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The association of a dysmorphic pelvis, absence of pubic rami, hip dysplasia, and genitourinary anomalies

✍ Scribed by Ravi K. Bashyal; Henrik Malchau; Katherine Nimkin; Lewis B. Holmes


Publisher
John Wiley and Sons
Year
2011
Tongue
English
Weight
535 KB
Volume
94
Category
Article
ISSN
1542-0752

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✦ Synopsis


Abstract

BACKGROUND

There have been reports of children who had absence of the pubic rami, hip dysplasia, and genitourinary anomalies.

CASE REPORT

We describe a 44‐year‐old woman with severe hip dysplasia, bilateral chronic congenital hip dislocations, abnormal development of the entire pelvis, and absence of the pubic rami in association with absence of the uterus.

CONCLUSION

We present an individual who has abnormal development of the entire pelvis, including absence of the pubic rami, and genitourinary anomalies. We suggest that this is a rare pattern of associated anomalies confined to a localized region of the body. Potential underlying developmental abnormalities include somatic mutations which affected the mesodermal cells from which the pelvis and mullerian structures develop. Birth Defects Research (Part A) 2012. © 2011 Wiley Periodicals, Inc.