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T-tube cholangiogram showing pancreatic duct, intrahepatic bile ducts, and duodenum 

T-tube cholangiogram showing pancreatic duct, intrahepatic bile ducts, and duodenum 

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One of the rare complications of choledochal cysts is rupture. In majority of the cases, the cause of rupture is unknown. Reconstructive surgery is the treatment of choice. We describe three patients with choledochal cyst rupture, who were admitted with acute abdomen. Diagnosis of biliary ascites with peritonitis was made in all the three patients....

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... placing the T-tube on posterior wall of the ruptured cyst, limbs of the T-tube were introduced into the common bile duct ends which were proximal and distal to the perforated cyst and purse string sutures were applied. Contrast study through the T-tube confirmed the free flow of bile to the duodenum without leakage and pancreatobiliary malunion (Fig. 2). Definitive surgery, that is, hepaticojejunostomy with Roux- en-Y anastomosis was done 12 weeks later. The patient was discharged on the 18th postoperative day without any remarkable event, and is doing well at 8 months of follow ...

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... Spontaneous CDC rupture is a rare complication reported in 1.8% to 2.8% of patients in different series [34]. The cause of the spontaneous CDC rupture is not known. ...
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PurposeComplicated choledochal cysts (CDC) have a variable presentation, and their management differs from an uncomplicated CDC. They are infrequently reported. We present our 15 years of experience in the management of complicated CDC.MethodologyWe reviewed the data of patients with CDCs managed at a tertiary level center from 2005 to 2020 from a prospectively maintained database.ResultsOf 215 patients with CDC, 123 patients presented with complicated CDC. The median age of complicated CDC was 31 years with a female preponderance (62.6%). The most common type of CDC associated with complications was type I (69.1%), followed by type IVA (29.3%). The Complicated CDC was presented as cholangitis with or without cystolithiasis (n = 45), cystolithiasis and hepatolithiasis(n = 44), malignancy(n = 10), complications associated with incomplete cyst excision (n = 10), acute pancreatitis (n = 8), chronic pancreatitis(n = 8), portal hypertension (n = 6), spontaneous rupture (n = 4), gastric outlet obstruction (n = 1). These patients were managed as a one-stage approach (52.03%) and a two-stage approach (47.96%). On univariate and multivariate analysis, increasing age, prolonged duration of symptoms, and presence of abnormal pancreaticobiliary ductal junction (APBDJ) were significantly associated with complicated CDC.Conclusion The management of complicated CDC varied depending on the associated pathology, many of them required a staged approach. Increasing age, prolonged duration of symptoms, and presence of APBDJ were significantly associated with complicated CDC.
... Type V CC typically presents with cholangitis and stone formation [17]. In 1-2% of cases, CC may present with rupture and biliary peritonitis prompting emergency biliary drainage [3,[43][44][45]. This presentation is typically seen in neonates and infants. ...
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Choledochal cysts are rare congenital disorders first described by Vater and Ezler in 1723. Their exact etiology remains incompletely understood; however, an anomalous pancreaticobiliary union (APBDU) and subsequent reflux of biliary contents into the biliary tree are thought to play a role. Accordingly, APBDU-associated choledochal cyst patients are significantly more likely to have evidence of hepatitis, cholangitis or pancreatitis and pathologically confirmed inflammation. In 1977, Todani and colleagues modified the original Alonso-Lej classification to include five types of CC. Type I and IV are the most common and most likely to be associated with malignancy. The majority of choledochal cysts are diagnosed in childhood. Clinical presentation varies and most often consists of nonspecific abdominal pain. Diagnosis is typically accomplished using multimodality imaging techniques including computed tomography, magnetic resonance imaging, ultrasound and MRCP. The use of diagnostic PTC and ERCP in CC has been largely replaced by MRCP. Appropriate management consists of prompt, complete cyst excision followed by restoration of biliary enteric continuity when necessary. Minimally invasive CC resection in the pediatric population has demonstrated acceptable outcomes. Prognosis is generally excellent; however, malignancy risk remains higher than the general population even after complete surgical excision.
... There has to be fine balance between the benefits as well as the risks associated with the treatment. The rupture of the cyst is common in children [3]. It is secondary to raised intracystic pressure because of distal stricture or stones [4,5]. ...
... The cumulative risk of spontaneous choledochal cyst rupture varies between 1.8% and 2.8% [11][12][13]. Rupture is more common during childhood and rare during post partum period of the pregnancy [3]. Various etiologies have been proposed for the spontaneous rupture of the cyst. ...
... There are multiple factors guiding the treatment. Friability of the cyst wall, hemodynamic instability, inflammation and edematous bowel mandate the drainage of the CBD either with a T-tube or percutaneous transhepatic drain [2,3]. This is followed by a definitive surgery once the acute crisis is controlled. ...
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With the advent of newer radiological investigations, choledochal cysts are being diagnosed more often in present era. These cysts are commonly diagnosed in early childhood and infancy, although some go undetected to be diagnosed in adulthood. These malformations are associated with multiple complications like cholangitis, jaundice, pancreatitis, rupture or even malignancy. Here we describe a post partum female, who was diagnosed to have choledochal cyst during sixth month of pregnancy. She presented with obstructive jaundice in cholangitis and was subjected to endoscopic retrograde cholangiopancreatography (ERCP) with stenting. This female delivered normally at term. She again had jaundice with cholangitis during early post partum period. Endoscopic stenting could be performed. She had features of peritonitis after 1 week of stenting. Investigations were performed and a diagnosis of spontaneous rupture of choledochal cyst was made. She underwent laparotomy and lavage with complete excision of the choledochal cyst and Roux-en-Y hepaticojejunostomy.
... 13 In recent years, minimally invasive treatment has become the forefront of treating conditions with minimal complications. Due to the possible severe complications [14][15][16] for expectant mothers and fetuses, it was unreasonable to conduct surgical resection and exploration immediately after the diagnosis has been made. Without a thoroughly conducted examination prior to the surgery it is difficult to create a detailed operation plan. ...
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Background & objective: Biliary cysts in pregnant women are a complex medical issue, especially when complicated with cholangitis. It is a serious and life-threatening diagnosis that can seriously endanger both the expectant mother and the fetus. However, during pregnancy, surgical treatment would lead to further complications and higher fetal mortality. Here, we propose a novel therapeutic approach that would be safe for both mother and child during pregnancy, with a definitive treatment postponed until after delivery. Methods: In this retrospective study we have summarized the clinical course of six adult patients diagnosed with choledochal cysts during pregnancy. Treatment was conducted in two stages, firstly, percutaneous cholecystostomy under ultrasound guidance and sustained negative pressure suction until delivery, and secondly, selective choledochal cyst excision when the patients recovered from delivery. Results: All the six patients gave birth to healthy babies. Four patients had Type-I choledochal cysts, and underwent Roux-en-Y hepaticojejunostomy surgery. Two patients had a Type-IV choledochal cyst. The first patient with Type-IV choledochal cyst underwent anastomosis between the secondary hepatic bile duct and jejunum and the second patient underwent laparoscopic cyst internal drainage. No serious complications were recorded after gallbladder drainage or during the perioperative period. Conclusions: Based on our single-centre experience we can conclude that treatment of choledochal cyst with cholangitis during pregnancy can be conducted safely and efficiently through the two stages strategy that we proposed in this paper. The first stage should be percutaneous cholecystostomy followed by elective surgical treatment following delivery.
... Choledochal cyst rupture is most commonly encountered in infancy or early childhood. 3 We present a rare case of choledochal cyst rupture during pregnancy with co-existent chronic pancreatitis. ...
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Choledochal cysts are a congenital disorder of the bile duct, usually presenting in childhood. As they cause weakening of the wall of the bile duct, any distal obstruction, infection or reflux of amylase-rich fluid in the biliary tract can predispose to spontaneous perforation. A 25-year-old pregnant woman at 35 weeks of gestation presented with acute abdominal and fetal distress. On emergency laparotomy, common bile duct perforations were detected. T-tube drainage and caesarean section were performed. A t-tube cholangiogram revealed the diagnosis of a choledochal cyst with an abnormal pancreatobiliary junction and chronic pancreatitis. The patient later underwent cyst excision with Roux en Y hepaticojejunostomy. Spontaneous choledochal cyst perforation in pregnancy can be successfully managed by a two-stage operation.
Article
Introduction. Choledochal cyst is enlargement of the external bile duct system that can lead to liver dysfunction and biliary cirrhosis in childhood and malignant degeneration of the liver and bile ducts in adulthood. There are many theories explaining the origin of the common bile duct cyst. However, none of them can explain the formation of all five different types. Most of them are congenital. However, some of them may be aquired. The theory of a long common biliopancreatic canal has become widespread and is still used to explain the formation of this type of cystic anomalies. If the common canal is long and its part is not surrounded by the sphincter of Oddi, the secret of the pancreas begins to be thrown into the choledochus. Proteolytic enzymes from the pancreas are quite active and can damage the epithelium and the wall of the bile ducts, which leads to their weakness and, as a result, dilatation of the choledochus. Ultrasound examination is the initial and main method for diagnosing choledochal cysts. In some cases, there is a need for endoscopic or magnetic resonance retrograde cholangiopancreatography, intraoperative cholangiography. Removal of the cyst and anastomosis of the common hepatic duct with the lumen of the jejunum or duodenum, performed through the subcostal approach, are standard procedures for the treatment of patients with a choledochal cyst. The advent of laparoscopy and the accumulation of experience in performing complex surgical interventions introduced a minimally invasive approach for the production of laparoscopic biliodigestive anastomoses. Intra- and postoperative complications include damage to the structures of the hepatic hilum, torsion of the abduction loop, incompetence and stricture of biliodigestive anastomosis, cholangitis, stone formation, adhesive intestinal obstruction, and malignancy of the cyst remnants. Conclusion. This literature review has demonstrated modern views on the origin, etiology, diagnosis and treatment of choledochal cysts. The scientific work discussed the versatile technical aspects of the surgical treatment of choledochal cysts in children and assessed its safety and effectiveness.