Hepatobiliary Flashcards
A 66-year-old Japanese female is referred by her
primary physician for long-standing biliary colic symptoms. She describes 10 to 15 years of intermittent right upper quadrant (RUQ) pain with nausea that typically resolves after 1 to 2 hours.
She went to the emergency room once 6 years ago and had an ultrasound that showed gallstones. Her medical history is significant for hypertension and osteoporosis. Her vitals and exam are unremarkable.
A repeat RUQ ultrasound ordered by her primary care physician now shows a large 3 cm gallstone, As well as a fixed mass in the fundus, 2 cm in diameter, that appears to originate from the gallbladder wall. The immediate surrounding gallbladder wall is thickened to 8 to 11 mm.
A CBC, basic chemistry, and liver function tests are all within normal limits.
Which of the following is a risk factor for gallbladder carcinoma?
A. Hemolytic anemia
B. Biliary dyskinesia
C. Clonorchis sinensis infection
D. Anomalous union of the pancreaticobiliary ductal
system or pancreaticobiliary maljunction (PBM)
E. Auto-immune diseases
D.
A history of gallstones is common, and 65% to
90% of those with biliary carcinoma, have a history
of gallstones. The relation of gallstones to gallbladder
cancer (GBC) is thought to be mediated by chronic
inflammation.
There is a relatively well-defined sequence of flat-epithelial premalignant changes leading to GBC. Chronic inflammation leads to intermediate low-grade dysplastic changes. Dysplastic progression over time leads to carcinoma-in-situ and finally invasive carcinoma.
Anomalous union of the pancreaticobiliary ductal system, where the pancreatic duct and common bile duct merge outside the wall of the duodenum and form a long common channel, is also associated with an increased risk of GBC. This pancreaticobiliary maljunction (PBM)
leads to chronic reflux of pancreatic enzymes.
The progression to GBC is likely mediated through
an epithelial hyperplasia with resultant papillary or
villous epithelial changes progressing to GBC.
Further evidence that this is a distinct pathway from
chronic inflammation is that the gene alterations of
cancers arising in the setting of cholelithiasis differ
from anomalies of the duct system associated cancers.
Adenomas do occur in the gallbladder and can
progress to cancer. However, this likely occurs much
less commonly than the other two pathways, given
a lack of cancer-related molecular changes in most
of these lesions.
Inflammatory bowel disease is also associated with increased risk for gallbladder carcinoma. Clonorchis infection is associated with risk for cholangiocarcinoma, but it has not been linked to carcinoma of the gallbladder.
Chronic Salmonella typhi or paratyphi infection is, however, associated with increased risk for gallbladder cancer.
Hemolytic anemia may be a cause of bilirubin type gallstones but is not a risk factor for gallbladder cancer, neither is biliary dyskinesia or auto-immune diseases.
Which of the following radiographic findings is associated with the highest incidence of gallbladder carcinoma?
A. Pancreaticobiliary maljunction (PBM) without biliary dilatation identified on magnetic resonance cholangiopancreatography (MRCP).
B. Strongly enhancing thick inner layer and a weakly enhancing or non-enhancing outer layer of the gallbladder wall on the portal phase of a multi-detector CT scan (MDCT).
C. Gallbladder polyp 10-20mm on ultrasound.
D. Gallbladder wall calcifications; “porcelain gallbladder”
E. Asymptomatic gallstone greater than 3 cm in size.
B.
A recent retrospective study of findings on MDCT associated with gallbladder cancer found two patterns most associated with finding malignancy at the time of surgery.
A strongly enhancing thick inner layer and a weakly enhancing or non-enhancing outer layer of the gallbladder wall on multi-detector CT scan was shown to have a 52% to 55% incidence for gallbladder cancer.
A single thick layer with heterogenous enhancement on MDCT had an incidence of 35% to 38% for gallbladder cancer.
Pancreaticobiliary malformation (PBM) without bile duct dilatation have an incidence of biliary tract cancer of 37.9%, of which 93.2% of these were gallbladder cancer.
Based on this data, prophylactic cholecystectomy is recommended for these patients.
The incidence rates of malignancy in gallbladder polyps varies widely in published reports but ranges from 9.6% to 40% for polyps 10 to 20 mm.
The wide variance is related to various imaging modalities used and populations studied.
Polyps >10mm, sessile polyps and rapidly growing polyps are all recommendations for gallbladder removal.
The finding of gallbladder calcifications or a porcelain gallbladder was found to be associated with a malignancy in 6% of cases in a recent systematic literature review. This is a far lower number than the historically quoted figure of approximately 25%.
Attempts in the review were made to limit inherent biases in a review of retrospective studies that favor overestimation but this value likely still overestimates the true incidence.
Given the incidence, the decision to perform a prophylactic cholecystectomy should not be absolute and should be weighed against the risks of surgery for the individual patient. The presence of gallstones is associated with an increased risk of gallbladder cancer.
The size and volume of stone burden have been identified as potential risk factors for developing gallbladder cancer.
However, there is no direct evidence of a causal relationship between gallstones and gallbladder cancer. The risk for gallbladder cancer development with a 3 cm or greater stone has been estimated to be a 2% risk over a 20 year period.
Review of all imaging studies shows a gallbladder tumor invades into the muscularis propria. There is no lymphadenopathy or distant metastases seen. What is the most appropriate next step in management?
A. Referral for definitive treatment with chemotherapy and radiation.
B. Referral for neoadjuvant chemotherapy and radiation.
C. Schedule for cholecystectomy.
D. Schedule for cholecystectomy with removal of
regional lymph nodes and en-bloc hepatic resection.
D.
The patient has a T1b lesion or early gallbladder
cancer.
Surgical resection is the only curative therapy
for gallbladder cancer.
The general consensus is for radical cholecystectomy for T1b or greater lesions. T1b tumors have been shown to have lymph node metastasis in 24% of cases. Lower recurrence rates and improved survival have been observed with radical resection including lymph nodes when compared to simple cholecystectomy.
A review of the SEER database showed that the evaluation of even a single lymph node improved overall survival and that radical resection without lymph node assessment was no better than cholecystectomy alone for early stage gallbladder cancer.
Neoadjuvant therapy has been evaluated in the setting of borderline and unresectable extra-hepatic biliary malignancies with good results in survival and obtaining negative margins but none of these addressed gallbladder cancer specifically and were confined to advanced disease.
Adjuvant chemoradiation does have a role in select
gallbladder cancer patients, especially with positive nodes or margins to improve local control.
The same patient with the same history of present
illness and past medical history instead presents
to the emergency room with RUQ pain.
The ultrasound in this case is read as 2 large gallstones
with diffuse gallbladder wall thickening up to 11 mm, pericholecystic fluid, and a normal common bile duct. Labs show white blood cell count of 13 and normal liver function. You take the patient for laparoscopic cholecystectomy.
There was no concern for malignancy during the
procedure. The gallbladder was removed without
spillage and with a retrieval bag. You see her 2 weeks
later in clinic, and review of the pathology report
shows T2 adenocarcinoma.
All surgical margins, including cystic duct margin, are reported as clear. What is the most appropriate course of action?
A. No additional surgery, surveillance with imaging
every 6 months.
B. Staging with imaging followed by radical cholecystectomy to include: liver resection with at
least 3 cm of margin around gallbladder bed and regional lymphadenectomy.
C. Staging with imaging followed by radical cholecystectomy and in addition, excision of the
previous laparoscopic port sites.
D. Staging with imaging followed by radical cholecystectomy and in addition, excision of the
common bile duct.
E. Staging with imaging followed by radical cholecystectomy and in addition, excision of both the
common bile duct and the laparoscopic port sites.
B.
Prognosis of gallbladder carcinoma is determined
by the depth of tumor infiltration and the ability to
obtain a tumor-free resection margin (R0).
For T2 or greater the definitive resection should include a minimal hepatic resection centered on the gallbladder
bed and a regional lymphadenectomy.
The majority of experts also extend this recommendation to T1b tumors but some controversy persists. There is uniform agreement that cholecystectomy alone is sufficient for Tis and T1a tumors. There is also general
agreement regarding several other technical points, such as resection of the common bile duct, and the need for port site excision. Excision of the common bile duct is only necessary for a positive cystic duct margin or direct invasion of the hepatoduodenal ligament.
Regarding routine common bile duct excision with radical cholecystectomy unless there is direct invasion of the hepatoduodenal ligament and/or of the cystic duct, bile duct resection does not result in decreased recurrence or better overall survival and does not increase the number of nodes in the specimen.
Peritoneal involvement with gallbladder cancer is common and there is theoretical adverse impact on this with pneumoperitoneum. However, the risk of port site recurrence is based on perforation of the gallbladder or extraction without a retrieval bag rather than the pneumoperitoneum.
Port-site excision does not improve overall or disease-free survival in large retrospective series. Port site excision does not need be routinely performed during secondary procedures for gallbladder cancer discovered
after laparoscopic cholecystectomy.
Which of the following is true regarding the surgical management for T2 or T3 gallbladder cancers?
A. Formal segmentectomy (4b + 5) improves overall survival over wedge resection.
B. Formal segmentectomy reduces local recurrence
rates compared to wedge resection, but overall survival is the same.
C. Formal segementetomy improves disease free survival, but not overall survival.
D. A clear survival benefit for formal segmentectomy
over wedge resection has not been demonstrated.
D.
Although some studies have reported anatomical resection improves survival and RO resection rate when compared to wedge resection, other reports have not demonstrated a benefit.
The majority of these studies dealt with liver resection for liver metastases. There are a few studies looking at gallbladder cancer specifically.
Pawlik et al. found that patients who underwent a major hepatic resection (e.g., formal segmentectomy of 4b + 5 or hemi-hepatectomy) had a similar risk of disease-specific death compared with patients who underwent a hepatic wedge resection. Horiguchi et al. found the overall survival rate and disease-free survival rate at 5 years did not differ significantly between wedge resection and 4a + 5 resection group for T2 tumors.
The available evidence in gallbladder cancer does not show a clear benefit to anatomic resection.
As such, the surgeon’s goal should be to resect all disease with negative histologic margins and chose the appropriate operation to achieve this with the fewest complications.
A 67-year-old female presents with sharp, burning right upper quadrant abdominal pain that radiates to her back. The pain awakens her from sleep. She has experienced nausea and three episodes of emesis. She reports burning substernal and epigastric pain. She denies any fevers or chills. She is hemodynamically normal and afebrile. Laboratory workup reveals a normal hepatic function panel, a normal basic chemistry, and a normal white blood cell count. Her abdominal exam is remarkable for mild tenderness in the right upper quadrant without peritoneal signs. After three hours, her pain resolves.
- What is the next best test in this patient scenario?
A. Plain films of the abdomen
B. Cholecystokinin stimulated cholescintigraphy
C. Right upper quadrant ultrasound
D. Computed tomography (CT) of the abdomen
E. Esophagogastroduodenoscopy (EGD)
- The appropriate imaging is ordered and shows no abnormality. Which is the next most appropriate
step in management?
A. Cholecystectomy
B. Esophagogastroduodenoscopy
C. Right upper quadrant ultrasound in two weeks
D. Proton pump inhibitor administration
E. Bile microscopy
1) C. This scenario describes symptomatic cholelithiasis, or biliary colic.
Plain films of the abdomen would not be a high yield study as less than 10% of gallstones are radio-opaque, nor would it offer specific imaging of the biliary system. Other yields from a plain film would be presence of nephrolithiasis or free air (Choice A).
A hepatobiliary (HIDA) scan is the gold standard for the diagnosis of acute cholecystitis—a clinical scenario in which she does not fit criteria (Choice B). With the cholecystokinin injection, the nuclear imaging study can be diagnostic for biliary dyskinesia.
CT can often visualize gallstones but is not the recommended imaging modality of choice for cholelithiasis as the sensitivity is 55% to 80% (Choice D); cholelithiasis is often found incidentally on CT scan when the imaging is performed for other reasons.
Esophagogastroduodenoscopy could be helpful to rule out peptic ulcer disease that can sometimes mimic symptoms of biliary colic, but this test would not be the ideal next choice in this situation (Choice E).
The most common initial imaging modality in biliary disease is the right upper quadrant ultrasound. Ultrasound has a sensitivity of 84% and specificity approaching 99% for the diagnosis of cholelithiasis (Choice C). Simultaneously, assessment for signs of acute cholecystitis, choledocholithiasis, and hepatic pathology can be sought.
2) C.
This patient should have had a right upper quadrant ultrasound performed in the first question above. If this imaging modality shows no abnormality, but the patient has biliary symptoms, she should have a repeat right upper quadrant ultrasound performed in two weeks with focus on areas that may have missed stones smaller than 3 mm (Choice C).
Esophagogastroduodenoscopy is an appropriate diagnostic modality to rule out peptic ulcer disease and can be utilized if the repeat ultrasound is still negative. Bile microscopy has been shown as an adjunct study to assess for microlithiasis and can be helpful (Choice E), although is not routinely performed; bile microscopy also requires endoscopy for collection. The sensitivity of bile microscopy for microlithiasis is 65% to 90%.
Cholecystectomy is not recommended at this time as other causes of the patient’s symptoms have not been ruled out. Cholecystokinin stimulated cholescintigraphy can help identify a functional gallbladder problem that
may warrant cholecystectomy if positive (Choice A).
Proton pump inhibitor therapy may help with reflux
symptoms but does not specifically address biliary
symptoms (Choice D).
A 67-year-old female presents with sharp, burning right upper quadrant abdominal pain that radiates to her back. The pain awakens her from sleep. She has experienced nausea and three episodes of emesis. She reports burning substernal and epigastric pain. She denies any
fevers or chills. She is hemodynamically normal and afebrile. Laboratory workup reveals a normal hepatic function panel, a normal basic chemistry, and a normal white blood cell count. Her abdominal exam is remarkable for mild tenderness in the right upper quadrant without peritoneal signs. After three hours, her pain resolves.
The patient returns to the emergency department one month later with similar symptoms. A right upper quadrant ultrasound is performed that shows gallstones without signs of acute cholecystitis. Her pain again resolves and she is referred to your clinic the following week. You discuss performing a cholecystectomy with the patient, which she refuses. Which of the following
is true regarding medical therapy of cholelithiasis?
A. Ursodiol therapy typically resolves biliary symptoms within one month.
B. Complete resolution of cholelithiasis is successful in less than 40% of patients.
C. If biliary symptoms persist while on ursodiol, cholecystectomy is indicated.
D. The mechanism of action of ursodiol includes reduction in bile synthesis.
E. Extracorporeal shockwave lithotripsy (ESWL)
has a symptomatic recurrence rate of 30%.
B.
Ursodiol may take up to 3 months to show
improvements in biliary symptoms, and can takeup to
3 years to completely dissolve gallstones (Choice A).
A meta-analysis of treatment with ursodiol showed
that only 37% of patients had complete resolution
of biliary symptoms; cholecystectomy remains the
preferred choice in surgical candidates (Choice B).
Many patients who undergo initial medical therapy
with ursodiol do progress to surgical treatment, but
they tend to show initial improvement in symptoms.
If biliary symptoms progress while being treated
with ursodiol, other causes must be ruled out, such
as sphincter of Oddi dysfunction or peptic ulcer
disease (Choice C).
The mechanism of action of ursodiol involves reducing absorption in the duodenum, resulting in disruption of micelles and reduced cholesterol absorption, thereby decreasing cholesterol concentration; ursodiol is not involved with the synthesis of bile (Choice D).
Extracorporeal shockwave lithotripsy can be utilized as nonsurgical therapy, but has a recurrence rate of approximately 20%.
Indications include patients with single stones, between the sizes of 5 mm and 2 cm.
ESWL is not commonly offered as medical therapy due to the efficacy and commonplace practice of cholecystectomy (Choice E).
A 67-year-old female presents with sharp, burning right upper quadrant abdominal pain that radiates to her back. The pain awakens her from sleep. She has experienced nausea and three episodes of emesis. She reports burning substernal and epigastric pain. She denies any fevers or chills. She is hemodynamically normal and afebrile. Laboratory workup reveals a normal hepatic function panel, a normal basic chemistry, and a normal white blood cell count. Her abdominal exam is remarkable for mild tenderness in the right upper quadrant without peritoneal signs. After three hours, her pain resolves.
The patient’s ultrasound, in addition to cholelithiasis, is also notable for a 1.5 cm non-mobile polypoid lesion in the fundus of the gallbladder. Which of the following is true?
A. Polyps greater than 5 mm are a risk factor for
gallbladder cancer.
B. Patients with large (> 2.5 cm) gallstones are more likely to develop gallbladder cancer than those without.
C. Laparoscopic cholecystectomy should be performed, a frozen analysis should be done, and if
positive, an oncologic resection should be done
laparoscopically.
D. Multiple pedunculated subcentimeter lesions are also a risk factor for gallbladder adenocarcinoma.
E. Extended cholecystectomy is not required for gallbladder adenocarcinomas with up to T3 lesions.
B.
This patient should undergo open cholecystectomy with a symptomatic, visualized preoperative intraluminal gallbladder lesion that is 1.5 cm in size.
The open cholecystectomy is preferred, because
gallbladder perforation or bile spillage during laparoscopic cholecystectomy can potentially seed the peritoneal cavity.
Polypoid lesion size greater than 1 cm is an independent risk factor for gallbladder adenocarcinoma, along with stone size greater than 2.5 cm (Choice A, B).
Gallbladder cancer has a 3:1 ratio of incidence in women and typically presents after age 60.
Extended cholecystectomy is not indicated if the lesion is confined below the muscle layer of the gallbladder.
If the lesion is T2 or greater, resection usually includes segments IVb and V of the liver (Choice E).
Choice D is descriptive of cholesterol polyps, which are not a risk factor for gallbladder adenocarcinoma.
A 70-year-old male presents to the emergency department with altered mental status. Family reports he was complaining of right upper quadrant abdominal pain prior to becoming altered mentally. On arrival, he is found to have a temperature of 102.5°F, heart rate of 112, and systolic blood pressure of 80 despite 2 liters of crystalloid infusion. On exam, he is visibly jaundiced with tenderness in the right upper quadrant. He has 3 out of 4 systemic inflammatory response syndrome
(SIRS) criteria. He is started on pipericillin/tazobactam and admitted to the intensive care unit for invasive monitoring and vasopressor support.
- Regarding this patients constellation of symptoms, what is the most common cause?
A. Gallstones
B. Biliary stricture
C. Malignancy
D. Genetic disorder
A.
Cholangitis is caused by obstruction of the biliary tree eventually leading to bile stasis and bacterial infection. The most common cause being gallstones, which account for around half of cases.
Other causes include stenosis/biliary stricture, malignancy and biliary stents.
Stents can cause obstruction from migration, occlusion, or colonization by bacteria leading to bacterial overgrowth and translocation into the bloodstream.
Regarding the pathophysiology of cholangitis, which of the following is correct?
A. Increased biliary pressure leads to decrease in production of IgG in the biliary mucosa leading to increased translocation of duodenal bacteria.
B. Intra-portal toxins and bacteria can cross through the biliary system due to stasis leading to infection.
C. Stones do not colonize with bacteria.
D. Biliary stents are not felt to contribute to or cause
cholangitis as they help decompress the biliary tree.
B.
Obstruction of the biliary tree via stricture/stenosis, stones, malignancy, or stent occlusion leads to increased biliary tract pressure. This pressure promotes stasis of bile and decreases production of IgA in the bile tract mucosa.
The lack of continuous bile flow, coupled with decrease mucosal protection allows for bacterial translocation from the duodenum through the biliary tract. This static bile and gallstones provide a healthy growth medium for bacteria.
The elevated intra-biliary pressure allows for translocation of these pathogens into the systemic
circulation causing septicemia. Less commonly, bacteria and toxins can enter through the portal circulation into the bile due to increase biliary pressure.
The most common bacteria are gram negative enteric pathogens: E coli, klebsiella, and enterobacter. They carry LPS that promotes cytokine release and leads to septic shock. Enterococcus is seen in a smaller set of cases.
Regarding the proper diagnostic workup, which noninvasive test has highest sensitivity and specificity for detecting the most common cause?
A. Abdominal ultrasound
B. Magnetic resonance cholangiopancreatography (MRCP)
C. CT Scan
D. Hepatobiliary (HIDA) scan
B.
MRCP is the best non-invasive test to confirm the presence of choledocholithiasis due to its high sensitivity (some studies quoting 100%) and nearly 100% specificity.
If the test is positive then this confirms diagnosis. It also is helpful in evaluating for stricture and ampullary masses.
Ultrasound is a good screening tool and can evaluate for common bile duct dilatation to perhaps lend clinical suspicion to presence of biliary obstruction. It is best at identifying the presence of cholelithiasis/cholecystitis. However, it has lower accuracy in identifying the presence of a choledocholith, roughly 80%.
A CT scan is less useful than ultrasound in detecting cholecystitis/common bile duct dilatation, but is helpful at evaluating for ampullary masses as a cause of a dilated common bile duct.
HIDA scans are not useful in the setting of cholangitis as the biliary tract infection reduces secretion of the radio nucleotide labeled marker into the biliary tree. It may show, however, obstruction with lack of flow into the duodenum.
Regarding the management of septic cholangitis caused by choledocholithiasis, which of the following is correct?
A. Urgent biliary tract decompression via endoscopic retrograde cholangiopancreatography (ERCP) is successful 60% of the time.
B. Percutaneous transhepatic cholangiography (PTC) is feasible for stone extraction and stent placement.
C. ERCP with a sphincterotomy is equivalent to cholecystectomy for reducing recurrence rates.
D. Should ERCP and PTC fail or are not feasible, operative choledochotomy and T-tube placement should be avoided because of the risk of surgery.
E. Broad spectrum antibiotic therapy alone will generally provide adequate treatment.
B.
In cases of cholangitis without septic shock, a trial of antibiotic therapy is recommended as this can resolve symptoms and ensure stability in up to 80% of patients.
Routine ERCP can be performed in this setting assuming the patient remains stable.
This patient displays Reynold’s pentad of fever, right upper quadrant pain jaundice, altered mental status, and hypotension, the first three signs constituting Charcot’s triad. This lends suspicion to suppurative cholangitis due to the patient’s state of septic shock.
Antibiotics, though required as initial therapy, are unlikely to complete resolve this patient’s septic physiology. Emergent/urgent biliary tree decompression is warranted and must be performed to prevent excessive morbidity/mortality.
ERCP with sphincterotomy has shown upwards of a 95% success rate in stone extraction and decreasing the rate of recurrence of cholangitis. It is, however, not superior to cholecystectomy in decreasing rates of recurrence and thus cholecystectomy is recommended after the ERCP/sphinceterotomy once the septic physiology has resolved. Arguments for early cholecystectomy have been made as waiting 6 to 8 weeks runs the risk of 20% recurrence rate of a gallstone related event.
Should ERCP fail, PTC is warranted as both these
procedures decrease the morbidity/mortality risk of a common bile duct exploration. PTC can be challenging if there is little intra-hepatic ductal dilatation and it also does not allow sphincterotomy.
Common bile duct exploration is warranted should ERCP and PTC fail at decompressing the biliary tree. In the setting of a patient who is in septic shock, choledochotomy with stone extraction and T-tube placement is recommended as this allows for decompression of the biliary tree and allows for sepsis to resolve prior to performing cholecystectomy to limit the morbidity and mortality associated with both procedures.
The exploration is performed through a choledochotomy on the common bile duct distal to the insertion of the cystic duct. Stay sutures are placed on either side of the choledochotomy and using balloon catheters, fluoroscopy with basket retractors, and flushing, stone extraction is performed. In general, the choledochotomy should be roughly the size of the largest stone. It is best done in dilated ducts as the risk of stenosis is high in
the setting of common bile duct size <6 mm.
A large bore T-tube is placed and the choledochotomy repaired over the T-tube with 4-0 absorbable sutures.
The tube is externalized and bile allowed to drain into an external bag. Due to lack of re-absorption of bile, a patient with this procedure is prone to being deficient in fat soluble vitamins (A, D, E, and K). It is the vitamin K deficiency which is most worrisome as it can lead to a coagulopathy.
A 32-year-old gravida 2 para 1 female at 28 weeks gestation presents with acute onset of right upper quadrant and right upper flank pain with associated nausea and vomiting over the preceding 24 hours. She has no significant medical or surgical history. She has had 1 uncomplicated vaginal delivery. At the time of her evaluation, her temperature is 99.8°F, heart rate is 110, and respiratory rate is 24. Her exam documents a positive Murphy’s sign and guarding in the right upper quadrant. Laboratory studies show the following: WBC - 20,000, H/H- 9/29, Platelets 130,000, ALT-60, and AST 90. Her bilirubin, lipase, and amylase levels are normal. Her urinalysis is within normal limits.
- Which of the following diagnoses is the least common disease in pregnancy presenting with right upper quadrant pain?
A. Acute fatty liver of pregnancy
B. Cholecystitis
C. Cholelithiasis
D. HELLP syndrome
E. Appendicitis
A.
The most common surgical disease in pregnancy is appendicitis with an incidence of 1 in 1000 to 2000 pregnancies.
Gallbladder disease is the second most common surgical disease in pregnancy with an incidence of 1 in 1200 to 1 in 10,000. Theoretically, the incidence of gall bladder disease including cholelithiasis, cholecystitis, and cholangitis should be increased in pregnancy.
The elevated level of serum estrogen seen in pregnancy increases cholesterol secretion, whereas the elevated level of progesterone reduces soluble bile acid secretion and slows emptying of gallbladder. Despite the predilection toward biliary sludge and stone formation, cholecystitis does not occur more frequently during pregnancy. Appendicitis occurs with equal frequency in each trimester and the incidence is not increased in the gravid patient.
The differential diagnosis for RUQ abdominal pain is expanded in pregnancy. It includes gastrointestinal disorders such as pancreatitis, peptic ulcer disease, hepatitis, and appendicitis, due to a superiorly displaced cecum, as well as pyelonephritis, nephrolithiasis, right lower lobe pneumonia, peptic ulcer disease, and myocardial infarction.
Obstetric specific diagnoses must also be included in the differential to include preeclampsia, HELLP (Hemolysis, Elevated Liver enzymes. Low Platelets syndrome and acute fatty liver of pregnancy (AFLP).
HELLP syndrome is a severe form of preeclampsia occurring in up to 8 of 1000 pregnancies presenting most commonly in the third trimester of pregnancy. This syndrome generally involves the characteristic hypertension and proteinuria seen with preeclampsia with evidence of liver dysfunction and a consumptive coagulopathy which can rapidly progress to fulminant
DIC.
Patients with preeclampsia may present with right upper quadrant or epigastric pain due to liver involvement and in the most severe cases subcapsular hemorrhage or hepatic rupture. AFLP is a rare diagnosis, seen in 1:20,000 pregnancies.
This patient presents with findings consistent with an inflammatory intra-abdominal process. Cholecystitis, choledocholithiasis, and cholangitis lead the differential diagnosis. The physical exam findings are highly suggestive of gallbladder disease. WBC counts and alkaline phosphatase levels are routinely elevated during pregnancy and therefore may not be as specific for inflammation during the assessment of the gravid patient.
Which of the following statements is correct concerning Acute Fatty Liver of Pregnancy (AFLP)?
A. AFLP presents most commonly in the second trimester of pregnancy.
B. AFLP commonly presents with serum aminotransferase levels similar to those found in gallbladder disease.
C. AFLP can present with hypoglycemia and occasionally renal failure which can help distinguish
it from HELLP (hemoconcentration, elevated liver enzymes, low platelet) syndrome and gallbladder disease.
D. In a preterm pregnancy, it is considered safe to
continue the pregnancy in a patient who has been diagnosed with AFLP.
C.
The AFLP syndrome almost always presents in the third trimester with serum aminotransferase elevations up to 1000IU/L, which is generally higher than those found in gallbladder disease.
AFLP can also present with hypoglycemia and renal failure, which is not characteristic of either HELLP or gallbladder disease.
Findings of AFLP can still significantly overlap with those of HELLP, making it very difficult to distinguish these two syndromes.
The treatment for both is the emergent delivery of the fetus.
Which of the following statements regarding radiographic imaging of biliary disease in pregnancy is
correct?
A. Classic sonographic signs of biliary disease are
altered in pregnancy.
B. The risk of radiation exposure to the fetus with ERCP (endoscopic retrograde cholangiopancreatography) is high throughout pregnancy.
C. The neuronal development of the fetus is most
sensitive to radiation between 20 to 28 weeks gestation.
D. Exposure to less than 5 rad of ionizing radiation has not been associated with an increased risk of fetal anomalies or pregnancy loss.
E. MR imaging has a higher sensitivity and specificity in the diagnosis of cholecystitis than ultrasonography.
D.
Due to the acuity of presentation and the myriad of diagnoses in the differential, imaging is an essential component in the diagnostic evaluation.
Risks of radiologic studies to the fetus must therefore be considered.
Sonography is the appropriate first line diagnostic modality in pregnancy for both biliary disease and appendicitis as this modality has a high diagnostic accuracy (90% to 100% for both diagnoses) and has no known risk to the fetus.
Classic ultrasound (US) findings to include wall edema, pericholecystic fluid, calculi, and sonographic Murphy’s sign maintain their sensitivity and specificity in pregnancy.
If ultrasound studies arc non-diagnostic, MR imaging without contrast has become the confirmatory test for appendicitis.
For biliary disease, an MRCP can be used in equivocal eases or in suspected eases of choledocholithiasis or cholangitis. It is not as sensitive as US for cholecystitis.
Intraoperative cholangiogram in combination with cholecystectomy is an option for diagnostic evaluation after fetal organogenesis is complete in the second trimester and does not appear to increase the risk for preterm delivery or adverse fetal outcomes.
If MRCP documents stone disease in the biliary tree, ERCP is considered a viable therapeutic option after the first trimester. The risks to the fetus with cholangiogram and ERCP can be reduced with shielding.
CT scan of the abdomen, which is the preferred imaging modality for appendicitis in the non-pregnant patient, confers radiation levels of 5 to 10 rads which approach the maximum permissible radiation dose for fetal exposure during pregnancy.
Fetal exposure to ionizing radiation increases risks of microcephaly, micropthalmia, mental retardation, growth restriction, and cataracts.
The concern of ionizing radiation is greatest during organogenesis which falls between 3-20 weeks of gestation.
The above patient is at 28 weeks and therefore the
risk of serious complications with ionizing radiation is limited. CT generally remains behind US and MR on the imaging algorithm for both appendicitis and cholecystitis even in the patient with a fetus of advanced gestational age due to the disputed twofold
increased risk of carcinogenesis ( 1:1000) in the fetus.
CT imaging should, however, not be abandoned as a diagnostic modality, as the risk of delay in diagnosis far outweighs the risk of radiation.
The consulting radiologist can design CT protocols to minimize the associated risks and counseling can minimize the associated anxiety of the patient.
In this patient, acute cholecystitis is diagnosed by ultrasound. Which of the following is correct regarding treatment of this patient?
A. The risk of adverse effects of laparoscopy is high
even with maximal intra-abdominal pressures limited to 9 mm Hg.
B. If left untreated, the most common complication
of acute cholecystitis in pregnancy is gangrenous
cholecystitis.
C. Available studies have shown significant differences regarding preterm delivery rates, birthweights or neonatal outcomes when comparing laparoscopic versus open cholecystectomies.
D. Nonsteroidal anti-inflammatory drugs (NSAID)
treatment for pain expected to last more than
48 to 72 hours is the pharmacologic option of
choice after 30 weeks gestation to avoid fetal
complications.
E. Beta-lactam antibiotics such as ampicillin-sulbactam or piperacillin-tazobactam are contraindicated in pregnant patients.
B.
Initial non-surgical management can be considered in hemodynamically normal pregnant patients experiencing cholelithiasis. This management plan generally involves bowel rest, intravenous hydration, and NSAID therapy.
A short course (< 48 to 72 h) of indomethacin treatment can provide effective analgesia but is generally avoided in late pregnancy due to the potential adverse fetal effects. Use in the third trimester increases the risk of premature closure of the patent ductus arteriosis and oligohydramnios.
Intravenous antibiotics to include ampicillin-sulbactam, piperacillin-tazobactam, and ticarcillin-clavulanate are not contraindicated in pregnant patients who need antibiotics for acute cholecystitis or choledocolithiasis.
Early surgery has been advocated for all types of biliary disease in pregnancy. If not treated, cholecystitis can lead to life threatening complications, the most common of which is gangrenous cholecystitis followed by abscess formation, perforation, fistula, ileus, or emphysematous cholecystitis. For symptomatic cholelithiasis with no evidence of cholecystitis, surgery can be delayed.
However the literature reports that surgical management of symptomatic cholelithiasis in pregnancy is safe, decreases hospital days, reduces emergency room visits, and the rate of preterm deliveries.
The second trimester (from 13 to 27 weeks gestation) is considered the best timeframe for cholecystectomy as the uterus is not obstructing the view of the surgical field, and the risk of miscarriage or preterm birth is lowest.
In this patient, with clear evidence of cholecystitis, surgical intervention is warranted to reduce risk of serious complications. If complications such as cholangitis or gallstone pancreatitis develop in a pregnant patient, maternal mortality may approach 15% and fetal loss up to 60%.
Surgical management of biliary disease has been revolutionized with the advent of laparoscopy. Laparoscopic technique can be utilized safely in pregnancy across all trimesters depending on the comfort level of the surgeon.
Although data is limited, laparoscopy does not confer an increased risk of adverse pregnancy outcomes to include preterm delivery as compared to laparotomy.
Proper positioning in left lateral tilt is important to reduce venal caval compression and maintain adequate placental blood flow, and open entry technique is recommended to prevent injury to the enlarged gravid uterus. Intraabdominal pressure with pneumoperitoneum should be limited to 10 to 12 mm Hg to reduce the theoretical concern of fetal acidosis associated with the effect of CO2.
There is no indication for intraoperative fetal monitoring.
Early surgery for appendicitis is also recommended in pregnant patients, as the consequences for both the mother and fetus can be catastrophic. Abbasi et al., in the largest case series to date involving 7000 patients, documents a markedly increased risk of severe complications with conservative management to include miscarriage and maternal sepsis. As with cholecystitis, non-operative management of appendicitis is contraindicated in pregnancy.
This patient undergoes an uncomplicated laparoscopic cholecystectomy. On postoperative day 2, she develops increasing pain in her right upper quadrant (RUQ) with fever and recurrent leukocytosis as well as elevated total bilirubin, transaminase, lipase and amylase levels. RUQ ultrasound documents dilated biliary ducts. Which of the following would be the most appropriate next step?
A. MRCP (magnetic resonance cholangio-pancreaticogram)
B. ERCP
C. Continued observation with antibiotic therapy
D. Repeat surgery with bile duct exploration
E. Delivery of the fetus
B.
Following cholecystectomy, this patient presents with findings consistent with choledocholithiasis, with associated gallstone pancreatitis and possible cholangitis.
The best option in this case is ERCP with the option of sphincterotomy to decompress the biliary tract. This approach appears safe in pregnant patients with early onset cholangitis with lower morbidity than conservative management.
In this patient, repeat surgery with intraoperative cholangiography or bile duct exploration would be a backup option, if the stones cannot be removed via ERCP.
Percutaneous biliary tract decompression would be another option in a high risk patient. MRCP is an excellent and safe diagnostic test in pregnancy and is a viable option in patients where the diagnosis is uncertain.
In a case with a high suspicion of cholangitis, this step could delay therapy which could have severe consequences for both fetus and mother.
More aggressive therapy for cholangitis is therefore indicated in pregnancy.
Although conservative treatment with continued intravenous antibiotics and observation may be appropriate in the non-gravid patient, the risks of this non-surgical approach are higher in the gravid patient and predispose her to grave complications.
Premature delivery of the fetus is not indicated for the treatment of biliary disease.
One year after an apparently uncomplicated cholecystectomy for chronic cholecystitis and cholelithiasis, a 37/F presents epigastric pain and jaundice of 2 wk duration. Ultrasonography reveals a dilated CBD along with dilated IHDs. To establish the nature of the obstruction, which diagnostic procedure is best:
a. ERCP
b. Percutaneous transhepatic cholangiography
c. CT scan
d. HIDA scan
a. ERCP
A 40/M from Davao presents with RUQ pain and fever. UTZ reveals a 6 cm complex mass in segment 7 of the liver. The treatment of choice is:
a. Percutaneous UTZ-guided aspiration
b. Laparoscopic surgery for drainage
c. Extra-peritoneal approach for drainage
d. Appropriate doses of metronidazole
d. Appropriate doses of metronidazole
A patient undergoes cholecystectomy, CBDE, T-tube choledochostomy for cholecystodocholithiasis. One week post-op, a T-tube cholangiogram shows multiple retained extrahepatic bile duct stones. Ideally this patient is best managed by:
a. choledochoscopic lithotripsy
b. re-exploration
c. extracorporeal shock wave lithotripsy
d. oral chenodeoxycholic acid
a. choledochoscopic lithotripsy
55/F consulted at the ER due to progressive painless jaundice, 1 day PTC, developed on and off of high grade fever associated with RUQ pain, on HBT-UTZ there was a cut off at the confluence of left and right IHDs, what will be your best treatment option at this time?
a. proceed with biliary exploration
b. ERCP with stenting
c. Bilateral percutaneous transhepatic biliary drainage
d. Conservative medical (antiobiotic / hydration) management
c. Bilateral percutaneous transhepatic biliary drainage
34/M underwent cholecystectomy, CBDE, t-tube choledochostomy. On follow up, the tube cholangiogram showed an impacted stone at the distal CBD. What is the best management for this patient?
a. flushing the t-tube with saline
b. choledochoscopy with stone extraction
c. ERCP, sphincterotomy with stone extraction
d. Re-exploration of the CBD
b. choledochoscopy with stone extraction
65/M came in at the ER due to abdominal pain and fever. On PE, the patient was noted to be jaundiced, tachycardic and hypotensive (70/40). Preliminary UTZ done at the ER showed dilated intrahepatic ducts. The best approach for this patient after resuscitation is:
a. Definitive surgery
b. Antibiotics alone
c. Immediate decompression
d. Conservative treatment
c. Immediate decompression
A 52-year-old male with a history of poorly controlled diabetes presents to the emergency department complaining of right upper quadrant pain and vomiting. An ultrasound is performed that shows gallstones with air in the gallbladder wall. Which of the following is the BEST next step in management of this patient?
a. IV antibiotics and cholecystectomy in the next 24-48 hours
b. Schedule elective cholecystectomy as an outpatient
c. ERCP
d. Emergent cholecystectomy
d. Emergent cholecystectomy