Choledochal CystS-U 5

Patient Profile• Name - Mahesh Ram• Age - 28 yrs• Sex - Male • Occupation - Labourer• Residence - Dausa• Marital Status - Married• Religion - Hindu• S E status - Class V (BG Prasad)

Presentation

• Abdominal pain, vague dull aching, Right upper part, since

4 months

• Episodes of vomiting, content food particles, about 10 – 12

times over 4 months

• Yellowish discolouration of skin and eyes since 2 months• No h/o fever, alteration in stool

colour/consistency/frequency, trauma, other significant complaints

Past History• ERCP on the 23rd October in SMS Dept of

Gastroenterology

Personal History• Loss of appetite (+)• Non smoker, Chews tobacco• Non alcoholic• Vegetarian• Undisturbed sleep• Regular bowel and bladder habits

General Physical Examination• Thin built and moderately nourished• Conscious, Cooperative, Well oriented

• Vital Statistics were Normal

• Pallor (-)• Icterus (+) (mild)• Clubbing (-)• Acyanotic• No palpable lymph nodes• No Edema

Local Examination • Per Abdomen –

INSPECTION

• Abdomen scaphoid, umbilicus central, • No visible lumps, pigmentation/discolouration• No visible veins, pulsations, peristalsis, scars• Hernial sites intact

Local Examination PALPATION

Abdomen soft, non-distendedTenderness in the Right Hypochondrium No rise of temperature, guarding / rigidityNo organomegaly, palpable lumps

PERCUSSION

Abdomen tympanic all over No free fluid Liver dullness in the 5th ICS with a span of 6cm

Local Examination

AUSCULTATION

• Normal bowel sounds heard with a frequency of 5-6/min• No bruit heard

SYSTEMIC EXAMINATION –

NORMAL

Investigations • CBC – Normal

• LFT – Sr Bilirubin – 1.5 mg/dl (D – 0.6; I – 0.9 mg/dl) , SGOT & SGPT – Normal

• Coagulation profile – Normal ; Sr. Alkaline Phosphatase – Normal

• Ultrasound (outside) - ? Double gb with calculi

• USG abdomen(SMSH) – 3 x 3 cm hypoechoic area with increased vascularity seen in the GB s/o GB fossa mass ; 3 x 2 cm Fusiform dilatation of the CBD s/o Choledochal cyst

• USG Guided FNAC was attempted - Unsuccessful

• CECT abdomen – Two cystic structures at the porta, one showing solid intraluminal lesion ? Double Gallbladder with mass ? Choledochal cyst with GB mass

Investigations•MRCP –

• Partially distended GB with soft tissue attenuation faceted areas within the lumen extending into cystic duct s/o likely soft calculi. Minimal extrinsic mass effect is noted on adjacent CBD with mild prominence of CHD & central biliary radicles

• ERCP - CBD cannulated, sphincterotomy doneThere is a large Choledochal cyst of the proximal CBD. No CBD stone found. 7 Fr DPT stent placed in CBD.

Operation Details

Laparoscopic Choledochal cyst excision with Laparoscopic-assisted Roux-en-Y

Hepaticojejunostomy GA

Intra Op findings –

• The choledochal cyst involved the supraduodenal CBD extending from CHD as evident in the MRCP

• Gallbladder was distended with ?mass in fundus

ProcedurePORT PLACEMENT - 1) Umbilical ( Camera port)

2) Epigastrium ( Surgeon’s Right Arm)

3) Right Subcostal ( Surgeon’s Left Arm)

4) Mid-Axillary 2 inch below Right costal margin

Steps – • Diagnostic Lap done. Pathology identified. Calot’s triangle dissected. Cystic

artery clipped.• Cystic duct dissected & isolated by looping with umbilical tape• CBD along with Choledochal Cyst dissected meticulously in the lesser

omentum and isolated from common & Right hepatic artery and portal vein• Upper end of the cyst transected at the level of CHD & stent removed• Lower end of the cyst transected after clipping the lower end of normal CBD • GB dissected from GB fossa and extracted along with the choledochal cyst

in a glove

Procedure…contd• Two extra ports placed on the Left & Right of the abdomen for making

the Roux-en-Y loop of Jejunum

• Roux-en-Y loop of jejunum made laparoscopically

• 2-inch long incision given between the epigastric & the right subcostal port

• Hand-sewn Hepaticojejunostomy done

• Drain placed in the Morrison’s pouch. Blood loss – 100-150ml

1

2

3

4

5

6

CYSTIC DUCT

Bile Duct Cyst

Lower end Clipped

Umbilical tape

Bile Duct Cyst

Hepatic Artery

Portal Vein

Duodenum

Upper end

Lower end

Gallbladder

Post Operative Course • Uneventful recovery

• Bowel Sounds present on POD 2

• NG tube removed on POD 3 Oral sips allowed

• Complete oral nutrition on POD 4

HISTOPATHOLOGY REPORT – • Gross – Gallbladder size 10cm, fundus shows grey-white irregular

proliferative growth 5 x 3 cm.

• Microscopy – Gallbladder shows evidence of Well-differentiated Papillary adenocarcinoma in the fundus reaching at one focus upto the muscle [pTIb]. Section from body and neck show chronic non-specific cholecystitis. Sections of the cyst are lined by cuboidal epithelium, wall shows inflammation, congestion, & fibrosis consistent with Choledochal Cyst. No evidence of malignancy seen

• Review report –Lymph Nodes negative for mets

Drain output –POD 1 - 150

ml biliousPOD 2 - 100

ml biliousPOD 3 - 100

ml biliousPOD 4 - 50

ml bilious

DISCUSSION

Choledochal Cyst

Cystic Disease of the Biliary Tree

Bile Duct Cysts

Bile Duct Cysts • Problem of infancy

• Sex predilection

• Bile Duct Cysts in adults

• Consensus on treatment in Extrahepatic & Intrahepatic cysts

Pathogenesis• Anomalous arrangement of the PANCREATOBILIARY duct junction

(A J P B D S)

KIMURA TYPE I -Pancreatic duct

enters the CBD

(10-58%)

KIMURA TYPE II - CBD drains into the

Pancreatic duct

KOMI TYPE I -CBD joins PD at a right angle

KOMI TYPE II -CBD joins PD at an acute angle

KOMI TYPE III - CBD & PD form a complicated network

subdivided according to “WARSHOW classification”

KOMI et al

Long Common Channel Theory• Associated along with an AJPBDS

• Pathological anatomy leading to reflux of enzymes

• Support –• Biliary Manometric Studies (Iwai et al, 1986)• High Pancreatic enzyme levels (Todani et al, 1990)• Histopathology of Cyst wall (Oguchi et al, 1988)

• Oligoganglionosis of the distal CBD

ClassificationTodani et al, 1977 ; Classification of Bile duct cysts

1. Solitary fusiform extrahepatic cyst

2. Extrahepatic supraduodenal diverticulum

3. Intraduodenal diverticulum ( CHOLEDOCHOCELE )

4. a- Fusiform extra & intra-hepatic cysts

b- Multiple extrahepatic cysts5. Multiple intrahepatic cysts

( CAROLI DISEASE )

Clinical Features• Asymptomatic

• Mimics calculus biliary tract disease

• Intermittent, recurring epigastric / hypochondriac pain, fever, jaundice, nausea, vomiting, anorexia

• Abdominal Mass - suspect malignancy

Clinical Features• Hepato-splenomegaly in cirrhosis & portal

hypertension with hematemesis, malena & ascites

• Symptoms of cholangitis & cyst-associated pancreatitis

• Classic triad of Bile duct Cyst – in < 10 % patients

Associated HB Pathology

• Inflammatory -• Cholangitis• Calculous

Cholecystitis• Cyst-associated

Pancreatitis• Cystolithiasis &

Hepatolithiasis• Hepatic Abscess

• Neoplastic• Cholangiocarcinoma• Carcinoma GB• Hepatocellular

Carcinoma• Pancreatic carcinoma

• Miscellaneous• Hepatic Cirrhosis• Portal Hypertension

Investigations• Ultrasound Abdomen

• CECT abdomen

• MRCP

• ERCP

• Direct Cholangiography• PTC

• H I D A scan

E R C P

Management- Principles

• Pre-operative work up - Complete cholangiographic definition

• Control of biliary infection

• Definitive treatment - SURGICAL

• Cyst Excision with Mucosa – mucosa biliary-enteric anastomosis

• Partial cyst excision (along with mucosectomy) with Roux-en-Y cystojejunostomy to an epithelial lined portion of remnant

1. Solitary fusiform extrahepatic cyst ( M C )a) dilatation of extrahepatic bile duct (entire)

b) dilatation of extrahepatic bile duct (focal segment)

c) dilatation of the common bile duct portion of extrahepatic bile duct

Total Cystectomy with Roux-en-Y Hepaticojejunostomy

TREATMENT – TYPE I

TREATMENT – TYPE II2. Extrahepatic supraduodenal diverticulum

EXCISION with PRIMARY REPAIR / T-tube placement

TREATMENT – TYPE III3. Intraduodenal diverticulum ( CHOLEDOCHOCELE )

• Duodenal mucosa

• Bile Duct mucosa

TRUE DIVERTICULUM

• Transduodenal Cyst excision +/- Sphincterotomy• Endoscopic Sphincterotomy with Cyst unroofing

TREATMENT – TYPE IVa) Fusiform extra & intra-hepatic cysts

b) Multiple extrahepatic cysts

EXCISION OF EXTRAHEPATIC CYST

With ROUX-en-Y H J

+/-

HEPATIC SEGMENT RESECTION

TREATMENT – TYPE V5. Multiple intrahepatic cysts ( CAROLI

DISEASE )

• UNILOBAR• DIFFUSE

Depends on -• Congenital Hepatic Fibrosis• Secondary Biliary Cirrhosis

HEPATIC RESECTION +/-

Roux-en-Y Cholangiojejunostomy

• SIMPLE• PERIPORTAL

FIBROSIS

TYPE VI Bile Duct Cyst• Cystic dilatation of the Cystic Duct

• Previously thought to be a subtype of Type 2 bile duct cyst

• Limited number of cases reported

MULTIPLANAR VOLUME RENDER

Modes of Surgery • LAPAROSCOPY• Safe, Effective• Technically challenging

• OPEN• Cyst excision

• Biliary-enteric anastomosis

• Roux-en-Y Hepaticojejunostomy

• Hepaticoduodenostomy?

FOLLOW UP• Long term follow up needed

• Age related risk of malignancy

• Complications - • Ascending Cholangitis• Pancreatitis• Anastomotic strictures • Bowel obstruction• Intrahepatic Bile Duct & terminal CBD (intrapancreatic) Stones• Cirrhosis of the liver & Portal Hypertension

Laparoscopic Advances• Technically challenging surgery

• Follows same principles of open surgery. Proper port placement is of significance & expertise in lap suturing

• Data restricted due to unavailability of series and comparative studies

• Largest series of 35 cases by Dr Palanivelu and colleagues

• Advantages to the patient

• Advantages to the operating surgeon

THANK YOU

ClassificationAlonso-Lej Classification in 1959

1. Fusiform/Saccular dilation of CHD & CBD

2. Supraduodenal diverticulum of CHD / CBD

3. Intraduodenal diverticulum of the distal CBD

(CHOLEDOCHOCELE)