Congenital non-parasitic hepatic cyst complicated by hepatoduodenal fistula: a rare case report

Abstract

Introduction and importance:

Simple hepatic cysts are benign and usually asymptomatic; however, they can rarely develop serious complications, such as hepatoduodenal fistula, due to infection, cyst size, or mechanical pressure.

Case presentation:

A 68-year-old male presented with fever, hematochezia, and severe right upper quadrant pain. Imaging revealed a 10 × 12 cm segment V–VII hepatic cyst with an air–fluid level, and endoscopy showed a fistulous opening in the second duodenum adjacent to a necrotic diverticulum. After stabilization, laparotomy confirmed a hepatoduodenal fistula; a 2 cm opening was closed with an omental patch, the cyst was deroofed, and a drain was placed, resulting in excellent recovery.

Clinical discussion:

This rare hepatoduodenal fistula arose from an infected simple hepatic cyst (pathology: cuboidal epithelium; negative Echinococcus serology), driven by inflammation and mechanical stress. Percutaneous drainage failed due to enteric communication; surgery was definitive.

Conclusion:

Infected simple hepatic cysts can rarely progress to a hepatoduodenal fistula with significant morbidity, but prompt diagnosis, resuscitation, antibiotics, and definitive surgical repair can result in full recovery.

Introduction

Cystic hepatic lesions affect approximately 2.5–18% of the population. Their pathogenesis remains unclear, and they are classified as true or false depending on the presence of an epithelial lining. True cysts include congenital cysts (simple cysts and polycystic liver disease), parasitic (hydatid) cysts, neoplastic cysts, and biliary duct-related cysts. False cysts result from spontaneous intrahepatic hemorrhage, post-traumatic hematoma, or intrahepatic biloma. These lesions are further divided into simple and complex cysts. Hepatic simple cysts (HSCs), the most common type, are benign and usually asymptomatic. They are congenital, lined by cuboidal epithelium, and arise from abnormal intrahepatic duct development in utero. They are typically stable but may enlarge and become symptomatic due to mass effect, rupture, hemorrhage, or infection. Complex hepatic cysts include congenital, premalignant, neoplastic, infectious, and post-traumatic lesions^[1,2^].

HIGHLIGHTS

• Rare hepatoduodenal fistula from an infected simple hepatic cyst mimicking hydatid disease.

• Failed percutaneous drainage due to enteric communication necessitates surgery.

• Omental patch repair with cyst deroofing achieved complete fistula healing.

• Postoperative endoscopic retrograde cholangiopancreatography resolved the biliary leak in a hydatid-endemic region.

• Multidisciplinary approach key for sepsis and hemorrhage stabilization.

Hydatid cyst is an endemic disease in Mediterranean and Middle Eastern countries[3]. In this case, the patient was thought to have a hydatid cyst, but it turned out to be a simple hepatic cyst after pathological tests were done.

We report a case of a 68-year-old male with a simple hepatic cyst that progressed to an abscess and fistulized into the duodenum.

Case presentation

We admitted a 68-year-old male with a complicated medical history that included unilateral optic neuropathy, peripheral neuropathy, and ischemic heart disease with coronary stenting. His medications included antiplatelet agents, azathioprine, and antihypertensive agents. He presented with upper gastrointestinal (GI) hemorrhage (hematemesis), lower GI hemorrhage (hematochezia), high temperature, and severe right upper quadrant abdominal pain. He was hemodynamically unstable at the time of admission and needed fluid resuscitation, blood transfusions, and broad-spectrum antibiotics (Table 1). After 6 days, an upper GI endoscopy revealed a 5 cm duodenal diverticulum in the second section of the duodenum with a necrotic base but no active bleeding. We suggest that the cyst was the primary source of bleeding, occurring secondary to fistulization into the duodenum, and that it stopped spontaneously.

Table 1.

Lab values.

	Upon admission	Prior to operation	Unit
WBC	4.5	6	109/L
Hb	6.8	11.1	g/dL
Plt	300	211	109/L
Na	128	130	mEq/L
K	-	4.2	mEq/L
Ast	20	17	U/L
Alp	392	-	U/L
TB	0.95	0.6	mg/dL
Glu	95	74	mg/dL
Amy	81	-	U/L
PT	76	-	s
aPTT	36.8	-	s
LDH	495	-	U/L
Creat	0.8	0.7	mg/dL

LDH, lactate dehydrogenase; PT, prothrombin time; TB, total bilirubin; WBC, white blood cell count.

A contrast-enhanced computed tomography (CT) scan with oral contrast of the abdomen and pelvis was performed and showed a well-defined hepatic cyst with a calcified wall and an air-fluid level, located in segments V–VII of the liver, measuring 10 × 12 cm, indicating a liver abscess. The contrast material was taken orally, which means that the presence of both an air-fluid level and the contrast material entering the cyst cavity confirms the existence of a communication between the intestinal tract and the cyst (Fig. 1). Percutaneous drainage was attempted but failed due to technical limitations. A CT scan was performed, and the findings confirmed that the pigtail catheter was located outside the cyst cavity. Following stabilization, the patient underwent an exploratory laparotomy. During surgery, we discovered a large hepatic cyst adherent to the second portion of the duodenum. While dissecting the cyst, a fistulous connection to the second duodenal segment was identified, with a fistulous opening measuring approximately 2 cm (Fig. 2). The fistula opening was not primarily closed; instead, an omental patch was used to close the fistula defect. The cyst wall was then deroofed, and an internal drain was placed, where a moderate amount of duodenal enteric fluid was found within the cyst cavity (Fig. 3). The patient recovered well after surgery. On day 3, we noted biliary drainage of approximately 300 mL/day from the surgical drain. Bile output rapidly decreased after endoscopic retrograde cholangiopancreatography (ERCP) was performed. On the seventh postoperative day, the drain was removed. A follow-up upper GI endoscopy 4 weeks later revealed that the duodenal fistula had almost fully healed. After 6 weeks, a short-term follow-up upper endoscopy was performed, which showed improvement in the fistula opening (Figure Supplemental Digital Content S1, available at: http://links.lww.com/IJSCR/A57) The work has been reported in line with the SCARE criteria and the PROCESS criteria^[4,5^].

Figure 1.

Contrast-enhanced axial CT of the abdomen showing hepatic cyst in segments V–VII with air-fluid level (arrow mark).

Figure 2.

Intraoperative image showing the fistula between the second segment of the duodenum and the hepatic cyst (blue arrow) and the cavity of the cyst (white arrow).

Figure 3.

Intraoperative images. (A) Intraoperative view of the cyst deroofing. (B) Intraoperative image showing the surgical closure of the duodenal fistulas opening with an omental patch (white arrow) and the cavity of the cyst after the wall was deroofed (blue arrow).

Discussion

Epidemiology and classification

Hepatic cystic lesions can originate from an infectious or non-infectious source. The non-parasitic category encompasses the most prevalent type, the simple hepatic cyst. This type of cyst has a minimal female predilection (about 4 to 1), usually diagnosed after the age of 40[6].

Differential diagnosis

The differential diagnosis of hepatic cysts includes a wide range of simple, infectious, and neoplastic conditions. Simple hepatic cysts are the most common and are typically asymptomatic[7]. Other simple lesions include biliary hamartomas, adult polycystic liver disease, and ciliated hepatic foregut duplication cysts. Neoplastic cystic lesions, post-traumatic or iatrogenic cysts, hematomas, and bilomas also enter the differential[8].

Clinical presentation and pathogenesis

HSCs are benign and rarely symptomatic lesions[1]. They occasionally become symptomatic due to mass effect, rupture, hemorrhage, or infection[2]. Pyogenic liver abscesses develop secondary to ascending cholangitis, hematogenous dissemination, or direct extension. The incidence is higher in middle-aged or elderly patients who present with low-grade evening fever, right upper quadrant pain, fatigue, tender hepatomegaly, and elevated white blood cell count[1]. Clinical features of fistulae into the duodenum are non-specific. The most frequently reported symptoms are abdominal pain, nausea, vomiting, and anemia^[9,10^] (Table 2).

Table 2.

Clinical presentation and pathogenesis compared with the literature.

Feature	Literature frequency	This case
RUQ pain	Common	Present
Anemia	Mostly hydatid cases	Present
Fistula	Rare	Hepatoduodenal

RUQ, right upper quadrant.

Fistulization pathogenesis

Factors like infection and inflammation might play a role in the pathogenesis of fistulation[11]. The repeated mechanical stress from ventilatory movement serves as a significant contributing factor. A case published in the American Journal of Respiratory and Critical Care Medicine described a hepatopulmonary fistula following mechanical ventilation in a patient with a previously asymptomatic hepatic cyst[12].

The persistent compression and subsequent mechanical friction due to the large cyst might have resulted in a progressive erosion of the adjacent duodenal wall. However, it is thought that larger cysts are at risk of infection, which will elevate the risk of fistulation[2]. The location of a hepatic cyst might affect the site of fistulation. There is a case report that describes an infected hepatic cyst in the left hepatic lobe with a fistula into the duodenum[11].

Diagnostic approach

An abdominal ultrasound is a rapid and non-invasive tool for the diagnosis of liver abscess. Air within an abscess cavity should prompt evaluation for hepatobronchial or hepatoenteric fistula, or secondary infection. To confirm the presence of a hepatoenteric fistula, a water-soluble barium swallow and/or CT with oral contrast is a pertinent radiological investigation[13]. The endoscopic assessment also becomes of critical value in such cases due to the direct communication of the liver with the GI system[14]. Furthermore, in our case, the resected tissue from the cyst wall underwent pathological examination, which revealed cuboidal epithelium consistent with a simple hepatic cyst (Figure Supplemental Digital Content S2, available at: http://links.lww.com/IJSCR/A58)

Management and treatment

Initial management typically involves broad-spectrum antimicrobial therapy and hemodynamic stabilization. However, definitive control of the infection source is paramount. While percutaneous catheter drainage (PCD) remains the first-line intervention for hepatic abscesses, its efficacy is significantly compromised in the presence of enteric fistulization. Persistent communication between the cyst and the duodenum allows ongoing contamination, impedes cavity resolution, and increases the risk of systemic sepsis. Saidani et al and Atri et al both describe cases where PCD failed due to fistula formation, necessitating surgical exploration^[9,15^].

Surgical repair remains the cornerstone of treatment. Literature supports the use of omental reinforcement in closing cysto-duodenal fistulas, given its vascularity and immunologic properties that promote healing in friable tissue. Saeki et al reported successful laparoscopic management of a similar cysto-duodenal fistula using omental reinforcement[11].

Postoperative biliary leakage, a known complication of cyst deroofing, was effectively managed with ERCP, underscoring the importance of multidisciplinary coordination in postoperative care. Imaging follow-up and serial inflammatory markers confirmed resolution of the infection and healing of the fistula tract.

Prognosis and follow-up

Recurrence remains a concern in hepatic cyst disease, particularly in hydatid cysts. In non-parasitic cysts (as we have in our case), recurrence is less frequent but may occur if drainage is inadequate or if the fistulous tract persists[16]. In this case, a 3-month CT follow-up confirmed complete cyst resolution.

Strengths and limitations

Strengths: Rare non-hydatid hepatoduodenal fistula with comprehensive imaging, pathology, and surgical detail. Limitations: Short-term follow-up limits long-term recurrence data.

Conclusion

Although congenital hepatic cysts are usually asymptomatic, rare life-threatening complications may occur, as in this case. Management requires prompt stabilization, antibiotics for infection, and definitive surgical repair of the fistula.

Ethical approval

Not applicable.

Consent

Not applicable.

Sources of funding

No funding.

Author contributions

A.M.: Surgical procedures, manuscript writing, and supervision. R.M.: Data collection, manuscript writing, and manuscript editing. A.M.A.M.: Data collection and manuscript writing. L.S.: Data collection and manuscript writing. L.A.T.: Data collection and manuscript writing. R.H.: Surgical procedures and supervision.

Conflicts of interest disclosure

No conflicts of interest to report.

Research registration unique identifying number (UIN)

Not applicable.

Guarantor

Ammar Mouhammad.

Provenance and peer review

Not applicable.

Data availability statement

Not applicable.