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foveal_hypoplasia [2023/09/26 16:14] – created Scott Larsonfoveal_hypoplasia [2026/02/02 19:35] (current) – [Systemic Associations] Scott Larson
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   * associated with poor vision and nystagmus   * associated with poor vision and nystagmus
  
-====== Ocular Associations ====== +==== Ocular Associations ==== 
-  * Aniridia+  * Aniridia (PAX6)
   * Achromatopsia   * Achromatopsia
   * Congenital retinal macrovessel   * Congenital retinal macrovessel
Line 13: Line 13:
   * Retinopathy of Prematurity   * Retinopathy of Prematurity
  
-====== Isolated Foveal Hypoplasia ======+==== Isolated Foveal Hypoplasia ====
   * Autosomal Dominant from PAX 6 gene variants   * Autosomal Dominant from PAX 6 gene variants
   * Autosomal Recessive from SLC38A8 gene variants   * Autosomal Recessive from SLC38A8 gene variants
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     * also associated with familial infantile nystagmus      * also associated with familial infantile nystagmus 
  
-====== Systemic Associations ======+==== Systemic Associations ====
   * [[albinism|Albinism]]   * [[albinism|Albinism]]
   * [[fevr|Familial Exudative Vitreoretinopathy]]   * [[fevr|Familial Exudative Vitreoretinopathy]]
   * [[incontientia_pigmenti|Incontientia pigmenti]]   * [[incontientia_pigmenti|Incontientia pigmenti]]
   * Prematurity   * Prematurity
-  * Stickler Syndrome+  * [[stickler_syndrome|Stickler Syndrome]] 
 + 
 +==== Diagnostic Procedures ==== 
 +  * Visual acuity 
 +    * VA is variable but can range from 20/40 to 20/200 
 +  * Oculomotor 
 +    * Nystagmus and strabismus 
 +  * Ophthalmoscopy 
 +    * absence of foveal reflex and foveal avascular zone and typical macular pigment 
 +  * [[optical_coherence_tomography|OCT]] 
 +    * Absence of foveal pit, persistent inner retinal layers through fovea  
 +    * Example of an OCT report of a child with foveal hypoplasia 
 +{{::foveal_hypoplasia.jpg}} 
 + 
 +  * OCT-A 
 +    * absence of superficial capillary plexus and variable decrease in deep capillary plexus in the fovea 
 + 
 +==== References ==== 
 +  * [[https://pubmed.ncbi.nlm.nih.gov/31937464/|Rufai SR, Thomas MG, Purohit R, Bunce C, Lee H, Proudlock FA, Gottlob I. Can Structural Grading of Foveal Hypoplasia Predict Future Vision in Infantile Nystagmus?: A Longitudinal Study. Ophthalmology. 2020 Apr;127(4):492-500.]] 
 +  * [[https://1drv.ms/b/c/31d83ae8e55e0542/EX01u0C3NulLoi7a60eswF8B-5H80Fsu4n4Cjz_gID-mMg?e=rHWQFw |Hendrickson A, Possin D, Vajzovic L, Toth CA. Histological development of the human fovea from midgestation to maturity. Am J Ophthalmol 2012;154:767-778.e2.]]