Orbit, 2014; 33(1): 1–4 ! Informa UK Ltd. ISSN: 0167-6830 print / 1744-5108 online DOI: 10.3109/13506129.2013.841715
REVIEW
The Management of Congenital Superior Conjunctival Fornix Prolapse Apjit Kaur Chhabra1, Priyanka Singh1, Manisha1, Rolli Khurana1, and Khusboo Srivastav1 King George’s Medical University, Department of Ophthalmology, Lucknow, India
ABSTRACT Aim: To present the management of bilateral congenital superior fornicial prolapse and provide its up-to-date review of literature. Methods: We present the management of congenital bilateral superior fornicial prolapse treated conservatively with topical antibiotics, lubricants and hypertonic saline. One-week, 2-weeks and 1-month follow-up is presented. Results were assessed in terms of reduction of chemosis and eyelid opening. Updated current English peer-reviewed literature was researched. Results: The superior fornicial prolapse resolved with conservative management within 10 days, resulting in normal eyelid motility at the end of 1 month. At present 61 cases have been reported in English peer-reviewed journals. The proposed etiology is varied. Conservative management with topical medication provides relief. Conclusions: Congenital fornicial prolapse is a rare condition. Topical medication provides complete resolution of prolapse. Authors emphasise the need for awareness of condition among ophthalmologists and neonatologists, because of its gruesome appearance. Keywords: Fornix, congenital, conjunctiva
INTRODUCTION
with prolapse of both superior fornices. The baby was born of normal vaginal delivery to a 22-year-old primi gravida, without history of difficult labour or instrumentation. No history of maternal infection was elicited. Examination revealed bilateral everted upper eyelids with superior forniceal and palpebral conjunctival chemosis, so much so that lower lid and globe could not be visualised (Figure 2). On retracting the chemosed tissue, the lower lids, bulbar conjunctiva and globe were found to be within normal limits. No abnormality was detected on systemic examination. The child was treated with topical hourly lubricants (carboxymethylcellulose 1.0%), antibiotic eye drop (Tobramycin base USP 0.3%) for 3 times, padding with 6% hypertonic saline-soaked gauze dressing. At 1 week follow-up, the condition improved and the child could open his eyes partially. The lids could
20 14
Congenital conjunctival forniceal prolapse is a rare entity with a predilection for the superior fornix. The eyelid is completely everted and is associated with chemosis and conjunctival prolapse. The underlying cause for eversion remains obscure, but several possible mechanisms and associations have been proposed (summarized in Figure 1). The chemosed conjunctiva protects the cornea from exposure and thus corneal complications are rare.
CASE REPORT The authors declare adherence to the tenets of Declaration of Helsinki. A written informed voluntary consent was taken from the patient’s father. A 6-hourold male neonate presented to us in November 2012
Received 4 January 2013; Accepted 18 August 2013; Published online 18 October 2013 Correspondence: Dr. Manisha, Department of Ophthalmology, King George’s Medical University, Lucknow-226003, U.P., India
1
2
A. K. Chhabra et al.
FIGURE 1. Factors contributing to etiology of congenital prolapse of superior fornix.
FIGURE 2. Photographs of the male neonate showing bilateral superior fornix prolapse at the time of presentation.
be manually repositioned, but reverted on releasing. No change was advised in treatment regimen. On subsequent follow-up at 2 weeks, the condition improved further, and the mother reported spontaneous eye opening. Examination revealed absence of chemosis with near normal lid blinks. Hyperosmotic agents and antibiotic instillation was stopped, and topical lubricant eye drops was continued. At followup after 4 weeks, the lids retained their normal position and the child could fixate and follow light. The conjunctiva was healthy and the cornea was clear (Figure 3). All topical medications were discontinued.
FIGURE 3. Photograph of same child after 1 month of conservative treatment.
REVIEW OF LITERATURE Congenital superior fornicial prolapse was first reported by Adams1 in 1896, who used the term ‘double congenital ectropion’. Sellar2 in 1992, reviewed the literature and identified 51 cases. Since then, ten more cases have been reported.3,4,5,6,7,8,9 Congenital forniceal prolapse is a rare condition, as demonstrated by paucity of cases reported in the last century. Hence, its incidence remains unknown. Most of the cases were found to be sporadic. However, two cases had evidence of family history, suggesting that a few cases may be inherited Orbit
Congenital Fornix Prolapse and its Management autosomal dominantly.2 There was a male preponderance with a ratio of 5:2 (38 males and 15 females). The eversion is usually present at birth and is typically bilateral,10 but late onset and unilateral total eversion of the upper eyelid has also been reported.2,4,6,9 The racial distribution suggests that the condition occurs more frequently in African population, with maximum number of reported cases (37). Other documented reports include 17 Whites, 3 Asian Indians and 2 Arabs. Its association has also been reported with Down’s syndrome2 and collodion skin disease.11 The commonly proposed aetiology in the patients of Down’s syndrome is generalised tissue hyperelasticity.12 It has also been associated with combination of short anterior lid lamellae and congenital levator disinsertion.2 The mechanism is slightly different in Africans which can be explained as overlapping of the lower lids by the everted upper lids, which irritates the bulbar surface of upper lids to cause blepharospasm and upper lid eversion.13 This overlap usually resolves within a couple of weeks after birth. The role of grand multiparity and post maturity on the occurrence of this condition has been questioned by earlier work in Nigeria3,8 and is still doubtful. Other maternal factors such as caesarean section, maternal infection, parity also appears to be incidental to its occurrence. Most of the authors reported that their patients had uncomplicated births, with no significant antenatal and postnatal history. Two patients were born of difficult labour and three were born by caesarean section. One patient showed signs of neonatal sepsis at the time of presentation.4 Advanced maternal age (430 years) has been observed in many of the cases.2 Isolated lid abnormalities were found associated with several cases (specially white population) such as conjunctival folds, absent lacrimal puncta, absent meibomian glands, absent tarsus plate, distichiasis, epicanthal fold, diminished levator action and laxity of lids. Most cases were associated with normal eyeball; however few cases had corneal changes as reported by Sellar et al. in his clinical review. Both conservative and surgical therapeutic options have been reported in the literature. Conservative management includes padding with hypertonic saline, lubricant and antibiotic. The use of subconjunctival hyaluronidase has been mentioned by Bentsi-Enchill.13 Surgical management has been suggested for delayed presentations and involves tarsorrhaphy, fornix forming sutures and full thickness skin graft to the upper lid.3,8 Babies responded equally well with both the modalities of treatment. All the recent reports emphasize a trial of conservative management before considering the patient for surgery.4–7,9 !
2014 Informa Healthcare USA, Inc.
3
A normal anatomical outcome with good functional recovery can be effectively achieved using topical lubrication, antibiotics and application of 5% hypertonic saline. The mechanism of action of 6% hypertonic saline-soaked gauze dressing is movement of fluid from edematous tissues through the semi permeable conjunctival membrane by the process of osmosis which leads to resolution of the edema and subsequent lid reversion.14 This method is highly recommended because it is inexpensive and noninvasive. Surgical management should only be done in recalcitrant cases and patients presenting late to prevent keratinisation of conjunctiva, amblyopia.
CONCLUSION Superior conjunctival prolapse is a rare condition which presents with striking clinical features. The excellent response to medical treatment noted by these authors is similar to the few previously reported cases. Reassurance of the parents is important in the management, and awareness of the condition and its management among ophthalmologists and neonatologists will help to avoid unnecessary referrals and investigations.
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
REFERENCES 1. Adams AL. A case of double congenital ectropion. Med Fortnightly 1896;9:337–338. 2. Sellar PW, Bryars JH, Archer DB. Late presentation of congenital ectropion of the eyelids in a child with Downs’s syndrome: A case report and review of the literature. J Pediatr Ophthalmol Strabismus 1992;29: 64–67. 3. Ibechukwu BI, Iya CK. Early surgical management of two cases of bilateral congenital prolapse of the superior conjunctival fornices with lid eversion. West Afr J Med 1991 Jan–Mar;10(1):433–438. 4. Adeoti CO, Ashaye AO, Isawumi MA, et al. Non-surgical management of congenital eversion of the eyelids. J Ophthalmic Vis Res 2010;5(3):188–192. 5. Maheshwari R, Maheshwari S. Congenital eversion of upper eyelids: Case report and management. Ind J Ophthalmol 2006;54:203–204. 6. Pereira FJ, Trindade SDEP, Cruz AA, et al. Congenital ectropion: three case reports and literature review. Arq Bras Oftalmol 2007;70(1):149–152. 7. Isawumi MA, Adeoti CO, Umar IO, et al. Congenital bilateral eversion of the eyelids. J Pediatr Ophthalmol Strabismus 2008;45(6):371–373. 8. Dawodu OA. Total eversion of the upper eyelids in a newborn. Niger Postgrad Med J 2001;8(3):145–147.
4
A. K. Chhabra et al.
9. Ren L, Liu J. Congenital total ectropion of upper lid. Vestn Oftalmol 1999;115:32–34. 10. Duke-Elder S. Congenital deformities. In: DukeElder S (ed). System of Ophthalmology. Vol 3. St. Louis: CV Mosby; 1963:866–867. 11. Shapiro RD, Soentgen ML. Collodion skin disease and everted eyelids. Postgrad Med 1969;45:216–219.
12. Sutture von H, Gottesleben HU. Konnatales Ektropium der Oberlinderund Mongolignus. Klin Monatsbl Augenheilkd 1967;150:552–556. 13. Bentsi-Enchill KO. Congenital total eversion of upper eyelids. Br J Ophthalmol 1981;65:209–213. 14. Voet D, Voet JG, Pratt CW. Fundamentals of Biochemistry. New York: John Wiley & Sons; 1999. p. 30.
Orbit
Copyright of Orbit is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
REVIEW
The Management of Congenital Superior Conjunctival Fornix Prolapse Apjit Kaur Chhabra1, Priyanka Singh1, Manisha1, Rolli Khurana1, and Khusboo Srivastav1 King George’s Medical University, Department of Ophthalmology, Lucknow, India
ABSTRACT Aim: To present the management of bilateral congenital superior fornicial prolapse and provide its up-to-date review of literature. Methods: We present the management of congenital bilateral superior fornicial prolapse treated conservatively with topical antibiotics, lubricants and hypertonic saline. One-week, 2-weeks and 1-month follow-up is presented. Results were assessed in terms of reduction of chemosis and eyelid opening. Updated current English peer-reviewed literature was researched. Results: The superior fornicial prolapse resolved with conservative management within 10 days, resulting in normal eyelid motility at the end of 1 month. At present 61 cases have been reported in English peer-reviewed journals. The proposed etiology is varied. Conservative management with topical medication provides relief. Conclusions: Congenital fornicial prolapse is a rare condition. Topical medication provides complete resolution of prolapse. Authors emphasise the need for awareness of condition among ophthalmologists and neonatologists, because of its gruesome appearance. Keywords: Fornix, congenital, conjunctiva
INTRODUCTION
with prolapse of both superior fornices. The baby was born of normal vaginal delivery to a 22-year-old primi gravida, without history of difficult labour or instrumentation. No history of maternal infection was elicited. Examination revealed bilateral everted upper eyelids with superior forniceal and palpebral conjunctival chemosis, so much so that lower lid and globe could not be visualised (Figure 2). On retracting the chemosed tissue, the lower lids, bulbar conjunctiva and globe were found to be within normal limits. No abnormality was detected on systemic examination. The child was treated with topical hourly lubricants (carboxymethylcellulose 1.0%), antibiotic eye drop (Tobramycin base USP 0.3%) for 3 times, padding with 6% hypertonic saline-soaked gauze dressing. At 1 week follow-up, the condition improved and the child could open his eyes partially. The lids could
20 14
Congenital conjunctival forniceal prolapse is a rare entity with a predilection for the superior fornix. The eyelid is completely everted and is associated with chemosis and conjunctival prolapse. The underlying cause for eversion remains obscure, but several possible mechanisms and associations have been proposed (summarized in Figure 1). The chemosed conjunctiva protects the cornea from exposure and thus corneal complications are rare.
CASE REPORT The authors declare adherence to the tenets of Declaration of Helsinki. A written informed voluntary consent was taken from the patient’s father. A 6-hourold male neonate presented to us in November 2012
Received 4 January 2013; Accepted 18 August 2013; Published online 18 October 2013 Correspondence: Dr. Manisha, Department of Ophthalmology, King George’s Medical University, Lucknow-226003, U.P., India
1
2
A. K. Chhabra et al.
FIGURE 1. Factors contributing to etiology of congenital prolapse of superior fornix.
FIGURE 2. Photographs of the male neonate showing bilateral superior fornix prolapse at the time of presentation.
be manually repositioned, but reverted on releasing. No change was advised in treatment regimen. On subsequent follow-up at 2 weeks, the condition improved further, and the mother reported spontaneous eye opening. Examination revealed absence of chemosis with near normal lid blinks. Hyperosmotic agents and antibiotic instillation was stopped, and topical lubricant eye drops was continued. At followup after 4 weeks, the lids retained their normal position and the child could fixate and follow light. The conjunctiva was healthy and the cornea was clear (Figure 3). All topical medications were discontinued.
FIGURE 3. Photograph of same child after 1 month of conservative treatment.
REVIEW OF LITERATURE Congenital superior fornicial prolapse was first reported by Adams1 in 1896, who used the term ‘double congenital ectropion’. Sellar2 in 1992, reviewed the literature and identified 51 cases. Since then, ten more cases have been reported.3,4,5,6,7,8,9 Congenital forniceal prolapse is a rare condition, as demonstrated by paucity of cases reported in the last century. Hence, its incidence remains unknown. Most of the cases were found to be sporadic. However, two cases had evidence of family history, suggesting that a few cases may be inherited Orbit
Congenital Fornix Prolapse and its Management autosomal dominantly.2 There was a male preponderance with a ratio of 5:2 (38 males and 15 females). The eversion is usually present at birth and is typically bilateral,10 but late onset and unilateral total eversion of the upper eyelid has also been reported.2,4,6,9 The racial distribution suggests that the condition occurs more frequently in African population, with maximum number of reported cases (37). Other documented reports include 17 Whites, 3 Asian Indians and 2 Arabs. Its association has also been reported with Down’s syndrome2 and collodion skin disease.11 The commonly proposed aetiology in the patients of Down’s syndrome is generalised tissue hyperelasticity.12 It has also been associated with combination of short anterior lid lamellae and congenital levator disinsertion.2 The mechanism is slightly different in Africans which can be explained as overlapping of the lower lids by the everted upper lids, which irritates the bulbar surface of upper lids to cause blepharospasm and upper lid eversion.13 This overlap usually resolves within a couple of weeks after birth. The role of grand multiparity and post maturity on the occurrence of this condition has been questioned by earlier work in Nigeria3,8 and is still doubtful. Other maternal factors such as caesarean section, maternal infection, parity also appears to be incidental to its occurrence. Most of the authors reported that their patients had uncomplicated births, with no significant antenatal and postnatal history. Two patients were born of difficult labour and three were born by caesarean section. One patient showed signs of neonatal sepsis at the time of presentation.4 Advanced maternal age (430 years) has been observed in many of the cases.2 Isolated lid abnormalities were found associated with several cases (specially white population) such as conjunctival folds, absent lacrimal puncta, absent meibomian glands, absent tarsus plate, distichiasis, epicanthal fold, diminished levator action and laxity of lids. Most cases were associated with normal eyeball; however few cases had corneal changes as reported by Sellar et al. in his clinical review. Both conservative and surgical therapeutic options have been reported in the literature. Conservative management includes padding with hypertonic saline, lubricant and antibiotic. The use of subconjunctival hyaluronidase has been mentioned by Bentsi-Enchill.13 Surgical management has been suggested for delayed presentations and involves tarsorrhaphy, fornix forming sutures and full thickness skin graft to the upper lid.3,8 Babies responded equally well with both the modalities of treatment. All the recent reports emphasize a trial of conservative management before considering the patient for surgery.4–7,9 !
2014 Informa Healthcare USA, Inc.
3
A normal anatomical outcome with good functional recovery can be effectively achieved using topical lubrication, antibiotics and application of 5% hypertonic saline. The mechanism of action of 6% hypertonic saline-soaked gauze dressing is movement of fluid from edematous tissues through the semi permeable conjunctival membrane by the process of osmosis which leads to resolution of the edema and subsequent lid reversion.14 This method is highly recommended because it is inexpensive and noninvasive. Surgical management should only be done in recalcitrant cases and patients presenting late to prevent keratinisation of conjunctiva, amblyopia.
CONCLUSION Superior conjunctival prolapse is a rare condition which presents with striking clinical features. The excellent response to medical treatment noted by these authors is similar to the few previously reported cases. Reassurance of the parents is important in the management, and awareness of the condition and its management among ophthalmologists and neonatologists will help to avoid unnecessary referrals and investigations.
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
REFERENCES 1. Adams AL. A case of double congenital ectropion. Med Fortnightly 1896;9:337–338. 2. Sellar PW, Bryars JH, Archer DB. Late presentation of congenital ectropion of the eyelids in a child with Downs’s syndrome: A case report and review of the literature. J Pediatr Ophthalmol Strabismus 1992;29: 64–67. 3. Ibechukwu BI, Iya CK. Early surgical management of two cases of bilateral congenital prolapse of the superior conjunctival fornices with lid eversion. West Afr J Med 1991 Jan–Mar;10(1):433–438. 4. Adeoti CO, Ashaye AO, Isawumi MA, et al. Non-surgical management of congenital eversion of the eyelids. J Ophthalmic Vis Res 2010;5(3):188–192. 5. Maheshwari R, Maheshwari S. Congenital eversion of upper eyelids: Case report and management. Ind J Ophthalmol 2006;54:203–204. 6. Pereira FJ, Trindade SDEP, Cruz AA, et al. Congenital ectropion: three case reports and literature review. Arq Bras Oftalmol 2007;70(1):149–152. 7. Isawumi MA, Adeoti CO, Umar IO, et al. Congenital bilateral eversion of the eyelids. J Pediatr Ophthalmol Strabismus 2008;45(6):371–373. 8. Dawodu OA. Total eversion of the upper eyelids in a newborn. Niger Postgrad Med J 2001;8(3):145–147.
4
A. K. Chhabra et al.
9. Ren L, Liu J. Congenital total ectropion of upper lid. Vestn Oftalmol 1999;115:32–34. 10. Duke-Elder S. Congenital deformities. In: DukeElder S (ed). System of Ophthalmology. Vol 3. St. Louis: CV Mosby; 1963:866–867. 11. Shapiro RD, Soentgen ML. Collodion skin disease and everted eyelids. Postgrad Med 1969;45:216–219.
12. Sutture von H, Gottesleben HU. Konnatales Ektropium der Oberlinderund Mongolignus. Klin Monatsbl Augenheilkd 1967;150:552–556. 13. Bentsi-Enchill KO. Congenital total eversion of upper eyelids. Br J Ophthalmol 1981;65:209–213. 14. Voet D, Voet JG, Pratt CW. Fundamentals of Biochemistry. New York: John Wiley & Sons; 1999. p. 30.
Orbit
Copyright of Orbit is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
