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. CASE: 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. SUMMARY AND CONCLUSION: 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.
Subject(s)
Cervix Uteri/pathology , Heart Defects, Congenital/complications , Hydrocolpos/complications , Polydactyly/complications , Puberty , Uterine Diseases/complications , Abnormalities, Multiple/surgery , Adolescent , Cervix Uteri/surgery , Female , Heart Defects, Congenital/surgery , Hematometra/etiology , Hematometra/surgery , Humans , Hydrocolpos/surgery , Polydactyly/surgery , Postoperative Complications , Pyometra/etiology , Pyometra/surgery , Tomography, X-Ray Computed , Uterine Diseases/surgeryABSTRACT
Pyosalpinx and hydrosalpinx are conditions mainly seen in adult women, but also among sexually active adolescents and can bring added hazard to fertility. However, these conditions are very rare in childhood, as well as in adolescent girls who are not sexually active. We are presenting two rare cases of young girls in early puberty with hydrosalpinx and pyosalpinx. Both girls had a history of abdominopelvic surgery in childhood for congenital bowel anomalies and fecal incontinence. Such cases are good reminders that girls with known abdominopelvic anomalies and surgical procedures in childhood need long term followup, in particular when entering puberty and maturation. The two cases show how fallopian tubes can be indirectly affected and present in adolescence with serious problems needing surgical procedures and potentially threatening future reproductive system performances.
El piosálpinx y el hidrosálpinx son condiciones vistas principalmente en mujeres adultas, pero también entre las adolescentes sexualmente activas, y pueden acarrear riesgos a la fertilidad. Sin embargo, estas condiciones son muy raras en la infancia, así como en las niñas adolescentes que no están sexualmente activas. Presentamos dos casos raros de jovencitas en la pubertad temprana con hidrosálpinx y piosálpinx. Ambas adolescentes tenían antecedentes de cirugía abdominopélvica en la infancia a causa de anomalías congénitas del intestino e incontinencia fecal. Tales casos son buenos recordatorios de que las muchachas con anomalías y procedimientos quirúrgicos abdominopélvicos en la niñez, necesitan seguimiento a largo plazo, en particular al entrar en la pubertad y la madurez. Los dos casos muestran cómo las trompas de Falopio pueden ser indirectamente afectadas, y presentarse en la adolescencia con graves problemas que necesitan procedimientos quirúrgicos y operaciones que constituyen una amenaza potencial al sistema reproductor.