Ectrópio congênito: relato de três casos e revisão de literatura

Abstract

To report 3 cases of congenital ectropion because of their rarity and confusing classification. Case 1: JPT, 2 days old, male, negro. Left upper eyelid eversion with chemosis was present, passive to mechanic reduction. Compressive occlusion was done with ectropion regression in 48 hours. Case 2: AJL, 6 years old, female, Caucasian, with Down syndrome. The left eye had hyperemia, lagophthalmos and inferior leucoma. She received horizontal shortening (superior and inferior tarsal strip) and skin grafts, and after 2 months the patient did not return. Case 3: GSD, 4 years old, male, Caucasian, with Down syndrome. His signs and treatment were the same as in case 2. According to Picó's classification the first case is classified as grade II due to eyelid eversion during the passage through the birth canal, more frequent in black people. Cases 2 and 3 represent grade III that is due to eyelid skin alteration, and the association with Down syndrome is observed. Treatment for ectropion grade III is always surgical, as it was done in these cases. We do not agree with Picó's classification, the only one found in medline, because there are no articles confirming the existence of grade I (absent tarsus), grade II should be called congenital upper eyelid eversion, grade IV (microphthalmos and orbital cyst) is a disease of the orbit. Grade III refers to true ectropion, because horizontal enlargement of superior and inferior eyelids (megaloblepharon).

Spectrum approach to congenital ectropion

“…(7) Lower eyelid is also rare and occurs in more severe cases with vertical deficiency of skin. (5,8) Main associations include difficult labor, male gender, Down syndrome, (9) collodion skin disease and lamellar ichthyosis. (3) Grandmultiparity and post-maturity have been questioned in Nigeria.…”

“…Many authors term it as congenital eyelid eversion and others as congenital ectropion. (5) Further, other authors describe a similar condition with intermittent eyelid eversion as congenital floppy eyelid. (6) Instead of this plethora of denominations, spectrum approach may join conditions that were previously considered separately.…”

Congenital upper eyelids ectropion in Down’s syndrome

“…Later, Gilbert and co-workers described two more cases associated with Down’s syndrome [ 5 ], [ 6 ]. This rare condition has been reported more frequently in black infants [ 1 ], [ 2 ], [ 3 ], [ 4 ], [ 7 ], [ 8 ] associated with ichthyosis [ 1 ], [ 4 ], [ 9 ], [ 10 ] and in infants with trisomy 21 [ 2 ], [ 5 ], [ 8 ], [ 11 ], [ 12 ], [ 13 ], [ 14 ]. Although the condition is generally bilateral and asymmetrical, some unilateral cases have been described [ 8 ], [ 11 ], [ 13 ].…”

“…Although the physiopathology of congenital upper eyelid ectropion is unknown, multiple factors have been implied, including absence of an effective lateral canthal ligaments, lateral elongation of the eyelid, hypotonia of the orbicularis, vertical shortening of the anterior lamella, and failure of the orbital septum to fuse with the levator aponeurosis [ 1 ], [ 2 ], [ 4 ], [ 6 ], [ 11 ], [ 12 ], [ 13 ], [ 14 ].…”

“…Surgical treatment for more severe cases that did not respond to conservative treatment include sub-conjunctival injection of hyaluronic acid [ 4 ], [ 8 ], [ 13 ], tarsorraphy [ 2 ], [ 3 ], [ 6 ], [ 7 ] tarsorraphy with excision of redundant conjunctiva [ 5 ], [ 7 ], fornix suture [ 3 ], [ 13 ], full-thickness skin graft [ 1 ], [ 2 ], [ 5 ], [ 11 ], full-thickness horizontal lid shortening [ 2 ], [ 6 ], and attachment of the orbital septum to the levator aponeurosis [ 2 ]. Most cases of congenital eversion of the eyelids without Down’s syndrome responded to patching or taping of the eyelids and the use of ointments [ 3 ], [ 6 ], [ 7 ], [ 11 ], [ 13 ], however surgical intervention may be necessary in patients with Down’s syndrome [ 6 ], [ 12 ], [ 14 ]. In our cases, the subconjunctival hyaluronic acid injection was not available at the moment and it was not likely to be effective due the lack of conjunctival chemosis.…”