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trisomy_18 [2025/09/29 14:41] – created Scott Larsontrisomy_18 [2026/01/26 22:15] (current) Scott Larson
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 ====== Trisomy 18 ====== ====== Trisomy 18 ======
 +a.k.a Edwards syndrome
 +====Main Features====
 +  *  Constellation of congenital anomalies affecting multiple organ systems (face, cardiac, renal, GI, CNS) 
 +    * Characteristic physical features
 +      * Small head, micrognatia, low-set malformed ears, overlapping fingers rocker bottom feet
 +      * Short sternum, underdeveloped thumbs, small fingernails
 +    * Neurologic impairment with severe psychomotor and cognitive disability
 +    * Feeding difficulty and failure to thrive
 +  * High infant mortality
 +    * most infants die in the first year of life 
 +  * [[https://my.clevelandclinic.org/-/scassets/images/org/health/articles/22172-edwards-syndrome|Cleveland Clinic Image Summary]]
  
 +====Eye Findings====
 +  * Present in 8-10%
 +  * Microphthalmia
 +  * Strabismus
 +  * Corneal opacities, Microcornia
 +  * Glaucoma 
 +  * Iris or choroid coloboma 
 +  * Cataract
 +  * Anterior segment dysgenesis
 +  * Eyelids
 +    * Ptosis
 +    * epicanthus
 +    * hypertelorism
 +  * Nystagmus reported but more rare, often in the setting of brain malformation
 +  * Retinal vascular tortuosity
 +====Other Findings====
 +  * Cardiac
 +    * VSD, ASD, PDA
 +    * Complex anomalies
 +  * Renal
 +  * GI
 +    * omphalocele, TE fistula
 +  * CNS
 +    * Cerebellar hypoplasia, choroid plexus cysts
 +====Etiology====
 +  * chromosomal abnormality 
 +====Resources====
 +  * [[https://medlineplus.gov/genetics/condition/trisomy-18/|Medline Plus Trisomy 18]]
 +  * [[https://pubmed.ncbi.nlm.nih.gov/26347425/|Congenital Anomalies associated with Trisomy 18 or Trisomy 13: A registry-based study in 16 European countries, 2000-2011. Springett A. et al. Am J Med Genet A. 2015 (12):3062-3069.]]
 +  * [[https://1drv.ms/b/c/31D83AE8E55E0542/AeixkAJ_9W5ApAulfN9vp6M?e=hwnAUH|A Case of Anterior Segment Dysgenesis with Iridolenticular Adhesions in Trisomy 18. Atwal PS. J Pediatr Genet 2015;4:207-208.]] 
 +  * Velzeboer CM, van der Harten JJ, Koole FD. Ocular pathology in trisomy 18. A histopathological report of three cases. Ophthalmic Paediatr Genet 1989;10(4):263–269
 +
 +{{tag>syndrome}}