Fibromuscular dysplasia in association with intrauterine cocaine exposure.
BACKGROUND
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.


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.


CONCLUSIONS
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.
Thomas
Kevin R
KR
Department of Pathology, Brody School of Medicine at East Carolina University and Pitt County Memorial Hospital, Greenville, NC 27858, USA. docin2004@earthlink.net
Thomas
Sandra P
SP
Hewan-Lowe
Karlene O
KO
Pestaner
Joseph P
JP
D001344:Autopsy; D019970:Cocaine-Related Disorders / Q000150:complications* / Q000473:pathology; D003331:Coronary Vessels / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D005352:Fibromuscular Dysplasia / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D005355:Fibrosis; D006801:Humans; D007223:Infant; D008297:Male; D011247:Pregnancy; D011297:Prenatal Exposure Delayed Effects; D012077:Renal Artery / Q000473:pathology; D014711:Vertebral Artery / Q000473:pathology