Thomas Kevin R, Thomas Sandra P, Hewan-Lowe Karlene O, Pestaner Joseph P
Department of Pathology, Brody School of Medicine at East Carolina University and Pitt County Memorial Hospital, Greenville, NC 27858, USA.
Cardiovasc Pathol. 2007 Sep-Oct;16(5):313-6. doi: 10.1016/j.carpath.2006.12.004. Epub 2007 Feb 21.
Fibromuscular dysplasia (FMD) is an idiopathic disease of small- and medium-sized arteries, involving one or more vascular beds. Patients may present with a range of symptoms, which may not readily lead to a diagnosis of FMD. While maternal cocaine abuse during pregnancy has previously been associated with vascular alterations in the fetus, an association specifically with FMD has not previously been described.
METHODS/RESULTS: In this case report, a 21-month-old male presented with a 3-week history of daily vomiting, with temporary improvement of symptoms, then relapse followed by loss of consciousness. His medical history was significant only for maternal cocaine use. Clinical evaluation revealed dilated cardiomyopathy, and a presumptive diagnosis of myocarditis was rendered. Respiratory arrest and death occurred 2 days after admission. Postmortem examination demonstrated intimal-type multivessel FMD, which was determined to be the cause of the clinical presentation.
Without a postmortem examination, it is unlikely that a diagnosis of intimal fibroplasia, a rare variant of FMD (5% of cases), would have been made. This case thus illustrates the continuing utility of the classic postmortem examination. More intriguingly, the case suggests a possible relationship between in utero cocaine exposure and the development of fibromuscular dysplasia in the child.
纤维肌性发育不良（FMD）是一种中小动脉的特发性疾病，累及一个或多个血管床。患者可能出现一系列症状，这些症状可能不易导致FMD的诊断。虽然先前已将孕期母亲滥用可卡因与胎儿血管改变相关联，但此前尚未描述过与FMD的具体关联。
方法/结果：在本病例报告中，一名21个月大的男性有3周每日呕吐病史，症状有短暂改善，之后复发并伴有意识丧失。他的病史仅以母亲使用可卡因显著。临床评估显示扩张型心肌病，并初步诊断为心肌炎。入院2天后发生呼吸骤停和死亡。尸检显示内膜型多血管FMD，这被确定为临床表现的原因。
如果没有尸检，不太可能做出内膜纤维增生症（FMD的一种罕见变体，占病例的5%）的诊断。因此，本病例说明了经典尸检的持续效用。更有趣的是，该病例提示子宫内可卡因暴露与儿童纤维肌性发育不良的发生之间可能存在关联。
