{
    "abstract": "Prepubertal vaginal bleeding is a common presentation for pediatric adolescent gynecologists with a broad differential diagnosis that historically may not have included complex lymphatic anomalies. However, given recent consensus criteria and imaging capabilities, this may be a condition that pediatric adolescent gynecologists see more frequently in the future.\nWe present a case of a 5-year-old pre-pubertal girl whose only presenting symptoms of a rare complex lymphatic anomaly was copious vaginal bleeding. After three vaginoscopies, two hysteroscopies, two pelvic MRIs, and a percutaneous ultrasound guided core needle biopsy, this patient was eventually diagnosed with Kaposiform lymphangiomatosis at age 9 years-old, and she is now being treated medically with sirolimus, a mammalian target of rapamycin (mTOR) inhibitor, with improvement in her symptoms.\nComplex lymphatic anomalies should be considered after initial and secondary workups for pre-pubertal vaginal bleeding or copious vaginal discharge are negative. Furthermore, this case illustrates the value of pelvic MRI in the setting of unknown cause of vaginal bleeding when typical workup is negative.",
    "authors": [
        {
            "affiliation": "Department of Pediatric and Adolescent Gynecology, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), Bethesda, Maryland; Children's National Hospital, Pediatric and Adolescent Gynecology Program, Washington. Electronic address: Kirsten.das@nih.gov.",
            "firstname": "Kirsten",
            "initials": "K",
            "lastname": "Das"
        },
        {
            "affiliation": "Unit on Vascular Malformations, Division of Intramural Research, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), Bethesda, Maryland.",
            "firstname": "Sarah",
            "initials": "S",
            "lastname": "Sheppard"
        },
        {
            "affiliation": "Pediatric Interventional Radiology, Children's National Hospital, Washington.",
            "firstname": "Bhupender",
            "initials": "B",
            "lastname": "Yadav"
        },
        {
            "affiliation": "Genetics and Metabolism, Children's National Hospital, Rare Disease Institute, Bethesda, Maryland.",
            "firstname": "Joyce Tannenbaum",
            "initials": "JT",
            "lastname": "Turner"
        },
        {
            "affiliation": "Pediatric Neurooncology, Children's National Hospital, Washington.",
            "firstname": "Miriam",
            "initials": "M",
            "lastname": "Bornhorst"
        },
        {
            "affiliation": "Center for Cancer and Blood Disorders, Children's National Hospital, Washington.",
            "firstname": "Alan H",
            "initials": "AH",
            "lastname": "Siegel"
        },
        {
            "affiliation": "Department of Pediatric and Adolescent Gynecology, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), Bethesda, Maryland; Children's National Hospital, Pediatric and Adolescent Gynecology Program, Washington.",
            "firstname": "Jacqueline C",
            "initials": "JC",
            "lastname": "Yano"
        },
        {
            "affiliation": "Department of Pediatric and Adolescent Gynecology, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), Bethesda, Maryland; Children's National Hospital, Pediatric and Adolescent Gynecology Program, Washington.",
            "firstname": "Veronica",
            "initials": "V",
            "lastname": "Gomez Lobo"
        }
    ],
    "conclusions": null,
    "copyrights": "Published by Elsevier Inc.",
    "doi": "10.1016/j.jpag.2024.06.005\n10.1542/peds.2014-3673\n10.3390/ijms23042358\n10.1016/j.jvscit.2019.10.003",
    "journal": "Journal of pediatric and adolescent gynecology",
    "keywords": [
        "Complex lymphatic anomaly",
        "Kaposiform lymphangiomatosis",
        "Nras",
        "Prepubertal vaginal bleeding"
    ],
    "methods": null,
    "publication_date": "2024-06-28",
    "pubmed_id": "38936506\n25307772\n36683202\n30542204\n33844431\n35202617\n37194624\n32894644\n25598153\n34103076\n26055853\n35216474\n31617676\n37264205\n31676179\n35246606\n27990590\n32025595",
    "results": null,
    "title": "Kaposiform Lymphangiomatosis as a Cause of Vaginal Bleeding & Discharge: A Case Report.",
    "xml": "<Element 'PubmedArticle' at 0x788502cf9170>"
}