TITLE

Triple A syndrome with ophthalmic manifestations in two siblings

AUTHOR(S)
Babu, Kalpana; Murthy, Krishna R.; Babu, Narendra; Ramesh, S
PUB. DATE
July 2007
SOURCE
Indian Journal of Ophthalmology;Jul2007, Vol. 55 Issue 4, p304
SOURCE TYPE
Academic Journal
DOC. TYPE
journal article
ABSTRACT
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.
ACCESSION #
25561923

 

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