Preoperative transdural collateral vessels in moyamoya as radiographic biomarkers of disease.

OBJECTIVE The prevalence of angiographically evident preoperative transdural collateral vessels in moyamoya is not well documented. The authors hypothesized that transdural collaterals could be used as radiographic biomarkers of disease, and that their presence is associated with more advanced moyamoya arteriopathy at diagnosis, which is a harbinger of more frequent operative complications and a predictor of better long-term angiographic results following surgery. METHODS The study consists of a single-institution case series of patients with moyamoya who underwent pial synangiosis between 2005 and 2013. RESULTS Moyamoya was diagnosed in a total of 204 patients (n = 121 [59%] female, 83 [41%] male); the average age at surgery was 9.5 years (range 0.4-35 years). Radiographically, 154 (75%) had bilateral disease for a total of 308 affected hemispheres; 152 (75%) had radiographic stroke; and 190 (93%) had "ivy sign" on FLAIR MRI, indicating slow flow. Of the 358 hemispheres, 324 were treated operatively. On preoperative angiography, 107 patients (52%) had transdural collaterals in 176 affected hemispheres (49%). The Suzuki stage was higher in patients with collaterals (3.4 vs 3.0, p = 0.002). Of 324 treated hemispheres, 84 (26%) had collaterals within the surgical field. Complications included 12 strokes (3.7% stroke rate/hemisphere), with 5 (42%) directly attributable to interruption of transdural collaterals. On 1-year postoperative arteriograms available in 222 hemispheres, Matsushima grades trended better in patients with preoperative collaterals (1.5 vs 1.8 [A = 1, B = 2, C = 3]; p < 0.003). CONCLUSIONS Transdural collaterals are present in nearly half of all preoperative arteriograms in patients with moyamoya. These collaterals are more common in advanced disease, are associated with stroke as a perioperative complication, and may suggest increased capacity to produce surgical collaterals postoperatively. These data support the utility of preoperative arteriography.

Sleep dysfunction in long term survivors of craniopharyngioma.

Craniopharyngiomas are slow growing tumors of the sellar and parasellar region and may also involve the hypothalamus. Treatment involves maximal surgical excision or subtotal resection followed by focal radiation therapy. Late effects of treatment include endocrinopathies, cognitive deficits, behavioral changes, obesity and sleep dysfunction. We conducted a retrospective review of all patients with craniopharyngioma more than 2 years off treatment and who were evaluated in the neuro-oncology survivorship clinic between 2003 and 2007. Clinical data, extent of resection, treatment modalities, endocrine status, patient symptom report and sleep study results were collected to evaluate the presence of patient reported daytime sleepiness and sleep disturbance and to determine possible risk factors. 28 patients were identified (25 %) female. 19/28 self-reported daytime fatigue or sleep disturbance; this included 4/6 patients with gross total resection and 15/22 with subtotal resection. 16/22 patients treated with cranial irradiation reported sleep-related abnormalities, compared to 3/6 patients who did not receive radiation. All but one patient had pituitary dysfunction requiring hormonal replacement. Patients with more than ≥2 sleep related complaints had a higher BMI (44.6 vs. 32.6, p = 0.0192). 8 patients underwent formal sleep evaluation. 3 patients had documented central or obstructive sleep apnea. The mean arousal index was 11.0/h (normal <5). Two patients were treated with melatonin for sleep disturbance and 2 were treated with stimulants for excessive daytime sleepiness. A majority of patients with craniopharyngioma have self-reported daytime fatigue and/or sleep dysfunction after treatment. Extent of resection did not increase the likelihood of patient-reported daytime sleepiness and/sleep dysfunction; however, patients who received radiation more frequently reported daytime sleepiness and/or sleep dysfunction. Patients with a higher BMI were more likely to experience sleep disturbance. Formal sleep evaluations should be considered in all patients with craniopharyngioma.

Anesthetic management of pial synangiosis and intracranial hemorrhage with a Fontan circulation.

We report the case of a 11-year-old girl with Moyamoya syndrome, who had undergone staged-repair of tricuspid atresia to a Fontan circulation, scheduled to undergo bilateral pial synangiosis. Surgery for the first hemisphere was complicated by intracranial hemorrhage requiring an emergency craniotomy. The case highlights the importance of understanding Fontan physiology and its interrelationship with the cerebral circulation in the setting of cerebrovascular insufficiency and raised intracranial pressure.