Table of Contents  
Year : 2022  |  Volume : 9  |  Issue : 1  |  Page : 101-104

Giant venous malformation of the trunk: Complications and therapeutic difficulty

1 Department of Plastic and Reconstructive Surgery, Mohamed IV Hospital University; Research Laboratory in Medical Sciences, Faculty of Medicine and Pharmacy of Oujda, Mohammed I University, Oujda, Morocco
2 Research Laboratory in Medical Sciences, Faculty of Medicine and Pharmacy of Oujda, Mohammed I University; Department of Vascular Surgery, Mohamed IV Hospital University, Oujda, Morocco

Date of Submission15-Aug-2021
Date of Acceptance15-Nov-2021
Date of Web Publication23-Mar-2022

Correspondence Address:
Ghita Belmaati Cherkaoui
Department of Plastic and Reconstructive Surgery, Mohamed IV Hospital University; Research Laboratory in Medical Sciences, Faculty of Medicine and Pharmacy of Oujda, Mohammed I University, Oujda

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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijves.ijves_89_21

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Venous malformations (VMs) are dyssembryogenias of the venous vascular system. Giant VMs are rare but frequently associated with hemostasis disorders of variable severity. Their treatment is long and complex. The combination of several therapeutic modalities can reduce symptoms without eliminating the VM. A multidisciplinary approach is essential. A clinical case illustrates the difficulty of managing these malformations.

Keywords: Giant venous malformation, localized intravascular coagulopathy, multidisciplinary approach

How to cite this article:
Cherkaoui GB, El Adak H, Oufkir AA, Benzirar A, El Mahi O. Giant venous malformation of the trunk: Complications and therapeutic difficulty. Indian J Vasc Endovasc Surg 2022;9:101-4

How to cite this URL:
Cherkaoui GB, El Adak H, Oufkir AA, Benzirar A, El Mahi O. Giant venous malformation of the trunk: Complications and therapeutic difficulty. Indian J Vasc Endovasc Surg [serial online] 2022 [cited 2022 Jul 1];9:101-4. Available from:

  Introduction Top

Venous malformations (VMs), according to the International Society for the Study of Vascular Anomalies classification, are slow-flow vascular anomalies consisting of dysplastic veins with walls deficient in smooth muscle cells,[1] located in any tissue.[2] The term giant VM is not clearly defined in the literature; in fact, it depends on its location and the degree of extension to deep structures, and it is only specified for hepatic cavernous hemangiomas when they are larger than 10 cm.[3] Coagulation disorders of varying severity, including localized intravascular coagulopathy (LIC), are frequently associated with limb and trunk VM particularly.[4] The treatment of giant VM remains a real challenge and involves a combination of therapeutic modalities for a sometimes disappointing result. We report a case of giant trunk VM complicated with LIC benefiting from both sclerotherapy and surgical treatment, which illustrates the difficulty of management.

  Case Report Top

A 24-year-old patient from a nonconsanguineous marriage, with no medical history, presented for management of a painful giant VM of the trunk, without a hemorrhagic syndrome. On examination, four enormous confluent masses occupied the back and flanks, and one less prominent mass was present in the right anterior abdominal wall.

  • The first: Left paravertebral measuring 13 cm × 17 cm occupying the lower scapular region
  • The second: Left paravertebral measuring 12 cm × 15 cm occupying the lumbar region
  • The third: Right paravertebral measuring 25 cm × 19 cm circumferential occupying the upper lumbar region
  • The fourth: Right paravertebral measuring 7 cm × 10 cm circumferential occupying the lower lumbar region
  • The fifth: At the level of the right anterior abdominal wall measuring 9 cm × 9 cm and located 4 cm from the iliac crest [Figure 1].
Figure 1: Preoperative photograph. Giant trunk VM, posterior view (a) ¾ right view (b)

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Angio-magnetic resonance imaging (MRI) showed the deep extension of the dorsal masses which invaded the entire muscle wall and even extended retroperitoneally on the left [Figure 2].
Figure 2: MRI angiography image in axial (a-c) and sagittal section (d) showing the invasion of the muscle wall and the retroperitoneal extension

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The biological evaluation revealed a LIC with a D-dimer level of 29.46 mg FEU/L (60 × the normal) and a low fibrinogen level of 0.9 g/l.

The strategy decided in the pluridisciplinary vascular anomalies meeting was (in view of the unavailability of sirolimus) to try sclerosis first, starting with the least extensive VM (anterior abdominal wall). Two sessions of sclerosis with Aetoxisclérol 2% were performed without any improvement. Thus, surgical treatment was proposed for the anterior and the right inferior VM of the back (the most superficial of the back VMs).

Preoperatively, heparin therapy with low-molecular weight heparin (LMWH) was started at a dose of 100 IU anti-Xa/Kg/d and continued until the D-dimer level decreased (becoming 10 × normal).

The surgical procedure took 6 h and consisted of a complete removal of the right dorsal VM creating a 12/7 cm skin defect. For the anterior abdominal location, a squeezing of the residual intramuscular VM (rectus abdominis muscle) completed the removal. The patient was transfused per operatively.

A minimal bleeding managed by compression occurred on the hours following surgery.

The defect was secondarily grafted with a split skin graft [Figure 3].
Figure 3: Intraoperative view of a complete excision of a back VM (a) Secondary skin graft (b)

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The remaining VMs are inaccessible to surgical treatment and the patient continues to wear a compression garment while awaiting sirolimus treatment [Figure 4].
Figure 4: 16 months postoperative: aggravation of the third VM, no recurrence of the surgically treated VM

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  Discussion Top

VMs are the most common of the vascular malformations: up to 1% of the population are affected.[5] Trunk location is not the most common (20%);[6] giant trunk VM is even more rare. Their diagnosis is easy, based on clinical findings. Further investigations confirm the diagnosis and look for complications, including LIC.

Standard radiography may show phleboliths. Doppler ultrasound confirms the diagnosis by showing venous lacerations with low or no flow and phleboliths. The computed tomography (CT) scan studies the deep extension of the VM and evaluates the bone changes. The MRI is the best imaging modality to assess the deep extension to the soft tissues and for follow-up.[7]

At the end of this evaluation, the severity of the VM can be assessed using the 2002 severity score[8] based on both the clinically visible locations and the tissue extension visible on imaging. Scored for 10 items, it allows a relatively accurate assessment of the superficial and deep extension of the vascular malformation and gives an idea of its evolution, more the score is high, more the evolution of the VM is problematic.[4] The score of our patient is 5.

LIC may be present in 40% of patients with large and extensive VMs with a risk of transition to disseminated intravascular coagulation (DIC), especially in the case of treatment.[9]

LIC is usually asymptomatic. However, it can cause swelling and pain due to thrombosis or bleeding within the malformation. Over time, intralesional thrombi develop into phleboliths that may be palpable.[10],[11]

It is therefore recommended, according to the “good practice guide” proposed by the International Union of Phlebology, to perform D-dimers as well as fibrinogen in the initial evaluation of the management of extensive extratruncal VMs as well as a more complete analysis (blood count, hemoglobin, platelets, D-dimer, fibrinogen, fibrin degradation product, and PT/ACT) during the follow-up of patients with extensive VMs or at risk.[12]

In the case of confirmed coagulation anomalies such as LIC, or in the case of extensive infiltrating and painful VMs, treatment with LMWH at a dosage of 100 IU/kg/d during 20 days is recommended.[12] A blood test for D-dimer and anti-Xa activity is performed 10 days after the beginning of the treatment to check its effectiveness and adjust the dosage if necessary. This treatment is indicated in preparation for a therapeutic procedure to prevent postoperative DIC.

The therapeutic strategy for VM is discussed at the multidisciplinary team meeting and is only started for symptomatic VM with esthetic, functional, or psychological repercussions, and if the proposed treatments respond to the patient's expectations.

Classically, surgery was the first-line treatment. All reconstructive surgical techniques (elliptical resection, local flaps, skin expansion, skin grafting, etc.) can be applied.[13],[14] All these methods quickly reach their limits in giant VMs.

Other therapeutic modalities include sclerotherapy, which consists of the percutaneous injection into the VM of a sclerosant: Aetoxisclérol® (polidocanol) 3% and Ethibloc® (absolute alcohol) 95%, either curative as a stand-alone procedure or preoperative with the aim of creating a better defined thrombosed mass, easier to excise surgically. In general, the effectiveness of sclerotherapy decreases in proportion to the size of the VM.[15],[16]

Sirolimus is an inhibitor of the mammalian target of rapamycin protein that is located downstream of the phosphatidylinositol 3 kinase/AKT pathway and is best known for its immunosuppressive effect. It has been used off-label for VMs, for which approved therapies were associated with unsatisfactory results or recurrence.

A review of 68 articles describing 324 patients with vascular malformations showed the efficacy of sirolimus in reducing the size of VMs (67% of patients receiving treatment for a mean of 12 months) and in resolving symptoms. Side effects, although common (53%), are generally mild (mucositis is the most common complication). On the other hand, a recurrence of symptoms was noted in 49% of patients after stopping treatment.[17]

Our patient was very demanding for a treatment of his VMs given the major functional (pain) and aesthetic discomfort. In spite of his motivation, the heaviness of the surgical treatment (prolonged anticoagulation, heavy and long procedure, bleeding complication, and prolonged hospitalization) for a limited result on only 1/5 of the volume of his VMs makes him lean more toward a medical treatment and he is waiting for a sirolimus treatment to be started.

  Conclusion Top

Giant VMs are delicate malformations, which are frequently accompanied by coagulopathy that must be systematically researched. Their management is difficult. A multidisciplinary approach is essential to obtain the best result with a minimum of complications.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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Di Carlo I, Koshy R, Al Mudares S, Ardiri A, Bertino G, Toro A. Giant cavernous liver hemangiomas: Is it the time to change the size categories? Hepatobiliary Pancreat Dis Int 2016;15:21-9.  Back to cited text no. 3
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. 4
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Mazoyer E, Enjolras O, Laurian C, Houdart E, Drouet L. Coagulation abnormalities associated with extensive venous malformations of the limbs: Differentiation from Kasabach-Merritt syndrome. Clin Lab Haematol 2002;24:243-51.  Back to cited text no. 8
Dompmartin A, Acher A, Thibon P, Tourbach S, Hermans C, Deneys V, et al. Association of localized intravascular coagulopathy with venous malformations. Arch Dermatol 2008;144:873-7.  Back to cited text no. 9
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Lee BB, Baumgartner I, Berlien P, Bianchini G, Burrows P, Gloviczki P, et al. Guideline. Diagnosis and treatment of venous malformations. Consensus document of the International Union of Phlebology (IUP): Updated-2013. Int Angiol 2014 Jun 10. Online ahead of print.  Back to cited text no. 12
Boon LM, Vanwijck R. Traitement médical et chirurgical des malformations veineuses. Ann Chir Plast Esthét 2006;51:403-11.  Back to cited text no. 13
Hein KD, Mulliken JB, Kozakewich HP, Upton J, Burrows PE. Venous malformations of skeletal muscle. Plast Reconstr Surg 2002;110:1625-35.  Back to cited text no. 14
Colletti G, Ierardi AM. Understanding venous malformations of the head and neck: A comprehensive insight. Med Oncol 2017;34:42.  Back to cited text no. 15
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Geeurickx M, Labarque V. A narrative review of the role of sirolimus in the treatment of congenital vascular malformations. J Vasc Surg Venous Lymphat Disord 2021;9:1321-33.  Back to cited text no. 17


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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