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Chapter-71 Management of Pediatric Retinal Diseases

BOOK TITLE: Surgical Techniques in Ophthalmology (Pediatric Ophthalmic Surgery)

Author
1. Hussain Anjli
2. Hussain Nazimul
3. Natarajan S
ISBN
9789350251485
DOI
10.5005/jp/books/11282_71
Edition
1/e
Publishing Year
2011
Pages
8
Author Affiliations
1. Smt. Kanuri Santhamma Retina and Vitreous Centre, LV Prasad Eye Institute, LV Prasad Marg, Banjara Hills Hyderabad, India, LV Prasad Eye Institute, Banjara Hills, Hyderabad, India, LV Prasad Eye Institute, Hyderabad, Macular Clinic, Aditya Jyot Eye Hospital, Mumbai, India, LV Prasad Eye Institute, LV Prasad Marg, Banjara Hills, Hyderabad, India
2. Al Zahra Pvt Hospital, Al Zahra Square, PO Box : 3499, Sharjah, UAE, Al Zahara Hospital, Sharjah, UAE, Al-Zahra Hospital, Shrajah, UAE, Vitreoretinal Surgery Clinic, Aditya Jyot Eye Hospital, Mumbai, India, Al Zahra Hospital, PO Box Sharjah, UAE, Al Zahra Hospital, PO Box 3499 Sharjah, UAE, LV Prasad Eye Institute, Hyderabad, Smt. Kannuri Santhamma Retina, Vitreous Center, LV Prasad Eye Institute, Hyderabad, India, Al Zahra Hospital, Sharjah, UAE, L.V. Prasad Eye Institute, Hyderabad, India, Smt. Kanuri Santhamma Retina and Vitreous Centre, LV Prasad Eye Institute LV Prasad Marg, Banjara Hills Hyderabad, Al Zahra Pvt. Hospital, Sharjah/Dubai, UAE, Al Zahra Pvt Hospital, Sharjah/Dubai, UAE
3. Aditya Jyot Eye Hospital, Pvt. Ltd., Plot No. 153, Road No. 9, Major Parmeshwaran Road, Opp. SIWS College, Gate No. 3, Wadala, Mumbai, India, Aditya Jyot Eye Hospital, Mumbai, Aditya Jyot Eye Hospital, Mumbai, India, Aditya Jyot Eye Hospital Pvt. Ltd., Plot No. 153, Road No. 9, Major Parmeshwaran Road, Opp. SIWS College Gate NO: 3, Wadala, Mumbai, India, Aditya Jyot Eye Hospital Pvt Ltd, Plot No. 153, Road No. 9, Major Parmeshwaran Road, Opposite SIWS College, Gate No. 3, Wadala, Mumbai, India, Aditya Jyot Eye Hospital Pvt Ltd, Plot No. 153, Road No. 9, Major Parmeshwaran Road, Opposite SIWS College, Gate No. 3, Wadala,Mumbai, India, Aditya Jyot Eye Hospital Pvt Ltd, Mumbai, Maharashtra, India, Aditya Jyot Eye Hospital, Aashirwad, Vikaswadi, Dadar TT, Mumbai, India, Adityajyot Eye Hospital Pvt. Ltd., Plot No. 153, Road No. 9, Major Parmeshwaran Road, Opp. SIWS College, Gate No. 3, Wadala, Mumbai, India, GKNM Hospital, Coimbatore, Tamil Nadu, Aditya Jyot Eye Hospital Pvt Ltd, Plot No 153, Road No 9, Major Parm
Chapter keywords

Abstract

Major pediatric retinal diseases are Retinopathy of prematurity (ROP), Best’s disease, gyrate atrophy. Retinopathy of prematurity (ROP) is a potentially blinding eye disorder that primarily affects premature infants weighing 1250 grams or less, that are born before 31 weeks of gestation. The disease improves and leaves no permanent damage in milder cases of ROP. The anteroposterior extent or the location was divided into 3 zones (zones 1–3), the circumferential extent into clock hours (1–12) and severity of the disease in 5 stages (stage 1–5). The presence of dilated and tortuous vessels denotes an aggressive, potentially sight threatening component and is called the PLUS disease. Rush disease is zone1 ROP with plus disease. In this the progression is rapid and fulminant. Proper planning is a key component of efficient screening in a neonatal intensive care unit. Differential diagnosis is done from familial exudative vitreoretinopathy, congenital falciform fold, incontinentia pigmenti. Retinal ablation, laser photo ablation, cryotherapy are the common treatment available. Best’s disease is an abnormality of the RPE associated with accumulation of yellowish lipofuscin like material in the macula. Known genetic mutation occurs at VMD2 gene. Genetic counseling and Low vision services are essential when needed. Gyrate atrophy is a rare autosomal recessive disorder associated with hyperornithinemia. Clinical features also follow a slowly progressive time course Amino acid analysis for plasma ornithine level is essential to restrict arginine free diet.

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