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Is Moyamoya Disease Congenital?

Moyamoya is a frequently found, but rare type of disease in children. The problem causes narrowing of the blood vessels and internal carotid arteries (arteries responsible for the supply of blood towards the brain) present in the brain. Due to progressive narrowing of arteries and blood vessels, formation of a new network consisting of small blood vessels takes place across the base of your brain to compensate for the reduction in blood flow. The newly formed blood vessels or arteries resemble any puff of smoke over the cerebral angiogram and hence, give the name Moyamoya i.e., something hazy similar to a smoke puff drifting in air.

Is Moyamoya Disease Congenital?

Analysis Of Patients With Moyamoya Disease And Congenital Heart Problem

With the objective to find out whether Moyamoya disease is a congenital one, doctors have conducted detailed analysis of 5 patients suffering from Moyamoya disease and structural congenital heart problems. After analysis, doctors observed-

Types Of Congenital Heart Problems

  • Coarctation of aorta in 3 different patients
  • Association with the defect of ventricular septal in 1 patient
  • Mitral valve and aortic valve stenoses in 1 patient
  • Fallot tetralogy in 1 patient
  • Combination of large paramenbranous ventricular septal type of defect and Fallot tetralogy in 2 other patients.

Note. 4 patients have undergone surgical treatment to cure their congenital heart problems in only 1 year of their lives and 1 patient had balloon dilation procedure to cure aortic coarctation at only 5 years age.

Diagnose Of Moyamoya Syndrome In The Mentioned Patients

Doctors diagnose Moyamoya syndrome in each of the aforementioned patients after their surgical intervention associated with congenital heart problem. Accordingly, diagnosis conducted-

  • At only 6 months in 1 patient
  • At 2 years in 3 different patients
  • At 6 years age in 1 patient

Results of the diagnosis show that 3 patients have already experienced strokes i.e. common symptom associated with Moyamoya disease and remaining 2 suffered from seizures. Other than this, most of the patients have to undergo with cerebral revascularization surgery to halt the Moyamoya disease progression.

Moyamoya takes place because of many reasons and the treatment depends on severity of the suffered disease. However, curing of the disease usually requires surgery, as it restores the flow of blood towards your brain. This successfully treats the problem of Moyamoya in many people, especially in large number of children even though clinical results depend primarily on whether a patient has experienced stroke or not.

Symptoms Of Moyamoya Disease

Unfortunately, Moyamoya disease does not cause any symptom until a patient suffers from mini-stroke or transient ischemic attack. Later on, the problem results in dizziness, headaches, weakness, seizures and paralysis in a particular part of one’s body. In most of the cases, symptoms associated with Moyamoya disease takes place because of progressive blockage of the major blood vessels of intracranial area and result in loss of permanent or transient neurological functions. This is a type of angiogram taking place in anterior-posterior direction i.e. front to back to show carotid artery obliteration, which intends to grow new arteries for supplying blood towards the brain.

Causes Of Moyamoya Disease

Until now, doctors have failed to identify the exact reason behind Moyamoya disease. However, about 10 percent of the patients suffer from the problem because of genetic reasons. In addition, the problem is common among Asian people. Even in some cases, Moyamoya disease takes place in combination with various other health issues.


To conclude, we should say that Moyamoya disease is of congenital type; however, the extent and underlying symptoms may vary among patients and their age groups.


Also Read:

Pramod Kerkar, M.D., FFARCSI, DA
Pramod Kerkar, M.D., FFARCSI, DA
Written, Edited or Reviewed By: Pramod Kerkar, M.D., FFARCSI, DA Pain Assist Inc. This article does not provide medical advice. See disclaimer
Last Modified On:April 3, 2019

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