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:
Iris coloboma
- Photophobia.
- Presence of double vision or blurring in the eyes.
Eyelid coloboma
- If the Exposure keratopathy and corneal ulceration, if the cornea is not protected.
Choroidoretinal coloboma
- Reduced visual acuity or visual field (variable, see 'Prognosis', below).
- Anisometropia, amblyopia and strabismus secondary to the visual deficit.
- Retinal detachment is the most common complication of choroidoretinal and optic disc colobomata, occurring in up to 42% of patients.[11]
- Retinal detachment can also occur with coloboma of the zonule or ciliary body.
- Cataracts of multiple types are associated with coloboma, including pigment deposits, which may be subcapsular, cortical, anterior and posterior polar, and can lead to total opacification.
- Lens subluxation has been associated with coloboma of the zonules and ciliary body.
- Subretinal neovascularisation can occur with retinochoroidal coloboma and may lead to macular detachment.
Other complications
- Morning glory disc anomaly may have an associated encephalocele. This may present as pulsating exophthalmos or a mass in the upper lid or nasopharynx.[10]Facial masses should not be biopsied without prior imagining.
- Cataracts (various types) are associated with coloboma.
- Secondary glaucoma due to faulty formation of fluid drainage systems in the eye. This may present later in life.
- Lens subluxation (uncommon).
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.