JOURNAL ARTICLE

Iris hypoplasia and aorticopulmonary septal defect: a neurocristopathy

Chris S Bergstrom, Richard A Saunders, Amy K Hutchinson, Scott R Lambert
Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus 2005, 9 (3): 264-7
15956947

BACKGROUND: Errors in neural crest development are responsible for a number of ocular disorders. In some cases, these ocular abnormalities may be associated with systemic disorders, some of which may be life threatening.

METHODS: We reviewed the medical record of 3 children from 2 institutions with a similar constellation of ocular and cardiac abnormalities.

RESULTS: All 3 children had iris hypoplasia with pupils 6-7 mm in diameter that had minimal or no response to light or pharmacological agents. The ocular examinations were otherwise normal. All 3 children also had large aorticopulmonary septal defects which were surgically repaired.

CONCLUSIONS: We report an association between iris hypoplasia and aorticopulmonary septal defects. Like the iris stroma, the aorticopulmonary septum forms from neural crest cells during embryogenesis which divide the truncus arteriosus into the ascending aorta and pulmonary trunk. Children with iris hypoplasia should undergo an immediate cardiac evaluation. It is important that opthalmologists be aware of the association between these two disorders since an aorticopulmonary septal defect is a life-threatening disorder.

Full Text Links

Find Full Text Links for this Article

Discussion

You are not logged in. Sign Up or Log In to join the discussion.

Related Papers

Remove bar
Read by QxMD icon Read
15956947
×

Save your favorite articles in one place with a free QxMD account.

×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"

We want to hear from doctors like you!

Take a second to answer a survey question.