S6211 Let Down Your Hair, Rapunzel—A Unique Case of Wernicke Encephalopathy in the Setting of Rapunzel Syndrome

Introduction: When the undigested hair (trichobezoar) encompasses the entire stomach and extends through the small or large intestine, this is termed Rapunzel Syndrome (RS). While trichobezoars are less than 1% in incidence, RS, specifically, is rarer. Wernicke encephalopathy (WE) from RS has no specific incidence or prevalence data available, making it a highly unique presentation. We introduce an exceptional case of a woman admitted for diabetic ketoacidosis treatment. Simultaneously, she was diagnosed with WE and dysphagia secondary to RS. Case Description/Methods: A 21-year-old woman presented with vomiting, altered mental status, fifteen pounds of weight loss, and dysphagia to solids for the last 2 months, which eventually progressed to liquids in the previous 2 weeks. At admission, she was hemodynamically stable. The pertinent examination revealed dry oral mucosa; she was alert and oriented to time and place. The labs were consistent with diabetic ketoacidosis, which was managed with aggressive intravenous (IV) fluids and insulin drip. During the hospitalization, she developed weakness in both lower extremities, intermittent confusion, and a new nystagmus. Magnetic resonance imaging (MRI) of the brain with contrast demonstrated enhancement of the bilateral mammillary bodies, a classic finding suggestive of Wernicke's Encephalopathy (WE). Autoimmune and infectious workups resulted negative. The iron panel was consistent with iron deficiency anemia. The MRI of the abdomen showed dilation of the stomach through the proximal jejunum and signs of partial obstruction of the jejunum. Subsequently, the patient underwent an esophagogastroduodenoscopy, which revealed a large trichobezoar extending up to the third portion of the duodenum. Attempted removal with a net or snare was unsuccessful, prompting anterior gastrostomy with successful removal of the trichobezoar. Following this, a psychiatrist evaluated her and attributed her symptoms to undiagnosed chronic anxiety. Her symptoms improved, and she was discharged on oral iron, thiamine supplements, and fluoxetine. Discussion: The RS complications include gastric obstruction and nutritional deficiencies. WE's association with RS has not been reported to date. Therefore, we aim to educate healthcare professionals about rare presentations and various nutritional complications of RS. Moreover, this case highlights how real-time collaboration, an integrated cross-specialty approach, can rapidly adapt to evolving complications of RS, and timely interventions can improve outcomes and survival.