Chronic Appendicitis-Induced Effusive-Constrictive Pericarditis and Hepatic Fibrosis: A Case Report

Introduction

Constrictive pericarditis is a rare but severe condition characterized by fibrosis, calcification, and adhesions within the pericardial layers. It leads to impaired diastolic filling of the heart and significant life-threatening hemodynamic compromise [1]. Effusive-constrictive pericarditis (ECP), an uncommon variant of pericarditis, involves the coexistence of pericardial effusion and constriction of the visceral pericardium. This dual pathology creates a tamponade-like effect despite fluid drainage and limits the diastolic relaxation of the heart [2]. ECP shares its etiology with constrictive pericarditis, including bacterial or viral infections, malignancy, trauma, uremia, post-myocardial infarction syndromes, and post-cardiac surgery complications. Rarely, inflammation from non-cardiac sources, such as intra-abdominal infections, can affect the pericardium through hematogenous or direct spread [3]. Recently, it has been reported as a complication of COVID-19 infection [4].

Appendicitis, a most frequent surgical emergency in the pediatric population, is complicated by atypical presentations in up to one-third of cases [5]. The initial missed diagnosis rate is between 28% and 57%, with a higher frequency in the younger population [6]. A potential distractor is an appendicolith, a calcified deposit within the appendix. It can create diagnostic and clinical decision-making dilemmas, particularly when it is isolated, without clinical signs. Appendicoliths are found incidentally in up to 32% of asymptomatic individuals but are present in approximately 28–30% of appendicitis cases [7].

This report presents the unique case of a 14.5-year-old boy with effusive-constrictive pericarditis and hepatic fibrosis secondary to chronic appendicitis associated with an appendicolith. It illustrates the diagnostic challenges of appendicitis and pericarditis, the limitations of pericardiocentesis, and the essential need for a multidisciplinary approach to managing complex and atypical presentations.

Case Report

INITIAL LABORATORY AND IMAGING FINDINGS:

Initial laboratory tests demonstrated an inflammatory response with mild leukocytosis (WBC 11.2×109/L, neutrophils 71%), an elevated C-reactive protein (CRP) level of 15.1 mg/L, and a high erythrocyte sedimentation rate (ESR) of 43 mm/h (Table 1). Liver function tests showed mild abnormalities, with aspartate aminotransferase (AST) at 48 U/L, alanine aminotransferase (ALT) at 52 U/L, and lactate dehydrogenase (LDH) at 312 U/L, suggesting hepatic involvement. Albumin levels were mildly decreased at 34 g/L, indicative of systemic inflammation. An extensive infectious workup, including serology and PCR for common viral and bacterial pathogens (eg, Epstein-Barr virus, cytomegalovirus, herpes simplex virus, and Mycoplasma), was negative. Autoimmune markers, including antinuclear antibodies (ANA) and antineutrophil cytoplasmic antibodies (ANCA), were also negative, making systemic autoimmune disease an unlikely cause. Imaging findings raised concerns about potential multisystem involvement. A chest X-ray revealed an enlarged cardiac silhouette with small bilateral pleural effusions (Figure 1A). Echocardiography showed a large pericardial effusion up to 3 cm in thickness, with diastolic collapse of the right-sided heart chambers, suggestive of tamponade (Figure 1B). Liver ultrasound demonstrated hepatomegaly (span 16 cm) with heterogeneous echotexture, splenomegaly (14 cm), mild ascites, and dilation of the hepatic veins and inferior vena cava, suggesting venous congestion.

Computed tomography (CT) confirmed the substantial pericardial effusion (Figure 2A) and identified a calcified appendicolith measuring 19×12 mm in the colon appendix (Figure 2B, 2C). At this stage, priority was given to managing the pericardial effusion and cardiac tamponade.

INITIAL MANAGEMENT AND PERICARDIAL INTERVENTION:

Urgent pericardiocentesis and drainage placement were performed, yielding 600 mL of serosanguineous fluid (Figure 3). The patient’s symptoms temporarily improved, but post-procedure echocardiography demonstrated persistently elevated right and left atrial pressures and constriction of left and right ventricular diastole. Pericardial fluid analysis confirmed an exudative profile with lymphocytic predominance, although microbiological cultures (including aerobic and anaerobic bacteria) and viral PCR were negative. Cytology showed lymphocyte predominance without malignant cells. Despite broad-spectrum antibiotics (ceftriaxone 2 g/day IV and metronidazole 500 mg IV every 8 hours) and dexamethasone 4 mg IV every 8 hours, the patient continued to experience low-grade fever and abdominal pain.

FURTHER INVESTIGATIONS AND DIAGNOSIS OF CONSTRICTIVE PERICARDITIS:

As symptoms persisted, additional imaging studies and consultations were undertaken. A follow-up echocardiogram demonstrated pericardial thickening, while thoracic CT scans revealed persistent pulmonary congestion, hepatomegaly, portal vein dilation, and thickening of the pericardium, all suggestive of constrictive pericarditis (Figure 3A, 3B). Rheumatology evaluation showed no evidence of systemic autoimmune disease, while genetic assessment noted marfanoid features but did not meet the diagnostic criteria for Marfan syndrome. Gastroenterological evaluation, including elastography, revealed hepatic fibrosis (METAVIR F3), likely secondary to chronic congestion from pericardial constriction.

The abdominal MRI indicated hepatomegaly with heterogeneous echotexture and edematous changes, suggestive of possible portal hypertension and compromised venous outflow (Figure 4A). Consistent with Doppler ultrasound findings, the MRI showed intrahepatic vascular flow with preserved respiratory phasic changes, although the hepatic veins widened. The MRI confirmed a thickened pericardial sac with significant diastolic constraint on the heart. Additionally, imaging reaffirmed the presence of a calcified appendicolith in the inflamed appendix, accompanied by mild ascites (Figure 4B).

A general surgery consultation advised deferring an elective appendectomy as there was no immediate need for intervention. However, the presence of the appendicolith and recurrent abdominal discomfort warranted close follow-up to consider appendectomy in the future if symptoms persisted.

SURGICAL INTERVENTION FOR CONSTRICTIVE PERICARDITIS:

Due to refractory symptoms and imaging findings, the patient underwent subtotal pericardiectomy and epicardial decortication via median sternotomy. Intraoperatively, a thick, fibrous white pericardial membrane tightly encasing the heart was visualized (Figure 5A), restricting diastolic expansion (Video 1). After pericardial resection (Figure 5B), cardiac function improved. Histopathological examination confirmed chronic fibrotic pericarditis without evidence of infectious agents.

RECURRENCE OF ABDOMINAL SYMPTOMS AND FINAL DIAGNOSIS:

Following pericardiectomy, the patient experienced significant respiratory and circulatory improvement, with resolution of inflammatory markers (Table 1). He was discharged home. Six weeks later, he presented with acute right lower-quadrant abdominal pain and high fever (39°C). Clinical examination suggested peritonitis. Abdominal ultrasound and CT scan revealed an inflamed, perforated appendix with periappendiceal fluid, indicative of an abscess. An urgent laparotomy confirmed a phlegmonous appendix with a necrotic tip and a surrounding abscess containing the previously noted appendicolith. Appendectomy was performed, and postoperative recovery was rapid, with resolution of fever and abdominal pain.

RESOLUTION AND FOLLOW-UP:

After appendectomy, all residual symptoms resolved. Inflammatory markers normalized within days, liver enzymes returned to baseline (Table 1), and follow-up echocardiography confirmed the absence of recurrent pericardial effusion. Abdomen ultrasound demonstrated resolution of hepatomegaly and splenomegaly, and subsequent elastography showed improvement in hepatic fibrosis, suggesting secondary hepatic changes due to chronic inflammation from appendicitis.

Discussion

Acute appendicitis remains the most common surgical emergency in the pediatric population, yet atypical presentations frequently complicate its diagnosis and management. While the classic symptoms of appendicitis are well known, they are often absent or misleading in children, contributing to delays in diagnosis and, in rare cases, life-threatening complications [8]. The advent of computed tomography (CT) imaging has markedly improved diagnostic accuracy for appendicitis, reducing the rate of negative appendectomies from approximately 20% to 7% [9]. The literature has previously documented pericardial involvement as a rare extra-abdominal manifestation of complicated appendicitis [10]. This case report highlights chronic appendicitis – without acute perforation – as a sustained inflammatory trigger leading to effusive-constrictive pericarditis, hepatic fibrosis, and systemic involvement. Unlike prior cases of acute appendicitis-related pericarditis, this case underscores the role of prolonged, low-grade inflammation, likely driven by an appendicolith. The failure of pericardiocentesis, persistence of cardiac constriction, and complete resolution of symptoms after appendectomy emphasize the need for early recognition of atypical appendicitis to prevent severe inflammatory sequelae.

The presence of an appendicolith is commonly associated with appendicitis, yet it is not a definitive marker of active disease. The literature suggests that although an isolated appendicolith has a high specificity, approaching 100%, for diagnosing appendicitis, its sensitivity remains low at approximately 44%. This indicates that while appendicoliths strongly are correlated with appendicitis, their presence alone is insufficient to establish a definitive diagnosis without additional inflammatory signs [7]. In our patient, the presence of an appendicolith on the initial CT scan raised clinical suspicion for appendicitis. However, surgical intervention was delayed due to the more urgent need to address pericardial effusion and associated cardiac tamponade symptoms. Cardiac catheterization was not performed; however, echocardiographic findings, including diastolic ventricular interdependence, atrial pressure, and respiratory variation in mitral inflow velocities, were consistent with a constrictive physiology. Imaging confirmed significant pericardial thickening, and the persistence of symptoms following pericardiocentesis strongly supported a diagnosis of effusive-constrictive pericarditis. Ultimately, near total pericardiectomy was performed, with special care taken in excising the pericardium around the pulmonary veins, caval veins, and arteries. Although the patient had a mild pectus carinatum deformity co-occurring with scoliosis (both included in the rehabilitation program), the median sternotomy approach was applied to have access to all parts of the heart and all vessels. As it turned out intraoperatively, the chosen access was the right one, as tight rings of constricted pericardium had formed around all the pulmonary veins and around the inferior vena cava, causing significant obstructions to blood flow to the heart.

Pericardial involvement in appendicitis has been documented in a limited number of case reports, yet its pathophysiology remains incompletely understood. Tan et al described 2 pediatric cases in which pericarditis developed as a complication of appendicitis, leading to severe systemic consequences, including pericarditis and intra-abdominal abscess formation [11]. These cases suggested that appendicitis can result in hematogenous or lymphatic dissemination of inflammation, provoking pericardial involvement. Similarly, in the present case, a chronic inflammatory state likely sustained a systemic response that contributed to effusive-constrictive pericarditis. Unlike the patients described by Tan et al, our patient initially exhibited predominantly systemic rather than abdominal symptoms, suggesting a more occult course of appendicitis with a delayed but profound inflammatory impact on the cardiovascular system.

Additional reports have documented similar pericardial complications following appendicitis. Ku et al described a pediatric patient who developed pericardial effusion following laparoscopic appendectomy for perforated appendicitis, ultimately requiring thoracoscopic pericardiotomy [12]. The presence of pericardial involvement after resolution of the primary intra-abdominal process suggests that pericardial inflammation can persist as part of an ongoing systemic response rather than being directly attributable to localized bacterial invasion.

The delayed onset of pericardial symptoms in this patient, as well as the presence of hepatic fibrosis, further supports the hypothesis that chronic, low-grade inflammation from appendicitis can precipitate multisystem involvement. While previous reports have not linked appendicitis to hepatic fibrosis, the findings in this case suggest that persistent inflammation and chronic venous congestion secondary to pericardial restriction contributed to progressive hepatic injury [13]. The presence of an appendicolith, which has been reported as a potential risk factor for complicated appendicitis, likely played a role in sustaining localized inflammation. In contrast to prior cases in which pericardial effusion occurred in the setting of acute perforated appendicitis, our case supports the hypothesis that prolonged subclinical inflammation can result in effusive-constrictive pericarditis, even in the absence of frank peritoneal contamination [14]. The presence of elevated inflammatory markers over an extended period, including persistently high C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), along with elevated liver enzymes (AST and ALT), aligns with a prolonged inflammatory process rather than an isolated acute event. Elastographic findings of significant hepatic fibrosis (METAVIR F3) reinforce the notion that systemic inflammation was sustained over weeks to months.

The prolonged disease course in this patient raises important clinical considerations. The initial deferment of appendectomy was justified given the life-threatening nature of cardiac tamponade; however, persistent systemic inflammation following pericardial drainage and pericardiectomy warranted a re-evaluation of the appendiceal pathology as a potential inflammatory source. In cases in which an appendicolith is present and systemic inflammation remains unexplained despite appropriate treatment of other organ-specific pathologies, early appendectomy should be considered to mitigate ongoing inflammatory responses and prevent further complications. This case, along with previous reports, suggests that if an appendicolith is present and systemic inflammation persists without a clear alternative etiology, early appendectomy should be considered to prevent potential systemic inflammatory sequelae [15].

Conclusions

This case highlights an unusual presentation of chronic appendicitis with an appendicolith leading to systemic inflammation, liver fibrosis, and effusive-constrictive pericarditis, ultimately requiring both pericardiectomy and appendectomy. The patient’s symptoms – exertional dyspnea, fever, and intermittent abdominal pain – represented an atypical and misleading clinical presentation, which initially led to a focus on cardiovascular involvement while delaying the recognition of appendicitis as the underlying source of systemic inflammation. The progression to effusive-constrictive pericarditis, hepatic congestion, and fibrosis demonstrates the far-reaching consequences of persistent low-grade inflammation.

The presented medical history underscores the critical need for clinicians to consider both thoracic and abdominal etiologies when evaluating patients with unexplained multisystem symptoms. Persistent systemic inflammation, particularly with elevated inflammatory markers and signs of cardiac compromise, should prompt an expanded differential diagnosis that includes chronic intra-abdominal sources such as appendicitis. Early identification and treatment of an underlying inflammatory focus, such as chronic appendicitis, can prevent severe complications, including pericardial fibrosis, the need for invasive cardiac surgery, and long-term organ damage.