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Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 437-438

Management of congenital ectropion of bilateral upper and lower eyelids in Down's syndrome


1 Consultant Orbital and Oculoplastic Surgeon, Manipal Hospitals, Salem, Tamilnadus, India
2 Baroda Children Eyecare and Squint Clinic, Vadodara, Gujarat, India

Date of Submission07-Sep-2020
Date of Acceptance25-Feb-2021
Date of Web Publication02-Jul-2021

Correspondence Address:
Dr. G Seethapathy
Department of Ophthalmology, Manipal Hospital, Salem, Tamilnadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_2902_20

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  Abstract 


Keywords: Congenital ectropion, Down's syndrome, lower eyelid, upper eye lid


How to cite this article:
Seethapathy G, Jethani J. Management of congenital ectropion of bilateral upper and lower eyelids in Down's syndrome. Indian J Ophthalmol Case Rep 2021;1:437-8

How to cite this URL:
Seethapathy G, Jethani J. Management of congenital ectropion of bilateral upper and lower eyelids in Down's syndrome. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 29];1:437-8. Available from: https://www.ijoreports.in/text.asp?2021/1/3/437/320025



Congenital ectropion is caused by vertical insufficiency of the anterior lamella of the eyelid and may give rise to chronic epiphora and exposure keratitis.[1],[2],[3] The association of congenital ectropion involving the upper and lower eyelids bilaterally with Down's Syndrome is relatively uncommon.[4],[5] Mild congential ectropion usually can be managed conservatively.[6] However, severe and symptomatic congential ectropion requires treatment which is similar to cicatricial ectropion.[7] We report a case of Down's syndrome with congenital ectropion and its management.

A 12-year-old female child presented to us with inability to close the eyes completely noticed by her parents since birth along with an abnormal everted appearance of both the upper and lower eyelids on both sides with long-standing exposure keratopathy.

On examination, the child was found to have bilateral grade 4 ectropion of both the upper eyelids exposing the tarsal conjunctiva and also a grade 2 uniform ectropion of the lower eyelids on both the sides. The child demonstrated a vertical anterior lamellar deficiency of all the four eyelids. In addition the corneas in both eyes were vascularized and opacified in the inferior 1/3 due to longstanding exposure keratopathy.

The child was uncooperative for visual acuity assessment as he had significant learning difficulties. We were also unable to perform corneal topography accurately (to rule out accompanying keratoconus which is also a well-known ocular association of Down's syndrome) as the parents were unwilling for a preoperative examination under anesthesia. Ocular examination was otherwise unremarkable with normal pupils, ocular movements, and normal posterior segment. In view of the severe learning disability and less cooperation, the post-operative management was difficult and challenging.

We decided to do the whole procedure in one sitting to avoid recurrent anesthesia and multiple staged surgeries. The precautions taken included avoiding short-acting depolarizing muscle relaxant, using small size endotracheal tube due to airway problems and to avoid sleep apnea we avoided narcotic drugs, an incremental dose of midazolam, and IV paracetamol for pain relief. The child had smooth recovery and post-operative course.


  Discussion Top


A complete eversion of the upper eyelids occasionally occurs in newborns. Possible causes include inclusion conjunctivitis, anterior lamellar inflammation or shortage or Down's syndrome.[1],[2],[3] True congenital ectropion can be primary[1],[2],[3] when associated with other eyelid abnormalities and secondary when it accompanies ocular/orbital/skin diseases. Distinctive clinical features help to differentiate the two entities (Congenital eversion of eyelids and True congenital ectropion).

Congenital eyelid eversion, a close differential diagnosis to be considered is an entity characterized by protrusion of the edematous conjunctiva from the everted eyelids.[8] The chemosed conjunctiva protects the cornea and hence corneal complications are rare. The proposed mechanisms include Orbicularis hypotonia, birth trauma, vertical shortening of the anterior lamella or vertical elongation of the posterior lamella, failure of the orbital septum to fuse with the levator aponeurosis, absence of an effective lateral canthal ligament, and lateral elongation of the eyelid. Once everted, the orbicularis spasm may act as a sphincter that leads to a vicious cycle of conjunctival strangulation and edema secondary to venous stasis. This usually resolves spontaneously or improves with non-surgical supportive measures.

In contrast, true congenital ectropion is definitively associated with a vertical anterior lamellar deficiency in the presence of a relatively normal posterior lamella. It is almost always accompanied by lagophthalmos and varying degrees of exposure keratopathy. This usually requires functional eyelid surgery for corneal protection.[9]

Our patient had a simultaneous correction of ectropion of all 4 eyelids by full-thickness skin grafting (the 2 upper eyelids were repaired by grafts from the respective ipsilateral supraclavicular regions and the 2 lower eyelids were corrected by grafts taken from the respective postauricular region). The skin overlying the posterior triangle (supraclavicular region) and over the postauricular region is excellent and relatively non-hair-bearing sites to be considered as donor sites for harvesting full-thickness skin grafts for eyelid tissue reconstruction. Once the line of shortening was released and the defective area of anterior lamellar deficiency was identified, the defect was measured and then the grafts harvested were deliberately chosen to be roughly 30% larger than the aforementioned eyelid defects. This was done to allow for late graft contracture as a part of routine wound healing and to prevent a residual late ectropion. In addition, the two lower eyelids were also tightened by a lateral tarsal strip procedure to tighten the lax lateral canthal tendon to facilitate better eyelid closure [Figure 1].
Figure 1: (a) Preoperative photograph of a child with Down's syndrome showing corneal opacity with bilateral severe upper lid ectropion with mild to moderate ectropion of lower lids. (b and c) Intraoeprative photograph showing right eye graft placement over upper and lower lid and showing complete closure of the palpebral fissure on the table. (d) Showing bilateral all four lids having a graft in place with no lagophthalmos

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The combination of lateral tarsal strip procedure to correct the lateral canthal laxity and the replacement of the deficient anterior lamella with full-thickness skin grafts helped in fully correcting the pre-existing ectropion.

We were able to achieve complete resolution of lagophthalmos and prevention of further corneal exposure. The preop, intraop, and postop photographs are being presented [Figure 2].
Figure 2: (a) Postoperative 8 weeks picture showing wonderfully corrected ectropion of both eyes. (b) Postoperative picture showing much improvement in the lagophthalmos

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True congenital ectropion with anterior lamellar deficiency needs to be distinguished from congential eversion of the eyelids that may resolve spontaneously.

We wish to report this case for the relative rarity of occurrence and also to highlight the fact that we were able to correct the four eyelid malposition in a single sitting thus avoiding multiple anesthetic inductions in this child with co-existing systemic concerns.

Acknowledgement

Dr. V. Paneerselvam, Director, Arasan Eye hospital.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ibraheem WA. Bilateral congenital upper eyelid eversion: The clinical course and outcome of conservative management. Pan Afr Med J 2014;17:215.  Back to cited text no. 1
    
2.
Pereira FJ, Trindade SP, Cruz AAV. Congenital ectropion: 3 case reports and review of literature review. Arq Bras Ofthalmol 2007;70:149-52.  Back to cited text no. 2
    
3.
Morax S, Gatinel D. Primary congenital ectropion. Apropos of 2 cases. J Fr Ophtalmol 1996;19:415-22.  Back to cited text no. 3
    
4.
Miller R, Martin F, Allen H. A case of congenital ectropion in Down's syndrome. Aust N Z J Ophthalmol 1988;16:119-25.  Back to cited text no. 4
    
5.
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;29:64-7.  Back to cited text no. 5
    
6.
Stern EN, Campbell CH, Faulkner HW. Conservative management of congenital eversion of the eyelids. Am J Ophthalmol 1973;75:319-20.  Back to cited text no. 6
    
7.
Alvarez EV, Wakakura M, Alvarez EI. Surgical management of persistent congenital eversion of the upper eyelids. Ann Ophthalmol 1988;20:353-4, 357.  Back to cited text no. 7
    
8.
Maheswari R, Maheashwari S. Congenital eversion of upper eyelids: Case report and management. Indian J Ophthalmol 2006,54:203-4.  Back to cited text no. 8
    
9.
Morris RJ, Collin JR. Functional lid surgery in Down's syndrome. Br J Ophthalmol 1989;73:494-7.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2]



 

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