Esophageal dysphagia as the sole symptom in type I Chiari malformation

Chiari malformation, also called Arnold-Chiari deformities, are rare hindbrain herniations that may present in children or adults. The most common symptoms include headache, syncope, disordered eye movement, sensory loss, weakness, and cerebellar features such as ataxia. Dysphagia occurs in 5-15% of patients, although only a few reports describe dysphagia as the only presenting symptom. We report a case of a 27-year-old woman who presented with a three-year history of dysphagia, chest pain, and weight loss. Esophageal manometrics revealed markedly disordered esophageal motility and gastroesophageal reflux. Her symptoms failed to respond to high doses of omeprazole, prokinetics, and eventually surgical fundoplication. The subsequent onset of neurological symptoms led to the diagnosis of Chiari type I malformation. Following posterior craniotomy with decompression, her dysphagia and chest discomfort completely resolved. Repeat esophageal manometrics revealed complete resolution of prior abnormalities.