|Year : 2016 | Volume
| Issue : 3 | Page : 300-304
Portwine stain with nodular thickening and intraoral hemangioma
Ankita Bohra1, Sumit Bhateja1, Geetika Arora2
1 Department of Oral Medicine and Radiology, Vyas Dental College and Hospital, Jodhpur, Rajasthan, India
2 Department of Public Health Dentistry, Vyas Dental College and Hospital, Jodhpur, Rajasthan, India
|Date of Submission||26-Jun-2015|
|Date of Acceptance||07-Dec-2016|
|Date of Web Publication||13-Dec-2016|
Dr. Ankita Bohra
Department of Oral Medicine and Radiology, Vyas Dental College and Hospital, Jodhpur, Rajasthan
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Vascular anomalies are a heterogeneous group of congenital blood vessel disorders more typically referred to as “birthmarks.” They are further categorized into vascular tumors and malformations, which are again subdivided into various types based on specific morphology, pathophysiology, clinical behavior and management of particular entity. Hemangiomas are the most common vascular tumors. Lymphatic, capillary, venous and arteriovenous malformations are the main subdivisions under the heading of vascular malformations. This paper discusses a case of co-occurrence of portwine stain with intraoral hemangioma.
Keywords: Hemangioma, portwine stain, vascular anomalies
|How to cite this article:|
Bohra A, Bhateja S, Arora G. Portwine stain with nodular thickening and intraoral hemangioma. J Indian Acad Oral Med Radiol 2016;28:300-4
|How to cite this URL:|
Bohra A, Bhateja S, Arora G. Portwine stain with nodular thickening and intraoral hemangioma. J Indian Acad Oral Med Radiol [serial online] 2016 [cited 2021 Apr 19];28:300-4. Available from: https://www.jiaomr.in/text.asp?2016/28/3/300/195649
| Introduction|| |
Vascular malformations are structural anomalies which involve the vascular plexus of the capillary, venous, lymphatic and arterial system that grows in proportion to the individuals growth. Capillary malformations include portwine stains, salmon stains and hereditary hemorrhagic telangiectasia. Portwine stain is defined as a macular telangiectasic patch present since birth and remains throughout life. They may be localized or extensive, affecting the whole limb., Hemangiomas are benign proliferative growth of the vascular endothelium. The Greek suffix “oma” means proliferation of tumor cells, and the term hemangioma means vascular anomalies with tumorous growth. Hemangiomas are usually present at or shortly after birth. Initial proliferative phase of 6- to 12-month period followed by stationary or plateau period. Period of regression starts by the age of 16–18 months and lasts for several years. Complete regression occurs in approximately 90% of the cases by the age of 9–11 years.
| Case Report|| |
A 30-year-old male patient reported to the Department of Oral Medicine and Radiology for a regular dental checkup. On extraoral examination, an asymptomatic erythematous macular patch was present unilaterally on the right side of the face with irregular margins. Superiorly it was extending from the right supraorbital margin descending downward involving the outer canthus of the eye, malar region, and limited inferiorly around 2 cm above the lower border of mandible. Superoinferiorly, it measured approximately 10 cm in dimension. Anteromedially, it passed along the midline and involved the inner canthus of the right eye, lateral surface of the nose, and limited at the right upper lip, whereas posterolaterally, it extended till the preauricular region approximately 2 cm short of tragus, measuring approximately 11 cm in anteroposterior dimension [Figure 1]. Nodular thickening was present on the lateral surface of the nose, which was not present earlier and was noticed by the patient since past 1 year. It gradually increased in size and was completely asymptomatic.
Facial asymmetry and macrochelia of the right upper lip was present [Figure 2]. Lesion was not involving the palatal region on the affected side. Patient reported that the lesion was present since birth. At initial stage, the size of the lesion was small which increased gradually along with the growth of the individual. Size of the lesion remained unchanged for approximately a decade and any color changes with respect to climate change were insignificant as per the information provided by the patient himself. No such lesions were seen elsewere in the body. No other relevant medical and dental history was noted. No history of central nervous system (CNS) disorder was reported by the patient. Anamnesis was noncontributory.
On intraoral examination, bright red-colored maxillary gingiva was noticed on the ipsilateral side adjoining the buccal mucosa [Figure 3]. Diascopy revealed blanching [Figure 4]. Hypervascular changes were unilateral, which is consistent with a trigeminal nerve distribution; in this case, following the second (maxillary) branch of the right trigeminal nerve. Differential diagnosis considered was the nevus of ota, which were ruled out due to their characteristic color difference. Lateral skull radiograph was advised [Figure 5] to rule out the possibility of intracranial calcification for any syndromic association. Any calcification was not appreciated in the radiograph. Thus, diagnosis of intraoral hemangioma with portwine stain on the right side of face was proposed.
| Discussion|| |
Vascular malformations are defects of vascular origin resulting in the formation of tortuous enlarged vascular channels. These arise as a result of abnormal signaling processes that control apoptosis, maturation, and growth of vascular cells during embryogenesis. These errors lead to the persistence of vascular plexus cells with a certain degree of differentiation. There are four major categories of vascular malformations based on their flow characteristics:
- Slow-flow – Capillary malformation, venous malformation, lymphatic malformation
- Fast-flow – Arteriole-venous malformation.
Commonly occurring capillary vascular malformations are salmon patches (nevus simplex), portwine stains (nevus flammeus), and strawberry naevi (infantile hemangiomas). Salmon patches, also known as “stork bite,” “angel's kiss,” or telangiectasic nevus, are the most common among all three. They are congenital macular lesions with a dull red color mostly involving the skin over the eyelids, bridge of the nose and nape of the neck, mostly fading during infancy. Strawberry naevi are soft raised vascular swellings on the skin, often with a bright red surface, that may have the appearance of a strawberry. They are also known as “infantile hemangiomas” and are present in approximately 5% of population worldwide. They usually appear after birth, often within the first month. Most strawberry naevi do not require treatment because they resolve spontaneously after few months.
Portwine stain, also called a fire mark, is a congenital pink or erythematous macular telangiectasic pigmentation that remains persistent throughout life. Portwine stains are named for their coloration, which is similar to a Portuguese fortified red wine. The most common locations are the head and neck, particularly the V1 and V2 dermatomes. The trunk and extremities are also frequently affected. The color change occurs due to increased hemoglobin content in the skin because of dilated capillaries and post-capillary venules in the affected regions. It occurs in both male and female newborns in approximately 1 in 20000 to 50000 live births. They may be localized or extensive, involving deeper vessels of dermis and subcutaneous tissues. It affects approximately 0.3–0.5% of newborns; the exact cause is unknown but portwine stains were shown to be caused by a somatic activating c. 548G → A mutation in the GNAQ gene. It has also been hypothesized to be a defect in embryological maturation of the sympathetic fibers, resulting in a loss of normal sympathetic control of the cutaneous vessels causing ectasia.
It commonly affects the face in the distribution of the trigeminal nerve. Initially, the lesions are pale pink; gradually, they evolve into a violaceous color and remain static or become nodular with progressing age. Portwine stains can develop thickening or nodules over time. Thickening is a further dilation of the ectatic blood vessels and nodules are vascular neoplasms or hyperplasias. Thickening often begins in early adulthood, however, its intensity and association with nodules continues to increase with age. Thickening and nodules were the most common in the area of the second and third branches of the trigeminal nerve innervating the face and were associated with a deepening color. The incidence of thickening alone was greater in male than in female patients. Later in life, portwine stains can become deeper red or purple in color and become raised or lumpy and more difficult to cover with makeup. The raised areas can bleed easily if they are scratched. Port wine stains of the eyelid area and upper jaw sometimes lead to increased pressure within the eye (glaucoma).
Histopathologically, portwine stains are composed of mature ectatic capillary channels in normal numbers within the superficial dermis surrounded by disorganized collagen. With progressive dilation, these blood vessels can be visualized in the deeper dermis and subcutaneous tissues. It is considered to be a hypodense zone of pericapillary neurons leading to reduced capillary tone and ectasia.
Portwine stains are associated with Sturge–Weber–Dimitri syndrome. A child born with a portwine stain on the face has approximately a 6% chance of having the Sturge– Weber syndrome More Details. It is characterized by portwine stain and leptomeningeal angiomas, which are diagnosed by the presence of gyriform “tramline” calcification on skull imaging studies, and are usually associated with ophthalmic manifestations, convulsive disorder, mental retardation, and contralateral hemiplegia. Because the patient progresses into adulthood, the stains tend to darken and thicken into a “cobblestone” appearance and can distort facial features, including the underlying bony structures. Klippel–Trenaunay syndrome is characterized by a portwine stain and angiomatosis of the extremities.
Diagnosis is usually made by clinical history and physical examination alone. In case of unclear diagnosis, a Doppler ultrasound/MRI may be performed. An MRI of the brain as well as an annual ophthalmological examination has been advised in case of suspicion for Sturge–Weber–Dimitri syndrome. Gradient echo T2-weighted imaging allows evaluation of high flow versus low flow lesions. Axial fat-saturated fats spin echo T1-weighted imaging is also useful in defining low-flow vascular anatomy and perfusion as well as to define the extent of the lesion.
The management of vascular malformations depends on the characteristics of the malformation, the functional impairment, disfigurement, the threat to life and on the expertise of the surgeon or the interventional radiologist. The therapeutic modalities such as sclerotherapy, embolization, laser photocoagulation, and surgical excision have been used. In sclerotherapy, direct injection of a sclerosing agent is given into the lesion. Sclerosing agents cause endothelial surface destruction and thrombosis, resulting into fibrosis and obliteration of the vessel lumen. Categorization of MRI findings in vascular malformations also provide information for the prediction of the probability of therapeutic success from sclerotherapy. Grade I lesions are well defined and less than 5 cm. Grade II lesions are either poorly defined or greater than 5 cm. Grade III lesions are poorly defined and greater than 5 cm. Lower grade lesions have better prognosis. Choice of the sclerosing agent depends on clinician's preference, dimensions and extent of the lesion, and draining veins within the lesions.
Laser treatment is effective only on surface lesions (size less than 3–4 mm). In case of presence of a lesion with a deep component such as lesions located in parotid gland, surgery is the preferred initial treatment. After the surgery when the lesion is well exposed, treatment with Nd:YAG laser can be performed. The pulsed dye laser (PDL; wavelengths = 585 or 595 nm) is the treatment of choice in many cases of portwine stains, and it is unusual for other treatment modalities to be utilized. It gives dramatic results if used early. Complications after laser therapy include hypopigmentation, hyperpigmentation, and scarring. Another nontraumatic treatment option is cosmetic camouflage. Today, different types of cosmetic products are available such as topical chemoexfoliation and collagen corticosteroids with hydroquinone bleaches. Cosmetic surgery can also be performed such as dermal abrasion and epilation. Depending on each patient and type and extent of lesion, treatment options are used. As soon as the lesion is diagnosed, treatment, if started, will give better results in younger age., In our case, the patient was educated regarding the pathology and benefits after taking treatment. He was referred to consult a dermatologist for further treatment and follow up after 2 months.
| Conclusion|| |
Individuals with vascular malformations should be treated with great care. Proper identification as well as multidisciplinary approach is required for treatment. Understanding the clinical options for interventions of each subtype of lesion will enable appropriate care and maximum results to be maximized. Being a dentist, any kind of such pathology present in the head and neck region should be thoroughly studied and diagnosed at an early stage so that it can be treated accordingly and has a good prognosis. Oral medicine specialists are supposed to be the first clinician to look after all these entities and educate patients for the associated risks of the lesion and to encourage them to start a treatment on a regular basis.
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
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]