Pipeline Embolization Device for Treatment of Craniocervical Internal Carotid Artery Dissections: Report of 3 Cases.

BACKGROUND: Dissecting pseudoaneurysms of the craniocervical circulation are uncommon, accounting for only 3% of all cerebral aneurysms. These aneurysms pose a challenge due to their location and anatomic configuration. The Pipeline Embolization Device (PED) is a flow diversion technique that successfully treats aneurysms by diverting blood flow away from the aneurysm and reconstructing the diseased parent artery by altering its hemodynamics. CASE DESCRIPTION: We report 3 cases in which the PED was used to treat craniocervical carotid artery dissection with associated pseudoaneurysms. A single PED was used in the first case, 4 PEDs were used in the second case, and 3 PEDs and a PRECISE PRO RX carotid stent were placed in the third case. All 3 patients achieved full neurologic recovery postoperatively. Cerebral angiography performed postoperatively demonstrated revascularization, good laminar flow, and no in-stent or adjacent stenosis. CONCLUSIONS: PED placement offers a safe and effective method of treating spontaneous or traumatic craniocervical carotid artery dissections with excellent neurologic outcomes postoperatively and complete long-term aneurysmal occlusion.

Surgical management of large scalp infantile hemangiomas.

BACKGROUND: Infantile Hemangiomas (IH) are the most common benign tumor of infancy, occurring in over 10% of newborns. While most IHs involute and never require intervention, some scalp IHs may cause severe cosmetic deformity and threaten tissue integrity that requires surgical excision. CASE DESCRIPTION: We present our experience with two infants who presented with large scalp IH. After vascular imaging, the patients underwent surgical resection of the IH and primary wound closure with excellent cosmetic outcome. We detail the surgical management of these cases and review the relevant literature. CONCLUSION: In some cases the IHs leave behind fibro-fatty residuum causing contour deformity. Surgery is often required for very large lesions causing extensive anatomical and/or functional disruption. The goal of surgical intervention is to restore normal anatomic contour and shape while minimizing the size of the permanent scar.

Resolution of trigeminal neuralgia by coil embolization of a persistent primitive trigeminal artery aneurysm.

The persistent primitive trigeminal artery (PTA) is a rare anastomosis between the carotid artery and basilar artery. While most PTAs are asymptomatic, lateral variants can occasionally compress the trigeminal nerve and precipitate trigeminal neuralgia. Aneurysms of the PTA are exceptionally rare in the literature and have not previously been associated with trigeminal neuralgia. We present the first case of an aneurysm of the PTA causing trigeminal neuralgia. The patient underwent coil embolization of the aneurysm which relieved her symptoms. We propose embolization as a viable therapeutic option for the resolution of trigeminal neuralgia when the condition is secondary to irritation by the high velocity pulsatile flow of an aneurysm.

Resolution of trigeminal neuralgia by coil embolization of a persistent primitive trigeminal artery aneurysm.

The persistent primitive trigeminal artery (PTA) is a rare anastomosis between the carotid artery and basilar artery. While most PTAs are asymptomatic, lateral variants can occasionally compress the trigeminal nerve and precipitate trigeminal neuralgia. Aneurysms of the PTA are exceptionally rare in the literature and have not previously been associated with trigeminal neuralgia. We present the first case of an aneurysm of the PTA causing trigeminal neuralgia. The patient underwent coil embolization of the aneurysm which relieved her symptoms. We propose embolization as a viable therapeutic option for the resolution of trigeminal neuralgia when the condition is secondary to irritation by the high velocity pulsatile flow of an aneurysm.

Open surgical disconnection for congenital, multi-hole, pial arteriovenous fistulae in non-eloquent cortex.

Intracranial pial arteriovenous fistulae (pAVFs), a direct shunt between a feeding artery and a venous channel with the absence of a true nidus characteristic of other types of arteriovenous malformations, are rare. We report a seven-year-old girl with an incidental intracranial pAVF. Following partial embolization with a combination of platinum coils and liquid embolic material, this lesion was surgically disconnected and a definitive cure was achieved. Based on the particular characteristics of this lesion-multiple, small arterial feeders, superficial location, and proximity to the non-eloquent cortex-we feel this vascular lesion represents a subset of pAVFs that may be most reasonably and safely treated by open surgery. While staged embolization has recently gained popularity as a treatment option, the additive risk of multiple embolizations as well as repeated exposure to ionizing radiation should not be understated, especially in the pediatric population. Furthermore, given the paucity of data on the long-term effectiveness of embolization, surgery remains an elegant and durable treatment option for pAVFs in carefully selected patients.

Cauda equina syndrome secondary to an absent inferior vena cava managed with surgical decompression.

The authors report on the case of a 24-year-old man who presented with back pain and radiculopathy due to epidural venous engorgement in the setting of a congenitally absent inferior vena cava. Despite initial improvement after steroid administration, the patient's health ultimately declined over a period of weeks, and signs and symptoms of cauda equina syndrome manifested. Lumbar decompression was performed and involved coagulation and resection of the compressive epidural veins. No complications occurred, and the patient made a full neurological recovery.

Pediatric cerebellar pilomyxoid-spectrum astrocytomas.

OBJECT: Pediatric cerebellar astrocytomas with pilomyxoid features include classic pilomyxoid astrocytomas (PMAs) and intermediate pilomyxoid tumors (IPTs). Since the original description of PMA in 1999, most reports in the literature have described PMAs arising from the hypothalamic/chiasmatic region. To the authors' knowledge, PMAs arising from the posterior fossa have not been discussed in the neurosurgical literature. Intermediate pilomyxoid tumors, or tumors with pathological features of both pilocytic astrocytoma (PA) and PMA, have only recently been described. In this article, the authors present 2 cases that fall within the spectrum of pediatric cerebellar PMA-including a classic PMA and an intermediate pilomyxoid tumor. The authors compare the radiological presentation, surgical results, and postoperative course to findings in a cohort of 15 patients with cerebellar PAs. METHODS: Between 2003 and 2010, 2 patients with pilomyxoid-spectrum astrocytomas underwent treatment at Vanderbilt Children's Hospital. One was a 22-month-old girl who presented with progressive gait disturbance and falls. The other was a 4-year-old girl who presented with ataxia and generalized weakness. In a retrospective review of pediatric cerebellar neoplasms resected by the senior author during this period, these tumors comprised 4% of cerebellar neoplasms and approximately 10% of cerebellar glial neoplasms. RESULTS: Both patients were treated with midline suboccipital craniotomy for resection. In both cases, tumor invasion anteriorly into the brainstem prevented gross-total resection. the patient in Case 1 was placed on chemotherapy following pathological diagnosis and later developed definitive evidence of leptomeningeal dissemination (LD) 3 years after the operation. The patient in Case 2 was placed on chemotherapy after exhibiting progressive evidence of local recurrence (findings were negative for LD) 12 months following resection. CONCLUSIONS: Pediatric patients with cerebellar pilomyxoid-spectrum astrocytomas appear to suffer higher rates of local recurrence and LD than pediatric patients with cerebellar PAs.

The spectrum of management practices in nontraumatic subarachnoid hemorrhage: A survey of high-volume centers in the United States.

BACKGROUND: There is a considerable variety of management practices for nontraumatic subarachnoid hemorrhage (ntSAH) across high-volume centers in the United States. We sought to design a survey which would highlight areas of controversy in the modern management of ntSAH and identify specific areas of interest fo further study. METHODS: A questionnaire on management practices in ntSAH was formulated using a popular web-based survey tool (SurveyMonkey(™), Palo Alto, CA) and sent to endovascular neurointerventionists and cerebrovascular surgeons who manage a high volume of these patients annually. Two-hundred questionnaires were delivered electronically, and after a period of 2 months, the questionnaire was resent to nonresponders. RESULTS: Seventy-three physicians responded, representing a cross-section of academic and other high-volume centers of excellence from around the country. On average, the responding interventionists in this survey each manage approximately 100 patients with ntSAH annually. Over 57% reported using steroids to treat this patient population. Approximately 18% of the respondents use intrathecal thrombolytics in ntSAH. Over 90% of responding physicians administer nimodipine to all patients with ntSAH. Over 40% selectively administer antiepileptic drugs to patients with ntSAH. Several additional questions were posed regarding the methods of detecting and treating vasospasm, as well as the indications for CSF diversion in patients with ntSAH further demonstrating the great diversity in management. CONCLUSION: This survey illustrates the astonishing variety of treatment practices for patients with ntSAH and underscores the need for further study.

CpG island methylation of genes accumulates during the adenoma progression step of the multistep pathogenesis of colorectal cancer.

Genetic alterations occur during the adenoma-carcinoma sequence of colon cancer formation and drive the initiation and progression of colon cancer formation. The aberrant methylation of genes is an alternate, epigenetic mechanism for silencing tumor suppressor genes in colon cancer. The aim of this study was to determine on a global and gene-specific level the role of CpG island methylation in the initiation and progression of colon cancer. Consequently, we assessed the frequency of gene methylation in tumors representative of the commonly recognized histological steps of the adenoma-carcinoma progression sequence through the analysis of eight genes previously identified to be methylated in colon cancer, MGMT, HLTF, MLH1, p14(ARF), CDKN2A, TIMP3, THBS1, and CDH1. We observed that the proportion of tumors carrying methylated alleles increased from adenomas to adenocarcinomas but that the proportion of tumors with methylated alleles was not different between adenocarcinomas and metastases (69% versus 90%, P = 0.01 and 90% versus 81%, P > 0.05). The most substantial difference occurred between early and advanced adenomas (47% versus 84%, P = 0.018). Furthermore, we observed that the frequency of gene methylation at the different steps of the progression sequence varied between genes. Thus, the aberrant methylation of genes appears to increase most significantly during the progression of early adenomas to advanced adenomas, and the frequency of specific gene methylation at the different steps of the adenoma-carcinoma progression sequence varies in a gene-specific fashion.

Aberrantly methylated CDKN2A, MGMT, and MLH1 in colon polyps and in fecal DNA from patients with colorectal polyps.

Colon cancer is the third leading cause of cancer-related death in the United States, affecting approximately 147,000 people each year. Most colon cancers arise from benign neoplasms and evolve into adenocarcinomas through a stepwise histologic progression sequence that starts from adenomas or hyperplastic polyps/serrated adenomas. Genetic alterations and, more recently, epigenetic alterations have been associated with specific steps in this polyp-adenocarcinoma sequence and likely drive the histologic progression of colon cancer. Consequently, we have assessed in colon adenomas and hyperplastic polyps the methylation status of MGMT, CDKN2A, and MLH1 to determine the timing and frequency of these events in the polyp-carcinoma progression sequence and subsequently to analyze the potential for these methylated genes to be molecular markers for adenomas and hyperplastic polyps. We have found that methylated MGMT, CDKN2A, and MLH1 occur in 49%, 34%, and 7% of adenomas and in 5%, 10%, and 7% of hyperplastic polyps, respectively, and that they are more common in histologically advanced adenomas. Furthermore, analysis of fecal DNA from persons who have undergone colonoscopic exams revealed methylated CDKN2A, MGMT, and MLH1 in fecal DNA from 31%, 48%, and 0% of individuals with adenomas and from 16%, 27%, and 10% of individuals with no detectable polyps, respectively. These results show that aberrant methylated genes can be detected frequently in sporadic colon polyps and that they can be detected in fecal DNA. Notably, improvements in the specificity and sensitivity of the fecal DNA-based assays will be needed to make them clinically useful diagnostic tests for polyps.