Allgrove'S syndrome: case report and literature review

Abstract

This report concerns two brothers aged 10 and 18 years with long-standing dysphagia that started at age three and six years respectively. They had been diagnosed as achalasia and treated accordingly. The appearance of additional symptoms and clinical signs required further investigations including abdominal sonography, esophago-gastroduodenoscopy, barium swallow, esophageal manometry, computerized tomography (CT) of abdomen and brain, biochemical profiles, and neurologic and ophthalmic evaluations. The results of these extensive investigations along with the clinical evaluations were consistent with Allgrove's syndrome.Glucocorticoid therapy was initiated. The management consisted of pneumatic cardiac dilatation and initiation of cortisone treatment. The patients' response was impressive and they resumed most of their usual activities.

Keywords: Achalasia; Adrenocortical impairment; Alacrima; Allgrove's syndrome; Autonomic neuropathy; Pneumatic dilatation; Steroid therapy; Triple A syndrome.

Figure 1

Patient during admission in the hospital

Figure 2

Patient during follow-up in OPD