Background

In the past, a choledochal cyst (CC) was thought to be an extrahepatic bile duct cystic dilatation. But names have changed over time, and choledochal cysts are now called biliary cysts, which include both intrahepatic and extrahepatic cystic dilatation. Cystic dilations in sections of the intrahepatic and extrahepatic bile ducts are a feature of biliary cysts.

Alonso-LEJ et al. first classified CC in 1959, classifying it into three categories according to the location of biliary duct dilatation. Todani et al. updated this categorization in 1977 and included two additional CC categories. Although the commonly used five-category classification is universally accepted by clinicians, some specialists disagree, arguing that each form of CC has its own distinct course, problems, and management. A classification scheme that prioritizes the pathophysiology above the biliary tree’s superficial anatomy is proposed. 

Epidemiology

With an incidence of roughly 1/100–150,000 in Western countries, choledochal cysts (CCs) cause 1 in 13,500 live births in the United States and 1 in 15 thousand live deliveries in Australia. They are incredibly rare. Despite a claimed prevalence of 1 in 1,000, the disease is more common in Asians, with two out of every three cases being of Japanese descent.

The gender distribution shows a notable majority of women (F(3.4)/M(1)). It is uncertain what is causing the majority of Asian and female ancestry. There appears to be a regional variance, with Japan accounting for two-thirds of the recorded cases in Asia. With an incidence of 1 in 1000 per live births, this ailment primarily affects the Asian population, whereas the Western population has an incidence of 1 in 100000–150000 live births.

The incidence of this condition in the United States is 1 in 13500 per live births, and Australia is 1 in 15000 per live births, respectively, despite the fact that it is uncommon in the Western population. The prevalence of CCs type I and IV is higher in females, with a 4:1 or 3:1 female-to-male ratio. The cause of Asian and female predominance is yet unknown. 

Anatomy

Pathophysiology

Various classifications exist for choledochal cysts, with Todani’s modified classification introduced in 1977 being the most widely adopted by clinicians. This classification encompasses five types of choledochal cysts (CCs): 

Type 1: 

Type 1 cysts, which make up between fifty percent and eighty percent of CCs, have common bile duct cystic dilatation. This type is further subdivided into three groups: 

• Type 1A: Whole extrahepatic biliary tree cystic dilatation associated with APBJ. 
• Type 1B: The extrahepatic biliary tract dilates segmentally in the absence of APBJ. 
• Type 1C: When there is APBJ, diffuse fusiform dilatation is seen in all of the extrahepatic biliary trees. Todani (2003) suggested that this dilatation could continue indefinitely into the channel (intrahepatic). 

Type 2: 

Diverticular dilation occurs anywhere along the duct (extrahepatic) in type 2 cysts, which make up 2% (CCs). 

Type 3: 

Choledochocele, or type 3 cysts, account for 1.4% of CCs to 4.5% of CCs. They involve the distal common bile duct’s intraduodenal cystic dilatation. 

Type 4: 

Type 4 cysts are many and further subdivided into two subgroups: they account for between 15 and 35 percent of CCs. 

• Type 4A: Numerous dilations that impact the biliary trees extrahepatic and intrahepatic.
• Type 4B: Multiple dilations are only present in the biliary tree (extrahepatic).

Type 5: 

Type 5 cysts, commonly known as Caroli’s disease, account for twenty percent of CCs. They are characterised by multiple dilations that are restricted to the biliary tree (intrahepatic). A combination of Type V CC with congenital hepatic fibrosis is referred to as “Carli syndrome”. 

Etiology

• Choledochal cystic disease’s etiology is yet unknown; however, as the deformities affect fetuses and infants, they are regarded as congenital. The fact that choledochal cysts are often linked to an aberrant pancreatobiliary duct junction (APBDJ) has led to the development of the most widely accepted explanation regarding these cysts. The “long common channel theory” explains how APBDJ permits pancreatic enzyme reflux into the common bile duct. Pancreatic juice reflux causes ductal wall disintegration and occlusion at the distal end of the cyst as a result of fibrosis and edoema. 
• According to a different view, this illness is caused by abnormalities in the ducts’ normal embryologic remodeling, which results in variable degrees of segmental dilatation and damaging inflammation. In individuals with a choledochal cyst, Kusunoki et al. found a lower-than-ideal ganglion cell count in the narrow section of the common bile duct as compared to controls. An obstructive component during the early embryonic stage was identified as the causal factor based on an experimental investigation in which cystic dilatation of the common bile duct was generated by the closure of the common bile duct’s distal end in the neonatal lamb. 
• The pancreatic junction and bile duct junction, known as the APBJ, are situated outside of the duodenal wall. According to Babbitt’s analysis, APBJ complicated the majority of choledochal cysts. Analyzing the fusion pattern between the pancreatic and bile ducts allows for the classification of APBJ into two types: P-C and C-P type. The common bile duct appears to join the main pancreatic duct in the C-P type, whereas the joining of the main pancreatic duct and the common bile duct in the P-C type. 
• APBJ was divided into three groups, type I, type II, and type III, by Komi et al. in 1991 based on the common channel’s dilatation and the auxiliary pancreatic duct’s course. APBJ’s C-P and P-C types are represented by types I and II, whereas type III is known as the complex type. The prevalence of APBJ type I (35.3%), type II (21.6%), and type III (43.1%), in that order. 
• After analyzing 1627 CDC patients, Tashiro et al. (2003) discovered that APBJ patients coincided with those in normal instances. Moreover, individuals without APBDJ have been observed to have pancreatic juice reflux. Given the circumstances that resemble APBJ, APBJ should be described as the lengthy common channel that has no connection to the sphincter of Oddi contraction. 

Genetics

Prognostic Factors

• Prognostic factors for choledochal cysts can vary, influenced by elements such as the specific cyst type, age at diagnosis, presence of complications, and the timing of surgical intervention. Each case is unique, and general considerations include: 

Cyst Type Influence: 

• The particular type of choledochal cyst can impact prognosis, with certain types carrying a heightened risk of complications. 

Early Detection Benefits: 

• Timely identification of choledochal cysts, especially in infants, can contribute to a more favorable prognosis by allowing prompt intervention and management. 

Complications Impact: 

• The existence of complications, such as cyst infections, pancreatitis, or malignancies, can significantly affect prognosis. Recognizing and treating complications early on is pivotal. 

Surgical Timing and Success: 

• The timing and success of surgical interventions, such as cyst excision and hepaticojejunostomy, play a crucial role in prognosis. These procedures aim to prevent complications and enhance long-term outcomes. 

Ongoing Follow-up Care: 

• Regular follow-up care and monitoring are vital for evaluating the postoperative course, detecting any recurrence or new complications, and ensuring the overall well-being of the patient. 

Histopathological Insights: 

• Examination of the cyst and surrounding tissues through histopathology can offer insights into the cyst’s nature and the presence of abnormal cellular changes. 

Patient’s Overall Health Impact: 

• The general health of the patient, inclusive of underlying medical conditions, can influence prognosis. Concurrent health issues may shape the management approach and overall outcomes. 

• These considerations underscore the need for a tailored and comprehensive approach to managing choledochal cysts, with close collaboration between healthcare professionals to optimize prognosis and patient well-being. 

Clinical History

Patient Presentation: 

The manifestation of choledochal cysts varies depending on the age of onset, with a dramatic presentation in infancy and a more diverse clinical picture in older children and adults. 

Infants: 

In infancy, choledochal cysts often present dramatically with features such as acholic stools and obstructive jaundice. This may prompt a diagnostic workup for conditions like biliary atresia, particularly in early infancy. Additionally, palpable masses in the right upper quadrant and hepatomegaly are commonly observed in infants with choledochal cysts. 

Children: 

Children diagnosed after infancy typically exhibit intermittent biliary obstruction or recurrent pancreatitis episodes. A right upper quadrant palpable mass, as well as jaundice, are possible symptoms of biliary obstructive pattern. In cases where pancreatitis is the primary manifestation, intermittent colicky abdominal pain becomes a significant symptom. Elevated amylase and lipase concentrations guide the diagnostic workup in such situations. 

Adults: 

Adults with choledochal cysts may face one or more severe complications, leading to common symptoms like jaundice, right upper quadrant pain, vague epigastric, or cholangitis. Abdominal pain is the predominant symptom in adults. While jaundice, a palpable right upper quadrant mass, and a classic triad of abdominal pain are described, it is evident in only 10-20% of cases; the majority exhibit one or two symptoms of the triad. 

Physical Examination

A palpable right upper quadrant mass is more frequently observed in infancy and early childhood. In patients presenting with pancreatitis, nonspecific mid-epigastric or diffuse abdominal pain is a common feature. 

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Biliary Obstruction of Various Causes: 

Prominent characteristics include a degree of dilatation less than that seen in choledochal cyst, recognisable primary lesions (such as  ampullary, pancreatic ductal, choledocholithiasis, or distal CBD (common bile duct) cancer, and proportionate (as opposed to fusiform) dilatation. 

Caroli Disease: 

Officially categorised as a type 5 choledochal cyst, it presents as a congenital dilatation of the major intrahepatic bile ducts that is nonobstructive. Multiple cyst-like formations of different sizes are the outcome of localised saccular ectasia, which is the condition’s defining feature. 

Acute Cholangitis: 

Features include obstructing choledocholithiasis and ductal wall thickening. 

Pancreatic Pseudocyst: 

Distinctive characteristics involve the absence of communication with bile ducts, a hyperintense cyst contiguous with a dilated pancreatic duct on magnetic resonance cholangiopancreatography (MRCP), a contrast-enhanced fibrous capsule on contrast-enhanced computed tomography (CECT), a well-defined cystic lesion related to the pancreatic head, and pseudocyst communication with the pancreatic duct, observed in 70% of cases on endoscopic retrograde cholangiopancreatography (ERCP). 

Recurrent Pyogenic Cholangitis: 

Key features include dilatation of both intra- and extrahepatic bile ducts, cast-like biliary stones, sludge, pneumobilia, and abscess. Rapid tapering of the peripheral intrahepatic duct, MRCP reveals ductal rigidity and straightening, and the condition is more commonly observed in Asians. 

Primary Sclerosing Cholangitis: 

This idiopathic inflammatory process leads to progressive strictures and fibrosis of extra- and intrahepatic bile ducts. Multifocal areas exhibit alternating biliary strictures and dilatation, along with abnormal bile duct wall thickening and enhancement. 

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

The comprehensive approach to treating choledochal cysts takes into account several factors, including the cyst type, patient age, complications, and overall health. Key components of this treatment paradigm encompass type-specific management, surgical intervention, early intervention in infants, complications management, long-term follow-up, individualized care, and histopathological examination: 

Type-Specific Management: 

Tailored strategies are employed for different choledochal cyst types. For instance, Type I cysts may involve procedures like cyst excision and hepaticojejunostomy, whereas Type II cysts may require diverticulum resection. Type-specific interventions aim to address each cyst type’s unique characteristics. 

Surgical Intervention: 

The primary treatment involves surgically removing the choledochal cyst by excising the cystic dilatation and reconstructing biliary drainage. Surgical techniques vary based on the cyst type and extent of involvement, with common procedures including cyst excision, hepaticojejunostomy, and roux-en-y hepaticojejunostomy. 

Early Intervention in Infants: 

Crucial in cases diagnosed in infancy, early intervention is essential for preventing complications and ensuring favorable long-term outcomes. 

Management of Complications: 

Complications such as infections, pancreatitis, or malignancies associated with choledochal cysts necessitate specific management. Timely recognition and treatment enhance prognoses. 

Long-Term Follow-up: 

Regular postoperative monitoring is vital for assessing recovery, identifying cyst recurrence, and managing potential complications. Long-term surveillance is particularly crucial due to complications, risks, and the potential for associated conditions. 

Individualized Care: 

Considering patient age, overall health, and specific circumstances, the treatment paradigm develops individualized care plans tailored to address each case’s unique aspects. 

Histopathological Examination: 

Examining the cyst and surrounding tissues histopathologically offers insights into the cyst’s nature and the presence of abnormal cellular changes. This step contributes to a more thorough understanding of the condition. 

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

lifestyle-modifications-in-treating-choledochal-cysts

1. Nutrition: A proper diet is crucial for recovery. Initially, the patient might receive intravenous fluids and nutrition to allow the digestive system to rest. Later, a gradual transition to a regular diet will be made, focusing on low-fat, easily digestible foods. 
2. Pain Management: Pain management is vital post-surgery. Adequate pain relief medications should be prescribed and administered as needed to ensure the patient’s comfort and facilitate movement and breathing exercises. 
3. Monitoring: Continuous monitoring of vital signs, incision site, and overall condition is essential. Regular check-ups by healthcare providers help in early identification of any complications. 
4. Activity and Rest: Balancing rest and appropriate activity is crucial. Too much activity may strain the surgical site, while excessive bed rest might delay recovery. The patient should gradually increase activity levels as advised by the healthcare team. 
5. Hygiene: Maintaining good hygiene, especially around the surgical site, is crucial to prevent infections. Proper wound care instructions should be followed diligently. 
6. Emotional Support: Surgery, especially in children, can be emotionally challenging. Providing emotional support and reassurance to both the patient and their family is important for overall well-being. 
7. Follow-up Care: Appointments for follow-up care with medical professionals are necessary to track the healing process, handle any issues, and guarantee a full recovery. 
8. Avoiding Certain Activities: Patients may need to avoid heavy lifting, strenuous activities, or certain foods that could cause strain on the abdomen or the digestive system, as advised by their healthcare provider. 

The typical approach to managing choledochal cysts involves surgical intervention for cyst removal and biliary system reconstruction. Environmental adjustments may not have a direct impact on choledochal cyst treatment, given its nature as a medical condition necessitating medical and surgical attention. Nevertheless, specific considerations can enhance the patient’s recovery environment during the perioperative and postoperative periods: 

Preoperative Care: 

• Ensure the patient is well-hydrated and nutritionally optimized before surgery. 
• Implement infection control measures to prevent preoperative infections. 
• Develop a comprehensive plan for adequate pain management. 

Surgical Environment: 

• Adhere strictly to aseptic techniques in the operating room to minimize infection risks. 
• Monitor and maintain optimal body temperature during surgery. 
• Ensure the availability of advanced imaging and surgical equipment to facilitate precise cyst removal and biliary reconstruction. 

Postoperative Care: 

• Implement effective pain management strategies. 
• Monitor vital signs and overall recovery closely in a well-equipped recovery room. 
• Apply infection prevention measures during the postoperative period. 
• Provide nutritional support to aid the healing process. 

Patient Education: 

• Offer clear and detailed information to patients and their caregivers regarding postoperative care, medications, and potential complications. 
• Emphasize the importance of follow-up appointments for monitoring and assessing the surgical site. 

Home Environment: 

• Establish a comfortable and supportive home environment to facilitate the patient’s recovery. 
• Ensure access to a balanced diet that supports healing. 
• If required, make arrangements for help with daily tasks during the initial phase of rehabilitation. 

Use of analgesics in the treatment of choledochal cysts

Analgesics, such as NSAIDs (nonsteroidal anti-inflammatory drugs) or acetaminophen, might be prescribed to address pain linked to the cyst or discomfort following surgery.  

Acetaminophen: It is an anti-inflammatory drug which is a derivative of anilide. It is used as an analgesic in the management of pain in patients with choledochal cysts. 

It is aimed at alleviating pain and may be suggested to manage pain linked to the fistula or to alleviate discomfort postoperatively. 

Use of antibiotics in the treatment of choledochal cyst

In certain instances, antibiotics may be recommended to prevent or treat infections associated with the choledochal cyst. This becomes especially significant if there are indications of infection or if the surgical procedure is delayed. 

Piperacillin/Tazobactam: Penicillins and beta-lactamase inhibitors are a class of antibiotics that includes piperacillin and tazobactam. This combo injection is used to treat post-operative wounds as well as bacterial infections in a variety of body areas, including the skin, lungs, female reproductive organs, stomach, and intestines. 

Nutritional Plan in choledochal cyst

Healthcare providers may suggest nutritional supplements to support recovery, considering factors like the specific surgical procedure and the nutritional status of the patient. This helps ensure adequate nourishment during the postoperative phase. 

It’s important to consult with healthcare professionals, such as gastroenterologists or hepatobiliary surgeons, for a comprehensive assessment and personalized treatment plan for choledochal cysts. 

surgical-intervention-in-the-treatment-of-choledochal-cysts

Fundamental Principles: 

Choledochal cyst surgical therapy is contingent upon the form of the cyst and any concomitant hepatobiliary pathology. In order to reestablish bile flow, biliary-enteric anastomosis (mucosa-to-mucosa) should be used after removal of all bile duct cysts.

The surgical therapy of this disease is not only dependent on external drainage. Long-term follow-up is necessary for adults treated without cyst resection due to the incidence of advanced anastomotic strictures and their age-related cancer risk.  

Cyst Type I 

The best course of treatment for adult type I bile duct cysts is a full cystectomy and Roux-en-Y hepaticojejunostomy. Benefits of this operation include decreased risk of stones, intracystic malignancy, anastomotic strictures, and cholangitis. Hepaticojejunostomy (Roux-en-Y) and complete cystectomy have demonstrated outstanding clinical outcomes.

There hasn’t been any higher morbidity or mortality than with choledochocystojejunostom (Roux-en-Y) drainage. In 10–25% of cases, recurrent cholangitis as a result of anastomotic strictures has been documented.

While several series have demonstrated that cyst excision can reduce malignancy, cancer has nonetheless emerged after cyst removal. In theory, adult cyst excision can be accomplished by first freeing the gallbladder from the liver bed in order to separate the cyst from the hepatic tissues.

Prior to dissecting the outermost layer of the cyst, the portal vein should be identified, and the liver artery needed to be isolated and controlled, particularly if extensive adhesions and hypervascularity are present. To locate the pancreaticobiliary ductal junction, the cyst (distal) (from pancreas) is removed prior to the cyst’s division.

The bile duct (distal) is carefully ligated proximal to its intersection with the duct of pancreas after the cyst has been cut laterally within the pancreatic head. Through a broad hepaticojejunostomy (mucosa-to-mucosa Roux-en-Y) at the hilum level, biliary-enteric flow is restored.  

There have been reports of laparoscopic therapy for type 1 choledochal cysts, and this approach needs more research. Cyst excision is rarely prevented by portal hypertension brought on by adhesions (inflammatory) from pancreatitis (severe) and secondary biliary cirrhosis previous drainage treatments.

In such cases, the chosen alternative treatment is choledochocystojejunostomy (Roux-en-Y). It may occasionally be necessary to do surgery (drainage) after 6 to 12 weeks following portal decompression via the central splenorenal shunt.

According to Lilly, the best way to treat this condition in elderly individuals with recurrent cholangitis and significant pericystic inflammation may be to remove the Cyst (anterolateral portion) and then remove the lining by the procedure for endocysts, leaving the rear wall next to the vein (portal) intact. 

Seventy percent of patients who have had a internal drainage procedure (cyst enterotomy) in the past need to have it redone due to complications, including hepatolithiasis and cholangitis.

In fact, even though the complications of surgery for excision following previous internal drainage procedure are more common than those seen after primary cyst excision, As a preventive measure against complications in certain situations of malignant cysts, it has been suggested that patients who have already undergone internal drainage treatments should have a second operation with cyst removal (even if asymptomatic). 

Cyst Type II 

Surgical excision is the treatment for type 2 cysts that develop as lateral diverticula of the bile duct (common). The cyst neck at the bile duct (common) junction may be mostly closed or undergo common bile duct T-tube decompression, depending on its size.

On the other hand, draining into the duodenum is theoretically simpler when these cysts originate from the intrapancreatic section of the common bile duct. There are not many reports of this kind of cyst (choledochal) because it only affects a small number of patients.  

Cyst Type III 

The actual cyst of the bile duct (distal common) that protrudes into the duodenum is known as a choledochoceles. Patients may exhibit pancreatitis, cholangitis, or biliary colic. The preferred course of treatment up until recently was transduodenal cyst removal, either with sphincterotomy or without sphincterotomy.

The common major pancreatic duct and bile duct must be carefully protected and reanastomosed to the mucosa (duodenal) because they open into the cyst. Nowadays, cyst unroofing and endoscopic sphincterotomy are the recommended courses of action.

Despite the fact that transduodenal excision removes the possibility of cancer, the danger of cancer by itself does not warrant surgical therapy for choledochoceles, as only three occurrences of carcinoma have been documented.  

Cyst Type IV 

Type I choledochal cysts are the term used to describe the component of extrahepatic type 4B and 4A cysts. Hepaticojejunostomy (Roux-en-Y) and transduodenal sphincteroplasty finish the type IVB choledochal cysts treatment, which have a choledochocele component. There is disagreement regarding how much of a type IVA cyst should be removed.

A few writers recommend hepaticoenterostomy and just extrahepatic component removal for management. On the other hand, cancer has been documented to develop in the intrahepatic cysts, as previously mentioned, as well as following hepaticojejunostomy-assisted extrahepatic cyst resection.  

It is obvious that intrahepatic cysts cannot be removed when they are extensive, but it would make sense to carry out the necessary hepatectomy (partial) when the intrahepatic illness is localised. hepatectomy (partial) has been used to treat Caroli illness for the same reason. 

There is evidence of dilation of cylindrical intrahepatic duct in type 4A illness, which regresses spontaneously after hepaticojejunostomy and common duct excision, in contrast to cystic intrahepatic disease, which does not return.

It seems more plausible that the intrahepatic (cylindrical) sickness is a type 1 cyst with some constriction causing proximal dilatation than it is a pure type 1. 

phases-of-management-in-the-treatment-of-choledochal-cysts

The management of choledochal cysts typically encompasses several phases, ranging from initial diagnosis to surgical intervention and subsequent postoperative care. The exact strategy may change based on the patient’s age, the nature of the choledochal cyst, and other variables. The following outlines the general stages of management: 

Diagnosis and Evaluation: 

• Clinical Assessment: Consideration of a patient’s medical history, physical examination, and symptoms. 

• Imaging Studies: Utilization of techniques like ultrasonography, magnetic resonance cholangiopancreatography (MRCP), and other imaging methods to visualize the cyst and assess its characteristics. 

Preoperative Preparation: 

• Consultation with Specialists: Collaboration among gastroenterologists, hepatobiliary surgeons, and other specialists for evaluation and planning. 

• Medical Stabilization: Addressing any associated medical issues, such as infections or inflammation, to ensure the patient is in optimal condition for surgery. 

Surgical Intervention: 

• Cyst Excision or Resection: The primary treatment involves surgically removing the choledochal cyst. Various procedures may be employed, including complete cyst excision or hepaticojejunostomy (connecting the bile duct to the small intestine). 

• Reconstruction: If necessary, reconstruction of the biliary and digestive tracts is performed to restore normal flow. 

Postoperative Care: 

• Monitoring and Recovery: Close monitoring of patients in the postoperative period for any signs of complications. 
• Pain Management: Ensuring adequate pain relief to enhance the patient’s comfort during the recovery phase. 

• Nutritional Support: Depending on the surgical procedure, provide necessary nutritional support and recommend dietary adjustments. 

Long-Term Follow-Up: 

• Regular Check-ups: Patients typically attend regular follow-up appointments to monitor their recovery and assess any potential complications. 

• Imaging Studies: Periodic imaging studies may be conducted to ensure the absence of recurrent cysts or other issues. 

Complications Management: 

1. Complication Identification and Treatment: Prompt identification and management of complications, such as infections, strictures, or abnormalities. 

Medication

Future Trends

Media Gallary

References

Diagnosis and Management of Choledochal Cysts – PMC (nih.gov)