Prevalence of and Risk Factors for Cerebral Microbleeds in Moyamoya Disease and Syndrome in the American Population.

BACKGROUND AND PURPOSE: Cerebral microbleeds (CMB) are reported to be frequent in moyamoya disease (MMD) and moyamoya syndrome (MMS) in the Asian population. It is associated with an increased risk of intracerebral hemorrhage. The significance of CMB in MMD/MMS in non-Asian populations has not been well established. Our study aimed to investigate the prevalence of CMB in MMD/MMS in a moymoya cohort with a majority of non-Asians and to identify risk factors for developing a CMB and its predictive value for subsequent vascular events. METHODS: The moyamoya database was compiled by screening for MMD/MMS among patients admitted to the Zale-Lipshy University Hospital at the University of Texas Southwestern Medical Center. We identified and analyzed data of 67 patients with MMD or MMS. Patients were characterized as CMB+ or CMB- based on MRI findings. In CMB+ patients, the total number and location of CMB were identified. Univariate and multivariate logistic regression were used to identify risk factors for developing CMB and whether CMB are associated with the development of subsequent vascular events. RESULTS: Out of a total of 67 patients, 11 (16%) had CMB. Males had significantly higher odds of having CMB as compared to females (OR 1.76; 95% CI 1.40-24.3, p = 0.021). The incidence of CMB was also associated with age at diagnosis (mean age of CMB+ patients vs. CMB- patients: 44 vs. 34 years, respectively, p = 0.024), smoking (p = 0.006), and hemorrhagic stroke at presentation (p = 0.034). Logistic regression with multivariate analysis found that gender and age at diagnosis remained statistically significant. New ischemic events occurred in 2 (20%) out of 10 CMB+ patients and 13 (23%) out of 55 CMB- patients, respectively (p = 0.79). While 2 (3%) CMB- patients had a new cerebral hemorrhage during follow-up, none of the CMB+ patients did. CONCLUSIONS: CMB are less prevalent in MMD/MMS in the USA than in Asia. An older age at diagnosis and male gender were associated with CMB. The presence of CMB was not associated with an increased risk of a subsequent ischemic or hemorrhagic stroke.

Novel application of percutaneous cryotherapy for the treatment of recurrent oral bleeding from a noninvoluting congenital hemangioma involving the right buccal space and maxillary tuberosity.

Cryotherapy is the application of varying extremes of cold temperatures to destroy abnormal tissue. The intent of this article is to describe a novel technique using percutaneous cryotherapy for treating a noninvoluting congenital craniofacial hemangioma (NICH). An 18-year-old woman with type 1 von Willebrand's disease, as well as a qualitative platelet aggregation disorder, presented with multiple recurrent episodes of oral bleeding from a NICH involving the right buccal space and maxillary tuberosity. The patient was initially treated with a combination of endovascular particulate embolization, percutaneous sclerotherapy, tissue cauterization, and laser therapy between the ages of 4 and 8 years of age. At 18 years of age, the patient presented with recurrent episodes of oral bleeding related to the NICH. Endovascular embolization was performed using particulate and a liquid embolic agent with limited success. Due to the refractory nature of this bleeding, the patient underwent successful lesion ablation using percutaneous cryotherapy. At 9-month follow-up, the patient is asymptomatic with no episodes of recurrent bleeding.

Fontan operation in the current era: a 15-year single institution experience.

OBJECTIVE: Evaluate current risk factors for mortality and morbidity in patients undergoing the Fontan procedure at a single institution in the current era. SUMMARY BACKGROUND DATA: An emphasis on early relief of volume and pressure overload culminating in the Fontan procedure has improved patient outcomes for patients with a single ventricle. METHODS: A cross-sectional retrospective study was performed for 636 primary Fontan procedures between July 1992 and June 2007. RESULTS: Anatomy included left ventricular hypoplasia in 64% and right ventricular hypoplasia in 36%. A lateral tunnel (LT) was performed in 92% and an extracardiac conduit (EC) in 8%. Hospital survival was 96%. Long-term survival was 97% at a mean follow-up of 50 months (range, 0-173 months). Ventricular anatomy and preoperative hemodynamics did not predict early or late survival. Longer aortic cross clamp (XC) time was associated with decreased late survival (P = 0.01). Fontan takedown was required in 3% and protein-losing enteropathy (PLE) developed in 6%. At follow-up, 98% of patients were either NYHA class I or II and 87% were in normal sinus rhythm. Patients with chest tube drainage >2 weeks had an increased risk of PLE (P < 0.0001) and diminished short- and long-term survival (P = 0.026 and P < 0.0001, respectively). CONCLUSIONS: The Fontan procedure can be performed with low risk regardless of ventricular anatomy. Duration of XC time is associated with survival. Prolonged CT drainage correlates with late PLE and diminished survival. There was a low prevalence of late rhythm disturbances and other complications.