Archives of Medicine and Health Sciences 2019 7(2):258-261
Vascular anomalies account as one among the most difficult diagnostic and therapeutic enigmas that can be encountered in the practice of medicine. These lesions are the result of an embryonic abnormality of the vascular system. The main characteristic feature of vascular malformations is that they never show the signs of involution. It is the most common neoplasm of the infancy. The clinical presentations are extremely protean and can range from an asymptomatic birthmark to life-threatening hemorrhage. Here, we present a case of vascular malformation in the upper lip of a 4-year-old patient.
| Introduction |  |
Most common congenital and neonatal abnormalities encountered in childhood are vascular abnormalities. Various terms have been used to describe this lesion such as arteriovenous aneurysm, cavernous hemangioma, cental hemangioma, pulsatile hemangioma, angioma, atriovenous shunt, and arteriovenous shunt.[1] Early to the 1980s, all vascular lesions were referred as hemangiomas. However, the works of Mulliken and Gloack have immensely broadened our understanding about vascular lesions; subsequently, vascular lesions have been divided into hemangiomas and vascular malformations. Benign vascular lesions are abnormalities of the blood vessel or endothelial cell proliferation. It represents 6.4% of all the diseases diagnosed by the Oral Diagnosis Service.[2] The two most common types of vascular birthmarks are hemangiomas and vascular malformations which may appear clinically to be very similar, but their course and treatment varies.[3]
Based on the biological behavior, vascular hemangiomas can be subdivided into low-flow and high flow lesions.[4] Oral vascular malformations are prevalent in the sixth and seventh decades of life. Histopathologically, it shows the proliferation of endothelial cells with stasis of blood. Surgical excision followed by embolization is the treatment of choice.[5] The most common complication of such lesions is excessive bleeding during excision. Here, we report a case of vascular malformation seen in the upper lip of a 4-year-old patient.
| Case Report | |
A 4-year-old patient reported to the outpatient department with a chief complaint of swelling in the left upper lip. The patient's mother gives a history of swelling seen after 6 months of birth which gradually increased in size with time. On clinical examination, there was a large swelling, sessile, measuring about 4 cm × 4 cm. The swelling was soft in consistency with irregular surface. The overlying mucosa was normal. The enlarged vein was visible, but no pulsation was present. There was no history of pain and paresthesia [Figure 1]. A clinical provisional diagnosis of hemangioma was given based on the above findings.
 | Figure 1: Preoperative image of the patient showing swelling in the upper lip Click here to view |
On gross examination, the specimen was measuring about 3 cm × 3 cm, reddish-brown in color, and soft in consistency [Figure 2].
 | Figure 2: Gross specimen which is reddish-brown in color Click here to view |
The histopathological feature showed connective tissue exhibiting multiple RBCs filled with the blood vessels of varying size and the nature ranging from small-sized capillaries to medium-sized arterioles which are interspersed with perineural arrangement of vascular channel suggestive of vascular malformation [Figure 3] and [Figure 4]. The lesion was surgically excised, and the patient was asked for follow-up to check for recurrence [Figure 5].
 | Figure 3: Histopathology image showing small sized blood filled capillaries Click here to view |
 | Figure 4: Histopathology image (×40) showing blood capillaries Click here to view |
 | Figure 5: Postoperative image Click here to view |
| Discussion | |
Benign vascular lesions are abnormalities of the blood vessel or endothelial cell proliferation. The diagnosis and classification of these malformations plays a paramount role in their treatment plan [Table 1] and [Table 2]. It was observed that benign oral vascular lesions represented 6.4% of all the diseases diagnosed by the Oral Diagnosis Service.[2] Mulliken and Glowacki proposed the classification of vascular anomalies based on the pathological features, i.e., endothelial cell turnover. According to this classification, vascular anomalies have been classified into two categories – (a) vasoproliferative neoplasm and (b) vascular malformations. Vascular malformation has less endothelial cell turnover (proliferates and undergo mitosis) compared to vasoprolifertive neoplasm. Instead, Vascular malformations (VMs) are structural abnormalities of venous, lymphatic, capillary, and arterioles which grow according to the proportion of the child.[6] The enlargement of vascular lesions is due to the changes in flow and pressure, dilatation of vascular channel, and collateral proliferation.[5] 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 3] shows the differences between hemangioma and vascular malformations.
 | Table 1: Simplified diagnostic approach to a congenital vascular lesion Click here to view |
 | Table 2: Current classification of hemangioma and vascular malformat Click here to view |
 | Table 3: Difference between hemangioma and vascular malformations Click here to view |
The hemangioma represents as a true vascular tumor resulting from a neoplastic overgrowth of normal vascular tissue. The hemangioma develops and grows due to the endothelial proliferation. In contrast with hemangiomas, vascular malformations result from abnormal vascular or lymphatic vessel morphogenesis and are not because of abnormal endothelial growth. Hemangiomas are usually present at birth and can be diagnosed by 1 year, whereas vascular malformations are present at birth but often not diagnosed until at a later stage of life (usually second decade). Hemangiomas show a rapid growth until 10 months and involute by the 10th year of life. Vascular malformations are indolent in nature and persist throughout life with an increase in response to infection, trauma, or hormonal fluctuation and they do not involute.[5]
VM are subdivided into (a) slow-flow and (b) high-flow malformations. Slow-flow VM has a prevalence of 1% in overall general population. The most common type in these subtypes is venous, lymphatic, and venolymphatic malformations. Venous malformation is formed due to the dilatation of superficial and deep veins due to the thin wall which lacks smooth muscle. Lymphatic subtypes of malformation are caused due to the collection of lymph vessels filled with serous fluid. Venolymphatic malformations are rare.[6]
High-flow VM is anterivenous malformation and anterivenus fistula. They are characterized by the formation of a cluster of arterial and venous channels without the formation of solid mass. The clinical presentations exhibit extremely considerable variety and can range from an asymptomatic birthmark to life-threatening hemorrhage. 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 10,000 people. They may continue to grow throughout the patient's life. Many patients with vascular malformations may be misdiagnosed as hemangiomas.[5] Oral VMs are more frequent in the upper lip, buccal mucosa, and lower lip and do not 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, cutaneomucosal venous malformation, and glomuvenous malformation are associated with the vascular lesions.[7] The investigation includes magnetic resonance imaging, computed tomography, and Doppler ultrasound. 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.
Various treatment modalities include surgical excision with blade or laser, cryosurgery, injection of corticosteroids or sclerosant (sodium tetradecyl), radiotherapy, and embolization with steel coil, gel foam, silicone beads, or cyanoacrylate. Whether they should be followed up or treated depends on the patient's age and site and size of the lesion. The above-mentioned modalities have notable disadvantages including excessive fibrosis, scarring, bleeding, cosmetic, and functional deficiency because of the long-term effect of steroid and radiation. Moreover, surgical excision and cryosurgery are effective for small lesions and superficial ones.
| Conclusion | |
Vascular lesions are of two types, hemangioma and vascular malformation. Hemangiomas involute as age advances, but vascular malformation persists for life long. The treatment of choice for both the entities is the surgical removal. Bleeding is the most common complication, and recurrence is rare.
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 | |
| 1. | Garzon MC, Enjolras O, Frieden IJ. Vascular tumors and vascular malformations: Evidence for an association. J Am Acad Dermatol 2000;42:275-9.  |
| 2. | Redondo P. Vascular malformations (I). Concept, classification, pathogenesis and clinical features. Actas Dermosifiliogr 2007;98:141-58. |
| 3. | Bhat VS, Aroor R, Bhandary BSK, Shety S. Traumatic anteriovenous malformation of cheek: A case report and review of literature. Int J Ortholaryangol Clin 2013;5:173-7. |
| 4. | Lowe LH, Marchant TC, Rivard DC, Scherbel AJ. Vascular malformations: Classification and terminology the radiologist needs to know. Semin Roentgenol 2012;47:106-17. |
| 5. | Doifode D, Damle SG. Vascular malformation of buccal mucosa: A case report. IJCD 2011;2:28-30. |
| 6. | Qiam F, Khan M, Ud Din Q. Mucosal venous hemangiomas of the oral cavity – An analysis of 43 cases and literature review. JKCD 2011;2:1-5. |
| 7. | Ethunandan M, Mellor TK. Haemangiomas and vascular malformations of the maxillofacial region – A review. Br J Oral Maxillofac Surg 2006;44:263-72. |
| Figures |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
| Tables |
[Table 1], [Table 2], [Table 3]
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