Papillorenal syndrome natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shivam Singla, M.D.[2]
Overview
Natural History
Complications
There are numerous complications associated with patients of renal coloboma syndrome mainly involving renal and ocular manifestations.
The moat common complications associated are:
End stage renal disease
VUR (Vesicoureteral reflux)
Eyelid coloboma
Iris coloboma
- Photophobia.
- Presence of double vision or blurring in the eyes.
Choroidoretinal coloboma
- Amblyopia
- Anisometropia,
- Retinal detachment ( Most common complication seen in patients with Choroidoretinal and optic disc coloboma)
- Strabismus
- Lens subluxation
- Cataracts
- Lens subluxation (uncommon).
- Glaucoma
- Morning glory disc anomaly
Prognosis
The prognosis for renal coloboma syndrome depends primarily on the treatment the patient is receiving. Whether the patient is receiving an appropriate treatment or not plays a major role in the prognosis of patients with RCS
Renal failure or ESRD can occur at any age and the treatment modalities reserved for this condition are dialysis and renal transplantation. Formal longitudinal studies of visual prognosis have not been carried out[1].
The prognosis for renal coloboma syndrome is worse if there is the presence of congenital anomalies in the patient[2]. Like if the patient with renal coloboma syndrome has underlying concomitant congenital heart disease ( cyanotic), anomalies with the brain and spinal cord, and esophageal atresia then the prognosis in this condition is bad.
Ocular prognosis or prognosis for the vision mainly depends on the severity or location of colobomas in the eye ( in relation to the optic nerve and maculopapular bundle and macula) as well as on the presence of any complications like amblyopia or retinal detachment.
The prognosis in patients with morning glory syndrome is usually poor[3].
Formal Longitudinal studies on visual prognosis of renal coloboma syndrome has not been carried out, However decreased visual acuity over time has been reported and not an uncommon manifestation seen in patients with renal- coloboma syndrome.