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We analyzed 82 cases of hepatobiliary fibropolycystic disease (FPD) that were seen at the Instituto Nacional de la Nutrición "Salvador Zubirán" in Mexico City in the thirty-year period comprised from 1956 to 1986. The different entities that compose FPD were distributed as follows: 61 (74%) cases of polycystic liver disease, 13 (16%) cases of choledochal cyst, and 8 (10%) cases of congenital hepatic fibrosis; there were 5 (6%) cases of Caroli's disease, 3 associated with congenital hepatic fibrosis and 2 with choledochal cyst. Polycystic liver disease predominated in females (67%) and presented at 54 +/- 12 years (mean +/- SEM) with pain, a mass, symptoms related to renal insufficiency or incidentally; polycystic kidneys were present in 61%. Liver function tests were normal in 94%. Choledochal cyst also predominated in females and presented at a mean age of 19 years with cholangitis. Liver function tests were abnormal in 69%. Congenital hepatic fibrosis (50% male) presented with variceal hemorrhage or cholangitis (in 3 patients associated with Caroli's disease). Polycystic kidneys were present in five patients. Four of the five patients with Caroli's disease were female and presented at a mean age of 19 years with cholangitis. It never presented as an isolated disease, but was associated more frequently to congenital hepatic fibrosis. The diseases that are part of the hepatobiliary polycystic disease vary in severity and thus the prognosis in an individual patient is determined by the type of fibropolycystic disease present. This is the largest series of this disease published in our country.

The author reports about five patients suffering from a primary fibrotic stenosis of the biliary system. All patients were assigned into hospital with a painless jaundice. In all cases a diagnosis was secured histologically. Four patients had a stenosis of the biliary system and a hepatico-jejunostomy as Y-anastomosis was possible to be applied. In one case both Ductus hepatici were taken ill symmetrically. A biliodigestive anastomosis was not practicable. After having widened the narrow intra-hepatitic biliary system, the stenosis appeared once more. It could not be found that the total intra- and extra-hepatic biliary system had fallen ill symmetrically. The cause of the illness is still uncertain. Based on clinical and histological findings an inflammatory process is not probable. The pathological anatomical findings were marked by a network-like filament of collagene without inflammation.

The etiology of choledochal cyst has been investigated by clinicians and pathologists primarily of Germany, Austria, United States, United Kingdom, and Japan from the end of the last century, but Japanese investigators contributed greatly to the study of the relationship between choledochal cyst and anomalous junction of the pancreaticobiliary ductal system. Heid (1893) is considered to be the first to document choledochal cyst associated with anomalous pancreaticobiliary junction, but it was probably Kozumi et al. (1916) that first evaluated anomalous junction in relation to the etiology of choledochal cyst. Yotsuyanagi (1936), who is noted for his work on the etiological studies of choledochal cyst, attached particular importance to anomalous junction and attempted to establish a relationship between dysplasia of the primordial common bile duct and its anomalous junction with the pancreatic duct. It is no exaggeration to say that this area was led by Japanese investigators even after the introduction of Babbitt's anomalous junction theory (1968). In this review, etiological theories of choledochal cyst and their changes with time were discussed separately for those before and after the advent of Babbitt's anomalous junction theory (1968) with particular reference to the causative relationship between anomalous junction and choledochal cyst. Although there is as yet no definite answer to this problem, the theories advanced to date are considered to be summarized as the following three major lines of thought: 1) bile duct dilatation and anomalous junction are both congenital dysplasia occurring simultaneously during the embryonic development, 2) bile duct dilatation is a secondary change induced by influx of pancreatic juice into the biliary tract due to anomalous pancreaticobiliary junction, and 3) bile duct dilatation is induced secondarily by stenosis of the distal bile duct, which is a part of complex anomaly of the pancreaticobility system. Which of these etiological mechanisms is more important than others must still be clarified, but these three factors are considered to be interrelated with one another in the etiology of choledochal cyst.

Histochemical and immunohistochemical studies on 47 consecutive specimens excised for choledochal cyst were performed to clarify possible metaplastic changes of the biliary duct in relation to carcinogenesis. An anomalous arrangement of the pancreaticobiliary ductal system was observed in all 39 cases examined. Among the 47 patients, 5 (10.6 per cent) had biliary carcinoma. 27.3 per cent mucous gland, 13.0 per cent goblet cell and 9.5 per cent argyrophil cell in 23 children. On the other hand, 81.8 per cent exhibited mucous gland, 41.7 per cent goblet cell and 27.3 per cent argyrophil cell in 24 adults. These metaplastic changes seemed to be an intestinal metaplasia and increased with age. Immunoreactive-gastrin or -somatostatin were evident immunohistochemically in 4 adults. These findings confirmed that intestinal metaplasia may develop in the biliary duct in cases of choledochal cyst. Although direct evidence between intestinal metaplasia and the development of biliary carcinoma was not found, reflux and stasis of pancreatic enzymes in the biliary duct may relate to the development of intestinal metaplasia and be an important factor related to the carcinogenesis of choledochal cyst.

The multifocal eosinophilic granuloma syndrome (Hand-Schüller-Christian disease) is characterized histologically by focal accumulations of histiocytes and eosinophils. The clinical manifestations of this syndrome reflect the site and extent of these focal accumulations which are usually found in membranous bone, skin, and lung. Significant hepatobiliary disease due to this disorder has not been previously described. We are reporting an adult female with the multifocal eosinophilic granuloma syndrome who developed a mixed histiocytic and eosinophilic infiltration of the common bile duct which resulted in obstructive jaundice. Therapeutic attempts have failed to reverse this process, and the patient has shown progressive intrahepatic biliary tract destruction.

A case of cholangiocarcinoma arising in a choledochal cyst (type II or biliary cyst in Alonso-Ley classification) in a 53-year-old woman is presented. Ultrasonography and computed tomography provided the correct preoperative diagnosis. The value of these modalities in presurgical planning is illustrated.

To investigate whether cystic dilation may be due to a reduction of postcholinergic cell bodies that leads to autonomic dysfunction, ganglion cell bodies were counted in five patients with choledochal cysts and five patients with normal choledochus. In the normal choledochus, the ganglion cell counts in the proximal and distal portions were 374.7 +/- 82.7/cm2 and 349.2 +/- 51.2/cm2, respectively. In cases of choledochal cysts, the ganglion cell counts in the dilated portion and narrow portions were 298.6 +/- 86.9/cm2 and 81.0 +/- 34.8/cm2, respectively. The cell ratio (narrow to dilated) was 0.271. Significant reductions of cell numbers were observed in all the choledochal cysts having various types of pancreatobiliary junctions. These findings indicate that the reduction in the number of ganglion cells in the narrow portion of the choledochal cysts supports the concept of oligoganglionosis in distal choledochus.

Radiographic studies including operative cholangiography, endoscopic retrograde cholangiopancreaticography and percutaneous transhepatic cholangiography were performed in 44 patients with congenital dilatation of the common bile duct. The relation between the maximum transverse diameter of the common bile duct (CBD) as an indicator of the degree of dilatation and various possible factors causing dilatation of CBD was studied. Among these factors, the length of the narrow segment (X) was closely related with the maximum transverse diameter (Y). The relation of these two was expressed by a simple equation; log Y = 0.068X + 1.06 (r = 0.934, p less than 0.001, n = 19) Dilatation of CBD has been successfully produced by narrowing the ductal lumen with a small plastic tube encirculating the distal common bile duct in dogs. Experimental dilatation was quite similar with congenital dilatation. In addition, the relation between the maximum transverse diameter (Y') and the length of the narrow segment (X') was also expressed in the following simple equation; log Y' = 0.060X' + 0.88 (r = 0.972, p less than 0.001, n = 18) The present studies did not support the hypothesis that abnormal junction of the pancreatico-biliary system might be the primary cause of congenital dilatation of CBD. They suggest that the stenosis of the distal CBD in the fetal life may determine dilatation of CBD.

Out of 921 endoscopic papillotomies (EPT) 110 (12%) were performed in patients with benign stenosis of the papilla of Vater. Among these patients were 96 after cholecystectomy and 14 with gallbladder in situ. The diagnostic aspects of the stenosis of papilla and the complications of EPT were investigated on the basis of these patients. The dilation of the bile ducts is not a specific criteria for the diagnosis of papilla-stenosis. Dilatation could develop after cholecystectomy despite the normal sphincter pressure. The complication rate in patients with stenosis of papilla of Vater is increased (13.6%) in comparison with the patients with biliary stones (8.8%). This can be account for the increased number of haemorrhage and cholangitis.

The use of percutaneous transhepatic biliary drainage (PTBD) has allowed the development of new diagnostic examinations, such as biliary exfoliative cytology. This is extremely important in patients with biliary tract strictures, in order to obtain a diagnosis about the real nature of the stricture. Forty-nine patients underwent exfoliative biliary tract cytology from bile samples. Exfoliative cytology was performed on bile samples taken at different times for each patient. The results of our study proved this examination to be highly specific (there were no false positive samples), although the diagnostic sensitivity was not as high (63.9%). It is important to underline that whenever the epithelium of the biliary tract is involved by neoplasm, the result of examination is always positive. These data may be useful to choose an appropriate surgical procedure.

With regard to observations of 6 children with choledochal cysts, the importance of the anatomic data obtained by ultrasonography is emphasized. In the most common types of cystic dilatation of the common bile duct, the precise details concerning the size and place of the extra-hepatic biliary abnormality make ultrasonography the best pre-operative investigation.

Cystic hydatid disease of the liver is uncommon in the United States. It is caused by the larval cestode Echinococcus granulosus. One complication of this disorder is rupture of the hepatic hydatid cyst into the biliary ducts. There have been few reported cases of this complication of hydatid disease in the US literature. We describe a case of this disorder and briefly review the literature.

Babitt was the first to suggest that the anomalous arrangement of the pancreatico-biliary ducts might be involved in the etiology of congenital choledochal cyst. Since then, many authors have reported this condition frequently associated with congenital choledochal cyst. The reflux of pancreatic juice into the common bile duct due to this anomaly has been thought to be responsible for the formation of choledochal cyst. Our previous study using the animal model with anomalous arrangement of pancreaticobiliary ducts, has proven that pancreatic juice in the common bile duct caused cystic dilatation of the common bile duct. However, the mode of action of pancreatic juice on the biliary duct has remained unclear. The present study using peroxidase as a tracer for permeability of the biliary duct showed early damage of the intercellular junction of biliary epithelium exposed by pancreatic juice, followed by affection of submucosal tissue.

Congenital choledochal cyst (CChC) has a very low frequency in our population; it is more frequent among females and its pattern of inheritance is multifactorial. The frequency of CChC in our population was 1 per 20,000 to 1 per 30,000 hospitalized patients in the pediatrics department of the Hospital Juarez and Hospital Infantil de Tacubaya, respectively. Four new cases of CChC are reported, two school children and two teenagers. Pre-operative diagnosis was accomplished clinically, which is the most precise non-invasive method. The most effective actual diagnostic methods include ultrasonography, CAT scan, and basic laboratory data, which can corroborate the clinical diagnosis of CChC with 100% certainty. The surgical treatment of CChC is controversial, but the surgical procedures of choice are choledochocystojejunostomy with total cyst removal, Roux's Y, and cholecystectomy. One case was treated with latero-terminal choledochocystojejunostomy and 3 cases with total removal of the cyst. The results were excellent.

A case in which a medium-sized common bile duct stone spontaneously passed through the papilla into the duodenum is presented. The naturel course of this patient was followed by repeated endoscopic retrograde cholangiography. Acute ulcerous changes caused by the passage of a gallstone and a surprisingly quick recovery are demonstrated by vivid illustrations. The clinical significance of the phenomenon with regard to the patulous sphincter of Oddi or, in contrast, papillary stenosis, is discussed.

Calculations were made to establish the action of probes of different thickness on both cicatric tissue and unstriated muscles of the major duodenal papilla. The findings are graphically represented. These calculations were based on histopathological findings recorded from the major duodenal papilla, expandibility, of collagenic fibres and unstriated muscles, and morphometric measurements. The conclusion was drawn that passage of a probe of more than 3 mm in diameter through a cicatrically narrowed papilla constituted an act of therapy or at least had a therapeutic component in addition to probing. The therapeutic range of papillary dilatation is limited to something from three to six millimetres.

The etiology of choledochal cysts is controversial. The authors report two cases which show that type 1 cysts (axial dilatation of the common bile duct and common hepatic duct) may be caused by a biliary web and that some choledochoceles (type 3 cyst) may actually be duodenal duplications (i.e., having an outer wall of smooth muscle lined with gastrointestinal mucosa and being adherent to the gut).

We report a case of a 23 year old woman who presented with symptoms of biliary tract disease to our University Hospital in Bayamón, Puerto Rico. A diagnosis of chronic cholelithiasis was made and the patient was taken to surgery. An adult Fasciola hepatica was recovered from the common bile duct. We review the pathobiology of this uncommon zoonosis.

The properties of the choledochal cyst were compared with those of the normal common bile duct. Cholecystokinin-octapeptide and a high concentration of acetylcholine produced smaller contractions in the strips of the narrow portion of the cyst than in the strips of the dilated portion and the normal common bile duct. Nicotine did not cause contractions in the strip of the narrow portion of the cyst. gamma-Aminobutyric acid (GABA) induced atropine-sensitive, tetrodotoxin-sensitive contractions in the bile ducts through bicuculline-sensitive, furosemide-sensitive GABAA receptors located on the postganglionic cholinergic neuron. The GABA did not induce contractions in the narrow portion of the cyst. The number of ganglion cells was decreased markedly in the same portion. These findings suggest that the choledochal cyst has postganglionic neural dysfunction. This character may be one of the causes of cyst formation.

Five patients with two periampullary choledochoduodenal fistulas diagnosed by endoscopic retrograde cholangiopancreatography (ERCP) are described. In two patients both fistulas were on the oral prominence of the papilla, whereas in the remaining three, one of the two fistulas was more proximal. All five patients presented with right upper abdominal pain, which was associated with chills, fever and jaundice in two. ERCP revealed choledocholithiasis in two patients, cholecystolithiasis in one, hepatocholedocholithiasis in one, and bile duct dilatation without stone in one. A previous history of pain episodes accompanied by jaundice was obtained in four cases, and surgical bougienage of the papilla had been performed during previous common bile duct exploration in two, which may be responsible for the occurrence of multiple fistulas by possible false passage.

A patient with recurrent episodes of acute pancreatitis found to be caused by the presence of a choledochocele is presented. Transhepatic cinecholangiography revealed a choledochocele receiving the terminations of the common bile duct and pancreatic duct, which emptied via a pinpoint opening into the duodenum. With duodenal peristalsis, the contrast material filling the choledochocele could be seen refluxing into the pancreatic duct. A discussion of the possible etiologies of choledochoceles and a review of the existing literature is presented.

In a prospective series of 53 consecutive explorations of the common bile duct, there were 12 patients in whom no contrast material entered the duodenum on postexploratory cholangiography. In 11 of these patients, the choledochoscope had been used to visualize the ductal anatomy and in each instance the ampulla of Vater was visualized from within and judged to be patent. Calibration, not dilation of the ampulla was performed upon ten of the patients. Transient edema or spasm of the ampulla secondary to operative trauma, stone removal or underlying disease was diagnosed and in each patient the operation was terminated after the cholangiogram. Follow-up cholangiography in each instance demonstrated a patent ampulla, free flow into the duodenum and no strictures or stones. It is concluded from these data that with operative choledochoscopy the anatomy and pathologic characteristics of the biliary tract can be visualized and the patency of the ampulla of Vater can be assessed.

Forceps biopsies of biliary stenoses are difficult to direct under fluoroscopy and for this reason may give spurious results. In addition, fluoroscopy does not prevent the electrohydraulic lithotripsy (EHL) probe from damaging the bile duct wall. Retrograde biliary endoscopy with the ultrathin endoscope (UTE) was tested in 12 patients to guide biopsies and in 6 patients to guide EHL. Results of biopsies were confirmed by surgery or the disease course in each of the 12 patients. Only one stone could not be fragmented and removed because the EHL probe could not be properly placed. The diagnosis of biliary stenosis may be markedly enhanced by using a UTE although at the risk of an endoscopic sphincterotomy and subsequent biliary infection. EHL with UTE is difficult and time consuming but further technical development may make it more practical immediately following sphincterotomy.

A choledochal cyst in the adult patient is not just an aneurysmal dilatation of the common bile duct, but it is the most obvious manifestation of a more widespread pancreaticobiliary ductal dysplasia. Most patients have abnormalities of the pancreatic ducts and, frequently, multiple areas of dilatation involving the intrahepatic bile ducts. Of our nine patients, six had pancreatograms, five of which were distinctly abnormal. Two of the five had recurrent pancreatitis. Six patients were found to have marked stenosis of the outflow tract from the cyst. Because the mucosa lining the choledochal cyst was chronically ulcerated, anastomoses to the cyst formed strictures in seven (64%) of 11 patients, and the long-term risk of carcinoma arising in the cyst was increased. The preferred therapy was excision of the cyst with Roux-en-Y hepaticojejunostomy.

The authors report a new case of biliary cancer occurring in a 23 year old asiatic girl with congenital cystic dilatation of the common bile duct. After a review of the world literature, the authors recall the principal features of this congenital disease, which is becoming more common in France with the influx of Asian refugees. They stress the risk of malignant degeneration which is related to irritation of the mucosa and biliary stasis. Ideal treatment involves complete excision of the cyst.