Choledochal Cyst and Gallbladder Disease Flashcards

Question

Describe the typical history and physical exam of a child presenting with acute cholecystitis.

Answer 1

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.

Answer 2

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.

Answer 3

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].

Answer 4

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].

Answer 5

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].

Answer 6

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].

Answer 7

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].

Answer 8

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.

Answer 9

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.

Answer 10

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].

Answer 11

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.

Answer 12

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).

Answer 13

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.

Answer 14

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.

Answer 15

C. Cyst excision with Roux-en-y hepaticojejunostomy

Answer 16

A Pancreatitis is more common after choledochocele (type III) surgery. For choledochocele surgery, a longitudinal duodenal incision required to expose the intraduodenal choledochocele. Unroofing of cyst and mucosa is reapproximated. Bile duct and pancreatic duct must be identified and calibrated. If opening is narrow, sphinteroplasty should be done. Rarely cyst located in the head of pancreas that are not amenable to internal drainage into the duodenum. For that, pancreaticoduodenectomy may be required. Syed/MCQ

Answer 17

E The histological type adenocarcinoma can occur. Syed/MCQ

Answer 18

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.

Answer 19

There are many theories to explain the development of a CC. However, none of these can explain the formation of the five different types of CC. CCs seem to be either congenital or acquired. Congenital cysts develop during fetal life. These appear to develop as a result of a prenatal structural defect in the bile duct. Shimotake et al. found that the total number of ganglion cells within the wall of these CCs is significantly lower than in control specimens. Also, smooth muscle fibers are more abundant in the cystic type than in the fusiform type. CCs, which develop later in life, are considered “acquired.” The theory of the long common biliopancreatic channel proposed by Babbitt has been widely accepted to explain the formation of this type. Normally, the terminal CBD and pancreatic duct unite to form a short common channel, which is well surrounded by the Oddi sphincter. This normal anatomic arrangement prevents the reflux of pancreatic fluid into the bile duct. If this common channel is long and part of it is not surrounded by the normal sphincter, pancreatic secretions can reflux into the biliary tree. Proteolytic enzymes from the pancreatic fluid are activated and can cause epithelial and mural damage that leads to mural weakness and dilatation of the choledochus. This theory is supported by the fact that high concentrations of activated pancreatic amylase and/or lipase have been found in patients with CC and long pancreaticobiliary channels. In an experimental study, CC was produced by creating a choledochopancreatic end-to-side ductal anastomosis. In addition, a high incidence of a common channel has been detected in CC patients. Obstruction at the level of the pancreaticobiliary junction may be an associated causal factor in choledochal dilatation. An experimental model for the study of cystic dilatation of the extrahepatic biliary system has been produced by ligation of the distal end of the CBD in the newborn lamb. High biliary pressure in patients with CC have also been recorded during operative correction. In adults, an anomalous union of the pancreaticobiliary duct is defined when the common pancreaticobiliary channel is longer than 15 mm or when its extraduodenal portion is more than 6 mm. In a study of 264 infants and children undergoing endoscopic retrograde cholangiopancreatography (ERCP), the maximal length of the common channel was found to be 2.7 mm among children 3 years or younger, 4 mm among children 4–9 years old, and 5 mm between 10 and 15 years old. A genetic basis for CC is suspected because its female predominance and different prevalence among countries in Asia and Europe versus United States. Using “trio-based” exome-sequencing of 31 patients with CC, Wong et al. recently reported 13 genes that were recurrently mutated at different sites.

Answer 20

An inflammatory reaction within the CC is noted in most cases. It is minimal in infants and gradually becomes more significant as the patient gets older. The degree of mucosal ulcerations and pericystic inflammation becomes more severe after repeated bouts of cholangitis. Stones or debris may be found in the CC, along with a dilated intrahepatic bile duct and a common biliopancreatic channel. Liver histology varies from normal to cirrhosis, depending on the patient’s age and degree of cholangitis.

Answer 21

Females are affected more often than males. In our series of 400 cases, the female-to-male ratio was 3.2:1. Clinical presentations differ according to the age of onset and the type of cyst. An abdominal mass or jaundice is a common finding in an infant with CC, whereas abdominal pain is more often seen in older children. The cystic form usually presents with an abdominal mass, whereas the fusiform type is usually found in patients presenting with abdominal pain. Choledochal cysts diagnosed antenatally are more likely cystic in nature. Clinical manifestations among our 400 cases included abdominal pain (88%), vomiting (46%), fever (28%), icterus (25%), discolored stool (12%), abdominal tumor (7%), and the classic triad (2%). Complications such as perforation and hemobilia are rare. However, pancreatitis is common. Malignant change is a late complication, mostly seen in adults.

Answer 22

Ultrasonography (US) is the initial imaging method of choice. Contour and position of the CC, the status of the proximal ducts, vascular anatomy, and hepatic echotexture can be evaluated on US. ERCP allows excellent definition of the cyst as well as the entire anatomy, including the pancreatobiliary junction. However, this investigation is invasive and has complications such as pancreatitis, perforation of the duodenal or biliary tracts, hemorrhage, and sepsis. Magnetic resonance cholangiopancreatography (MRCP) is highly accurate in the detection and classification of the cyst. The overall detection rate of MRCP for a CC is very high (96–100%) and should be considered a first-choice imaging technique for evaluation. Intraoperative cholangiography is indicated when the anatomic detail of the biliary tract cannot be demonstrated by MRCP or ERCP. Contrast-enhanced computed tomography (CT) may be indicated in some patients with pancreatitis or if an associated tumor is suspected.

Answer 23

Cystoduodenostomy and cystojejunostomy have been abandoned due to cholangitis, stone formation, and malignant degeneration. External drainage is indicated for a perforated cyst in patients whose condition is too unstable to perform cystectomy and a bilio-enteric anastomosis. Cyst excision and a bilio-enteric anastomosis is the preferred approach for most patients. The cyst should be excised at the level of the common biliopancreatic channel orifice at its distal end and approximately 5 mm from the confluence of the right and left hepatic ducts at the proximal end. Postoperative malignancy in a residual cyst on either the hepatic duct side or from the distal part has been reported. A review from the English language and Japanese literature of 23 patients with carcinomas of the bile duct developing after CC excision found that malignancy developed in the intrapancreatic remnant of the bile duct or CC in 6 patients, in the remnant of the CC at the hepatic side in 3 patients, in the hepatic duct at or near the anastomosis in 8 patients, and in the intrahepatic duct in 6 patients. Abdominal pain and pancreatitis due to leaving a remnant of the cyst in the pancreatic head also have been described. Operative correction can be performed safely in all age groups.

Answer 24

Biliary infection should be treated before operation. A prolonged prothrombin time secondary to cholestasis should be corrected with intravenous vitamin K. Drugs for elimination of ascaris are given in areas where ascaris is prevalent.

Answer 25

Many surgeons use hepaticojejunostomy, whereas others prefer hepaticoduodenostomy. Fat malabsorption and duodenal ulcer are the main concerns with a hepaticojejunostomy. In addition, the operative time is longer in comparison with hepaticoduodenostomy. Complications after Roux-en-Y hepaticojejunostomy, such as a twist of the Roux limb, intestinal obstruction, and duodenal ulcers, have been reported. On the other hand, cholangitis and gastritis due to bilious reflux are the main concerns with hepaticoduodenostomy. However, the operative time is shorter in comparison with hepaticojejunostomy. A hepaticoduodenostomy is considered more physiologic because the bile drains directly into the duodenum. This anastomosis is performed above the transverse colon mesentery, which may help prevent intestinal obstruction from adhesions. Hepaticojejunostomy The ligament of Treitz is identified. A 5-0 silk suture is placed 10 cm distal to the ligament of Treitz in the newborn, 20 cm in infants, and 30 cm in children. A second suture (5-0 PDS, Ethicon, Inc., Somerville, NJ) is placed 2 cm below the first suture to mark the jejunal limb, which will be anastomosed to the hepatic duct. The jejunal segment containing the two sutures is grasped with a locking instrument. The previously made transumbilical vertical incision is extended 1.0 cm cephalad. The jejunum is then exteriorized, and the jejunojejunostomy is performed extracorporeally. The jejunum is then returned into the abdominal cavity. The extended umbilical incision is closed, and the laparoscopic instruments are reinserted. The Roux limb is passed through a window in the transverse mesocolon to the porta hepatis. The jejunum is opened longitudinally on its antimesenteric border a few millimeters from the end of the Roux limb to avoid the creation of a significant blind pouch as the child grows. The hepaticojejunostomy is fashioned using two running sutures of 5-0 PDS (interrupted sutures are used when the diameter of the common hepatic duct is <1 cm). The anastomosis is performed from left to right using 3-mm instruments. If the diameter of the common hepatic duct is too small, ductoplasty is performed by opening the common hepatic duct and incising the left hepatic duct longitudinally for a variable distance. Different techniques of ductoplasty have been also described by other surgeons. Mesenteric defects in the transverse mesocolon and the small bowel mesentery are closed. The gallbladder is detached from its bed, and the cyst and gallbladder are removed through the umbilicus. The operative field is washed with warm saline, and a subhepatic closed suction drain is left. Hepaticoduodenostomy Following cyst resection, the duodenum is mobilized as much as possible, and a hepaticoduodenostomy is constructed 2–3 cm distal to the pylorus using two running sutures of 5-0 PDS. As noted previously, interrupted sutures are used when the diameter of the common hepatic duct is less than 1.0 cm. The rest of the operation is performed as previously described. Alternative Reconstruction Techniques An alternative biliary reconstruction technique is an end-to-end hepaticojejunostomy (in contrast to end-to side). This should be performed only if there is no significant size discrepancy between the hepatic duct and Roux limb. A hepaticoduodenostomy with jejunal interposition can be performed if desired. Open Operation The open technique is used in patients with a perforated cyst, in patients who have had previous hepatobiliary operation, or if the surgeon does not feel comfortable with the laparoscopic approach. A high transverse upper quadrant incision is used. The operative technique then follows that described for the laparoscopic approach. The cyst is mobilized as previously described and divided and removed, and either a hepaticojejunostomy or hepaticoduodenostomy is performed. Single-Incision Laparoscopic Operation The single incision laparoscopic operation has been described by several authors in an attempt to achieve better cosmetic outcomes. A transumbilical or periumbilical incision is utilized. Three ports (3 or 5 mm) are then inserted through separate sites in the umbilical fascia. The liver is secured to the abdominal wall with a stay suture placed at the round ligament. The cystic artery and cystic duct are isolated and divided. A second traction suture is placed at the junction of the distal cystic duct and gallbladder fundus to elevate the liver and splay the liver hilum. The third suture is placed at the CC to facilitate the dissection around the cyst. The rest of the operation is continued similar to the conventional laparoscopic operation. Robotic Approach for Choledochal Cyst The robotic approach also has been described. This technique appears safe and effective, but the operative time is quite long.

Answer 26

Laparoscopic Approach Endotracheal intubation and general anesthesia are standard. Epidural analgesia can provide excellent postoperative pain relief. Broad-spectrum intravenous antibiotics are best given at induction of anesthesia and continued for 5 days postoperatively. A nasogastric tube, rectal tube, and urinary catheter are used to decompress the stomach, colon, and bladder. The patient is placed in a 30° lithotomy position (Fig. 44.5). The surgeon stands or sits at the lower end of the operating table between the patient’s legs. The first laparoscopic operation for CC was reported in 1995. This approach quickly became popular and has become the routine approach in many centers. The laparoscopic approach is preferred for most types of CC: I, II, and IV. Relative contraindications are in patients with perforation, previous biliohepatic surgery, or especially newborns with damaged hepatic functions. A 10-mm cannula is inserted through the umbilicus for the telescope. Three additional 5- or 3-mm ports are introduced for the working instruments: one in the right flank, another in the left flank, and one in the left hypochondrium Carbon dioxide pneumoperitoneum is maintained at a pressure of 8–12 mmHg. The liver is secured to the abdominal wall with a suture placed around the round ligament. The cystic artery and the cystic duct are identified, clipped, and divided. A second traction suture is placed at the junction of distal cystic duct and gallbladder fundus to elevate the liver and expose the hepatic hilum. The appearance of the cyst, liver, and spleen is noted. Intraoperative cholangiography via the gallbladder can be performed if the anatomy has not been clearly defined preoperatively. With a large cyst, bile can be aspirated through a catheter, which reduces the cyst size to facilitate the pericystic dissection. The duodenum is retracted downward using a dissector inserted through the left lower port. The mid-portion of the cyst is dissected circumferentially. Separation of the cyst from the hepatic artery and portal vein is meticulously performed until a dissector can be passed through the space between the posterior cyst wall and the portal vein going from left to right. The cyst is then divided at this site. The inferior part of the cyst is separated from the pancreatic tissue down to the common biliopancreatic duct using a 3-mm dissector for cautery and dissection. Protein plugs or calculi within the cyst and common channel are washed out and removed. The inferior part of the cyst is opened longitudinally and inspected to identify the orifice of the common biliopancreatic channel. A small catheter is then inserted into the common channel. Irrigation with normal saline via this catheter is performed to eliminate protein plugs until clear fluid returns and the catheter can be passed down into the duodenum. A cystoscope can be used to measure the length of the common channel and remove protein plugs in it if its diameter permits. The distal CC is then clipped and divided at the level of the orifice of the common channel. The cephalad portion of the cyst is further dissected to the common hepatic duct. The cyst is initially divided at the level of the cystic duct. After identifying the orifice of the right and left hepatic ducts, the rest of the cyst is removed, leaving a stump approximately 5 mm from the bifurcation of the hepatic ducts. Irrigation with normal saline through a small catheter inserted into the right and then into the left hepatic duct is performed to wash out protein plugs or calculi until the effluent is clear. If the cyst is intensely inflamed with extensive pericystic adhesions, the cyst is opened by a transverse incision on its anterior wall. The dissection of the cyst wall from the portal vein is then carefully performed while viewing the cyst from inside and outside. After dividing the mid-portion of the cyst, the upper and lower parts of the cyst are removed as previously described.

Answer 27

Injury to the Portal Vein This complication can be prevented by keeping the dissection as close to the cyst wall as possible. When severe pericystic inflammation and adhesions are present, opening the cyst on its anterior wall and carefully separating the left and posterior walls of the cyst from the portal vein, while viewing internally and externally, can help prevent injury to the portal vein. Transection of Both Hepatic Ducts This situation can happen when the hepatic bifurcation is low and away from the liver hilum. This complication can be avoided by identifying the orifice of the right and left hepatic ducts by internal inspection before excising the cyst from the hepatic duct. Injury to the Pancreatic Duct Understanding the anatomy of the common biliopancreatic channel via MRCP, ERCP, or perioperative cholangiography is important. Internal inspection of the distal choledochus to identify the orifice of the common biliopancreatic duct helps the surgeon decide where the distal part of the cyst should be divided. Twist of Roux Limb This complication can happen during cystectomy and hepaticojejunostomy. Careful inspection of the Roux limb before performing hepaticojejunostomy is mandatory.

Answer 28

Oral feeding is initiated after the fluid from the gastric tube becomes clear, usually by postoperative day 2 or 3. The abdominal drain is removed on day 5 if there is no evidence of leak from the bilio-enteric anastomosis. Complications from the laparoscopic approach are similar or lower when compared with the open operation. Early postoperative complications include bleeding, anastomotic leak, pancreatic fistula, and intestinal obstruction. The anastomotic leak and pancreatic fistula often resolve with drainage, intravenous antibiotics, nasogastric decompression, and parenteral nutrition. Significant late complications included cholangitis, anastomotic stricture, intrahepatic calculi, and bowel obstruction. Cholangitis without anastomotic stricture or intrahepatic calculi is treated with antibiotics, whereas endoscopic maneuvers or reoperation are used for anastomotic stricture or intrahepatic calculi. The intrapancreatic remnants are difficult to manage. Partial or complete removal of the pancreatic head may be necessary.

Answer 29

Caroli Disease (Type V) Partial hepatectomy is indicated for the localized type of Caroli disease, and liver transplantation is usually needed for diffuse disease. Choledochocoele (Type III) Endoscopic unroofing of a choledochocele, with sphincterotomy of the CBD or sphincterotomy alone, are considered the preferred treatment for choledochocele. (IOC, duodenotomy, cyst excision, marsupialization, or ERCP sphincterotomy)

Answer 30

Patients with cholelithiasis and biliary dyskinesia present with similar symptoms, including postprandial pain, nausea, and vomiting. Although patients with cholelithiasis are classically thought to have biliary colic, their symptom complex overlaps with those of biliary dyskinesia such that it is difficult to discern the etiology of the symptoms if they are arising from gallbladder disease. The initial imaging study for abdominal complaints is often an abdominal film, although gallstones are rarely radiopaque. Usually, this study is nonspecific and not helpful, and an abdominal US is then obtained. Cholelithiasis is readily documented on US, but the US is usually normal with biliary dyskinesia. When considering the diagnosis of biliary dyskinesia, a cholecystokinin (CCK)-stimulated hepatobiliary iminodiacetic acid (HIDA) scan is the next appropriate step. After fasting for 6 hours, the patient is given an intravenous injection of technetium-99–labeled iminodiacetic acid analog. When peak gallbladder filling occurs, CCK is administered over 60 minutes and the gallbladder ejection fraction is calculated. A gallbladder ejection fraction less than 35% is diagnostic of biliary dyskinesia. Laparoscopic cholecystectomy is recommended for symptomatic cholelithiasis, and the symptoms usually resolve following cholecystectomy. For biliary dyskinesia, the ideal treatment is unclear. Nevertheless, many patients, both children and adults, undergo laparoscopic cholecystectomy with good, although not uniform, results. Resolution or improvement in symptoms after cholecystectomy has been reported in 44–97%. In a recent series, pain resolution was found in almost 90% of patients with a median follow-up of 3 weeks. However, there is no reliably consistent preoperative indicator of symptomatic relief following cholecystectomy for biliary dyskinesia. Symptom resolution may be more likely the lower the gallbladder ejection fraction. In one report, unless the child had an ejection fraction of less than 15%, there was not predictable relief of symptoms. In another study with a median follow-up of 3.7 years, the authors found that 60% of patients’ symptoms resolved if they had an ejection fraction of less than 35% or pain with CCK administration. Therefore, prior to laparoscopic cholecystectomy for biliary dyskinesia, a careful and thorough discussion with the family is important to inform them that their symptoms may not completely resolve following cholecystectomy.

Answer 31

The incidence of cholelithiasis is increasing secondary to the epidemic of childhood obesity. Excess cholesterol causing the supersaturation of bile leads to cholesterol stones, which is the most common type of stone encountered in children today. Other types of stones include pigmented stones from hemolytic disorders such as sickle cell anemia, hereditary spherocytosis (HS), and thalassemias. The incidence of pigmented stones is around 15% in children and is secondary to the increased release of hemoglobin from hemolysis resulting in an increase in bilirubin. The increased bilirubin becomes conjugated with calcium leading to the pigmented stones. Other reasons for the development of cholelithiasis include long-term total parenteral nutrition (TPN), dehydration, cystic fibrosis, short bowel syndrome, ileal resection, mast cell activation, and use of oral contraceptives.

Answer 32

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.

Answer 33

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.

Answer 34

Acute acalculous cholecystitis is defined as inflammation of the gallbladder without the presence of gallstones. This entity can result from bile stasis, ischemia, or both. Risk factors include TPN, prolonged fasting, volume depletion, multiple transfusions, and sepsis. Symptoms are similar to those of biliary colic; however, the right upper quadrant pain can be more severe. The diagnosis is made with an US that reveals gallbladder wall thickness (>4 mm), edema, and pericholecystic fluid without the presence of gallstones.

Answer 35

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.

Answer 36

The revolution in minimally invasive surgery began with the laparoscopic approach for cholecystectomy. The standard four-port technique has been modified, and now surgeons can perform this procedure through a single umbilical incision. A recent prospective randomized trial enrolled 60 children to either a single-incision laparoscopic cholecystectomy or the standard four-port technique. The results from that trial showed that children who underwent a single-incision laparoscopic cholecystectomy had longer mean operative times when compared with the standard fourport technique as well as greater surgical difficulty. Furthermore, patients undergoing a single-incision laparoscopic cholecystectomy described having more pain, requiring higher doses of postoperative analgesia, as well as accruing more significant hospital charges. Regardless of whether the patient is undergoing the fourport technique or a single-incision approach, the patient is placed supine on the operating table and two video monitors are positioned at the head of the table. An orogastric tube is inserted for decompression of the stomach. For the single-incision approach, some surgeons prefer to stand between the patient’s legs, whereas the surgeon usually stands on the patient’s left side for the four-port technique. For both approaches, the patient is prepped and draped widely. For single-site umbilical laparoscopic cholecystectomy, it is necessary to use an umbilical incision of approximately 2 cm in length. In the United States a premanufactured port is often utilized. The two main devices used are the SILS Port (Covidien, Norwalk, CT) and the TriPort (Olympus America, Center Valley, PA). The SILS Port is a foam port with three working channels. The fourth instrument can usually be placed along the left side of the port. Although the TriPort is designed for three instruments, a fourth 3-mm instrument can be inserted through one of the insufflation channels. It is helpful to have a long telescope so that the telescope holder is standing out of the way of the operating surgeon. Outside the United States, many surgeons place a single port in the umbilicus with instruments inserted through the fascia surrounding the umbilicus. Sometimes, low-profile, 5-mm individual ports are utilized. Regardless of the technique and orientation of the instruments through the umbilicus, the principles of the procedure are the same as for the traditional four-port laparoscopic cholecystectomy. Lateral retraction of the infundibulum is important for visualization of the triangle of Calot and critical view of safety. The cystic duct and cystic artery are similarly ligated and divided as with the four-port technique. One difference between the two approaches is that it is best to irrigate and suction all the fluid prior to exteriorizing the gallbladder as gallbladder removal entails removing the premanufactured port (if utilized). It can often be difficult to reinsert these ports, so it is best to irrigate and suction prior to extracting the gallbladder. After removing the gallbladder and umbilical port, the umbilical fascia is closed with either interrupted or continuous 0-absorbable suture. The skin is approximated with interrupted 5-0 plain sutures. The main advantage of the single-incision approach appears to be cosmesis, but this advantage is marginal in most patients.

Answer 37

Answer: 1 - Cholangitis Explanations: • Cholangitis is a feared postoperative complication after choledochal cyst excision and Roux-en-Y hepaticojejunostomy, with an incidence of 2% to 5%. • The mechanism behind postoperative cholangitis is the formation of a stricture at the site of anastomosis or residual debris present in dilated intrahepatic ducts that could cause bile stasis, cholelithiasis, and cholangitis. • To avoid these complications, choledochoscopy is performed during surgery to reduce the risk of cholelithiasis and cholangitis by removing intrahepatic stones. • To reduce the risk of stricture formation postoperatively, it is recommended during surgery to do a wide anastomosis to allow free and smooth drainage of bile into the bowel. StatPearls

Answer 38

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

Answer 39

C During the course of normal biliary development the epithelial ductal plates induce vasculogenesis in the portal mesenchyme resulting in development of hepatic artery branches. These structures in turn influence development of the embryonic bile ducts. Arteriopathy resulting in abnormal vascular-ductal interactions is a possible mechanism in the development of biliary atresia and the focus of renewed interest. other choices are possible aetiologies of choledochal cyst. SPSE 1

Answer 40

D The classic triad of jaundice, pain and right hypochondrial mass is presented in less than 10% of patients. Infants typically present with obstructive jaundice, acholic stools with or without vomiting and an abdominal mass or hepatomegaly. Fever and failure to thrive may be noted. Recurrent abdominal pain is the dominant presenting feature in older children with or without intermittent jaundice. occasionally signs of hepatic fibrosis are present even in young patients. The degree of histological damage and the rate of epithelial metaplasia and dysplasia is related to the age of the patient, and malignancy risk mainly affect adults. other choices are true. SPSE 1

Answer 41

A The common hepatic duct and the cystic duct form the base and inferior margin of Calot’s triangle. The superior border was formerly the cystic artery; however, the inferior surface of the right hemiliver is accepted as a better working definition. Dissection of this area, a crucial part of cholecystectomy, endangers the structures mentioned. The structures within the hepatoduodenal ligament are the common bile duct, hepatic artery and portal vein. The right and left hepatic arteries usually lie posterior to the bile ducts at the liver hilum. The common bile duct contains two main arteries running longitudinally at the medial and lateral aspects of the duct. Intramural resection of the posterior wall of the cyst may help to avoid damage to the portal vein and hepatic arteries if there is dense inflammation or portal hypertension. SPSE 1

Answer 42

B Even in the presence of pancreaticobiliary malunion, hyperamylasaemia is not found in infants with choledochal cyst. This is due to pancreatic immaturity and only reaches significant levels at about 1–2 years of age. However, biliary concentrations of pancreatic lipase, elastase and trypsin are significantly elevated in infants with a common channel. other choices are true. SPSE 1

Answer 43

E In infants, choledochal cyst needs to be distinguished from cystic varieties of biliary atresia and other intra-abdominal cysts. ovarian cysts are usually easily distinguishable by their location in the pelvis. In the rare instance that ovarian cysts reach the size that allows them to extend into the subhepatic space, clinical details allow differentiation from choledochal cysts. SPSE 1

Answer 44

A Complications of choledochal cyst can occur at multiple levels from: ● pancreas – acute/chronic pancreatitis, pancreatolithiasis ● extrahepatic bile ducts – obstructive jaundice, cholangitis, cholelithiasis, cyst rupture and malignant transformation ● intrahepatic – biliary cirrhosis/hepatic fibrosis, portal hypertension, and liver abscess. Sclerosing cholangitis is a chronic cholestatic liver disease, characterised by progressive inflammation and fibrosis of the bile ducts resulting in biliary cirrhosis and is associated with a high risk of cholangiocarcinoma. The majority of patients are young, male and have coexisting inflammatory bowel disease. SPSE 1

Answer 45

E In more than 75% of cases, there is an anomalous junction between the distal common bile duct and the pancreatic duct, whereby they unite outside the duodenal wall proximal to the ampulla of Vater creating a long common channel. Consequently, pancreatic juice refluxes into the biliary tree producing chronic inflammation and is believed to be a potential cause of dilatation of the wall. Hepatic fibrosis may develop rapidly in prenatally detected cases with obstruction and is known to be reversible with surgical repair. occasionally, in older patients, mild periportal fibrosis can be noted. obstruction of the distal common bile duct has been a popular aetiologic theory for many years. Bile sludge may gather at the site of the stenosis or, less commonly, small stones. Whether the obstruction is congenital from a developmentally abnormal and stenotic bile duct or secondary to inflammation is not always clear. It may be difficult to differentiate congenital bile duct dilatation from dilatation secondary to an obstruction in cases where the dilatation of the duct is minimal or there is an absence of an anomalous junction. ‘Forme fruste’ is a term applied to cases believed to represent a choledochal cyst that has not yet reached a size that is easily detectable through ultrasonography and may require more invasive imaging such as magnetic resonance cholangiopancreatography or endoscopic retrograde cholangio-pancreatography (ERCP) to detect subtle changes. Rarely, choledochal cyst can also be associated with ectopic and multiseptate gallbladder. SPSE 1

Answer 46

C ultrasonography is the initial investigation of choice not only to demonstrate the details of the cyst but also the vascular anatomy and hepatic echotexture as well. Stones or sludge in the ducts or extension of the cyst into the liver may be noted. Assessment of biliary anomalies in the liver may also be noted. ERCP and percutaneous transhepatic cholangiography give excellent visualisation of the duct anatomy. However, both invasive techniques carry a risk of iatrogenic pancreatitis and biliary sepsis, and should be avoided during and immediately after episodes of acute pancreatitis. Intraoperative cholangiography by injecting into the cyst itself is inefficient and awkward and may fail to fill the intrahepatic ducts. It is best performed through the cystic duct and may require large volumes of contrast to fill out the distal duct including the pancreatic duct and the ducts inside the liver. CT with intravenous and oral contrast may be used in patients in whom the ultrasound does not provide sufficiently detailed imaging, the cyst is not very dilated, or where an additional problem is suspected. In most patients a detailed ultrasonography supplemented by intraoperative cholangiography provides sufficient information. Hepatobiliary scintigraphy may be used after surgery to evaluate secretion of bile into the liver but serves almost no purpose in the preoperative workup. SPSE 1

Answer 47

B The optimum treatment for most types of choledochal cyst is complete cyst excision to the upper limit of the dilated portion of the common hepatic duct portion of the cyst and reconstruction by hepaticoenterostomy. Care must be taken not to injure any hepatic ducts that enter the cyst in an anomalous location, typically the posterior sectoral duct of the right lobe. Cholecystectomy should always be performed since the cystic duct is almost always involved in the cystic dilatation and empties into the cyst itself. Cystenterostomy is unacceptable due to the risk of long-term complications including cholangitis, cholelithiasis, pancreatolithiasis, anastomotic stricture, biliary cirrhosis and malignancy. Thus cyst excision, cholecystectomy and Roux-en-Y hepaticojejunostomy would be the treatment of choice and patients treated previously by cystenterostomy should undergo revisional surgery. Hepaticoduodenostomy may be an alternative technique, although it carries a risk of bilious gastric reflux, gastritis and oesophagitis; ulceration and malignant transformation such as hilar adenocarcinoma has been reported. Transduodenal cyst excision is the technique used for large cysts of type III choledochoceles. Excision of the mucosa only is a technique that has been used when the cyst wall is extremely inflamed and very adherent to the underlying portal vein, but should not be utilised except under extreme circumstances. SPSE 1

Answer 48

A Progressive enlargement of a fetal choledochal cyst with a normal or distended gallbladder or hepatic ducts, or dilatation of the intrahepatic bile ducts demonstrated during pregnancy, favours choledochal pathology. Biliary atresia is not diagnosed prenatally and is rarely associated with large cysts in the hilum of the liver. In biliary atresia, the gallbladder is not typically enlarged and may be undetectable by ultrasound. Hepatic fibrosis may develop rapidly in prenatally detected cases and is known to be reversible with surgical repair. The optimal timing for surgical treatment of prenatally diagnosed choledochal cyst in infants who are otherwise well, is 3 months of age when the baby has established normal feeding patterns, has grown in size and has developed an established pulmonary circulation with resolution of fetal circulatory patterns. The results of surgical treatment at this age are generally excellent. SPSE 1

Answer 49

D The distal common bile duct is divided just outside the pancreas and oversewn with absorbable suture. It is not followed down through the pancreas, to avoid injury to the pancreatic duct. operative cholangiography is always advisable since it demonstrates the pancreaticobiliary junction, the anatomy of the intrahepatic ducts and provides a guide to the distal level of bile duct transection necessary. It also shows the position of the individual hepatic ducts in relation to the hepatic end of the cyst where the cyst must be transected without injuring anomalous hepatic ducts. The common hepatic duct is transected at the level of the bifurcation and cholecystectomy should always be performed despite the cystic duct being normal in size. Primary external T-tube drainage is helpful in patients with cyst rupture or uncontrolled cholangitis where cyst excision must be performed subsequently. SPSE 1

Answer 50

B Cholangiocarcinoma is the type of cancer that develops in patients with poorly drained or untreated choledochal cysts. long-term follow-up is indicated in all patients because of the sporadic reports of malignant degeneration in bile ducts near the anastomosis or even in ducts located in the liver. ultrasound of the liver and pancreas should be done annually or when necessary if patients become symptomatic particularly in those who had intrahepatic ductal dilatation preoperatively. malignancy may affect residual extrahepatic or dilated intrahepatic ducts or residual intrahepatic cysts, and is more likely with associated chronic inflammation or bile stasis. Some surgeons advocate hepaticoduodenostomy in preference to hepaticojejunostomy because of a more physiological outcome, associated with a smaller risk of adhesive small bowel obstruction and late anastomotic stricture. However, good results can be achieved with hepaticojejunostomy provided that a wide anastomosis at the level of the common hepatic duct bifurcation is constructed. Hepaticoduodenostomy has been reported to have a higher risk of bile reflux gastritis. Pancreatitis is rare after cyst excision and Roux-en-Y hepaticojejunostomy, although it may develop many years after cyst excision in patients with a dilated or complex common channel containing protein plugs or calculi, or in those with a substantial segment of retained terminal common bile duct. Cholangitis may have preceding causes including anastomotic stricture, bile duct stricture, calculi or Roux-loop obstruction. Type IVa cysts are particularly prone to developing intrahepatic calculi. Thus appropriate follow-up with standard imaging methods is recommended to detect and correct these complications early. liver transplantation is necessary if cholestasis develops in patients with type V cysts in the liver because of the development of biliary cirrhosis in whom no drainage procedure can be done to improve biliary stasis. SPSE 1

Answer 51

C Primary cholelithiasis is now the most common aetiology of gallbladder disease in the paediatric population. TPN use is commonly reported in association with cholelithiasis and contributes to the incidence of primary cholelithiasis. Haemolytic cholelithiasis secondary to disorders such as sickle-cell disease, spherocytosis, thalassaemia, and so forth. is the second most common cause of gallstones. Congenital abnormalities of the biliary tract are rarely associated with gallbladder disease. Ileal resection does contribute to the formation of pigment stones, but is not by itself the most common cause of gallbladder disease. SPSE 1

Answer 52

E The incidence of cholelithiasis and resulting cholecystitis in the paediatric population has increased over the past 2 decades. This has been attributed to several factors including earlier pregnancy age, increased prevalence of child hood obesity, poor diet, increased use of prolonged TPN, and increased use of oral contraceptives. ultrasound has aided in the increased sensitivity of detection. SPSE 1

Answer 53

A Hydrops of the gallbladder presents in conditions such as septic shock, severe diarrhoea and dehydration, hepatitis, Kawasaki’s disease, and mesenteric adenitis. A palpable mass may be felt on physical exam. The gallbladder becomes acutely distended and develops wall oedema. most cases will resolve spontaneously with supportive care. Antibiotics and cholecystectomy may become necessary if supportive care fails or cholecystitis develops. SPSE 1

Answer 54

B This clinical scenario is consistent with biliary dyskinesia secondary to gallbladder dysmotility. laboratory workup and ultrasound are typically unremarkable. The most appropriate next step is to order a HIDA scan. Studies have shown that using an ejection fraction less than 35% for a diagnosis of biliary dyskinesia and subsequent cholecystectomy, will achieve 80% or better success rates. ERCP and CT would be expected to be negative and would not be helpful in this workup. Expectant management may be appropriate pending the HIDA results, but would not be the appropriate next step in this case. SPSE 1

Answer 55

A This question demonstrates straightforward indications to perform an intraoperative cholangiogram. A dilated common bile duct, history of jaundice, history of pancreatitis, and elevated liver function tests and GGT are all preoperative findings that may represent choledocholithiasis. unclear anatomy during a cholecystectomy may lead to iatrogenic damage to the common bile duct. Intraoperative cholangiogram should be performed in all of the above situations. Acalculous cholecystitis is not an indication to perform an intraoperative cholangiogram. SPSE 1

Answer 56

E The clinical scenario is an example of acalculous cholecystitis that develops as a result of an extensive bowel resection and prolonged TPN use. Prolonged TPN leads to decreased gallbladder contractibility, distension and stasis. These changes lead to an increased risk of infection and cholecystitis. The ultrasound findings of gallbladder-wall oedema, pericholecystic fluid, and sludge are all consistent with acalculous cholecystitis. After workup is completed for other causes for sepsis, cholecystectomy should be performed. A HIDA scan would not be needed after an ultrasound demonstrating findings consistent with acalculous cholecystitis. Antibiotics are an appropriate next step, but cholecystectomy should follow. Stopping the TPN and a trial of enteral nutrition is not appropriate in the setting of acute cholecystitis. SPSE 1

Answer 57

A In this question stem a cystic mass is diagnosed in the porta hepatis. The differential diagnosis includes choledochal cyst, cholangiocarcinoma, diverticulum of the biliary tree, other biliary anomalies and duodenal duplication and so forth. The ultrasound, while discovering the mass, cannot give a definitive diagnosis and does not give clear information regarding the association of the mass with the biliary tree. ERCP will be the best choice to give this information and is certainly less invasive than an operative diagnosis. Repeat ultrasound would not give additional information. Endoscopic ultrasound also will not give much additional information leading to a diagnosis. likewise a HIDA scan would not be specific for any of the above conditions. SPSE 1

Answer 58

D Anomalies of the gallbladder and cystic duct are rare causes of gallbladder disease. These anomalies are usually diagnosed incidentally. Reassurance and education is the next most appropriate step in management. Cholecystectomy is not necessary without the presence of coexisting gallbladder disease. No further workup is needed for asymptomatic anomalies and additional imaging would not add more information. SPSE 1

Answer 59

B The most common types of gallstones are cholesterol stones, especially in children without haematological diseases. Cholesterol stones are seen with increasing age, obesity and poor diet, oral contraceptive use and pregnancy. Approximately 10%–20% of gallstones are composed of calcium bilirubinate and are classified as pigment stones. These stones are mostly seen in patients with decreased gallbladder contractility, prolonged TPN use, history of significant ileal resection, or a chronic haemolytic state, e.g. hereditary spherocytosis and sickle-cell disease. SPSE 1

Answer 60

C Cholelithiasis occurs in 43%–63% of patients with hereditary spherocytosis. ultrasound is therefore recommended before splenectomy. If cholelithiasis is documented, cholecystectomy should be performed at the time of splenectomy. Cholelithiasis is present in 10%–55% of patients with sickle-cell disease; however, cholecystectomy is not recommended unless the patient is symptomatic. Cholecystectomy should be performed electively and not during a haemolytic crisis. An ejection fraction of less than 35% is used most commonly to aid in the diagnosis of biliary dyskinesia. An ejection fraction of 42% should be considered normal and cholecystectomy should not be recommended based on the information given. Although an ileal resection can predispose to the development of gallbladder disease, a limited resection for meckel’s diverticulum should not. A cystic duct diverticulum is a rare anomaly of the biliary tree. The presence of this anomaly in an asymptomatic patient is not an indication for cholecystectomy. SPSE 1

Answer 61

E There is currently no evidence to suggest that leaving a drain after cholecystectomy improves outcomes or prevents morbidity. Drain use should be selective and based on generally appropriate surgical considerations. The remainder of the answers are all principles of a laparoscopic cholecystectomy. SPSE 1

Answer 62

B ERCP is effective in the paediatric population with documented success rates of 90% and greater by experienced practitioners. These success rates are similar to that in the adult population. Complication rates are similar or lower than those in the adult groups. Complications generally involve pancreatitis, haemorrhage, perforation and infection. Pancreatitis is the most common of these complications with a rate probably in the range of 3%–7%. most of these cases of pancreatitis are mild. There is currently no age cutoff for the performance of ERCP. Success has been obtained safely in patients as young as 1 year of age. A history of pancreatitis is not a contraindication to ERCP; in fact, ERCP is a diagnostic tool for the evaluation of chronic or recurrent pancreatitis. SPSE 1

Answer 63

D This question tests management of iatrogenic injury to the common bile duct during cholecystectomy. limited injuries to the lateral aspect of the common bile duct can be repaired primarily with or without the use of a T-tube. Injuries involving greater than 50% of the duct diameter or involving electrocautery injury should be repaired with choledochojejunostomy or choledochoduodenoscopy, depending on the location of the injury. Repair of these more extensive injuries primarily leads to the development of significant biliary stricture and secondary morbidity. SPSE 1

Answer 64

E There are 5 types. Type I consists of a cystic dilation of the common bile duct with normal size right and left hepatic ducts and a normal gallbladder entering the cyst. This is the most common type, making up 85–95 percent of the cases. Type II cysts are diverticular outpouchings from the common bile duct. Type III cysts are choledochocele, dilations of the distal common bile duct located either in the duodenal wall or the head of the pancreas. Type IV choledochal cysts are similar in appearance to the extrahepatic ducts of the type I choledochal cysts with the addition of intrahepatic biliary duct dilation. Type V choledochal cysts or Caroli’s disease, consists of a normal extrahepatic biliary duct system with dilated intrahepatic bile ducts. Syed/MCQ

Answer 65

D All of the above are true. Syed/MCQ

Answer 66

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. Syed/MCQ

Answer 67

C Type I is the commonest variety, so for cyst excision with Roux-en-Y, hepaticojejunostomy is the commonest procedure. Residual cyst can develop malignant changes. Syed/MCQ

Answer 68

A Pancreatitis is more common after choledochocele (type III) surgery. For choledochocele surgery, a longitudinal duodenal incision required to expose the intraduodenal choledochocele. Unroofing of cyst and mucosa is reapproximated. Bile duct and pancreatic duct must be identified and calibrated. If opening is narrow, sphinteroplasty should be done. Rarely cyst located in the head of pancreas that are not amenable to internal drainage into the duodenum. For that, pancreaticoduodenectomy may be required. Syed/MCQ

Answer 69

E The histological type adenocarcinoma can occur. Syed/MCQ

Answer 70

Several factors are known to cause cholelithiasis in children. By far, the most commonly reported condition associated with the development of nonhemolytic cholelithiasis, particularly in neonates and infants, is the use of total parenteral nutrition (TPN). Up to 43% of children receiving long-term TPN develop gallstones. The mechanism of gallstone formation related to TPN remains unclear but may be associated with changes in bile composition caused by the amino acid infusion. Also, the lack of enteral feeding leads to ineffective gallbladder contraction and results in reduced enterohepatic circulation of bile. The administration of fat is believed to ameliorate the deleterious effects of the amino acids in the TPN. Because many infants receive TPN and only a few develop gallstones, other factors are probably necessary for the development of cholelithiasis in this patient population. Septicemia, dehydration, chronic furosemide therapy, cystic fibrosis, short-bowel syndrome, and ileal resection for necrotizing enterocolitis also may be important contributing factors. The cause of gallstone formation is probably multifactorial. Stasis, sepsis, a lack of enteral feedings, and some alteration of the enterohepatic pathway for the recirculation of bile are all important for stone development. Moreover, neonates and, particularly, premature infants are susceptible to the cholestatic effects of TPN because of the immaturity of their enterohepatic circulation of bile salts. Finally, the composition of gallstones in children may be different than that found in adults. Whereas stones in adults are primarily cholesterol in composition, calcium carbonate and black pigment stones are often found in pediatric patients, especially those younger than 10 years of age. Causes associated with gallstones in older children include the use of oral contraceptives, cystic fibrosis, pregnancy, obesity, and ileal resection. Cholelithiasis has also been reported in children undergoing cardiac transplantation who are receiving cyclosporine and in patients who have previously required extracorporeal membrane oxygenation as a newborn. Coran

Answer 71

Patients with SCD represent the largest group of patients at risk for postoperative complications after cholecystectomy. In a report of 364 cases from a national sickle cell disease study group, the total complication rate was 39%, with sickle cell events representing 19%; intraoperative or recovery room problems, 11%; transfusion complications, 10%; postoperative surgical events, 4%; and death, 1%. The open operation was performed in 58% of the patients, and the laparoscopic route was used in 42%. The complication rates were similar between the two groups. From that study, it was believed that the incidence of sickle cell events may be higher in patients who were not preoperatively transfused. Moreover, the laparoscopic approach is now recommended for this patient population. The acute chest syndrome can be seen in up to 20% of sickle cell patients undergoing abdominal operation. The laparoscopic approach did not decrease the incidence of this complication. Meticulous attention to perioperative management, transfusion guidelines, and pulmonary care may reduce the incidence of the acute chest syndrome. Coran

Answer 72

The classic triad of abdominal pain, jaundice, and right upper quadrant mass is seen in only a minority of patients. The diagnosis should be suspected in children with chronic relapsing abdominal pain, recurrent pancreatitis, acute cholangitis without cholelithiasis, and cholelithiasis or biliary sludge without obvious risk factors. The latter group is particularly important, as a cholecystectomy without adequate imaging of the biliary tree represents the wrong intervention in these patients. Presentation with recurrent mild pancreatitis seems to be quite common in older children in whom the diagnosis is often delayed. However, even older children can present with giant cysts. The spectrum of presentation is therefore quite wide. Sherif

Answer 73

There are two main theories, “pancreaticobiliary maljunction” and “congenital stenosis of bile ducts”, proposed for the pathogenesis of CC. Pancreaticobiliary maljunction is a congenital malformation in which the pancreatic and bile ducts join outside the duodenal wall, forming an abnormally long common channel. In patients with pancreaticobiliary maljunction, the sphincter of Oddi fails to regulate the function of the pancreaticobiliary junction, resulting in a two-way regurgitation and a mixing of pancreatic juice and bile, which activates the pancreatic enzymes in the pancreatic juice, leading to bile duct damages and inflammation, which subsequently result in a dilation of the bile duct as seen in CC. However, pancreaticobiliary maljunction can only be identified in about 50–80% of patients with CC. Congenital stenosis of bile ducts is characterized by a reduced number of ganglions and neurons of the CBD, which causes a dysregulated contraction and increased intraluminal pressure in the proximal bile duct, leading to a cystic distention of the proximal CBD in CC. https://www.mdpi.com/2073-4425/13/6/1030 — CCs, which develop later in life, are considered “acquired.” The theory of the long common biliopancreatic channel proposed by Babbitt has been widely accepted to explain the formation of this type. Normally, the terminal CBD and pancreatic duct unite to form a short common channel, which is well surrounded by the Oddi sphincter. This normal anatomic arrangement prevents the reflux of pancreatic fluid into the bile duct. If this common channel is long and part of it is not surrounded by the normal sphincter, pancreatic secretions can reflux into the biliary tree. Proteolytic enzymes from the pancreatic fluid are activated and can cause epithelial and mural damage that leads to mural weakness and dilatation of the choledochus. This theory is supported by the fact that high concentrations of activated pancreatic amylase and/or lipase have been found in patients with CC and long pancreaticobiliary channels. In an experimental study, CC was produced by creating a choledochopancreatic endto-side ductal anastomosis. In addition, a high incidence of a common channel has been detected in CC patients. Obstruction at the level of the pancreaticobiliary junction may be an associated causal factor in choledochal dilatation. An experimental model for the study of cystic dilatation of the extrahepatic biliary system has been produced by ligation of the distal end of the CBD in the newborn lamb. High biliary pressure in patients with CC have also been recorded during operative correction. In adults, an anomalous union of the pancreaticobiliary duct is defined when the common pancreaticobiliary channel is longer than 15 mm or when its extraduodenal portion is more than 6 mm. In a study of 264 infants and children undergoing endoscopic retrograde cholangiopancreatography (ERCP), the maximal length of the common channel was found to be 2.7 mm among children 3 years or younger, 4 mm among children 4–9 years old, and 5 mm between 10 and 15 years old. A genetic basis for CC is suspected because its female predominance and different prevalence among countries in Asia and Europe versus United States. Using “trio-based” exome-sequencing of 31 patients with CC, Wong et al. recently reported 13 genes that were recurrently mutated at different sites. H&A