temporary celiac ganglion block as a test before celiac ganglion resection for dysautonomia-related bowel dysmotility

Conditions: Dysautonomia

Summary: dysautonomia is malfunction of the autonomic nervous system. it usually results from overactivity of the sympathetic portion and over-secretion of acetylcholine. symptoms depend on the organ involved by this sympathetic overstimulation. involvement of the gastrointestinal system results in chronic dysmotility, nausea, vomiting, food intolerance, weight loss and need for feeding tube placement and/or parenteral feeding. autonomic celiac ganglia resection has been shown to alleviate symptoms as it interrupts the sympathetic stimulation to the gastrointestinal (gi) system, however there is no pre surgery test to confirm the diagnosis. the investigators' objective is to temporarily block the celiac ganglion with a long acting anesthetic (liposomal bupivacaine). if symptoms abate the diagnosis is confirmed and patient will proceed to surgery.

problem: dysautonomia is malfunction of the autonomic nervous system. it usually results from overactivity of the sympathetic portion and over-secretion of acetylcholine. symptoms depend on the organ involved by this sympathetic overstimulation. a partial list of dysautonomia-related conditions is shown below: affected level manifestation cardiac innervation postural orthostatic tachycardia syndrome skin hyperhidrosis arteries raynaud's phenomenon kidneys hypertension small nerve fibers reflex sympathetic dystrophy pain fibers complex regional pain syndrome gi system chronic gi dysmotility/irritable bowel syndrome (ibs) the last one (chronic gi dysmotility) usually affects young females, presenting after puberty. symptoms include chronic abdominal pain, intestinal angina, chronic nausea/vomiting, inability to take po, need for total parenteral nutrition (tpn) or g-tube feeding. further, the patients' condition is often complicated by opioid dependence, malnutrition, weight loss, social isolation etc. many such patients are misdiagnosed as having median arcuate ligament syndrome (mals) and are referred to surgery for ligament release. though some of the patients do get partial relief, this temporary relief is due to the partial interruption of the sympathetic nerves during surgery. repeat surgery to complete celiac ganglion resection is often necessary. occasionally surgeons refer patients for a computer tomography (ct) guided temporary celiac ganglion block to confirm (or exclude) sympathetic system dysfunction as the cause of the patients symptoms. however negative or positive predictive value of this test has not been studies rigorously. research hypothesis: the investigators' hypothesis is that a low-risk, outpatient test can confirm (or exclude) dysautonomia as the cause of the patient's symptoms. the experimental test is ct-guided, celiac ganglion temporary block with liposomal bupivacaine. importance of the research: some of the patients who are diagnosed as having mals have in fact dysautonomia and have the wrong surgery. many other patients with dysautonomia-related gi symptoms are not diagnosed at all and offered only symptomatic treatment. the development of ct-guided, celiac ganglion temporary block with liposomal bupivacaine as a low-risk confirmatory test for dysautonomia-related gi symptoms, will improve surgical outcomes and afford a novel treatment option to many patients. 2. objectives ct-guided, celiac ganglion temporary block with liposomal bupivacaine will eliminate sympathetic input to the bowel. its half-life is 24 hours and therefore symptom relief can be distinguished from overlap due to procedural sedation. primary: 1. improved tolerance to per os (po) solid food intake 2. decrease in abdominal pain (both at baseline and that associated with po intake) secondary: 1. decrease/elimination of pre-existing nausea/vomiting frequency and severity 2. decrease/elimination of analgesic use 3. background experience with procedure: ct-guided, celiac ganglion temporary block with liposomal bupivacaine. the pi, dr. georgiades has performed ct-guided nerve blocks hundreds of times, including celiac ganglion block, over the past 15 years. nerve and specifically celiac ganglion block is an approved procedure for abdominal pain treatment, and is performed by interventional radiology at johns hopkins. dr. georgiades is a full time faculty in the division of interventional radiology. he has privileges for performing ct-guided celiac ganglion block, as well as conscious sedation. dr. georgiades will be the only investigator performing this procedure in the test population. clinical data (with medication): liposomal bupivacaine has long been used as an effective local analgesic, especially in orthopedic and plastic surgery. clinical data (celiac ganglion block): celiac ganglion block has been used for over a century for the treatment of abdominal pain. ct-guided celiac ganglion block has been introduced in the 1950s and most commonly performed with a combination of lidocaine and alcohol for permanent celiac ablation. experience with medication: liposomal bupivacaine is an (food and drug administration) fda approved drug and has long been used as a local anesthetic. it is simply a long acting formulation of bupivacaine. bupivacaine's half-life is 2.7 hours. since the procedure is performed under conscious sedation, the effects of bupivacaine cannot be distinguished from those of the medication given for sedation (versed and fentanyl). the half-life of liposomal bupivacaine on the other hand, is approximately 24 hours. therefore any symptom relief on post-test day #1 or 2 can be attributed to celiac ganglion blockade and not to sedation medication. liposomal bupivacaine is available in 266 mg vials which is the maximum recommended single dose for adults. 4. study procedures a. study design, including the sequence and timing of study procedures (distinguish research procedures from those that are part of routine care). the research protocol is highlighted in light brown below. all else is part of the patient's standard of care treatment. there are two potential patient populations than are candidates for the research protocol. 1. those with dysautonomia-related gi dysmotility, misdiagnosed as having mals after mals surgery fails to relieve symptoms, and 2. those with known dysautonomia-related dysmotility and related symptoms. the research procedure includes the ct-guided celiac ganglion block only. this test will be used to confirm (or exclude) dysautonomia as the patient's cause of gi symptoms. those who have symptom relief after the test block, will proceed with open celiac ganglion resection. treatment description: 1. selection/recruitment potential population pool (ppp): adolescents and adults with diagnosis of : 1. median arcuate ligament syndrome (mals) 2. both mals \& postural orthostatic tachycardia syndrome (pots) 3. pots or other dysautonomia symptoms \& bowel dysmotility study population (subgroup of ppp): 1. mals patients with little or no improvement after laparoscopic arcuate ligament release 2. mals \& pots patients with little or no improvement after laparoscopic arcuate ligament release 3. pots/dysautonomia patients with bowel dysmotility symptoms: required: chronic nausea, food intolerance, abdominal pain, additional: chronic vomiting, need for enteral or parenteral nutrition, hyperhidrosis, complex regional pain syndrome, raynaud's exclusion criteria: evidence for non-dysautonomia related causes of the patient's symptoms. must exclude: chronic cholecystitis, gastritis, peptic ulcer disease, gastro esophageal reflux, celiac disease, mesenteric atherosclerotic disease, vasculitis, anorexia, depression, other psych issues, etc complete compass 31 (validated autonomic function score)

Eligibility criteria:
inclusion criteria: * patients with dysautonomia-related gi dysmotility, misdiagnosed as having mals after mals surgery fails to relieve symptoms * patients with known dysautonomia-related dysmotility and related symptoms exclusion criteria: * allergy to liposomal bupivacaine * platelets \< 50 thousand * international normalised ration (inr) \> 1.7

Keywords: celiac ganglion block resection bowel dysmotility