scholarly journals Disfiguring high-flow cervicofacial arteriovenous malformations

2020 ◽  
Vol 2020 (10) ◽  
Author(s):  
Alaa Nabawi ◽  
Nader Abraham ◽  
Ayman Nabawi

Abstract Arteriovenous malformations (AVMs) are congenital vascular anomalies resulting from defects in angiogenesis. Approximately 40% of AVMs go undetected after birth and only experience the delayed clinical onset of symptoms in adulthood. AVMs are rare, representing only 1.5% of all vascular anomalies. The most common sites for the aberrant vascular nidus are the oral cavity and maxillofacial region, which represent 50% of the cases. AVMs are the most challenging and life-threatening form of vascular malformation. Exsanguination, thrombus detachment and embolization are the most hazardous operative risks. Small case series revealed a 75% recurrence rate during a 5-year follow-up, which adds another layer of complexity to their management. Large lesions in the head and neck cause deformation to the patient and present a challenge to the surgeon during their excision among vital structures and reconstruction of the 3D complex defects.

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xu Ma ◽  
Bing Jie ◽  
Dong Yu ◽  
Ling-Ling Li ◽  
Sen Jiang

Abstract Background The life-threatening haemorrhagic complications of pulmonary arteriovenous malformations (PAVMs) are extremely rare, and only described in isolated cases. This study was designed to comprehensively investigate management of ruptured PAVMs. Methods We retrospectively assessed clinical and imaging data of ruptured PAVMs to summarize incidence, clinical characteristics, and outcomes following embolisation between January 2008 and January 2021. Results Eighteen of 406 (4.4%) patients with PAVMs developed haemorrhagic complications. Twelve of 18 patients were clinically diagnosed with hereditary haemorrhagic telangiectasia (HHT). Haemorrhagic complications occurred with no clear trigger in all cases. Eight of 18 patients (44.4%) were initially misdiagnosed or had undergone early ineffective treatment. 28 lesions were detected, with 89.3% of them located in peripheral lung. Computed tomography angiography (CTA) showed indirect signs to indicate ruptured PAVMs in all cases. Lower haemoglobin concentrations were associated with the diameter of afferent arteries in the ruptured lesions. Successful embolotherapy was achieved in all cases. After embolotherapy, arterial oxygen saturation improved and bleeding was controlled (P < 0.05). The mean follow-up time was 3.2 ± 2.5 years (range, 7 months to 10 years). Conclusions Life threatening haemorrhagic complications of PAVMs are rare, they usually occur without a trigger and can be easily misdiagnosed. HHT and larger size of afferent arteries are major risk factors of these complications. CTA is a useful tool for diagnosis and therapeutic guidance for ruptured PAVMs. Embolotherapy is an effective therapy for this life-threatening complication.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Yuya Kato ◽  
Yoshikazu Ogawa ◽  
Teiji Tominaga

Abstract Background Pregnancy is a known risk factor for pituitary apoplexy, which is life threatening for both mother and child. However, very few clinical interventions have been proposed for managing pituitary apoplexy in pregnancy. Case presentation We describe the management of three cases of pituitary apoplexy during pregnancy and review available literature. Presenting symptoms in our case series were headache and/or visual disturbances, and the etiology in all cases was hemorrhage. Conservative therapy was followed until 34 weeks of gestation, after which babies were delivered by cesarean section with prophylactic bolus hydrocortisone supplementation. Tumor removal was only electively performed after delivery using the transsphenoidal approach. All three patients and their babies had a good clinical course, and postoperative pathological evaluation revealed that all tumors were functional and that they secreted prolactin. Conclusions Although the mechanism of pituitary apoplexy occurrence remains unknown, the most important treatment strategy for pituitary apoplexy in pregnancy remains adequate hydrocortisone supplementation and frequent hormonal investigation. Radiological follow-up should be performed only if clinical symptoms deteriorate, and optimal timing for surgical resection should be discussed by a multidisciplinary team that includes obstetricians and neonatologists.


Author(s):  
Virender Malik ◽  
Harshith Kramadhari ◽  
Jawahar Rathod ◽  
Yadav W. Munde ◽  
Uday Bhanu Kovilapu

AbstractThe peripheral high-flow vascular malformation (HFVM) comprises arteriovenous malformation (AVM) and fistula (AVF), shows varied clinical presentation (ranging from subtle skin lesion to life-threatening congestive heart failure), and frequently poses diagnostic and therapeutic challenges. Importance of assigning a specific diagnosis to the vascular malformation cannot be overstated, as the treatment strategy is based on the type of vascular anomaly. Although the International Society for the Study of Vascular Anomalies (ISSVA) classification system is the most commonly accepted system for classifying congenital vascular anomalies in clinical practice, the Cho–Do et al classification is of utmost help in guiding optimal mode of treatment in peripheral AVM. Although transarterial approach remains the most commonly employed route for peripheral AVM embolization, the role of transvenous and direct percutaneous approach is ever increasing and the final decision on the approach depends on angioarchitecture of the AVM. In this article, we review various commonly employed classification systems for congenital vascular anomalies, and describe clinical features, imaging and treatment strategies for peripheral arteriovenous malformation (PAVM).


Author(s):  
Parag B. Lad ◽  
N. Venkateshwaran ◽  
M. R. Thatte ◽  
Sanket Tanpure

AbstractManagement of child abuse with flexor tendons, neurovascular injuries, and life-threatening conditions is challenging. It needs a multisectoral coordinated and synchronized team effort for successful outcomes. We present a case series of children abused by a parent with a sharp object. The children sustained multiple flexor tendon injuries, neurovascular injuries in upper limbs, and tracheal injury compromising respiration. We performed a tracheostomy to save a child and subsequently repaired numerous flexor tendons, nerves, and arteries. During follow-up, these children required secondary reconstruction (tenolysis, tendon lengthening, nerve reconstruction) for flexor contractures, stiffness, and sensory loss in distal forearms. We measured the range of movements and assessed the children’s functional outcome using the Strickland score at 3-year follow-up. The range of movement and functional outcome was excellent in both children in our series. A timely performance of surgery, aided with efficient intensive care, therapy, and consistent posttraumatic psychosocial rehabilitation, produced excellent results in our series.


2019 ◽  
Vol 131 (4) ◽  
pp. 1297-1307 ◽  
Author(s):  
Eric S. Nussbaum ◽  
Kevin M. Kallmes ◽  
Jeffrey P. Lassig ◽  
James K. Goddard ◽  
Michael T. Madison ◽  
...  

OBJECTIVEBecause simple intracranial aneurysms (IAs) are increasingly treated endovascularly, neurovascular surgery has become focused on complex IAs that may require deconstructive aneurysm therapy with concomitant surgical bypass. The authors describe the decision-making process concerning cerebral revascularization and present outcomes that were achieved in a large case series of complex IAs managed with cerebral revascularization and parent artery occlusion.METHODSThe authors retrospectively reviewed the medical records, including neuroimaging studies, operative reports, and follow-up clinic notes, of all patients who were treated at the National Brain Aneurysm Center between July 1997 and June 2015 using cerebral revascularization as part of the management of an IA. They recorded the location, rupture status, and size of each IA, as well as neurological outcome using the modified Rankin Scale (mRS), aneurysm and bypass status at follow-up, and morbidity and mortality.RESULTSThe authors identified 126 patients who underwent revascularization surgery for 126 complex, atheromatous, calcified, or previously coiled aneurysms. Ninety-seven lesions (77.0%) were unruptured, and 99 (78.6%) were located in the anterior circulation. Aneurysm size was giant (≥ 25 mm) in 101 patients, large (10–24 mm) in 9, and small (≤ 9 mm) in 16 patients. Eighty-four low-flow bypasses were performed in 83 patients (65.9%). High-flow bypass was performed in 32 patients (25.4%). Eleven patients (8.7%) underwent in situ or intracranial-intracranial bypasses. Major morbidity (mRS score 4 or 5) occurred in 2 (2.4%) low-flow cases and 3 (9.1%) high-flow cases. Mortality occurred in 2 (2.4%) low-flow cases and 2 (6.1%) high-flow cases. At the 12-month follow-up, 83 (98.8%) low-flow and 30 (93.8%) high-flow bypasses were patent. Seventy-five patients (90.4%) undergoing low-flow and 28 (84.8%) high-flow bypasses had an mRS score ≤ 2. There were no statistically significant differences in patency rates or complications between low- and high-flow bypasses.CONCLUSIONSWhen treating challenging and complex IAs, incorporating revascularization strategies into the surgical repertoire may contribute to achieving favorable outcomes. In our series, low-flow bypass combined with isolated proximal or distal parent artery occlusion was associated with a low rate of ischemic complications while providing good long-term aneurysm control, potentially supporting its wider utilization in this setting. The authors suggest that consideration should be given to managing complex IAs at high-volume centers that offer a multidisciplinary team approach and the full spectrum of surgical and endovascular treatment options to optimize patient outcomes.


Neurosurgery ◽  
2019 ◽  
Vol 66 (Supplement_1) ◽  
Author(s):  
Rebecca Y Du ◽  
Melissa LoPresti ◽  
Vijay Ravindra ◽  
Ionela Iacobas ◽  
Katie Bergstrom ◽  
...  

Abstract INTRODUCTION Cerebral arteriovenous malformations (AVMs) are the most common cause of spontaneous intracranial hemorrhage in pediatric patients. Recurrence after complete surgical resection is a recognized risk that occurs primarily in children. While Pediatric Neurosurgery practices traditionally focus on clinical management, recent advances have found gene mutations to play a role in pathogenesis and development of AVMs It is not known how many pediatric AVMs are sporadic versus arising from familial inheritance patterns. We aimed to see how identified genetic mutations are represented in our AVM patient population in Texas and the southern United States. METHODS We developed a multidiscipinary pediatric neurovascular program at our institution. Patients and families were screened in their first-time and/or follow-up outpatient clinic encounters by our hematologist-oncologist and genetics counsellor from the Pediatric Vascular Anomalies program. Genetic testing recommendations and results were recorded in our IRB-approved ongoing prospective neurovascular database. RESULTS 50 patients were screened in Vascular Anomalies program. Diagnoses ranged from AVMs (n = 20), cerebral cavernous malformations (n = 13), non-NF/non-sickle-cell moyamoya (n = 7), and other lesions such as cerebral proliferative angiopathy and megelencephaly-capillary malformation. Of the 50 patients, 30 underwent genetic testing. 7 had identified mutations. 3 patients with cavernous angiomas had Krit1 mutations. 2 patients with AVMs had Rasa1 mutations, and 2 patients with megenecephaly-capillary malformations had PIK3CA mutations. CONCLUSION These preliminary findings highlight the importance of thinking beyond neurosurgical intervention for pediatric neurovascular diseases. Genetic mutations have been found in 23% of patients screened thus far. Such findings provide insight into disease biology and may likely have clinical implications for risk assessment, family screening, and follow-up surveillance. Further work needs to be done to capture all our AVM population for genetic screening over time, while expanding our neurovascular program's study focus to encompass all neurovascular pathologies.


2009 ◽  
Vol 37 (4) ◽  
pp. 619-623 ◽  
Author(s):  
G. D. Puri ◽  
I. Sen ◽  
J. R. Bapuraj

This report describes three children, aged eight to 11 years, with high-flow cerebral arteriovenous malformations who underwent interventional neuroradiological procedures involving glue (N-butyl cyanoacrylate) embolisation under general anaesthesia. The procedure was facilitated by relative hypotension induced by esmolol infusion and intravenous adenosine boluses. To allow controlled deposition of N-butyl cyanoacrylate into the arteriovenous malformations, glue injection was synchronised with the onset of adenosine-induced brief cardiac standstill. This resulted in satisfactory obliteration of the arteriovenous malformations nidus in all cases. The haemodynamic modulations, including the adenosine-induced brief cardiac standstill, was noted to not affect the BIS values in our patients. All patients had satisfactory obliteration of their arteriovenous malformations and had good neurological outcomes at one-year follow-up.


2012 ◽  
Vol 18 (3) ◽  
pp. 309-313 ◽  
Author(s):  
H. Maekawa ◽  
M. Tanaka ◽  
H. Hadeishi

Cerebral proliferative angiopathy (CPA) is a rare vascular abnormality with several angiomorphological features that are distinct from brain arteriovenous malformations (AVMs). The natural history of CPAs indicates a lower risk for hemorrhage compared to brain AVMs. A 62-year-old woman presented with gait instability and dysarthria. MRI and angiography revealed a diffuse vascular network involving the tectum and cerebellar vermis with intermingled brain parenchyma. This lesion had no dominant feeder, high-flow arteriovenous shunt, flow-related aneurysm or highly dilated veins on angiogram. These findings were consistent with a diagnosis of CPA. During follow-up, she developed progressive gait instability and eye movement abnormalities, but no remarkable change was detected on the repeated MRI and angiography. Nine years later, she died of mesencephalic hemorrhage originating from the CPA. To the best of our knowledge, this is the first description of a patient with CPA who died as a result of the initial hemorrhage. It is important to recognize that a part of CPAs is aggressive and can be more vulnerable to critical hemorrhage.


2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Elvira Ferrés-Amat ◽  
Jordi Prats-Armengol ◽  
Isabel Maura-Solivellas ◽  
Eduard Ferrés-Amat ◽  
Javier Mareque-Bueno ◽  
...  

Intraosseous arteriovenous malformations (AVMs) in the head and neck region are uncommon. There are several types and they can have a wide range of clinical presentations. Depending on the blood flow through the AVM, the treatment may be challenging for the attending team and may lead to life-threatening hemorrhages. A clinical case report is presented. A 9-year-old girl, seen for gingival bleeding during oral hygiene, was found to have a high-flow AVM located within and around the mandible. Two-stage treatment consisted of intra-arterial embolization followed by intraoral injection of a sclerosing agent 8 weeks later. At the 8-year follow-up, imaging study showed no evidence of recurrent lesion inside or outside the bone. The final outcome is a correct occlusion with a symmetric facial result. This case shows that conservative treatment may be the first treatment option mostly in children. Arteriography and transcortical injection were enough to control the AVM.


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