Clinical significance of manometric assessment of both pancreatic duct and bile duct sphincter in the same patient

In this study both pancreatic and bile duct sphincter pressures were measured on the same occasion by means of endoscopic manometry in 42 patients with long-standing upper abdominal pain. Nine (53%) of the 17 patients with abnormal sphincter function had a marked difference between the pancreatic duct sphincter pressure (PSOP) and the bile duct sphincter pressure (BSOP): 6 patients with a clinical diagnosis of biliary dyskinesia showed elevated BSOPs, whereas the PSOPs were normal. The reverse, an abnormal PSOP but normal or only a slightly elevated BSOP, was registered in the three patients with chronic pancreatitis. These findings indicate that a motor abnormality may be restricted to only one of the sphincters. Thus, when the sphincter of Oddi is investigated only from the pancreatic duct, manometry may either fail to show an abnormal BSOP in some patients with biliary dyskinesia, or it may falsely suggest this diagnosis in patients with unrecognized pancreatitis.     

Bile duct disorders

Drug-induced bile duct injury related prolonged or chronic cholestasis is recognized as a common side effect of treatment with several drugs. The severity and duration of the clinical symptoms suggest that this increase in number of reports is not only related to clinician and pathologists being increasingly aware of the condition, but also may represent a true increase in incidence likely related to a time-related growing experience with newer drugs. This clinical presentation encompasses a wide variety of features that may be the source of diagnostic difficulties, especially in the cases where cholestasis occurs days or weeks after the completion of therapy. Even more puzzling is the initial picture of hepatocholangitis, which may be silent and ensuing bile duct paucity with chronic anicteric cholestasis may be another source of diagnostic difficulties in the long-term. These diagnostic difficulties suggest that some of the cases of the so-called "idiopathic adulthood ductopenia" may originate from overlooked drug induced vanishing bile duct syndrome. The pathogenesis of the syndrome remains largely unknown and the determinants of prognosis and outcome. From reproducible data obtained in different studies investigating HLA-dependent predisposition, one may assume that genetics plays a major role even if other unknown additive factors are also likely involved. Severity of initial hepatocholangitis is likely to represent another important determinant of severity and prognosis, however to be assessed in larger longitudinal studies. Therapy of large bile duct injury mimics that of primary sclerosing cholangitis. Treatment of small bile duct injury remains disappointing. Corticosteroids are invariably ineffective. Ursodeoxycholic acid as been shown to induce improvement of clinical and biochemical cholestasis in some selected cases, its efficacy being however unpredictable. Preliminary data about the natural history of the vanishing bile duct syndrome suggest that therapy might be more effective when initiated early.     

Clinical aspects and manometric criteria in achalasia.

Achalasia is the best known primary motility disorder of the esophagus. Dysphagia is the main symptom, intermittent at the beginning, but becoming more marked with evolution. Although some peculiarities are noted, they are not sufficiently characteristic to establish the diagnosis. Chest pain is often associated with dysphagia and may be the prominent complaint in the early stage of the disease. Dynamic investigations, mainly esophageal manometry, are needed for the diagnosis and follow-up after treatment. Three findings are commonly recorded: increase in lower esophageal sphincter pressure, lack of relaxation and absence of peristalsis, the latter being indispensable for the diagnosis of achalasia. On the basis of manometric findings, achalasia is easily differentiated from other primary motility disorders, i.e. diffuse esophageal spasm, nutcracker esophagus, but non-specific esophageal motility disorders are frequent. Manometry is also an objective method of assessing the effectiveness of treatment--i.e. surgical myotomy or balloon dilatation--of the lower esophageal sphincter.     

Bile duct laparoscopic exploration

The use of ERCP with endoscopic esphincterotomy (ES) for the management of choledocholithiasis has replaced almost completely common bile duct exploration in the era of laparoscopic cholecystectomy. Once the procedure is completed it is followed by laparoscopic cholecystectomy in the same hospitalization. During the last few years, the development of new technology and equipment with possibilities of minimal invasive procedures and diagnosis, as well as the ongoing surgical skills has allowed surgeons to solve the problem of choledocholithiasis in one minimal invasive procedure, decreasing effectively morbidity with a high success rate, changing again ERCP with ES as a complement and not as a substitute for surgery. Nevertheless the initial step in the use of these techniques require of a special technical support and what is most important capacitation to achieve the desire objectives. Transcystic technique seems to be the most promising and choledochotomy with primary closure in cases that cannot be solved with the transcystic approach. Regardless of the approach the surgeon must be familiar with both and increasingly with the use of the endoscope in order to provide patients with advantages of minimal invasive surgery and all its advantages. We also discuss our series of 81 patients operated on with laparoscopic surgery with a high rate of success, low morbidity and mortality.     

Bile duct carcinoma mimicking chronic liver disease.

In a case of cholangiocarcinoma involving the bifurcation of the common duct, the association with striking intermittence of jaundice and with signs of chronic liver disease presented a diagnostic enigma. Disappearance of jaundice does not rule out the diagnosis of bifurcation tumors and may be misleading. Adequate demonstration of the biliary tree, best achieved with percutaneous transhepatic cholangiography, is essential for early diagnosis of this entity.     

Clinical and ultrastructural study on primary ciliary dyskinesia

We evaluated laboratory and radiological findings and examined tracheobronchial cilia by transmission electron microscopy in 9 patients with primary ciliary dyskinesia (PCD), in order to elucidate the clinical pictures of PCD and the relationship between PCD and diffuse panbronchiolitis (DPB) which was proposed as a new disease entity in Japan in 1969. The clinical pictures of our PCD patients were almost the same as that already described in several articles in Europe and North America; early onset of respiratory symptoms, high incidence of chronic sinusitis and otitis media exudative as well as infertility, continuous infections in the lower respiratory tracts (Hemophilus influenzae, Pseudomonas aeruginosa etc.). Tracheobronchial cilia obtained by brushing technique were immotile (6 out of 8 patients) or dyskinetic (2 out of 8 patients). Ultrastructural study of cilia revealed the lack of dynein arms in all patients: the lack of both outer and inner arms (4 patients), the lack of outer arms (2 patients), the lack of inner arms (2 patients). Chest X-ray films revealed situs inversus in six out of nine patients. According to the radiological findings (chest X-ray film, CT-scan, bronchogram), the patients were divided into three groups; I: localized bronchiectasis (5 patients), II: diffuse micronodular lesions without definite bronchiectasis (3 patients), III: diffuse micronodular lesions with bronchiectasis (1 patient). Two patients of the second group satisfied the clinical diagnostic criteria for DPB (Chest 83:63, 1983). In conclusion, PCD can cause a variety of respiratory tract lesions such as bronchiectasis, DPB and other types of peripheral airway disorders.     

Bile duct hepatocellular carcinoma thrombi

Hepatocellular carcinoma (HCC) is the most common primary liver malignancy and the third cause of death related to cancer. Through the application of surveillance programs the percentage of early diagnosis has increased but the diagnosis is still made at advanced stages in some patients. The presentation of HCC as progressive jaundice secondary to bile duct tumor thrombi is uncommon. In such cases it is extremely difficult to distinguish such biliary lesions from cholangiocarcinoma or even common bile stones. We report a case of a 34-year-old male patient with common bile duct invasion secondary to HCC that mimicked choledocholithiasis. The diagnosis of HCC was confirmed after thrombi extraction during ERCP.     

Bile duct emptying in response to fat: a validation study

Fatty meal sonography has been suggested to assess patients with biliary pain after cholecystectomy, but the effects of gallbladder removal on biliary dynamics has not been studied prospectively. Before elective cholecystectomy, 25 patients had their common hepatic ducts' diameter measured by ultrasonography before and after a fat stimulus. In 23, tests were repeated 1 month, 1 year, and 5 years after surgery. In preoperative studies, 5 patients showed dilatation after fat and 2 of these had stones in the common bile duct. However, another 4 patients with stones or sludge in the duct did not show dilatation, so that the response to fat was a poor indicator of patients requiring common bile duct exploration. No patient had major symptoms after surgery. At 1 month and 12 months, the response to fat was variable with more than half of those tested showing no decrease in duct size. A more consistent pattern emerged at 5 years, when 14 of 18 patients tested showed a decrease in common hepatic duct after fat; 3 were unchanged and 1 increased by 1 mm. The response to fat was less consistent and more difficult to measure in the common bile duct, even 5 years after operation. It was concluded that not all patients with indications for exploration of the common bile duct on operative cholangiography show a dilatation response to fat on preoperative testing. Also, fatty meal sonography should be used with caution because the response to fat in asymptomatic patients soon after operation is unpredictable, with occasional patients showing dilation without apparent obstruction. Measurement of common hepatic duct is preferred to common bile duct and increases in diameter of 1 mm are probably not significant.     

Bile duct cysts in adults. Pitfalls in diagnosis and management

Six women have been referred to us for treatment of bile duct cysts during the past 6 years. In three patients the disorder was misdiagnosed by the referring institution, and they were admitted because of continuing severe symptoms; two of them had been treated with internal drainage procedures which led to unnecessary treatment delay and complications. Two patients had developed cyst carcinoma, the most feared complication of cyst disease. It is concluded that clinical symptoms are nonspecific, and the diagnosis is only ascertained after complete cholangiographic imaging of the biliary tree. Primary cyst excision and hepaticojejunostomy is the treatment of choice.     

Episodic laryngeal dyskinesia. Clinical and psychiatric characterization

We have obtained physiologic and psychiatric evaluations on five subjects with episodic laryngeal dyskinesia (LD) and compared them with three patients with expiratory laryngeal stridor and asthma (ELS), and five with chronic asthma (CA). Laryngoscopy confirmed adduction of the vocal cords. Diminished inspiratory flow rates with an expiratory/inspiratory ratio of 1.5 to 3.3 was demonstrated by flow volume studies. Flows improved strikingly while breathing an 80 percent helium/20 percent oxygen mixture. Patients with LD showed varying degrees of depression and sought some form of secondary gain. A histrionic personality, conversion or factitious disorders are not an essential part of this syndrome. Tracheostomy may seldom be necessary in the managing of the acute crisis of LD. Reassurance, oxygen, intermittent positive pressure, and sedation may be sufficient. Mildly depressed patients decreased the frequency and severity of wheezing episodes after receiving reassurance and a clear explanation of ventilatory mechanics.     

Bile duct hamartomas-the von Meyenburg complex

IntroductionA 42-year-old female patient with symptomatic cholelithiasis presented clinically with dyspepsia. The results of preoperative clinical and laboratory tests were normal. No co-morbidities were observed. Abdominal ultrasonography did not display any alterations in the hepatic parenchyma, but a gallbladder full of calculi and slight thickening of the gallbladder wall, characteristic of acute cholecystitis. Upper gastrointestinal endoscopy demonstrated gastro- esophageal reflux diseas…     

Bile duct hamartomas--the von Meyenburg complex

Hamartomas of the bile duct (von Meyenburg complex) are benign neoplasms of the liver, constituted histologically cystic dilatations of the bile duct, encompassed by fibrous stroma. We report a 42-year-old female patient with symptomatic cholecystitis, whose gross and ultrasonic appearance suggestive of multiple liver metastases. Magnetic resonance imaging and liver biopsy are the gold standards for diagnosis of this rare hepatobiliary condition.