Congenital absence of the pericardium associated with congenital diaphragmatic hernia and hepatic hemangioendothelioma: case report and review of the literature

Abstract

An unusual case of a neonate with absent pericardium, left-sided diaphragmatic hernia, and hepatic hemangioendothelioma is described. The posterolateral diaphragmatic hernia was successfully repaired, and agenesis of the pericardium did not interfere with cardiac function postoperatively. The hepatic tumour was a coincidental radiological finding, while liver function and the biochemical profile were normal. We also present a review of the literature on congenital absence of the pericardium and conclude that all cases should be screened for cardiac and hepatic anomalies.

Congenital complete absence of pericardium presenting with chest pain: MRI characteristics

“…Likewise, congenital absence of pericardium is frequently left sided. They have been associated with intracardiac anomalies like atrial septal defect, ventricular septal defect, tetralogy of Fallot [1] and extracardiac anomalies like diaphragmatic hernia [2], lung anomalies like left lung hypoplasia and bronchogenic cyst [5]. The presented case herein exemplifies an isolated complete absence of the entire pericardium.…”

“…The presented case herein exemplifies an isolated complete absence of the entire pericardium. Analysis of published data on complete absence of the entire pericardium reveals that though the defect has been present from birth, the diagnosis has been made across different age groups, from as early as the neonatal age group to as late as the eighth decade of life [2,6]. Almost always, neonatal and infantile patients are being diagnosed at the time of surgery for other congenital anomalies usually a congenital diaphragmatic hernia [2].…”

“…But it can also be symptomatic and infrequently associated with life threatening complications like strangulation of heart chambers and sudden cardiac death making it a clinically relevant entity. Congenital absence of the pericardium is usually an autopsy diagnosis or an incidental finding at the time of cardiothoracic surgeries [1,2]. Its incidence in autopsy studies was reported as 1 in 14000 [3].…”

Congenital total absence of the pericardium: A case report

“…Brought to you by | MIT Libraries Authenticated Download Date | 5/12/18 12:39 PM such as bronchogenic cyst, pulmonary sequestration, hypoplasia of the left lung and left pulmonary artery, dextroisomerism, diaphragmatic hernia, hepatic hemangioendothelioma, ruptured type A aortic dissection, or tricuspid regurgitation [4][5][6][7][8]. There was no coexisting heart anomaly in our case.…”

“…Primary closure, pericardiectomy, left atrial appendectomy, division of adhesions, extension of the defect, or pericardioplasty are the surgical procedures employed for partial defects or symptomatic patients [1,6]. For our patient, we recommended a β-blocker to treat palpitations, with close follow-up to ensure symptom resolution.…”