Medical Case Reports

Keywords

Benign vascular lesions; Vascular malformation; Hemorrhage; Neoplasm

Introduction

Benign vascular lesions are abnormalities of blood vessel or endothelial cell proliferation. It was observed that benign oral vascular lesions represented 6.4% of all the diseases diagnosed by Oral Diagnosis Service [1,2]. Two most common types of vascular birthmarks are hemangiomas and vascular malformations which may appear to be very similar, but their course and treatment varies [3,4].

About 12% of newborns are thought to have a hemangioma, although most of them disappear during the first year of life instead vascular malformations are always present from birth, though they might not be apparent. Hemangiomas disappear with age but vascular malformations grow spontaneously with time. Hence, in general, most hemangiomas can be considered insignificant tumors that do not require any treatment except for aesthetic correction [4].

Based on the biological behaviors, VM can be subdivided into low flow and high flow lesions [5-7]. Oral vascular malformations are prevalent in the 6^th and 7^th decades of life. Histopathologically, it shows proliferation of endothelial cells with stasis of blood. Surgical excision followed by embolization is the treatment of choice for such lesions [8]. The commonest complication of such lesions is excessive bleeding during excision. Here we report a case of vascular malformation seen in upper lip of four and half year patient.

Case Report

A four and half year-old patient had reported to OPD with a chief complaint of swelling in the left upper lip. The patient’s mother gives history of swelling seen after 6 months of birth which gradually increased in size with time. On clinical examination the swelling was large, sessile, measuring about 4 × 4 cm. The swelling was soft in consistency with irregular surface. The overlying mucosa was normal. Enlarged vein was visible, no pulsation present. There was no history of pain and paresthesia (Figure 1). On gross examination the specimen was measuring about 3 × 3 cm, reddish brown in color, soft in consistency (Figure 2).

Figure 1: Pre-operative image showing swelling in upper lip.

Figure 2: Image showing gross specimen.

The histopathological feature showed connective tissue exhibiting multiple RBC’s filled with blood vessels of varying size and the nature ranging from small sized capillaries to medium sized arterioles which are interspersed with perinural arrangement of vascular channel suggestive of vascular malformation (Figures 3 and 4). The lesion was surgically excised (Figure 5) and patient was asked for follow up to check for recurrence.

Figure 3: Image showing multiple blood vessels and extravasated RBC ‘s (4x).

Figure 4: Image showing blood vessels in high power (10x).

Figure 5: Post-operative images after treatment.

Discussion

Benign vascular lesions are abnormalities of blood vessel or endothelial cell proliferation, it was observed that benign oral vascular lesions represented 6.4% of all the diseases diagnosed by Oral Diagnosis Service [2] Mulliken and Glowacki in 1982 proposed classification of vascular anomalies based on pathological features, i.e., endothelial cell turnover. According to this classification, vascular anomalies have been classified in to two categories - (a) Vasoproliferative neoplasm (b) Vascular malformations. Vascular malformation has less endothelial cell turnover (proliferates and undergo mitosis) compared to vasoprolifertive neoplasm. Instead VM are structural abnormalities of Venous, lymphatic, capillary and arterioles which grow according to the proportion of the child [9]. Enlargement of vascular lesions are due to changes in flow and pressure, dilatation of vascular channel, and collateral proliferation [8]. Active endothelial cells are the important feature in all series consistent with a progressing vascular lesion, but it is unclear whether the endothelial proliferation is a primary event or results from vascular expansion by means of hemodynamic mechanism [1]. Table 1 showing difference between haemangioma and vascular malformations.

Table 1: Differences between haemangioma and vascular malformations.

VM are subdivided into (a) Slow-flow (b) High-flow malformations. Slow flow VM has prevalence of 1% in overall general population. Most common type in these subtypes is Venous, Lymphatic and Venolymphatic malformations. Venous malformation is formed due to dilatation of superficial and deep veins due to thin wall which lacks smooth muscle. Lymphatic subtypes of malformation are caused due to collection of lymph vessels filled with serous fluid. Venolymphatic malformations are rare [9].

High flow VM is anterivenous malformation and anterivenus fistula. They are characterized by formation of cluster of arterial and Venous channels without formation of solid mass. The clinical presentations exhibit extremely considerable variety and can range from an asymptomatic birthmark to lifethreatening haemorrhage. These lesions commonly occur in the head and neck region with a predilection for the oral cavity, respiratory and muscle groups. The overall incidence of VM is about 1 in 10000 people. They may continue to grow throughout the patient’s life. Many patients with vascular malformations may be misdiagnosed as hemangiomas [8].

Conclusion

Oral VM are more frequent in the upper lip, buccal mucosa and lower lip, doesn’t show any gender predilection [2]. Vascular malformations can cause significant morbidity and even mortality in both children and adults. Few syndromes such as blue rubber bleb nevus syndrome, cutaneo-mucosal venous malformation (VMCM), glomu-venous malformation (GVM) are associated with the vascular lesions [6]. Investigation includes MRI, CT and Doppler US. Benign oral vascular lesions may be treated by sclerotherapy, systemic corticosteroids, interferon α, laser, embolization, cryotherapy, and surgery. Management and treatment decisions depend on the patient’s age, and on the lesion’s site and size. The first line of treatment of VM includes embolization. Complete surgical excision or combination therapy is also suggested in children’s [9].

References

1. Correa PH, Nunes LC, Johann AC, Aguiar MC, Gomez RS, et al. (2007) Prevalence of oral hemangioma, vascular malformation and varix in a Brazilian population. Braz Oral Res 21: 40-45.
2. Redondo P (2007) Vascular malformations (I). Concept, classification, pathogenesis and clinical features. Actas Dermosifiliogr 98: 141-158.
3. Foco F, Brkic A (2013) Vascular anomalies of the maxillofacial region: Diagnosis and management. Intechopen.
4. Bhat VS, Arror R, Bhandary S, Shetty S (2013) Traumatic anteriovenous malformation of cheek: A case report and review of literature. Int J Ortholaryangol Clin 5: 173-177.
5. Lowe LH, Marchant TC, Rivard DC, Scherbel AJ (2012) Vascular malformations: Classification and terminology the radiologist need to know. Semin Roentgenol 47: 106-117.
6. Lakkasetty YK, Malik S, Shetty A, Nakhaei K (2014) Multiple vascular malformations in head and neck – A rare case report. Journal of Oral and Maxillofacial Pathology. J Oral Maxillofac Patho 18: 137-142.
7. Barrett WA, Speight PM (2000) Superficial arteriovenous hemangioma of the oral cavity. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 90: 731-738.
8. Doifode D, Damle SG (2011) Vascular malformation of buccal mucosa: A case report. IJCD 2.
9. Qiam F, Khan M, Din MQ (2011) Mucosal venous hemangiomas of the oral cavity – An analysis of 43 cases and literature review. JKCD 1: 2.