Cerebral Hyperperfusion Syndrome After Revascularization Surgery in Patients with Moyamoya Disease: Systematic Review and Meta-Analysis

World Neurosurg. 2020 Mar:135:357-366.e4. doi: 10.1016/j.wneu.2019.11.065. Epub 2019 Nov 20.


Background: Cerebral hyperperfusion syndrome (CHS) after bypass surgery is known as a complication of moyamoya disease (MMD). However, the incidence of CHS has not been accurately reported, and there is no consensus on related risk factors.

Objective: To evaluate the incidence and characteristics of CHS in patients with MMD after revascularization surgery via meta-analysis.

Methods: Relevant cohort studies were retrieved through a literature search of PubMed, Embase, and Ovid until December 1, 2018. Eligible studies were identified per search criteria. A systematic review and meta-analysis were used to assess the CHS total incidence, incidence in pediatric patients with MMD and adult patients with MMD, incidence for direct and combined bypass surgery, progress rate, and proportion of each symptom (including transient neurologic deficits [TNDs], hemorrhage, and seizure).

Results: A total of 27 cohort studies with 2225 patients were included in this meta-analysis. The weighted proportions per random-effects model were 16.5% (range, 11.3%-22.3%) for CHS total incidence, 3.8% (range, 0.3%-9.6%) for pediatric patients with MMD, 19.9% (range, 11.7%-29.4%) for adult patients with MMD, 15.4% (range, 5.4%-28.8%) for direct bypass surgery, and 15.2% (range, 8.4%-23.2%) for combined bypass surgery. Progress rate was 39.5% (range, 28.7%-50.8%). The most common CHS-related symptom was TNDs (70.2%; range, 56.3%-82.7%), followed by hemorrhage (15.0%; range, 5.5%-26.9%) and seizure (5.3%; range, 0.6%-12.9%).

Conclusions: CHS is a common complication after revascularization surgery in MMD. It is more frequently seen in adult patients. The most common CHS-related symptom was TNDs, followed by hemorrhage and seizure.

Keywords: Bypass surgery; Cerebral hyperperfusion syndrome; Incidence; Moyamoya disease.

Publication types

  • Meta-Analysis
  • Systematic Review

MeSH terms

  • Age Distribution
  • Cerebral Revascularization*
  • Cerebrovascular Circulation*
  • Cerebrovascular Disorders / diagnostic imaging
  • Cerebrovascular Disorders / epidemiology
  • Cerebrovascular Disorders / physiopathology
  • Humans
  • Intracranial Hemorrhages / epidemiology
  • Magnetic Resonance Angiography
  • Magnetic Resonance Imaging
  • Moyamoya Disease / surgery*
  • Perfusion Imaging
  • Postoperative Complications / diagnostic imaging
  • Postoperative Complications / epidemiology*
  • Postoperative Complications / physiopathology
  • Postoperative Hemorrhage / epidemiology
  • Seizures / epidemiology
  • Syndrome
  • Tomography, Emission-Computed, Single-Photon