BRAIN BYPASS SURGERY FOR MOYA MOYA DISEASE

1. BRAIN BYPASS SURGERY
FOR MOYA MOYA DISEASE
DR AMIT KUMAR GHOSH
Consultant Neurosurgeon
Institute of Neurosciences,
Kolkata, India

2. Purpose of this presentation and
study
1) EDAMS is very simple and safe procedure as compared to STA-MCA
bypass
2) It does not require very high end microscope or high end expertise,
almost all the neurosurgeon can do it.
3) Very applicable for resource constraint places
4) Clinically proven efficacious to prevent further stroke in Moya Moya
disease
5) In our study , adults also shown good results

3. Presentation (n-75)
Children
(<18 years)
Adult Total
Infarct 33 22 55
Haemorrhage 2 12 14
Hge & infarct 5 0 5
Cognitive dec. 0 1 1
Children--- 40 (Male--- 12, Female– 28)
Adult--- 35

4. 6-Vessel
DSA
To diagnose
Moya Moya disease
And to assess
STA and MCA
MR perfusion scan
To assess cerebral blood flow pre operatively
with Acetazolamide,
To assess improvement post-operatively
To decide need for surgery on asymptomatic hemisphere
CT/MRI
To diagnose
Hemorrhage or
infarct
PRE-OPERATIVE EVALUATION

5. PRE-OPERATIVE EVALUATION--
MRI--
DSA--
MR
perfusion--

6. ENCEPHALO-DURO-
ARTERIO-MYO-SYNANGIOSIS
( EDAMS )
In which the STA and the muscle flap are
approximated to the surface of the brain
and sutured to the dural edge was
proposed and developed in 1984 by
Kinugasa et al.

8. 1. Normotension
2. Normocarbia or slightly raised EtCO2(40-
45 mmHg)
3. Normothermia to mild hypothermia
ANAESTHETIC CONSIDERATIONS

9. CLINICAL---
PLAIN CT SCAN OF BRAIN---
MRI – occasional
MR perfusion --- 6 weeks, 3 months, 6
months,1 year.
DSA/CT angio--- after 6 months.
POST OPERATIVE EVALUATION---

10. Outcome of revascularization was measured by----
1) Regress or halt the intensification of basal
moyamoya vessels,
2) Increase of the trans-dural collaterals and
the lepto-meningeal collaterals
leading to
increased perfusion and favorable
hemodynamic compensation clinically
resulting in preventing further episodes of
stroke in patients with MMD.

11. Criteria criteria No of patients
Moya moya
vessels
regression 57
same 18
Intensification 0
Transdural
vessels
regression 0
same --
intensification 75
Perfusion
(Matsushima
grade)
<1/3rd 0
1/3rd---2/3rd 52
>2/3rd 23
Angiographic outcome

12. le
Clinical outcome—
Minimum Follow up------1 year
Recurrent haemorrhage— 0
Recurrent infarct----- 0
Improvement of hemiparesis and speech ---- 55
No improvement of deficit (vision and
hemiparesis)---- 18
Mortality--- 2

14. Results were clinically categorized in 4 groups---
Excellent---- who had completely normal and started
their school/job. ( Modified Rankin scale---- 0 and 1)
Good—who had mild deficit, Gone back to work
( Modified Rankin scale---- 2)
Average--- who had residual disability. Restricted work
pattern. ( Modified Rankin scale---- 3).
Poor—Can not work, had significant neurodeficit.
( Modified Rankin scale---- 4 and 5)

15. Functional outcome No of patients
Excellent 48( children-28,adult-20)
Good 16 (10-children, 6-adult)
Average 6 (1-no improvement, 2—
complications, 3-partial
improvement)
poor 3 (no improvement)-
adults—2, children—1)
Mortality 2 (adults)
Functional outcome at 6 months – children—40, adults--35

16. le
Complications No of patients
Hemiparesis 4 (3 –partially improved)
Speech deficit (motor or sensory) 2 (improved)
Wound infection 2 (one required bone flap removal)
Subdural haemorrhage 2( non-surgical)
Mortality 2 ( due to multiple bi-hemispheric
infarct)
Complications--- 12 Patients

17. EDAMS was very effective, safe, simple
procedure with excellent results.
83.33% patient returned to their job (school for
the children) after 6 months of surgery.
OBSERVATION—

18. Comparison between adults and pediatrics
In a study by Houkin et al. in patients of MMD presenting with intra-cerebral
haemorrhage, 100% paediatric patients undergoing EDAMS had a good
outcome as compared to 68% with direct bypass;
whereas in adults, 100% patients treated with direct bypass had a good
outcome as compared to 38% patients treated with EDAMS.
Dusick et al. showed good response to EDAS in 100%
paediatrics and 90% adults, where the good response
was defined as the absence of new ischemic events after
1-month postoperatively..

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

20. The disease is more common in females as compared to
males (1.8:1).
The ischemic type predominates in childhood (69% of
cases in patients <10 years old).
The hemorrhagic type is more characteristic of adults. It is
extremely rare for children to present with hemorrhage
(2.8%).
In children, headache is a frequent presenting symptom,
which may persist even after successful revascularization.
Epilepsy and involuntary movements are also commonly
seen.

21. Bimodal onset
1st decade
3rd& 4th deacde
Children presents
with
Stroke, seizure,
headache
Adult presents with
Hemorrhage or
stroke

22. Stages of MMD observed on DSA in different patients: (a) narrowed
ICA branches; (b) appearance of moyamoya vessels; (c) attenuated
ACA and MCA with significant moyamoya vessels; (d) occluded ICA,
attenuated moyamoya vessels; (e) occluded ICA, disappearance of
moyamoya vessels; and (f) occlusion extending to posterior circulation
supporting the anterior circulation
Suzuki's classification for ICA (grade 1-6) & Mugikura's
classification of posterior cerebral artery (PCA; grade 1-4)

23. Postoperative collateral grading on
external carotid angiography (Matsushima and Inaba)

24. Potential mechanisms of moyamoya disease.
The association between genetic, circulating, and environmental factors.
RNF213, Ring finger 213; EPCs, endothelial progenitor cells; SPCs, smooth
muscle progenitor cells; miRNAs, microRNAs; CRABP-1, cellular retinoic acid-
binding protein-I.

25. • Arteriosclerosis,
• Neurofibromatosis,
• Irradiation,
• Sickle cell anemia,
• Connective Tissue Abnormalities,
• Down syndrome
Secondary causes of Moya Moya disease —

26. -
• 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

27. 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)
Omental transposition
multiple burr holes procedure
Superior cervical ganglionectomy
Cervical pericarotid sympathectomy

28. 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

29. 4. Raphael Guzman, Michael Kelly and Gary K. Steinberg :
Clinical outcome after 450 revascularization procedures for
moyamoya disease:J. Neurosurg. / May 22, 2009
5. Steven D. Chang and Gary K. Steinberg:
Surgical Management of Moyamoya Disease
www.moyamoya.com
6. Dianna M. Milewicz et al. Mutations in smooth muscle
Alpha-Actin ( ACTA2) cause coronary artery
disease,stroke,Moya Moya disease and Thoracic aortic
disease : The American Journal of Human
Genetics,84,617—627, May 15,2009

30. THANK YOU