case presentation

2. Demographic Profile.
• Name Hafsa Saeed D/O Mohammad Saeed
• Age/Sex 22years/Female
• Occupation: Teacher
• Address: Wazirabad
• Date of Admission: 19/09/16 through OPD

3. Presenting Complaints.
• Vomiting 4 months

4. History Of Presenting illness.
• The patient was refered from Liver Clinic to Surgical
OPD. She was in her usual state of health about 4
months back when she started having vomiting..
• Vomitus being yellow in colour
• Occurring once a week
• 20-30 minutes after food intake
• Containing food particles
• Half cup in quantity.

5. • There is no history of abdominal pain.
• There is no history of anorexia, fever, rigors or chills
or sweating.
• There is no history of yellowish discoloration of
sclera, melena, bleeding per rectum, hemetemesis or
haemoptysis.
• There is no history of weight loss.
• There is no history of diarrhea, constipation.
• There is no history of shortness of breath, headache,
bone pain.

6. Systemic Inquiry.
• Gastrointestinal System
• Vomiting, Nausea, vomiting, abdominal pain, diarrhea,
constipation, hemetemesis, melena, jaundice
• Respiratory System
• Cardiovascular System Insignificant
• Respiratory System
• Urogenital System

7. Past Medical & Surgical History.
• No history of Diabetes Mellitus, Hypertention,
Ishcemic Heart Disease, Asthma, Tuberculois or any
malignancy
• No history of any blood transfusions.
• No history of previous surgery.

8. Family History.
• Not Significant
Drug History And Allergy
• Not Significant
• No known allergies.

9. Personal History.
• Non smoker
• Non addict
• Normal bowel and sleep
habits
• Unmarried
• Teacher by Profession
Socio Economic History.
• Belongs to middle class
family

10. General Physical Examination.
• A young female lying in bed, well oriented in
time, place and person with following…
• Vitals:
• Pulse 80/min
• BP 110/70
• Temp 99 F
• R/R 16/min

11. • Pallor Negative
• Jaundice Negative
• Cyanosis Negative
• Clubbing Negative
• JVP Not Raised
• Lymph Nodes Not Palpable
• Thyroid Not Enlarged

12. Systemic Examination.
Gastrointestinal System:
• Flat abdomen moving with respiration, umblicus central and
everted.
• No visible pulsations, swelling, scar marks, veins
• Hernial orifices intact
• Soft, non tender abdomen
• No mass palpable.
• No visceromegaly
• Bowel sounds present

13. Systemic Examination.
• Respiratory System:
• Normal Vesicular Breathing on both sides
• Cardiovascular System:
• S1+S2+0
• Central Nervous System:
• GCS 15/15, Concious, oriented

14. Summary.
• A 22 years old female referred to surgical floor
from Liver clinic with complaints of Vomiting
for the last 4 month with no other associated
symptoms and no significant finding on clinical
examination.

15. Differential Diagnosis.
• Acid Peptic Disease.
• Cholelithiasis.

16. Investigations.
investigations 20-September-2016
HB 10.3
TLC 8.3
Platelets 270
Total Bilirubin 0.5
ALT 16
ALP 185
Urea 15
Creainine 0.8
PT 13/13
APTT 33/33
Albumin 4.0

17. Abdominal Ultrasound.
• Fusiform dilatation of
common bile duct.
• Type 1 choledochal cyst

18. CT Scan ABDOMEN WITH
CONTRAST.
• Cholelithiasis
• Fusiform dilatation of
proximal common bile
duct, Type 1
Choledochal cyst.

19. MRCP
• Cholelithiasis.
• Fusiform dilatation of
proximal CBD with
multiple calculi, Type 1
Choledochal cyst.

20. Final Diagnosis.
• Type 1 Choledochal Cyst with Cholelithiasis.

21. Management.
• The patient was counseled regarding the disease and
its management.
• Anesthesia fitness was done.
• Blood and blood products were arranged.
• Open cholecystectomy with excision of cyst and
Roux-en-y Hepaticojejunostomy was performed on
29/09/2016.

22. Post Operative Period.
Zero Post Op Day:
The patient was shifted to PACU.
• Vitals:
Pulse 90/min
BP 120/80
Temp A/F
Drain 150ml
Urine output adequate
Adequate analgesia via epidural was given.

23. Literature Review.

24. Choledochal Cyst
• Choledochal cysts represent congenital cystic dilatations of
the Biliary Tree. Diagnosis relies on the exclusion of other
conditions (e.g. tumour, gallstones, inflammation) as a cause
of biliary duct dilatation.

25. • Cystic diseases of the biliary tree are rare.
• Aetiology remains unknown.
• Thought to represent congenital weakness of
common bile duct with distal obstruction.
• Another theory postulates unequal proliferation of
duct epithelium.

26. • Higher incidence among Japanese.
• Although they may be discovered at any age, 60% are
diagnosed before the age of 10 years.
• Strong female predominence, 70% cases reported in
West, M:F ratio of 1:4.

27. Pathology.
• Their aetiology is uncertain but a close association
with anomalous formation of the pancreaticobiliary
ductal junction is reported in some subtypes.
• Due to this anomaly, there is a large common
channel draining pancreatic and bile duct.
• Pancreatic juices cause cholangitis and bile duct wall
destruction, which together with distal stenosis due
to scarring, result in formation of choledochal cyst.

28. Types of Choledochal Cyst.
• Type I (Commonest)
• Diffuse cystic dilatation
• Type II
• Localized dilatation of supraduodenal bile duct.
• Type III
• Supraduodenal diverticulum.
• Type IV
• Intraduodenal dicerticulum or Choledochocle.
• Type V
• Solitary intrahepatic cyst.
• Type VI (Caroli Disease)
• Multiple intrahepatic cysts.
• Type VII
• Multiple intra- & extrahepatic cysts

29. Todani Classification.

30. Type I Choledochal Cyst

31. Type II Choledochal Cyst

32. Type III Choledochal Cyst
(Choledochocele).

33. Type IV Choledochal Cyst
(Extrahepatic And Intrahepatic Disease).

34. Type V choledochal cyst
(Caroli disease).

35. Caroli Disease.
• Hereditary disorder, autosomal recessive.
• Poor prognosis.
• Recurrent cholangitis, intrahepatic stones, liver abscess
and eventually cirrhosis.
• Intrahepatic cystic disease is, sometimes, accompanied
by congenital hepatic fibrosis. This condition is called
“Grumbach Disease.”

36. Clinical Features.
• Classically presentation includes the triad of
• Cholestatic Jaundice
• Abdominal mass
• Pain
• This triad is however only present in 40% (range 19-
60%) of cases, with palpable mass being the least
common manifestation.

37. Complications:
• Calculous disease (most common).
• Recurrent cholangitis.
• Pancreatitis
• Hepatic abscess formation
• Biliary cirrhosis.
• Rupture of cyst with biliary peritonitis (rare).
• Cholangiocarcinoma (life time incidence 10-15%)
• Portal vein thrombosis.

38. Investigations.
(ultrasound, CT, direct contrast studies (ERCP, PTC) or MRI.)

39. Laboratory Investigations.
No laboratory studies are specific for the diagnosis of a choledochal cyst.
• CBC count: An elevated white blood cell count with increased numbers of
neutrophils and immature neutrophil forms may be observed in the
presence of cholangitis.
• Liver function tests may be useful in narrowing the differential diagnosis.
Hepatocellular enzyme and alkaline phosphatase levels may be elevated.
• Serum amylase and lipase concentrations may be increased in the
presence of pancreatitis. Serum amylase concentrations also may be
elevated in biliary obstruction and cholangitis.
• Serum chemistry may be abnormal if the patient is vomiting.

40. Ultrasound.
• Key to the diagnosis is a dilated cystic lesion which
communicates with the bile duct and is separate
from the gall bladder.
• Careful search for other causes needs to also be
undertaken, keeping in mind that stone formation
and malignancy are associated with choledochal
cysts.

41. CT Scan & MRCP & ERCP.
• Findings are similar to Ultrasound, with a greater
ability to demonstrate intrahepatic disease.
• CT with IV Contrast mixing in the cyst may be
inhomogeneous. It does however have the
advantage that it conclusively demonstrates
communication with the biliary tree.
• Demonstrates the anomalous junction of the bile and
pancreatic ducts and its angle.

42. Computed tomography (CT) scan of large, saccular type I choledochal cyst

43. Treatment.
• Treatment of choice is Surgical Excision with
Roux-en-Y anastomosis.

44. Medical Care.
• Appropriate antibiotic therapy and supportive care
should be given to patients presenting with
cholangitis.
• Patients who present at a late stage, after the
development of advanced cirrhosis and portal
hypertension, may not be good candidates for
surgery because of the prohibitive morbidity and
mortality associated with these co-morbid
conditions.

45. • No medical therapy specifically targets the etiology of
choledochal cysts, nor is any drug or any type of
nonsurgical modality curative.
• Patients who present with Cholangitis should be
treated with broad-spectrum antibiotic therapy
directed against common biliary pathogens, such as
Escherichia coli and Klebsiella species, in addition to
other supportive measures, such as volume
resuscitation.
• Again, it must be emphasized that these means are
supportive and that surgery is the only currently
available definitive therapy.

46. Surgical Care.
• The treatment of choice for choledochal cysts is
complete excision of the cyst with construction of a
biliary-enteric anastomosis to restore continuity with the
gastrointestinal tract.
• According to Jordan and associates, both partial
resection of the cyst and internal drainage procedures
expose patients to increased risks of cholangitis,
pancreatitis, and cholangiocarcinoma.
• Ulas M, Polat E, Karaman K, et al. Management of choledochal cysts in adults: a retrospective analysis of 23
patients. Hepatogastroenterology. 2012 Jun. 59(116):1155-9.
• Edil BH, Olino K, Cameron JL. The current management of choledochal cysts. Adv Surg. 2009. 43:221-32

47. Surgical Management By
Choledochal Cyst Type

48. Type I Choledochal Cyst.
• The treatment of choice is
complete excision of the
involved portion of the
extrahepatic bile duct. A
Roux-en-Y
hepaticojejunostomy is
performed to restore
biliary-enteric continuity.
• Ahn SM, Jun JY, Lee WJ, et al. Laparoscopic total
intracorporeal correction of choledochal cyst in pediatric
population. J Laparoendosc Adv Surg Tech A. 2009 Oct.
19(5):683-6.

49. Roux-en-Y hepaticojejunostomy to restore biliary-enteric continuity following resection of
choledochal cyst

50. Type II Choledochocele.
• The dilated diverticulum
comprising a type II
choledochal cyst is
excised in its entirety.
The resultant defect in
the common bile duct is
closed over a T-tube.

51. Type III Choledochal Cyst
(choledochocele).
• The choice of therapy depends
upon the size the cyst.
• Choledochoceles measuring 3
cm or less can be treated
effectively with endoscopic
sphincterotomy.
• Lesions larger than 3 cm
typically produce some degree
of duodenal obstruction. These
lesions are excised surgically
through a transduodenal
approach.
• If the pancreatic duct enters the
choledochocele, it may have to
be re-implanted into the
duodenum following excision of
the cyst.

52. Type IV Choledochal Cyst
• The dilated extrahepatic duct
is completely excised and a
Roux-en-Y
hepaticojejunostomy is
performed to restore
continuity.
• Intrahepatic ductal disease
does not require dedicated
therapy unless hepatolithiasis,
intrahepatic ductal strictures,
and hepatic abscesses are
present. In such instances, the
affected segment or lobe of
the liver is resected.

53. Type V Choledochal Cyst
(Caroli Disease)
• Disease limited to one hepatic
lobe is amenable to treatment by
hepatic lobectomy. When this
occurs, the left lobe usually is
affected.
• Hepatic functional reserve should
be examined carefully in all
patients before committing to
such therapy.
• Patients with bilobar disease who
begin to manifest signs of liver
failure, biliary cirrhosis, or portal
hypertension may be candidates
for liver transplantation.

54. Lilly Technique.
• Occasionally, the cyst adheres densely to the portal vein
secondary to long-standing inflammatory reaction.
• In this situation, a complete, full-thickness excision of the
cyst may not be possible.
• In the Lilly technique, the serosal surface of the duct is
left adhering to the portal vein, while the mucosa of the
cyst wall is obliterated by curettage or cautery.
Theoretically, this removes the risk of malignant
transformation in that segment of the duct.

55. Prognosis.
• Prognosis is excellent after complete excision
of cyst.

56. • Shimotakahara and coworkers compared Roux-en-Y
hepaticojejunostomy to hepaticoduodenostomy for
biliary reconstruction following choledochal cyst
excision.
• They concluded that hepaticojejunostomy was a
better choice because of an unacceptably high rate
of duodenogastric bile reflux (33.3%) in the
hepaticoduodenostomy group.
• Mukhopadhyay B, Shukla RM, Mukhopadhyay M, et al. Choledochal cyst: A review of 79 cases and the role of
hepaticodochoduodenostomy. J Indian Assoc Pediatr Surg. 2011 Apr. 16(2):54-7.

57. Recent
Advances.

58. Jang JY, Kim SW, Han HS, Yoon YS, Han SS, Park YH. Totally laparoscopic
management of choledochal cysts using a four-hole method. Surg Endosc.
2012 Nov. 20(11):1762-5.
• Jang and coauthors described their experience with
laparoscopic surgical management of choledochal cysts in a
series of 12 adult patients (mean age=37.3 y).
• Complete cyst excision and reconstruction via Roux-en-Y
hepaticojejunostomy was accomplished in all patients using
laparoscopic techniques.
• No mortalities and no anastomotic complications occurred.
• The mean operative time was 228 minutes. Patients were
discharged from the hospital after an average stay of 5.8 days.

59. Woo R, Le D, Albanese CT, Kim SS. Robot-assisted laparoscopic
resection of a type I choledochal cyst in a child. J Laparoendosc
Adv Surg Tech A. 2013 Apr. 16(2):179-83.
• Reports of Robotically-assisted laparoscopic resection of
choledochal cysts are beginning to appear.
• Woo and colleagues reported such a case in the management
of a 5-year-old child with a type I choledochal cyst.
• The cyst was excised successfully.
• The total robotic operative time was 390 minutes, and the
time for the entire procedure was 440 minutes.
• No complications occurred. The patient was reported well
after 6 months of follow-up.

60. Meschino M, García-Ochoa C, Hernandez-Alejandro R.
Hepatobiliary Surg Nutr.
2015 Feb;4(1):E8-E12. doi: 10.3978/j.issn.2304-3881.2014.08.09.
• We present the rare case of a RUPTURED CHOLEDOCHAL
CYST (CC) in a young woman presenting with a two-day
history of worsening upper abdominal pain.
• Imaging revealed a contracted gallbladder, dilated common
bile duct (CBD), and a large amount of peritoneal fluid.
• Percutaneous paracentesis was performed, obtaining
bilious fluid. Further imaging revealed cystic dilatation of
the CBD and the diagnosis of rupture CC type I was made.

61. • The patient was initially managed conservatively with percutaneous
drains, IV antibiotic therapy, and sphincterotomy through an ERCP.
• Elective cyst resection and Roux-en-Y hepatojejunostomy was
performed 8 weeks later. It is important to differentiate a ruptured
CC from other surgical emergencies without exploratory
laparotomy.
• Initial conservative management could be considered, followed by
elective resection once inflammation, infection, and other
complications have resolved, avoiding the increased risk associated
with an emergency operation or two-stage laparotomy.