Choledochal cysts in adults: a report of two cases and review of the literature

A choledocal cyst is a dilation of some component of the biliary tract that may include both intra- and extra-hepatic sites. They are classified into six types, all of which are relatively rare. Previously, choledochal cysts were treated with biliary-enteric bypass procedures. The current recommendation is to attempt complete excision to minimize the known risk of malignancy and the development of recurrent cholangitis or pancreatitis that may occur in patients with these cystic lesions. Two cases are discussed in which type I choledochal cysts presented. One was removed from a 31-yr-old man who presented with vague abdominal complaints the other from a 32-yr-old man who presented with pancreatitis. The epidemiology, diagnosis, surgical treatment, and risk of cancer in choledochal cysts is described.     

Cystic dilation of extrahepatic bile ducts in adulthood: diagnosis, surgical treatment and long-term results

To evaluate the long-term results of surgery for choledohal cyst in adulthood, a series of 13 patients over the age of 16 operated on for choledochal cyst during a period of six years and followed-up for a minimum of 3 years was analyzed. Patients with type I and IVa cysts underwent extrahepatic cyst resection and Roux-en-Y hepatico-jejunostomy. Choledochoceles (type III) were managed endoscopically. No operative mortality or morbidity occurred. Type I and III cysts showed almost ideal follow-up with no sign of stricture on HIDA scan. One type IVa cyst patients developed recurrent cholangitis due to anastomotic stricture, managed percutaneously. Whenever possible, complete cyst resection and Roux-en-Y reconstruction is the treatment of choice for all extrahepatic biliary cysts. Intra- and extrahepatic dilatations are adequately treated by extrahepatic resection and careful endoscopic or radiologic surveillance. Small choledochoceles can be safely managed by endoscopic sphincterotomy.     

Malignant change in the biliary tract after excision of choledochal cyst

BACKGROUND: Choledochal cyst is a rare congenital condition with a high risk of malignant change if untreated. The risk of malignancy after surgical excision of choledochal cyst is not known. METHODS: Forty-eight patients with choledochal cysts managed over a 21-year period were reviewed, to determine the risk of malignant change after cyst excision. Thirty-nine of 48 patients had no carcinoma at first admission; their mean(s.d.) age was 20(18) years. Thirty-seven of 39 patients underwent cyst excision and cholecystectomy followed by hepaticoenterostomy. RESULTS: Cyst excision was incomplete in 28 of the 37 patients because dilated portions of the biliary ducts remained proximally and/or distally. In these 37 patients, no carcinoma has developed in the remnant proximal hepatic duct or the terminal bile duct after mean(s.d.) follow-up of 9.1(6.4) years. In the remaining nine patients, biliary carcinoma was diagnosed at the first visit. Six patients died from recurrence with a mean(s.d.) survival time of 13(11) months, while three patients were alive and free from recurrence 2 months, 1 year and 7 years after operation. CONCLUSION: Malignant change has not been observed after total or subtotal excision of choledochal cysts in this series.     

Scanning electron microscopy studies of the human lens capsule after capsulorhexis

Choledochal cysts are malformations of the biliary ductal system, which rarely occur in infancy. In neonates and infants, choledochal cysts are congenital, may be associated with distal biliary atresia and typically presents with cholestatic jaundice and acholic stools characteristic of biliary obstruction. In older children and adults, it may be acquired in association with an anomalous pancreaticobiliary union and presents with any combination of intermittent jaundice, abdominal pain, and a palpable abdominal mass. Early detection of choledochal cysts is important in the prevention of the fatal complications of biliary obstruction. Here, we present two cases of congenital choledochal cyst in which the patients were 9 weeks old and 7 weeks old respectively. Both of them presented with jaundice, acholic stools, and hepatomegaly. Combined use of sonography, computed tomography and hepatobiliary scintigraphy demonstrated the presence of choledochal cysts. Thereafter, they underwent surgical excision of the cysts with Roux-en-Y reconstruction of the biliary tree; the results were successful.     

Choledochal cyst and neoplasm: a comprehensive review of 106 cases and presentation of two original cases

Choledochal cysts are prone to complications: cholangitis, biliary cirrhosis, portal hypertension, lithiasis, rupture, pancreatitis, and carcinoma. The coincidence of choledochal cysts and neoplasia ranges from 2.5 to 26 per cent. One hundred six cases of choledochal cysts with neoplasms have been collected from the literature. We have tabulated the results of 68 cases found to have a neoplasm at the initial laparotomy and of 38 patients with pristine choledochal cysts treated electively who subsequently developed a neoplasm. Two original cases are presented. The primary site of neoplasia was not confined only to choledochal cysts. There appears to be a propensity for malignancy to develop anywhere in the biliary tract or gallbladder or pancreas in conjunction with the choledochal cyst. Accompanying choledochal cysts is a high incidence of an anomalous relation at the pancreaticobiliary junction with subsequent malignancy formation. A pathogenetic basis is postulated.     

Bile duct cysts in adults

INTRODUCTION: Bile duct cysts are rare, congenital dilations of the intrahepatic and/or extrahepatic biliary tract. Most of them present during childhood. The classical triad right upper quadrant pain, jaundice and abdominal mass is present only in a few instances. We report here the bile duct cysts which were diagnosed at our institution from 1989 to 1996. METHODS: 3245 consecutive endoscopic retrograde cholangiopancreatograms (ERCP) were evaluated retrospectively. Diagnosis was made when localized cystic dilations of the intrahepatic and/or extrahepatic biliary tract were present. Diffuse dilations of the intrahepatic and extrahepatic biliary tract were excluded. RESULTS: Bile duct cysts were found in 20 patients (17 females, 3 males) among 3245 ERCPs. Their mean age was 56 +/- 20 (median 64, range 10 to 83) years. The cyst types (according to the Alonso-Lej classification with the Todani modification) were type I in 11 (55%), type II, III and IV in two instances each (10%), and type V (or Caroli's disease) in 3 patients (15%). Leading symptoms were cholestasis in 14 patients, 10 of whom had abdominal pain, jaundice in 4 patients, and single cases of pancreatitis, cholangitis, and abdominal mass. In 2 patients the diagnosis was made incidentally. 10 patients had bile duct stones. We performed endoscopic sphincterotomy in 15 patients with concretions or persistent symptoms, 3 patients had cyst resection. One of these, with a type I cyst, already had a disseminated cholangiocarcinoma. 10 of 17 patients without cyst resection are currently symptom-free after complete removal of all gallstones. One male patient with cholecystolithiasis, who is not operable due to advanced liver disease, has recurrent cholangitis, 4 patients have died from causes unrelated to the bile duct cysts, and 2 patients are lost to follow up. CONCLUSION: Bile duct cysts in adults are rare. There is a preponderance in the female gender, and the most common type is the extrahepatic (choledochal) cyst. The leading symptoms are cholestasis and right upper quadrant pain. There is an increased risk of cholangiocarcinoma. In young patients the cysts should be entirely removed to prevent malignancy. Older persons are usually symptomless after complete removal of gallstones.     

Successful management of ruptured choledochal cyst by primary cyst excision and biliary reconstruction

One to two percent of patients who have choledochal cysts present with cyst rupture and bile peritonitis. Reported cases have been managed with external drainage of the cyst followed by a second procedure to excise the cyst and reconstruct the biliary tract. The authors report two cases of ruptured choledochal cyst treated with primary cyst excision and biliary drainage. The satisfactory outcome of these patients suggests that this is the preferred management.     

Immunoglobulin gene organization and the mechanism of repertoire development

Twenty-two cases of suprascapular nerve entrapment caused by supraglenoid cyst compression were reviewed. Pain and weakness were the presenting symptoms in 14 shoulders and pain alone in 8. Twenty of the cysts were diagnosed by magnetic resonance imaging, and two were confirmed at surgical exploration. Electromyography of 20 shoulders was positive for neurologic involvement for both the infraspinatus and supraspinatus in 4 cases, for the infraspinatus only in 12, and negative in 4. Sixteen shoulders were treated by open excision, arthroscopy, or both. Superior labral lesions were diagnosed in 11 of 12 patients who underwent arthroscopy. At follow-up 10 of the patients who underwent surgery had complete resolution of symptoms, 5 had occasional pain or weakness, and 1 recurrence required a second surgery. Of six patients treated without surgery, two improved and four had no change. Supraglenoid ganglion cysts are common and can easily be diagnosed by magnetic resonance imaging. For patients with symptoms arthroscopy with repair of the superior labral lesion and either arthroscopic debridement or direct open decompression and excision of the cyst is recommended.     

Parameatal cyst: presentation of two cases and a review of the literature

OBJECTIVE: To describe two additional cases of parameatal urethral cyst. The literature is reviewed and the etiology and treatment of this disease are discussed. METHODS: Two patients aged 20 and 24 years with parameatal urethral cyst are described. The patients were seen at the Gea González Hospital (México) during the period 1987-1988. The first case was asymptomatic and the cyst had been present since birth and in the other case, the cyst appeared at age 24 and caused irregular stream. Laboratory tests, surgical excision and pathological analysis of the cyst were performed. RESULTS: The laboratory tests were normal, no problems were encountered during surgical excision, the symptoms disappeared and there were no postoperative complications or recurrence. Pathological analysis demonstrated columnar epithelium in both cases. CONCLUSIONS: Parameatal urethral cyst is a rare benign condition that is asymptomatic in most of the cases. It may be present since birth or appear later and is prevalent in young males. Its etiology remains unclear and treatment is by complete surgical excision to avoid complications and recurrence.     

Mitchell osteotomy for adolescent hallux valgus

BACKGROUND: Complications often follow if a choledochal cyst is treated simply by drainage, either internal or external. This study reviews 17 patients who had had previous cystoenterostomy (n = 9) or external drainage (n = 8) and who required reoperation and cyst excision. METHODS: The study was a retrospective review including ten women and seven men managed over 9 years. The indications for reoperation were stone formation (10 patients), pancreatitis (three), portal hypertension (two) and hepatic abscess (one); two patients were asymptomatic. RESULTS: Definitive surgery with cyst excision was possible in all patients who had previously had external cyst drainage and in seven of nine who had had previous cystoenterostomy. There were no deaths. Two postoperative biliary leaks and two duodenal fistulas resolved spontaneously. CONCLUSION: Excision of a choledochal cyst is possible and desirable even after a previous drainage operation. In severely ill patients with a complication of choledochal cyst, external drainage may be a preferable initial manoeuvre.     

Biliary atresia and biliary cysts

The authors present a review of the classification, aetiology, presentation, treatment and long-term outcome of children and adults with biliary atresia and choledochal cyst disease. Biliary atresia should be suspected in any infant with jaundice beyond the second week of life. Although the aetiology and pathogenesis remain unclear, early management with portoenterostomy has significantly improved the course of this disease. Recent advances in immunosuppression have made liver transplantation a valuable and necessary adjunct to biliary bypass. With choledochal cyst disease, adults, unlike children, often present with acute biliary tract symptoms or pancreatitis. The treatment of choice remains extrahepatic cyst excision and biliary bypass. This treatment has excellent long-term results that minimize the development of malignancy.     

Evaluation of axis alignment system for correction of myopic astigmatism with the excimer laser

BACKGROUND: Anomalous pancreaticobiliary junction (APBJ) without congenital choledochal cyst (CCC) carries a high risk of gallbladder carcinoma development. The aim of this study was to obtain information allowing early diagnosis and appropriate management. METHODS: The clinical features, imaging findings and surgical outcome of 18 patients with APBJ without CCC were analysed retrospectively. RESULTS: Fourteen patients had symptoms, including those of acute pancreatitis (five patients). In 16 patients the gallbladder showed abnormalities, including carcinoma (eight) and mucosal hyperplasia (11). Ultrasonography detected gallbladder carcinoma with 100 per cent sensitivity and mucosal hyperplasia with 91 per cent sensitivity. A long common channel was demonstrated by endoscopic retrograde cholangiopancreatography (ERCP) in all patients, endoscopic ultrasonography in nine of ten, and magnetic resonance cholangiopancreatography (MRCP) in five of five. Five of eight patients with gallbladder carcinoma underwent extended cholecystectomy with bile duct excision. Three patients with cancer and eight with no cancer had cholecystectomy alone. None developed bile duct carcinoma or acute pancreatitis after operation. All patients without malignancy remained asymptomatic for a mean follow-up period of 4.7 years. CONCLUSION: Prophylactic cholecystectomy is recommended for patients with APBJ without CCC. For early diagnosis of APBJ, gallbladder abnormalities on ultrasonography or acute pancreatitis of unknown aetiology should prompt further investigation with ERCP or less invasive imaging modalities such as endoscopic ultrasonography and MRCP.     

Ultrasonography of cystic thyroid nodules: sonographic-pathologic correlation

Retrospectively the ultrasonographic findings of 153 surgically resected cystic thyroid nodules were reviewed. The pathologic findings in this series revealed that 86% were degenerating benign adenomas or adenomatous goiters, and 14% were malignant tumors. The sonographic appearance of these lesions was classified into 7 groups as follows: type I: entirely cystic (less than 1cm), type II: cystic(more than 1cm) [II(a)], and with small polyp or dome-like elevation on the cyst wall [II(b)], type III: larger cyst with projection (more than 1cm) into the lumen, type IV: cyst with a peripherally localized solid component, type V: irregularly mixed cystic and solid components, type VI: a solid mass with multiple crescentic cysts [VI(a)], or round cysts [VI(b)], type VII: a solid mass with only one or two cysts. Pathologic correlation revealed that malignancy in this series ranged from 80% in type III and V to only 4% in type II, where most of the lesions in this group were composed of granulation tissue in degenerating adenomatous polyps and cyst walls. Lesions in type IV showed malignancy rate of 40%. Type III showed characteristic sonographic findings seen in cystic papillary carcinomas (CPCs), with multiple punctate echogenic foci in large pedunculated projections. The typical psammomatous calcifications specific in this group were confirmed in 6 of the 8 type III CPCs. The multiple crescentic cysts in type VI(a) lesions were characteristic sonographic signs seen in adenomatous goiters, representing the pathologic finding of cysts forming around each of multiple adenomatous nodules in this group. Type VII represented non specific appearing lesions, included adenomas, adenomatous goiters, CPCs and follicular carcinomas.(ABSTRACT TRUNCATED AT 250 WORDS)     

Radiation and concurrent carboplatin administration in locally advanced head and neck cancer. A Hellenic Cooperative Oncology Group Study

We report our first experience with a laparoscopic treatment of congenital choledochal cysts involving the total cyst resection and the reconstruction of the biliary and gastrointestinal tracts through a transmesocolic hepatic-jejunal Roux-en-Y loop anastomosis. The procedure was carried out in a 14-kg 6-year-old girl with a congenital choledochal cyst of the first type, according to the Alonso-Lej classification. The cyst was divided using a Multifire EndoGIA 30 stapler. Hepatic-jejunal and jejunojejunal anastomoses were made with 4.0 chrome catgut interrupted sutures. Intestinal recanalization occurred on the 2nd postoperative day and the postoperative course was uneventful. The laparoscopic approach affords several advantages: excellent intraoperative visualization of tiny structures and, therefore, great surgical accuracy; early resumption of peristalsis; no postoperative pain; no laparocele; prevention of adhesions; excellent esthetics; and quicker resumption of school and sports activities.     

Ultrasound in the management of acute pancreatitis

Thirty-eight patients have been examined by ultrasound when symptoms or sign suggested the development of a pseudocyst following an attack of proven acute pancreatitis. Pseudocyst was diagnosed in 23 of the 38 cases. Five patients had multilocular cysts, four of which were shown to be communicating. Laparotomy was carried out on 14 of the 23 patients and surgical drainage was performed in 12 cases. The remaining nine cases were monitored and showed steady regression. Small cysts arising in the head of pancreas may give rise to recurrent or persistent pancreatitis and may be demonstrated pre-operatively by ultrasound but not readily by other means. A further 12 patients showed an area of irregular absorption of ultrasound interpreted as an inflammatory mass. Monitoring of these cases showed progressive resolution without cyst formation. Three of these cases subsequently required laparotomy-one developed an abscess and one necrosis of the body and tail of pancreas while a third developed severe pancreatic fibrosis of the area identified by ultrasound. The ability to distinguish between pseudocysts and inflammatory masses and to demonstrate communication between multiple cysts is of considerable value in pre-operative diagnosis.     

Presentation and surgical management of bronchogenic and esophageal duplication cysts in adults

OBJECTIVE: Bronchogenic and esophageal duplication cysts are congenital anomalies of the tracheobronchial tree and foregut that are often asymptomatic at initial presentation in adults. Surgery is always recommended, even for patients with asymptomatic disease, because of the possible development of symptoms and complications during the natural course of the disease and because definitive diagnosis can be established only on surgical specimen. METHODS: Twenty-seven patients with bronchogenic and esophageal duplication cysts were treated in our institution over the last 2 decades. Ten patients (37%) were asymptomatic at initial presentation. Chest pain and dysphagia were the most common complaints in symptomatic patients affected by bronchogenic and duplication cysts, respectively. RESULTS: A complete excision of the cyst was performed in 26 cases, whereas one patient with intrapulmonary cyst underwent a right upper pulmonary lobectomy. A posterolateral thoracotomy was performed in 23 patients, and a video-assisted thoracoscopy using a three-port technique was performed in the last 4 patients. No postoperative morbidity was recorded. All patients, except one, were asymptomatic at a median follow-up time of 4 years. CONCLUSIONS: Surgery is the treatment of choice for bronchogenic and esophageal duplication cysts. Video-assisted thoracoscopy should represent the first-line approach in these patients.     

Value of MR cholangiopancreatography in evaluating choledochal cysts

OBJECTIVE: The aim of this retrospective study was to clarify whether MR cholangiopancreatography (MRCP) is a suitable replacement for ERCP in evaluation of the choledochal cyst. MATERIALS AND METHODS: Sixteen patients (six adult and 10 pediatric) with choledochal cysts underwent MRCP using a half-Fourier acquisition single-shot turbo spin-echo sequence. Extent of the cyst, defects within the biliary tree, and presence or absence of the anomalous junction of the pancreaticobiliary duct were evaluated. Findings were compared with those of ERCP. RESULTS: MRCP better defined the proximal biliary tree than did ERCP in two patients. Defects within the biliary tree were diagnosed correctly on MRCP in eight patients; however, two defects within the distal common bile duct were missed in pediatric patients. The presence of the anomalous junction of the pancreaticobiliary duct was revealed accurately by MRCP in all adult patients but was revealed accurately in only four of the 10 pediatric patients. CONCLUSION: MRCP appears to offer diagnostic information that is equivalent to that of ERCP for assessment of choledochal cysts in adults. In pediatric patients, MRCP should not replace ERCP; however, MRCP can play an important role as a noninvasive examination and should be considered a first-choice imaging technique for evaluation of choledochal cysts.     

Prenatal diagnosis of biliary atresia associated with choledochal cyst

We report a case of biliary atresia promptly detected by its association with a choledochal cyst, prenatally diagnosed at 20 weeks' gestation. The baby was operated at 14 days of life. A choledochal cyst was found and fibrosis of the extrahepatic bile ducts was also noted. So, excision of the choledochal cyst and a Roux-en-Y porto-jejunostomy was done. Nine months later, despite an appropriate biliary drainage, echographic and histological changes compatible with liver cirrhosis have been detected. Including this one, six cases have been reported.     

Orbital lobe lacrimal ductal cyst

Lacrimal ductal cysts are rare, especially in the orbit. A case of lacrimal ductal cyst in the orbital lobe, revealed by computed tomography and magnetic resonance imaging, is described in a 16-year-old man who was successfully treated by complete surgical excision. The radiologic and clinical features of this type of lacrimal ductal cyst are discussed.     

Is it possible to differentiate between choledochal cyst and congenital biliary atresia (type I cyst) by antenatal ultrasonography?

Both choledochal cyst and congenital biliary atresia (type I cyst) may share the same ultrasonographic pattern. We report 2 cases which were shown to have cystic structures on the upper abdomen by antenatal ultrasonography. The size of the choledochal cyst found at 24 weeks' gestation increased steadily as gestational age advanced. But the size of the cyst in congenital biliary atresia found at 29 weeks' gestation remained unchanged throughout the remaining pregnancy. Cyst enlargement, therefore, may suggest the possibility of a choledochal cyst.