A 35 year old nurse of Hispanic origin is referred to a gastroenterologist by her primary care physician (PCP) because of several episodes of severe upper abdominal pain that have occurred over a six month period. When it occurs, the pain is always located in the right upper quadrant of the abdomen, builds up to a steady and intense level, lasts 30 minutes to one hour, and is of sufficient severity to disrupt her normal activities (Box 1, Fig 1). It often radiates to the right subscapular region. She has not been able to identify any definite precipitating factors to the pain, although on two occasions it has occurred soon after her evening meal. The pain has woken her from sleep on one occasion. On several occasions she has experienced diaphoresis and nausea and vomiting during the episode of pain. The pain is not relieved by defecation or passage of flatus (Box 2), and she reports that it is not triggered by movements or lifting (Box 4). She has taken an antacid on two occasions during an episode of pain but this did not produce any improvement in the pain (Box 6). In between attacks, she does not suffer from other gastrointestinal symptoms apart from occasional heartburn, and her weight has been steady. The patient has no significant past medical history. She takes no regular medications and does not smoke or drink alcohol. In her family history, the patient reports that her mother suffered from “gallbladder trouble” that had been difficult to diagnose, but had eventually been cured by cholecystectomy.
The gastroenterologist obtains the further history that one attack of severe pain had caused her to be taken to the emergency room, where she was found to be afebrile and to have no specific abdominal abnormalities on physical examination. The discharge summary from that hospital visit states that her white cell count was normal, as were standard liver biochemistry and serum amylase and lipase. An abdominal ultrasound scan (Box 10) had also been performed and was reported as unremarkable, although the quality of the images was poor because of her body habitus and the presence of bowel gas.
Physical examination performed by the gastroenterologist does not reveal any specific abnormalities. Despite the apparent lack of response to non-prescription antacids, the gastroenterologist recommends upper GI endoscopy (Box 8). This reveals no relevant abnormality. The gastroenterologist then requests a CT scan to exclude any intra-abdominal lesion, and to obtain better images of the gall bladder (Box 12). The CT scan is also negative, with a normal appearing gallbladder and normal bile duct size, and a normal appearing pancreas.
The patient continues to experience similar episodes of severe abdominal pain, despite avoiding high-fat meals. A trial of antispasmodic therapy prescribed by her PCP does not produce any improvement in the pain. The patient and her mother are both convinced that the gallbladder is causing her symptoms, and press the gastroenterologist for a surgical referral. However the gastroenterologist feels that further investigation is warranted; she discusses with the patient the pros and cons of several possible additional tests to more definitively exclude gallstone and gallbladder disease, including magnetic resonance cholangio-pancreatography (MRCP), endoscopic ultrasound (EUS) of the gallbladder and biliary tree, and duodenal aspiration for biliary crystals (Box 12). EUS is only available at a town 50 miles away, while the gastroenterologist is not convinced of the value of examination for biliary crystals. After discussion, she recommends a nuclear medicine DISIDA scan, to determine the ejection fraction of the gallbladder, having confirmed that the patient is not taking any medications that could affect the results of the test. The investigation reports her ejection fraction to be only 20% (Box 13). On this basis the gastroenterologist makes a diagnosis of functional gallbladder disorder (Box 15). She suggests a two month therapeutic trial with a low dose tricyclic antidepressant agent (Box 16), but the pain episodes continue despite this. She then recommends that the patient see a biliary surgeon for consideration of cholecystectomy (Box 17).
The surgeon had just attended a seminar which raised doubt on the value of the DISIDA test in this setting, as several studies had shown little or no correlation with the outcome after cholecystectomy. However, he agrees with the gastroenterologist that the clinical features are strong, that the family history adds some further support, and that there is no evidence for other disorders that may explain the pain. He performs laparoscopic cholecystectomy (Box 17) without complication. Histology of the gallbladder reveals a mild degree of “chronic cholecystitis”. The patient made a good recovery and was free of symptoms when seen a year later by her internist for a routine check-up (Box 18). Had the outcome not been satisfactory, the gastroenterologist had planned to reassess the patient for other functional GI disorders (Box 14), and to consider the possibility of sphincter of Oddi dysfunction (Box 19) (see ‘post-cholecystectomy biliary-like pain’ algorithm).
