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Triple A syndrome with ophthalmic manifestations in two siblings.
[triple a syndrome]
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
.
Diseases
Validation
Diseases presenting
"absence of palpebral portion"
symptom
triple a syndrome
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