Manoly Imthiaz, Viola Nicola, Fowler Darren, Roman Kevin, Haw Marcus
Wessex Cardiac Centre, Southampton University Hospitals NHS Trust, Southampton, UK.
World J Pediatr Congenit Heart Surg. 2011 Apr;2(2):321-3. doi: 10.1177/2150135110390718.
Primary cardiac tumors are very rare, with a reported incidence of 0.15% to 0.2% in autopsy series. They can be life threatening because of myocardial compression and ventricular dysfunction. Once diagnosed during pregnancy, the clinical condition of the baby is monitored because of the risk of rupture of the tumor capsule. The authors report a rare case of a neonate who presented with respiratory and cardiac compromise due to cardiac tamponade necessitating emergency exploration of the pericardium and excision of tumor. A well-encapsulated tumor measuring around 5 cm and bigger than the heart was completely excised. This was diagnosed to be an immature teratoma. Follow-up echocardiogram was normal, and on serial monitoring, alpha-fetoprotein was within normal limits. The baby was discharged home with no complications. Intrapericardial teratoma in neonates is a surgical emergency if presented with significant pericardial effusion. It can be a challenge if diagnosed in utero with rupture before the viability of pregnancy. A multidisciplinary team approach is necessary to manage such situations. Complete excision is necessary because of its association with tissues of malignant potential.
原发性心脏肿瘤非常罕见,尸检系列报道的发病率为0.15%至0.2%。由于心肌受压和心室功能障碍,它们可能危及生命。一旦在孕期确诊,由于肿瘤包膜破裂的风险,需对胎儿的临床状况进行监测。作者报告了一例罕见的新生儿病例,该新生儿因心脏压塞出现呼吸和心脏功能障碍,需要紧急探查心包并切除肿瘤。一个包膜完整、大小约5厘米且比心脏还大的肿瘤被完全切除。诊断为未成熟畸胎瘤。随访超声心动图正常,连续监测时甲胎蛋白在正常范围内。婴儿无并发症出院。如果新生儿心包内畸胎瘤出现大量心包积液,是外科急症。如果在子宫内诊断且在孕期可存活之前发生破裂,则可能是一项挑战。处理这种情况需要多学科团队协作。由于其与具有恶性潜能的组织有关,完全切除是必要的。