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Case Report
A case of McKusick-Kaufman syndrome
Se-Hyung Son, Yoon Joo Kim, Eun Sun Kim, Ee-Kyung Kim, Han-Suk Kim, Beyong Il Kim, Jung-Hwan Choi
Clin Exp Pediatr. 2011;54(5):219-223.   Published online May 31, 2011

McKusick-Kaufman syndrome (MKS) is an autosomal recessive multiple malformation syndrome characterized by hydrometrocolpos (HMC) and postaxial polydactyly (PAP). We report a case of a female child with MKS who was transferred to the neonatal intensive care unit of Seoul National University Children's Hospital on her 15th day of life for further evaluation and management of an abdominal cystic mass. She...

A Case of Neonatal Hydrometrocolpos Accompanying Urinary Tract Obstruction
Kyoung Eun Lee, Ok Yeon Cho, Eun Jung Sim, Do Jun Cho, Dug Ha Kim, Ki Sik Min, Ki Yang Yoo, Guan Sup Lee
Clin Exp Pediatr. 2004;47(7):789-793.   Published online July 15, 2004
Neonatal hydrometrocolpos is the cystic dilatation of the vagina and uterus, which is caused by the combination of vaginal obstruction such as imperforate hymen, transverse vaginal septum, or vaginal atresia, and glandular secretion by maternal estrogenic stimulation. Although hydrometrocolpos is a rare congenital abnormality unlike pubertal hematocolpos, it is one of the relatively common abdominal masses in neonates. In typical...
Two Cases of Hydrometrocolpos in Neonate
Min Young Park, Bo Ra Son, So Young Kim, Young You Kim, Hyun Hee Kim, Won-Bae Lee, In Kyung Sung, Chung Sik Chun, Su Aa Lim
Clin Exp Pediatr. 2002;45(2):278-283.   Published online February 15, 2002
Imperforated hymen, vaginal atresia or high transverse vaginal septum are caused by incomplete vaginal canalization. The infant may be present with distention of the vagina and the uterus with glandular secretions stimulated by maternal estrogens, known as hydrometrocolpos. We report two cases of hydrometrocolpos. In the first case, distal vaginal atresia with cystovaginal fistula was revealed by a contrast fluoroscope...
A Case of Neonatal Hydrometrocolpos Causing Urinary Obstruction
Chang Soo Oh, Myung Lye Kim, Byung Tae Kim, Sang Jhoo Lee, Soo Hyoung Seo
Clin Exp Pediatr. 1992;35(11):1597-1601.   Published online November 15, 1992
Neonatal hydrometrocolopos is a rare congenital anomaly, which is thought to be caused by both vaginal obstruction such as imperforate hymen, transverse vaginal septum or vaginal atresia and sufficient maternal estrogenic stimulation to provoke secretion from the glands in the neonatal reproductive tract. The presence of lower abdominal mass in a female infant should always arouse the suspicion of hydrometrocolpos...
A Case of McKusick-Kaufman Syndrome
Han Joo Kong, Young Mi Jee, In Kyung Sung, Byung Churl Lee
Clin Exp Pediatr. 1992;35(6):829-833.   Published online June 15, 1992
We experienced a case of Mckusick-Kaufman syndrome in a 14 day-old female neonate. She had hydrometrocolpos with vaginal atresia and polydactyly of feet associated with bilateral hydronephrosis and umbilical hernia. Hydronetrocolpos with vaginal atresia was corrected by abdominoperineal-vaginal pull through operation on 21st day of life. A brief review of related literatures was made.
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