Introduction
Constrictive pericarditis is an acquired disorder typically resulting from pericardial inflammation, which leads to fibrosis and thickening of the pericardium. Reference Nahass and Kassotis1 This restricts the heart’s diastolic function, ultimately causing heart failure. Reference Nahass and Kassotis1 Common causes include infections, trauma, postoperative complications from cardiac surgery, tumours, or systemic diseases. Reference Birtolo and Shahini2 Protein-losing enteropathy is a clinical syndrome characterised by excessive loss of plasma proteins through the gastrointestinal tract, leading to hypoalbuminemia, ascites, and oedema. Reference Ozen3 Protein-losing enteropathy is not an independent disease, but rather a manifestation of various underlying conditions. Reference Ozen3 In this report, we present a case of protein-losing enteropathy secondary to constrictive pericarditis in a 3-year-old boy.
Case presentation
The patient, a 3-year-old boy, initially presented one month prior with unexplained eyelid oedema, accompanied by mild oedema of the abdominal wall and lower extremities. On taking a history, the patient was found to have no history of chest trauma, etc. Liver function tests indicated hypoalbuminemia. In February 2023, he sought treatment, and he was hospitalised multiple times and received intravenous albumin infusions.
Due to recurrent symptoms, he was referred to Shanghai for further evaluation. After comprehensive examinations, he was diagnosed with “intestinal lymphangiectasia, hypoalbuminemia, abnormal liver function, hepatomegaly, hemorrhagic gastritis, duodenal ulcer, thyroid dysfunction, oedema, ascites, influenza, and left atrial enlargement.” The doctors recommended further investigation and treatment in Beijing. During his hospitalisation, he received multiple albumin infusions to correct hypoalbuminemia and was treated with oral hydrochlorothiazide and spironolactone for diuresis. After his condition stabilised, he was discharged. Following discharge, the patient continued taking ursodeoxycholic acid capsules, hydrochlorothiazide, and spironolactone, effectively controlling his symptoms.
In July 2023, the patient underwent further evaluation in Beijing. Protein-losing enteropathy imaging confirmed intestinal protein loss (see figure 1), and echocardiography revealed elevated pressures in both atria, with an unclear aetiology but suspected constrictive pericarditis (see figure 2). Doppler ultrasound of the central lymphatic vessels showed dilation of the thoracic duct in the left cervical segment. Enhanced abdominal and pelvic CT revealed pelvic oedema and multiple small lymph nodes in the bilateral inguinal region and pelvis. Enhanced abdominal CT showed abnormal enhancement of the liver parenchyma, presenting with a mottled or pseudolobular appearance, along with multiple nodules and cystic lesions in the peritoneal and retroperitoneal areas, suggesting lymphangiectasia and ascites. Chest CT indicated uneven lung transparency, likely related to impaired lymphatic drainage. Nodules were observed in the anterior segment of the right upper lobe and the lateral segment of the middle lobe, raising the possibility of lymphoma. Multiple small cystic lesions were also found in the anterior and middle mediastinum, suspected to be lymphangiomas.
Protein-losing enteropathy imaging.
Preoperative echocardiogram.
In September 2023, the child was diagnosed with constrictive pericarditis and was treated with torasemide and potassium citrate granules. Multiple follow-up tests showed albumin levels ranging from 27 to 35 g/L, during which intravenous human albumin and diuretic therapy were continued.
In December 2023, the child underwent pericardiectomy. Postoperative pathology revealed fibrous tissue proliferation, increased collagen fibres, indistinct pericardial layers, along with neovascularization and scattered inflammatory cell infiltration. No granulomatous inflammation was observed, and the diagnosis was chronic non-specific pericarditis.
Following the surgery, the child no longer experienced symptoms such as oedema or oliguria, and repeat tests showed normal albumin levels and liver function. The final diagnosis was protein-losing enteropathy secondary to lymphangiectasia caused by constrictive pericarditis. After a 10-month follow-up, the child has fully recovered.
Discussion
According to the literature, protein-losing enteropathy secondary to intestinal lymphangiectasia caused by constrictive pericarditis is rare. Reference Birtolo and Shahini2 However, the cardiac and gastrointestinal abnormalities resulting from constrictive pericarditis are typically reversed following complete pericardiectomy. Reference Birtolo and Shahini2 In this case, the patient’s albumin levels returned to normal after pericardiectomy.
In this particular case, the child had no common underlying causes of constrictive pericarditis, such as malignancy, tuberculosis, autoimmune diseases, or a history of surgery. Additionally, the patient was born at the onset of the COVID-19 pandemic, and the first symptoms appeared in January 2023. Therefore, the possibility that constrictive pericarditis was a rare complication of either COVID-19 infection or vaccination cannot be ruled out.
Previous reports have documented cases of constrictive pericarditis associated with COVID-19 infection Reference Beckerman, Alarfaj and Tracy4–Reference Kaya and Yalta6 or vaccination Reference Bain and Guglin7–Reference Aikawa, Ogino and Aikawa9 , with five cases occurring in males aged 16 to 55, and one case in a 70-year-old female. The onset of symptoms ranged from a few days to several years after COVID-19 infection or vaccination. However, no cases in children under five years of age have been reported.
In conclusion, this report presents a rare case of constrictive pericarditis in a 3-year-old boy, with hypoalbuminemia as the main symptom, possibly linked to COVID-19 infection or vaccination. Although such cases are extremely rare, clinicians should consider this possibility when encountering pericarditis of unknown origin and inquire about the patient’s history of COVID-19 infection and vaccination to ensure timely diagnosis.
Conclusion
In conclusion, this case report presents a rare instance of constrictive pericarditis in a 3-year-old boy, leading to protein-losing enteropathy and severe hypoalbuminemia. Despite the absence of common causes such as malignancy, tuberculosis, autoimmune diseases, or prior surgeries, the patient’s condition responded well to pericardiectomy, with complete resolution of symptoms and normalisation of albumin levels. The potential link to COVID-19 infection or vaccination raises the possibility that constrictive pericarditis may be a rare complication in paediatric patients, especially given the timing of symptom onset in this case. While constrictive pericarditis causing protein-losing enteropathy is uncommon, this case highlights the importance of considering it in patients with unexplained pericardial inflammation and hypoalbuminemia. Clinicians should also be vigilant about the patient’s history of COVID-19 infection or vaccination, as early diagnosis and surgical intervention can lead to a full recovery.
Acknowledgements
The authors thank the patient and her family for allowing them to study this case.
Financial support
Not applicable.
Competing interests
The authors declare no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Ethical standards
Ethical approval is not required for the case report in our institution, Jining First People’s Hospital.
