Tadele Henok, Chanie Abeje
Department of Pediatrics and Child Health, Hawasa University, Ethiopia.
Department of Surgery, Hawasa University, Ethiopia.
Ethiop J Health Sci. 2017 Mar;27(2):203-205. doi: 10.4314/ejhs.v27i2.14.
Ectopia Cordis is defined as complete or partial displacement of the heart outside the thoracic cavity. It is a rare congenital defect with failure of fusion of the sternum with extra thoracic location of the heart. The estimated prevalence of this case is 5.5 to 7.9 per million live births.
We had a case of a 16-hour-old male neonate weighing 2.9kg with externally visible, beating heart over the chest wall. Initial treatment included covering the heart with sterile-saline soaked dressing, starting systemic antibiotics and supportive care. A staged surgical approach to this defect with the initial aim of replacement of the heart to the thoracic cavity was opted. The neonate died twenty minutes after the surgical intervention due to cardiogenic shock despite adequate resuscitative measures.
This case report underscores the missed opportunity of antenatal ultra-sonographic diagnosis and the challenge of Ectopia Cordis treatment in Ethiopia.
心外畸形是指心脏完全或部分移位至胸腔外。它是一种罕见的先天性缺陷，表现为胸骨融合失败且心脏位于胸外。这种病例的估计患病率为每百万活产儿5.5至7.9例。
我们有一例16小时大、体重2.9千克的男婴，其心脏在胸壁外可见且跳动。初始治疗包括用无菌生理盐水浸湿的敷料覆盖心脏、开始使用全身性抗生素并给予支持治疗。针对此缺陷选择了分期手术方法，最初目标是将心脏放回胸腔。尽管采取了充分的复苏措施，但该新生儿在手术干预后20分钟因心源性休克死亡。
本病例报告强调了埃塞俄比亚产前超声诊断错失的机会以及心外畸形治疗面临的挑战。
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