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Home , Congenital cataract, Enophthalmos, Hypertropia, Keratoconus

DJO Vol. 30, No. 1, July-September 2019 Case Report Oculodigital phenomenon leading to advanced in 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

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 Abstract both eyes, along with oculodigital sign and . 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 later in life. Oculodigital sign can further compromise the vision in such cases due to associated keratoconus, , and .

Delhi J Ophthalmol 2019;30;46-48; Doi http://dx.doi.org/10.7869/djo.479 Keywords: Retinopathy of prematurity, Cicatricial ROP, Keratoconus in ROP, Oculodigital phenomenon

Introduction care unit stay and oxygen supplementation. The parents Better health care facilities and improved survival rates denied a history of previous ophthalmic examination and of premature infants in developing countries have led to intervention. Other than developmental delay, there were an increase in the risk of ROP. With increasing awareness no systemic complaints. There were no similar complaints in about ROP among gynecologists, pediatricians and other family members. ophthalmologists most of the preterm babies are now On examination, was hand movements close to screened and treated in time, but there are still some face in the right eye and no perception of light in the left eye. unfortunate ones who are missed out and become victim Enophthalmos was noted in both eyes (Figure 1A), while to this potentially blinding condition. The purpose of this a positive oculodigital sign was observed in the right eye case report is to highlight the grave sequelae of ROP if left (Figure 1B). The child had horizontal manifest untreated and the presence of oculodigital phenomenon as with null in dextroversion and left eye with an additive risk factor in increasing complications in these . On slit-lamp examination, the right eye showed cases. conical protrusion of with apical thinning, Vogt’s striae and positive Munson sign suggestive of advanced Case report keratoconus (Figure 2A and 2C). Rest of the anterior segment A 14-year-old male child presented to the outpatient was unremarkable in the right eye. The left eye had band- department with complaints of poor vision in both the eyes shaped keratopathy in addition to keratoconus (Figure 2D). since infancy. The parents gave a history of preterm vaginal A dense cataract with posterior synechiae was also noted in delivery at 26 weeks of gestation due to maternal cervical the left eye (Figure 2B). There were no cells, flare or rubeosis incompetence. Also, there was a history of neonatal intensive iridis in the anterior chamber.

Figure 1: Face photograph (A) Bilateral enophthalmos with left eye exotropia and hypertropia; (B) Positive oculodigital phenomenon with the child poking his right eye with the index finger. (C) Ultra-wide field fundus photograph of the right eye on Optos Tx200. The is pale with temporally arising retinal vessels. Retinal vessels are straight and blunted with avascular temporal periphery and nasal hemi . Widespread retinal pigmentary changes can be seen suggestive of self-settled retinal detachment. (D) B/A scan ultrasonography of the left eye shows minimal vitreous echoes and a thick echogenic membrane attached to the disc suggestive of retinal detachment. High amplitude spikes can be seen in the ocular coat suggestive of calcification.

E-ISSN: 2454-2784 P-ISSN: 0972-0200 46 www.djo.org.in O Vol. 30, No. 1, July-September 2019

Dilated fundus examination of the right eye revealed poking or rubbing of the eye with thumb or fist.1 It is a self- an average-sized pale optic disc. The temporal vascular stimulatory phenomenon wherein a flash of light is perceived arcades showed straightening and sudden blunting along on mechanical stimulation of the [pressure with avascular temporal and nasal retina with widespread phosphene].2 The sign though commonly reported in Leber’s pigmentary changes (Figure 1C). The left eye fundus could congenital amaurosis is also associated with other conditions not be assessed due to the presence of dense cataract. with poor vision such as retinopathy of prematurity, bilateral Intraocular pressure as measured on Goldmann applanation , congenital , congenital corneal tonometer was 12 mmHg and 6 mmHg in the right and the leucoma, congenital rubella syndrome, Norrie's disease, and left eye respectively. B scan ultrasonography of the left eye congenital retinal degenerations.3,4 (Figure 1D) revealed minimal vitreous echoes and a thick Chronic eye rubbing can lead to keratoconus, cataract, echogenic membrane attached to the disc with poor after- enophthalmos, and retinal detachment, all of which were movements, suggestive of retinal detachment. Calcification present in our case.5 This further increases the ocular of the outer ocular coats was also seen without any mass morbidity in patients with previously compromised vision. lesion. (Pentacam) revealed a Kmax of ROP sequelae can present with posterior segment changes 66.2 D and 63.1 D, thinnest pachymetry of 461µm and 336 such as abnormal peripheral retinal changes, retinal pigment µm and posterior elevation of +134 µm and +52 µm in the epithelial changes, vitreoretinal interface changes, vitreous right and left eye respectively. membranes, high , and lattice-like degenerations.6 Differential diagnosis in such a case includes cicatricial Retinal detachment may occur due to significant vitreoretinal retinopathy of prematurity, familial exudative traction and/or posterior retinal breaks.7 Neglected cases with vitreoretinopathy, pigmentosa, Norrie's disease, very poor vision may develop oculodigital phenomenon and and Retinoblastoma. Bilateral disease, history of prematurity its complications like retinal detachment and keratoconus.8 and oxygen supplementation, absence of family history, Keratoconus occurring due to oculodigital phenomenon absence of hearing impairment, and absence of mass lesion (as a result of repetitive corneal micro-trauma), has been in fundus pointed towards a clinical diagnosis of ROP. No described rarely.9-11 further ophthalmic intervention was advised in both eyes This case highlights the association of oculodigital sign with because of poor visual prognosis. Subjective keratoconus in patients with cicatricial ROP, which can was done for the right eye, but no visual improvement was further compromise the residual vision in these patients. Also, seen. The parents were counseled regarding the hazards of it emphasizes on the need for a detailed anterior segment oculodigital sign and need for regular follow up. Complete examination along with fundus evaluation for all cases of systemic and neurological evaluation of the child was done regressed ROP on all follow-ups. Proper documentation under pediatrics department. of the patients' and keratometry on every follow up is a must and would help in early detection and Discussion timely intervention of the cases developing keratoconus. Oculodigital sign of Franceschetti is often seen in eyes with The diverse presentation of regressed ROP later in life as was poor vision and is characterized by repetitive pressing, seen in our case include advanced keratoconus, band-shaped

Figure 2: Slit-lamp photograph under (A) Focal slit illumination of the right cornea showing inferior paracentral corneal protrusion with thinning and Vogt's striae suggestive of keratoconus. (B) Diffuse illumination of the left eye showing complicated cataract with posterior synechiae (C) Diffuse illumination showing a lateral view of the left eye showing advanced conical protrusion of the cornea; (D) Diffuse illumination photograph of the left eye showing band-shaped keratopathy.

E-ISSN: 2454-2784 P-ISSN: 0972-0200 47 Delhi Journal of DJO Vol. 30, No. 1, July-September 2019 keratopathy, cataract and retinal detachment stressing again Cite This Article as: Kumawat D, Sahay P, Goel S, Kumar V, Chandra P. on the need for regular and prolonged follow up of ROP Oculodigital phenomenon leading to advanced keratoconus in Retinopathy of patients. Prematurity – A case report.

References Acknowledgments: Nil 1. Gerald M Fenichel. Disorders of the . In: Clinical Conflict of interest: None declared Paediatric Neurology: A approach. 2009; 6:343. Source of Funding: None 2. Troster H, Brambring M, Beelmann A. Prevalence and situational causes of stereotyped behaviours in blind infants and pre- Date of Submission: 23 May 2019 schoolers. J Abnorm Child Psychol, 1991;19:569-90. Date of Acceptance: 10 July 2019 3. Roy FH. Ocular autostimulation. Am J Ophthalmol, 1967;63:1776. 4. Franklin AH. Norrie's disease. Am J Ophthalmol, 1971;72:947-8. 5. Brodsky MC. Nystagmus in infancy and childhood. In: Paediatric Address for correspondence Neuro-Ophthalmology, 2010; 2:383-443. 6. The committee for the classification of retinopathy of prematurity. Pranita Sahay An international classification of prematurity: the classification MD DNB, FICO, FAICO (Ref SX.), FICO (Cornea) of retinal detachment. Arch Ophthalmol, 1984; 102:1130-4. Dr. Rajendra Prasad Centre for Ophthalmic 7. Sneed Sr, Pulido JS, Blodi CF. Surgical management of late-onset Sciences, All India Institute of Medical retinal detachments associated with regressed retinopathy of Sciences, New Delhi, India prematurity. Ophthalmology, 1990; 97:179-83. Email id: [email protected] 8. Kumawat D, Sahay P, Alam T, Bhari A, Chandra P. Ocular auto-stimulation and its morbidity in stage 5 retinopathy of prematurity. Indian J Ophthalmol, 2019; 67:912-916. 9. Lorfel RS, Sugar HS. Keratoconus associated with retrolental fibroplasia. Ann Ophthalmol, 1976; 8:449-50. 10. Kogoleva LV, Pleskova AV. Keratoconus in children with retinopathy of prematurity. Vestn Oftalmol, 2012;128:32-4. 11. Hittner HM, Rhodes LM, McPherson AR. Anterior segment abnormalities in cicatricial retinopathy of prematurity. Ophthalmology, 1979; 86:803-16. Quick Response Code

E-ISSN: 2454-2784 P-ISSN: 0972-0200 48 www.djo.org.in