Choledochal Cyst and Gallbladder Disease Flashcards
What is the appropriate surgical repair for the most common type of choledochal cyst?
A. Hepaticojejunostomy
B. Choledochojejunostomy
C. Cyst resection alone
D. Observation only
E. Cholecystectomy
ANSWER: A
COMMENTS: Although it is possible to diagnose choledochal cysts in utero, more often they present by the age of 10 with right upper quadrant pain, jaundice, and occasionally a palpable mass.
Ultrasound is the appropriate first diagnostic step, examining both the intra- and extrahepatic biliary systems.
When there is a concern for biliary atresia versus a cyst, a HIDA scan can be performed to delineate the anatomy.
There are five major types of choledochal cysts:
Type I is the most common, representing about 90% of cases. This is a fusiform dilatation of the common bile duct but does not involve the intrahepatic biliary system. Surgical management of type I cysts includes resection of the affected portion of the common bile duct with a hepaticojejunostomy and Roux-en- Y limb.
Type II cysts are a diverticulum off of the side of the common bile duct, and there is no intra- or extrahepatic ductal dilation. This is managed with a simple cyst resection. Often, this may be done laparoscopically.
A type III cyst is dilatation of the very distal common bile duct and is usually either intrapancreatic or intraduodenal. These cysts are also known as choledochoceles. There is no dilatation of the proximal common bile duct or intra- hepatic ducts. These cysts may be managed with either marsupialization or with endoscopic retrograde cholangiopancreatography (ERCP) and sphincterotomy.
A type IV disease is defined as multiple cysts that may be intrahepatic, extrahepatic, or both. It is recommended that the common bile duct cyst be resected up to the hepatic ducts with a hepaticojejunostomy and Roux-en-Y limb.
Type V, also known as Caroli disease, is multiple exclusively intrahepatic biliary cysts. The operative management of this disease is not straightforward and ranges from segmental resection to liver transplantation. Cyst excision is advocated not only to avoid a biliary obstruction leading to pancreatitis and cholangitis but also to reduce the risk of malignancy.
Types I, IV, and V cysts have the highest malignant potential. This risk of malignant transformation increases with incomplete cyst excision as well as older age at the time of resection.
Discuss choledochal cysts.
Choledochal cysts are congenital, cystic dilatations of the biliary tree that can occur with an incidence of approximately 1:100,000–1:150,000 in the Western hemisphere and a reported incidence of 1:1,000 in the Asian population.
The majority of patients with choledochal cysts remain asymptomatic and diagnosis is made due to an incidental finding.
Only a minority presents with clinical signs of jaundice/obstruction, cholangitis/pancreatitis, or an acute abdomen due to secondary complications.
Diagnostics include abdominal ultrasound and blood tests, an MRCP can further specify the anatomy if necessary and in cases of acute obstruction an ERCP with stenting is required.
Choledochal cysts are premalignant conditions; therefore, the therapy consists of a complete choledochal cyst excision, early after diagnosis.
This can be performed using “open” or “laparoscopic” surgery.
The reconstruction afterwards includes a biliodigestive anastomosis, which can be either a hepatico-jejunostomy with a Roux-en-Y loop, or a hepatico-duodenostomy.
Currently, there is no evidence for the superiority of any of these techniques.
After choledochal cyst excision the lifetime risk for malignancies is reduced, but recent data suggest a lifelong elevated risk of up to 11% of cancer development even following operation.
Therefore, lifelong follow-ups are necessary.
What are choledochal cysts?
Choledochal cysts are rare congenital, cystic dilatations of the biliary tree that can occur anywhere between the duodenum and the intrahepatic bile ducts [1].
The etiology of this condition is still unknown.
The first anatomical description was made in 1723 by the German anatomist and botanist Abraham Vater (1684–1751), who is also known for the ampulla of Vater and Lamellar corpuscles. Golder Lewis McWhorter (1888–1938) was the first surgeon to report on the surgical “cure” of a 49-year-old lady with choledochal cyst at the Presbyterian Hospital in Chicago/ USA in 1924.
What is the common classification for choledochal cysts?
Todani classification. It was introduced in 1977 by Takuji Todani at the Department of Surgery/University Medical School in Okayama, Japan. Since then it has been modified, including information on the location and appearance of the choledochal dilatation (e.g. isolated or disseminated cysts) [Lee et al., Korean J Radiol. 2009 Jan-Feb;10(1):71–80)].
Type I: saccular/diffuse fusiform dilatation of common bile duct, typically inferior to the confluence of the left and right hepatic ducts (the most common type)
Type II: diverticulum of the common bile duct
Type III: intraduodenal dilatation of the common bile duct
Type IV: multiple cysts of the intra- or extrahepatic bile ducts (or both)
Type V: single or multiple intrahepatic cysts
What is the common channel?
The common channel describes the unification of the common bile duct and the pancreatic duct.
In the majority of choledochal cysts, this unification already appears outside the duodenal wall, called pancreatobiliary maljunction and leading to a “long” common channel [Kamisawa T et al. (2017), J Gastroenterol., Feb;52(2):158–163].
What are “forme fruste choledochal cysts”?
The term “forme fruste” was introduced by John R Lilly of the Children’s Hospital Denver in 1985, describing a stenosis of the distal common bile duct of unknown origin, with a “long common channel” and classical histopathological features of choledochal cysts (in the common bile duct wall).
In the majority of cases the minimal (or nonexistent) dilatation of the extrahepatic bile duct and the pancreaticobiliary maljunction are present at first admission, without signs of stones, tumor, or inflammation.
How common are choledochal cysts?
The incidence of choledochal cysts is approximately 1:100,000–1:150,000 in the Western hemisphere, with high geographical variations and a reported incidence of 1:1,000 in the Asian population.
While the majority is diagnosed during childhood, in some patients the diagnosis is delayed until adulthood.
Higher numbers are expected, due to the fact that many patients remain undiagnosed during their lifetime.
How do patients with choledochal cysts usually present?
The majority of patients with choledochal cysts remain asymptomatic and diagnosis is made due to an incidental finding.
Only a minority presents with clinical signs of jaundice/obstruction, cholangitis/pancreatitis, or an acute abdomen due to secondary complications.
Recently, prenatal ultrasound diagnosis was reported.
What are the possible complications of choledochal cysts?
Choledocholithiasis and hepatolithiasis, leading to bile duct obstruction, jaundice, cholangitis, cholecystitis, pancreatic reflux and pancreatitis, and sepsis.
Ascending cholangitis is a suspected carcinogen for malignant transformation of the cyst.
Cyst rupture and peritonitis are rare complications of choledochal cysts.
How does the diagnostic workup for suspected choledochal cyst look like?
The diagnostic workup includes serologic markers for inflammation, cholestasis as well as imaging by sonography, CT scan, hepatobiliary scintigraphy with Technetium 99 (HIDA), MRCP, or ERCP (Figs. 37.1 and 37.2).
Apart from an abdominal ultrasound, imaging in the typical age group of the patients has the disadvantage of a general anesthesia or sedation.
In large centers an abdominal ultrasound is the only preoperative imaging needed.
An MRCP can be added in unclear cases.
ERCP and potential stenting are mainly reserved for patients with acute biliary obstruction.
What is the most common complication following ERCPs?
Acute pancreatitis, which occurs in approximately 5% of cases after ERCP.
Less common complications include bleeding after sphincterotomy, duodenal perforation, and retroperitoneal abscesses.
What is the treatment of choledochal cysts?
Surgery with complete cyst excision.
Based on preoperative imaging and classification, this may include cyst excision and hepatic resections.
In rare cases with involvement of the pancreatic duct, a Whipple procedure needs to be considered.
What is the best timing of surgery?
Shortly after diagnosis, from three months of age onwards. If diagnosed during an inflammatory episode, broad-spectrum antibiotics should be given first followed by surgery after an inflammatory-free interval of 6–8 weeks.
What is the major risk if a child with choledochal cyst is left untreated?
Patients with untreated choledochal cysts have an increased risk of cholangiocarcinoma and gallbladder carcinoma.
While cases of malignant transformation during childhood are extremely rare, the lifetime carcinoma risk in adults with untreated choledochal cysts is 6–30%, increasing with age.
Furthermore, patients have a high risk of hepatolithiasis, recurrent cholangitis, and pancreatitis.
What is Caroli’s disease and how is it treated?
Caroli’s disease is a rare congenital condition with non-obstructive dilatation of the intrahepatic ducts, which can be localized in one hepatic lobe or disseminated all over the intrahepatic bile ducts.
Based on the localization an (extended) hepatectomy in unilateral appearance and liver transplantation in bilateral, disseminated cases have been described [Lee HK, et al. (2009), Korean J Radiol., Jan–Feb; 10(1):71–80].
What are surgical approaches to choledochal cyst excision?
Choledochal cyst excisions can be performed by laparotomy or laparoscopy [2].
Based on the current literature, none of the approaches is superior.
However, recent studies suggest that children undergoing laparoscopic cyst excision have longer operating times, less intraoperative bleeding (and blood transfusions), shorter hospital stay, and faster recovery.
Is complete cyst excision always feasible?
Any operation should always aim to excise the cyst completely.
However, in some cases the cyst can expand into the porta hepatis, the head of the pancreas, or the duodenum.
The extent of the resection then needs to be determined by the surgeon as remnants of the cyst predispose to malignancies.
Furthermore, in patients with recurrent inflammation prior to surgery, the cyst can be adherent the fragile tissues surrounding it.
In these cases, the risk of vascular injury is increased.
Whenever the decision for incomplete cyst excision is made, the surgeon should resect as much of the biliary epithelium as possible.
Which anatomic reconstructions are performed following cyst excision?
Hepatico-jejunostomy with a Roux-en-Y loop or hepatico-duodenostomy.
Based on the lack of comparative studies in the current literature, none of the techniques is superior [3, 4].
However, recent studies suggest that while the frequency of postoperative bile leaks and anastomotic strictures are equivalent using both techniques, the rates of cholangitis and biliary reflux are higher following hepatico-duodenostomy.
Cholecystectomy is always part of the operation.
Is there any indication for cysto-enteric drainage?
No. While cysto-enteric drainage was performed in the past, a complete cyst excision needs to be achieved according to the current consensus.
What are the intraoperative complications of choledochal cyst excisions?
Major intraoperative complications include bleeding, intraoperative cyst perforation, and narrowing of the pancreatic duct.
Furthermore, all surrounding tissue/ organs—especially the hepatoduodenal ligament should be handled with great caution.
What are postoperative complications following choledochal cyst
excisions?
The most important postoperative complication is a bile leak or stricture of the bil- iodigestive anastomosis with recurrent episodes of cholangitis.
In addition, post- operative anastomotic leaks of enteric anastomosis, fluid collections (hematoma, biloma, abscess), peritonitis, sepsis, bleedings, hepatolithiasis, biliary reflux, and pancreatitis can occur [5].
Is there a potential postoperative risk of malignancies?
After choledochal cyst excision the lifetime risk for malignancies is reduced significantly.
However, recent data suggest a lifelong elevated risk of up to 11% of a local malignancy even following operation [6].
Are postoperative follow-ups necessary?
Due to the persistent risk of malignancies, even following cyst excision, life- long follow-ups are necessary including transition to adult gastroenterology.
For adults, a yearly abdominal ultrasound including measurement of CA19-9 are recommended.
Discuss pediatric gallbladder disease.
Pediatric gallstone disease is on the rise in the United States, for two main reasons.
First, improved diagnostic tools including high-resolution ultrasound have made the detection of small stones more readily apparent.
Secondly, dietary changes have led to an increased incidence of cholesterol stones.
Additionally, improved survival in the neonatal population, many of whom are maintained on parenteral nutrition for an extended period of time, has led to pathophysiology previously seen in very limited instances.
Laparoscopic cholecystectomy remains the standard operation of choice for patients requiring surgical extirpation of the gallbladder alone.
Regional variation in expertise dictates the management of choledocholithiasis using either laparoscopic common bile duct exploration or endoscopic retrograde cholangiopancreatography.
Describe the typical history and physical exam of a child presenting with acute cholecystitis.
The classic history and physical exam of a patient with acute cholecystitis includes right upper quadrant pain associated with nausea and possibly fever.
Older children and adolescents may provide additional details such as pain following ingestion of a fatty meal.
However, younger children may manifest simply with decreased oral intake and dehydration.
In infants, the development of jaundice following an illness or TPN use may be the first clues to gallbladder disease.
Co-morbid conditions (described below) can increase the clinical suspicion for acute cholecystitis.
What laboratory tests and imaging would you order to evaluate a child with right upper quadrant pain?
CBC to evaluate for evidence of infection/inflammation.
Chemistry to assess hydration status and electrolyte imbalances.
Transaminases and bilirubin to determine if there is an obstructive component.
Elevated direct bilirubin raises the concern for choledocholithiasis.
Right upper quadrant ultrasound to evaluate for the presence of gallstones, sludge, pericholecystic fluid, gallbladder wall thickening, or biliary ductal dilatation.
What are the causes of acute cholecystitis?
a. Acalculous: often occurs in the setting of severe illness/sepsis, lack of enteral feeding, and utilization of TPN.
Since the gallbladder is not subjected to the hormonal impulses stimulating contraction, biliary stasis ensues.
This, when combined with gallbladder distension and a low flow state, are a set up for infection.
b. Calculous
i. Hemolytic: This was previously the most common cause of cholelithiasis in the pediatric population, producing pigmented stones due to increased serum levels of unconjugated bilirubin. Pediatric patients with sickle cell disease and concurrent gallbladder sludge or cholelithiasis should be considered for elective cholecystectomy with appropriate pre-operative hydration to prevent perioperative sickle cell crisis [1].
ii. Non-hemolytic: The pathophysiology of non-hemolytic cholelithiasis in the pediatric population resembles that in adults, with cholesterol stone formation in association with obesity and estrogen exposure. Other lithogenic factors include conditions predisposing to gallbladder stasis, decreased enterohepatic circulation of bile (ileal resection, prolonged lack of enteral nutrition, TPN) or systemic illness. The incidence of non-hemolytic cholelithiasis has increased in incidence over time in children [3].
Which pediatric patients are at increased risk of developing acute cholecystitis?
Those with short gut/intestinal failure, history of severely prematurity, hemolytic diseases (sickle cell, hereditary spherocytosis, cystic fibrosis, obesity, and meta- bolic syndrome [1–3].
What is the treatment for cholelithiasis and acute cholecystitis?
The standard surgical treatment for both symptomatic cholelithiasis and acute cholecystitis is laparoscopic cholecystectomy.
In acute cholecystitis, the patient should first be fluid resuscitated and given intravenous antibiotics [1–3].
What are the important anatomical structures to identify during a laparoscopic cholecystectomy?
The gallbladder is first grasped at the fundus and retracted cephalad. It is impor- tant to establish the “critical view of safety,” by retracting the infundibulum laterally.
This allows the surgeon to visualize the infundibulum joining with the cystic duct and deflects these structures away from the common bile duct, which is located more posteriorly.
This view allows easy access to the cystic artery.
Important anatomic considerations in this area include the Triangle of Calot, which is bordered by the cystic duct, the common bile duct and the inferior edge of the liver; however, the common bile duct is often not exposed during the operation [4].
What are the most common complications of the surgical treatment for acute cholecystitis? How are they managed?
a. Bile duct injury: if recognized during the time of operation, bile duct recon- struction with roux-en-Y hepaticojejunostomy is typically the operation of choice. If recognized post-operatively, percutaneous drainage of the biloma, decompression of the biliary tree, and delineation of the anatomy should be performed in order to determine the next step for operative reconstruction.
b. Bleeding: The most commonly injured artery during the operation is the right hepatic artery. If recognized at the time of operation this should be addressed by either repair or ligation (if repair proves impossible). Post-operative hemorrhage is most often from the cystic artery. This, or the right hepatic artery, can be embolized with interventional radiology if necessary.
c. Bile leak: Usually from a displaced clip off of the cystic duct stump. This is treated with ERCP with sphincterotomy and/or stent placement to provide a path of least resistance for bile to flow allowing the stump to heal [3].
Describe the symptoms, physical exam and laboratory findings for a child with a stone is in their common bile duct.
If a stone escapes the gallbladder and becomes lodged in the common bile duct, this is referred to as choledocholithiasis.
If this is associated with obstruction that leads to infection it is referred to as ascending cholangitis.
The presentation is similar to that for acute cholecystitis, however the child frequently has associated jaundice.
Laboratory findings are notable for elevation of the transaminases, total and direct bilirubin due to obstruction of the common bile duct.
What is the treatment for choledocholithiasis and cholangitis?
Choledocholithiasis alone can be managed with either laparoscopic cholecystectomy and common bile duct exploration or ERCP followed by laparoscopic cholecystectomy in the same hospitalization.
There are pros and cons of each treatment approach, but ultimately the decision relies on the regional expertise of the surgeon and the interventional gastroenterology team [3].
Ascending cholangitis should be treated with fluid resuscitation, IV antibiotics, and once stabilized, ERCP with sphincterotomy and/or stone retrieval.
Laparoscopic cholecystectomy should be performed during the same hospital admission, after the patient has recovered from their acute illness but prior to discharge.
What are the indications for performing intra-operative cholangiogram?
Most commonly to evaluate for retained stones in the common bile duct, based on preoperative direct hyperbilirubinemia.
It is also helpful with unclear anatomy and concern for intra-operative bile duct injury [1, 2].
What is biliary dyskinesia and what are the elements from the history and physical that would heighten the clinical suspicion for this problem?
Biliary dykinesia is usually defined as decreased/ineffective gallbladder contractility with an ejection fraction <20–35% on a cholecystokinin (CCK) HIDA scan [1–3].
Patients with biliary dyskinesia often have typical biliary symptoms including nausea, vomiting, & right upper quadrant pain however usually lack signs of systemic infection.
Their pain may be more chronic in nature as opposed to intermittent episodes of acute pain.
What are the typical laboratory and imaging findings of a patient with biliary dyskinesia?
The laboratory data for a patient with biliary dyskinesia are often normal.
Typically, gallbladder ultrasounds on these patients fail to demonstrate evidence of gallstones.
The diagnosis is made by measuring the biliary ejection fraction using CCK-stimulated biliary scintigraphy (HIDA scan).
What is the treatment for biliary dyskinesia?
Laparoscopic cholecystectomy is offered for the treatment for biliary dyskinesia.
Oftentimes this diagnosis is one of exclusion, and unfortunately this does not always provide symptomatic relief.
As a generalization, patients with very low biliary ejection fraction are more likely to have symptomatic relief from cholecystectomy [2].
Persistent pain despite cholecystectomy will require further work-up.
Regarding the diagnosis of choledochal cyst, which of the following is true?
A. Adult form occurs in children older than 7 years.
B. Classical trait is acholic stool, jaundice, and hepatomegaly.
C. Infantile form occurs in 1 to 3 months of age.
D. High serum unconjugated bilirubin.
E. Preoperative liver biopsy is not indicated.
C
Adult form occurs in children older than two years.
The classical triad is abdominal pain, palpable mass, and jaundice.
Infantile form occurs in 1–3 months of age, characterized by jaundice, acholic stool and hepatomegaly.
Serum conjugated bilirubin is high.
Pre-operative liver biopsy is indicated to exclude cirrhosis and malignancy.
The most common surgical procedure for choledochal cyst is:
A. Cystoduodenostomy
B. Roux en y cystojejunostomy
C. Cyst excision with Roux-en-y hepaticojejunostomy
D. End-to-side Roux-en-y choledochojejunostomy
E. Longitudinal duodenoscopy for choledochocele.
C. Cyst excision with Roux-en-y hepaticojejunostomy
What is the normal common bile duct diameter for pediatric patients?
How are choledochal cysts classified?
Different classifications have been proposed for choledochal cyst. However, the Todani classification has been the most widely accepted. According to this classification, CCs are classified into five types:
□ Type I
□ Ia: Cystic dilatation of the CBD
□ Ib: Fusiform dilatation of the CBD
□ Type II: Diverticulum of the CBD
□ Type III: Choledochocele (dilatation of the terminal CBD within the duodenal wall)
□ Type IV
□ IVa: Multiple cysts of the extrahepatic and intrahepatic ducts
□ IVb: Multiple extrahepatic duct cysts
□ Type V: Intrahepatic duct cyst (single or multiple, as in Caroli disease).
Forme fruste is a special variant of CC characterized by pancreaticobiliary malunion, but little or no dilatation of the extrahepatic bile duct.
Children present with symptoms similar to those in patients with a CC.
Excision of the extrahepatic bile duct is recommended in these children because of the likely eventual development of cancer due to chronic pancreaticobiliary reflux.
Type I CCs predominate. Together with type IVa cysts, they account for more than 90% of cases.
Caroli disease is characterized by segmental saccular dilatation of the intrahepatic bile ducts. It may affect the liver diffusely or be localized to one lobe.
How is pediatric biliary disease diagnosed?
The classic symptom of right upper quadrant abdominal pain from biliary disease occurs more commonly in adolescents than in younger children.
Younger children will more commonly present with nausea and vomiting and may not exhibit the classic symptoms of abdominal pain.
Overall, approximately 60% of children and adolescents with cholelithiasis are symptomatic.
The classic pain of biliary colic includes sharp, crampy abdominal pain that radiates to the right shoulder and scapula.
It most commonly occurs after a fatty meal and can last several hours.
Transabdominal US is the diagnostic imaging of choice with a sensitivity and specificity greater than 95% for detecting gallstones. However, the sensitivity of US for detecting gallstones is lower in children than in adults.
Furthermore, US can identify involvement of the common and hepatic ducts, evidence of gallbladder inflammation, and other abnormalities in the liver and pancreas.
CT has little utility for diagnosing cholelithiasis and can expose children to unnecessary radiation.
If the US identifies stones with severe gallbladder wall thickening (>4 mm), edema, and pericholecystic fluid, a nuclear medicine study can help determine the presence of acute cholecystitis.
In patients with acute cholecystitis, the radioactive analogs are excreted in the liver, but do not pass into the gallbladder due to obstruction of the cystic duct.
In addition, MRCP can be a useful imaging modality for suspected pathology of the gallbladder. It is a noninvasive test and can evaluate pancreaticobiliary anatomy for evidence of a stricture, obstruction, or injuries related to trauma.
However, the biggest limitation for using MRCP is the need for sedation or general anesthesia.
A second diagnostic adjunct for cholelithiasis is the use of endoscopic US, which can help identify stones not seen on transabdominal US.
What are the special considerations needed when evaluating children with gallbladder disease?
There are four special considerations when evaluating children with gallbladder disease.
1) The first is the child with sickle cell disease. The most important principle for improved operative outcomes in children with sickle cell anemia relies on adequate hydration and transfusion to an acceptable hemoglobin level of 10 mg/dL.
A recent retrospective review reported that elective cholecystectomy for cholelithiasis in patients with sickle cell disease decreases morbidity when compared with children undergoing an emergent operation. This result is most likely secondary to optimization of hydration prior to the elective cholecystectomy.
2) The second circumstance arises in the patient with HS who is undergoing splenectomy. A right upper quadrant US should be performed to evaluate for cholelithiasis prior to splenectomy as it is relatively straightforward to remove the gallbladder at the same time if gallstones are noted.
However, in a study of 17 patients with spherocytosis, but not cholelithiasis, none of these patients developed cholelithiasis with a mean follow-up of 15 years.
Thus, it is probably not necessary to prophylactically remove the gallbladder in patients with HS undergoing splenectomy.
3) Another area of increasing debate is the routine use of an intraoperative cholangiogram during a laparoscopic cholecystectomy. There is little evidence for its routine use. However, if there is a concern about the anatomy or a concern about the presence of a common duct stone, surgeons should consider performing this study.
4) A fourth situation involves the child or adolescent who presents with known or suspected choledocholithiasis. A recent report noted that approximately 11% of children undergoing cholecystectomy were found to have a CBD stone.
Associated signs include jaundice, dark urine, and acholic stools.
In adults, this situation is most commonly handled by ERCP with sphincterotomy and stone extraction either before or after the laparoscopic cholecystectomy.
However, in children, many pediatric gastroenterologists are not trained in this technique and many children’s hospitals require the help of a gastroenterologist for adults.
One approach in children with suspected choledocholithiasis is to perform the ERCP and sphincterotomy before performing the laparoscopic cholecystectomy.
In addition, CBD exploration can be a safe approach for choledocholithiasis in situations in which there is limited availability of ERCP.
This can be performed laparoscopically by experienced surgeons and with an open approach for those who are less experienced.
What is the typical technique for a laparoscopic cholecystectomy?
Four small incisions are generally used for the traditional laparoscopic cholecystectomy.
A 10-mm port is introduced in the umbilicus, and a 10-mm telescope is then inserted. (Although the optics are satisfactory with a 5-mm telescope, it is helpful to have a 10-mm port in the umbilicus to extract the gallbladder, especially if it is inflamed, so there is no real benefit to using a 5-mm umbilical port and telescope.)
A 5-mm cannula is inserted in the epigastrium to the right of the patient’s midline, which becomes the main operating site for the surgeon.
Two instruments are then placed on the patient’s right side, one in the right mid-abdomen and one in the right lower abdomen.
A stab incision technique is often possible for these two right lateral instruments as they are not exchanged during the operation. Also, 3-mm instruments can be utilized in younger patients at these two sites as well.
The patient is then rotated into reverse Trendelenburg and to the patient’s left. The gallbladder is grasped using the right lower abdominal instrument and rotated cephalad over the liver to expose the triangle of Calot. The surgeon then utilizes the right upper abdominal instrument and the epigastric instrument to perform the procedure.
Initial attention is directed toward lysing adhesions to the infundibulum. Blunt dissection follows to identify the cystic duct and cystic artery. At this point, lateral retraction of the infundibulum is essential to orient the cystic duct at 90° to the CBD to help prevent misidentification of these two structures.
It is important to visualize the critical view of safety to correctly identify the anatomy. This critical view is bounded by the CBD medially, the cystic duct inferiorly, the gallbladder laterally, and the liver superiorly.
After the cystic duct and common duct have been correctly identified, two options exist.
A cholangiogram can be performed if the anatomy is unclear or if there is suspicion of common duct stones.
If the anatomy is clear and there is no suspicion of choledocholithiasis, it is reasonable to ligate the cystic duct with endoscopic clips and then divide it. Similarly, the cystic artery is ligated and divided.
Once these two structures have been ligated and divided, the gallbladder is then detached from the liver using retrograde dissection with cautery.
The hook cautery, spatula cautery, or Maryland dissecting instrument connected to cautery can be used for this purpose.
Prior to complete detachment of the gallbladder from the liver, the area of dissection should be inspected to ensure hemostasis, and then the gallbladder is completely detached. If there is little to no inflammation, the gallbladder can usually be exteriorized through the umbilical incision without using an endoscopic bag. However, for inflamed gallbladders, it is best to remove the specimen using a bag.
A 2-month-old girl presents with jaundice. She was born at full-term and spent two weeks in the NICU. Total bilirubin is 9 and direct bilirubin is 7. MRCP of the abdomen is shown. What is the most appropriate next step in management?
Choices:
1. Observation
2. Treatment with ursodiol
3. Percutaneous drainage
4. Resection and hepaticojejunostomy
Answer: 4 - Resection and hepaticojejunostomy
Explanations:
•Choledochal cyst is a congenital cystic dilatation of the biliary tree, classified by Todani into five types. It is occasionally premalignant, as it can transform into cholangiocarcinoma.
• Most choledochal cysts are incidentally diagnosed during childhood. The triad of abdominal pain, palpable mass, and jaundice is found in only 20% of patients.
• Evaluation may be performed with ultrasound, CT, MRCP, or ERCP.
MRCP is the gold standard for diagnosis with a sensitivity of
90-100%. Type I choledochal cyst appears as solitary fusiform or cystic dilation of the common bile duct. Choledochal cysts should mirror the signal intensity of normal bile ducts. Abnormal wall enhancement or thickening should raise concern for superinfection or malignancy.
• The management of choledochal cysts depends on the type and the extent of hepatobiliary pathology. As a rule, all cysts should be resected and bile flow restored. Type I and type IV choledochal cysts are managed with complete excision of the choledochus and restoration of bile flow by Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy.
StatPearls
What is the classification of bile duct injury?
