|Year : 2021 | Volume
| Issue : 2 | Page : 283-285
Bilateral corneal haze and unilateral glaucoma in a preterm infant with congenital erosive vesicular disease – A case report
Monisha Mohan, Shashikant Shetty
Department of Pediatric Ophthalmology and Strabismus, Aravind Eye Hospital, Madurai, Tamil Nadu, India
|Date of Submission||13-May-2020|
|Date of Acceptance||11-Sep-2020|
|Date of Web Publication||01-Apr-2021|
Dr. Monisha Mohan
Aravind Eye Hospital, Madurai - 625 025, Tamil Nadu
Source of Support: None, Conflict of Interest: None
We report a rare ocular presentation of bilateral corneal haze and unilateral glaucoma in a 33-week old preterm infant diagnosed to have congenital erosive and vesicular dermatosis.
Keywords: Buphthalmos, congenital erosive vesicular dermatosis, congenital glaucoma, prematurity, corneal haze
|How to cite this article:|
Mohan M, Shetty S. Bilateral corneal haze and unilateral glaucoma in a preterm infant with congenital erosive vesicular disease – A case report. Indian J Ophthalmol Case Rep 2021;1:283-5
|How to cite this URL:|
Mohan M, Shetty S. Bilateral corneal haze and unilateral glaucoma in a preterm infant with congenital erosive vesicular disease – A case report. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Apr 20];1:283-5. Available from: https://www.ijoreports.in/text.asp?2021/1/2/283/312330
Congenital erosive and vesicular dermatosis (CEVD), first reported by Cohen et al. in 1985, is a rare disorder characterized by erosions and vesicles at birth, hydrocephalus, and developmental delay.
Ocular involvement such as corneal scarring, conjunctival inflammation, lacrimal duct obstruction, and vitreous hemorrhage have been already reported in the literature. Other features associated with this skin condition include neurodevelopmental abnormalities like seizures, cerebral palsy, mild hemiparesis, microcephaly.,
Rare associations include sensorineural hearing loss, neonatal thrombocytopenia, dystrophic ears, erosive lichen planus, digital tip gangrene, murky appearance of amniotic fluid on prenatal ultrasound, and hydronephrosis.,
In this report, we describe a rare occurrence of unilateral congenital glaucoma in a preterm infant diagnosed with CEVD.
| Case Report|| |
A 42-week-old preterm infant (9 weeks chronological age), was referred by the pediatrician to our out-patient care for bilateral corneal haze. Born out of a non-consanguineous marriage, the child was delivered pre-term at 33 weeks of gestation by spontaneous vaginal delivery and weighed approximately 1.67 kg. No oxygen therapy or photo-therapy was administered. General examination revealed healing vesicular lesions over scalp region [Figure 1]a with sparing of the face [Figure 1]b and supple reticulate scarring over the trunk [Figure 2] and limbs with sparing of palms and soles. Bilateral corneal haze with left eye buphthalmos [Figure 3]a was noted. Earlier photographs taken at birth, by her mother revealed increased corneal haze compared to the present corneas. Child was examined under anesthesia (EUA). Anterior segment findings showed diffuse corneal haze with edema noted in both eyes, more in the left eye than the right. Intraocular pressure (IOP) checked by ICare Pro rebound tonometer (ICare, Helsinki, Finland) showed 14 mmHg in the right eye and was un-recordable in the left eye. Corneal diameters measured with Castrovejo calipers measured 11.5 mm and 13 mm in the right and left eye respectively suggesting associated buphthalmos in the left eye. Axial length measured using PalmScan AP2000 (Micro Medical Devices Inc, California, USA) was found to be 18.24 mm in the right eye and 19.44 mm in the left eye. Fundus examination revealed both eye immature vessels in all zones with large discs and optic disc appeared suspicious of glaucomatous changes with a cup-to-disc ratio of 0.5 and 0.8 in the right and left eye respectively.
|Figure 1: Clinical photograph shows the healing vesicular lesions involving scalp and forehead (a) and sparing the face (b). Note the left eye blepharospasm in Figure 1b|
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|Figure 2: Clinical photograph showing the supple reticular scarring of the healed lesion on the back of the child at 42 weeks (area between the dotted lines)|
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|Figure 3: Clinical photograph showing the diffuse corneal haze in the left eye with buphthalmos taken at 33 weeks (a) and at 42 weeks (b). Note the relative corneal clearing in Figure 3b|
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Laboratory investigations to rule out mucopolysacchardosis and abnormal amino-acids were done that turned out to be negative. Blood investigations were within the normal range. In view of buphthalmos with corneal haze, suspicious optic disc changes, and increased axial length in the neonatal period, a diagnosis of congenital glaucoma was made and the child was started on both eyes Dorzolamide hydrochloride 2% eye drops twice daily.
Child was reviewed again after 3 months of using Dorzolamide hydrochloride 2% eye drops twice daily. The vesicular lesions were seen to be in multiple stages of healing. Child was examined for the second time under EUA on follow up. Anterior segment findings showed clear cornea in the right eye with very minimal corneal haze in the left eye [Figure 3]b. Intraocular pressure showed 10 mmHg in the right eye and was 10 mm Hg in the left eye. Corneal diameters measured 10.5 mm and 11.5 mm in the right and left eye respectively. Axial length measured 18.93 mm in the right eye and 19.89 mm in the left eye. Well perfused optic disc with a cup-to-disc ratio of 0.5 and 0.6 in the right and left eye respectively with mature retina was noted. All these findings are suggestive of findings of congenital glaucoma responding to medical therapy.
| Discussion|| |
With a prevalence of <1/100000 live births, congenital erosive vesicular dermatosis is a rare disease. A disease that presents at birth, mostly seen in the premature infant, is characterized by superficial erosions, multiple vesicles, and bullae that affect almost about 75% of the body surface, involving mainly the trunk and extremities. Volar surface and face is spared.
There is no genetic predisposition noted until now. Exact mechanism or the mode of inheritance is not known but proposed etiopathogenesis includes birth trauma, premature rupture of membranes, intrauterine infections, chorioamniotis, amniotic adhesions, purulent or discolored amniotic fluid and a developmental defect with unusual healing in preterm skin.
Our case did not have any significant history with regard to the cause mentioned. Previous literature have reported CEVD cases mostly in the preterm and about six cases in the term babies.
With less than 30 cases, reported in the literature, ocular manifestations are seen in 36% of the cases. Ocular manifestations described are corneal scarring, conjunctival inflammation, naso-lacrimal duct obstruction, vitreous hemorrhage, and scarring of eyelid margins. Neurodevelopmental disorders and ophthalmological manifestation may well be the manifestation of prematurity. Their association with CEVD still remains unclear.
There have not been any previous reports of corneal edema or suspicious glaucomatous findings in these children. Our case of bilateral corneal haze, buphthalmos, suspicious glaucoma with increased axial length responding to dorzolamide hydrochloride 2% eye drops with a decrease in corneal edema and reversal of cupping in the left eye shows the possibility of this rare association of congenital glaucoma in CEVD. The fact that corneal haze improved after starting topical anti-glaucoma medication strongly suggests the diagnosis of raised IOP and possible glaucoma with CEVD.
| Conclusion|| |
We thus report the clinical presentation and management of bilateral corneal haze and left eye buphthalmos with suspicious glaucomatous optic nerve head changes and recommend a thorough clinical examination in terms of axial length measurement, corneal diameter measurement, IOP recording, and optic disc evaluation in all cases of CEVD with corneal haze and/or buphthalmos.
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
Conflicts of interest
There are no conflicts of interest.
| References|| |
Cohen BA, Esterly NB, Nelson PF. Congenital erosive and vesicular dermatosis healing with reticulated supple scarring. Arch Dermatol 1985;121:361-7.
Tlougan BE, Paller AS, Schaffer JV, Podjasek JO, Mandell JA, Nguyen XH, et al
. Congenital erosive and vesicular dermatosis with reticulated supple scarring: unifying clinical features. J Am Acad Dermatol 2013;69:909-15.
Mashiah J, Wallach D, Leclerc-Mercier S, Bodemer C, Hadj-Rabia S. Congenital erosive and vesicular dermatosis: A new case and review of the literature. Pediatr Dermatol 2012;29:756-8.
Metz BJ, Hicks J, Levy M. Congenital erosive and vesicular dermatosis healing with reticulated supple scarring. Pediatr Dermatol 2005;22:55-9.
Ciorba A, Corazzi V, Conz V, Tazzari R, Aimoni C. Sensorineural hearing loss in congenital erosive and vesicular dermatosis. J Int Adv Otol 2016;12:208-9.
Sidhu-Malik NK, Resnick SD, Wilson BB. Congenital erosive and vesicular dermatosis healing with reticulated supple scarring: report of three new cases and review of the literature. Pediatr Dermatol 1998;15:214-8.
Srinivas SM, Mukherjee SS, Hiremagalore R. Congenital erosive and vesicular dermatosis healing with reticulated supple scarring: Report of four cases. Indian J Dermatol Venereol Leprol 2018;84:73-5.
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