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Conservative management of congenital upper eyelid eversion
Journal Pre-proof Conservative management of congenital upper eyelid eversion Peter Daniel, MD, Martin Cogen, MD PII:
S1091-8531(19)30568-3
DOI:
https://doi.org/10.1016/j.jaapos.2019.10.005
Reference:
YMPA 3111
To appear in:
Journal of AAPOS
Received Date: 1 July 2019 Revised Date:
9 October 2019
Accepted Date: 29 October 2019
Please cite this article as: Daniel P, Cogen M, Conservative management of congenital upper eyelid eversion, Journal of AAPOS (2020), doi: https://doi.org/10.1016/j.jaapos.2019.10.005. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. Copyright © 2019, American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.
Conservative management of congenital upper eyelid eversion Peter Daniel, MD, and Martin Cogen, MD Author affiliations: Department of Ophthalmology, School of Medicine, University of Alabama at Birmingham Financial support: Research to Prevent Blindness, New York, NY, and the Department of Ophthalmology and Visual Sciences at the University of Alabama at Birmingham. Submitted July 1, 2019. Revision accepted October 29, 2019. Correspondence: Peter Daniel, 311 Twin Lake Rd, Greenville, SC 29609 (email: [email protected]). Word count: 1,032
We report 2 cases of congenital upper eyelid eversion and highlight nonsurgical treatment options, including a novel approach. Congenital upper eyelid eversion (CUEE) is a rare congenital condition characterized by completely everted upper eyelids, with prominent chemosis. It can lead to significant ocular morbidity, including exposure keratopathy, corneal scarring, and perforation.1-3 We report 2 cases of CUEE treated at the University of Alabama at Birmingham and discuss therapeutic options, including nonsurgical treatment. Case 1 An otherwise healthy 1-day-old African American girl was referred from an outside hospital for bilateral CUEE after an uneventful Caesarian delivery performed for nonreassuring fetal heart tracings at approximately 38 weeks’ gestation. The mother had no prenatal care, but all of her lab tests were normal. On ophthalmological examination, both upper eyelids were everted, with prominent conjunctival hyperemia and chemosis (Figure 1A). The remainder of the examination was otherwise unremarkable. Attempts to manually reposition the eyelids were unsuccessful. The patient was started on a topical antibiotic-corticosteroid ophthalmic ointment, for lubrication, infection prophylaxis, and chemosis reduction. To osmotically decrease the chemosis, saline eyedrops 5% were instilled every 2 hours, and saline-soaked gauze pads were applied for 1 hour three times daily. Within 3 days, the chemosis in the left eye had diminished sufficiently to permit manual repositioning of the eyelid, at which point the topical hypertonic saline was discontinued, and the left upper lid was taped closed using a hydrocolloidal dressing. Four days later, the left eyelid remained in normal anatomic position and no longer needed to be taped (Figure 1B). However, the right eye failed to improve after 7 days, and 6 doses of systemic dexamethasone were administered (0.9 mg total, orally four times daily at 0.05 mg/kg); a steroid
taper was not indicated. Within 2 days, the right eye improved dramatically, with marked reduction of the conjunctival chemosis, allowing manual repositioning of the eyelid (Figure 1CD). The repositioned right eyelid was taped closed. By day 13, both upper lids were anatomically normal, without mechanical support or medical therapy. At 2 months’ follow-up, both eyelids were normal, and there were no ocular sequelae. Case 2 An otherwise healthy 4-day-old African American boy was transferred from an outside hospital for treatment of bilateral CUEE. The patient was born at 39 weeks’ gestation via spontaneous vaginal delivery. He had been started on topical 5% saline drops in addition to topical antibioticcorticosteroid drops prior to transfer. Aside from everted, chemotic upper eyelids, the examination was normal. The eyelids could be manually repositioned and were taped shut. Three days later, the eyelids remained in normal anatomic position without mechanical support. Treatment was discontinued, and the patient had no ocular sequelae. Discussion Sellar and colleagues3 described 51 patients with congenital upper eyelid eversion and noted that whereas the majority of cases had no systemic association, a higher prevalence was seen in patients with colloidal skin disease, Down syndrome, and African ancestry. Most cases are bilateral, although unilateral cases have been reported. Patients usually reach medical attention shortly after birth. Delayed presentation is associated with an increased risk of serious corneal complications, including ulceration and perforation.1,3,4 Although the exact cause of CUEE is not known, birth trauma has been proposed as a possible mechanism. Although most infants with CUEE are born vaginally, the fact that cases have been reported following Caesarian section casts doubt on trauma as the sole etiology. It has also been hypothesized that CUEE arises from
orbicularis hypotonia, vertical elongation of the posterior lamella or shortening of the anterior lamella of the upper eyelid, or failure of fusion between the orbital septum and levator aponeurosis.1,2,4,5 However, histopathologic examination of a deceased 9-day-old infant with CUEE failed to show any anatomic lid abnormalities to explain the condition.6 The venous stasis theory proposes that vascular congestion within the eyelid causes chemosis, which swells the lid tissues enough to cause lid eversion. The lid eversion triggers orbicularis spasm, which further exacerbates the venous stasis and chemosis, thereby leading to a vicious cycle of worsening lid eversion. Nonoperative treatment of CUEE is aimed at reducing the conjunctival chemosis sufficiently to allow anatomic repositioning of the upper eyelids. Application of hypertonic agents to osmotically dehydrate the swollen conjunctiva using saline solution and/or ointment results in improvement within a week in most cases and complete resolution in approximately 1 month.1,2,4,5,7,8 A topical antibiotic is added as an adjunct treatment to decrease the risk of infection of the exposed ocular surface. To help address any inflammatory component of the subconjunctival edema, topical corticosteroids may be added as well. Witherspoon and colleagues9 described the effectiveness of subconjunctival injection of a combination corticosteroid and lidocaine with epinephrine for persistent postoperative conjunctival chemosis resistant to topical therapy. The authors felt that the epinephrine augmented the corticosteroid-sensitized vasoconstriction. Corticosteroid effects on vasculature include vasoconstriction due to reduced prostacycline production, increased alpha-adrenergic receptors, and inhibition of nitric oxide synthetase. This led us to speculate that the addition of a systemic corticosteroid in case 1 might be helpful. We decided the systemic route was less risky than periocular injection in a neonate, and the improvement was dramatic; to our knowledge, this
is the first reported use of systemic steroids to address this condition. In cases where conservative management failed, surgical procedures, such as tarsorrhaphy, fornix fixation sutures, subconjunctival injection of hyaluronic acid, mattress sutures to compress the eyelid tissues, or full-thickness skin grafts of the upper eyelids have been reported.3,5 However, most patients respond fully to appropriate conservative management; surgery should be reserved for truly refractory cases. Caution is advised: in 1 reported case lid manipulation resulted in respiratory arrest.10 Based on available evidence and our own experience, most infants with CUEE can have excellent anatomic and functional results if managed promptly with topical hypertonic therapy combined with topical antibiotic-corticosteroid therapy. A trial of systemic corticosteroid may help in resistant cases. Prompt management of CUEE is necessary to restore proper eyelid position and prevent potentially blinding sequelae. Literature Search PubMed, Library of Congress, and LISTA were searched for English-language results in June 2019. Search terms included eyelid eversion, congenital eyelid eversion, congenital ectropion, and congenital upper eyelid eversion.
References 1.
Dohvoma VA, Nchifor A, Ngwanou AN, et al. Conservative management in congenital bilateral upper eyelid eversion. Case Rep Ophthalmol Med 2015:2015: 389289.
2.
Maheshwari R, Maheshwari S. Congenital eversion of upper eyelids: case report and management. Indian J Ophthalmol 2006;54:203-4.
3.
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.
4.
Ibraheem WA. Bilateral congenital upper eyelid eversion: the clinical course and outcome of conservative management. Pan Afr Med J 2014;17:215.
5.
Krishnappa NC, Deb AK, Poddar C. Congenital total eversion of upper eyelids in a newborn with Down’s syndrome. Oman J Ophthalmol 2014;7:98-9.
6.
Young RJ. Congenital ectropion of the upper lids. Arch Dis Child 1954;29:97-100.
7.
Kirkpatrick A, Ledlow D, Dixon E, Philips JB Congenital bilateral eyelid eversion and chemosis: a case study. Neonatal Netw 2018;37:137-40.
8.
Shinder R, Langer PD. Unilateral congenital eyelid eversion causing marked chemosis in a newborn. J Pediatr Ophthalmol Strabismus 2011;48 online:e1-2.
9.
Witherspoon CD, Cogen MS, Kuhn F. Chemotic prolapse of the conjunctiva. Ophthalmic Surg 1991 22:241-2.
10.
Watts MT, Dapling RB. Congenital eversion of the upper eyelid: a case report. Ophthal Plast Reconstr Surg 1995;11:293-5.
Legends FIG 1. Clinical photographs of case 1. A, On presentation. B, After 5 days of topical therapy, resulting in resolution of eyelid edema in the left eye, refractory chemosis in the right eye. C, Day 10 of treatment, showing prompt resolution of chemosis in the right eye following systemic corticosteroid. D, Day 11 of treatment, showing anatomic repositioning of right eyelid using a hydrocolloidal dressing.
S1091-8531(19)30568-3
DOI:
https://doi.org/10.1016/j.jaapos.2019.10.005
Reference:
YMPA 3111
To appear in:
Journal of AAPOS
Received Date: 1 July 2019 Revised Date:
9 October 2019
Accepted Date: 29 October 2019
Please cite this article as: Daniel P, Cogen M, Conservative management of congenital upper eyelid eversion, Journal of AAPOS (2020), doi: https://doi.org/10.1016/j.jaapos.2019.10.005. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. Copyright © 2019, American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.
Conservative management of congenital upper eyelid eversion Peter Daniel, MD, and Martin Cogen, MD Author affiliations: Department of Ophthalmology, School of Medicine, University of Alabama at Birmingham Financial support: Research to Prevent Blindness, New York, NY, and the Department of Ophthalmology and Visual Sciences at the University of Alabama at Birmingham. Submitted July 1, 2019. Revision accepted October 29, 2019. Correspondence: Peter Daniel, 311 Twin Lake Rd, Greenville, SC 29609 (email: [email protected]). Word count: 1,032
We report 2 cases of congenital upper eyelid eversion and highlight nonsurgical treatment options, including a novel approach. Congenital upper eyelid eversion (CUEE) is a rare congenital condition characterized by completely everted upper eyelids, with prominent chemosis. It can lead to significant ocular morbidity, including exposure keratopathy, corneal scarring, and perforation.1-3 We report 2 cases of CUEE treated at the University of Alabama at Birmingham and discuss therapeutic options, including nonsurgical treatment. Case 1 An otherwise healthy 1-day-old African American girl was referred from an outside hospital for bilateral CUEE after an uneventful Caesarian delivery performed for nonreassuring fetal heart tracings at approximately 38 weeks’ gestation. The mother had no prenatal care, but all of her lab tests were normal. On ophthalmological examination, both upper eyelids were everted, with prominent conjunctival hyperemia and chemosis (Figure 1A). The remainder of the examination was otherwise unremarkable. Attempts to manually reposition the eyelids were unsuccessful. The patient was started on a topical antibiotic-corticosteroid ophthalmic ointment, for lubrication, infection prophylaxis, and chemosis reduction. To osmotically decrease the chemosis, saline eyedrops 5% were instilled every 2 hours, and saline-soaked gauze pads were applied for 1 hour three times daily. Within 3 days, the chemosis in the left eye had diminished sufficiently to permit manual repositioning of the eyelid, at which point the topical hypertonic saline was discontinued, and the left upper lid was taped closed using a hydrocolloidal dressing. Four days later, the left eyelid remained in normal anatomic position and no longer needed to be taped (Figure 1B). However, the right eye failed to improve after 7 days, and 6 doses of systemic dexamethasone were administered (0.9 mg total, orally four times daily at 0.05 mg/kg); a steroid
taper was not indicated. Within 2 days, the right eye improved dramatically, with marked reduction of the conjunctival chemosis, allowing manual repositioning of the eyelid (Figure 1CD). The repositioned right eyelid was taped closed. By day 13, both upper lids were anatomically normal, without mechanical support or medical therapy. At 2 months’ follow-up, both eyelids were normal, and there were no ocular sequelae. Case 2 An otherwise healthy 4-day-old African American boy was transferred from an outside hospital for treatment of bilateral CUEE. The patient was born at 39 weeks’ gestation via spontaneous vaginal delivery. He had been started on topical 5% saline drops in addition to topical antibioticcorticosteroid drops prior to transfer. Aside from everted, chemotic upper eyelids, the examination was normal. The eyelids could be manually repositioned and were taped shut. Three days later, the eyelids remained in normal anatomic position without mechanical support. Treatment was discontinued, and the patient had no ocular sequelae. Discussion Sellar and colleagues3 described 51 patients with congenital upper eyelid eversion and noted that whereas the majority of cases had no systemic association, a higher prevalence was seen in patients with colloidal skin disease, Down syndrome, and African ancestry. Most cases are bilateral, although unilateral cases have been reported. Patients usually reach medical attention shortly after birth. Delayed presentation is associated with an increased risk of serious corneal complications, including ulceration and perforation.1,3,4 Although the exact cause of CUEE is not known, birth trauma has been proposed as a possible mechanism. Although most infants with CUEE are born vaginally, the fact that cases have been reported following Caesarian section casts doubt on trauma as the sole etiology. It has also been hypothesized that CUEE arises from
orbicularis hypotonia, vertical elongation of the posterior lamella or shortening of the anterior lamella of the upper eyelid, or failure of fusion between the orbital septum and levator aponeurosis.1,2,4,5 However, histopathologic examination of a deceased 9-day-old infant with CUEE failed to show any anatomic lid abnormalities to explain the condition.6 The venous stasis theory proposes that vascular congestion within the eyelid causes chemosis, which swells the lid tissues enough to cause lid eversion. The lid eversion triggers orbicularis spasm, which further exacerbates the venous stasis and chemosis, thereby leading to a vicious cycle of worsening lid eversion. Nonoperative treatment of CUEE is aimed at reducing the conjunctival chemosis sufficiently to allow anatomic repositioning of the upper eyelids. Application of hypertonic agents to osmotically dehydrate the swollen conjunctiva using saline solution and/or ointment results in improvement within a week in most cases and complete resolution in approximately 1 month.1,2,4,5,7,8 A topical antibiotic is added as an adjunct treatment to decrease the risk of infection of the exposed ocular surface. To help address any inflammatory component of the subconjunctival edema, topical corticosteroids may be added as well. Witherspoon and colleagues9 described the effectiveness of subconjunctival injection of a combination corticosteroid and lidocaine with epinephrine for persistent postoperative conjunctival chemosis resistant to topical therapy. The authors felt that the epinephrine augmented the corticosteroid-sensitized vasoconstriction. Corticosteroid effects on vasculature include vasoconstriction due to reduced prostacycline production, increased alpha-adrenergic receptors, and inhibition of nitric oxide synthetase. This led us to speculate that the addition of a systemic corticosteroid in case 1 might be helpful. We decided the systemic route was less risky than periocular injection in a neonate, and the improvement was dramatic; to our knowledge, this
is the first reported use of systemic steroids to address this condition. In cases where conservative management failed, surgical procedures, such as tarsorrhaphy, fornix fixation sutures, subconjunctival injection of hyaluronic acid, mattress sutures to compress the eyelid tissues, or full-thickness skin grafts of the upper eyelids have been reported.3,5 However, most patients respond fully to appropriate conservative management; surgery should be reserved for truly refractory cases. Caution is advised: in 1 reported case lid manipulation resulted in respiratory arrest.10 Based on available evidence and our own experience, most infants with CUEE can have excellent anatomic and functional results if managed promptly with topical hypertonic therapy combined with topical antibiotic-corticosteroid therapy. A trial of systemic corticosteroid may help in resistant cases. Prompt management of CUEE is necessary to restore proper eyelid position and prevent potentially blinding sequelae. Literature Search PubMed, Library of Congress, and LISTA were searched for English-language results in June 2019. Search terms included eyelid eversion, congenital eyelid eversion, congenital ectropion, and congenital upper eyelid eversion.
References 1.
Dohvoma VA, Nchifor A, Ngwanou AN, et al. Conservative management in congenital bilateral upper eyelid eversion. Case Rep Ophthalmol Med 2015:2015: 389289.
2.
Maheshwari R, Maheshwari S. Congenital eversion of upper eyelids: case report and management. Indian J Ophthalmol 2006;54:203-4.
3.
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.
4.
Ibraheem WA. Bilateral congenital upper eyelid eversion: the clinical course and outcome of conservative management. Pan Afr Med J 2014;17:215.
5.
Krishnappa NC, Deb AK, Poddar C. Congenital total eversion of upper eyelids in a newborn with Down’s syndrome. Oman J Ophthalmol 2014;7:98-9.
6.
Young RJ. Congenital ectropion of the upper lids. Arch Dis Child 1954;29:97-100.
7.
Kirkpatrick A, Ledlow D, Dixon E, Philips JB Congenital bilateral eyelid eversion and chemosis: a case study. Neonatal Netw 2018;37:137-40.
8.
Shinder R, Langer PD. Unilateral congenital eyelid eversion causing marked chemosis in a newborn. J Pediatr Ophthalmol Strabismus 2011;48 online:e1-2.
9.
Witherspoon CD, Cogen MS, Kuhn F. Chemotic prolapse of the conjunctiva. Ophthalmic Surg 1991 22:241-2.
10.
Watts MT, Dapling RB. Congenital eversion of the upper eyelid: a case report. Ophthal Plast Reconstr Surg 1995;11:293-5.
Legends FIG 1. Clinical photographs of case 1. A, On presentation. B, After 5 days of topical therapy, resulting in resolution of eyelid edema in the left eye, refractory chemosis in the right eye. C, Day 10 of treatment, showing prompt resolution of chemosis in the right eye following systemic corticosteroid. D, Day 11 of treatment, showing anatomic repositioning of right eyelid using a hydrocolloidal dressing.