Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 8  |  Issue : 3  |  Page : 269-273

Lifesaving successful embolization of aggressive vertebral body hemangioma and a large pulmonary arteriovenous malformation


Department of Interventional Radiology, AVBRH, Sawangi, Wardha, Maharashtra, India

Date of Submission23-Jul-2020
Date of Acceptance02-Nov-2020
Date of Web Publication6-Jul-2021

Correspondence Address:
Prashant Gajanan Pote
Department of Interventional Radiology, AVBRH, Sawangi, Wardha, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_105_20

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  Abstract 


Vertebral hemangiomas are incidental finding and most of the time do not necessitate immediate attention unless symptomatic. Vertebral hemangiomas can be very aggressive to the extent that they can produce cord compression and paraplegia/paraparesis. Such cases need evaluation with magnetic resonance imaging to assess the cause, extent, and mass effect on the spinal cord. Transcatheter embolization and decompression surgery are well established modes of treatment as single or combined approach. Pulmonary arteriovenous malformation (AVM) is caused by the direct communication of main pulmonary artery with pulmonary vein, leading to a high-flow right-to-left shunt. This usually presents clinically as hypoxia, cyanosis, and dyspnea. The clinical signs and symptoms vary depending on the size, number, and flow of fistula as well as the associated vascular malformations. Transcatheter embolization is a treatment of choice for pulmonary AVM. However, this method may result in incomplete resolution if the malformation is large in size or supplied by multiple feeding arteries. Surgical resection may be considered in those kinds of cases. As AVM can lead to hemodynamical instability, these lesions need to be addressed first. In this case, pulmonary AVM came to notice post surgical decompression. Here, we report a case of aggressive vertebral body hemangioma and a large pulmonary AVM in the same patient, which were treated by embolization in two different settings.

Keywords: Embolization, pulmonary arteriovenous malformation, vertebral hemangioma


How to cite this article:
Pote PG, Banode P, Rawekar S. Lifesaving successful embolization of aggressive vertebral body hemangioma and a large pulmonary arteriovenous malformation. Indian J Vasc Endovasc Surg 2021;8:269-73

How to cite this URL:
Pote PG, Banode P, Rawekar S. Lifesaving successful embolization of aggressive vertebral body hemangioma and a large pulmonary arteriovenous malformation. Indian J Vasc Endovasc Surg [serial online] 2021 [cited 2021 Jul 25];8:269-73. Available from: https://www.indjvascsurg.org/text.asp?2021/8/3/269/320607




  Introduction Top


Vertebral body hemangiomas usually do not require any clinical attention unless they are large and produce clinical feature due to cord compression or vertebral fracture. These are picked with magnetic resonance imaging (MRI) in both symptomatic and asymptomatic patients. Identification of any other vascular malformation necessitates syndromic workup. Pulmonary arteriovenous malformation (AVM) is caused by the direct visualization of main pulmonary artery with pulmonary vein, leading to a high-flow right-to-left shunt. Despite the lack of clinical symptoms in most cases, pulmonary arterial venous fistulous communication requires treatment due to considerable high rate of mortality and morbidity. Both the pathologies when encountered in the same patient make management challenging and multidisciplinary approach critical.

Before the era of intervention radiology, surgical resection was the only available option for treatment of these lesions. Currently, transcatheter embolization is the treatment of choice for both vertebral hemangioma and pulmonary AVM. In the current report, we describe the case of a paraplegic patient with a large vertebral body hemangioma and pulmonary AVM treated by transcatheter embolization.


  Case Report Top


A 16-year-old female presented with the only complaint of paraparesis. Power in the bilateral lower limb was Grade 1. There were no other neurologic or respiratory symptoms. As a part of routine physical examination, there was no evidence of cyanotic changes on her lips and fingertips or evidence of clubbing in the fingers. On admission, the patient's vitals were within normal range.

The patient underwent MRI spine which demonstrated an aggressive vertebral body hemangioma at D6 level. There was extraosseous intraspinal extension of the vascular component from the inner wall of the D6 vertebra and D5–D6 disc level. There was significant cord compression and cord edema which was responsible for the patient's paraparesis. Considering the aggressive nature of hemangioma, a decision for embolization with particulate embolic material was taken. Preoperative angiogram showed vascular blush in the involved vertebra and prominent vascular channels in the extradural intraspinal space [Figure 1] and [Figure 2]. The lesion was promptly supplied by the bilateral intercostal arteries at this level. No artery of Adamkiewicz or spinal artery was seen arising from the feeding arteries supplying the hemangioma. The feeders were selectively cannulated with a PROGREAT® catheter and embolization was done with polyvinyl alcohol (PVA) particle size 300. Almost complete embolization was achieved with residual lesion <10% [Figure 3] and [Figure 4]. There was no intraoperative–perioperative complication. Still, there was no evidence of hypoxia, and the patient was maintaining well on room air. Power in the lower limb had improved to Grade 3 within 2 days post embolization.
Figure 1: Right T6 intercostal artery angiogram

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Figure 2: Left T6 intercostal artery angiogram

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Figure 3: Post embolization images from right T6 intercostal artery angiogram confirming almost complete embolization with minimal residual blush

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Figure 4: Post embolization images left T6 intercostal artery angiogram confirming almost complete embolization with minimal residual blush

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Surgical decompression with laminectomy and stabilization with a metallic screw was planned. The patient underwent a spinal surgery in general anesthesia which lasted for approximately 6 h and resulted in significant blood loss. This was the first time the patient was unable to maintain oxygen saturation despite being on ventilation with FiO2 of 100%. Maximum oxygen saturation achieved was 65%. There was a high suspicion of associated pulmonary AVM. Computed tomography pulmonary angiogram was done on the following day which showed a large pulmonary malformation in the left lower lobe [Figure 5] and [Figure 6]. The largest feeding vessel had a maximum diameter of 12 mm.
Figure 5: Coronal maximum intensity projection images from pulmonary angiogram demonstrating a large pulmonary arteriovenous malformation (thick arrow). Prominent left pulmonary artery is shown by thin arrow

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Figure 6: Axial maximum intensity projection images from pulmonary angiogram demonstrating a large pulmonary ar teriovenous malformation (thick arrow). Prominent left pulmonary artery is shown by thin arrow

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Retrospectively, the patient's skin, oral cavity, and nasal cavity were then carefully examined suspecting hereditary hemorrhagic telangiectasia (HHT), but no identifiable lesion was found. The patient's parents also denied any history of recurrent or spontaneous epistaxis. There were no family members who had been diagnosed in the past with visceral AVM. As there were no supportive findings for the diagnosis of HHT other than visceral AVM, this case was regarded as non-HHT-related pulmonary AVM and vertebral hemangioma.

Immediately, we took the patient for embolization as any delay would have proved fatal. We used multiple Nester soft platinum coils: two coils of size 35-14-12 – one of 35-14-10 and one of 35-14-8 [Figure 7], [Figure 8], [Figure 9], [Figure 10]. Postembolization angiogram showed no more abnormal vascular connection. At the beginning of the procedure, the patient's SpO2 was at 65% with FiO2 of 100%. There was satisfactory clinical improvement as we succeed to achieve SpO2 of 98% with FiO2 of 100% within 10 min of the last coil packing. The patient was shifted to the intensive care unit and extubation was done on the following day. The patient was kept on nasal oxygen at 5 L/min and the very next day, she was able to maintain saturation (SpO2 of 95%) on room air.
Figure 7: A large pulmonary arteriovenous malformation in the left marked by thick arrow. Prominent left pulmonary artery is shown by thin arrow

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Figure 8: A large pulmonary arteriovenous malformation in the left marked by thick arrow. Prominent left pulmonary artery is shown by thin arrow

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Figure 9: Postcoil embolization, almost complete disappearance of the arteriovenous malformation

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Figure 10: Day 2 postcoil embolization – the patient had pneumothorax in the right hemithorax (thin arrow) due to central line which was managed conservatively. Coils are shown in thick arrow

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At the time of discharge, bilateral lower limb power improved to Grade 4, otherwise the patient was clinically asymptomatic.


  Discussion Top


In 1927, Makrykostas described how ballooning of a vertebra or epidural extension of vertebral hemangioma results in cord compression and neurological symptoms.[1] Various modalities were considered since then including radiation therapy, vertebroplasty, ethanol ablation, and surgery and endovascular embolization. The optimal goal of treatment is relieving the cord compression and providing stability to the vertebral column.

Vertebral hemangioma is a highly vascular lesion and thus imposes the risk of uncontrollable hemorrhage during surgical interventions.[2] The study conducted by Manning in 1951 before the modern era of surgery estimated mortality rate as higher as 25% due to uncontrolled bleeding.[1] Endovascular embolization can be a definitive therapy for aggressive hemangioma which was demonstrated earlier by Gross et al. (1976) who reported neurological improvement within 48 h following endovascular embolization with Gelfoam®. Raco et al. (1990) shared their experience of treatment of five cases suggesting improvement in to patients which did not require further treatment. In 2009, a case report by Kiroglue et al. demonstrated successful treatment of aggressive vertebral hemangioma with endovascular embolization, but the patient had recurrent spinal cord compression and required surgical treatment. In a study by Jayakumar et al. (1997), clinical improvement was reported in 11 out of 12 patients who were treated with embolization and laminectomy, thus establishing the role of preoperative embolization in combination with laminectomy. However, embolization is contraindicated when the feeding vessel supplies the artery of Adamkiewicz in addition to hemangioma.

Similarly, in the case under description, we have taken decision to go for the combined approach of embolization followed by surgery.

Pulmonary AV fistula (AVF) is a rare disease entity. There is a recognized female predilection with F: M ratio ranging around 1.5–1.8:1. The estimated incidence is thought to be around 2–3/100,000.[3] Aggressive vertebral hemangioma and pulmonary AVF in the same patient is a rare occurrence. Pulmonary AVF or AVM is commonly associated with HHT which is an autosomal dominant genetic disorder.[4] It is reported that at least 33% of those with a single PAVM and at least 50% of those with multiple PAVMs have HHT.[5] However, in this case, there was pulmonary AVF with aggressive vertebral hemangioma, which had no family history.

Many patients with pulmonary AVM/AVF do not present early in the disease process unless there is a precipitating factor. In our case, the precipitating factor was the long duration of surgery and blood loss which manifested AVF due to a high-flow right-to-left shunt. However, many a time, patients present with neurological complications such as transient ischemic attack, brain abscess, migraine, or even stroke, none of which was observed in this case.[6] Combining the three contemporary studies of consecutive patients with untreated PAVM, the incidence of stroke was 11.4%, the incidence of brain abscess was 6.8%, and total morbidity and mortality was 23%.[7] When followed over time, PAVM typically remains unchanged in size, although about 25% enlarge gradually.

Due to high morbidity rate, identification and timely treatment of these vascular malformations and symptoms is of paramount importance in patient care. Currently, all pulmonary AVMs/AVFs are amenable to embolization via coil or vascular plug.[8] Multiple series have demonstrated anatomic resolution of PAVMs, reduction in right-to-left shunting, and improvement in oxygenation after embolization.[4],[9],[10] After embolization of multiple feeding vessels, regression of pulmonary AVM is noted immediately, resulting in improvement in oxygenation which can be witnessed on the operation table.

There are several reports of efficacy of embolization for the treatment of large pulmonary AVM.[9],[10] An interesting feature in the present case was just with the use of four coils, the fistula was completely occluded and significant symptomatic improvement was noted in the patient's oxygen saturation.

There have been multiple case reports/series on aggressive vertebral hemangiomas and pulmonary AVM, but the occurrence of both pathologies in the same patient and their successful endovascular embolization have not been reported yet. This case also emphasizes on identifying pulmonary malformation in all cases of aggressive vertebral hemangioma, thus their embolization can be planned before undergoing the definitive surgical procedure.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Manning HJ. Symptomatic hemangioma of the spine. Radiology 1951;56:58-65.  Back to cited text no. 1
    
2.
Alexander J, Meir A, Vrodos N, Yau YH. Vertebral hemangioma: An important differential in the evaluation of locally aggressive spinal lesions. Spine (Phila Pa 1976) 2010;35:E917-20.  Back to cited text no. 2
    
3.
Khurshid I, Downie GH. Pulmonary arteriovenous malformation. Postgrad Med J 2002;78:191-7.  Back to cited text no. 3
    
4.
Gossage JR, Kanj G. Pulmonary arteriovenous malformations. A state of the art review. Am J Respir Crit Care Med 1998;158:643-61.  Back to cited text no. 4
    
5.
Suchin CR, Whitman GJ, Chew FS. Pulmonary arteriovenous malformation. AJR Am J Roentgenol 1996;167:648.  Back to cited text no. 5
    
6.
White RI, Lunch-Nyhan A, Terry P, Buescher PC, Farmlett EJ, Charnas L, et al. Pulmonary arteriovenous malformations: Techniques and long-term outcome of embolotherapy. Radiology 1988;169:663-9.  Back to cited text no. 6
    
7.
Dines DE, Arms RA, Bernatz PE, Gomes MR. Pulmonary arteriovenous fistulas. Mayo Clin Proc 1974;49:460-5.  Back to cited text no. 7
    
8.
Hsu CC, Kwan GN, Thompson SA, Evans-Barns H, van Driel ML. Embolisation for pulmonary arteriovenous malformation. Cochrane Database Syst Rev 2012;1:CD008017.  Back to cited text no. 8
    
9.
Shovlin CL, Letarte M. Hereditary haemorrhagic telangiectasia and pulmonary arteriovenous malformations: Issues in clinical management and review of pathogenic mechanisms. Thorax 1999;54:714-29.  Back to cited text no. 9
    
10.
Lacombe P, Lacout A, Marcy PY, Binsse S, Sellier J, Bensalah M, et al. Diagnosis and treatment of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: An overview. Diagn Interv Imaging 2013;94:835-48.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]



 

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