颅骨板障内纤维鞘瘤伴左顶叶血管畸形一例

颅骨板障内纤维鞘瘤伴左顶叶血管畸形是一种相对独立的相邻两部位同时发生病变的疾病,在临床较少见,本文通过回顾性分析2016年9月收治的1例颅骨板障内纤维鞘瘤伴左顶叶血管畸形患者的相关资料,对颅内肿瘤和血管畸形并存的这一现象进行报道。     

Craniofacial osseointegration

In year 2007, 30 years have elapsed since the first patient was supplied with a craniofacial osseointegrated implant. The reason for implanting in this patient was a severe conductive hearing loss, which necessitated the use of a bone conduction hearing aid. By utilizing the possibility to transmit sound to the cochlea via direct bone conduction, a new era in audiology was established. Further applications of osseointegration in the craniofacial field is related to the rehabilitation of patients with defects from cancer therapy, malformations, traumatic amputations and burns. Specific fields of osseointegration in this respect are due to possible side effects from radiotherapy and chemotherapy that will affect osseointegration negatively. Other aspects are related to osseointegration in children. This review will focus on the knowledge gained during the first 30 years of craniofacial osseointegration.     

EML4::ALK fusions in complex lymphatic malformations

Gorham–Stout disease (GSD) and generalized lymphatic anomaly (GLA) are subtypes of complex lymphatic malformations (CLMs) with osseous involvement that cause significant complications, including pain and pathologic fractures. As with other vascular anomalies, somatic mosaic mutations in oncogenes are often present, and the mTOR inhibitor sirolimus alleviates symptoms in some, but not all, patients. We describe two patients, one with GSD and one with GLA, found to have EML4::ALK fusions. This report of a targetable, oncogenic fusion in vascular malformations expands our understanding of the genetic basis for CLMs and suggests additional targeted therapies could be effective.     

Embolization using hydrogel-coated coils for pulmonary arteriovenous malformations

PurposeTo prospectively evaluate the efficacy and safety of embolization using hydrogel-coated coils for the treatment of pulmonary arteriovenous malformations (PAVMs).Materials and methodsThe outcomes of 21 PAVMs in 19 patients (3 men and 16 women; mean age, 58.8 ± 15.2 [SD] years; age range 14–78 years) treated by venous sac embolization (VSE) with additional feeding artery embolization were prospectively evaluated. For VSE, using one or more 0.018-inch hydrogel-coated coils was mandatory. Recanalization and/or reperfusion were evaluated by pulmonary arteriography 1 year after embolization.ResultsThe mean feeding artery and venous sac sizes were 4.0 mm and 8.5 mm, respectively. Embolization was successfully completed in 20/21 PAVMs, yielding a technical success rate of 95%. The feeding artery was also embolized in 17/20 successful PAVMs (85%). A technical failure occurred in one PAVM, where embolization was abandoned because of migration of one bare coil to the left ventricle. The mean numbers of hydrogel-coated coils and bare platinum detachable coils used for VSE were 3.3 ± 2.1 (SD) (range, 1–8) and 4.4 ± 3.9 (SD) (range, 1–17), respectively. The mean percentages of hydrogel-coated coils in number, length, and estimated volume were 42.9%, 33.3%, and 72.7% respectively. One patient with one PAVM was lost to follow-up after 3 months. Neither recanalization nor reperfusion was noted in the remaining 19 PAVMs (success rate, 19/19 [100%]). One grade 4 (coil migration) adverse event occurred, and it was treated without any sequelae.ConclusionVSE using hydrogel-coated coils with additional feeding artery embolization is a safe and effective treatment for PAVM.     

Complete versus partial excision of infected arteriovenous grafts: Does remnant graft material impact outcomes?

ObjectiveInfected arteriovenous grafts necessitate intervention to obtain source control. However, excising the graft material can be challenging and can lead to complications. Leaving a cuff of graft at the sites of anastomosis allows for the avoidance of potential risks. However, it is unclear whether doing so places patients at risk of recurrent graft infection. The purpose of the present study was to investigate the effect of complete vs partial excision of infected arteriovenous prosthetic dialysis access grafts.MethodsThe data from all patients who had undergone surgical intervention for infected arteriovenous grafts at a single institution were retrospectively reviewed. The patients were grouped according to intervention type: complete excision and partial excision of arteriovenous prosthetic grafts. Partial excisions were further substratified based on whether flow had been restored through the arteriovenous access. The primary outcome was freedom from subsequent intervention for infection, defined as the number of days from excision to subsequent reoperation for reinfection. Freedom from infection was analyzed using the Kaplan-Meier method.ResultsA total of 117 patients had undergone surgical intervention for 122 infected arteriovenous grafts from 2003 to 2016. Of these 117 patients, 79 (64.8%) had undergone partial excision of infected arteriovenous grafts, and 43 (35.2%) had undergone complete excision with vascular repair. Within the partial excision cohort, 71 infected arteriovenous grafts (58.2%) were not flow restored and 8 (6.6%) were flow restored using either prosthetic or autogenous interpositions. The median follow-up time was 2.4 years (interquartile range, 0.6-4.5 years). The most common causative organisms included methicillin-resistant Staphylococcus aureus (n = 34; 27.9%), methicillin-sensitive S. aureus (n = 17; 13.9%), and S. epidermidis (n = 15; 12.3%). The recurrent infection rate in the partial excision group was 16.5% (n = 13) compared with 2.3% (n = 1) in the complete excision group. In the flow-restored subcohorts, those with restoration using prosthetic interposition grafts had the greatest reinfection rate at 57.1% (n = 4), and those with restoration using autogenous conduits did not experience reinfection (P = .033).ConclusionsIncomplete excision of infected arteriovenous prosthetic grafts was associated with a higher rate of reinfection compared with complete graft excision. Complete excision presents technical challenges but could provide superior source control in managing infected dialysis access. Complete excision with vascular reconstruction should be performed when possible to avoid leaving remnant prosthetic material.     

Fatal glioblastoma after Gamma Knife radiosurgery for arteriovenous malformation in a child

We describe a fatal case of glioblastoma multiforme that was induced by Gamma Knife radiosurgery (GKS; Elekta AB, Stockholm, Sweden) for an arteriovenous malformation (AVM). A 4-year-old girl presented with repeated convulsions. Imaging studies revealed an AVM located in the right thalamus. One year after initial symptoms, GKS was performed to obliterate the nidus. The maximum and marginal radiation doses were 32 and 16 Gy, respectively. Seventy months after GKS, the patient represented with severe headache. MRI showed a poorly demarcated tumor with heterogeneous gadolinium enhancement in the right thalamus and adjacent to the white matter of the temporal lobe. After a generalised convulsion, the patient deteriorated into a deep coma. CT scans showed severe brain swelling with intratumoral hemorrhage. An emergency craniotomy was performed, and the hematoma was removed. During this surgery, a tumor mass, which was found adjacent to the hematoma, was resected. Microscopic examination revealed glioblastoma multiforme. Despite intensive treatment, the patient died 1 month after surgery. A GKS-induced secondary tumor is a rare but serious complication. It is important to be aware of the adverse effects of GKS, including secondary neoplasms, before its clinical application, especially in young patients.     

Brainstem arteriovenous malformation presenting with dyspraxic handwriting in a young girl

We report the case of a 11-year-old girl who developed an isolated hand-writing disorder with dysgraphia at the beginning of the school year in the sixth grade. A brain magnetic resonance angiography showed a round arteriovenous malformation sited in the left side of the midbrain extending to the ipsilateral medio-basal thalamus. Child neurologists should never neglect a thorough neurological evaluation in case of isolated worsening of handwriting, to rule out possible underlying organic causes.