Doubly complicated: A case of massive non-parasitic liver cyst presenting with dyspnea and inferior vena cava compression: A rare case report

Abstract

Introduction and importance

Simple hepatic cysts, common benign liver conditions, are increasingly detected incidentally due to advancements in imaging technologies. While typically asymptomatic, complications such as compression of neighboring structures can arise, presenting unique diagnostic and management challenges. We present a doubly complicated case of a massive non-parasitic liver cyst in a 61-year-old female patient, manifesting with dyspnea and compression of the inferior vena cava.

Case presentation

A 61-year-old female with a history of treated hypertension presented with worsening dyspnea over six months. Physical examination revealed a large, painless abdominal mass, and imaging confirmed a 20 cm cystic liver mass compressing the inferior vena cava and exerting a mass effect on the diaphragm. Surgical exploration and deroofing of the cyst led to successful resolution.

Clinical discussion

The presentation of dyspnea in non-parasitic liver cysts is rare but notable, highlighting the importance of considering hepatic etiologies in respiratory symptoms. Imaging modalities such as ultrasound and CT play crucial roles in diagnosis, while MRI aids in ruling out biliary-cystic fistulas. Surgical management, particularly subcostal laparotomy, remains a viable option for complex cases.

Conclusion

This case underscores the need for heightened awareness of atypical presentations of non-parasitic liver cysts and the significance of imaging in diagnosis. Subcostal laparotomy, though associated with limitations, remains valuable in select cases. Further research comparing surgical approaches is warranted to optimize management strategies for symptomatic non-parasitic liver cysts.

Highlights

• •This presentation details a rare case of a massive non-parasitic liver cyst, complicated by dyspnea and compression of the inferior vena cava.
• •Imaging modalities including CT scans and biliary MRI were crucial in diagnosis, revealing compression not only of adjacent structures such as the inferior vena cava and the ipsilateral kidney but also demonstrating displacement of the liver against the diaphragmatic dome.
• •Successful resolution achieved through cyst deroofing, emphasizing the doubly complicated nature of the case.
• •This case highlights the atypical presentation and complications associated with hepatic cysts, emphasizing the importance of considering hepatic etiologies in such cases and the crucial role of imaging in guiding management strategies.

1. Introduction

Simple hepatic cysts, which are non-parasitic in nature, represent a prevalent form of benign liver ailment, affecting roughly 1–5 % of individuals within the general population [1]. Advancements in imaging technology have led to a rise in the inadvertent identification of these cysts through abdominal ultrasonography or computed tomography (CT) [2].

These cysts typically present as asymptomatic, permitting conservative follow-up without targeted intervention [1,3]. Nevertheless, certain cysts may be linked with complications including infection, hemorrhage, obstructive jaundice, portal hypertension, and rupture [1,4]. Exceptionally, it can be revealed by respiratory symptoms such as dyspnea [1]. Herein, we elucidate a particularly intricate scenario involving a massive non-parasitic liver cyst in a 61-year-old female patient, further complicated by dyspnea and compression of the inferior vena cava. Successful resolution was attained through cyst deroofing, underscoring the doubly complex nature of the case. This work has been reported in line with the SCARE 2023 criteria [5].

2. Case presentation

This is a 61-year-old female patient with a history of treated hypertension and no surgical history. She presented to the emergency department with dyspnea progressing over the past 6 months, with recent worsening of her symptoms. Further questioning did not reveal any other associated functional signs.

The physical examination revealed stable hemodynamic parameters with a blood pressure of 120/80 mmHg and a heart rate of 80 bpm. She had a respiratory rate of 16 cycles per minute and normal oxygen saturation. Cardio-pulmonary auscultation showed decreased breath sounds at the right lung base. She had soft pitting edema in both lower limbs. The abdominal examination revealed a distended, soft, easily depressible, and painless abdomen with the presence of a mass in the right hypochondrium. The mass was fixed, with a soft consistency, and measured 20 cm in diameter.

The electrocardiogram showed no abnormalities. Laboratory findings were unremarkable, notably with normal troponin levels. The chest X-ray showed an elevation of the right diaphragmatic dome with no other abnormalities.

The thoraco-abdominal CT scan revealed the existence of a large cystic mass in the liver's right lobe. It exhibited an exophytic growth beneath the liver, likely originating from segment VI. The mass appeared unilocular, lacking intra-cystic buds or septa, and possessed a thin enhanced wall measuring 21 cm. Additionally, the cyst was noted to exert a mass effect on both the inferior vena cava and the right diaphragmatic dome (Fig. 1). The complementary investigation by biliary MRI concluded the presence of the same exophytic cystic mass measuring 21 cm in its largest dimension, compressing the inferior vena cava. It also displaces the gallbladder and the ipsilateral kidney and presses the liver against the diaphragmatic dome and the anterior abdominal wall. Furthermore, there is no communication between the cyst and the bile ducts (Fig. 2).

Fig. 1.

Axial (A) and sagittal (B) CT scan images demonstrating the bile cyst displacing the liver against the right diaphragmatic dome (red arrow).

Fig. 2.

Biliary MRI showing the bile cyst compressing the inferior vena cava (blue arrow) and the ipsilateral kidney (yellow arrow).

Given negative hydatid serologies and the clinical-radiological findings, the most likely diagnosis was a large symptomatic biliary cyst causing dyspnea.

The patient underwent surgery via the right subcostal approach: exploration revealed a cystic mass affecting segment VI of the liver, measuring 20 cm with a thin wall, serous content, pushing forward the gallbladder, medially the gastric antrum, and compressing the inferior vena cava (IVC) from behind, suggesting the appearance of a giant biliary cyst. Additionally, it was noted that the cyst exerted a mass effect on the upper portion of the diaphragmatic dome without intra-thoracic communication (Fig. 3, Fig. 4). The cyst was subjected to deroofing, extending to the margins of the liver parenchyma, and meticulous examination was conducted on the internal surfaces of the cyst walls to identify the location of biliary communication. The exploration did not find any bile fistula. Drainage of the residual cavity was performed. Pathological analysis revealed the presence of a non-parasitic simple hepatic cyst.

Fig. 3.

Intraoperative image of the bile cyst.

Fig. 4.

Intraoperative image after opening and aspiration of the cyst.

The postoperative course was uneventful, and the patient was discharged on the third day after surgery. The clinical and radiological follow-up after 3 months did not show any abnormalities.

3. Discussion

The biliary cyst of the liver refers to an intrahepatic formation with serous liquid content, circumscribed by an epithelium identical to that of the bile ducts, with which it has no communication [4].

Hepatic simple cysts typically manifest asymptomatically and are commonly discovered incidentally via abdominal imaging modalities [1,6]. Simple cysts may present as solitary or multiple lesions, varying in size from millimeters to exceeding than 20 cm in diameter [2,4,7]. While symptoms are infrequent, complications may arise due to intracystic bleeding, rupture, or secondary bacterial infection [2,6]. Compression of neighboring structures may ensue, such as compression of the inferior vena cava leading to lower extremity edema, compression of the portal vein culminating in portal hypertension, or compression of the biliary tree leading to cholestasis [1,2,6]. The lower limb edema observed in our patient may be attributed to the mass effect of the cyst on the inferior vena cava in the absence of any other evident cause. The dyspnea represents an exceptional mode of presentation of symptomatic biliary cysts, such as in our case [1]. In our case, this phenomenon arises from a mass exerting pressure on the diaphragmatic dome.

The escalating utilization of diagnostic imaging techniques, such as ultrasonography and CT scans, has facilitated the detection of a growing prevalence of hepatic cysts within the general population [1,8]. In our case, abdominal ultrasound was not performed due to the absence of any functional signs or physical examination findings suggestive of hepatobiliary pathology. Ultrasound typically confirms the diagnosis of uncomplicated biliary cysts, while preoperative CT scanning is imperative for assessing complicated forms and strategizing the surgical approach [1,6]. Classically, simple hepatic cysts are well-defined, rounded, hypodense, both before and after intravenous contrast injection [1,9]. The main differential diagnoses are mainly with hydatid cyst and cystadenoma [8,9]. Hence, the importance of preoperative hydatid serology and postoperative histological examination [3]. Biliary MRI offers the advantage of providing better visualization of the biliary tree to rule out a biliary-cystic fistula [[8], [9], [10]]. In our case, MRI ruled out any communication with the bile ducts.

The standard treatment for symptomatic biliary cysts is optimal resection of the protruding dome via a laparoscopic approach [1]. The occurrence of pain is the main indication for this [1,4]. Due to its minimally invasive nature, this method has the advantages of shortening both the operative time and the hospital stay [1]. However, this intervention, practically effective in symptom regression, is not without the risk of recurrence [1,6]. Factors for recurrence include adhesions (preventing proper excision), deep cyst localization, or a cyst located in the posterior segment of the liver [1,9].

These situations can be easily approached conventionally [2]. Nevertheless, these recurrences were better accepted than the results generated by other surgical procedures, including cystectomy, lobectomy, or hepatectomy [1,2]. In curative intent, these radical methods were definitively effective but seem excessive and burdened with quite significant morbidity (50 %) [1,4,9]. Alternatively, the method of puncture-aspiration alone has been abandoned due to resulting recurrences (100 %) [1,9]. Percutaneous sclerotherapy allows a significant improvement in long-term results, but it can be poorly tolerated due to debilitating abdominal pain, especially after alcohol use [1]. It is contraindicated in the case of a cyst with hemorrhagic content [1,3,6]. However, compared to conventional or laparoscopic resection, it represented in the literature a feasible but unvalidated alternative, as no randomized controlled study comparing these two methods has been reported [1,2,9].

In our case, sclerotherapy was not available in our hospital. Subcostal laparotomy was the sole surgical approach considered, as laparoscopic intervention was not pursued owing to insufficient expertise in managing giant biliary cysts via laparoscopy.

With this method, we achieved better exposure of the liver and the cyst, allowing for a cautious optimal resection. Despite its complications (bile leakage) and drawbacks (long operative time and hospital stay), it retains a crucial role in three situations: treatment of recurrences, failure of the laparoscopic approach requiring conversion, and drainage of an associated pancreatic pseudocyst [1].

The technique of transcystic air injection during the deroofing operation for hepatic cysts with a small cyst–biliary communication was useful for the detection of the biliary orifice inside the cyst cavity. Surgeons should be aware of this technique as a useful option to obtain favorable outcomes, with reduced postoperative bile-related morbidity [10]. Additionally, the utilization of indocyanine green fluorescence as a similar technique can enhance intraoperative visualization and aid in identifying the biliary structures, further contributing to successful surgical outcomes and decreased postoperative complications [8].

4. Conclusion

In summary, this case highlights two noteworthy aspects: the uncommon presentation of dyspnea in patients with symptomatic non-parasitic liver cysts and the notable compression effect on the inferior vena cava by these cysts. While ultrasound confirms uncomplicated biliary cysts, preoperative CT scanning is crucial for complex cases [9].

Although subcostal laparotomy has limitations, it remains a viable option for select cases, particularly for recurrent cysts or when laparoscopic intervention is not feasible [1]. Further studies comparing surgical approaches are warranted to guide optimal management strategies for symptomatic non-parasitic liver cysts [1,9].

Patient consent

Written informed consent was obtained from the patient for the publication of this case report and its accompanying images. A copy of the written consent is available for the Editor-in-Chief of this journal to review upon request.

Ethical approval

Ethical approval is not applicable/waived at our institution.

Funding

This research did not receive funding from any specific grant provided by public, commercial, or not-for-profit organizations.

Guarantor

Dr. Ahmed Omry.

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CRediT authorship contribution statement

Hager Behi and Ahmed Omry contributed to manuscript writing and editing, and data collection; Amel Changuel and Rania Dallagi contributed to data analysis; Hanene Guelmami and Med Bachir Khalifa contributed to conceptualization and supervision; all authors have read and approved the final manuscript.

Declaration of Generative AI and AI-assisted technologies in the writing process

AI tools were not used for the elaboration of the manuscript.

Declaration of competing interest

No conflicts of interest.