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Mirizzi Syndrome

WhitneyN.Jones andLeahC.Tatebe
60
Scenario
A 55-year-old male presents to the emergency room with worsening right upper quadrant and epigastric pain, nau­sea, vomiting with oral intolerance for the last 4days. He has a history of cholelithiasis and has had chronic episodic epigastric pain radiating to his back and right side for the last year. His pain normally resolves itself within 24–36h. He previously presented 9months ago with similar signs and symptoms and a right upper quadrant ultrasound demon­strated a distended gallbladder with multiple gallstones. Past medical history includes hypertension and obesity. He was subsequently discharged and the patient failed to follow up with surgery for further evaluation.
Physical exam is notable for epigastric and right upper
quadrant tenderness and scleral icterus.
Labs were notable for mildly elevated white blood cells (13.3×103/L), total bilirubin (7.4mg/dL), and direct biliru­bin (4.8mg/dL).
What differential diagnosis should be considered for this patient? (Khokhar etal. 2022)
• Acute cholecystitis
• Choledocholithiasis
• Mirizzi syndrome
• Gallbladder cancer
• Cholangiocarcinoma
• Pancreatic cancer
• Sclerosing cholangitis
• Metastatic disease
What steps should be taken for denitive diagnosis?
Abdominal Ultrasound (Chen etal. 2018)
• Typically the initial imaging modality to evaluate biliary
disease due to it being noninvasive and widely available
• Evidence of MS includes atrophic gallbladder, ectatic common hepatic duct with a normal distal CBD
MRCP
• Next imaging modality when signs of obstructive jaun­dice are seen on US (Grohol etal. 2023)
• Increased sensitivity to detect MS when compared to US
• Useful in dening the biliary anatomy and detect charac­teristics of MS
• Ineffective inlocalizing stulas (Chen etal. 2018)
ERCP (Chen etal. 2018)
• Gold standard for diagnosing MS, as it is highly sensitive and provides therapeutic options
• Provides superior visualization of the biliary tree and can visualize if stulation has occurred
• Allows for decompression of biliary tree
• Pre or post operative stenting can be helpful in dening the anatomy
CT
• Most useful imaging modality to distinguish MS from malignancy (Chen etal. 2018)
• Location and cause of biliary obstruction can be seen (Chen etal. 2018)
Describe the stages of progression of MS?
• Initially, isolated compression of hepatic bile duct or common bile duct is the rst stage
• Progresses to inammation, ulceration, and the formation of cholecystoenteric and cholecystobiliary stulas as the compression becomes prolonged and chronic
What are intraoperative ndings found during operation
may indicate Mirizzi syndrome?
W. N. Jones · L. C. Tatebe (*) Department of Surgery, Northwestern University, Chicago, IL, USA e-mail: whitney.jones@nm.org; ltatebe@nm.org
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2025 M. Neff et al. (eds.), Passing the General Surgery Oral Board Exam, https://doi.org/10.1007/978-3-031-78244-2_60
• Hard brous adhesions between gallbladder and CBD
• Thick-walled gallbladder
• Contracted gallbladder
185
186
W. N. Jones and L. C. Tatebe
MS is treated surgically with a cholecystectomy. What are the challenges of operating on patients with MS and what are common complications?
• Challenges include difcult, distorted anatomy with
dense adhesions secondary to inammation
• CBD injury
• Massive hemorrhage
What is the operative approach for MS? (Klekowski etal.
2021)
• Open cholecystectomy is generally accepted as the stan-
dard of care. There should be a high rate of conversion to
open and increased risk of bile duct injury, up to 17%
• However laparoscopic cholecystectomy could be an
option if a stula is not present
• If MS diagnosis is made intraoperatively, recommend
intraoperative cholangiogram to evaluate for a stula and
to further dene the biliary anatomy. If a stula is present
repair or stent bile injury
• Concerning involving a hepatobiliary surgeon
• Can consider endoscopic therapies until denitive surgi-
cal treatment in poor surgical candidates
What characteristic anatomic distortions can you expect to see in MS? (Klekowski etal. 2021)

Clean Kills

• Failing to dene the biliary anatomy with the proper imaging modality
• Failing to consider malignancy and other biliary pathol­ogy including acute cholecystitis, cholangitis, and pan­creatitis that could be occurring concurrently with MS
• Failing to address biliary obstruction
• Surgical plan should consider and address known difculties
• No leaving a drain if there is concern for a stula or bile leak
Words ofWisdom
MS is a very rare sequelae of biliary disease with an inci­dence of roughly 1% in the United States and slightly higher in low and middle income countries and it is very challeng­ing to diagnose preoperatively. MS should be considered in differential diagnosis of any patient presenting with obstruc­tive jaundice as preoperative diagnosis allows for proper operative planning to limit and prevent high mortality and morbidity associated with MS. While surgery remains the mainstay of treatment, preoperative diagnosis of MS allows a collaborative multidisciplinary approach to both diagnosis and treatment.
1. Atrophic gallbladder with thick or thin walls
2. Obliterated cystic duct
3. Cystic duct—long, parallel to the common bile duct and with low insertion
4. Cystic duct—short cystic duct with another anatomical variation
5. Bile duct—partially obstructed due to the external com­pression or a gallstone eroding from the gallbladder
6. Distal bile duct with normal thin walls and no distended lumen
7. Proximal bile duct—dilated with inamed walls
8. Abnormal communication between the bile duct and the gallbladder
9. Fistula between the gallbladder and stomach, duodenum, colon, or other structures

Bibliography

Chen H, Siwo EA, Khu M, Tian Y. Current trends in the manage-
ment of Mirizzi syndrome. Medicine. 2018;97:e9691. https://doi.
org/10.1097/md.0000000000009691.
Grohol B, Fortin GT, Ingold T, Bennett P. Mirizzi syndrome: a
case report. Cureus. 2023;15:e34783. https://doi.org/10.7759/
cureus.34783.
Jones MW, Troy F. Mirizzi syndrome. StatPearls Publishing; 2023.
https://www.ncbi.nlm.nih.gov/books/NBK482491/. Accessed 21
Aug 2023.
Khokhar I, Adourian M, Delia E, Mohan G, Mathew M. Mirizzi
syndrome: a case report and review of the literature. Cureus. 2022;14:e24375. https://doi.org/10.7759/cureus.24375.
Klekowski J, Piekarska A, Góral M, Kozula M, Chabowski M.The
current approach to the diagnosis and classication of Mirizzi syn­drome. Diagnostics. 2021;11:1660.
Part VII
Pancreas

Acute Pancreatitis

RachaelPalumbo andAditiKapil
61
A 52-year-old male presents to the emergency department with a 1-day history of severe epigastric pain with mild nau­sea and decreased appetite. He denies previous similar symptoms, vomiting, changes in bowel habits, weight loss, or fever. He has no history of alcohol or drug use. There is no signicant past medical or surgical history. Lab data include WBC 12, Hb 14, bicarb 19, creatinine 1.2, lactic acid 3, and lipase 3000. Vitals: HR 110 (regular), BP 100/60, Temp 36°F, RR 18. Physical exam signicant for epigastric pain and dry mucous membranes but otherwise normal. How would you manage this?
• Based on the Revised Atlanta Criteria, the patient has
acute pancreatitis.
– This includes presence of at least two of the following:
(1) upper abdominal pain; (2) serum amylase or lipase level more than three times the upper limit of normal; (3) characteristic imaging ndings (contrast enhanced CT, MRI, and etc.).
• Initial management includes uid resuscitation and close
monitoring. Recommend goal directed uid resuscitation (e.g., 10cc/kg bolus of Ringer’s lactate with continuous rate at 1.5cc/kg given evidence of hypovolemia on vitals) that is adjusted based on urine output, vital signs, and lab data after admission (De-Madaria etal. 2022).
• Early initiation of nutrition is recommended, including a
trial of regular diet within 24h of admission. Parenteral nutrition should only be employed when enteral feeds cannot be tolerated and nutritional support is required (Crockett etal. 2018).
• Prophylactic antibiotics are not recommended (Crockett
etal. 2018).
After 3days of resuscitation and initiation of regular diet, the patient has improved. Vital signs and labs have normal­ized, and the patient’s pain has much improved. What inves-
R. Palumbo · A. Kapil (*) Department of Surgery, Yale School of Medicine, New Haven, CT, USA e-mail: aditi.kapil@yale.edu
tigational studies and potential interventions should be done prior to discharge?
• The patient should have an evaluation of biliary sources of pancreatitis. This would include a RUQ ultrasound to evaluate for cholelithiasis. Gallstone pancreatitis is the most common cause of acute pancreatitis.
• If cholelithiasis is present, a cholecystectomy is recom­mended prior to discharge. Normalization of serum enzymes or resolution of pain is not specically required prior to undergoing cholecystectomy in mild–moderate gallstone pancreatitis.
Alternate Scenario
A 52-year-old male presents to the emergency department with a 1day history of severe epigastric pain with nausea and vomiting. Denies previous similar symptoms, changes in bowel habits, weight loss, or fever. No history of alcohol or drug use. No signicant past medical or surgical history. Lab data including: WBC 15, Hb 16, bicarb 16, creatinine 2.1, lactic acid 5, lipase 3000. Vitals: HR 120 (regular), BP 90/50, Temp 36 °F, RR 18. Physical exam signicant for altered mental status (confusion), epigastric pain. How would you manage this?
• Similar to the above scenario, the patient has acute pan­creatitis. However, he is showing signs of hemodynamic instability with tachycardia, hypotension, and altered mental status with lab data showing concern for hypovolemia.
• Initial management would include close monitoring with ICU admission and uid resuscitation. Prophylactic anti­biotics are not recommended.
Fluid resuscitation and close monitoring initiated.
However, despite this, over the next 48h, the patient is per­sistently hypotensive (MAP 55) and becomes progressively lethargic and altered. His temperature is now 39°C with a
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2025 M. Neff et al. (eds.), Passing the General Surgery Oral Board Exam, https://doi.org/10.1007/978-3-031-78244-2_61
189
190
R. Palumbo and A. Kapil
leukocytosis of 24 and hemoglobin of 14. How would you manage this patient?
• This patient is progressively critically ill from his pancre­atitis, requiring intubation and initiation of vasopressors.
• Given development of fever, worsening leukocytosis, and persistent hemodynamic instability, would recommend CT imaging to evaluate pancreatitis.
– If a patient with acute pancreatitis fails to show clinical
improvement within 72h of admission or infected nec­rotizing pancreatitis is suspected, cross sectional imag­ing is recommended with initiation of antibiotics with superimposed infection.
Contrast enhanced cross sectional imaging performed,
showing areas of both pancreatic parenchymal enhancement and nonenhancement with evidence of a small amount of peripancreatic emphysema. How would you manage this patient?
• This patient has necrotizing pancreatitis with associated infection. Would initiate broad spectrum antibiotics and continue supportive care, including nutritional support with enteral feeds.
The patient is started on antibiotics and enteral feeds.
Initially, he clinically improves, allowing for extubation and weaning from vasopressor support. However, on hospital day 9, he decompensates with Tmax 39°C and hypotension requiring vasopressor support. Repeat imaging shows a 6cm retroperitoneal peripancreatic uid collection with gas and necrotic debris. How would you manage this patient?
• The patient is now showing an acute necrotic collection with associated infection.
– The Revised Atlanta Criteria characterizes acute pan-
creatitis based on morphologic subtypes and timeline of the disease process, specically peripancreatic uid collections. The morphologic subtypes include inter­stitial edematous acute pancreatitis and necrotizing acute pancreatitis. Peripancreatic uid collections that develop within 4 weeks of the onset of interstitial edematous pancreatitis are termed acute peripancreatic uid collections; after 4 weeks, as pseudocysts. The collections that develop within 4weeks in the setting of necrotizing pancreatitis are acute necrotic collec­tions, and after 4weeks, considered walled off necro­sis. This can be seen in Table.
• Given the patient’s decompensation despite antibiotics and development of a peripancreatic uid collection, it
would be reasonable to recommend a step-up approach for drainage of the collection. Options include both an endoscopic and operative step-up approach, with spe­cic intervention depending on the institution resources.
– The step-up approach is a minimally invasive manage-
ment technique to peripancreatic uid collections, rst developed from the Panther Trial (Dutch Pancreatitis Group) compared to open necrosectomy. Treatment includes a series of percutaneous drain placements, with ultimate video assisted retroperitoneal debride­ment if clinically indicated. Since its development, endoscopic step up approaches have been employed with similar outcomes, with lower rates of pancreati­cocutaneous stulas (van Brunschot etal. 2018).
Interventional GI is not available at your institution, so over the next several days, the patient undergoes a series of percutaneous drain placements with upsizing ×2. However, despite upsizing and ushing the drain, he continues to have fevers, leukocytosis, and poor PO tolerance. How would you manage this patient?
• Given the lack of clinical improvement, would recom-
mend a video assisted retroperitoneal debridement, gen-
eral steps:
– Positioned supine, left side elevated. Prep and drape
the left ank and abdomen, including drains.
– Subcostal incision made adjacent to the retroperitoneal
drain, allowing digital exploration and following of the drain into the collection.
– Collection opened and necrosectomy performed with
ringed forceps and suction.
– Long 10 mm trocar and laparoscope then placed
through incision into the retroperitoneum for further debridement.
– Two large bore drains placed and incision closed.

Clean Kills

• Recommending open necrosectomy as operative inter-
vention prior to considering Step Up approach or trans-
gastric debridement of necrotic pancreatic collections.
• Failing to initiate initial management of pancreatitis,
including uid resuscitation, close monitoring, and early
nutrition. Remember, pancreatitis can be a critical care
scenario.
• Failing to recognize a biliary etiology as the cause of
pancreatitis.
61 Acute Pancreatitis
191

Bonus Points

• Management of peripancreatic uid collections is com­plex, and it often requires input from an interdisciplinary team and close attention to institutional resources. Knowing the surgical Step Up approach can provide a reasonable treatment algorithm for most cases.
• Be familiar with the Revised Atlanta Criteria for diagno­sis and classication of pancreatitis subtypes.
Words ofWisdom
Management of acute pancreatitis for the general surgeon includes not only the medical and critical care aspects of treatment but also the interventional algorithms such as the Step Up approach in necrotizing pancreatitis. Initial care of pancreatitis regardless of severity includes uid resuscita­tion, close monitoring, and nutrition. Cholecystectomy is recommended during index admission for gallstone pancre­atitis but may be postponed 8weeks for necrotizing pancre­atitis. Initiate antibiotics only when there is evidence of superimposed infection. Care for peripancreatic uid collec­tions is complex, with the most common indication for drain­age being infection.

Bibliography

Colvin SD, Smith EN, Morgan DE, Porter KK.Acute pancreatitis: an
update on the revised Atlanta classication. Abdom Radiol (NY). 2020;45(5):1222–31.
Crockett SD, Wani S, Gardner TB, Falck-Ytter Y, Barkun
AN. American Gastroenterological Association Institute guide­line on initial management of acute pancreatitis. Gastroenterology. 2018;154(4):1096–101.
De-Madaria E, Buxbaum JL, Maisonneuve P, García García De Paredes
A, Zapater P, Guilabert L, etal. Aggressive or moderate uid resusci­tation in acute pancreatitis. N Engl J Med. 2022;387(11):989–1000.
Hallensleben ND, Timmerhuis HC, Hollemans RA, Pocornie S, van
Grinsven J, van Brunschot S, etal. Optimal timing of cholecystec­tomy after necrotising biliary pancreatitis. Gut. 2022;71(5):974–82.
Lankisch PG, Apte M, Banks PA. Acute pancreatitis. Lancet.
2015;386(9988):85–96.
Maatman TK, Zyromski NJ.Open pancreatic debridement in necrotiz-
ing pancreatitis. J Gastrointest Surg. 2021;25(1):331–2.
Mederos MA, Reber HA, Girgis MD. Acute pancreatitis: a review.
JAMA. 2021;325(4):382–90.
Rayman S, Jacoby H, Guenoun K, Oliphant U, Nelson D, Kaiser A,
etal. Diagnosis and contemporary management of necrotizing pan­creatitis. Am Surg. 2023;89:4817.
van Brunschot S, van Grinsven J, van Santvoort HC, Bakker OJ,
Besselink MG, Boermeester MA, etal. Endoscopic or surgical step­ up approach for infected necrotising pancreatitis: a multicentre ran­domised trial. Lancet. 2018;391(10115):51–8.
van Santvoort HC, Besselink MG, Bakker OJ, Hofker HS, Boermeester
MA, Dejong CH, etal. A step-up approach or open necrosectomy for necrotizing pancreatitis. N Engl J Med. 2010;362(16):1491–502.

Chronic Pancreatitis

ClaraZhu andYoungK.Hong
62
Way Question May BeAsked?
A 60-year-old male with a history of smoking comes to your ofce with a known history of chronic pancreatitis. He has constant and signicant abdominal pain, which he states is impairing his quality of life. He has undergone a CT scan, which demonstrates diffuse pancreatic calcications without a visible mass.
How toAnswer?
Full History
• Frequency of abdominal pain
• Length of pain episodes
• Tolerance of food
• Quality of stool
• Recent weight loss
• Family history of cancer
• History of smoking
• History of alcohol use
Full Physical Examination
• Vital signs
– Cachexia
• Temporal wasting (signs of malnutrition)
• Abdominal exam
• Scleral icterus
• Jaundice
• CT scan in work-up of differential diagnosis. CT can demonstrate characteristic ndings of chronic pancreati­tis, e.g., calcications, pancreatic duct dilation, heteroge­neity of parenchyma
• MRCP to evaluate for ductal characteristics, including diameter and pancreas divisum. Diameter determines the feasibility of a drainage operation

Surgical Procedure

• IR evaluation—celiac plexus block for pain, although this procedure must be repeated every several weeks
• Consider rst-line endoscopic drainage—ERCP with sphincterotomy—if pancreatic divisum is the underlying cause of chronic pancreatitis
• Surgical approach to be considered after failure of symp­tom relief after nonoperative management, obstruction, or variceal bleeding
• Puestow operation—lateral pancreaticojejunostomy for ductal dilation (ductal decompression)
• Beger operation—subtotal pancreatic head resection with duodenal preservation (resection of diseased tissue)
• Frey procedure—coring out of the pancreatic head with lateral pancreaticojejunostomy (ductal decompression with resection of diseased tissue)

Common Curveballs

Diagnostic Tests
• Labs including CBC, BMP, lipase, amylase, albumin, prealbumin
C. Zhu · Y. K. Hong (*) Department of Surgery, Cooper University Healthcare, Camden, NJ, USA e-mail: Zhu-clara@cooperhealth.edu;
Hong-young@cooperhealth.edu
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2025 M. Neff et al. (eds.), Passing the General Surgery Oral Board Exam, https://doi.org/10.1007/978-3-031-78244-2_62
• Lack of interventional GI availability
• Recurrent symptoms after standard operations. Patients should be considered for total pancreatectomy, though this will cause signicant morbidity, including brittle diabetes
193
194
C. Zhu and Y. K. Hong

Clean Kills

• Forgetting to order imaging
• Failing to consider endoscopic interventions before surgery
• Not ordering testing to elucidate the underlying etiology of chronic pancreatitis
• Choosing drainage operations for a nondilated pancreatic duct
Words ofWisdom
Chronic pancreatitis is an irreversible disease of the pancre­atic parenchyma that can progress to diabetes, weight loss from malabsorption, and pancreatic malignancy. Radiographic tests can indicate whether there is tissue in need of resection, drainage, or both. Patients who have recur­rent symptoms after standard operations for chronic pancre­atitis should be considered for a total pancreatectomy.

Pancreatic Cancer

ClaraZhu andYoungK.Hong
63
Way Question May BeAsked?
A 60-year-old male presents to the emergency room with painless jaundice and weight loss over the last month. A CT scan performed identied a pancreatic mass. You are con­sulted to evaluate the patient.
How toAnswer?
Full History
• Constitutional symptoms (fatigue, weight loss)
• Abdominal pain
• Nausea/vomiting
• Pruritus
• Medical history
• Assessment of functional status
• Cancer history
• Substance use history (ETOH, smoking)
• Family history
Full Physical Examination
• Abdominal exam
• Lymph node exam
• Assessment of patient’s functional status
Diagnostic Tests
• Laboratory studies, including LFTs, CBC, and lipase
– Serum bilirubin, alkaline phosphatase, liver enzymes
• Staging evaluation
– Tumor markers (CEA, CA 19-9 [most sensitive, but
falsely elevated with hyperbilirubinemia]) – CT with triple-phase contrast pancreatic protocol – CT chest for evaluation of metastatic disease
C. Zhu · Y. K. Hong (*) Department of Surgery, Cooper University Healthcare, Camden, NJ, USA e-mail: Zhu-clara@cooperhealth.edu;
Hong-young@cooperhealth.edu
– ERCP/EUS for endoscopic assessment and biopsy
Stent placement can be performed if there is con­cern for obstructive jaundice
– Staging laparoscopy (Controversial)

Surgical Management

Resectable Disease → No vascular abutment or less than 180° of PV/SMV
Borderline Resectable → Pancreatic head disease: less than 180° abutment of SMV or Celiac artery→Neoadjuvant chemotherapy
Unresectable disease or metastatic disease → Locally advanced with greater than 180° encasement of SMA, encasement of the celiac axis and/or aorta→Denitive sys­temic therapy
Key Steps for Whipple’s Procedure
1. Full abdominal exploration to evaluate for resectability
2. Clockwise rotation:
(a) Cattell–Braasch maneuver (b) Identication of intrapancreatic superior mesenteric
vein (SMV) (c) Extended Kocher maneuver (d) Portal dissection (e) Ligation of gastroduodenal artery (GDA) (f) Cholecystectomy (g) Transaction of common hepatic duct proximal to cys-
tic duct (h) Transect distal stomach (i) Transect jejunum distal to LOT (j) Transect pancreas at the level of the portal vein with
frozen margin
3. Counterclockwise reconstruction: (a) End-to-side pancreaticojejunostomy (PJ) in two lay-
ers over pediatric feeding tube as a stent (b) End-to-side choledochojejunostomy (c) End-to-side gastrojejunostomy
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2025 M. Neff et al. (eds.), Passing the General Surgery Oral Board Exam, https://doi.org/10.1007/978-3-031-78244-2_63
195
196
C. Zhu and Y. K. Hong
Braun jejunojejunostomy to reduce risk of bile
reux
(d) Drain placement at PJ anastomosis

Common Curveballs

The patient’s functional status should be taken into consider­ation before offering surgery.
• Those with limited ECOG status should be considered for denitive chemoradiation therapy
• Intraoperative discovery of metastatic disease in obstructed biliary tree
Borderline resectable disease → Neoadjuvant therapy
FOLLOWED by restaging.
Distal pancreatectomy/splenectomy is an option for a dis-
ease of the pancreatic body/tail.
Explain the course of replaced hepatic arteries.

Surgical complications

• Anastomotic leak
• Pancreatic leak at PJ→Management options if a leak is high output
• Delayed gastric emptying
• Marginal ulcer management
• Chyle leak
What to do in the setting of metastatic disease (be able to
describe palliative bypass procedures).

Clean Kills

• Inadequate staging workup
• Lack of understanding of anatomic resectability
• Never operate with very elevated serum biliru­bin→Biliary stent before surgery to reduce the risk of cholestatic liver failure
Words ofWisdom
Pancreatic cancer is a deadly disease, but surgical resection with perioperative chemotherapy offers a meaningful sur­vival benet for patients with resectable disease. It is essen­tial to understand the criteria for resectability, as well as complications that can occur in the postoperative setting.