Journal article
McKusick Kaufman Syndrome, Complications Arising at Puberty
Journal of pediatric & adolescent gynecology, Vol.27(6), pp.e125-e126
12/2014
DOI: 10.1016/j.jpag.2013.10.002
PMID: 24656697
Abstract
McKusick Kaufman Syndrome (MKS), a rare genetic condition, presents in the neonatal period with a classic triad of postaxial polydactyly, congenital heart disease, and hydrometrocolpos. The diagnosis is typically clinical, based on the presence of polydactyly and hydrometrocolpos.
We report the case of a 13-year-old female, who was diagnosed with MKS in infancy and underwent vaginal reconstructive surgery for a urogenital sinus. She was lost to follow-up thereafter. She presented to our institution at age 13 with pyometra, pyosalpinx, and tubo-ovarian abscess due to a stenotic cervix obstructing menstrual outflow.
Gynecologic follow-up is imperative in patients with history of vaginal reconstruction to monitor for hematometra from outflow obstruction to prevent life threatening secondary bacterial infections.
Details
- Title: Subtitle
- McKusick Kaufman Syndrome, Complications Arising at Puberty
- Creators
- Erin T Lueth - University of Colorado, Denver, COKelly E Wood - University of Iowa Children's Hospital, Iowa City, IA
- Resource Type
- Journal article
- Publication Details
- Journal of pediatric & adolescent gynecology, Vol.27(6), pp.e125-e126
- Publisher
- Elsevier Inc
- DOI
- 10.1016/j.jpag.2013.10.002
- PMID
- 24656697
- ISSN
- 1083-3188
- eISSN
- 1873-4332
- Language
- English
- Date published
- 12/2014
- Academic Unit
- Stead Family Department of Pediatrics; Hospital Medicine
- Record Identifier
- 9984093322602771
Metrics
19 Record Views