Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 7  |  Issue : 3  |  Page : 306-308

Management of a curious case of a large recurrent truncal vascular anomaly in a young adult


Department of Surgery, Armed Forces Medical College, Pune, Maharashtra, India

Date of Submission10-Jan-2020
Date of Decision18-Feb-2020
Date of Acceptance07-Mar-2020
Date of Web Publication12-Sep-2020

Correspondence Address:
Basil Badarudeen
Department of Surgery, Armed Forces Medical College, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_4_20

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  Abstract 


Venous malformations (VMs) as the name implies contain only venous blood which is slow moving and are hence classified under low-flow vascular malformations. Sclerotherapy is the main modality of treatment for small VMs. However, bigger lesions require surgical excision in addition to interventional procedures. Hereby, we present a successfully resected case of a large, diffuse, multiloculated, and multiplanar vascular anomaly of the trunk, which presented to us after incomplete resolution following multiple sittings of interventional therapy. Surgical excision was tricky and challenging because of its large size and complex anatomical involvement. Complete resolution of the lesion was achieved with no neurological deficit. There was full recovery of movements at the shoulder joint on follow-up.

Keywords: Sclerotherapy, surgical excision, venous malformations


How to cite this article:
Roy N, Badarudeen B, Pillai HJ. Management of a curious case of a large recurrent truncal vascular anomaly in a young adult. Indian J Vasc Endovasc Surg 2020;7:306-8

How to cite this URL:
Roy N, Badarudeen B, Pillai HJ. Management of a curious case of a large recurrent truncal vascular anomaly in a young adult. Indian J Vasc Endovasc Surg [serial online] 2020 [cited 2020 Oct 26];7:306-8. Available from: https://www.indjvascsurg.org/text.asp?2020/7/3/306/294905




  Introduction Top


Venous malformations (VMs) are congenital abnormalities belonging to the group of vascular malformations. VMs are by definition slow-flow, simple malformations containing abnormal venous network.[1] They can occur in almost any part of the body. 40% of VMs have been reported to occur in the head-and-neck region, while another 40% are seen in the extremities. The trunk region accounts for only 20%.[2] They can affect any tissue and may involve multiple tissue types or organs at the same time. They may not confine themselves to anatomical planes.[3]

Classification

Based on clinical behavior and endothelial characteristics, Mulliken and Glowacki proposed a classification for vascular anomalies in 1982.[4] The International Society for the Study of Vascular Anomalies (ISSVA) accepted this classification in 1992. It was updated at the ISSVA Workshop, Melbourne, in April 2014 and revised last in May 2018.[5] It still retains the general biological scheme of the classification and is now the most accepted one. It helps to avoid confusion in the naming of lesions; enabling clinicians across specialties to communicate, make coding, and generation of statistical data accurate.[6]

Vascular anomalies were divided into two groups:

  1. Vascular tumors (underlying endothelial hyperplasia)
  2. Vascular malformations (dysmorphogenesis and abnormal cellular turnover).


Vascular malformations are further divided into:

  1. Low flow (capillary, venous, lymphatic, and combined)
  2. High flow (arteriovenous malformations, arteriovenous fistula).


VMs are slow-flow vascular anomalies.[7] They are the most common low-flow vascular malformations, with a prevalence of 1% in the general population.[8]

Magnetic resonance imaging (MRI) is the imaging of choice for VMs. On T1-weighted images, slow-flow VMs are typically hypointense, and on T2-weighted images, they appear bright and show marked contrast enhancement. Large VMs also show venous lakes and phleboliths on T2 imaging, which help in the diagnosis.[9]

Sclerotherapy is the main modality of treatment for diffuse VMs that may involve multiple muscle groups. Absolute ethanol is among the most commonly used sclerosing agents. It causes inflammation and thus obliteration of the abnormal veins. Success rate being complete resolution in 95% of cases after multiple sessions taken over a period of 8 months.[10] However, in large, diffuse, multiloculated, and multiplanar lesions, sclerotherapy alone may be inadequate for complete resolution. In these cases, ablative surgeries become mandatory following failure of the interventional therapies, as was evident in our rare case.


  Case Report Top


A 16-year-old boy presented with a large soft tissue swelling in the right scapular area. This painless swelling was apparent to his mother since birth as a small swelling, which gradually increased in size to involve the entire right posterolateral chest wall displacing the scapula outward measuring 25 cm in the craniocaudal axis and 20 cm in the transverse axis [Figure 1].
Figure 1:(a) Preoperative lateral view. (b) Preoperative posterolateral view

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In 2006, at the age of 3½ years, he underwent a wide local excision of swelling in a private hospital [Figure 2]. However, the swelling reappeared after about 6 months of symptom-free postoperative period. He underwent multiple sittings of foam sclerotherapy as an outpatient department case before presenting to this center. MRI of the chest wall revealed multiplanar low-flow VMs involving the entire right chest wall, deep to the scapula, latissimus dorsi, and extending medially deep to the rhombhoid group of muscles. The lesion extends from the lateral border of the erector spinae muscles posteriorly to the lateral edge of the pectoralis major anteriorly with vascular communication from axilla, thoracic paravertrebral and intercostals plexuses with large venous lakes within and deep to latissimus dorsi muscle and the scapula [Figure 3].
Figure 2: X-ray showing soft tissue diffuse mass in the chest wall with minimal chest wall deformity at age 3

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Figure 3: (a and b) Preoperative magnetic resonance imaging chest wall

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The patient underwent exploration through the old posterolateral thoracotomy scar with both superior and inferior extension. The malformation was extensively involving the latissimus dorsi, trapezius, subscapularis, and rhomboid major and minor [Figure 4]. There were both intramuscular and intermuscular large venous lakes with communications with the axilla and vertebral and intercostals plexuses. A skin preserving wide local excision of the lesion was successfully carried out. The intraoperative bleed was minimized with the use of harmonic and vessel-sealing devices. However, the latissimus dorsi, rhomboid major/minor, and part of trapezius muscles were sacrificed as they were involved in the lesion [Figure 5].
Figure 4:Intraoperative, the lesion is seen deep to the scapula involving the latissimus dorsi muscle extending from the midline to lateral border of pectoralis major muscle

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Figure 5: The specimen which weighed 2 kg

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The postoperative period was uneventful. The skin flaps were healthy. There was complete resolution of the lesion. The range of movements of the right shoulder joint was full and free with no neurological deficits [Figure 6].
Figure 6:Postoperative status at suture removal with preservation of the full range of right shoulder joint

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The patient presented for follow-up at 6-month intervals with no evidence of recurrence at 1 year [Figure 7].
Figure 7: Follow-up at 1 year showing no recurrence and full range of movements at shoulder

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  Discussion Top


Sclerotherapy is a primary mode of treatment for vascular malformations. Multimodalilty therapy combining both interventional and surgical ablation is the treatment of choice in bigger lesions or in failure of interventional therapy. Surgical excision is tricky and challenging as there is loss of planes due to previous intervention, presence of large residual venous lakes, and multiplanar involvement, which often leads to subtotal excision. This leads to high chances of recurrence. Adequate exposure and meticulous dissection with knowledge of anatomy assisted with energy sources such as harmonic and vessel-sealing devices have made such extensive and complete ablation possible. This case is being highlighted as a rare case not only because of its large size and complex anatomical involvement but also because of the effective postoperative result, which has been possible only due to correct, cohesive steps taken cross specialties.

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.
Dubois J, Soulez G, Oliva VL, Berthiaume MJ, Lapierre C, Therasse E. Soft-tissue venous malformations in adult patients: Imaging and therapeutic issues. Radiographics 2001;21:1519-31.  Back to cited text no. 1
    
2.
Choi DJ, Alomari AI, Chaudry G, Orbach DB. Neurointerventional management of low-flow vascular malformations of the head and neck. Neuroimaging Clin N Am 2009;19:199-218.  Back to cited text no. 2
    
3.
McCafferty I. Management of low-flow vascular malformations: Clinical presentation, classification, patient selection, imaging and treatment. Cardiovasc Intervent Radiol 2015;38:1082-104.  Back to cited text no. 3
    
4.
Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: A classification based on endothelial characteristics. Plast Reconstr Surg 1982;69:412-22.  Back to cited text no. 4
    
5.
ISSVA Classification for Vascular Anomalies. Melbourne: 20th ISSVA Workshop; April 2014. Available from: http://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf. [Last updated on 2018 May 30].  Back to cited text no. 5
    
6.
Kapadia S, Thakore V, Patel H. Vascular malformations: An update on classification, clinical features, and management principles. Indian J Vasc Endovascular Surg 2017;4:152-62.  Back to cited text no. 6
    
7.
Mazoyer E, Enjolras O, Bisdorff A, Perdu J, Wassef M, Drouet L. Coagulation disorders in patients with venous malformation of the limbs and trunk: A case series of 118 patients. Arch Dermatol 2008;144:861-7.  Back to cited text no. 7
    
8.
Eifert S, Villavicencio JL, Kao TC, Taute BM, Rich NM. Prevalence of deep venous anomalies in congenital vascular malformations of venous predominance. J Vasc Surg 2000;31:462-71.  Back to cited text no. 8
    
9.
Walker EA, Fenton ME, Salesky JS, Murphey MD. Magnetic resonance imaging of benign soft tissue neoplasms in adults. Radiol Clin North Am 2011;49:1197-217, vi.  Back to cited text no. 9
    
10.
Lee BB, Do YS, Byun HS, Choo IW, Kim DI, Huh SH. Advanced management of venous malformation with ethanol sclerotherapy: Mid-term results. J Vasc Surg 2003;37:533-8.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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