SURGICAL MANAGEMENT OF MOYA MOYA DISEASE

SURGICAL MANAGEMENT OF MOYA MOYA DISEASE

----AFTER 15 CASES

DR AMIT KUMAR GHOSH Consultant Neurosurgeon

Institute Of Neurosciences, Kolkata

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)

Presentations ---

Infarcts--- 12

Hemorrhage--- 10

Progressive cognitive decline---- one

Both infarct and hemorrhage--- Two

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

SURGERY–

Indication– Individualized depending on general and neurological status

EDAMS only or EDAMS and STA-MCA bypass

ENCEPHALO-DURO-ARTERIO-MYO-SYNANGIOSIS

STA-MCA BYPASS

MCA—M4

STA

MCA Temporary clipping

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

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 their deficit were completely resolved.

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.

POST-OPERATIVE ANGIOGRAPHY AND MR perfusion scan

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

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.

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.

• 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

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

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

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.

References1) 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

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