{
  "abstract": "Background Paediatric hemorrhagic moyamoya disease (hMMD) is rare, with unclear clinical features and long-term outcomes owing to limited data. We comprehensively evaluated the clinical characteristics, prognosis and optimal treatment strategies for pediatric hMMD.Methods We screened paediatric patients with hMMD hospitalised between November 2003 and December 2024. Clinical features, treatments and long-term outcomes were retrospectively analysed. Multivariate logistic regression was used to evaluate risk factors for rebleeding and poor prognosis.Results During the first decade, hemorrhagic events were rare. However, in the second decade, females experienced hemorrhage at age 13–15 years and males at 10–12 years. Over 1103 patient-years, 96 hemorrhagic events occurred. Before encephaloduroarteriosynangiosis (EDAS), seven rehemorrhage instances occurred among 94 patients over 93.2 patient-years of observation. A modified Rankin Scale score ≥3 on admission independently predicted rehemorrhage (p=0.040; OR 5.280; 95% CI 1.083 to 25.739), while early EDAS (within 3 months of the first hemorrhage) significantly reduced this risk (p=0.043; OR 0.107; 95% CI 0.012 to 0.933). EDAS patients had lower rehemorrhage rates compared with their natural history. Anterior choroidal artery dilation and early-onset and delayed-onset posterior cerebral artery involvement increased postoperative complication risk. Early EDAS reduced postoperative complications (p=0.028; OR 0.219; 95% CI 0.057 to 0.845).Conclusion Although rare, paediatric hMMD carries a high rebleeding rate. The second decade of life may represent the peak period for bleeding in paediatric patients with MMD, necessitating close monitoring. Early EDAS reduces rebleeding and postoperative complications in hMMD. Compared with the natural course, EDAS safely reduces paediatric rehemorrhage.",
  "authors": [
    {
      "affiliations": [
        "Medical School of Chinese PLA, Beijing, Beijing, China",
        "Department of Neurosurgery, the First Medical Center of Chinese PLA General Hospital, Beijing, China"
      ],
      "name": "Cheng Chen"
    },
    {
      "affiliations": [
        "Medical School of Chinese PLA, Beijing, Beijing, China",
        "Department of Neurosurgery, the First Medical Center of Chinese PLA General Hospital, Beijing, China"
      ],
      "name": "Gan Gao"
    },
    {
      "affiliations": [
        "Department of Neurosurgery, the First Medical Center of Chinese PLA General Hospital, Beijing, China"
      ],
      "name": "Desheng Li"
    },
    {
      "affiliations": [
        "Department of Neurosurgery, the First Medical Center of Chinese PLA General Hospital, Beijing, China"
      ],
      "name": "Hui Wang"
    },
    {
      "affiliations": [
        "Medical School of Chinese PLA, Beijing, Beijing, China",
        "Department of Neurosurgery, the First Medical Center of Chinese PLA General Hospital, Beijing, China"
      ],
      "name": "Minjie Wang"
    },
    {
      "affiliations": [
        "Medical School of Chinese PLA, Beijing, Beijing, China"
      ],
      "name": "Jingjie Li"
    },
    {
      "affiliations": [
        "Medical School of Chinese PLA, Beijing, Beijing, China",
        "Department of Neurosurgery, the First Medical Center of Chinese PLA General Hospital, Beijing, China"
      ],
      "name": "Simeng Liu"
    },
    {
      "affiliations": [
        "Medical School of Chinese PLA, Beijing, Beijing, China"
      ],
      "name": "Zhangwei Zeng"
    },
    {
      "affiliations": [
        "Medical School of Chinese PLA, Beijing, Beijing, China"
      ],
      "name": "Shuainan Ning"
    },
    {
      "affiliations": [
        "Department of Neurosurgery, the First Medical Center of Chinese PLA General Hospital, Beijing, China"
      ],
      "name": "Ronghui Gu"
    },
    {
      "affiliations": [
        "Department of Neurosurgery, the First Medical Center of Chinese PLA General Hospital, Beijing, China"
      ],
      "name": "Songtao Pei"
    },
    {
      "affiliations": [
        "Department of Neurosurgery, the First Medical Center of Chinese PLA General Hospital, Beijing, China"
      ],
      "name": "Cong Han"
    },
    {
      "affiliations": [
        "Department of Neurosurgery, the First Medical Center of Chinese PLA General Hospital, Beijing, China"
      ],
      "name": "Lian Duan"
    }
  ],
  "title": "Clinical features, treatment and long-term outcomes of hemorrhagic moyamoya disease in children: an extensive follow-up study",
  "uid": "b4345c6b-3bbf-5e63-b28c-76119abad5b6"
}