Oligohydramnios sequence revisited in relationship to arthrogryposis, with distinctive skin changes. Issue 11 (26th August 2014)

• Record Type: Journal Article
• Authors: Hall, Judith G.
• Is Part Of: American journal of medical genetics. Volume 164:Issue 11(2014.)
• Page Start: 2775
• Page End: 2792
• Publication Date: 2014-08-26
• Subjects: Medical genetics -- Periodicals; 616.14205
• DOI: 10.1002/ajmg.a.36731
• Languages: English
• ISSNs: 1552-4825

Abstract

<abstract abstract-type="main"> <title> <x xml:space="preserve">Abstract</x> </title> <sec id="ajmga36731-sec-0001" sec-type="section"> <p>Thirty cases of arthrogryposis associated with longstanding oligohydramnios were identified among 2, 500 cases of arthrogryposis (1.2%) and were reviewed for clinical features and natural history. None had renal agenesis or renal disease. Twenty‐two had a history of known rupture of membranes. Only 50% had pulmonary hypoplasia at birth and only two died (7%). Sixty percent (18/30) seemed to have their multiple congenital contractures (MCC) primarily on the basis of compression related to the longstanding oligohydramnios and responded well to physical therapy. On average they did not have intrauterine growth restriction. "Potter" facies and remarkable skin changes were present in all. An excess of males was observed in spite of the lack of genitourinary anomalies. © 2014 Wiley Periodicals, Inc.</p> </sec> </abstract>