Introduction:Choroidal neovascularization (CNVM) is an important cause of visual impairment in pediatric age group (1). The CNVM in children has different causesfrom that of adult, maculopathies in children can cause CNVM such as best disease and inflammatory diseases (2), we are presenting 2 cases ofCNVM in childrenCASE (1)A case of seven-year old girl presented with decreased vision in both eyes. Examination showed Visual Acuity (right eye 6/12, left eye 6/18), normalanterior segments, fundus showed right active CNVM in the macular area and left macular scar (Fig 1) which was confirmed by Optical CoherenceTomography (OCT) (Fig 2,3), Fundus Fluorescein Angiography(FFA) (Fig 4); the etiology of which could not be identified. She was treated withintravitreal injections of Ranibizumab (Lucentis). Over a course of one and half year follow-up she had multiple recurrences in both eyes thatnecessitated further injections, five injections each eye, last injection was in July 2015. Screening of the family showed bilateral Vitilliformmaculopathy in her 5-year old brother. Electrophysiological testing of the patient and her brother showed abnormal electro-oculogram (EOG) andreduced macular and con responses both eyes (Fig 5,6).

Fig.2 OCT of the right eye showing

disruption of the retinal pigment

epithelium with detachment of the

sensory retina due to active CNVM.

Fig.1.Color fundus photograph ofthe right (a) and the left (b)eye of case1 at presentation,showing bilateral macularscar with fresh subretinalhemorrhage in the right eye

Fig.4

FFA of the right eye showing

area of blocked fluorescence

corresponding to the area of

hemorrhage in the colored

fundus photo and area of

hyper-fluorescence (a) and

late leakage (b) indicating

active CNVM.

Fig.3 OCT of the left eye showing

subfoveal scar.

Fig 5ERG showing Macular & ConeResponses are reduced in botheyesAbnormal EOG (OU)Arden Ratio < 1.8

Fig.6ERG of Patient Brother showing ReducedMacular & Cone Reponses in both eyesEOG reduced in OD ( Arden = 1.2) and Normalin OS ( Arden 2.2)

Fig.7Color fundus photograph of thebrother’s right and left eye(a) & (b) showing the yellowish fovealdeposits typical of Best’s disease egg-yolk stageOCT of OD and OS (c & d) showingsubfoveal Deposits

CASE (2)Thirteen-year old girl who presented with left eye decreased vision that she discovered accidently. There is no family history of anyocular disease. Examination showed right eye vision 6/6 unaided, normal IOP, normal anterior and posterior segment. Left eye visionclose counting fingers (CCF), normal IOP, and anterior segment, there was serous macular detachment with raised whitish supero-temporal peripapillary scar (Fig 8,9), and OCT showed elevated disc margin OS (Fig 10). Autofluorescence and B-Scan examinationexcluded the presence optic nerve drusen (Fig 11, 12 ). All blood investigations including serology to rule out any inflammation werenormal, chest x-ray was normal. She was diagnosed as idiopathic peripapillary CNVM which regressed spontaneously over 2 months (Fig13)

Fig.10 OCT of

the optic nerve

head showing

elevated disc

margin

Fig.9 showing

sensory

detachment

of the macula

secondary to

disc edema

Fig.12 B-scan of the left eye showing no Drusen

Fig.11 Autofluorescence of right (a) and left (b)

showed no Autofluorescence both eyes.

Fig.8 color fundus photograph of the right eye (a) and the left eye(b) showing normal fundus (a) and peripapillary scar at

the superotemporal quadrant of the disc (b) . FFA of the left eye showing staining of the scar( c, d)

(a) (b) (c) (d)

(a) (b)

(a) (b)

(a) (b)

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Choroidal Neovascular Membrane in ChildrenMaha Elshafei,MD.MS.FRCSI, Fareed AL-Laftah MD, FCRS-Ed, Baber Ali MD

Fig. 13 showing

marked

reduction in the

sensory

detachment of

the macula

Discussion:CNVM in children has different etiologies from that affecting Adults. In children, etiology of CNVM can be classified in to 3 categories: (A) Inheritedconditions: like macular dystrophies, myopia, and Angiold streaks, (B) Acquired conditions like ocular inflammation i.e. retinal choroiditis or posttraumatic. (C) Idiopathic CNVM (3). In case 1 the diagnosis of Best’s disease could not be reached at the beginning because of the late presentation of thecase i.e. in the scarring stage. It was made only after the examination of the brother and was confirmed by the electrophysiological testing In the past CNVMused to be treated with laser photocoagulation, photodynamic therapy, transpupillary Thermo-therapy, or surgical removal of the membrane. (4,5,6).. Most ofthese treatment options are replaced nowadays with intravitreal injections of anti VEGF agents. Several reports of case series or case reports showedgood response to this treatment option (7) Case 1 one was treated with repeated intravitreal injection of Ranibizumab. The condition settled down withVA 6/9 and 6/18 in the right and left eyes respectively. There was no ocular or systemic side effect related to the injection. In case 2, no treatment was givenas it showed spontaneous regression. It is known that Best’s Disease has Abnormal EOG and Normal ERG in previous studies (8) ; however there are manyreports proved that ERG is also affected in early stages of best disease (9).. and this is correlated with case 1.

Conclusions

CNVM is considered a significant cause of visual decline in young age group., Beside OCT, Electrophysiology testing is an important diagnostic tool in CNVMin Children, Anti VEGF is considered the treatment of choice nowadays.