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

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