SUCCESSFUL MANAGEMENT OF 21 YRS OF DYSPHAGIA DUE TO AN UNUSUAL CAUSE

Abstract

A 24 years old lady presented with dysphagia
and regurgitation since her childhood,
inadequate weight gain and delayed
mile stones. History of consanguineous
marriage and history of surgery
for dysphagia to her paternal aunt were
present. At 3 years of her age, barium
swallow and thyroid function tests were
reported to be normal while ophthalmological
consultation revealed she had
refractory error and dry eye for which
corrective glasses and artificial tears
were prescribed. Psychiatric consultation
for delayed mile stones revealed she had
mild mental retardation. At 14yrs of age
upper GI scopy was reported to be normal.
At 19 yrs of age, barium swallow
showed features suggestive of achalasia
and she has been treated medically. At
24yrs of age, barium swallow showed
sigmoid esophagus with tight LES. She
presented to our institution only at this
juncture and evaluation revealed her to
have 4A or Allgroves syndrome .After
complete evaluation, she underwent long
cardiomyotomy and she is fine on regular
follow up. This case is presented for its rarity
and need for high index of suspicion