Todani Type I Common Bile Duct Cyst in an Adult Patient: Clinical Case and Literature Review

Marco Antonio Medina-Ortega; Guillermo Vallejo-Vallecilla; Ana Valeria Gómez-Mosquera

Report of cases

Quiste de colédoco Todani I en una paciente adulta: caso clínico y revisión de la literatura

Marco Antonio Medina-Ortega ^* mmedinao@unicartagena.edu.co

Universidad de Cartagena, Colombia

Guillermo Vallejo-Vallecilla

Universidad del Cauca, Colombia

Ana Valeria Gómez-Mosquera

Universidad del Cauca, Colombia

Todani Type I Common Bile Duct Cyst in an Adult Patient: Clinical Case and Literature Review

Revista colombiana de Gastroenterología, vol. 39, no. 1, pp. 105-111, 2024

Asociación Colombiana de Gastroenterología

Received: 28 April 2023

Accepted: 05 June 2023

DOI: https://doi.org/10.22516/25007440.1074

Abstract: Bile duct cysts are characterized by intrahepatic or extrahepatic bile duct dilation. It is a rare congenital pathology, diagnosed mainly in children. The clinical manifestation in adults is usually nonspecific but essential due to their increased risk of developing carcinoma. We present the case of a 37-year-old female patient who consulted for pain in the epigastrium radiating to the right hypochondrium, associated with emesis and choluria. The hepatobiliary ultrasound was normal, but due to the risk of choledocholithiasis, a magnetic resonance cholangiopancreatography was performed, revealing a cystic dilation of the proximal common bile duct of approximately 2 cm, classified as Todani type I. Bile duct cysts have been associated with several complications, and cholangiocarcinoma is the most important. The probability of malignancy is higher in adults and Todani type I cysts.

Keywords: Common bile duct cyst, common bile duct, abdominal pain, Roux-en-Y anastomosis, case reports.

Resumen: Los quistes de vía biliar se caracterizan por la dilatación de los conductos biliares intrahepáticos o extrahepáticos. Es una patología congénita poco frecuente, diagnosticada principalmente en niños. La presentación clínica en adultos suele ser inespecífica, pero importante debido a su riesgo aumentado de desarrollar carcinoma. Se presenta el caso de una paciente de 37 años que consulta por dolor en epigastrio irradiado al hipocondrio derecho, asociado a emesis y coluria. La ecografía hepatobiliar resultó normal, pero debido al riesgo de coledocolitiasis se realizó una colangiopancreatografía por resonancia magnética en la que se evidenció una dilatación quística del colédoco proximal de aproximadamente 2 cm, clasificada como Todani tipo I. Los quistes de vía biliar se han asociado a varias complicaciones, y el colangiocarcinoma es la más importante. La probabilidad de malignización es mayor en adultos y en los quistes Todani tipo I.

Palabras clave: Quiste del colédoco, conducto del colédoco, dolor abdominal, anastomosis en Y de Roux, informes de casos.

Introduction

Choledochal cysts, also known as bile duct cysts, are congenital abnormalities characterized by the dilation of the intrahepatic or extrahepatic biliary ducts. They account for 1% of hepatic anomalies with estimated incidences ranging from 1 in 100,000 to 1 in 150,000 in Western populations and as frequent as 1 in 1,000 in certain Asian populations¹^,², with a female predominance³. Although typically identified in childhood, accounting for approximately 25% of cases⁴, the incidence in adults is on the rise⁵^,⁶.

The underlying cause of persistent choledochal cysts is unclear, but the most widely accepted hypothesis is that proposed by Babbit in 1969⁷. This theory posits an anomalous junction of the pancreatic duct and the bile duct, which merge 1.5-4 cm distal to the sphincter of Oddi, forming a “common channel” that permits the reflux of pancreatic secretions into the biliary duct, a condition known as “pancreatobiliary reflux”. The resulting activation of pancreatic enzymes leads to inflammation and thickening of the bile duct walls, prompting dilation. This is supported by reports of elevated amylase in the bile in patients with this conditions compared to those without it⁸^,⁹. Babbit’s theory accounts for 50%-80% of cases since not every patient with this long common channel develops a cyst; likewise, not every patient with a choledochal cyst has such a channel¹⁰. Alternative theories propose the absence of ganglion cells, dysfunction of the sphincter of Oddi, and a weakened bile duct wall¹¹^,¹².

In 1959, Alonso-Lej¹³ categorized choledochal cysts; subsequently, Todani and colleagues¹⁴ revised this classification, which is now widely adopted. Five types of choledochal cysts are delineated based on location and morphology (Table 1)¹⁵. Clinical presentation varies according to age groups.

Table 1

Todani’s Classification of Choledochal Cysts and Their Frequency.

Author’s own research.

Prenatal ultrasound diagnoses have been made at an average gestational age of 20 weeks¹⁶^,¹⁷. The classical triad of pain, right hypochondrial mass, and jaundice is primarily observed in children¹⁸. However, studies suggest that this triad’s prevalence is relatively low¹⁸^-²⁰. In adults, symptom presentation is typically vague and nonspecific, with abdominal pain being the most frequently reported symptom¹^,²⁰. When more specific symptoms do occur, they tend to be associated with complications such as cholangitis and pancreatitis¹^,⁸^,²¹. Laboratory parameters are not diagnostic, though findings may include hyperamylasemia, raised levels of direct and total bilirubin, aminotransferases, and alkaline phosphatase²²^,²³.

Diagnosis of a bile duct cyst relies on identifying biliary dilation once other causes have been excluded, such as neoplasia or stones²⁴. Ultrasound often serves as the initial imaging modality, proving highly effective in detecting cystic structures with a sensitivity rate between 71%-97%, particularly in pediatric cases²^,¹¹^,²³^,²⁵. Computed tomography is instrumental in illustrating the cyst’s continuity with the biliary tree, as well as assessing its relations and potential malignancy²⁶. Endoscopic retrograde cholangiopancreatography (ERCP) has largely been superseded by magnetic resonance cholangiopancreatography (MRCP), which remains useful in diagnosing choledochoceles⁸^,²⁷^,²⁸. MRCP is lauded for its precise delineation of anatomical pathology, including abnormal pancreatobiliary junctions, and for detecting ductal stones and tumors, rendering it the gold standard without the associated risks of ERCP²⁹^,³⁰.

Case presentation

We present the case of a 37-year-old female with a surgical history inclusive of hysterectomy and three prior hospitalizations for epigastric pain, which ultrasound assessments had interpreted as acid peptic disease. The patient sought consultation for epigastric pain radiating to the dorsolumbar region described as a burning sensation, rated 8/10 in intensity, accompanied by multiple episodes of food and bilious vomiting, oral intake intolerance, and choluria. Physical examination did not reveal signs indicative of an acute abdomen. Laboratory tests indicated positive C-reactive protein (CRP), with elevated total bilirubin at 3.16 mg/dL, direct bilirubin at 2.90 mg/dL, alkaline phosphatase at 175 U/L, alanine aminotransferase (ALT) at 300.7 U/L, aspartate aminotransferase (AST) at 248.2 U/L, and urinalysis displaying bilirubin at 1 mg/dL with no additional anomalies.

Subsequent abdominal ultrasound reported a gallbladder containing small calculi, with normal-caliber intra- and extrahepatic bile ducts and a common bile duct measuring 4.5 mm. Owing to the risk of choledocholithiasis, an MRCP was performed, which depicted a normal-sized gallbladder devoid of calculi, cystic dilation of the proximal common bile duct approximately 2 cm below the hepatic duct bifurcation extending to the ampulla of Vater, absence of stones in the common bile duct, and non-dilated intrahepatic bile duct (Figure 1).

Figure 1
Magnetic resonance cholangiopancreatography (coronal section) with a green arrow pointing to the choledochal cyst.
Author’s own research.

The patient was diagnosed with a choledochal cyst and underwent cyst resection followed by bilio-digestive bypass via Roux-en-Y hepaticojejunostomy (Figure 2).

Figure 2
Roux-en-Y hepaticojejunostomy.
Author’s own research.

The surgical exploration revealed a distended gallbladder, a 3 mm cystic duct, and a choledochal cyst measuring 5 x 4 cm with a proximal neck situated 5 mm from the bifurcation of the hepatic ducts and a distal retro-duodenal neck with thickened walls adherent to the portal vein (Figure 3). Two pathological samples were taken: one from the choledochal cyst and one from the gallbladder (Figure 4).

Anatomical structures. 1. Confluence of the hepatic ducts. 2. Portal vein. 3. Right hepatic artery originating from the superior mesenteric artery.

Figure 3
Anatomical structures. 1. Confluence of the hepatic ducts. 2. Portal vein. 3. Right hepatic artery originating from the superior mesenteric artery.
Author’s own research.

Figure 4
Pathological specimen of the choledochal cyst.
Author’s own research.

Results

The surgical procedure was carried out without any adverse events, and the patient’s postoperative recovery was free of complications. At a follow-up visit fifteen days post-surgery, the patient presented a pathology report which confirmed acute cholecystitis and a benign choledochal cyst.

Discussion

The diagnosis of choledochal cysts in the adult population is becoming increasingly frequent¹^,¹⁹^,²⁴^,³⁰. In the case under discussion, the definitive diagnosis was established via MRCP after the initial ultrasound yielded inconclusive results. The clinical symptoms and laboratory findings were indicative of cholestasis, prompting the decision to proceed with more definitive imaging. While ultrasound remains the most accessible and cost-effective imaging technique, its variable sensitivity ranging from 71% to 97% can miss choledochal cysts, unlike MRCP, which boasts a detection rate of 96% to 100%²⁹. Prompt diagnosis and intervention are paramount given the elevated risk of complications associated with choledochal cysts in adults, which include obstructive jaundice, symptomatic choledocholithiasis, pancreatitis, secondary biliary cirrhosis, and notably, cholangiocarcinoma¹^,³¹^,³². The malignancy risk is reported to be approximately 10%-30%⁶^,²⁹^,³⁰, and it escalates with age-from 0.7% in the first decade to over 10% beyond the second decade, with more than half of the patients older than 60 years with choledochal cysts showing malignancy¹^,²². The cyst type is another risk factor, with type I presenting the highest incidence at 68%, followed by type IV at 21%⁶^,¹⁴^,³³.

Consequently, the management of choledochal cysts consists of complete surgical excision⁶^,³⁴. Resection of the cyst along with Roux-en-Y hepaticojejunostomy is the procedure of choice for type I and IV cysts²⁹^,³⁰, and partial hepatectomy may be necessary for type IVA. Type II cysts warrant excision and possibly biliary-enteric repair²^,²⁹^,³⁵. Treatment for type III cysts may involve sphincteroplasty, sphincterotomy³⁶, or the insertion of a drainage tube into the duodenum¹¹. For Todani type V, options include segmental liver resection, liver transplantation, or close patient monitoring to preempt complications²⁹^,³⁷^,³⁸.

The emergence of minimally invasive surgery has established laparoscopic surgery as a safe and valid alternative. When compared to open surgery, the laparoscopic approach reduces the need for transfusions and shortens the recovery period, despite a typically longer operative duration³⁹^,⁴⁰.

Conclusions

Diagnosing biliary cysts is a significant challenge due to the nonspecific nature of the symptoms and signs associated with the condition. Ultrasound is an effective and cost-effective first-line imaging modality for diagnosing biliary cysts; however, as evidenced in the presented case, a negative ultrasound does not eliminate the possibility of disease. Hence, MRCP is crucial for definitive diagnosis, serving as the gold standard in the detection of choledochal cysts and in ruling out lithiasis and neoplasia. Cyst resection remains the preferred treatment approach due to the significant risk of malignant transformation associated with these cysts.

Acknowledgments

This research did not receive any specific grant funding.

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Notes

Citation: Medina-Ortega MA, Vallejo-Vallecilla G, Gómez-Mosquera AV. Todani Type I Common Bile Duct Cyst in an Adult Patient: Clinical Case and Literature Review . Revista. colomb. Gastroenterol. 2024;39(1):105-111. https://doi.org/10.22516/25007440.1074

Author notes

*Correspondence: Marco Antonio Medina-Ortega. mmedinao@unicartagena.edu.co