Metachronous spinal pial arteriovenous fistulas: case report.

Spinal pial arteriovenous fistulas (spAVFs) are believed to be congenital lesions, and the development of a de novo spAVF has not been previously described. A 49-year-old female with a childhood history of vascular malformation-induced right lower-extremity hypertrophy presented in 2004 with progressive pain in her right posterior thigh and outer foot. Workup revealed 3 separate type IV spAVFs, which were treated by combined embolization and resection, with final conventional angiography showing no residual spinal vascular lesion in 2005. Ten years later, the patient returned with new right lower-extremity weakness, perineal pain, and left plantar foot numbness. Repeat spinal angiography demonstrated 2 de novo intertwined conus medullaris spAVFs.

Safety and outcome of combined endovascular and surgical management of low grade cerebral arteriovenous malformations in children compared to surgery alone.

PURPOSE: To evaluate the outcomes of combined preoperative embolization and microsurgical resection in comparison with microsurgical resection alone as the current standard of care for low-grade cerebral arteriovenous malformations (AVM) in the pediatric population. MATERIALS & METHODS: We performed a single-center retrospective study of pediatric patients presenting with Spetzler-Martin (SM) grade I and II cerebral AVMs at a high-volume tertiary pediatric hospital between January 2005 and September 2016. Low grade AVM patients were divided into two groups: pre-operative embolization with subsequent microsurgical resection or microsurgical resection alone. Patient demographics, clinical and imaging presentations, AVM morphological characteristics, post-operative complications, and mid to long-term clinical outcomes were studied. Post-embolization and post-surgical outcomes were assessed prior to and after treatment, at 3 months and at final follow-up using the modified Rankin Scale (mRS) to compare both final independent (mRS 0-2) and favorable (no change or improved mRS) clinical outcomes for comparison between study groups. Statistical associations of patient demographics, AVM characteristics/SM grading, and treatment modality group with post-operative complications were performed using univariate logistic regression analysis. RESULTS: Thirty-four patients with low grade cerebral AVMs met the study inclusion criteria (mean age 10.6 ± 3.4 years; range 3-16 years, 22M:12 F). Twenty patients (59%) presented with ruptured AVMs. Twenty-five patients (73.5%) underwent combined treatment with embolization and microsurgical resection, while 9/34 (26.5%) underwent microsurgical resection alone. A total of 35 embolization procedures performed in 25 patients (Mode, 1; Range, 1-7) were associated with two minor post-embolization and 7 subsequent post-surgical (28%) complications, resulting in clinical deterioration in a single patient. Microsurgical resection alone was associated with 3 post-surgical complications (33%), resulting in permanent neurological disability in a single patient. There was no significance association of post-operative complications with either treatment modality group, combined treatment versus surgical resection alone [OR:1.13; 95% CI:0.23-5.62; p-value 0.88]. SM Grade II and eloquent locations were found to be significantly associated with post-surgical complications of low grade pediatric cerebral AVMs [OR 13.2 and OR 8 respectively, p-value 0.004 and 0.005). On mean follow-up time of 35.7 months, final clinical outcome was favorable in the majority of both treatment arms with no dependent (mRS>2) patients in the combined endovascular and surgical cohort. Two patients in the surgical cohort failed to achieve independent functional status, primarily due to a pre-operative morbid status (p-value 0.015). However, there was no significant difference in favorable outcomes between the treatment groups [p-value 0.14]. CONCLUSION: Our study suggests equivalent safety and favorable clinical outcomes related to combined endovascular embolization and microsurgical resection of low grade pediatric cerebral AVMs in comparison to microsurgical resection alone. On long term clinical follow-up, the vast majority of patients achieved an independent and favorable functional status irrespective of pre-operative embolization.

Choroidal artery embolization in the management of cerebrospinal fluid overproduction: case report and review of the literature.

Diffuse villous hyperplasia of the choroid plexus (DVHCP) is a rare cause of communicating hydrocephalus. DVHCP may be diagnosed radiographically and through histological evaluation. It may be associated with genetic abnormalities, particularly involving chromosome 9. Due to CSF overproduction, patients with DVHCP often fail management with shunting alone and may require adjuvant interventions. The authors present the case of a child with partial trisomy 9p and delayed diagnosis of hydrocephalus with radiographic evidence of DVHCP who was successfully managed with ventriculoperitoneal shunt (VPS) placement, adjuvant bilateral endoscopic choroid plexus coagulation (CPC), and the novel application of anterior choroidal artery embolization. In addition, a systematic MEDLINE search was conducted using the keywords "diffuse villous hyperplasia," "choroid plexus hypertrophy," and "idiopathic cerebrospinal fluid overproduction." Clinicopathological characteristics and outcomes of the present case were reviewed and compared to those in the literature.A 14-month-old girl with partial trisomy 9p presented with macrocephaly and radiographic evidence of communicating hydrocephalus and DVHCP. Ventriculoperitoneal shunting resulted in distal failure due to inadequate CSF absorption, and ventriculoatrial shunt (VAS) placement was not possible due to multiple cardiac anomalies. Daily CSF production was reduced via endoscopic third ventriculostomy and bilateral CPC, followed by distal choroidal artery embolization, enabling VPS re-internalization. The embolization was complicated by radiographic evidence of an iatrogenic cerebral infarct, but this was clinically occult. Thirty-two additional cases of communicating hydrocephalus due to DVHCP are reported in the literature: 27 pediatric, 3 adult, and 2 postmortem. Genetic abnormalities were noted in 14, with 7 (50%) involving chromosome 9. Twelve patients underwent plexectomy (9 bilateral, 2 unilateral, 1 partial), and 10 underwent CPC (4 bilateral, 3 unilateral, and 3 unspecified), with or without shunting. Eight patients were successfully managed with shunting alone (6 VASs, 2 VPSs), and none underwent arterial embolization.DVHCP is a rare cause of communicating hydrocephalus that may be associated with genetic abnormalities. A thorough review of the literature highlights diagnostic criteria and interventional options involved in managing this cause of CSF overproduction. The present case demonstrates that angiographic confirmation of prominent choroidal arteries may contribute to the diagnosis DVHCP. In addition, embolization of the distal choroidal arteries may be considered as a potential adjuvant treatment in patients for whom conventional treatments have failed or are not feasible.

MynxGrip vascular closure device use in pediatric neurointerventional procedures.

OBJECTIVE The aim of this paper was assess the efficacy and safety of using the MynxGrip arterial closure device in pediatric neuroendovascular procedures where the use of closure devices remains off-label despite their validation and widespread use in adults. METHODS A retrospective review of all pediatric patients who underwent diagnostic or interventional neuroendovascular procedures at the authors' institution was performed. MynxGrip use was predicated by an adequate depth of subcutaneous tissue and common femoral artery (CFA) diameter. Patients remained on supine bedrest for 2 hours after diagnostic procedures and for 3 hours after therapeutic procedures. Patient demographics, procedural details, hemostasis status, and complications were recorded. RESULTS Over 36 months, 83 MynxGrip devices were deployed in 53 patients (23 male and 30 female patients; mean age 14 years) who underwent neuroendovascular procedures. The right-side CFA was the main point of access for most procedures. The mean CFA diameter was 6.24 mm and ranged from 4 mm to 8.5 mm. Diagnostic angiography comprised 46% of the procedures. A single device failure occurred without any sequelae; the device was extracted, and hemostasis was achieved by manual compression with the placement of a Safeguard compression device. No other immediate or delayed major complications were recorded. CONCLUSIONS MynxGrip can be used safely in the pediatric population for effective hemostasis and has the advantage of earlier mobilization.

The rare case of a large complex intraosseous cranial arteriovenous malformation with successful multidisciplinary management.

Intraosseous cranial arteriovenous malformations (AVMs) are very rare, challenging entities. The authors report the case of an extracranial parietooccipital vascular lesion. A 12-year-old boy presented with accelerated growth of a right scalp lesion over a few months. Digital subtraction angiography showed a large, right parietooccipital intraosseous AVM with multiple complex arterial feeders. Treatment of these lesions is difficult and can necessitate a multidisciplinary approach. In the featured case, 6 embolization procedures were performed over 1 year, including both transarterial and transvenous approaches, followed by total resection. The authors describe what is thought to be the second case of an AVM originating in the cranial bones and the first case with successful multidisciplinary management.

Long-term evaluation of a historical cohort of Iranian common variable immunodeficiency patients.

OBJECTIVES: Common variable immune deficiency (CVID) is the most frequent form of symptomatic primary immunodeficiency disease, characterized by hypogammaglobulinemia, recurrent infections and increased predisposition to autoimmunity and malignancies. The aim of this study was to reconsider important points of previously performed studies on Iranian CVID patients diagnosed and followed from 1984 to 2013. METHODS: Diagnosis was made using approved criteria including reductions of serum levels of immunoglobulins and exclusion of well-known single gene defects in individuals with an age >4 years and evidence of specific antibody deficiency. RESULTS: Detailed information on demographic data, survival rates, clinical phenotypes, immunologic and genetic data and treatment of 173 patients are provided. The early onset presentation (74.5%) and rate of consanguineous marriage (61.2%) were considerably higher in our cohort. Our study revealed clinically related correlations regarding consanguinity, the population of naïve CD4(+) T cells and switched-memory B cells, cytokine levels and special genetic factors (including HLA and AID genes). CONCLUSION: Despite current efforts, more comprehensive studies are needed, especially for classification and investigation of the genetic background and prognostic factors for patients with CVID in order to better managment and followup of patinets.

A review on guidelines for management and treatment of common variable immunodeficiency.

Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency in adults. As symptoms of CVID are usually heterogeneous and unspecific, diagnosis and follow-up of CVID can be challenging. In light of this, a broad review of advances in management and treatment of CVID is performed here in order to reach a distinct protocol. However, it should be noted that owing to the nature of the disease, it can only be treated symptomatically but not cured. There is little evidence to guide appropriate or universal guidelines to improve the current status of management of the disease. The most satisfactory treatments of CVID could be achieved by the use of immunoglobulin replacement, antibiotics, immunosuppressants and hematopoietic stem cell transplantation. This review is written based on the importance of clinical surveillance of asymptomatic CVID cases and early recognition of different clinical complications. Moreover, for each complication, appropriate interventions for improving outcomes are mentioned.