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Year : 2007  |  Volume : 55  |  Issue : 4  |  Page : 304-306

Triple A syndrome with ophthalmic manifestations in two siblings

1 Vittala International Institute of Ophthalmology, Bangalore, India
2 Indira Gandhi Institute of Child Health Sciences, Bangalore, India

Correspondence Address:
Kalpana Babu
100, 29th cross, Banashankari, 2nd stage, Bangalore-560 070
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0301-4738.33048

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Triple A syndrome (Allgrove syndrome) is a rare, autosomal recessive disorder characterized by adrenocorticotropic hormone resistant adrenal insufficiency, alacrima, achalasia of the esophageal cardia, progressive neurological degeneration and occasionally autonomic instability. We report the ophthalmic manifestations in 2 siblings from a consanguineous family with this syndrome. A routine ophthalmic examination showed absence of palpebral portion of lacrimal gland, dry eyes and sluggish pupillary reflexes in both eyes of these patients. Both of these patients had achalasia cardia while the boy additionally had increased serum cortisol levels. Topical ocular lubricants were prescribed for both of them and Heller's cardiomyotomy with fundus plication was done for achalasia. These cases would create an awareness of this rare condition in ophthalmology in addition to suggesting its management.

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