Delhi Journal of Ophthalmology

Oculodigital phenomenon leading to advanced keratoconus in Retinopathy of Prematurity – A case report

Devesh Kumawat, Pranita Sahay, Siddhi Goel, Vinod Kumar, Parijat Chandra
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi, India

Corresponding Author:

Pranita Sahay
MD DNB, FICO, FAICO (Ref SX.), FICO (Cornea)
Dr. Rajendra Prasad Centre for Ophthalmic
Sciences, All India Institute of Medical
Sciences, New Delhi, India
Email id: drpranitasahay@gmail.com

Received: 23-MAY-2019

Accepted: 10-JUL-2019

Published Online: 24-DEC-2019

DOI:http://dx.doi.org/10.7869/djo.479

Abstract
A 14-year-old male child presented with complaints of poor vision in both eyes since infancy. The child had a history of preterm delivery, neonatal intensive care unit stay, and oxygen supplementation. He was diagnosed to have cicatricial Retinopathy of Prematurity [ROP]. Keratoconus was evident in both eyes, along with oculodigital sign and enophthalmos. This case highlights the grave sequelae of untreated ROP and its complications. A varied presentation of ROP is to be borne in mind when evaluating such patients later in life. Oculodigital sign can further compromise the vision in such cases due to associated keratoconus, cataract, and retinal detachment.

Keywords :Retinopathy of prematurity, Cicatricial ROP, Keratoconus in ROP, Oculodigital phenomenon

Introduction

Better health care facilities and improved survival rates of premature infants in developing countries have led to an increase in the risk of ROP. With increasing awareness about ROP among gynecologists, pediatricians and ophthalmologists most of the preterm babies are now screened and treated in time, but there are still some unfortunate ones who are missed out and become victim to this potentially blinding condition. The purpose of this case report is to highlight the grave sequelae of ROP if left untreated and the presence of oculodigital phenomenon as an additive risk factor in increasing complications in these cases.

Case report

A 14-year-old male child presented to the outpatient department with complaints of poor vision in both the eyes since infancy. The parents gave a history of preterm vaginal delivery at 26 weeks of gestation due to maternal cervical incompetence. Also, there was a history of neonatal intensive care unit stay and oxygen supplementation. The parents denied a history of previous ophthalmic examination and intervention. Other than developmental delay, there were no systemic complaints. There were no similar complaints in other family members. On examination, visual acuity was hand movements close to face in the right eye and no perception of light in the left eye. Enophthalmos was noted in both eyes (Figure 1A), while a positive oculodigital sign was observed in the right eye (Figure 1B). The child had horizontal manifest nystagmus with null in dextroversion and left eye exotropia with hypertropia. On slit-lamp examination, the right eye showed conical protrusion of cornea with apical thinning, Vogt’s striae and positive Munson sign suggestive of advanced keratoconus (Figure 2A and 2C). Rest of the anterior segment was unremarkable in the right eye. The left eye had bandshaped keratopathy in addition to keratoconus (Figure 2D). A dense cataract with posterior synechiae was also noted in the left eye (Figure 2B). There were no cells, flare or rubeosis iridis in the anterior chamber.
Dilated fundus examination of the right eye revealed an average-sized pale optic disc. The temporal vascular arcades showed straightening and sudden blunting along with avascular temporal and nasal retina with widespread pigmentary changes (Figure 1C). The left eye fundus could not be assessed due to the presence of dense cataract.

Intraocular pressure as measured on Goldmann applanation tonometer was 12 mmHg and 6 mmHg in the right and the left eye respectively. B scan ultrasonography of the left eye (Figure 1D) revealed minimal vitreous echoes and a thick echogenic membrane attached to the disc with poor aftermovements, suggestive of retinal detachment. Calcification of the outer ocular coats was also seen without any mass lesion. Corneal topography (Pentacam) revealed a Kmax of 66.2 D and 63.1 D, thinnest pachymetry of 461µm and 336 µm and posterior elevation of +134 µm and +52 µm in the right and left eye respectively.

 


Differential diagnosis in such a case includes cicatricial retinopathy of prematurity, familial exudative vitreoretinopathy, Retinitis pigmentosa, Norrie's disease, and Retinoblastoma. Bilateral disease, history of prematurity and oxygen supplementation, absence of family history, absence of hearing impairment, and absence of mass lesion in fundus pointed towards a clinical diagnosis of ROP. No further ophthalmic intervention was advised in both eyes because of poor visual prognosis. Subjective refraction was done for the right eye, but no visual improvement was seen. The parents were counseled regarding the hazards of oculodigital sign and need for regular follow up. Complete systemic and neurological evaluation of the child was done under pediatrics department.

Discussion

Oculodigital sign of Franceschetti is often seen in eyes with poor vision and is characterized by repetitive pressing, poking or rubbing of the eye with thumb or fist.1 It is a selfstimulatory phenomenon wherein a flash of light is perceived on mechanical stimulation of the optic nerve [pressure phosphene].2 The sign though commonly reported in Leber’s congenital amaurosis is also associated with other conditions with poor vision such as retinopathy of prematurity, bilateral congenital cataract, congenital glaucoma, congenital corneal leucoma, congenital rubella syndrome, Norrie's disease, and congenital retinal degenerations.3,4
Discussion Oculodigital sign of Franceschetti is often seen in eyes with poor vision and is characterized by repetitive pressing, poking or rubbing of the eye with thumb or fist.1 It is a selfstimulatory phenomenon wherein a flash of light is perceived on mechanical stimulation of the optic nerve [pressure phosphene].2 The sign though commonly reported in Leber’s congenital amaurosis is also associated with other conditions with poor vision such as retinopathy of prematurity, bilateral congenital cataract, congenital glaucoma, congenital corneal leucoma, congenital rubella syndrome, Norrie's disease, and congenital retinal degenerations.3,4
This case highlights the association of oculodigital sign with keratoconus in patients with cicatricial ROP, which can further compromise the residual vision in these patients. Also, it emphasizes on the need for a detailed anterior segment examination along with fundus evaluation for all cases of regressed ROP on all follow-ups. Proper documentation of the patients' refractive error and keratometry on every follow up is a must and would help in early detection and timely intervention of the cases developing keratoconus. The diverse presentation of regressed ROP later in life as was seen in our case include advanced keratoconus, band-shaped keratopathy, cataract and retinal detachment stressing again on the need for regular and prolonged follow up of ROP patients.

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CITE THIS ARTICLE

Kumawat D, Sahay P, Goel S, Kumar V, Chandra POculodigital phenomenon leading to advanced keratoconus in Retinopathy of Prematurity – A case report.DJO 2019;30:46-48

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Kumawat D, Sahay P, Goel S, Kumar V, Chandra POculodigital phenomenon leading to advanced keratoconus in Retinopathy of Prematurity – A case report.DJO [serial online] 2019[cited 2020 Jan 25];30:46-48. Available from: http://www.djo.org.in/articles/30/1/Oculodigital-phenomenon-leading-to-advanced-keratoconus-in-Retinopathy-of-Prematurity.html