DR.

PAWAN SAPKOTA
A CASE OF
MBBS, MS (BPKIHS)
BHARATPUR HOSPITAL
CHOLEDOCHAL
CYST
SEQUENCE OF FLOW
• Case introduction
• Discussions
• Conclusions
• References
CASE INTRODUCTION..

• 10 Y/M
• complaints of: pain over right upper quadrant of abdomen for 1 day,

- sudden, dull aching, gradually progressive, aggravated on supine,

fever for 1 day and
vomiting for 1 episode (nonbilious)
O/E:
• Vitals-stable, PILCOD- nil
• P/A: soft and mild tenderness over right hypochondrium, non distended and no mass appreciated
Advised for routine Ultrasonography:
Findings- A simple cyst of 62 x 32 mm was noted in the right lobe of the liver with septa and
internal echoes. No communication with the biliary system. ?hydatid cyst
Biochemical profile:
• TLC- 8090N53
• Hb-13
• TB/DB- 0.3/0.1
• SGPT/SGOT/ALP: 5.7/22.9/203
Cect abdomen and Pelvis
• Fusiform dilation of the whole course of CBD and CHD measuring 65 x 42 x 37mm.
Mild dilatation of central IHBDS likely Choledochal cyst Type I

MRCP:
• Liver is normal in size. Normal signal intensity of liver parenchyma is seen. No evidence
of IHBRD noted. No mass lesion seen.
• Gall bladder is well distended with normall wall thickness. Minimal sludge isn noted in
GB lumen. Fusiform dilation of the extrahepatic CBD measuring 6.9 x 4.5 cm is seen. No
evidence of filling defects noted within lumen of CBD. Likely choledochal cyst IA
• Pancreas is normal in size. Normal parenchymal intensity seen.
OT PROCEDURE

• Exploratory laparotomy + excision of cyst + Roux-en-Y HJ Under GA on 2080/03/06

• Findings: Normally distended gallbladder with gross dilation of CBD 6x4 cm up to the
hepatic confluence
• Post operatively patient was shifted to ICU and then shifted to ward on 2 nd
day.
• Hospital stay was uneventful besides some hypoglycemic episodes which
was managed accordingly.
• Patient was discharged on 5th day of admission
• Routine follow-up was made on 7th day of discharge and on 1 month
• Biopsy report: feature suggestive of chronic cholecystitis. Negative for
malignancy:
• At 1 month: patient had episodes of loose motion ~1-2 episodes per day. No
other issues beside that.
DISCUSSIONS..
• Dilatation at any level of the bile duct (average normal diameter 5-6 mm in adult) (1)
• Incidence: Asian (1 in 1000 live births) vs Western population (1 in150000 live births)
• Female to male- 4:1 (7)
ETIOLOGY AND PATHOPHYSIOLOGY

• Long common channel theory by Babbit

• Pancreaticobiliary reflux and activation of proenzymes
• Abdominal pain, vomiting, jaundice, and increased aminotransferases in children
• In one research,20, jaundice if amylase <20U/l and abdominal pain in those with amylase
>200u/l
• In adulthood, PBR presents as dilatation of bile ducts and malignancy
• Site of malignancy- within cyst but can be anywhere within biliary tree
• PBM is present in 50-80% of cases
CLINICAL FEATURES

• Classic triad- abdominal pain, abdominal mass and jaundice-5-10% (33)

• Abdominal pain (61-94%) (36)
• Infantile presentation- intermittent obstructive cholestatic syndrome
• Abdominal mass (52.4% vs 21.2%) and jaundice (33.3% vs 0%) (44)
• Ass with PBM (85.7% vs 59.6%)
• Adult had more abdominal pain than children( 71.8% vs 40.7%)
DIAGNOSIS

• Ultrasonography
• Technectium-99 HIDA scan
• Ct cholangiography : sensitivity 90% (48)
• MRCP- sensitivity 90-100% (49)
MANAGEMENT RATIONALE (NEW)

• Medical management: antibiotics for cholangitis or supportive therapy for pancreatitis

• Malignancy- 6% to 30% in adult with CC
• Life-long malignancy risk after excision- 4%
• Higher in APBJ
SURGICAL PRINCIPLES (NEW)

• Historically- cystenterostomy or drainage procedure

• Cyst excison and bilioenteric reconstruction ( Roux-en-Y HJ or HD)
• Disadvantage of HD is bile reflux (5%) (59)
INTRAOPERATIVE
CHOLANGIO
PANCREATICOGRAPHY
OUTCOMES AND COMPLICATIONS

• Hepaticoenterostomy vs Cystenterostomy
• MC complications- anastomotic leakage, anastomotic stricture (2.5-17%) and cholangitis (23-
40%) (68)
• Perioperative morbidity in adult and children (35.1% vs 16.3%) (36)
• Adult: Seroma (3.1%), wound infection (9.7%), perihepatic abscess (7.7%)
• Children: anastomotic leaks(3%) and gastrointestinal tract perforation (3%) (36)
• Laparoscopic vs open procedure
• For stricture, balloon dilation should be first step; success rate 81% (69)
• Long term follow-up warranted
• Malignant degeneration more often in type I and IV cysts (46)
• Biliary cancer reported in 5-10% of patients
• After complete surgical excision- 0.7-5.4%
• 5 year survival rate: 95.5% (adults 94.6%; children 97.2%) (36)
FOLLOW-UP

• Lifelong follow-up with USG, liver profile and CA19-9 annually recommended
• AST every 4 months for 2 years; then every 6 months for 5 years (46)
CONCLUSIONS

• Is a biliary anomaly often diagnosed at early age

• Early treatment is helpful before serious sequelae arise
• Knowledge of different variants is required to have better results and to minimise
complications
• Follow-up is necessary
REFERENCES

1. Witcombe JB, Cremin BJ. The width of the common bile duct in childhood. Pediatric Radiology. 1978;7:147-149

2. O'Neill JA. Choledochal cyst. Current Problems in Surgery. 1992;29(6):361-410

3. Jung SM, Seo JM, Lee SK. The relationship between biliary amylase and the clinical features of Choledochal cysts in
pediatric patients. World Journal of Surgery. 2012;36:2098-210

4. Shah OJ, Shera AH, Zargar SA, et al. Choledochal cysts in children and adults with contrasting profiles: 11-year experience
at a tertiary care center in Kashmir. World Journal of Surgery. 2009;33:2403-2411

5. Soares KC, Kim Y, Spolverato G, et al. Presentation and clinical outcomes of Choledochal cysts in children and adults: A
multi-institutional analysis. JAMA Surgery. 2015;150(6):577-58

6. Huang CS, Huang CC, Chen DF. Choledochal cysts: Differences between pediatric and adult patients. Journal of
Gastrointestinal Surgery. 2010;14:1105-1110
7. Lam W, Lam TP, Saing H. Cholangiography and CT cholangiography of pediatric patients
with choledochal cysts. AJR. American Journal of Roentgenology. 1999;173:401-405
8. Ndoye NA, Wellé IB, Cissé L, Guèye D, Diouf C, Mbaye PA, et al. Choledochal cyst in
children in Dakar: Diagnostic and therapeutic aspects. African Journal of Paediatric Surgery.
2021;18(3):168-170
9. Wiseman K, Buczkowski AK, Chung SW, Francoeur J, Schaeffer D, Scudamore CH.
Epidemiology, presentation, diagnosis, and outcomes of choledochal cysts in adults in an
urban environment. American Journal of Surgery. 2005;189(5):527-531
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