title: McKusick Kaufman syndrome, complications arising at puberty.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: University of Colorado, Denver, CO.
authors.forename: Erin T
authors.initials: ET
authors.lastname: Lueth
authors.affiliation: University of Iowa Children's Hospital, Iowa City, IA. Electronic address: Kelly-wood@uiowa.edu.
authors.forename: Kelly E
authors.initials: KE
authors.lastname: Wood
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery; D000293:Adolescent; D002584:Cervix Uteri / Q000473:pathology* / Q000601:surgery; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000601:surgery; D006409:Hematometra / Q000209:etiology / Q000601:surgery; D006801:Humans; D052202:Hydrocolpos / Q000150:complications* / Q000601:surgery; D017689:Polydactyly / Q000150:complications* / Q000601:surgery; D011183:Postoperative Complications; D011627:Puberty; D055112:Pyometra / Q000209:etiology / Q000601:surgery; D014057:Tomography, X-Ray Computed; D014591:Uterine Diseases / Q000150:complications* / Q000601:surgery
keywords: Hydrometrocolpos; McKusick Kaufman Syndrome; Pyometra; Pyosalpinx