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trisomy_13 [2025/09/29 15:01] Scott Larsontrisomy_13 [2025/09/29 15:05] (current) Scott Larson
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 a.k.a. Patau syndrome a.k.a. Patau syndrome
 ====Main Features==== ====Main Features====
-  *  +  *  Severe congenital and neurodevelopmental impairment 
 +  * Midline defects 
 +    * cleft lip and palate, holoprosencephaly, scalp defects (aplasia cutis), congenital heart disease.  
 +  * Poor prognosis for life with high infant mortality, most infants die in the first days or weeks of life only 5-10% survive beyond the first year. 
  
 ====Eye Findings==== ====Eye Findings====
-  * Microphthalmia+  * Microphthalmia/ Anophthalmia
   * Coloboma of iris    * Coloboma of iris 
   * Corneal Opacities   * Corneal Opacities
   * Cataracts- often sectoral associated with coloboma   * Cataracts- often sectoral associated with coloboma
-  * Retinal dysplasia, Retinal Detachment +  * Retinal dysplasia, Retinal Detachment. Coats Disease
-  * Anopthalmia+
   * Persistent fetal vasculature   * Persistent fetal vasculature
   * Glaucoma   * Glaucoma
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   * Cerebral Vision impairment    * Cerebral Vision impairment 
 ====Other Findings==== ====Other Findings====
-  * +  * Congenital heart disease 
 +  * Polydactyly 
 +  * Microcephaly 
 +  * GU malformations 
 +    * cryptorchidism and renal anomalies 
 +  * Ear anomalies 
 +  * Omphalocele
 ====Etiology==== ====Etiology====
-  * +  * Chromosome abnormality
 ====Resources==== ====Resources====
   * [[https://pubmed.ncbi.nlm.nih.gov/16765673/|Clinical ocular abnormalities in infants with trisomy 13. Leuder, Gregg T. Am J Ophthalmol. 2006;(141(6):1057-60]]   * [[https://pubmed.ncbi.nlm.nih.gov/16765673/|Clinical ocular abnormalities in infants with trisomy 13. Leuder, Gregg T. Am J Ophthalmol. 2006;(141(6):1057-60]]
 +  * [[https://medlineplus.gov/genetics/condition/trisomy-13/|Medline Plus Trisomy 13]]
  
 {{tag>syndrome}} {{tag>syndrome}}