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trisomy_13 [2025/09/29 14:57] – created Scott Larsontrisomy_13 [2025/09/29 15:05] (current) Scott Larson
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 ====== Trisomy 13 ====== ====== Trisomy 13 ======
 a.k.a. Patau syndrome a.k.a. Patau syndrome
 +====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====
 +  * Microphthalmia/ Anophthalmia
 +  * Coloboma of iris 
 +  * Corneal Opacities
 +  * Cataracts- often sectoral associated with coloboma
 +  * Retinal dysplasia, Retinal Detachment. Coats Disease
 +  * Persistent fetal vasculature
 +  * Glaucoma
 +  * Kerato-irido-lenticular dysgenesis
 +  * Optic Nerve Hypoplasia
 +  * Cerebral Vision impairment 
 +====Other Findings====
 +  * Congenital heart disease
 +  * Polydactyly
 +  * Microcephaly
 +  * GU malformations
 +    * cryptorchidism and renal anomalies
 +  * Ear anomalies
 +  * Omphalocele
 +====Etiology====
 +  * Chromosome abnormality
 +====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://medlineplus.gov/genetics/condition/trisomy-13/|Medline Plus Trisomy 13]]
 +
 +{{tag>syndrome}}