(LECTURES)

JAUNDICE AND SURGERY

Dr.Manoj K.Gupta MBBS,MS,FICS(Uro),FAIS  

manojgupta@surgsocietyddn.cjb.net

There are basically  three instance where a surgeon would be confronted with a jaundiced patient.

* A truly surgical jaundiced patient

* A patient with an acute or a chronic surgical problem who also has concomitant medical jaundice, which may complicate the surgical procedure.

* A jaundiced patient with a positive hepatitis 'B'  surface antigen coming in for an emergency operation. The threat to the surgeon, the operating team, and the nursing personnel is real.

I will be concentrating on management of the surgically jaundiced  patient.

DIAGNOSIS OF SUSPECTED SURGICAL JAUNDICE

Liver Function Test (LFT) :‑ There must be a good definition     

                                   between medical and surgical

                                   jaundice.

Ultra Sono Graphy (USG) :‑ One of the  best screening tests.

                             ‑ Dilated ducts indicate surgical     

                               jaundice.

                             ‑ Undilated ducts do not  entirely

                               rule out surgical jaundice.

Endoscopic Retrograde Cholangio Pancreatography (ERCP):‑

                         ‑ This is the  best method for      

                           diagnosing surgical jaundice.

Percutaneous Transhepatic Cholangiography (PTC) :‑

                         ‑ This is the better  for hilar   

                           lesions , and gives  distal anatomy

                           above the stricture.

Endoscopic Cholangio Retrograde Pancreatography:‑

                        ‑ This the better for distal   lesions or

                          stones .

                          Operative and T tube cholangiography.

CAUSES OF BILIARY DUCT OBSTRUCTION

INTRINSIC

* Ductal calculi

* Acute cholangitis

* Carcinoma of the bile duct (cholangiocarcinoma)

* Carcinoma  of the ampulla of Vater

* Intraductal  secondary tumor seeding

* Benign biliary tumors

* Benign post‑traumatic strictures

* Sclerosing cholangitis (primary or secondary)

* Idiopathic biliary strictures

* Parasites

* Haemobilia

EXTRINSIC

* Carcinoma of the pancreas

* Carcinoma of the gall bladder

* Metastatic carcinoma

* Reticuloses

* Mirizzi syndrome

* Pancreatitis , acute and chronic

* Pancreatic pseudocysts

CONGENITAL

* Biliary Atresia

* Choledochal  cyst

* Caroli's disease

BILE DUCT STONE

DEFINITION :‑

* Primary bile stones develop de novo in the bile  ducts.

* Secondary bile duct stones migrate from the gall bladder (this includes' residual or retained' stones inadvertently left after cholecystectomy).

Bile duct stones most commonly present with obstructive jaundice, colic, cholangitis or acute pancreatitis.

MANAGEMENT

Primary bile duct stones do not develop in a normal bile duct and imply bile stasis and infection. Treatment is aimed at achieving free drainage of bile.

This can bee done surgically (choledo­choduodenostomy, surgical sphincteroplasty or hepaticojejunostomy), by incising the papilla, using diathermy at ERCP.

Secondary bile duct stones are increasingly treated by removal at ERCP. In many cases, when there are also stones in the gall bladder, cholecystectomy with some form of manipulation of the bile  duct is indicated.

Conventionally , if stones are found in the common bile duct on operative cholangiography with open cholecystectomy, surgical exploration, and stone extraction , is performed through an incision low in the bile duct, and a T tube is left in place. Some days later a T tube cholangiogram is performed, and if it showed no residual  stones the T tube is removed. If the cholangiogram showed stones still within the ducts the T tube was left in until the track had matured, and at six weeks percutaneous extraction of  the stones is performed using steerable wires and basket (the Burthenne technique). With the increasing popularity of laparoscopic surgery, many surgeons prefer to submit their pa­tients  to endoscopic sphincterotomy, rather than convert to an open operation.

This  policy is successful in 85 % of patients with a low morbid­ity ( 5‑10 %), and  mortality (/_ 1%). Stones thought to be too large  to pass  spontaneously can be extracted using Dorima baskets or balloon catheters at the time of papillotomy. Large stones can be broken down in the ducts using contact lithotripsy, lasers, or crushing baskets. However, these practices have led to a marked  increase throughout the world in the  demand of ERCP and sphincterotomy. The clinical and economic consequences of this have yet to be fully evaluated.

PYOGENIC CHOLANGITIS

Pyogenic cholangitis is an acute suppurative or non suppurative inflammatory process, within the bile ducts, that occurs in the presence of bile stasis. In one large series a third of the cases were due to stones, another third were due to benign biliary strictures, and the rest  were due to tumours, foreign bodies, blocked biliary tubes and radiological intervention.

Acute suppurative cholangitis is usually due to complete biliary

obstruction , with pus under pressure within the ducts. Patients present with Charcot's triad of jaundice, rigors and upper abdom­inal  pain. Mental stupor and septicaemic  shock supervene in advanced cases ( Reynolds' pentad ). The common  bacteria in­volved are Escherichiam coli, Klebsiella spp. and enterococcus. In 20‑40 %  of cases anaerobes are present . Mixed growths are the most common.

The biliary  system should be decompressed urgently. Morbidity and mortality in patients treated by open operation are high and, in some series, mortality approaches 60 % . Endoscopic sphincte­rotomy  with removal of stones, or passage of a drainage tube beyond the stones or stricture , is the treatment of choice with a mortality of less than 10 %.

Chronic recurrent cholangitis occurs in the presence of partial bile duct obstruction with fever  and rigor, but only mild jaundice. The condition settle rapidly with antibiotics.

However , because of the risk of secondary to ecure free flow of bile is required.

Recurrent  pyogenic cholangitis (oriental cholangiohepatitis) is a form of cholangitis  that is common in Japan and south East Asia.

Parasitic infestation of the biliary tree causes multiple stric­tures and beading  of the extrahepatic and intrahepatic ducts leading to bile stagnation , stone formation and abscesses.

The common parasites are clonorchis sinensis and ascaris lumbro­coides. Indeed, the corpses of theses parasites  may be  found within primary bile duct stones. Management is surgical with cholecystectomy and Rous-en-Y hepaticojejunostomy. Resection of severely damaged liver lobes may be necessary.

STRICTURE OF THE BILE DUCTS:‑

Sclerosing cholangitis is a chronic stenosing inflammatory process involving  the intrahepatic and extrahepatic biliary tree. It is conventionally divided into primary sclerosing cholangitis, which is idiopathic , or secondary  sclerosing cholangitis, which is due to chronic obstruction of the extrahepatic ducts.

Primary sclerosing cholangitis  may present with the gradual onset of cholestatic jaundice, and can be diagnosed in patients with a biliary stricture who do not have gallstones o  a history of previous surgery, and has had a sufficiently long follow up to exclude cholangiocarcinoma. There is no  predilection for either sex. Most patients have ulcerative colitis though only 1‑2% of patients with ulcerative colitis develop this complication . Others have Crohn's disease though this is less common . The aetiology remains  uncertain.

Laboratory tests demonstrate an elevated and often fluctuating serum bilirubin with a persistently elevated alkaline phosphatase level. Hypergammaglobulinaemia and auto‑antibodies , in particu­lar antineutrophil cytoplasmic antibody , may be present, and there is an excess of patients with the HLA‑B8 haplotype. Cholan­giographic appearances at ERCP vary from strictures and beading throughout the biliary tree to an isolated or "dominant" extrahe­patic stricture.

Histologically  , there are characteristic periportal inflammato­ry changes, but often the features are not specific and are those of long‑standing  biliary obstruction. The disease process is variable and , in some patients with ulcerative colitis, the liver condition may be clinically silent.

Primary sclerosing cholangitis mat lead inexorably to liver failure, with chronic biliary obstruction and secondary biliary cirrhosis.

To manage  an obstruction ., the stricture can be dilated and endobiliary tubes placed at ERCP. Pyogenic cholangitis can compli­cate primary sclerosing cholangitis and antibiotics should be used at an early stage . Biliary drainage can be achieved surgi­cally in patients with dominant  extrahepatic stricture and only a little liver damage by excising the stricture and performing Roux‑en‑Y hepaticjejunostomy. Dysplastic changes and cholangio­caricnoma may supervene in cases of primary sclerosing cholangi­tis . This may pose a diagnostic problems.

Surgical excision  is sometimes  indicated because of suspected malignancy . Apart from these cases, major surgery is best avoid­ed, as this may prejudice the success of hepatic transplantation , which may be the only  option in the advanced stages. Colectomy does not alter the course of the disease in patients with ulcera­tive colitis. Corticosteroids have no proven place in management , and trials of colchicine and oral ursodeoxycholic acid are in progress.

Traumatic iatrogenic strictures , accidental injury to the bile ducts at cholecystectomy are , most commonly inflicted by sur­geons in training. The risk seems to be greater with laparoscopic than with open  cholecystectomy, though  some of this may reflect a "learning curve" during development of the new technique. The mechanism may be by direct damage to the bile duct, disruption of its blood and scarring around the bile ducts.

Presentation may be early if complete division or ligation of the bile duct has taken place, It may take years after surgery. Long‑standing strictures result in secondary sclerosing cholangitis, leading to hepatic fibrosis and secondary biliary cirrhosis with portal hypertension and varices.

Treatment  is aimed at complete drainage of the biliary tree by anastomosis to a long (70 cm) Roux‑en‑Y loop of jejunum. Follow­ing successful biliary drainage the liver architecture and func­tion partially resolves.

TUMOURS

Benign; papillomas  and adenomas are rare in the proximal ducts. They are relatively common distally and merge with periampullary cancer.

Cholangiocarcinoma is an uncommon adenocarcinoma. It occurs in  400 people per year in the  united  kingdom and usually presents between the ages of fifty and years . It has a propensity to occur at or near the confluence of the right   and left hepatic ducts; the tumours and its surrounding desmoplastic reaction cause a tight stenosis (the  "Klatskin" tumour). There is perineural and local liver and vascular invasion with metastases to regional lymph nodes. Predisposing factors are thought to be  ulcerative colitis, with or without  primary sclerosing cholangitis, and, in the far East, chronic parasitic infestation. There is no specif­ic association with gallstones.

PTC is the most helpful investigation and indicates how much involved duct  remains above the stricture . Angiography may be performed to exclude vascular invasion of the hepatic artery or portal vein.

Adequate palliation can be achieved with stenting at either PTC or ERCP, but about 20% of patients are suitable for surgery , which improves the year survival from less than 5% to 20‑40%.This involves associated excision liver resection , and reconstruction of the hepatic ducts to a Roux‑en ‑Y lop of jejunum.

Secondary Tumours; metastases to the hilar lymph nodes may causes biliary obstruction that is difficult to differentiate from primary cancer. Metastases within the liver may also compress the hilar ducts, but metastases to the bile duct itself are rare, though they have been reported from melanomas.

CHOLEDOCHAL CYSTS

Choledochal cysts are thought to be congenital and are fusiform or diverticular dilatation of the biliary ducts. They may be single or multiple and may  be intrahepatic or extrahepatic. Most choledochal cyst ( 70‑80%) present in childhood. The condi­tion is particularly  common in japan where it occurs in 1/1000 live births , compared with 1/100,00 live births in western  Europe.

There is a female preponderance of 4:1.

The cyst wall consists of fibrous tissue without a smooth muscle coat, and with an attenuated and often inflamed or dysplastic epithelial  lining. There is usually a characteristic anomalous junction between the common bile and pancreatic  ducts, often with distal stenosis , and the bile in the cyst has a high amy­lase concentration.

Children present with cholestatic jaundice , pain, and occasional an  abdominal mass. Biliary cirrhosis and liver failure will follow if left untreated. Adults present  with jaundice, cholan­gitis , or pancreatitis. There is an  estimated 10% risk of malignant change in the biliary tract in adults presenting with the disease; only 57% of tumours are intracystic. The aim of treatment therefore is to  excise all redundant extrahepatic bile ducts and achieve free drainage of the bile, with reconstruction to a Roux‑en‑Y loop of jejunum.

HAEMOBILIA

Blood in the biliary tract is usually due to iatrogenic injury from PTC, Liver biopsy or surgery. It can follow blunt or pene­trating trauma, or rupture of hepatic artery aneurysm. T may also occur with vascular tumours. It may present with jaundice , bil­iary colic, melaena or haematemesis. Investigations are ultrasound scanning, endoscoping (where blood is seen issuing from the papilla) and arteriobiliary fistula or hepatic arterial aneurysm is seen.

 DISORDERS OF THE PAPILLA OF VATER

The papilla of Vater contains the finely balanced sphincter of oddi.

The sphincter is under neural and hormonal control and is able to withstand a pressure of 300mm HG when closed. A variety of disor­ders may effect sphincter function.

Functional disorders; some  patients who suffer recurrent attacks of biliary pain or of acute pancreatitis without apparent cause may suffer from dyskinesia of the sphincter. It is best diagnosed by endoscopic manometric studies. Surgical  or endoscopic sphincterotomy may cure the syndrome.

Stenosis; anatomical stenosis may result from periampullary duodenal ulceration, the repeated passage of stones, or from surgical manipulation. Surgical exploration with the  usage of large rigid  instruments through the papilla should be avoided. Patients will have abnormal sphincter pressure measurements and may respond to  spincterotomy.Small  stones or sludge passing through the sphincter obstruct the ampulla, and there is a risk of acute pancreatitis. This is caused by reflux of bile from the common channel between the pancreatic and common bile ducts, with activation of pancreatic enzymes.

Ampullary adenocarcinoma; in the ampullary region adenocarcinoma may arise from the distal bile duct epithelium. the ampulla of Vater, the pancreas , or the duodenal mucosa. Patients present with painless jaundice and in the absence of stones, a distended gall bladder.

Investigation requires ultrasound to identify bile duct dilata­tion, the level of obstruction, and the presence or otherwise of a mass. ERCP will demonstrate the tumour and allow biopsy or cytological bushings to be taken to confirm the diagnosis. Endoscopic sphincterotomy or stenting, allows temporary decompres­sion and relief of jaundice before surgery. In patients who are unfit for surgery, or who have advanced tumours, endoscopic sphincterotomy or stenting may be the definitive management.

Local excision or radical excision  by means of pancreatoduode­nectomy  has a mortality of less than 5% with a acceptable mor­bidity.

The five year survival rate is 60 % , which is much higher than for carcinoma of the head of the pancreas. Prognosis depends on the degree of differentiation , the depth of invasion, and the presence of regional lymph node metastases.