McKusick-Kaufman syndrome as a cause of acute abdomen in the neonatal period
Franke, B.; Missbach, D.; Römer, K.H.
Zentralblatt für Chirurgie 113(4): 254-258
1988
ISSN/ISBN: 0044-409X PMID: 3364056 Document Number: 312762
The McKusick-Kaufman-syndrome, also known by the name of hydrometrocolpos-hexadactyly syndrome, is an autosomal-recessive hereditary disease or spontaneous malformation. A hydrometrocolpos is formed in the process by a transversal vaginal septum and subsequently acts like an abdominal tumour to compress the iliac veins and to result in supravesical obstruction, eventually causing a condition of ileus and respiratory problems. Transvaginal drainage is used as primary therapy.