• Advances in pediatric surgery
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• v.18 no.1
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• pp.1-11
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• 2012

담관 낭종은 흔하지는 않지만, 외과적 절제가 필요한 주요 질환이다. 절제하지 않고 남겨두면 다른 질환으로의 이환이나 다양한 합병증에 의한 사망까지도 야기할 수 있기 때문에 적절한 수술적 치료가 필수적이다. 최근 수술에 따른 다양한 문제들이 계속 보고되고 있으며, 출생 전이나 건강 검진 시 발견되는 무증상의 담관 낭종의 수술 시기에 대해서도 아직 논란이 되고 있다. 저자들은 1995년부터 2009년까지 담관 낭종으로 수술 받은 환자 중 수술 받을 당시의 연령이 18세 이하인 32명 환자의 임상 양상과 수술 기록을 후향적으로 분석하여, 수술 성적에 영향을 주는 요인들을 알아보고자 하였다. 전체 32명 중 남자 10명, 여자 22명이었으며, 수술 당시의 평균 연령은 5.4세였다. 췌담관 합류이상은 9명(28.1%)이었으며, 평균 추적 관찰 기간은 34.6개월이었다. 술 전 증상을 호소한 환자는 30명(93.8%)이었으며, 복통(63.3%), 황달(40.0%), 구토(23.3%), 복부종물(16.7%), 발열(16.7%)등의 순서였다. 술 전 혈액 검사에서 AST/ALT의 상승이 18명(56.3%), 고빌리루빈혈증이 8명, 백혈구 증가증이 7명(21.9%)이었다. 술 후 합병증은 5명(15.6%)에서 나타났는데, 2명은 담관염이었고, 나머지 3명은 문합부 협착, 술 후 출혈, 장 폐쇄가 각각 1명 있었다. 술 후 평균 재원 기간은 12.2일 이었으며, 추적 관찰 기간 중 3명(9.0%)이 재입원 하였다. 수술 성적에 영향을 미치는 요인으로, 수술 당시의 나이가 많을수록 평균 수술 시간과 재원 기간이 증가하였다(p=0.004, p=0.028). 증상이 있었던 환자군에서 무증상 환자군보다 재원 기간이 더 길었다(p=0.001). 또한 췌담관 합류이상이 있었던 환자군에서 재입원율이 유의하게 높았다(p=0.005). 소아의 담관 낭종은 진단이 되면 증상 발현 전이라도 조기에 수술하는 것이 수술 시간 및 재원 기간을 단축시킬 수 있으며, 췌담관 합류이상이 동반된 경우에는 술 후에 재입원할 가능성이 높으므로 주의깊게 추적 관찰하는 것이 바람직할 것이다.

• Journal of Yeungnam Medical Science
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• v.2 no.1
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• pp.299-306
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• 1985

Congenital dilatation of the common bile duct is relatively rare anomaly. Its pathogenesis has not been completely understood. Complications of the choledochal cyst are mainly suppurative cholangitis, liver cirrhosis, stone formation, malignant change, bile peritonitis due to spontaneous and traumatic rupture. We experienced one case of choledochal cyst associated with hemorrhagic tendency and a cerebral hematoma, which is extremely rare complication. The 3 monthes old male patient reported here was treated with complete excision of cyst and Roux-en-Y choledochojejunostomy after correction of bleeding tendency and removal of crebral hematoma. Postoperative course was relatively uneventful, 11 days after operation, the patient was discharged with full improvement.

• Advances in pediatric surgery
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• v.9 no.1
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• pp.45-51
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• 2003

A nationwide survey on choledochal cyst was undertaken among 39 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and the case registration form for each patient during the five year period of 1997 to 2001. Three hundred and forty eight patients were registered from 32 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1:3.4. The age of patients on diagnosis was $49.0{\pm}44.4$ months. The geographic distribution was 34.8% in Seoul and Kyoungki-do, 33.3% in Kyoungsang-do, 17.9% in Cholla-do, and 8.5% in Choongchung-do, in order of frequency. The three common clinical presentations were abdominal pain (63.8%), vomiting (35.3%), and jaundice (29.1%). Only seven patients (2%) presented with classic triad, and 25 patients were diagnosed by antenatal ultrasonographic examination. According to the Todani Classification, 238 patients (7l.3%) were type 1, 3 (0.9%) type 11, and 93 (27.8%) type IV. At the time of the operation, three important associated conditions were choledocholithiasis in 45 patients (15.1%), liver fibrosis (Grade 1-4) in 35, and previous operative procedure for biliary diseases in 10. Associated anomalies were observed in 13 patients (3.8%). Three hundred thirty nine (98.8%) of 343 lesions were treated by Cyst excision and Roux-Y hepaticoiejunostomy. One hundred seventy-six patients had an anomalous arrangement of the pancreatobiliary ductal system (APBD): APBD was not in 92 patients, biliary duct joined to the pancreatic duct in 51, and pancreatic duct joined to the biliary duct in 26. There were 8.5% early, and 7.7% late phase operative complications. The major complications were bleeding, anastomotic leakage, and acute pancreatitis. The combination of acute abdomen and choledochal cyst may suggest spontaneous rupture. Because of the development of late intrahepatic bile duct stones, long term follow up after cyst excision and hepaticojejunostomy is required. The optimal time of surgical intervention should also be considered in the situation of routine use of antenatal ultrasonographic examination. This is the first review of the choledochal cyst in Korea and provides baseline data for future comparisons.

• Advances in pediatric surgery
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• v.5 no.2
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• pp.126-129
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• 1999

A 6 and a half year-old girl developed recurrent cholangitis following hepatic portoenterostomy for biliary atresia. Computed tomogram showed an ovoid cyst ($4.5{\times}4.0$ cm in size) in the left hepatic lobe and another tubular dilatation ($2.0{\times}0.8$ cm in size) in the right hepatic lobe. Percutaneous transhepatic cholangiodrainage(PTCD) and cystogram showed an ovoid cyst in the left hepatic lobe (Tsuchida type A), measuring $6.6{\times}5.0$ cm in size. Following drainage and administration of parenteral antibiotics she became afebrile and anicteric. However she continued to drain 45-150 cc of bile per day via the tube over the next 2 weeks. The patient successfully underwent intrahepatic cystojejunostomy with intraoperative ultrasonographic guidance. This case illustrates relapsing cholangitis caused by Tsuchida type A intrahepatic cyst, successfully managed with PTCD followed by an internal drainage procedure.

• Advances in pediatric surgery
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• v.14 no.2
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• pp.178-182
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• 2008

Forme fruste choledochal cyst (FFCC) is one of the diverse types of choledochal cyst with little or no dilatation of the extrahepatic bile duct (EHBD). It is considered that FFCC has to do with the pancreatobiliary malunion (PBM). In children, 3 to 6 millimeters of EHBD is assumed to be normal. Even though there is no clear-cut definition, FFCC is likely to be associated with bile duct dilatation less than 10 millimeters. Almost all cases have PBMs and symptoms of the pancreatitis or cholangitis. We experienced a case of FFCC in a 4-year-old boy. His EHBD measures 10 mm diameter. He had symptoms of pancreatitis and elevated hepatic transaminases. The pancreatobiliary common channel was 28 millimeters. He underwent EHBD resection and Roux-en-Y hepaticojejunostomy and was discharged with no specific complications.

• Clinical and Experimental Pediatrics
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• v.45 no.11
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• pp.1381-1388
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• 2002

Purpose : Magnetic resonance cholangiopancreatography(MRCP) is a noninvasive method for imaging the pancreaticobiliary tree. The aim of this study was to evalute the usefulness of MRCP for the diagnosis of pancreaticobiliary diseases in children. Methods : From October 1996 to May 2001, 67 patients with obstructive jaundice and three patients with chronic recurrent pancreatitis were evaluated with abdominal ultrasonography and MRCP. The final diagnosis was based on the operative and pathologic findings with biopsy specimen including clinical and laboratory findings. Results : A total of 70 patients, consisting of 31 males and 39 females, with a mean age of $2.6{\pm}3.3$ years were studied. The final diagnosis was biliary atresia in 25, neonatal cholestasis in 18, choledochal cyst without anomalous pancreatobiliary duct union(APBDU) in nine, choledochal cyst with APBDU in seven, cholestatic hepatitis in five, chronic recurrent pancreatitis in three, sclerosing cholangitis in two, and secondary biliary cirrhosis in one case. The overall diagnostic accuracy of abdominal ultrasonography was 75.7% and that of MRCP was 97.1%. The sensitivity and specificity of MRCP were 100% and 98% for biliary atresia, 87.5% and 100% for choledochal cyst with APBDU, 100% and 100% for choledochal cyst without APBDU, sclerosing cholangitis and chronic recurrent pancreatitis, respectively. Conclusion : MRCP is a fast, non-invasive and reliable method for diagnosing pancreaticobiliary diseases in children and will be the standard diagnostic procedure in the future.

• Advances in pediatric surgery
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• v.5 no.2
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• pp.121-125
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• 1999

To study the role of anomalous pancreatico-biliary ductal union (APBDU) in the development of choledochal cyst, we reviewed 23 cases. APBDU is defined as a long common channel(>0.4 mm). The patients ages ranged from 1 week to 112 months and the mean age was 22.5 months. Right upper quadrant pain was the most prevalent symptom. The diagnosis was made by ultrasonography and operative cholangiography in most patient. The preoperative diagnosis was made in 100 % of the cases. Gallstones were found in 5 cases. Todani type I and type IV were prevalent. A long common channel was found in all cases. The operative treatment consisted of cyst excision and Reux-en-Y hepaticojejunostomy or choledochojejunosotmy. One patient had postoperative pancreatitis. There was no mortality. We conclude that detection of choledochal cyst is occurring at a younger age and APBDU seems to play an important role in the pathogenesis of type I and IV cysts. Cyst excision is the treatment of choice to eliminate repeated cholangitis and malignant transformation.

• Advances in pediatric surgery
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• v.17 no.1
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• pp.72-80
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• 2011

Although laparoscopic surgery for hepatobiliary disease in children is possible, it is technically challenging. In an attempt to overcome these difficulties, the da Vinci Robotic Surgical System$^{(R)}$ was used to facilitate the minimally invasive treatment of choledochal Cyst in six children. In early consecutive three cases, we experienced three complications; a case of laparotomy conversion, a case of late stenosis of the hepaticojejunostomy, and a case of leakage from a hepaticojejunostomy. However, in the last three cases the complete resection of the choledochal cyst and Roux-en-Y hepaticojejunostomy were performed using the robotic surgical system without complication. We think robot-assisted choledochal cyst resection in children appears safe and feasible, and may increase the variety of complex procedures in pediatric surgical fields.

• Advances in pediatric surgery
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• v.11 no.2
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• pp.186-191
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• 2005

A 10-year-old-girl was referred to our hospital due to abdominal pain. She underwent Roux-en-Y cystojejunostomy for a choledochal cyst at another hospital at the age of 3 months. Abdominal ultrasonography (USG) and computed tomography (CT) showed type I choledochal cyst and multiple gallbladder stones. Because of severe inflammation and adhesion, partial resection of the choledochal cyst and Roux-en-Y hepaticojejunostomy was performed. Two and one half years later, intermittent abdominal pain, fever, nausea and vomiting occured. Abdominal CT scan showed a polypoid nodular lesion in the remnant of the choledochal cyst and probable metastasis at segment 7 of the liver. The duodenum was obstructed by the mass. Liver biopsy revealed moderately differentiated adenocarcinoma. A palliative gastrojejunostomy was performed to relieve duodenal obstruction. She died of hepatic insufficiency 4 months later.

• Investigative Magnetic Resonance Imaging
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• v.6 no.2
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• pp.129-136
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• 2002

Purpose : To evaluate the usefulness of MRCP in the diagnosis of the variable causes of common bile duct(CBD) dilatation, except stone or tumor Materials and methods : Twenty-six patients(M:F=15:11, mean age; 62 years) with both MRCP and ERCP were included in this study. Dynamic MRCP(n=12) and contrast-enhanced MRI(n=10) of abdomen were also added. Dilatation of CBD, intrahepatic ducts and pancreatic duct was evaluated, including coexistence of intrahepatic ductal stone, pancreatic pseudocyst, and papillary or papillary edema. The criteria of CBD dilatation was over than 7mm(n= 21, without cholecystectomy) or 10 mm(n=5, with cholecystecto-my) in diameter on T2-weighted coronal image. Results : The mean diameter of CBD was 12.7mm without cholecystectomy(9-19 mm) and 13.0 mm with cholecystectomy(10-15mm), respectively(p 〉0.05). Cholangitis(n=11, 42.3%), chronic pancreatitis(n=8, 30.8%), stenosis of distal CBD(n= 6, 23.1%), periampullary diverticulum(n=3, 11.5%), stenosis of ampulla of Vater(n=2, 7.7%), dysfunction of sphincter of Oddi(n=2, 7.7%), acute focal pancreatitis in the pancreatic head(n=2, 7.7%), papillitis(n=1, 3.8%), pseudocyst in the pancre atic head(n = 1, 3.8%), and ascaris in CBD(n=1, 3.8%) were noted. Pancreatic duct dilatation(n=10, 38.5%) and duodenal diverticulum(n=3, 11.5%) were also seen on MRC P. On dynamic MRCP(12 patients), distal CBD was visualized in 2 patients(16.7%), which was not shown on routine MRCP. Only 1 patient(10.0%) showed papillitis with slightly enhancing papilla on contrast-enhanced MRI (10 patients). Conclusion : MRCP was thought to be helpful in the evaluation of the causes of CBD dilatation, not caused by stone or tumor, especially in the cases of stenosis of distal CBD and chronic pancreatitis, dysfunction of sphincter of Oddi on dynamic MRCP and cholangitis and pericholangitic abnormality on contrast-enhanced MRI.