From the Institue of Ophthalmology and Visual Science, New Jersey Medical School, Newark, New Jersey.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Paul D. Langer, MD, Institute of Ophthalmology and Visual Science, New Jersey Medical School, 90 Bergen Street, DOC, Suite 6168, Newark, NJ 07103.
Congenital eyelid eversion is a rare phenomenon characterized by the protrusion of chemotic palpebral conjunctiva from an everted eyelid in a newborn. There have only been approximately 50 reported cases in the literature, and associations have been made with African-American race and Down syndrome. The condition usually resolves spontaneously with supportive topical lubrication and pressure patching, rarely necessitating surgical intervention. We present a case of an African-American newborn with congenital eversion of the right upper eyelid causing considerable chemosis, which resolved with topical lubrication.
A full-term African-American male infant was born uneventfully to a healthy nulliparous 27-year-old mother via spontaneous vaginal delivery at 37 weeks and weighing 3,410 g. Delivery was atraumatic and forceps were not used. The pregnancy had been routine, and there was no history of fetal distress or evidence of Down syndrome. Apgar scores at birth were normal. Physical examination at birth was normal except for severe injection and edema of the right upper eyelid noted by the obstetrician, who requested an ophthalmology consultation.
Ophthalmic examination performed 4 hours after delivery showed complete eversion of the right upper eyelid, with marked hyperemia and chemosis of the palpebral conjunctiva (Fig. 1). Attempts to restore the eyelid to a normal position via manual manipulation were unsuccessful. The remainder of the ophthalmic examination was normal, including normal anterior segment, extraocular movements, left eyelid anatomy, and no signs of birth trauma. There was no family history of eyelid pathology.
Figure 1. (A and B) Patient at Presentation with Right Upper Eyelid Eversion Showing Extensive Palpebral Conjunctival Hyperemia and Chemosis.
Treatment was started with dexamethasone/tobramycin ophthalmic ointment three times daily to the chemotic conjunctiva. Over the next several days, the hyperemia and chemosis gradually diminished, the eyelid returned to its normal position, and the infant was discharged home (Fig. 2).
Figure 2. Patient Several Days Following Topical Lubrication Displaying Resolution of Conjunctival Congestion.
Congenital eversion of the upper eyelid is a rare anomaly that can be seen in otherwise healthy newborns. In other instances, causes such as birth trauma, prolonged and difficult delivery, infection, and congenital malformations are present. Approximately 50 cases have been reported since the original description of this entity in 1896 by Adams.1 Most reported cases described the pathology at the time of birth, although late onset has been described as late as 11 years of age.2,3 Of the reported cases, a higher preponderance was noted in males (almost 2:1) and African Americans (60%).3 The predilection for African-American infants remains unexplained. Most reports involved bilateral involvement (86%), whereas the remaining seven unilateral cases were all seen in African-American infants.3 Involvement of all four eyelids has also been reported.4 Ten of the cases had Down syndrome, and this association is most apparent in whites (9 of 17 white patients had Down syndrome compared to only 1 of 30 African-American patients).3 Two cases had a positive family history of the condition, suggesting inheritance may be an etiologic factor.3 Most of the cases had uncomplicated vaginal deliveries.
There is no consensus as to the etiology of congenital eyelid eversion. Proposed theories include birth trauma, inclusion conjunctivitis, eyelid hypotonia, eyelid spasm, vertically short anterior eyelid lamellae, tissue hyperelasticity, congenital levator disinsertion, and failure of the orbital septum to fuse with the levator palpebrae aponeurosis with adipose interposition.4,5
Conservative treatment aimed at protecting the exposed conjunctiva from desiccation and infection usually leads to rapid resolution within 1 to 3 weeks, if recognized early. This can be accomplished with moist dressings, eyelid taping, pressure patching, and topical anti-inflammatory and antibiotic lubricants. The cornea is rarely involved because the protruding conjunctiva protects the cornea from exposure. Surgery is reserved for resistant cases or cases with late presentation. Surgical techniques include suturing procedures, tarsorrhaphy, skin grafts, horizontal eyelid shortening, subconjunctival injection of hyaluronic acid, and excision of redundant conjunctiva.
This case describes only the eighth report of unilateral congenital eversion of the eyelid and, like the others, it occurred in an African-American newborn. It also highlights the importance of early recognition and conservative treatment to avoid corneal sequelae, the need for subsequent eyelid surgery, and parental anxiety.
- Adams AL. A case of double congenital ectropion. Medical Fortnightly. 1896;337–338.
- Silverstone B, Hirsch I, Sternberg MD, Berson D. Late onset of total eversion of the upper eyelids. Ann Ophthalmol. 1982;14:477–448.
- Sellar PW, Bryars JH, Archer DB. Late presentation of congenital ectropion of the eyelids in a child with Down’s syndrome: a case report and review of the literature. J Pediatr Ophthalmol Strabismus. 1992;29:64–67.
- Raab EL, Saphir RL. Congenital eyelid eversion with orbicularis spasm. J Pediatr Ophthalmol Strabismus. 1985;22:125–128.
- Blechman B, Isenberg S. An anatomical etiology of congenital eyelid eversion. Ophthalmic Surg. 1984;15:111–113.