Multifactorial Etiology of Worsening Gastroparesis in a Patient With Ehlerʼs Danlos

Abstract

A 27 yo female with a history of dystonia, chronic migraines, motor tics, Ehler's Danlos Syndrome and Chiari malformation presented with persistent nonbilious emesis, headaches, nausea and early satiety. The patient had a posterior fossa craniotomy with placement of a DuraGen dural graft matrix 6 weeks prior, and lost 15 pounds due to severe nausea and vomiting with inability to tolerate liquids or solids. On MRI of the head she was found to have a stable suboccipital craniotomy. She also underwent a lumbar puncture with negative cultures. A NM gastric emptying study was performed which demonstrated significant retention of gastric contents calculated to be 25% at 4 hours. Endoscopy with botox injection of the pylorus was performed. Over the next few months her nausea and vomiting persisted despite treatment with ondansetron, prochlorperazine maleate, trimethobenzamide, dexamethasone, domperidone, dronabinol, anti-migraine medications, IV caffeine drip and promethazine. A jejunostomy tube was placed given ongoing malnutrition. During this time, she developed a pruritic urticarial rash at the site of her cranial decompression. In order to control her pruritus she required high doses of diphenhydramine, given lack of response to hydroxyzine; 100mg oral 3-4 times a day. An allergic reaction to the prolene sutures or gortex dura graft was suspected with the recent posterior fossa craniotomy. Allergy skin testing was performed with placement of prolene sutures and a gortex patch on the patient's forearm. She was positive for a prolene allergy and was subsequently taken to the operating room with removal of her prolene sutures. As the urticaria and pruritus resolved she no longer required diphenhydramine. Her nausea and vomiting resolved and she gained 28 pounds. She no longer required J tube feedings. This case emphasizes the importance of medication-induced delay in gastric emptying from anti cholinergic agents. Medication induced gastroparesis should be considered even in patients with EDS in whom an etiological diagnosis of gastroparesis is common. It is critical to discontinue narcotics and other medications that affect gastric emptying at least 48 hours prior to a gastric emptying test. Once diagnosis is confirmed, management should include assessment and correction of nutritional state, relief of symptoms, improvement of gastric emptying, and glycemic control in diabetics.