# Rosai-Dorfman Disease as Constrictive Pericarditis: An Unusual Childhood Presentation

## TL;DR

A six-year-old child presented with heart-related symptoms as the first sign of a rare disease called Rosai-Dorfman disease.

## Contribution

This case report highlights an unusual initial presentation of Rosai-Dorfman disease as constrictive pericarditis in a child.

## Key findings

- RDD can present as constrictive effusive pericarditis with congestive heart failure in children.
- Treatment with steroids and pericardiectomy resolved the symptoms in this case.
- RDD should be considered in the differential diagnosis of constrictive pericarditis in children.

## Abstract

Rosai-Dorfman disease (RDD) is a rare, multisystemic, histiocytic disorder that usually presents with painless cervical lymphadenopathy. We describe a case of constrictive effusive pericarditis with congestive cardiac failure in a six-year-old child as an initial presentation of RDD. The child underwent pericardiectomy and was treated with steroids, following which the symptoms resolved entirely. While tuberculosis accounts for the majority of cases presenting with constrictive pericarditis in developing countries, the presentation of RDD can be easily missed if not kept in mind as a possibility. This case report documents the unique presentation of a rare disease.