Bilateral Congenital Upper Eyelid Eversion (Ectropion): A Rare Presentation Responded Well to Conservative Treatment at Temeke Regional Referral Hospital-Tanzania ()
1. Introduction
Described for the 1st time in 1896 by Adams as a case of double congenital ectropion, in which the eyelid is completely turned out, with prolapsed conjunctiva and chemosis. Most of the cases described in literature were present at birth, but some cases of late onset have also been reported [1] [2].
2. Case Presentation
This is case report of a 3-day-old male baby who was referred to our facility 24 hours after delivery with bilateral eversion of the upper eyelids. The baby was born at term by spontaneous vertex delivery with 3.4 kg and no reported birth trauma. The prenatal history was uneventful.
On examination: There were out turning of the both eyes (more on the left eye), with chemosis of conjunctiva of both eyes, and no discharge. The rest of anterior segment examination findings were normal (Figure 1).
A diagnosis of bilateral congenital ectropion was reached and possible differentials like Downs syndrome and TORCHES were ruled out. The patient was managed conservatively with pressure dressing by sterile gauze soaked in normal saline. The patient completely resolved with 7 days of treatment with no complications (Figure 2). We had a holistic approach of which the treatment team to this rare condition included ophthalmologist, general surgeon and pediatrician.
Figure 1. Appearance of the 1-day-old baby with congenital bilateral upper eye lid eversion on admission to our facility
Figure 2. Complete resolution of ectropion after 7 days of conservative therapy.
3. Discussion
Congenital eversion of upper eyelids is a very rare presentation. The underlying cause of the disease remains largely unknown, yet several possible mechanisms have been postulated. Birth trauma, orbicularis muscle hypotonia, vertical shortening of the anterior eyelid lamella or vertical elongation of the posterior eyelid lamella, lateral elongation of the lid, failure of the orbital septum to fuse with the levator aponeurosis, and absence of an effective lateral canthal ligament have been implied as probable underlying causes [2] [3] [4] [5]. Our 3 days neonate had no reported birth trauma and on physical examination did not reveal any skeletal deformity.
The incidence appears higher in black infants, infants with trisomy 212 and infants born with collodion skin disease [6] [7]. Moreover most cases are bilateral and present at birth; however, unilateral and late onset have been reported as well [3] [5]. The index case is of black descent and presented at birth with bilateral disease. In addition he did not have neither clinical nor radiological features of Down’s syndrome no collodion skin disease.
Generally, conservative management is favoured and surgical options usually become necessary in recalcitrant type. Conservative treatment is mostly supportive and it includes the use of 5% hypertonic saline and lubricant. Documented surgical options include the use of compression sutures, tarsorrhaphy with excision of redundant conjunctiva, subconjunctival injection of hyaluronic acid, fornix sutures, and full thickness skin graft to the upper lid [4] [8] [9]. The present case was treated conservatively with pressure dressing of sterile gauze soaked in normal saline. Complete recovery was achieved within a week of treatment.
4. Conclusion
Despite of the debate on the best mode of treatment of these cases, based on our experience on the management of the index case we still recommend on conservative approach over surgery when patients present early.