Moyamoya syndrome increases the risk of stroke in sickle cell disease, but revascularization surgery can modify this risk. Collaborative management between hematology and neurosurgery offers effective strategies to reduce stroke risk in these patients. We describe a challenging case where a patient with sickle cell disease undergoing standard of care management as prescribed by the Stroke Prevention Trial in Sickle Cell Anemia and revascularization with pial synangiosis subsequently developed rapidly progressive disease in other cerebral vessels and suffered ischemic hemispheric stroke. This case not only demonstrates the success of management in accordance with the American Heart Association (AHA) and American Stroke Association (ASA) guidelines but also demonstrates critical areas where we lack understanding of disease progression.

1.
Scott
RM
,
Smith
ER
.
Moyamoya disease and moyamoya syndrome
.
N Engl J Med
.
2009
;
360
(
12
):
1226
37
. .
2.
Ohene-Frempong
K
,
Weiner
SJ
,
Sleeper
LA
,
Miller
ST
,
Embury
S
,
Moohr
JW
,
Cerebrovascular accidents in sickle cell disease: rates and risk factors
.
Blood
.
1998
;
91
(
1
):
288
94
.
3.
Lawrence
C
,
Webb
J
.
Sickle cell disease and stroke: diagnosis and management
.
Curr Neurol Neurosci Rep
.
2016
;
16
(
3
):
27
. .
4.
Griessenauer
CJ
,
Lebensburger
JD
,
Chua
MH
,
Fisher
WS
 3rd
,
Hilliard
L
,
Bemrich-Stolz
CJ
,
Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease
.
J Neurosurg Pediatr
.
2015
;
16
(
1
):
64
73
.
5.
Ferriero
DM
,
Fullerton
HJ
,
Bernard
TJ
,
Billinghurst
L
,
Daniels
SR
,
DeBaun
MR
,
Management of stroke in neonates and children: a scientific statement from the American heart association/American stroke association
.
Stroke
.
2019
;
50
(
3
):
e51
96
.
6.
Newman
S
,
Boulter
JH
,
Malcolm
JG
,
Pradilla
I
,
Pradilla
G
.
Outcomes in patients with moyamoya syndrome and sickle cell disease: a systematic review
.
World Neurosurg
.
2020
;
135
:
165
70
. .
7.
Aguilar-Salinas
P
,
Hayward
K
,
Santos
R
,
Agarwal
V
,
Sauvageau
E
,
Hanel
RA
,
Surgical revascularization for pediatric patients with sickle cell disease and moyamoya disease in the prevention of ischemic strokes: a single-center case series and a systematic review
.
World Neurosurg
.
2019
;
123
:
435
42 e8
.
8.
Smith
ER
,
McClain
CD
,
Heeney
M
,
Scott
RM
.
Pial synangiosis in patients with moyamoya syndrome and sickle cell anemia: perioperative management and surgical outcome
.
Neurosurg Focus
.
2009
;
26
(
4
):
E10
. .
9.
Scott
RM
,
Smith
JL
,
Robertson
RL
,
Madsen
JR
,
Soriano
SG
,
Rockoff
MA
.
Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis
.
J Neurosurg
.
2004
;
100
(
2
):
142
9
. .
10.
Adegoke
SA
,
Figueiredo
MS
,
Vicari
P
,
Carrete
H
 Jr
,
Idagawa
MH
,
Massaro
AR
,
Posterior circulation evaluation in patients with sickle cell anemia
.
J Stroke Cerebrovasc Dis
.
2016
;
25
(
3
):
717
21
.
11.
Yuan
S
,
Jordan
LC
,
Davis
LT
,
Cogswell
PM
,
Lee
CA
,
Patel
NJ
,
A cross-sectional, case-control study of intracranial arterial wall thickness and complete blood count measures in sickle cell disease
.
Br J Haematol
.
2021
;
192
(
4
):
769
77
.
12.
Adams
R
,
McKie
V
,
Nichols
F
,
Carl
E
,
Zhang
DL
,
McKie
K
,
The use of transcranial ultrasonography to predict stroke in sickle cell disease
.
N Engl J Med
.
1992
;
326
(
9
):
605
10
.
13.
Adams
RJ
,
McKie
VC
,
Hsu
L
,
Files
B
,
Vichinsky
E
,
Pegelow
C
,
Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography
.
N Engl J Med
.
1998
;
339
(
1
):
5
11
.
14.
Hulbert
ML
,
McKinstry
RC
,
Lacey
JL
,
Moran
CJ
,
Panepinto
JA
,
Thompson
AA
,
Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle cell disease
.
Blood
.
2011
;
117
(
3
):
772
9
.
15.
Seidman
C
,
Kirkham
F
,
Pavlakis
S
.
Pediatric stroke: current developments
.
Curr Opin Pediatr
.
2007
;
19
(
6
):
657
62
. .
16.
Merkel
KH
,
Ginsberg
PL
,
Parker
JC
 Jr
,
Post
MJ
.
Cerebrovascular disease in sickle cell anemia: a clinical, pathological and radiological correlation
.
Stroke
.
1978
;
9
(
1
):
45
52
. .
17.
Drew
JM
,
Scott
JA
,
Chua
GT
.
General case of the day. Moyamoya syndrome in a child with sickle cell disease
.
Radiographics
.
1993
;
13
(
2
):
483
4
. .
18.
Garza-Mercado
R
.
Pseudomoyamoya in sickle cell anemia
.
Surg Neurol
.
1982
;
18
(
6
):
425
31
. .
19.
Kirkham
FJ
,
Hogan
AM
.
Risk factors for arterial ischemic stroke in childhood
.
CNS Spectr
.
2004
;
9
(
6
):
451
64
.
20.
Dobson
SR
,
Holden
KR
,
Nietert
PJ
,
Cure
JK
,
Laver
JH
,
Disco
D
,
Moyamoya syndrome in childhood sickle cell disease: a predictive factor for recurrent cerebrovascular events
.
Blood
.
2002
;
99
(
9
):
3144
50
.
21.
Ware
RE
,
Davis
BR
,
Schultz
WH
,
Brown
RC
,
Aygun
B
,
Sarnaik
S
,
Hydroxycarbamide versus chronic transfusion for maintenance of transcranial doppler flow velocities in children with sickle cell anaemia-TCD with transfusions changing to hydroxyurea (TWiTCH): a multicentre, open-label, phase 3, non-inferiority trial
.
Lancet
.
2016
;
387
(
10019
):
661
70
.
22.
Lin
N
,
Baird
L
,
Koss
M
,
Kopecky
KE
,
Gone
E
,
Ullrich
NJ
,
Discovery of asymptomatic moyamoya arteriopathy in pediatric syndromic populations: radiographic and clinical progression
.
Neurosurg Focus
.
2011
;
31
(
6
):
E6
.
23.
Titsworth
WL
,
Scott
RM
,
Smith
ER
.
National analysis of 2,454 pediatric moyamoya admissions and the effect of hospital volume on outcomes
.
Stroke
.
2016
;
47
(
5
):
1303
11
. .
24.
DeBaun
MR
,
Jordan
LC
,
King
AA
,
Schatz
J
,
Vichinsky
E
,
Fox
CK
,
American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults
.
Blood Adv
.
2020
;
4
(
8
):
1554
88
.
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