. Injury to the cornea is possible but uncommon with careful technique.
In congenital upper eyelid eversion, prognosis is excellent with appropriate management. Complete resolution is expected in most cases without long-term sequelae, if treated promptly. Corneal exposure complications are uncommon but have been reported, particularly with delayed treatment.[20]
Additional Resources
References
- ↑ 1.0 1.1 1.2 1.3 Orbit, Eyelids, and Lacrimal System, Section 7. Basic and Clinical Science Course. San Francisco: American Academy of Ophthalmology; 2009.
- ↑ 2.0 2.1 American Academy of Ophthalmology Focal Points: Ectropion and Entropion, Volume 12, Number 10, 1994.
- ↑ Adams A. A case of double congenital ectropion. Med Fortnightly. 1896;9:337-338.
- ↑ Blechman B, Isenberg S. An anatomical etiology of congenital eyelid eversion. Ophthalmic Surg. 1984;15(2):111-113.
- ↑ 5.0 5.1 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(1):64-67. doi:10.3928/0191-3913-19920101-13.
- ↑ 6.0 6.1 Raab EL, Saphir RL. Congenital eyelid eversion with orbicularis spasm. J Pediatr Ophthalmol Strabismus. 1985;22(4):125-128. doi:10.3928/0191-3913-19850701-03.
- ↑ Wolfley DE. Preventing conjunctival prolapse and tarsal eversion following large excisions of levator muscle and aponeurosis for correction of congenital ptosis. Ophthalmic Surg Lasers Imaging. 1987;18(7):491-494. doi:10.3928/1542-8877-19870701-04.
- ↑ 8.0 8.1 Cavuoto KM, Hui JI. Congenital eyelid eversion. J Pediatr Ophthalmol Strabismus. 2010;47(1):1-3. doi:10.3928/01913913-20100507-02.
- ↑ 9.0 9.1 Shinder R, Langer PD. Unilateral congenital eyelid eversion causing marked chemosis in a newborn. J Pediatr Ophthalmol Strabismus. 2010;47(1):1-2. doi:10.3928/01913913-20091218-05.
- ↑ Atitallah S, Ben Ayed M, Bouyahia O, Mazigh S, Yahyaoui S, Boukthir S. Unilateral congenital upper eyelid eversion: a rare presentation in a neonate. Eur J Ophthalmol. 2025;35(6). doi:10.1177/11206721251350821.
- ↑ Kirkpatrick A, Ledlow D, Dixon E, Philips JB. Congenital bilateral eyelid eversion and chemosis: a case study. Neonatal Netw. 2018;37(3):137-140. doi:10.1891/0730-0832.37.3.137.
- ↑ Shapiro RD, Soentgen ML. Collodion skin disease and everted eyelids. Postgrad Med. 1969;45(4):216-219. doi:10.1080/00325481.1969.11697104.
- ↑ Hegde V, Robinson R, Dean F, et al. Drug-induced ectropion: what is best practice? Ophthalmology. 2007;114:362-366.
- ↑ Durairaj VD, Horsley MB. Resolution of pityriasis rubra pilaris-induced cicatricial ectropion with systemic low-dose methotrexate. Am J Ophthalmol. 2007;143:709-710.
- ↑ Nerad JA. Techniques in Ophthalmic Plastic Surgery: A Personal Tutorial. Philadelphia: Saunders; 2010.
- ↑ Adeoti CO, Ashaye AO, Isawumi MA, Raji RA. Non-surgical management of congenital eversion of the eyelids. J Ophthalmic Vis Res. 2010;5(3):188-192.
- ↑ Dohvoma VA, Nchifor A, Ngwanou AN, et al. Conservative management in congenital bilateral upper eyelid eversion. Case Rep Ophthalmol Med. 2015;2015:1-3. doi:10.1155/2015/389289.
- ↑ Daniel P, Cogen M. Conservative management of congenital upper eyelid eversion. J AAPOS. 2020;24(1):46-48. doi:10.1016/j.jaapos.2019.10.005.
- ↑ Watts MT, Dapling RB. Congenital eversion of the upper eyelid: a case report. Ophthalmic Plast Reconstr Surg. 1995;11(4):293-295. doi:10.1097/00002341-199512000-00014.
- ↑ 20.0 20.1 Al-Hussain H. Congenital upper eyelid eversion complicated by corneal perforation. Br J Ophthalmol. 2005;89(6):771-771. doi:10.1136/bjo.2004.053348.
