RETINAL NON ATTACHMENT

Congenital nonattachment of the retina is caused by the failure of the invaginating anterior optic cup (future sensory retina) to contact the posterior layer (future retinal pigment epithelium), leaving a fluid filled space.

ULTRASOUND

1. Extreme microphthalmia.
2. Conical structure within the globe with its conical base toward the lens and its apex pointing posteriorly towards the optic nerve.
3. Other intraocular anomalies:
• Persistent hyperplastic primary vitreous.
• Colobomas of the lens and iris (1,2).
4. Other extraocular anomalies:
• Hydrocephalus (3).

ASSOCIATED SYNDROMES

• Walker-Warburg Syndrome.

REFERENCES

1. Foos RY, Kiechler RJ, Allen RA. Congenital nonattachment of the retina. Am J Ophthalmol 1968;65:202
2. Boniuk M, Hittner HM. Congenital retinal disinsertion syndrome. Trans Am Acad Ophthalmol Otolaryngol 1975;79:827
3. Warburg M. Hydrocephaly, congenital retinal nonattachment, and congenital falciform fold. Am J Ophthalmol 1878;85:88
4. Farrel SA, Leadman TML, Davidson RG et.al. Prenatal diagnosis of retinal detachment in Walker-Warburg syndrome. Am J Med Genet 1987;28:619-624
5. Vohra N, Ghidini A, Alvarez M et.al. Walker-Warburg syndrome: Prenatal ultrasound findings. Prenat Diagn 1993;13:575-579.