1. SURGICAL MANAGEMENT
OF MOYA MOYA DISEASE
----AFTER 15 CASES
DR AMIT KUMAR GHOSH
Consultant Neurosurgeon
Institute Of Neurosciences,
Kolkata

2. TOTAL NO OF MOYA MOYA DISEASE TREATED—25
OPERATED (revascularizations)—15
CHILDREN—10
ADULT—5
EDAMS—10 ( All children)
STA-MCA BYPASS and EDAMS—5 (All adult)

3. Presentations ---
Infarcts--- 12
Hemorrhage--- 10
Progressive cognitive decline---- one
Both infarct and hemorrhage--- Two

4. PRE-OPERATIVE EVALUATION—
CT / MRI SCAN– To diagnose hemorrhage and infarcts
6 VESSELS DSA --- To diagnose Moya Moya disease and
also To assess STA and MCA
MR Perfusion scan–
To assess cerebral blood flow pre operative,
To assess improvement post-operatively
To decide need for surgery on asymptomatic hemisphere

5. SURGERY–
Indication– Individualized
depending on general and
neurological status
EDAMS only or EDAMS and
STA-MCA bypass

6. ENCEPHALO-DURO-
ARTERIO-MYO-
SYNANGIOSIS

8. STA-MCA BYPASS

9. MCA—
M4
STA

10. MCA
Temporary
clipping

11. ANAESTHETIC CONSIDERATIONS -----
1. Normotension
2. Normocarbia or slightly raised EtCO2(40-45 mmHg)
3. Normothermia to mild hypothermia
4. No anesthesia technique has been shown to be superior
to other

12. RESULTS AND FOLLOW UP– FOR EDAMS
only UPTO 1 YEAR
Mortality—Nil
One wound infection because of perforation of skin
during STA dissection—However cured with
conservative treatment
One chronic headache due to mal-positioning of bone
flap.
No other morbidity. All Childs are going to school and

13. RESULTS AND FOLLOW UP OF STA-MCA bypass
along with EDAMS—
Mortality –one, On 1st
post operative day patient developed
multiple infarcts in bilateral hemisphere and patient died on 7th
post-operative day
( patient of Quasi-Moya Moya ds due to bilateral ICA occlusion
with progressive cognitive decline)
One patient--- Did well clinically, but bypass did not work in post-
op DSA.
Other patients– Did well clinically and bypass also worked in
post-operative DSA.

14. POST-OPERATIVE ANGIOGRAPHY AND MR
perfusion scan

15. Moyamoya disease is an extremely rare
disorder in most parts of the world except in
Japan, where the overall incidence is higher
(0.35 per 100,000).
The pathogenesis of Moyamoya disease is
unknown
Moyamoya disease is characterized by
progressive intracranial vascular stenoses of
the circle of Willis
LITERATURE REVIEW

16. It is a disease that tends to affect children and adults in the
third to fourth decades of life.
In children it tends to cause strokes or seizures.
In adults it tends to cause bleeding or strokes.
The clinical features are cerebral ischaemia (strokes),
recurrent transient ischaemic attacks,haemorrhage,
convulsions and/or migraine-like headaches.

17. The diagnosis is initially suggested by CT, MRI, or
angiogram.
The name derives from its angiographic image--
the "puff of smoke," refers to the appearance of
multiple collateral vessels seen on angiography.

18. • There is no Medical treatment for Moya
Moya disease.
• The role of Aspirin: thought by some to
reduce the incidence of microemboli from
the micro-collaterals
• Surgery is the Mainstay of treatment to
prevent furthur Ischemic and
Hemorrhagic events

19. Several surgical approaches have been suggested in treating
moyamoya disease
These approaches are generally divided into two groups:
Direct Revascularisation- STA-MCA anastomosis
Indirect Revascularisation---
EDAMS (encephalo-duro-arterio-myo-synangiosis)
EMS (encephalomyosynangiosis),
EDAS (encephaloduroarteriosynangiosis),
multiple burr holes procedure
Superior cervical ganglionectomy
Cervical pericarotid sympathectomy

20. With the direct STA-MCA surgery, increased blood supply
is immediate
After the in-direct EDAMS, EDAS, EMS, and multiple burr
holes surgeries, symptoms may seem to improve almost
immediately and it will take probably 6-12 months before
new vessels (blood supply) can develop sufficiently.
Post-operative angiography is best to be done between 6
months and 1 year
The development of EDAMS was an attempt to increase
collateral circulation from both the middle meningeal artery
and the superficial temporal artery

21. A definite conclusion about whether to choose
EDAMS or another procedure to treat
moyamoya disease is unclear.
Clinical results of EDAMS show a greater reduction of
the ischemic attacks in moyamoya disease, and
angiograms taken after surgery show extensive
collateral vessels on the ischemic brain. Therefore,
EDAMS seems to be one of the more effective
treatments for moyamoya disease.

22. References
1) Kuriyama S, Kusaka Y, Fujimura M, et al (2008). "Prevalence
and clinicoepidemiological features of moyamoya disease in
Japan: findings from a nationwide epidemiological survey".
Stroke 39 (1): 42–7.
2) Wakai K, Tamakoshi A, Ikezaki K, et al (1997).
"Epidemiological features of moyamoya disease in Japan:
findings from a nationwide survey". Clin Neurol Neurosurg 99
Suppl 2: S1–5.
3) Surgical Treatment of Moyamoya Disease: Operative Technique
for Encephalo-duro-arterio-myo-synangiosis, Its Follow-up,
Clinical Results, and Angiograms; Kinugasa, Kazushi M.D.;
Mandai, Shinya M.D.; Kamata, Ichiro M.D.; Sugiu, Kenji
M.D.; Ohmoto, Takashi M.D. Volume 32(4), April 1993, p
527–531

23. THANK YOU