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sotos_syndrome [2025/07/01 22:53] – created Scott Larsonsotos_syndrome [2025/07/01 23:13] (current) – [Main Features] Scott Larson
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 ====== Sotos Syndrome ====== ====== Sotos Syndrome ======
 +====Main Features====
 +  * Distinctive Facial Features
 +    * Broad, prominent forehead with a dolichocephalic head shape, sparse frontotemporal hair,downslanting palpebral fissures, malar flushing, long and narrow face, tall chin 
 +<WRAP round box 60%>
 +{{:sotos_syndrome.jpg |}} \\
 +From Reference 2 \\
 +</WRAP>
 +  * Learning disability
 +  * Overgrowth > 2 SD above the mean (98th percentile)
 +    * Tall stature
 +    * Macrocephaly 
 +====Eye Findings====
 +  * Strabismus
 +  * Nystagmus
 +  * Cataract
 +  * Refractive error
 +    * astigmatism
 +    * myopia
 +    * hyperopia
 +  * Optic atrophy
 +  * Retinal atrophy
 +  * Megalophthalmos 
 +====Other Findings====
 +  * Behavioral problems especially autism
 +  * Advance bone age
 +  * Cardiac anomalies
 +    * PDA, septal defects or more complex anomalies
 +  * Cerebral ventriculomegaly
 +  * Joint hyperlaxity with or without pes planus
 +  * Renal anomalies- most commonly VUR
 +  * Scoliosis 
 +  * Seizures
 +====Etiology====
 +  * Mutations in the NDS1 gene
 +  * 5q35 microdeletion encompasing NDS1
 +====Resources====
 +  - [[https://www.ncbi.nlm.nih.gov/books/NBK1479/|GeneReviews Article]]
 +  - [[https://1drv.ms/b/c/31d83ae8e55e0542/ERAf3o98dU5Pg3q0-0ZGuNABGuqSz-t3L3IZ3NkB5FygsQ?e=CHzckN |Tatton-Brown K. et al. Clinical Features of NSD1-Positive Sotos Syndrome. Clinical Dysmorphology 2004;13:199-204.]] 
  
 +{{tag>syndrome}}