Table of Contents  
ORIGINAL ARTICLE
Year : 2019  |  Volume : 6  |  Issue : 1  |  Page : 10-12

Vascular anomalies: Presentation and response to medical and surgical management, our experiences in the last 5 years


Department of Surgery (CTVS), Dhulikhel Hospital, Kathmandu University Hospital, Dhulikhel, Nepal

Date of Web Publication8-Mar-2019

Correspondence Address:
Dr. Robin Man Karmacharya
Department of Surgery (CTVS), Dhulikhel Hospital, Kathmandu University Hospital, Dhulikhel
Nepal
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_28_18

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  Abstract 


Aim: Vascular anomalies, lesions of abnormal vascular development pose a significant challenge in some of the cases for diagnosis and management. The International Society for the Study of Vascular Anomalies has classified this condition into two broad types as vasoproliferative or vascular neoplasms such as hemangioma and vascular malformation. We have analyzed all the cases with vascular anomalies that have presented in outpatient department (OPD) in the past 5 years. Materials and Methods: All patients presented to vascular OPD of Dhulikhel Hospital in the year 2013–2017, if patients are diagnosed with vascular anomalies, are included in the study. Details on age, sex, site of the lesion, and medical or operative management performed are analyzed. Results: Of the total 51 vascular anomalies cases, the mean age of the patient was 22.4 years (standard division. 8.4, range 6 months to 51 years). Female-to-male ratio was 2.4:1. Forty cases (70.48%) belonged to vascular malformation while 11 cases (21.56%) were of hemangioma. In case of vascular malformation, 18 cases were of venous malformation while 8 cases were of capillary malformation. There were a total of 14 arteriovenous malformations. The highest number of anomalies was noticed in the lower limb (39.22%) followed by the head (25.49%). Regarding treatment modality opted, in five cases, medical management with oral steroids and propranolol was advised. In 35 cases, surgical resection was done. Conclusion: Vascular anomalies are detected in fairly young patients with preponderance in the female. Treatment of vascular anomalies is complex and is based on stage, type, and location of each anomaly.

Keywords: Hemangioma, vascular anomalies, vascular malformation


How to cite this article:
Karmacharya RM, Shrestha BK, Shrestha B, Devbhandari M, Tuladhar SM. Vascular anomalies: Presentation and response to medical and surgical management, our experiences in the last 5 years. Indian J Vasc Endovasc Surg 2019;6:10-2

How to cite this URL:
Karmacharya RM, Shrestha BK, Shrestha B, Devbhandari M, Tuladhar SM. Vascular anomalies: Presentation and response to medical and surgical management, our experiences in the last 5 years. Indian J Vasc Endovasc Surg [serial online] 2019 [cited 2019 Mar 21];6:10-2. Available from: http://www.indjvascsurg.org/text.asp?2019/6/1/10/253733




  Introduction Top


Vascular anomalies are lesions of abnormal vascular development and still pose a significant challenge in some of the cases for diagnosis and management. Various terminologies have been used for this condition, but guidelines by the International Society for the Study of Vascular Anomalies (ISSVA) has classified this condition into two broad types as vasoproliferative or vascular neoplasms such as hemangioma and vascular malformation.[1]

Notable pioneer study had been done by Mulliken and Glowacki in 1982, and this condition was described in detail based on clinical and biological nature.[2]

Hemangioma is a vascular tumor which grows rapidly in infancy and can involute later in some cases. Histopathologically, there is cellular hyperplasia. Hemangioma is classified into two different types as involuting and noninvoluting type. In 90% of the patients, there is a regression of hemangioma and termed as involuting type while in rest, it continues to grow and fall into noninvoluting type.[3]

In contrast, vascular malformations are disorder that happens during embryogenesis whereby there is the persistence of vascular cluster lined by single endothelial cells.[4] It is divided into two types as slow flow (venous malformation, capillary malformation, and lymphatic malformations) and high flow (arteriovenous malformation) malformation based on the velocity of flow in the lesion. Rarely, in some syndromes like Klippel-Trenaunay Syndrome, these different vascular malformations can coexist making complex vascular malformations.[5]

We have analyzed all the cases with vascular anomalies that have presented in outpatient department (OPD) in the last 5 years.


  Materials and Methods Top


All patients presented to vascular OPD of Dhulikhel Hospital in the year 2013–2017, if patients are diagnosed with vascular anomalies, are included in the study. The variables studied are age, sex, site of the lesion, and medical or operative management performed. Diagnosis of vascular anomaly is confirmed if the patient presents with typical pigmentation with lesion depicting vessel in the screening Doppler. If the lesions are extensive, magnetic resonance imaging is also performed. In some cases, computed tomography angiogram is also done with venous/arterial phase where relevant.


  Results Top


A total of 51 cases of vascular anomalies identified during the study. Mean age of the patient was 22.4 years (standard deviation. 8.4, range 6 months to 51 years). Of them, 36 (70.59%) were female and rest 15 (29.41%) were male with female-to-male ratio of 2.4:1. Of the cases, 40 cases (70.48%) belonged to vascular malformation while 11 cases (21.56%) were of hemangioma. Different subtypes of hemangioma and vascular malformation are mentioned in [Table 1]. Three cases in hemangioma were of involuting type while 8 cases were of noninvoluting type. In case of vascular malformation, 18 cases were of venous malformation while 8 cases were of capillary malformation. There were a total of 14 arteriovenous malformation.
Table 1: Number of vascular anomalies in cases presenting at outpatient department

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In relation to the location of vascular anomalies, we divided the anomalies into location as head, upper limb, chest, abdomen-pelvis, and lower limb. The highest number of anomalies was noticed in the lower limb (39.22%) followed by the head (25.49%) as shown in [Figure 1].
Figure 1: Number of vascular anomalies in different location

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Regarding treatment, modality opted, in five cases, medical management with oral steroids and propranolol was advised. Some improvements were noticed in these cases. Most of the cases were young children and in cases where surgical treatment is extremely difficult. In 35 cases, surgical resection was done (7 of them required more than one setting of surgery). During surgery, in selected cases, intralesional steroid injection and/or sclerotherapy were also done, especially in low-flow vascular anomalies. In remaining, 11 cases only intralesional steroid injection and/or sclerotherapy were done. Two of the cases did not improve with this treatment and decided to consult in another hospital.

Examples of some of the cases are shown in [Figure 2], [Figure 3], [Figure 4].
Figure 2: Venous malformation in the right lower limb involving persistent embryonic lateral marginal vein of leg

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Figure 3: Arteriovenous malformation in the right palm

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Figure 4: Venous malformation in right lower limb treated by staged surgery with good results

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  Discussion Top


Studies to see epidemiology of vascular anomalies is scarce. In a study involving 3573 children, it was found that venous anomalies existed in 1.2% of children. In the cases of vascular anomalies, arteriovenous malformation was the most common with 37% and hemangioma was 35%.[6]

In our study, of the cases, venous malformation was the most common followed by arteriovenous malformation. Regarding the progression of arteriovenous malformation, Schobinger has classified into four systems as a Quiescent stage (Stage I), Progressive stage (Stage II), Destructive stage with pain, bleeding, and ulceration (Stage III), and Congestive cardiac failure stage (Stage IV).[7] In our case, none of the cases were in Stage IV.

About the management of vascular anomalies, it depends on the type, size, site, and stage of malformation. In slow flow malformation, sclerotherapy is one modality of treatment. Various agents are available, but foam sclerotherapy agents such as sodium tetradecyl sulfate and polidocanol have good results with lesser side effects. Agents such as absolute alcohol have been found to damage nerves, and also induce systemic toxicity in some cases.[8],[9]

Sclerotherapy has significant risk in high flow malformation, owing to washout of the sclerosing agent by the flow. Newer endovascular treatment modalities like embolization have good results but are of limited practice in our setup owing to extensive costs associated with the procedure until date.[10],[11],[12] Surgical treatment possible is excisional biopsy if the lesions are easy and safe to excise. In some cases, feeder ligation and staged surgery are better.


  Conclusion Top


ISSVA does make uniformity among the wide array of anomalies. Decision-making of treatment modality is complex and is based on stage, type, and location of each anomaly. Regarding surgical treatment, staged treatment is also required in some selected cases. Sclerotherapy is an endovascular treatment modality which is possible in some slow flow anomalies. Newer but expensive modalities like coiling are yet to be a regular management modality in our setup.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Enjolras O. Classification and management of the various superficial vascular anomalies: Hemangiomas and vascular malformations. J Dermatol 1997;24:701-10.  Back to cited text no. 1
    
2.
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. 2
    
3.
Chiller KG, Passaro D, Frieden IJ. Hemangiomas of infancy: Clinical characteristics, morphologic subtypes, and their relationship to race, ethnicity, and sex. Arch Dermatol 2002;138:1567-76.  Back to cited text no. 3
    
4.
Cohen MM Jr. Vascular update: Morphogenesis, tumors, malformations, and molecular dimensions. Am J Med Genet A 2006;140:2013-38.  Back to cited text no. 4
    
5.
Servelle M. Klippel and trénaunay's syndrome 768 operated cases. Ann Surg 1985;201:365-73.  Back to cited text no. 5
    
6.
Tasnadi G. Epidemiology of vascular malformation. Hemangiomas and Vascular Malformations. Springer-Verlag Italia 2009. p. 109-10.  Back to cited text no. 6
    
7.
Kohout MP, Hansen M, Pribaz JJ, Mulliken JB. Arteriovenous malformations of the head and neck: Natural history and management. Plast Reconstr Surg 1998;102:643-54.  Back to cited text no. 7
    
8.
Berenguer B, Burrows PE, Zurakowski D, Mulliken JB. Sclerotherapy of craniofacial venous malformations: Complications and results. Plast Reconstr Surg 1999;104:1-11.  Back to cited text no. 8
    
9.
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. 9
    
10.
Mulligan PR, Prajapati HJ, Martin LG, Patel TH. Vascular anomalies: Classification, imaging characteristics and implications for interventional radiology treatment approaches. Br J Radiol 2014;87:20130392.  Back to cited text no. 10
    
11.
Lee BB, Lardeo J, Neville R. Arterio-venous malformation: How much do we know? Phlebology 2009;24:193-200.  Back to cited text no. 11
    
12.
Do YS, Kim YW, Park KB, Kim DI, Park HS, Cho SK, et al. Endovascular treatment combined with emboloscleorotherapy for pelvic arteriovenous malformations. J Vasc Surg 2012;55:465-71.  Back to cited text no. 12
    


    Figures

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

  [Table 1]



 

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