Temporary Celiac Ganglion Block as a Test Before Celiac Ganglion Resection for Dysautonomia-Related Bowel Dysmotility
Temporary Celiac Ganglion Block as a Test Before Celiac Ganglion Resection for Dysautonomia-Related Bowel Dysmotility
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)
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