a case recently operated in Benazir Bhutto Hospital Rawalpindi, Pakistan

1. Case discussion
Dr Muhammad Ihtesham
PGT SU2
BBH RWP.

2. Brief history
Patient rubab, 13 year old female, presented
through opd with C/O
Pain epigastrium for 2 years
vomiting on and off for 2 years

3. History of presenting complaints
Epigastric pain was gradual in onset, severe in
intensity and radiating to back. Pain was related
to intake of fatty and spicy diet and relieved by
taking medicine.
Vomiting was non projectile and related to pain
and yellowish green in colour containing food
particles.

4. • There was no history of weight loss,
dysphagia, anorexia, bleeding PR,
hematemesis, yellowish discoloration of
sclera, clay colored stool or dark colored urine.
• No history of flatulence or heart burn.

5. Systemic inquiry
• Respiratory system: not significant
• Cardiovascular system: not significant
• Gastrointestinal system: pain
epigastrium, vomiting, nausea, on
and off constipation, indigestion
• Nervous system: not significant
• Musculoskeletal system: not significant

6. Previous history
• There is no significant previous history, drug
history of family history.
• Previous medical and surgical history is also
insignificant

7. General physical examination
• BP= 110/70
• Pulse= 80/min
• Temperature= A/F
• R/R= 16/min
• There was no clinical jaundice, cervical or
axillary lymph adenopathy, anemia or dilated
veins in neck. Rest of the the GPE is normal.

8. Systemic examination
GIT
 Inspection: Non distended abdomen, central umbilicus,
normal hair distribution. No visible dilated veins or pulsations
 Palpation: Abdomen soft non-tender, no visceromegaly or
mass palpable
 Percussion: resonant note, no ascites detected
 Auscultation: Bowel sounds were audible
• Respiratory system: normal vesicular breathing
• CNS: conscious and oriented
• CVS: S1+S2+0

9. Differential diagnosis
• Acid peptic disease
• Gastritis
• Esophagitis
• Acute cholecystitis
• Acute pancreatitis
• Choledochal cyst
• Pancreatic divisum

10. Investigations
• Blood cp:
Hb=12g/dl
TLC=7*103
• LFT’s
TBIL=0.8
ALT=22
ALP=74
• RFT’s= WNL
• PT/APTT= WNL

11. USG abdomen
• It showed cystic area of 4.8 * 3.1cm seen in
proximal part of CBD/CHD.
• Rest of the USG was normal

12. MRCP
• Well defined cystic lesion with calculi, lying
adjacent to common bile duct. Features
suggestive of choledochal cyst type 2.
• Contracted GB with cholelithiasis.

14. Diagnosis
Type 1 diffuse choledochal cyst

15. Management
Excision of the choledochal cyst with Roux en y
loop formation

16. Per op findings
• Fusiform choledochal cyst starting from about
1.5cm proximal to insertion into duodenum
and extending all the way up to CHD.
• Cystic duct opening into the cyst. All the
planes surrounding the cyst were well
preserved.
• Abdominal drain placed in subhepatic space.

19. Post op status of the patient
• Patient is maintaining the vitals throughout
the post op period.
• Urine output is adequate.
• Drain output: day 1= 200ml/ 24hrs
day 2=350ml/24hrs
day 5= 1000ml/ 24hrs
day 6=

20. Post op investigations
• Hb= 8.9g/dl
• Hct = 27
• Plt= 189* 103
• TBIL= 0.8
• ALP= 78
• Urea = 18
• Creat= 0.3
• Na+ = 136
• K+ = 3.8
• Cl- = 97
• S. amylase= 3267 and 499
• S.lipase= 318

21. Active complaints
• 2 spikes of high grade fever
• Nausea
• Productive cough

22. Discussion
• Can ERCP and PTC be useful techniques for
the hepatobiliary imaging ?

23. Literature review
 Choledochal cysts are congenital cystic dilatations
of the extrahepatic and/or intrahepatic biliary
tree. They are rare—the incidence is between
1:100,000 and 1:150,000 in populations of
Western countries.
 Choledochal cysts affect females three to eight
times more often than males. Although
frequently diagnosed in infancy or childhood, as
many as one half of the patients have reached
adulthood when diagnosed.

24. Cause
 weakness of the bile duct wall and increased
pressure secondary to partial biliary
obstruction are required for biliary cyst
formation.
 Adults commonly present with jaundice,
cholangitis or pancreatitis.
 Less than one half of patients present with the
classic clinical triad of abdominal pain,
jaundice, and a mass.

25. Types of choledochal cysts

26. Caroli’s disease
• Type VI( both intra and extrahepatic)
choledochal cyst which is a hereditary
disorder, is known as Caroli disease and
carries a poor prognosis from recurrent
cholangitis and the development of
intrahepatic stones, liver abscess formation
and, eventually, cirrhosis.

27. Grumbach disease
• In some patients, multiple intrahepatic cystic
disease is accompanied by congenital hepatic
fibrosis. This condition is known as Grumbach
disease.

28. How to investigate
Ultrasonography or CT scanning will confirm the
diagnosis, but endoscopic cholangiography,
transhepatic cholangiography, or MRCP is
required to assess the biliary anatomy and to
plan the appropriate surgical treatment.

29. Treatment
 For types I, II, and IV, excision of the
extrahepatic biliary tree, including
cholecystectomy, with a Roux-en-Y
hepaticojejunostomy is ideal.
 In type IV, additional segmental resection of
the liver may be appropriate, particularly if
intrahepatic stones, strictures, or abscesses are
present or if the dilatations are confined to one
lobe.

30. • The risk of cholangiocarcinoma developing in
choledochal cysts is as high as 15% in adults
and supports complete excision when they are
diagnosed. For type III, sphincterotomy is
recommended.

31. Thank you