Download choledochalcyst Changed

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CASE
PRESENTATION
Dr. Sana Anum,
Dr. Ahmed Javed
PGR-1
Surgical Unit
DHQ Hosp Rwp
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PATIENT’S PROFILE
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NAME: Naveeda D/O Mehboob
AGE: 16 years
Gender: Female
Address: Tehsil Bagh Azad Kas
hmir
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Presenting Complaints
Patient presented in O.P.D with
complaints of
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Pain right hypochondrium
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Vomiting

Fever
Since last 3 days
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History of Present Illness
Patient was in usual state of health 2 years back
when she started having Intermittent episodes of

pain right hypochondrium,
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Multiple episodes of vomiting,

And Fever
lEach episode lasted from few days to a week,
relieved after taking prescribed medications from
local G.P
lNow, presented with acute aggravation of these
symptoms for 3 days
l
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Intermittent episodes of pain
right hypochondrium
Gradual in onset
Moderate in severity,
Sharp in character,
Non radiating,
Continuous,
Progressively increasing
Related with the intake of food,
Relieved with medication
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Associated with 3 to 4
episodes of vomiting per day
Pale coloured,
Non projectile
Profuse,
Associated with intake of food,
Containing undigested food particles
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Fever
Gradual onset
High grade,
Continuous,
Associated with rigors and chills
Relieved with medication
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No h/o yellowing of sclerae/ pale
coloured stool;
No h/o loss of apetite, loss of
weight;
no h/o blood in vomitus/ blood in
stool
No h/o trauma
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History
SYSTEMIC
REVIEW
RESP: no cough, hemoptysis, stridor
CVS: no s.o.b, palpitations, chest pain
CNS: no dizziness, diplopia, seizures
Genitourinary: no urinary complaints,
menarche at 12 years of age, regular c
ycles, no h/o discharge
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History
PAST MEDICAL
H/o multiple appointments with local G.P but detail
s of medications not available
No h/o drug allergies
No h/o transfusions
PAST SURGICAL
No prev surgery, No recent instrumentation
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History
•PERSONAL
•Student, unmarried
•FAMILY
•No h/o diabetes, hypertension, T.B, asthma in immediate relativ
es
•No h/o malignancy
•SOCIAL
•Lives with parents and 3 siblings in a house of 3 rooms, no pets
at home, no recent travel
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Physical Examination
General Appearance:
Conscious, alert, slightly anxious, in moderate pain,
lying in bed
No pallour/jaundice
No cyanosis, clubbing, koilonychia
Dry oral mucosa
No edema
No cervical/ axillary/ inguinal lymphadenopathy
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Physical Examination
VITALS:
Pulse: 101 bpm
R/R: 13 bpm
Temp: 98 F
B.P: 100/60 mmHg
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Physical Examination
Abdominal Examination:
Scaphoid abdomen, central, inverted umbilicus, no
scar mark, striae, dilated veins
Tenderness in epigastrium and right
hypochondrium, Murphy's sign (-)
Liver palpable 2 fingerbreadths below right subcostal
margin
No splenomegaly, no palpable mass, no ascites
Hernial Orifices intact
No supraclavicular lymphadenopathy
DRE: no exeternal pathology, anal tone normal, no
mass, tenderness
Bowel sounds audible on auscultation
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Physical Examination
th
Cardiovascular
Apex beat left 5 ICS
S1+ S2+ no murmurs
Chest
Bilaterally equal air entry with no ad
ded sounds
Extremities
No edema, no cyanosis, brisk capill
ary refill
Neurologic Exam
No focal neurologic findings
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Provisional
Diagnosis
ACUTE CHOLECYSTITIS
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DIFFERENTIALS
Acute Cholecystitis
lAcute Pancreatitis
lchronic pancreatitis
lGastritis
lCholedochal Cyst
l
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Laboratories
Hgb
13.6 g/dL
Na+
135 mEq/L
Hct
33.5%
K+
3.5 mEq/L
WBC
7.1x 10^3/uL
Cl-
103 mEq/L
N
50%
T. Bilirubin
0.8
L
40%
AST
111 IU/L
M
7%
ALT
17 IU/L
E
2%
Alk Phos
748 IU/L
Amylase
66 IU/L
USG ABDOMEN
Gall Bladder distended
lCystic dilation of distal
CBD(3.0x2.1cm) with
multiple echogenic foci
(calculi), largest 8 mm in
size
lIntrahepatic Biliary
channels not dilated
l
CT ABDOMEN WITH ORAL & I/V CONTRAST
Cystic dilation of distal CBD 2.2x2.8 cm, lateral to head of pancreas,
Containing multiple faintly radiolucent shadows likely to be stones
Homogenous liver with no focal lesion
CT ABDOMEN WITH ORAL & I/V CONTRAST
Cystic dilation of distal CBD 2.2x2.8 cm, lateral to head of pancreas,
Containing multiple faintly radiolucent shadows likely to be stones
Homogenous liver with no focal lesion
MRCP
Cystic dilation of common bile duct
measuring upto 1.5 cm in diameter
with cranio caudal extent of 3.2cm
Intraluminal calculusof 10-12mm
noted dependently in dilated CBD
Intra-pancreatic part of CBD normal
Impression: Choledochal Cyst Typre
1b
MRCP
M
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DIAGNOSIS: Choledochal Cyst,
Type 1b
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PRE OPERATIVE PREPARATION
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Informed Consent
Blood grouping & cross matching
Fitness for General Anesthesia
Arrangement of Ventillatory support Post opera
tively
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PROCEDURE: Open
Cholecystectomy, Choledochal
Cystectomy & Hepaticojejunostomy
Incision: Rooftop incision
Per-Op Findings:
lCholedocholithiasis
lFusiform Choledochal cyst
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PROCEDURE
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After taking aseptic measures, skin incision made. Subcutaneous tissues incised, abdo
men opened and findings noted.
Duodenum kocherized
Gall bladder mobilized from fossa
Choledochal cyst dissected away from portal vein
Cystectomy done distally at the level of entrance into duodenum, & proximally1 cm from
confluence of right and left hepatic ducts
Roux loop formed 30 cm distal to duodenojejunal junction
Hepaticojejunostomy and end to side entero-enterostomy done
Drain placed in subhepatic region
Abdomen washed with warm normal saline and then closed in layers
Aseptic dressing done
Specimen of resected Choledochal cyst and gall bladder was labelled and sent for histo
pathology.
Cholecochal
cyst
Gall Bladder
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POST OPERATIVE COURSE
Patient was shifted in ICU care for 48 hours post
operatively
1st post op day
Pulse: 93 bpm
B.P. 110/60 mmHg
RR: 16/min
spO2: 98%
NG: 100 ml/24 hrs
Urine O/P: 1600 ml/24 hrs
Subhepatic Drain: 125 ml/24 hrs
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POST OPERATIVE COURSE
3rd Post Operative Day
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Pulse: 76 bpm
B.P. 120/70
R/R: 18/min
sPO2: 98%
NG: nill
Urine O/P: 2100ml/ 24 hrs
Subhepatic Drain: 100 ml/24 hrs
• S/amylase, LFTs within normal limits
• NG, Folley catheter removed & patient mobilized
• Oral sips allowed
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POST OPERATIVE COURSE
8th Post Operative Day
• Pulse: 80 bpm
• B.P. 120/70
• R/R: 12/min
• Subhepatic Drain: nill/24 hrs
• Repeat S/amylase, LFTs within normal limits
• Subhepatic drain removed
• Patient discharged & advised followup with histopathology report
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LITERATURE
REVIEW
Biliary Cysts
Dr Malik Irfan Ahmed
Senior Registrar Surgery,
DHQ Hospital
•Biliary cysts are cystic dilations that
may occur singly or in multiples
throughout the biliary tree.
• They were originally termed
choledochal cysts due to their
involvement of the extrahepatic bile
duct. However, the clinical
classification was revised in 1977 to
include intrahepatic cysts.
Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts:
Classification, operative procedures, and review of thirty-seven cases
including cancer arising from choledochal cyst. Am J Surg 1977; 134:263.
ALONSO-LEJ F, REVER WB Jr, PESSAGNO DJ. Congenital choledochal cyst, with
a report of 2, and an analysis of 94, cases. Int Abstr Surg 1959; 108:1.
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EPIDEMIOLOGY
• Incidence of biliary cysts has been estimated to be 1:100,000 to 1:150,000
• The incidence is higher in some Asian countries (up to 1:1000)
• Between one-half and two-thirds of the reported cases occurring in Japan
• More common in women, with a female to male ratio of 3:1 to 4:1
Lipsett PA, Pitt HA, Colombani PM, et al. Choledochal cyst disease. A changing pattern of presentation. Ann Surg 1994; 220:644.
O'Neill JA Jr. Choledochal cyst. Curr Probl Surg 1992; 29:361.
Singham J, Yoshida EM, Scudamore CH. Choledochal cysts: part 1 of 3: classification and pathogenesis. Can J Surg 2009; 52:434
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PATHOGENESIS (Congenital)
• Unequal proliferation of embryologic biliary epithelial
cells before bile duct cannulation is complete.
• Fetal viral infection
• Ductal obstruction or distension during the prenatal or
neonatal period.
Lu, SC. Biliary cysts. In: Textbook of gastroenterology, Yamada, T (Eds), Lippincott Williams and Williams, Philadelphia 1999. p.2292.
Yotuyangi S. Contribution to etiology and pathology of idiopathic cystic dilatation of the common bile duct, with a report of three cases. Gann (Tokyo)
1936; 30:601.
Tyler KL, Sokol RJ, Oberhaus SM, et al. Detection of reovirus RNA in hepatobiliary tissues from patients with extrahepatic biliary atresia and
choledochal cysts. Hepatology 1998; 27:1475.
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PATHOGENESIS (Acquired)
ABNORMAL PANCREATICO-BILIARY JUNCTION
It is present in about 70 percent of patients with biliary
cysts and may be a significant risk factor for malignancy
with the cyst.
Yamao K, Mizutani S, Nakazawa S, et al. Prospective study of the detection of anomalous connections of pancreatobiliary ducts during
routine medical examinations. Hepatogastroenterology 1996; 43:1238.
Song HK, Kim MH, Myung SJ, et al. Choledochal cyst associated the with anomalous union of pancreaticobiliary duct (AUPBD) has a more
grave clinical course than choledochal cyst alone. Korean J Intern Med 1999; 14:1.
Funabiki T, Matsubara T, Miyakawa S, Ishihara S. Pancreaticobiliary maljunction and carcinogenesis to biliary and pancreatic malignancy.
Langenbecks Arch Surg 2009; 394:159.
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BABBITT’S THEORY of APBJ
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Failure of the embryological ducts to migrate into the duodenum
Junction of the bile duct and pancreatic duct outside the duodenal wall &
sphincter of oddi
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Long common duct channel (8 to 20mm)
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Reflux of pancreatic juice into the biliary tree
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Intraductal activation of proteolytic enzymes
Biliary epithelial damage, inflammation, ductal distension
• cyst formation
Funabiki T, Matsubara T, Miyakawa S, Ishihara S. Pancreaticobiliary maljunction and carcinogenesis to biliary and pancreatic malign
ancy. Langenbecks Arch Surg 2009; 394:159.
Li L, Yamataka A, Yian-Xia W, et al. Ectopic distal location of the papilla of vater in congenital biliary dilatation: Implications for patho
genesis. J Pediatr Surg 2001; 36:1617.
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PAEDIATRIC PRESENTATION
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The majority of patients with biliary cysts present before
the age of 10 years with
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Conjugated hyperbilirubinemia (80 percent)
Failure to thrive, or an abdominal mass (30 to 60 perc
ent)
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Lipsett PA, Pitt HA, Colombani PM, et al. Choledochal cyst disease. A changing pattern of presentation. Ann Surg 1994; 220:644.
Singham J, Yoshida EM, Scudamore CH. Choledochal cysts: part 2 of 3: Diagnosis. Can J Surg 2009; 52:506.
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PRESENTATION IN ADULTS
• Chronic and intermittent abdominal pain (50 to 96
percent)
• Intermittent jaundice and recurrent cholangitis (34 to 55
percent)
• Abdominal mass (10 to 20 percent)
• Pancreatitis (20 percent)
• Biliary lithiasis (8 percent).
Singham J, Schaeffer D, Yoshida E, Scudamore C. Choledochal cysts: analysis of disease pattern and optimal treatment in
adult and paediatric patients. HPB (Oxford) 2007; 9:383.
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Classic Triad of
•Abdominal pain
•Jaundice
•RUQ mass
seen in only 10-20 % cases
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Rarely, biliary cysts present with
• Intraperitoneal rupture
• Bleeding due to erosion into adjacent vessels
• Portal hypertension
• Secondary biliary cirrhosis due to prolonged biliary
obstruction and recurrent cholangitis.
• In addition, type III cysts can case gastric outlet
obstruction due to the obstruction of the duodenal
lumen or intussusception.
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COMPLICATIONS
• Cholangitis
• Biliary stone formation
• Anastomotic stricture
• Residual debris in the intrahepatic bile
ducts
• Intrahepatic bile duct dilatation
• Malignancy
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CANCER RISK
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Cancer Risk by Age
 0.7% in patients under 10 years of age,
 6.8% in patients 11 to 20 years of age,
 14.3% in patients over 20 years of age
 10 to 30% (16 percent in the largest report) , with a mean
age at diagnosis of 32 years
 50% has been reported in older patients
Singham J, Yoshida EM, Scudamore CH. Choledochal cysts: part 2 of 3: Diagnosis. Can J Surg 2009; 52:506.
Søreide K, Søreide JA. Bile duct cyst as precursor to biliary tract cancer. Ann Surg Oncol 2007; 14:1200.
Todani T, Watanabe Y, Toki A, Urushihara N. Carcinoma related to choledochal cysts with internal drainage operations. Surg Gynecol
Obstet 1987; 164:61.
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Cancer Risk by Pathology
 68% in type I cysts,
 21% in type IV cysts .
 5 and 2% in types II & type III respectively. Cancer in
patients with type III cysts may be limited to those with
choledochoceles lined by biliary, rather than duodenal, epithelium
 7 to 15% in type V Caroli disease
Todani T, Tabuchi K, Watanabe Y, Kobayashi T. Carcinoma arising in the wall of congenital bile duct cysts. Cancer 1979;
44:1134.
Ohtsuka T, Inoue K, Ohuchida J, et al. Carcinoma arising in choledochocele. Endoscopy 2001; 33:614.
Dayton MT, Longmire WP Jr, Tompkins RK. Caroli's Disease: a premalignant condition? Am J Surg 1983; 145:41.
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Cancer Risk Post Operative
• 0.7 to 6 percent due to remnant cyst
tissue
• Undetected,
subclinical
malignant
disease at the anastomotic site in the
pancreas
Ono S, Fumino S, Shimadera S, Iwai N. Long-term outcomes after hepaticojejunostomy for choledochal cyst: a
10- to 27-year follow-up. J Pediatr Surg 2010; 45:376.
Eriguchi N, Aoyagi S, Okuda K, et al. Carcinoma arising in the pancreas 17 years after primary excision of a
choledochal cysts: report of a case. Surg Today 2001; 31:534.
+Investigations
No laboratory studies are specific for the diagnosis of a
choledochal cyst
• Complete blood count with differential cell Count
• Liver function studies:
•Elevated hepatocellular enzyme
•Alkaline phosphatase levels are nonspecific for
choledochal cysts
• Serum amylase and lipase levels
• Serum Electrolytes
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RADIOLOGICAL
•Transabdominal ultrasound
•Computed tomography
•CT cholangiogarphy
•Endoscopic ultrasound
•Intraductal ultrasound
•Endoscopic retrograde cholangiopancreatography
•Magnetic resonance cholangiopancreatography
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Transabdominal ultrasound
• First imaging modality used for the evaluation
• Can not detect type III and type V cysts.
• Sensitivity of 71 to 97 %
• Factors that may limit the usefulness of an ultrasound
include the patient's body habitus, the presence of bowel
gas, and limited visualization due to overlying
structures.
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Computed tomography
 CT can detect all types of biliary cysts.
 Can evaluate for the presence of malignancy.
 It is also useful for determining the extent of intrahepatic disease in
patients with type IVA or V cysts.
It has high sensitivities for visualizing the biliary tree (93 percent),
diagnosing biliary cysts (90 percent), and diagnosing intraductal stones
(93 percent). However, its sensitivity is lower for imaging the pancreatic
duct (64 percent).
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Endoscopic ultrasound (EUS)
 Can demonstrate extrahepatic biliary cysts and provide
detailed images of the cyst wall and pancreaticobiliary
junction.
 It is not limited by body habitus, bowel gas, or overlying
structures.
Intraductal ultrasound (IDUS)
 Diagnosis of early malignant changes in a biliary cyst
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Cholangiography
• Direct cholangiography (whether intraoperative,
percutaneous, or endoscopic) has a sensitivity of up to 100
percent for diagnosing biliary cysts and previously was a
commonly obtained test.
• Can identify abnormal pancreatobiliary junction, and filling
defects due to stones or malignancy.
• Increase risk of cholangitis and pancreatitis.
[ Patients
with cystic disease are greater risk for these complications ]
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Magnetic resonance cholangiopancreatography [MRCP]
• Does not have the risks of cholangitis and pancreatitis as direct
cholangiography
• Sensitivity 73 - 100 %.
• The data are variable with regard to its ability to diagnose an
abnormal pancreatobiliary junction. [46-75%]
•In Unobstructed cases it is the Gold Standard and Investigation of
Choiced
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Hepatobiliary scintigraphy
•Using radio-labeled dyes : technetium-99m-labeled hepatic
iminodiacetic acid (HIDA), which is selectively taken-up by
hepatocytes and excreted into the bile.
•HIDA scanning is useful for extrahepatic cysts, with a
sensitivity up to 100% for type I cysts. However, it is
inadequate at visualizing the intrahepatic bile ducts
•HIDA scanning may also be useful in cases of cyst rupture
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CT cholangiography
• It has high sensitivities for visualizing the
•Biliary tree (93%),
•Biliary cysts (90%),
•Intraductal stones (93%)
• However, its sensitivity is lower for imaging
the pancreatic duct (64 %)
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SURGICAL
TREATMENT
Surgical resection/drainage remains the
mainstay of management in choledochal cyst
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SURGICAL TREATMENT
Type I: Complete excision of the involved portion of the extrahepatic bile
duct; a Roux-en-Y hepaticojejunostomy is performed to restore biliaryenteric continuity
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SURGICAL TREATMENT
Type II:
Complete excision of the dilated diverticulum comprising a type II
choledochal cyst; the resultant defect in the common bile duct is closed
over a T-tube
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SURGICAL TREATMENT
Type III (choledochocele):
 Choledochoceles measuring 3 cm or less:
 Endoscopic sphincterotomy,
 Lesions larger than 3 cm (which typically produce some
degree of duodenal obstruction):
 Transduodenal approach—if the pancreatic duct
enters the choledochocele, reimplantation into the
duodenum may be required following excision of the
cyst
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SURGICAL TREATMENT
Type IV:
Complete excision of the dilated extrahepatic duct
Roux-en-Y hepaticojejunostomy to restore continuity
Segmental resection: intrahepatic ductal disease does not require
dedicated therapy unless hepatolithiasis, intrahepatic ductal strictures, and
hepatic abscesses are present (in such instances, resection of the affected
hepatic segment or lobe is performed)
 Percutaneous hepaticojejunostomy: in diffuse involvement, may permit
repeated stone extractions and dilations
 Surgical unroofing of intrahepatic cysts has been reported
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SURGICAL TREATMENT
Type V (Caroli disease):
 Hepatic lobectomy for disease limited to one
hepatic lobe (left lobe usually affected)
 Liver transplant: patients with bilobar disease
manifesting signs of liver failure, biliary cirrhosis, or
portal hypertension may require liver
transplantation
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l
Lilly technique:
 When the cyst adheres densely to the portal vein secondary to long-standing
inflammatory reaction
 Serosal surface of the duct is left adhering to the portal vein
 The mucosa of the cyst wall is obliterated (mucosectomy) by curettage or cautery
 Theoretically, this removes the risk of malignant transformation in that segment
of the duct.
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Alternatives to surgery
•In patients who refuse surgical resection or who are poor
surgical candidates, lesser interventions (such as ERCP)
may treat symptoms caused by gallstones or sludge.
•No proven effective method of screening biliary cysts for
dysplasia or intramucosal cancer. If screening is attempted,
an intraductal ultrasound is probably the most sensitive
test for detecting early malignancy in the cyst wall.
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Follow Up
Lifelong follow-up because of the increased risk
of cholangiocarcinoma(10-15%), even after
complete excision of the cyst
(even at site of anastomosis)
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Complication of hepaticojejunostomy
• Stenosis
of
the
biliary-enteric
anastomosis (25% cases) leading to
• Cholangitis
• Jaundice
• Cirrhosis
• Patients should be monitored for evidence
of stricture formation with annual serum
liver function tests
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Robotic surgery for type I Choledochal Cyst
• 10 Cases
• The operation time was 321 min in laparosco
pic cases, while 489 min in robotic surgery ca
ses
Naitoh T1, Morikawa T2, Tanaka N2, Aoki T2, Ohtsuka H2, Okada T2, Sakata N2, Ohnuma S2, Nakagawa K2, Haya
shi H2, Musha H2, Yoshida H2, Motoi F2,Katayose Y2, Unno M2.
•
J Robot Surg. 2015 Jun;9(2):143-8. doi: 10.1007/s11701-015-0504-5. Epub 2015 Feb 26.