Case Presentation:

A 32–year–old woman presented with non bloody vomitus and generalized weakness for 3 days. Medical history was significant for diabetes mellitus type 1 and diabetic ketoacidosis (DKA) in 2006. She was not compliant with medication. On admission, the patient was tachypneic, tachycardiac but her exam was otherwise unremarkable. Initial laboratory work included serum glucose of 1250 mg/dL, anion gap of 27 mmoL/L and a urinalysis corroborating the diagnosis of DKA. By hospital day 2, after aggressive insulin therapy and fluid resuscitation, her hyperglycemia improved; however, she developed right ear pain and sore throat. She denied hearing loss, dysphagia and odynophagia. Although otalogic and oropharyngeal inspection were normal, amoxicillin/clavulanic acid was started for suspected acute otitis media (AOM). Throat culture was negative. Symptoms persisted and could not be explained by CT of the temporal bones. On hospital day 9, our patient noted epigastric pain reproducible by palpation. Hematocrit remained stable. Esophagogastroduodenoscopy (EGD) performed demonstrated necrosis of the distal and mid esophagus with severe mucosal erythema and erosion demarcated by a normal gastroesophageal (GE)junction. Biopsy specimens revealed extensive inflammation without evidence of infection or malignancy. Treatment with esomeprazole and sucralfate was initiated. Over the next 2 weeks our patient’s sore throat and ear pain slowly resolved and at discharge she tolerated a full diet and was discharged home with a diagnosis of Acute Esophageal Necrosis (AEN).

Discussion:

AEN is rare condition associated with significant mortality. EGD often demonstrates black pigmentation of the distal esophagus ending at the GE junction. Men are more often affected, usually in the sixth decade of life. Common causes include gastric outlet obstruction, ischemia or any condition causing hypoperfusion and volume depletion. Patients usually present with upper gastrointestinal(GI) bleeding. Treatment is focused towards the underlying cause as well as conservative management. Esophageal stricture is the most common complication. We present a case of AEN in a young woman with DKA presenting as otalgia and sore throat.

Conclusions:

While AEN is rare, an association with DKA has been described. Unlike our patient, elderly men are most often affected. AEN involving more than the distal esophagus is unusual. To our knowledge, this case depicts the first presenting symptoms of AEN solely as otalgia and sore throat. The mechanism for this referred pain may be explained by the proximal location of disease. Early recognition of AEN is important to facilitate diagnosis and appropriate management, and reduce mortality.