Dural arteriovenous fistula (D-AVF): a rare cause of symmetrical bithalamic changes with free diffusivity changes.

We report a rare case of an encephalopathic presentation of a posterior fossa dural arteriovenous fistula with concomitant florid symmetrical bithalamic free diffusivity changes on MRI in a previously healthy individual. We describe the structural and functional imaging findings and the role of catheter angiography in diagnosis, prognostication and the timing and strategy in the management of this challenging vascular entity.

[Bilateral thalamic ischaemia secondary to a tentorial dural fistula]. / Isquemia talamica bilateral secundaria a fistula dural tentorial.

TITLE: Isquemia talamica bilateral secundaria a fistula dural tentorial.

Traumatic arteriovenous fistula of the superficial temporal artery: a histopathologic report.

Traumatic aneurysms, pseudoaneurysms, and arteriovenous (AV) fistulas of the superficial temporal artery (STA) are uncommon, accounting for only 0.5% to 2.0% of all aneurysms subjected to surgery. In the literature that relates to the STA, the general term "aneurysm following traumatic injury" often includes a wide array of pathologic entities, including proper aneurysms, pseudoaneurysms, and AV fistulas. In 75% of cases, such aneurysms are due to blunt trauma. Within this group, STA AV fistulas constitute an exceedingly rare subset. The case presents the occurrence of a traumatic AV fistula of the STA following blunt trauma (karate kick) and provides the first description of the histologic appearance of this lesion.

Cavernous malformation of brainstem, thalamus, and basal ganglia: a series of 176 patients.

BACKGROUND: Cavernous malformations (CMs) in deep locations account for 9% to 35% of brain malformations and are surgically challenging. OBJECTIVE: To study the clinical features and outcomes following surgery for deep CMs and the complication of hypertrophic olivary degeneration (HOD). METHODS: Clinical records, radiological findings, operative details, and complications of 176 patients with deep CMs were reviewed retrospectively. RESULTS: Of 176 patients with 179 CMs, 136 CMs were in the brainstem, 27 in the basal ganglia, and 16 in the thalamus. Cranial nerve deficits (51.1%), hemiparesis (40.9%), numbness (34.7%), and cerebellar symptoms (38.6%) presented most commonly. Hemorrhage presented in 172 patients (70 single, 102 multiple). The annual retrospective hemorrhage rate was 5.1% (assuming CMs are congenital with uniform hemorrhage risk throughout life); the rebleed rate was 31.5%/patient per year. Surgical approach depended on the proximity of the CM to the pial or ependymal surface. Postoperatively, 121 patients (68.8%) had no new neurological deficits. Follow-up occurred in 170 patients. Delayed postoperative HOD developed in 9/134 (6.7%) patients with brainstem CMs. HOD occurred predominantly following surgery for pontine CMs (9/10 patients). Three patients with HOD had palatal myoclonus, nystagmus, and oscillopsia, whereas 1 patient each had limb tremor and hemiballismus. At follow-up, 105 patients (61.8%) improved, 44 (25.9%) were unchanged, and 19 (11.2%) worsened neurologically. Good preoperative modified Rankin Score (98.2% vs 54.5%, P = .001) and single hemorrhage (89% vs 77.3%, P < .05) were predictive of good long-term outcome. CONCLUSION: Symptomatic deep CMs can be resected with acceptable morbidity and outcomes. Good preoperative modified Rankin Score and single hemorrhage are predictors of good long-term outcome.

Ondine's curse with accompanying trigeminal and glossopharyngeal neuralgia secondary to medullary telangiectasia.

BACKGROUND: Central hypoventilation syndrome ("Ondine's Curse") is an infrequent disorder that can lead to serious acute or chronic health consequences. This syndrome, especially in adults, is rare, and even less frequent in the absence of clear pathogenic lesions on MRI. In addition, we are not aware of any previously reported cases with associated cranial nerve neuralgias. METHODS: We describe a patient with baseline trigeminal and glossopharyngeal neuralgia, admitted with episodes of severe hypoventilatory failure of central origin, consistent with "Ondine's Curse". After evaluation, she was found to have a medullary capillary telangiectasia, thought to be the causative lesion, and which could explain her complete neurologic and hypoventilatory syndrome. The patient was treated with placement of a diaphragmatic pacing system, which has been effective thus far. RESULTS: This case illustrates the need for investigation of centrally mediated apnea, especially when co-occurring cranial nerve neuralgia is present and cardiopulmonary evaluation is negative. It provides an example of capillary telangiectasia as the causative lesion, one that to our knowledge has not been reported before. CONCLUSIONS: Placement of a diaphragmatic pacing system was warranted and became lifesaving as the patient was deemed to be severely incapacitated by chronic ventilatory insufficiency.

[A case report of spinal dural AV fistula which showed drastic recovery].

Spinal dural AVF is the most common type of spinal AVMs. It affects especially old male with slowly progressive paraparesis in most cases. We have experienced a rare case of a middle aged male with spinal dural AVF who showed rapid deterioration in its course. The patient was treated with surgical interruption of AVF after transarterial embolization with NBCA. The patient improved well after the treatment, and intramedullary lesion on MRI disappeared after 4 years. The authors would like to stress the importance of early diagnosis and proper treatment for spinal dural AVFs.

[Cavernous sinus dural arteriovenous fistula complicated by edematous cerebral lesions from venous etiology]. / FISTULE ARTERIOVEINEUSE DE LA LOGE CAVERNEUSE COMPLIQUEE DE LESIONS CEREBRALES OEDEMATEUSES D'ORIGINE VEINEUSE.

The authors report the case of a 38-year-old woman with a cavernous DAVF resulting in edematous lesions located in the territory of the ipsilateral basal vein. Transarterial embolization led to subtotal regression of the fistula associated with the regression of cerebral abnormalities. The authors discuss the pathophysiology of the cerebral edematous lesions and the therapeutic consequences according to the venous drainage of the cavernous sinus.

Vascular malformations of the colon in children.

PURPOSE: The aim of this study was to evaluate clinical, radiologic, surgical, and histopathologic characteristics of vascular malformations of the colon (VMC) in children. METHODS: Eighteen patients with lower intestinal bleeding (LIB) and VMC were included. Clinical data, angiographic features, surgical treatments, and histopathologic findings were analyzed. RESULTS: Age of clinical onset was from 1 week to 16 years. Duration of LIB before diagnosis was from 1 month to 9 years. Admission hemoglobin level was 2 to 9 g/dL in 13 patients and in the other 5 was normal. Scintigraphy, colonoscopy, and barium enema results were normal or suggested a vascular problem. Mesenteric arteriography detected VMC in all 18 cases. Left hemicolon and rectum were affected in 83% of the cases. VMC were resected in 14 patients, 10 were anastomosed or pulled-through, and the other 4 required an intestinal stoma. Ten patients are asymptomatic, and 4 have minimal postoperative bleeding. The histopathologic study made in 15 cases (14 surgical specimens and 1 endoscopic biopsy), revealed arterial-venous VMC in 12 and arterial-venous-lymphatic VMC in 3 cases. CONCLUSIONS: The identification of VMC in children with LIB often is delayed. An early mesenteric arteriography should be added to the routine evaluation. Surgical treatment presents a challenge in rectal cases. To avoid confusion in the nomenclature a simplified morphologic classification is suggested.