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PubMedDate not supplied

[Surgical revision and correction of choledochal cysts].

Choledochal cyst are a rare congenital abnormality, seldom treated by the General Surgeon. We report six cases of re-operation found on a eighth year period. Six cysts type I, and one type II (Todani's Classification) were found. On five of them, an internal derivation had been performed, in one patient only a celiotomy was performed, and in another one a colecistectomy. Complete resection of the cyst was performed in six cases. In one female patient, this was impossible, because a carcinoma was found, she died postoperatively. We recommend external bile duct drainage for those cases where a complete resection can not be performed. This option alleviates the symptoms, allows morphological studies and do not compromise the final procedure which must be the complete excision of the cyst.

Adolescent↗

PubMed1989

Radiologic approach to choledochal cysts.

Choledochal cysts, being uncommon and having a nonspecific presentation, require a high index of suspicion for their diagnosis. In this series of 11 patients, the correct diagnosis was established in all using a combination of real-time ultrasound (US) scanning (9 positive) and endoscopic retrograde cholangiopancreatography (ERCP; 10 positive). Real-time US is suitable as the initial imaging modality and ERCP is complementary.

Adolescent↗

PubMed1989

[Congenital hepatic fibrosis associated with choledochal cyst].

The purpose of this work is to report the case of a one-year and eleven-month-old female child who presented with an asymptomatic hepatic mass; on x-ray examination a well limited round tumor was seen as well as renal cysts. A choledochal cyst was found upon laparotomy; the liver biopsy exhibited congenital hepatic fibrosis. This association is not clearly defined in the literature review made, making this perhaps the first well-documented case with this combination. We analyze the pathogenic mechanisms of both disorders and conclude that choledochal cyst is probably not related to hepatorenal fibropolycystic disease; this could be a casual association in our patient.

Choledochal Cyst↗

PubMed1991

Occult carcinoma in an adult choledochal cyst.

The complications of choledochal cyst are avoidable if diagnosed early, and adequate resection undertaken. This case report describes the long history of right subcostal pain in a young man of 26 who had a squamous carcinoma in a choledochal cyst diagnosed after serial section of the excised cyst. Subsequent resection of the head of the pancreas showed histological residual tumour from which he died 4 months later. This case illustrates the need for complete early excision of a choledochal cyst to prevent this complication.

Adenocarcinoma↗

PubMed1990

Complications of choledochal cysts in adulthood.

Choledochal cyst is a well-recognised entity, presenting primarily in infants and young children. Where symptoms are delayed until adulthood, associated hepatobiliary pathology may complicate the presentation. These problems may be aggravated by previous treatment with bypass surgery rather than resection. We report seven cases from our recent experience presenting with complications in adulthood. These included cholangitis, hepatic abscess, pancreatitis and malignancy within the cyst. Two patients presented during pregnancy. These complications and their implications for management are discussed.

Adenoma, Bile Duct↗

PubMed1977

Operative procedures for choledochal cyst.

Fifty one operations for choledochal cyst were carried out upon 46 patients: 41 as a primary operation and 10 as reoperation. An excision of choledochal cyst with hepaticojejunostomy in Roux-en-Y fashion at the second laparotomy gave a favorable results in all patients who suffered recurrent symptoms after the previous anastomotic procedure. Comparing operation time, blood loss and operative results of the reoperations with those of the primary, it was concluded that all choledochal cyst should be excised at the first operation unless other reason avoiding excision exists. Hepatic portojejunostomy is recommended for choledochal cyst with an associated distal atresia and the hypoplastic hepatic duct.

Adolescent↗

PubMed1989

Biliary malignancies occurring in choledochal cysts.

Eight cases of choledochal cyst associated with biliary malignancy (gallbladder carcinoma in three and bile duct carcinoma in five) were reviewed to evaluate the roles and limitations of computed tomography (CT) (n = 8), ultrasound (US) (n = 6), cholangiography (n = 8), and angiography (n = 6). In cases of gallbladder carcinoma, both CT and US revealed mass lesions in the gallbladder consistent with cancer. CT also depicted either a mass lesion or an irregular thickened wall in all cases of bile duct carcinoma; however, US failed to demonstrate a thickened wall in one of three cases in which CT findings were positive. Cholangiography universally revealed malunion of the pancreatico-biliary duct, and the findings were suggestive of malignancy in seven cases in which CT depicted abnormalities. Angiography showed tumor stain in three of six cases. Lymph node metastases were present in four cases but were detected preoperatively in only one. One case showing a thickened bile duct wall was erroneously diagnosed as malignancy among 27 cases of choledochal cyst unassociated with biliary malignancy.

Adult↗

PubMed1991

[Anomaly of the pancreatico-biliary junction and etiology of choledochal cysts].

The etiology of choledochal cysts isn't yet clarified unequivocably. Numerous theories have been worked out. One of them is the 1969 by Babbitt postulated "common-channel"-theory, which is based on an anomaly of the pancreaticobiliary connection. In case of a fusion of ductus choledochus and ductus pancreaticus widely before the papilla of Vater and the formation of a common channel with a minimum length of 15 mm the reflux of pancreatic secretion into the off-leading biliary ducts may occur and choledochal cysts may develop. In order to check up this hypothesis we evaluated retrospectively pictures of a direct cholangiography (ERCP, PTC and/or intraoperative cholangiography) of 26 patients suffering from type I, IV and V ectasias of the biliary ducts according to Todani. We found a common channel with an abnormal length in 8 of 12 patients suffering from extrahepatic ectasias of the biliary duct type I and IV (66%), but we didn't find it in patients with type V intrahepatic ectasias of the biliary duct. This analysis may be recognized as a reference to the truth of the Babbitt-theory.

Cholangiography↗

PubMed1988

Choledochal cysts in the adult.

Although choledochal cyst is generally considered a disorder of childhood, in approximately 20% of patients a cyst is first recognized in adult life. We reviewed nine adults who underwent operation for choledochal cyst from 1974-1985 at Yale New Haven Hospital. Presenting symptoms included: crampy right upper quadrant pain, jaundice, pruritus, cholangitis, pancreatitis, hepatomegaly, and elevated liver function tests. Seven patients had undergone previous biliary surgery. The eight patients with type I cysts underwent complete excision of the cysts with Roux-Y choledochojejunostomy. One patient with a type IVa cyst underwent Roux-Y cystjejunostomy. All patients had complete resolution of biliary symptoms. There were no deaths, serious complications, or development of biliary malignancy in the 6 months to 13 years of follow-up. Optimum treatment of choledochal cyst is complete excision with reconstruction by Roux-Y choledochojejunostomy.

Adolescent↗

PubMed1988

The role of endoscopic retrograde cholangiopancreatography in the diagnosis and treatment of adult choledochal cyst.

Five patients with adult choledochal cyst were diagnosed by endoscopic retrograde cholangiopancreatography (ERCP). Four patients had type I and one patient had type III choledochal cyst with abnormal pancreaticobiliary duct anatomy. One patient had a tubular adenoma of the distal common bile duct. All patients were treated surgically (excision of cyst with biliary and enteric reconstruction in two patients; cystojejunostomy, two, and transduodenal sphincterotomy, one patient). The follow-up period ranged from two to nine years, with a mean of four years. One patient had recurrent cholangitis develop and was treated successfully by nasobiliary tube flushing. ERCP should be used when choledochal cyst is suspected by ultrasonography, aiming at demonstrating both biliary and pancreatic duct anatomy and enabling the planning of the surgical procedure. Nasobiliary tube flushing provides an alternative therapeutic modality in patients in whom surgical treatment is contraindicated.

Adolescent↗

PubMed1978

Surgical treatment of choledochal cyst.

A case of multiloculated choledochal cyst occurring in an 18-year-old girl is presented. The choledochus which contained multiple pigment stones was excised as the primary treatment and the patient remains well on two year follow-up. Ten additional cases of choledochal cyst treated in the past 20 years have been reviewed. It is concluded that excision of the choledochus is a safe and acceptable treatment of choledochal cyst in young low risk patients and that the alternative treatment of Roux-en-Y choledochocyst-jejunostomy gives satisfactory results.

Adolescent↗

PubMed1989

Hepatico-jejuno-duodenostomy reconstruction following excision of choledochal cysts in children.

Ten children with choledochal cysts were treated at Children's Memorial Hospital in Chicago between 1979 and 1980 and at Henrietta Egleston Hospital for Children or Grady Memorial Hospital in Atlanta between 1980 and 1987. Age at the time of operation ranged from one to 46 months. There were eight girls and two boys with Type I choledochal cysts. One child with a dilated but histologically normal common bile duct underwent a choledochoduodenostomy. The remaining nine patients had excision of the choledochal cyst. Continuity with the GI tract was then re-established with a valved isolated jejunal segment interposed between the common hepatic duct and the duodenum. The valve serves to prevent reflux of enteric contents up into the biliary tract. The short jejunal segment allows bile to drain into the duodenum with minimal stasis; this more closely approximates the natural physiologic state than does a Roux-en-Y reconstruction. There was no mortality, with follow up ranging from six months to over eight years. Two post-operative complications (one anastomotic stricture and one small bowel obstruction) occurred, both in the same patient. There were no anastomotic leaks and no episodes of post-operative cholangitis. All patients are currently free of jaundice and none have signs of portal hypertension. The treatment of choledochal cysts is excision. Biliary tract reconstruction with the valved common hepatico-jejuno-duodenostomy is safe, effective, and physiologically appealing.

Child, Preschool↗

PubMed1979

The surgical treatment of choledochal cyst.

Eleven consecutive infants and children with choledochal cyst were evaluated for operative resection of the cyst as the primary surgical therapy. Total excision and biliary reconstruction by choledochojejunostomy was successfully performed in nine patients. Simple intestinal drainage of the cyst was used in two instances because of severe liver disease. Four patients had coexisting biliary malformations. In all five infants, biopsy of the liver was consistent with biliary atresia. In addition, the extrahepatic bile ducts proximal to the cyst were obliterated in two infants. The findings suggest that pathogenesis of choledochal cyst may be different in infants than in older children. The indications for excision of the cyst in four patients were due to complications from earlier internal drainage procedures. In most patients, including all those having had a cystoenterostomy earlier, resection was done from the inside of the cyst, thus minimizing the danger of injury to the neighboring vascular structures. There were no operative deaths, and morbidity was minor. The traditional operation for a choledochal cyst has been internal drainage of the cyst into the intestine. Because of the high incidence of late complications and the frequent association of major co-existing biliary malformations, the procedure should be reserved for highly specific indications. Total surgical excision is the procedure of choice for a choledochal cyst.

Adolescent↗

PubMed1989

Ultrasonography and 99mTc-DISIDA cholescintigraphy in the diagnosis of choledochal cyst in children.

Eighteen children with choledochal cyst have underwent abdominal ultrasonography and 99mTc-DISIDA (di-isopropylphenylcarbamoyl-methylimidodiacetic acid) cholescintigraphy before surgery. All patients had typical sonographic findings of a large cystic mass in the porta hepatis with direct continuity to the biliary system. The gallbladder could be easily recognized and distinguished from the cystic mass in every except one case. Dilatation of the intrahepatic ducts was noted in 12 patients (67%). Coexisting biliary stones were found in 3 patients (17%). On the cholecintigrams, 14 patients (78%) had characteristic findings of a round or ovoid photon-deficient area in the porta hepatis with delayed filling and stasis of the tracer in the same area. Three infants (17%) showed a persistent round photon-defect over the porta hepatis without visualization of gallbladder or bowel radioactivity, which was thought to suggest a choledochal cyst with obstruction. A combination of ultrasonography and cholescintigraphy can provide a very accurate preoperative diagnosis of choledochal cyst.

Bile Ducts↗

PubMed1988

Adenocarcinoma in a choledochal cyst during pregnancy: a case report and guidelines for management.

Choledochal cysts, although more common in females than males, have only rarely been encountered in association with pregnancy. We report a patient with a large choledochal cyst that was excised at the time of cesarean section. Pathologically, the cyst wall was found to contain a focus of adenocarcinoma. The development of carcinoma is a recognized risk in patients with choledochal cysts that mandates cyst excision, rather than bypass, whenever possible. The literature regarding choledochal cysts in pregnancy is reviewed. Pregnant patients with choledochal cysts require careful management so as to avoid cyst rupture; and cesarean section is preferable to normal vaginal delivery in most cases. Although the preferred management of a choledochal cyst is excision and Roux-en-Y reconstruction, this may have to be deferred until after delivery, depending on gestation age, because of the risk of fetal mortality and maternal morbidity that is associated with this procedure.

Adenocarcinoma↗

PubMed1989

Etiological relationships between choledochal cyst and anomalous junction of the pancreaticobiliary ductal system.

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.

Biliary Fistula↗

PubMed1979

Portal hypertension secondary to choledochal cyst.

Two patients with portal hypertension secondary to choledochal cyst are reported and combined with four others previously reported in the literature. Choledochal cysts may, by extrensic compression, cause partial or complete portal vein obstruction, portal hypertension and esophageal varices. Needle biopsy of the liver is probably not a reliable means of differentiating choledochal cyst from other intrahepatic causes of portal hypertension. Internal drainage of the choledochal cyst has been performed in four patients and in each instance resulted in satisfactory portal decompression.

Adolescent↗

PubMed1990

Increased secondary bile acids in a choledochal cyst. Possible role in biliary metaplasia and carcinoma.

Choledochal cysts are uncommon congenital or acquired lesions of the biliary tree. The incidence of biliary tract carcinoma in patients with choledochal cysts is 5-35 times greater than that of the general population. Factors responsible for the increased risk of carcinoma are unknown. The case of a young woman who underwent excision of a choledochal cyst 16 years after initial diagnosis and treatment by choledochocystduodenostomy is reported. Metaplasia of the epithelial lining of the cyst was found in the resected specimen. The relative composition of bile acids in cyst contents was as follows: lithocholate, 2%; deoxycholate, 88%; chenodeoxycholate, 5%; and cholate, 5%. Virtually all bile acids were recovered in unconjugated form. In contrast, the bile acid composition of hepatic bile was as follows: lithocholate, 0%; deoxycholate, 34%; chenodeoxycholate, 43%; and cholate, 23%. Bile acids were fully conjugated. These data suggest that stasis of bile within choledochal cysts contributes to bacterial overgrowth and generation of unconjugated secondary bile acids.

Adenoma, Bile Duct↗