Cerebrovascular moyamoya disease in a 5-year-old girl who underwent interruption of the right common carotid artery in the neonatal period

The authors report on the case of a girl with cerebrovascular moyamoya disease born with severe respiratory failure caused by a congenital diaphragmatic hernia. Cardiopulmonary management included extracorporeal membrane oxygenation until the diaphragm defect was repaired. The right common carotid artery (CA) was interrupted and cannulated for extracorporeal membrane oxygenation. When she was 5 years of age, the patient experienced ischemic symptoms in her right extremities. Angiography revealed stenosis of the terminal portion of the internal CA (ICA) with the development of moyamoya vessels on the left side of the brain; the right ICA was supplied by extracranial anastomotic arteries. Indirect extracranial–intracranial bypass surgery was performed in the left hemisphere, and the hypoperfusion improved. The same change in the intracranial ICA with the development of moyamoya vessels occurred on her right side when she was 7 years old. Decreased cerebral blood flow occurred twice, and the moyamoya vessels developed to compensate for the cerebral ischemia. However, the occlusion of the extracranial common CA in infancy induced extracranial anastomosis rather than moyamoya vessel proliferation, and collateral circulation was formed at the lesion site. This finding indicates that neoangiogenesis requires both cerebral ischemia and growth factors derived from the lesion.

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Circle of Willis Blood Velocity and Flow Direction After Common Carotid Artery Ligation for Neonatal Extracorporeal Membrane Oxygenation

PEDIATRICS ◽

10.1542/peds.83.3.343 ◽

1989 ◽

Vol 83 (3) ◽

pp. 343-347

Author(s):

Tonse N. K. Raju ◽

Shin Y. Kim ◽

Janet L. Meller ◽

Gopal Srinivasan ◽

Vivek Ghai ◽

...

Keyword(s):

Blood Flow ◽

Carotid Artery ◽

Extracorporeal Membrane Oxygenation ◽

Cerebral Artery ◽

Common Carotid Artery ◽

Circle Of Willis ◽

Artery Ligation ◽

Membrane Oxygenation ◽

Carotid Artery Ligation ◽

The Right

The velocity and direction of blood flow in the circle of Willis arteries were measured in three infants who underwent right common carotid artery ligation for extracorporeal membrane oxygenation treatment. Within 15 minutes of common carotid artery ligation, blood flow was detected in one infant's right middle cerebral artery; however, the velocity was reduced to 50% of the preextracorporeal membrane oxygenation level. The velocity remained 50% to 70% lower than normal during the 88 hours of extracorporeal membrane oxygenation therapy. In the other two infants, the velocity changes were less severe. By 2 to 10 weeks after weaning from extracorporeal membrane oxygenation, the velocities in the left cerebral arteries were increased to 116% to 217% of the corresponding right cerebral vessels. Following common carotid artery ligation, a retrograde direction of flow was noted in the first (A1) segment of the right anterior cerebral artery and in the right posterior communicating artery, whereas the direction of flow was normal in the corresponding vessels on the left. After common carotid artery ligation, the vertebrobasilar and the contralateral internal carotid systems appear to be the main sources of reperfusion of the right cerebral hemisphere via the circle of Willis. Furthermore, because of the known variants of the circle anatomy, a noninvasive pulsed Doppler method could be used to evaluate the flow patterns in the circle of Willis arteries, both before and after common carotid artery ligation for extracorporeal membrane oxygenation.

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Quantitative Electroencephalography Values of Neonates During and After Venoarterial Extracorporeal Membrane Oxygenation and Permanent Ligation of Right Common Carotid Artery

Artificial Organs ◽

10.1111/j.1525-1594.2006.00240.x ◽

2006 ◽

Vol 30 (6) ◽

pp. 447-451 ◽

Cited By ~ 7

Author(s):

Gerhard Trittenwein ◽

Sandra Plenk ◽

Elisabeth Mach ◽

Gehan Mostafa ◽

Harald Boigner ◽

...

Keyword(s):

Carotid Artery ◽

Extracorporeal Membrane Oxygenation ◽

Common Carotid Artery ◽

Quantitative Electroencephalography ◽

Membrane Oxygenation ◽

Venoarterial Extracorporeal Membrane Oxygenation

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Morbidity for Survivors of Extracorporeal Membrane Oxygenation: Neurodevelopmental Outcome at 1 Year of Age

PEDIATRICS ◽

10.1542/peds.83.1.72 ◽

1989 ◽

Vol 83 (1) ◽

pp. 72-78

Author(s):

Penny Glass ◽

Marilea Miller ◽

Billie Short

Keyword(s):

High Risk ◽

Carotid Artery ◽

Extracorporeal Membrane Oxygenation ◽

Jugular Vein ◽

Long Term Outcome ◽

Membrane Oxygenation ◽

Cardiopulmonary Support ◽

High Risk Infants ◽

The Right

Extracorporeal membrane oxygenation is an important technology in the treatment of high-risk infants whose long-term outcome is being followed prospectively at our institution. The extracorporeal membrane oxygenation procedure allows temporary cardiopulmonary support for critically ill full-term neonates who are refractory to maximum ventilatory and medical management as a consequence of severe persistent pulmonary hypertension. The technique necessitates both the permanent ligation of the right common carotid artery and jugular vein and systemic heparinization. The survivors constitute a unique group of high-risk infants, from the standpoint of the hypoxic-ischemic insults preceding extracorporeal membrane oxygenation and the risks associated with the procedure. Our results indicate that most of our survivors are developing normally at 1 year. Major morbidity, in terms of either significant developmental delay (Bayley mental and motor indices less than 70) or significant neuromotor abnormality, occurred in only 10% of these infants. Poor outcome was associated with major intracranial hemorrhage and chronic lung disease. Ligation of the right carotid artery and jugular vein was not associated with a consistent lateralizing lesion. Long-term follow-up through school age is essential.

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