Surgical Treatment for Patients with Moyamoya Syndrome and Type 1 Neurofibromatosis

Jose L. Porras, Wuyang Yang, Tomas Garzon-Muvdi, Risheng Xu, Jaishri Blakeley, Allan Belzberg, Justin M. Caplan, Syed Khalid, Geoffrey P. Colby, Alexander Coon, Rafael J. Tamargo, Edward Ahn, Judy Huang

Research output: Contribution to journalArticlepeer-review

3 Scopus citations


Introduction The current study describes the impact of surgery in preventing follow-up ipsilateral transient ischemic attacks (TIAs)/strokes in an East Coast North American cohort of patients with both moyamoya syndrome (MMS) and neurofibromatosis type 1 (NF1) (MMS-NF1). Methods We retrospectively reviewed records of patients with MMS and NF1 at the Johns Hopkins Medical Institutions from 1990–2014. Baseline characteristics and follow-up results including subsequent ipsilateral strokes were collected and compared between a revascularization group (group 1) and a conservatively managed group (group 2) on a per-hemisphere basis. Results A total of 9 patients (14 hemispheres) were included in our study. The average age of all patients at NF1 diagnosis was 2.1 ± 7.7 years, with 6 being female (66.7%). The average age of all patients at MMS diagnosis was 10.4 ± 16.6 years with the median age being 7.7 years (range: 4.1–27.0 years). Race distribution was: White (n = 4, 44.4%), Black (n = 3, 33.3%), and Asian (n = 2, 22.2%). Four patients (44.5%) experienced cerebrovascular manifestations of MMS before MMS diagnosis. Group 1 was younger at MMS diagnosis (P = 0.009), likely with a more acute symptom onset (P = 0.077). Management strategies were: pial synangiosis (n = 3, 21.4%) and conservative (n = 11, 78.6%). During an average follow-up period of 6.28 ± 2.0 years, no ipsilateral TIAs/strokes were observed for group 1; conversely, 2 ipsilateral TIAs (18.2%) and 2 ipsilateral strokes (18.2%) occurred in group 2. Conclusions In our study of non-Asian patients with MMS-NF1, revascularization reduced stroke recurrence and deterioration of symptoms. However, more studies are warranted to further explore the role of revascularization procedures given the rarity of this disease combination.

Original languageEnglish (US)
Pages (from-to)19-25
Number of pages7
JournalWorld neurosurgery
StatePublished - Mar 1 2017


  • Moyamoya
  • Neurofibromatosis 1
  • Revascularization
  • Stroke

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology


Dive into the research topics of 'Surgical Treatment for Patients with Moyamoya Syndrome and Type 1 Neurofibromatosis'. Together they form a unique fingerprint.

Cite this