Triple A syndrome with ophthalmic manifestations in two siblings.
Indian J Ophthalmol
;
2007 Jul-Aug; 55(4): 304-6
Article
in English
| IMSEAR
| ID: sea-70979
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.
Full text:
Available
Index:
IMSEAR (South-East Asia)
Main subject:
Female
/
Humans
/
Male
/
Addison Disease
/
Radiography, Thoracic
/
Dry Eye Syndromes
/
Esophageal Achalasia
/
Child
/
Siblings
/
Diagnosis, Differential
Type of study:
Diagnostic study
Language:
English
Journal:
Indian J Ophthalmol
Year:
2007
Type:
Article
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