Common bile duct stones:

Like gall bladder stones there can be stones in bile duct as well. This could be either primary CBD Stones or secondary CBD Stones.

Risk factors:

Cholesterol stones should be differentiated from pigment stones, which may form within the biliary system as a consequence of bile duct infections, haemolytic anaemia, small bowel disease such as Crohn's disease, or cystic fibrosis. Risk factors for the development of cholesterol stones include increasing age, female sex, family history, obesity, dyslipidaemia and oestrogen therapy. Exercise and moderate alcohol consumption appear to be protective. Risk factors for biliary sludge include pregnancy, drugs (such as ceftriaxone, octreotide and thiazide diuretics), total parenteral nutrition and fasting.

Investigations:

Transabdominal ultrasound and liver function tests to be done.

Unlike gallbladder stones, common bile duct stones can be difficult to visualise on transabdominal ultrasound. However, a combination of biliary colic with a dilated bile duct on ultrasound and/or abnormal LFTs is suggestive of the diagnosis.

Alkaline phosphatase (ALP) is often raised, normal levels can be seen. In the context of acute biliary obstruction, the dominant abnormality can be an alanine transferase (ALT) of several hundred international units or higher.

When there is a persistent suspicion of common bile duct stones and results are non-diagnostic, further investigation may be necessary.

For patients requiring assessment before or after gallbladder removal, other options include magnetic resonance cholangiopancreatography (MRCP) and endoscopic ultrasound. Both are sensitive and specific tests which avoid the risks associated with the gold standard investigation endoscopic retrograde cholangiopancreatography (ERCP), which is now generally reserved for therapeutic intervention.

Managing the condition:

Stones in the gallbladder can be managed conservatively if they are not causing the patient pain, but most clinicians would offer to remove stones in the common bile duct regardless of presenting symptoms.

In patients who have already undergone cholecystectomy, the treatment of choice is ERCP. This can typically be performed as a day case procedure, either under conscious sedation or general anaesthetic.

Therapeutic ERCP entails identifying the orifice of the common bile duct, it enters the duodenum, using a flexible video endoscope passed via the oesophagus.

The duct is then cannulated and cholangiography is undertaken. Following this, a biliary sphincterotomy is performed, whereby the papilla is cut using electrocautery. Stones can then be removed using a balloon catheter or metal basket.

ERCP is associated with some significant risks. Acute pancreatitis is historically the most common complication and occurs more frequently in young female patients. However, the incidence can be reduced by the use of non-steroidal anti-inflammatory medications at the time of intervention. Other complications include bleeding, perforation and sepsis.

Although most adverse events settle with supportive management, there is a small mortality rate associated with the procedure of 0.5%.

This means that careful case selection, good operator technique and thorough discussion with the patient are essential. Sometimes patient may require multiple sessions of treatment for removal or large stones.

What are the treatment options for this patient?

At repeat ERCP is usually done after 1 month following cholecystectomy, the stent is generally removed if the CBD clearance is achieved, in small percentage of patients re do stenting is desired.

It is very import to remove the stent placed after ERCP in due time because after longer period the stent itself can act as nidus for stones and there will be stone formation around it.

Spontaneous passage of calculi occurs in up to 10% of patients, with 80% requiring removal of stone fragments during repeat ERCP. Although recurrence of bile duct calculi is estimated at 14% after one year, most of these are amenable to endoscopic treatment.

Gall bladder cancer:

Gallbladder cancer is cancer that begins in the gallbladder.

Gallbladder cancer is uncommon in western and southern part of India. When gallbladder cancer is discovered at its earliest stages, the chance for a cure is very good. But most gallbladder cancers are discovered at a late stage, when the prognosis is often not good. Geographically it is seen more common in northern part of India.

Gallbladder cancer is difficult to diagnose because it often causes no specific signs or symptoms. Also, the relatively hidden nature of the gallbladder makes it easier for gallbladder cancer to grow without being detected.

Diagnosing gallbladder cancer

Tests and procedures used to diagnose gallbladder cancer include:

•Blood tests: Blood tests to evaluate your liver functions as well as tumour markers like CA 19.9.

•Procedures to create images of the gallbladder: Imaging tests that can create pictures of the gallbladder include ultrasound, computerized tomography (CT) and magnetic resonance imaging (MRI).

•PET Scan: If needed could also be ordered to see for the distant extents of the disease.

Determining the extent of gallbladder cancer

It is best done with high speed Multi slice CT Scan to look for local spread of disease.

Second thing is presence of jaundice and distant lymph node spread.

Presence of jaundice makes it very difficult for surgeon to operate and reduction of jaundice has to be done with either ERCP /PTBD. This procedure

can lead to reduction in jaundice but at the same time the disease can progress and make the tumour unresectable.

Stages of gallbladder cancer

The stages of gallbladder cancer are:

•Stage I. At this stage, gallbladder cancer is confined to the inner layers of the gallbladder.

•Stage II. This stage of gallbladder cancer has grown to invade the outer layer of the gallbladder.

•Stage III. At this stage, gallbladder cancer has grown to invade one or more nearby organs, such as the liver, small intestine or stomach. The gallbladder cancer may have spread to nearby lymph nodes.

•Stage IV. The latest stage of gallbladder cancer includes large tumors that involve multiple nearby organs and tumors of any size that have spread to distant areas of the body.

Treatment

What gallbladder cancer treatment options are available to you will depend on the stage of your cancer, your overall health and your preferences.

The initial goal of treatment is to remove the gallbladder cancer, but when that isn't possible, other therapies may help control the spread of the disease and keep you as comfortable as possible.

Surgery for early-stage gallbladder cancer

Surgery may be an option if you have an early-stage gallbladder cancer. Options include:

•Surgery to remove the gallbladder. Early gallbladder cancer that is confined to the gallbladder is treated with an operation to remove the gallbladder (cholecystectomy). Only for stage T1a.

•Surgery to remove the gallbladder and a part of the liver and lymph nodes. Gallbladder cancer that extends beyond the gallbladder and into the liver is sometimes treated with surgery to remove the gallbladder, as well as portions of the liver and bile ducts that surround the gallbladder.

It's not clear whether additional treatments after successful surgery can increase the chances that your gallbladder cancer won't return. Some studies have found this to be the case, so in some instances, your doctor may recommend chemotherapy, radiation therapy or a combination of both after surgery.

Prognosis and life expectancy after gall bladder cancer

•As said ahead this is one of the deadliest cancers of all kind, one is because of its late presentations and secondly because of its virulence and aggressive disease nature.

•Even despite surgery the chances of recurrence rate of ca GB is very high.

•Only papillary type of GB Cancer has some better and favourable prognosis.

•Rest all tend to have poorer outcome and nearly 10% five-year survival rate.

Treatments for late-stage gallbladder cancer

Surgery can't cure gallbladder cancer that has spread to other areas of the body. Instead, doctors use treatments that may relieve signs and symptoms of cancer and make you as comfortable as possible.

Options may include:

•Chemotherapy. Chemotherapy is a drug treatment that uses chemicals to kill cancer cells.

•Radiation therapy. Radiation uses high-powered beams of energy, such as X-rays and protons, to kill cancer cells.

Procedures to relieve blocked bile ducts

Advanced gallbladder cancer can cause blockages in the bile ducts, causing further complications. Procedures to relieve blockages may help. For instance, surgeons can place a hollow metal tube (stent) in a duct to hold it open or surgically reroute bile ducts around the blockage (biliary bypass).

Palliative care:

Supportive and pain killer treatment.

Treatment for nutrition and vomiting.

Treatment for ascites or bleeding from vomitus.

Choledochal cyst

Choledochal cysts (CC) are a rare congenital cystic dilation of the biliary tract. They present primarily in female infants and young children and are more prevalent in East Asian populations.

Although benign, CC can be associated with serious complications including malignant transformation, cholangitis, pancreatitis, and cholelithiasis.

< p style="Font-size:1.20em">Approximately 80% of CC are diagnosed in infants and young children within the first decade of life. The incidence of CC ranges from 1 in 100,000 to 1 in 150,000 individuals in Western countries to one in 13,000 individuals in Japan.

The exact etiology is unknown, anomalous pancreaticobiliary duct junction (APBDJ) is seen in 30% to 70% of all CC where the common bile duct (CBD) and pancreatic duct junction occurs outside the duodenum, allowing reflux of pancreatic fluid into the biliary tree. The exposure of biliary epithelium to digestive and caustic pancreatic enzymes may contribute to CC formation.

Diagnosis:

Choledochal cysts are usually diagnosed in childhood, although in utero and adult diagnosis is also common.

Common presentations include abdominal pain, jaundice, and right upper quadrant mass and are most commonly seen in paediatric patients.Cholangitis, pancreatitis, portal hypertension, and liver function test abnormalities are common and are thought to be a result of ABPDJ or stone obstruction. Biliary amylase levels can be elevated in CC patients, and clinical features correlate with degree of elevation.

The classic triad of abdominal pain, right upper quadrant mass, and obstructive jaundice is mainly seen in the paediatric population.

Adults are more likely to present with biliary or pancreatic symptoms and abdominal pain; children are more likely to present with an abdominal mass and jaundice.Adults with CC are more likely to have symptomatic gallstones (45% to 70% of patients) or acute cholecystitis, both of which are attributed to biliary stasis. As a result, adult patients with CC are more likely to have undergone previous biliary procedures including surgery and stenting.

The best way to diagnose the cc is have USG followed by MRCP examination.

In case of mass within the CC one may be subjected to CECT abdomen or an EUS FNAC for the material confirmation of diagnosis.

Why do they need the treatment?

Biliary malignancy is seen in 10% to 30% of CC. Malignancy is rarely seen in pediatric CC; however, CC-associated biliary malignancy carries a dismal prognosis.

Malignancy is most commonly associated with types I and IV cysts, while types II, III, and V have minimal neoplastic risk.

Now a days this surgery can be carried out with minimally invasive laparoscopic and robotic techniques. These techniques have made the recovery and outcome of the surgery very good in recent times. Three dimensional laparoscopy and robotic surgery has made this surgery very easy for the operating surgeon.

Treatment for the CC varies upon types.

Type 1 – Surgical excisioin and RYHJ

Type 2 – Surgical excisioin and RYHJ OR Stenting

Type 3 – ERCP and papillotomy with close monitoring

Type 4 – Surgical excisioin and RYHJ +/- Liver resection

Type 5 – Liver transplantation. (The risk of neoplasia in Caroli's disease is less than 7%, but surgical management is usually indicated secondary to cholangitis and liver complications)

Outcome and Prognosis

Postoperative morbidity and mortality are typically very low in children, while postoperative complications are more commonly seen in adult patients. Late complications (greater than 30 days postoperatively) occur in up to 40% of adult patients if surgery is not done in properly. At times it can lead to stricture formation of RYHJ as well as post-operative hepatolithiasis.

Although malignancy is rare, CC resection does not reduce it to baseline levels, so long-term surveillance is indicated given the increased likelihood of developing postexcision biliary malignancy.

Benign Biliary stricture

Benign biliary strictures (block) (BBSs) may form from chronic inflammatory pancreaticobiliary pathologies, postoperative bile-duct injury (after gall bladder surgery), or at biliary anastomoses following liver transplantation.

Presentation of patient / signs and symptoms:

Most of the times patients are presented with slowly rising bilirubin may or may not be associated with fever, pain always negligible or mild, may or may not be associated with weight loss and associated with clay coloured stool and darkening of urine. Presentation is almost always mild and gradual. The signs and symptoms are progressive or in some cases it may be episodic.

The causes in brief could be

Common	Less common
Postsurgical
Liver transplantation	Vasculitis: SLE- and ANCA-associated
Cholecystectomy	Radiation therapy
Bilioenteric anastomosis	Portal biliopathy
Inflammatory	Post–radiofrequency ablation
Chronic pancreatitis	Tuberculosis
Primary sclerosingcholangitis	Postsphincterotomy
Trauma	Parasitic infection
	Mirizzi syndrome
	IgG4 cholangiopathy

Diagnosis:

Is best done with complete assessment of liver function testes. The treating doctor may order other associated blood tests and tumour markers test as well. USG is the primary line of investigation for liver and later on MRCP is usually needed to classify the type and its extents.

The types:

Bismuth class Location

I >2 cm distal to hepatic confluence

II <2 cm distal to hepatic confluence

III At the level of the hepatic confluence

IV Involves the right or left hepatic duct

V Extends into the left or right hepatic branch ducts

Treatment:

Treatment aims to relieve symptoms of biliary obstruction, maintain long-term drainage, and preserve liver function.

Endoscopic therapy

Endoscopic therapy, including stricture dilatation and stenting, is effective in most cases and the first-line treatment of BBS. For severe fibrotic strictures, initial balloon or bougie dilatation may be required, followed by placement of one or more plastic stents or an FCSEMS

Response to treatment is dependent upon the technique and accessories used, as well as stricture etiology.

Recommended endoscopic treatments for causes of benign biliary strictures

Condition	Dilatation	≥1 plastic stent	FCSEMS
Chronic Pancreatitis	–	Yes	Yes
Primary Sclerosing Cholangitis	Yes	–	–
Liver transplantation	–	Yes	Yes
Surgical injury	–	Yes	Yes
IgG4 cholangiopathy	–	Yes	–
Bilioenteric anastomosis	Yes	Yes	–

Although SEMSs are more expensive than plastic stents, they have significantly wider diameters (10 vs 3.3 mm, respectively), and are technically easier to insert than placing multiple plastic stents. Even the results are also better for the FCMS.

Surgical

Surgery may be required for patients with refractory BBS or those who are noncompliant with endoscopic therapy. For example, patients with CP found those with pancreatic head calcifications were 17-times more likely to have failure of response to endoscopic stenting.

Surgical treatment may provide better long-term outcomes for patients with persisting CP-associated BBS after more than three endoscopic procedures.Post-liver-transplantation nonanastomotic BSs also have lower treatment success following endoscopic drainage than those with anastomotic strictures.

Surgical alternatives to repair of BBS include excision of strictures with end-to-end repair, Roux-en-Y hepaticojejunostomy (RYHJ), choledochojejunostomy, and choledoduodenostomy. The choice of repair depends on such variables as extent and location of BBS. However, choice of treatment remains RYHJ.

Surgery lasts typically between 2 to 4 hours and the recovery time is usually 4-6 days of hospital stay followed by couple of weeks rest at home.

Radiological therapies are reserved for patients whose strictures are refractory to endoscopic and surgical interventions.

Outcome:

The procedure is safe and durable, with a rate of success of 80%–99% in the hands of experienced surgeons.

Cholangio carcinoma

Cholangiocarcinoma is cancer that forms in the tubes (bile ducts) that carry the bile from liver to intestine. This condition, also known as bile duct cancer, is an uncommon form of cancer that occurs mostly in people older than age 50, though it can occur at any age.

Doctors divide cholangiocarcinoma into different types based on where the cancer occurs in the bile ducts:

• Intrahepatic cholangiocarcinoma occurs in the parts of the bile ducts within the liver and is sometimes classified as a type of liver cancer. We shall discuss there.

• Hilar cholangiocarcinoma occurs in the bile ducts just outside of the liver. This type is also called perihilar cholangiocarcinoma.

• Distal cholangiocarcinoma occurs in the portion of the bile duct nearest the small intestine or within the intra pancreatic part of bile duct. The presentation & management as well as the outcomes are similar to that of the pancreatic head tumours, hence shall be discussed in pancreatic head cancer region.

Hilar cholangiocarcinoma:-

Symptoms:

Signs and symptoms of cholangiocarcinoma include:

Yellowing of your skin and the whites of your eyes (jaundice)

Intensely itchy skin

White-coloured stools

Fatigue

Abdominal pain

Unintended weight loss

When to see a doctor

See your doctor if you have persistent fatigue, abdominal pain, jaundice, or other signs and symptoms that bother you.

Diagnosis

Liver function tests. Blood tests to measure your liver function can give your doctor clues about what's causing your signs and symptoms. It checks Bilirubin and liver enzymes levels as well as coagulation parameters.

• Tumor marker test. Checking the level of cancer antigen (CA) 19-9 in your blood may give your doctor additional clues about your diagnosis. A high level of CA 19-9 in your blood doesn't mean you have bile duct cancer, though. This result can also occur in other bile duct diseases, such as bile duct inflammation and obstruction. Value of such marker does not corelate with the severity of disease.

• MRI with MRCP- This is a test for the visualization of bile duct intra hepatic and extra hepatic area. One of the best tests to delineate the duct size and its anatomy.

• CECT Whole abdomen- This is the test to see the vessels involvement for the tumour and surrounding area. It also determines the extents of the disease as well as spread in surrounding area.

• A test to examine your bile duct with a small camera. During endoscopic retrograde cholangiopancreatography (ERCP), a thin tube equipped with a tiny camera is passed down your throat and through your digestive tract to your small intestine. The camera is used to examine the area where your bile ducts connect to your small intestine. Your doctor may also use this procedure to inject dye into the bile ducts to help them show up better on imaging tests.

• A procedure to remove a sample of tissue for testing. A biopsy is a procedure to remove a small sample of tissue for examination under a microscope.

If the suspicious area is located very near where the bile duct joins the small intestine, your doctor may obtain a biopsy sample during ERCP. If the suspicious area is within or near the liver, your doctor may obtain a tissue sample by inserting a long needle through your skin to the affected area (fine-needle aspiration – EUS- FNAB).

If your doctor confirms a diagnosis of cholangiocarcinoma, he or she tries to determine the extent (stage) of the cancer. Often this involves additional imaging tests. Your cancer's stage helps determine your prognosis and your treatment options.

Treatment

Bile duct cancer consultation

The results of your imaging tests will help guide your treatment. Treatments for cholangiocarcinoma (bile duct cancer) may include:

• Surgery. When possible, doctors try to remove as much of the cancer as they can. For very small bile duct cancers, this involves removing part of the bile duct and joining the cut ends. For more-advanced bile duct cancers, nearby liver tissue, pancreas tissue or lymph nodes may be removed as well.

• Liver transplant. Surgery to remove your liver and replace it with one from a donor (liver transplant) may be an option in very much selective & certain cases for people with hilar cholangiocarcinoma.

• For many, a liver transplant is a cure for hilar cholangiocarcinoma, but there is a risk that the cancer will recur after a liver transplant.

• Chemotherapy. Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be used before a liver transplant. It may also be an option for people with advanced cholangiocarcinoma to help slow the disease and relieve signs and symptoms.

• Radiation therapy. Radiation therapy uses high-energy sources, such as photons (x-rays) and protons, to damage or destroy cancer cells. Radiation therapy can involve a machine that directs radiation beams at your body (external beam radiation). Or it can involve placing radioactive material inside your body near the site of your cancer (brachytherapy).

• Photodynamic therapy. In photodynamic therapy, a light-sensitive chemical is injected into a vein and accumulates in the fast-growing cancer cells. Laser light directed at the cancer causes a chemical reaction in the cancer cells, killing them. You'll typically need multiple treatments. Photodynamic therapy can help relieve your signs and symptoms, and it may also slow cancer growth. You'll need to avoid sun exposure after treatments.

• Biliary drainage. Biliary drainage is a procedure to restore the flow of bile. It can involve bypass surgery to reroute the bile around the cancer or stents to hold open a bile duct being collapsed by cancer. Biliary drainage helps relieve signs and symptoms of cholangiocarcinoma.

Because cholangiocarcinoma is a very difficult type of tumor to treat, don't hesitate to ask about your doctor's experience with treating the condition. If you have any doubts, get a second opinion.

Supportive (palliative) care

Palliative care is specialized medical care that focuses on providing relief from pain and other symptoms of a serious illness. Palliative care specialists work with you, your family and your other doctors to provide an extra layer of support that complements your ongoing care. Palliative care can be used while undergoing aggressive treatments, such as surgery.

When palliative care is used along with other appropriate treatments — even soon after your diagnosis — people with cancer may feel better and may live longer.

Palliative care is provided by teams of doctors, nurses and other specially trained professionals. These teams aim to improve the quality of life for people with cancer and their families. Palliative care is not the same as hospice care or end-of-life care.