Objective: To explore the effects of pregnancy complicated with moyamoya disease on maternal and fetal outcomes. Methods: The general clinical data and maternal and fetal outcomes of 20 pregnancies of 15 patients with moyamoya disease admitted to the First Affiliated Hospital of Zhengzhou University from January 2012 to October 2022 were retrospectively analyzed. Results: (1) General information: among the 20 pregnancies of 15 clearly diagnosed pregnant women complicated with moyamoya disease, 12 were diagnosed before pregnancy (60%, 12/20), 3 were diagnosed during pregnancy (15%, 3/20), and 5 were diagnosed during puerperal period (25%, 5/20). There were 7 cases of primipara (35%, 7/20) and 13 cases of multipara (65%, 13/20). (2) Pregnancy complications and maternal and infant outcomes: among the 20 pregnancies of 15 pregnant women with moyamoya disease, there were 9 pregnancy complications (45%, 9/20), including 5 gestational hypertension (25%, 5/20), 2 severe pre-eclampsia (10%, 2/20), 1 hyperlipidemia and 1 gestational diabetes mellitus (5%, 1/20). There were 2 case of drug abortion in the first trimester, 3 cases of labor induction in the second trimester, and 15 cases of delivery during the third trimester. All the 15 deliveries were cesarean section, of which 11 (11/15) were cesarean sections with medical indications, and 4 (4/15) were cesarean sections caused by personal factors. General anesthesia was used in 5 cases (5/15), epidural block anesthesia in 7 cases (7/15), and combined spinal and epidural anesthesia in 3 cases (3/15). The median gestational age of 15 neonates was 37.2 weeks (34.0 to 40.8 weeks), with 10 cases (10/15) were full-term infants, and 5 (5/15) were preterm infants (3 of which were associated with hypertensive disorder complicating pregnancy). The birth weight of 15 neonates was (2 853±454) g. Four neonates were admitted to neonatal intensive care unit (NICU), of which 3 cases were admitted to NICU due to premature delivery and 1 case was admitted to NICU due to neonatal jaundice. There was no neonatal asphyxia or death. All neonates were followed up from 4 months to 6 years after birth, and all grew well. (3) Neurological symptoms during pregnancy: 8 cases (40%, 8/20) had neurological symptoms during pregnancy, and 6 cases (30%, 6/20) had hemorrhagic symptoms, of which 3 cases occurred during the puerperal period (3/6). There were 2 cases of ischemic symptoms (10%, 2/20), all of which occurred during the puerperal period (2/2). (4) Analysis of factors related to the occurrence of cerebral hemorrhage: the incidence of cerebral hemorrhage in patients with moyamoya disease diagnosed before pregnancy was significantly lower than that in those without a clear diagnosis, and the incidence of cerebral hemorrhage in women with moyamoya disease was lower than that in primipara (all P<0.01). The incidence of cerebral hemorrhage in moyamoya patients without hypertensive disorder complicating pregrancy was lower than that in patients with hypertensive disorder complicating pregrancy, but the difference was not statistically significant (P>0.05). Conclusions: Pregnancy combined with moyamoya disease has adverse effects on maternal and infant outcomes, and the incidence of pregnancy complications increases. Cerebral hemorrhage occurres in prenatal and puperium, while cerebral ischemia occurres mainly in puperium.
Infant ; Pregnancy ; Infant, Newborn ; Female ; Humans ; Pregnancy Outcome ; Cesarean Section ; Pregnant Women ; Infant, Premature ; Moyamoya Disease/complications* ; Retrospective Studies ; Pregnancy Complications/epidemiology* ; Cerebral Hemorrhage

Anticoagulants ; Atrial Appendage/surgery* ; Atrial Fibrillation/complications* ; Brain Ischemia ; Cardiac Catheterization ; Female ; Humans ; Moyamoya Disease/surgery* ; Stroke ; Treatment Outcome

Adult ; Hemoglobinuria, Paroxysmal ; complications ; pathology ; Humans ; Male ; Moyamoya Disease ; diagnosis ; etiology ; pathology ; Young Adult

The etiology and pathogenesis of moyamoya disease (MMD) remain elusive. Some inflammatory proteins, such as cyclooxygenase (COX)-2, are believed to be implicated in the development of MMD. So far, the relationship between COX-2 and MMD is poorly understood and reports on the intracranial vessels of MMD patients are scanty. In this study, tiny pieces of middle cerebral artery (MCA) and superficial temporal artery (STA) from 13 MMD patients were surgically harvested. The MCA and STA samples from 5 control patients were also collected by using the same technique. The expression of COX-2 was immunohistochemically detected and the average absorbance (A) of positively-stained areas was measured. High-level COX-2 expression was found in all layers of the MCA samples from all 5 hemorrhagic MMD patients, while positive but weak expression of COX-2 was observed only in the endothelial layer of the MCA samples from most ischemic MMD patients (6/8, 75%). The average A values of COX-2 in the hemorrhagic MMD patients were substantially higher than those in their ischemic counterparts (t=4.632, P=0.001). There was no significant difference in the COX-2 expression among the "gender" groups, or "radiographic grade" groups, or "lesion location" groups (P>0.05 for all). The COX-2 expression was detected neither in the MCA samples from the controls nor in all STA specimens. Our results suggested that COX-2 was up-regulated in the MCA of MMD patients, especially in hemorrhagic MMD patients. We are led to speculate that COX-2 may be involved in the pathogenesis of MMD and even contribute to the hemorrhagic stroke of MMD patients.
Adult ; Case-Control Studies ; Cyclooxygenase 2 ; genetics ; metabolism ; Female ; Humans ; Intracranial Hemorrhages ; enzymology ; etiology ; Male ; Middle Aged ; Middle Cerebral Artery ; metabolism ; Moyamoya Disease ; complications ; enzymology

PURPOSE: Leptomeningeal collateral, in moyamoya disease (MMD), appears as an ivy sign on fluid-attenuated inversion-recovery (FLAIR) images. There has been little investigation into the relationship between presentation of ivy signs and old brain lesions. We aimed to evaluate clinical significance of ivy signs and whether they correlate with old brain lesions and the severity of clinical symptoms in patients with MMD. MATERIALS AND METHODS: FLAIR images of 83 patients were reviewed. Each cerebral hemisphere was divided into 4 regions and each region was scored based on the prominence of the ivy sign. Total ivy score (TIS) was defined as the sum of the scores from the eight regions and dominant hemispheric ivy sign (DHI) was determined by comparing the ivy scores from each hemisphere. According to the degree of ischemic symptoms, patients were classified into four subgroups: 1) nonspecific symptoms without motor weakness, 2) single transient ischemic attack (TIA), 3) recurrent TIA, or 4) complete stroke. RESULTS: TIS was significantly different as follows: 4.86+/-2.55 in patients with nonspecific symptoms, 5.89+/-3.10 in patients with single TIA, 9.60+/-3.98 in patients with recurrent TIA and 8.37+/-3.39 in patients with complete stroke (p=0.003). TIS associated with old lesions was significantly higher than those not associated with old lesions (9.35+/-4.22 vs. 7.49+/-3.37, p=0.032). We found a significant correlation between DHI and motor symptoms (p=0.001). CONCLUSION: Because TIS has a strong tendency with severity of ischemic motor symptom and the presence of old lesions, the ivy sign may be useful in predicting severity of disease progression.
Adolescent ; Adult ; Aged ; Brain/metabolism/*pathology ; Cerebral Arteries/*pathology ; Child ; Child, Preschool ; Collateral Circulation ; Disease Progression ; Female ; Humans ; Magnetic Resonance Imaging/*methods ; Male ; Meninges/*pathology ; Middle Aged ; Moyamoya Disease/complications/*pathology ; Severity of Illness Index ; Stroke ; Young Adult

PURPOSE: Moyamoya disease (MMD) occurs predominantly in Korean and Japanese women. The aim of this study was to investigate clinical features and pregnancy outcomes in women with MMD. MATERIALS AND METHODS: We conducted a retrospective chart review of women with MMD who visited our Department of Obstetrics and Gynecology between January 2005 and October 2013. For all study subjects, clinical features, demographic characteristics, and perinatal outcomes were recorded. RESULTS: We identified 28 pregnancies in 22 patients who had been diagnosed with MMD. The mean maternal age at delivery was 31.9+/-3.5 years old. The mean gestational age at delivery was 38.0+/-0.9 weeks. Among the 28 pregnancies, 25 (92.5%) underwent cesarean section; 19 (76.0%) of them were performed under regional anesthesia and six (24.0%) under general anesthesia. The mean newborn weight was 3233.7+/-348.2 g. The 5-minute Apgar score in 85% of the newborns was higher than 8, with no other apparent complications. During the puerperal period, transient ischemic attack symptom or seizure occurred in 4 cases, although patients recovered within a few days. CONCLUSION: For pregnant women with MMD, it is important to control blood pressure and prevent hyperventilation during the intrapartum period, and the best methods of delivery and anesthesia should be considered to avoid unfavorable sequelae. Additionally, a multidisciplinary approach (i.e., neurosurgery) is necessary to constantly manage underlying diseases.
Adult ; Blood Pressure/physiology ; Cesarean Section ; Female ; Gestational Age ; Humans ; Infant, Newborn ; Maternal Age ; Moyamoya Disease/*diagnosis/epidemiology ; Pregnancy ; *Pregnancy Complications ; Pregnancy Outcome/*epidemiology ; Republic of Korea/epidemiology ; Retrospective Studies

The purpose of this study was to determine the prevalence and characteristics of symptomatic coronary heart disease (CHD) in patients with moyamoya disease (MMD). This retrospective study evaluated 456 patients who received examination for MMD between 1995 and 2012. We reviewed the patients' medical history and coronary imaging, including conventional coronary angiography and coronary computed tomography angiogram (CTA). Among 456 patients with MMD, 21 (4.6%) patients were found to have symptomatic CHD. Ten patients were treated with coronary artery bypass graft or percutaneous coronary intervention for unstable angina or myocardial infarction. Eleven were treated with medication for stable angina (n = 6) and variant angina with mild degree of stenosis (n = 5).The median age of these patients was 44 yr (range, 27-59). The median Framingham score at diagnosing MMD was < 1% (range, < 1%-16%). The old age was associated with CHD in uni- and multivariate analyses (P = 0.021, OR, 1.053; 95% CI, 1.008-1.110). Considering low age of onset and low stroke risk factor, CHD might be a systemic manifestation that is clinically relevant to MMD.
Adult ; Age Factors ; Aged ; Coronary Angiography ; Coronary Artery Disease/*etiology ; Female ; Humans ; Logistic Models ; Male ; Middle Aged ; Moyamoya Disease/*complications ; Retrospective Studies

No abstract available.
Adult ; Brain Ischemia/diagnosis/*etiology ; Cerebral Angiography ; Fatal Outcome ; Female ; Humans ; Hyperventilation/complications ; Moyamoya Disease/*complications/diagnosis/therapy ; Risk Factors ; Stroke/diagnosis/*etiology ; Thyroid Crisis/*complications/diagnosis/therapy

No abstract available.
Angiography, Digital Subtraction ; Cerebral Angiography ; Cerebrovascular Circulation ; Collateral Circulation ; Female ; Humans ; Infarction, Middle Cerebral Artery/diagnosis/*etiology/physiopathology ; Intracranial Arteriosclerosis/diagnosis/*etiology/physiopathology ; Magnetic Resonance Angiography ; Middle Aged ; Moyamoya Disease/diagnosis/*etiology/physiopathology ; Predictive Value of Tests ; Scleroderma, Systemic/*complications ; Tomography, Emission-Computed, Single-Photon

Child ; Humans ; Male ; Moyamoya Disease ; etiology ; Takayasu Arteritis ; complications ; diagnosis