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Congenital total eversion of upper eyelids in a newborn with Down’s syndrome Nagesha Chokkahalli Krishnappa, Ashish Kumar Deb, Chanchal Poddar Department of Ophthalmology, Silchar Medical College and Hospital, Silchar, Assam, India
Description
eye [Figure 5]. At the end of 5th week, the child achieved normal eyelids with complete eye opening in both the eyes. [Figure 6].
A six-day-old female neonate was seen with parents complaining of fleshy protrusion of the lids and inability to open both eyes since birth. The mother was a 24-year-old-gravida 2 para 1. The full-term infant weighing 1.985 kg was born after prolonged labor of more than 20 hrs but without any instrumentation. Ocular examination revealed eversion of both upper eyelids and severe conjunctival chemosis with prolapse of forniceal conjunctiva [Figure 1]. The lid could not be repositioned to the normal position, even with pressure. Cornea was clear with brisk pupillary reactions. Pediatric assessment revealed that the child had peripheral stigmata of Down’s syndrome like Single transverse palmar crease and short 5th digit with clinodactyly and joint hyperflexibility [Figures 2 and 3]. A diagnosis of bilateral congenital eversion with Down’s syndrome was made. The child was put on 5% hypertonic saline two hrly, moxifloxacin 0.5% eye drops six times a day, carboxymethyl cellulose six times a day, and Tobramycin eye ointment two times a day and padding of the eyelids with 5% hypertonic saline-soaked gauze dressing.
Figure 1: Six-days-old baby - Note conjunctival eversion with severe chemosis
Figure 2: Single transverse palmar crease and short 5th digit with clinodactyly
At 2nd week follow-up, condition improved and child could open her left eye partially with regression of prolapsed conjunctiva. In the right eye, condition persisted but was of less severity. [Figure 4]. By the end of 4th week, there was complete resolution in the left eye but incomplete resolution in the right Access this article online Quick Response Code: Website: www.ojoonline.org DOI: 10.4103/0974-620X.137174 Figure 3: Joint hyperflexibility
Copyright: © 2014 Krishnappa CK, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Correspondence: Dr. Nagesha Chokkahalli Krishnappa, Department of Ophthalmology, Sri Devaraj Urs Medical college, Kolar, India. E-mail: [email protected]
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Oman Journal of Ophthalmology, Vol. 7, No. 2, 2014
Krishnappa, et al.: Congenital total eversion in a newborn with Down’s syndrome with the levator aponeurosis, absence of an effective lateral canthal ligament, and lateral elongation of the eyelids.[1]
Figure 4: 2nd week follow-up: Partial regression of eversion
Venous stasis during delivery also caused marked chemosis and prolapse of the conjunctiva, leading to eversion of the eyelids.[2] The conjunctival chemosis protects the cornea from exposure and hence corneal complications are rare. Congenital eyelid eversion can be treated conservatively by topical lubrication, antibiotics, and hypertonic saline[3] or surgically by lid sutures, sub-conjunctival hyaluronidase, and eye padding.[4] Our case was managed by topical antibiotics, lubricants, hypertonic saline, and padding where improvement was observed within one week and complete resolution occurred in five weeks.
Figure 5: 4th week follow-up: Complete resolution of eversion in LE
Although both minor and major surgical procedures have been advocated, their efficacy must be questioned because conservative measures appear very successful as shown in this case. To conclude, despite the alarming presentation of congenital eyelid eversion, its benign course justifies a conservative approach in anticipation of an excellent result.
Figure 6: 5th week end: Complete resolution of eversion in BE
References 1.
Dilated fundoscopy with 0.5% tropicamide drops disclosed normal fundus. Congenital eyelid eversion is reported to be very rare. The incidence appears higher in black infants, infants with trisomy 21, and infants born with collodion skin disease. Several factors have been implicated in its pathophysiology including orbicularis oculi hypotonia, birth trauma, vertical shortening of anterior lamella or vertical elongation of posterior lamella of the eyelids with failure of the orbital septum to fuse
Oman Journal of Ophthalmology, Vol. 7, No. 2, 2014
2.
3. 4.
Al Hussain HA, AI-Rajhi AA, AI-Qahtani S, Meyer D. Congenital upper eyelid eversion complicated by corneal perforation. Br J Ophthalmol 2005;89:771. Conlon MR, Sutula FC. Congenital eyelid anomalies. In Albert DM, Jakobiac FA (editors): Principles and Practice of Ophthalmology, Vol. 4. 2nd ed. Philadelphia, PA: WB Saunders Co; 1994. Adeoti CO, Ashaye AO, Isawumi MA, Raji RA. Nonsurgical management of congenital eversion of the eyelids. J Ophthalmic Vis Res 2010;5:188-92. Bentsi-Enchill KO. Congenital total eversion of the upper eyelids. Br J Ophthalmol-1981;65:209-13.
Cite this article as: 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. Source of Support: Nil, Conflict of Interest: None declared.
99
Copyright of Oman Journal of Ophthalmology is the property of Medknow Publications & Media Pvt. 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.
Congenital total eversion of upper eyelids in a newborn with Down’s syndrome Nagesha Chokkahalli Krishnappa, Ashish Kumar Deb, Chanchal Poddar Department of Ophthalmology, Silchar Medical College and Hospital, Silchar, Assam, India
Description
eye [Figure 5]. At the end of 5th week, the child achieved normal eyelids with complete eye opening in both the eyes. [Figure 6].
A six-day-old female neonate was seen with parents complaining of fleshy protrusion of the lids and inability to open both eyes since birth. The mother was a 24-year-old-gravida 2 para 1. The full-term infant weighing 1.985 kg was born after prolonged labor of more than 20 hrs but without any instrumentation. Ocular examination revealed eversion of both upper eyelids and severe conjunctival chemosis with prolapse of forniceal conjunctiva [Figure 1]. The lid could not be repositioned to the normal position, even with pressure. Cornea was clear with brisk pupillary reactions. Pediatric assessment revealed that the child had peripheral stigmata of Down’s syndrome like Single transverse palmar crease and short 5th digit with clinodactyly and joint hyperflexibility [Figures 2 and 3]. A diagnosis of bilateral congenital eversion with Down’s syndrome was made. The child was put on 5% hypertonic saline two hrly, moxifloxacin 0.5% eye drops six times a day, carboxymethyl cellulose six times a day, and Tobramycin eye ointment two times a day and padding of the eyelids with 5% hypertonic saline-soaked gauze dressing.
Figure 1: Six-days-old baby - Note conjunctival eversion with severe chemosis
Figure 2: Single transverse palmar crease and short 5th digit with clinodactyly
At 2nd week follow-up, condition improved and child could open her left eye partially with regression of prolapsed conjunctiva. In the right eye, condition persisted but was of less severity. [Figure 4]. By the end of 4th week, there was complete resolution in the left eye but incomplete resolution in the right Access this article online Quick Response Code: Website: www.ojoonline.org DOI: 10.4103/0974-620X.137174 Figure 3: Joint hyperflexibility
Copyright: © 2014 Krishnappa CK, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Correspondence: Dr. Nagesha Chokkahalli Krishnappa, Department of Ophthalmology, Sri Devaraj Urs Medical college, Kolar, India. E-mail: [email protected]
98
Oman Journal of Ophthalmology, Vol. 7, No. 2, 2014
Krishnappa, et al.: Congenital total eversion in a newborn with Down’s syndrome with the levator aponeurosis, absence of an effective lateral canthal ligament, and lateral elongation of the eyelids.[1]
Figure 4: 2nd week follow-up: Partial regression of eversion
Venous stasis during delivery also caused marked chemosis and prolapse of the conjunctiva, leading to eversion of the eyelids.[2] The conjunctival chemosis protects the cornea from exposure and hence corneal complications are rare. Congenital eyelid eversion can be treated conservatively by topical lubrication, antibiotics, and hypertonic saline[3] or surgically by lid sutures, sub-conjunctival hyaluronidase, and eye padding.[4] Our case was managed by topical antibiotics, lubricants, hypertonic saline, and padding where improvement was observed within one week and complete resolution occurred in five weeks.
Figure 5: 4th week follow-up: Complete resolution of eversion in LE
Although both minor and major surgical procedures have been advocated, their efficacy must be questioned because conservative measures appear very successful as shown in this case. To conclude, despite the alarming presentation of congenital eyelid eversion, its benign course justifies a conservative approach in anticipation of an excellent result.
Figure 6: 5th week end: Complete resolution of eversion in BE
References 1.
Dilated fundoscopy with 0.5% tropicamide drops disclosed normal fundus. Congenital eyelid eversion is reported to be very rare. The incidence appears higher in black infants, infants with trisomy 21, and infants born with collodion skin disease. Several factors have been implicated in its pathophysiology including orbicularis oculi hypotonia, birth trauma, vertical shortening of anterior lamella or vertical elongation of posterior lamella of the eyelids with failure of the orbital septum to fuse
Oman Journal of Ophthalmology, Vol. 7, No. 2, 2014
2.
3. 4.
Al Hussain HA, AI-Rajhi AA, AI-Qahtani S, Meyer D. Congenital upper eyelid eversion complicated by corneal perforation. Br J Ophthalmol 2005;89:771. Conlon MR, Sutula FC. Congenital eyelid anomalies. In Albert DM, Jakobiac FA (editors): Principles and Practice of Ophthalmology, Vol. 4. 2nd ed. Philadelphia, PA: WB Saunders Co; 1994. Adeoti CO, Ashaye AO, Isawumi MA, Raji RA. Nonsurgical management of congenital eversion of the eyelids. J Ophthalmic Vis Res 2010;5:188-92. Bentsi-Enchill KO. Congenital total eversion of the upper eyelids. Br J Ophthalmol-1981;65:209-13.
Cite this article as: 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. Source of Support: Nil, Conflict of Interest: None declared.
99
Copyright of Oman Journal of Ophthalmology is the property of Medknow Publications & Media Pvt. 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.
