|Year : 2015 | Volume
| Issue : 2 | Page : 81-85
Dental considerations of capillary malformation
Deepa Jatti Patil, Ajay Singh Kataria, Gagan Puri, Aravinda Konidena
Department of Oral Medicine, Diagnosis and Radiology, Swami Devi Dayal Hospital and Dental College, Golpura, Panchkula, Haryana, India
|Date of Web Publication||28-Jan-2016|
Deepa Jatti Patil
Department of Oral Medicine, Diagnosis and Radiology, Swami Devi Dayal Hospital and Dental College, Golpura, Panchkula - 125 121, Haryana
Source of Support: None, Conflict of Interest: None
Hemangiomas and vascular malformations (VMs) are the two types of vascular lesions. VMs are further subdivided according to the type of involved vessels present as arterial, arterio-venous, venous, and capillary or lymphatic malformations. The capillary malformation (CM) previously was considered to be a hemangioma and was known as port wine stain, and hereby presenting a case report on the orodental manifestations of CM.
Keywords: Capillary malformation; diascopy; vascular lesions
|How to cite this article:|
Patil DJ, Kataria AS, Puri G, Konidena A. Dental considerations of capillary malformation. Indian J Multidiscip Dent 2015;5:81-5
| Introduction|| |
Vascular lesions are among the most common congenital and neonatal abnormalities. An understanding of vascular lesions has been greatly facilitated by the work of Mulliken and Glowacki. He worked exhaustively and formulated a biological classification of vascular anomalies based on physical findings, clinical behavior, and cellular kinetics. According to their classification, there are two types of vascular lesions: Hemangiomas and vascular malformations (VMs). Two main characteristics distinguish these two types. While hemangiomas are not usually present at birth, they are apparent within the first-week of life; in contrast, VMs are present at birth but may not be clinically evident. Furthermore, hemangiomas show rapid proliferation during the first 2 years of life, followed by a slow involution. In contrast to these characteristics, VMs show proportionate growth in relation to body volume and show no signs of spontaneous involution.
Mulliken and Glowacki have further classified the different types of VMs according to the type of involved vessels present as arterial, arterio-venous, venous, and capillary or lymphatic malformations. These occur equally in females and males. By definition, all VMs are present at birth. Capillary malformations (CM) are usually present at birth. Lymphatic malformations are often also recognized at birth and will become noticeable in 80% of children during the first year of life. In contrast to this, venous malformations appear at any time from birth to early adulthood. Arterial and arterio-venous malformations often become evident at times of hormonal changes such as puberty or pregnancy. In comparison to hemangiomas, VMs do not proliferate but grow proportionately with the infant throughout the course of the individual's life. While hemangiomas expand by hyperplasia, VMs enlarge by hypertrophy. Progressive ectasia of existing vascular structures caused by sepsis, trauma, and pregnancy or puberty results in the expansion of VMs. The present case is about CM in a 45-year-old male patient that caused esthetic disfigurement to the patient.
| Case Report|| |
A 45-year-old male patient reported to the Department of Oral Medicine and Radiology with a chief complaint of a reddish discoloration on the outer and inner side of cheeks. This discoloration was present since childhood and has increased in size and color intensity eventually over a period of few years. The lesion was asymptomatic except for the esthetic disfigurement. He also stated that the color of the lesion deepens during winter. He also complained of bleeding from the gingiva on slightest provocation. His medical history was insignificant for any epileptic episodes. No significant occlusal manifestations were elicited. His mental IQ was within normal limits. Examination of trigeminal and facial nerve revealed no focal deficit.
Extraoral examination revealed two reddish pink well-defined macules irregular shaped one on the right malar region extending along the inner canthus of eye and other on the pretragus area measuring approximately 2 cm × 2 cm [Figure 1] and[Figure 2]. The surface texture is pebbly, and no visible pulsations were seen. On palpation, the macule was nontender, nonpulsatile and diascopy test was positive that is, the lesion blanched on pressure.
On intraoral examination, three irregular well-defined macules were seen on the right buccal vestibule, right-mid buccal mucosa, and right side of the hard palate [Figure 3]. Diascopy test was positive and lesion was nonpulsatile. The patient had good oral hygiene with mild stains and calculus. Remarkable bleeding on probing was present which lasted around 10–12 min and regressed on its own. A provisional diagnosis of CM/port wine stain (PWS) involving the maxillary and mandibular branch of trigeminal nerve was given. Capillary hemangioma, arterio-venous malformation, Sturge– Weber syndrome More Details, and ecchymosis were considered under the differential diagnosis. Complete blood count with prothrombin time, international normalized ratio was advised, and all values were within the normal limits. Postero-anterior view of skull and lateral cephalogram did not reveal any evidence of tramline calcifications.
Based on the clinical features and investigations, a final diagnosis of CM was given. The patient was referred for laser therapy.
| Discussion|| |
VMs are extremely common lesions accounting for approximately 7% of all benign tumors. Although the head and neck constitute <14% of the total surface area of the body, approximately 50% of all VMs occur in these regions. VM are errors of morphogenesis that are populated by stable mature vascular endothelium. Both sexes are equally affected. They are always present at birth (though some may not be apparent until a later stage) and in contrast to hemangiomas, they never proliferate or involute. Instead, they expand slowly and relentlessly throughout life, in pace with the growth of the patient. Trauma, puberty, and pregnancy can cause accelerated growth. These lesions are sub-classified according to the predominant type of vessel and characteristics of flow. Unlike hemangiomas, VMs are associated with skeletal abnormalities in up to 35% of the cases. Intracranial locations are relatively common and often associated with potential complications of great concern that may occur in case of rupture or bleeding. Generally, extracranial VMs represent only an esthetic problem and normally do not require treatment.,
CM (previously called PWS capillary hemangioma) is made up of postcapillary venules within the papillary and superficial reticular dermis. They are congenital vascular birthmarks, occurring in an estimated 0.3% of newborns. A PWS is a slow-flowing CM, described as a cluster of pink to purple, sharply demarcated patches. They are typically located in a unilateral distribution on the head and neck and may involve the mucous membranes. Like most VMs, PWS are present at birth and do not involute spontaneously. In the present case, the CM was present since childhood and located in a unilateral distribution along the distribution of trigeminal nerve.
These lesions are characterized histologically by ecstatic vessels and a deficiency of nerves in the papillary plexus of the skin in the affected area. It is hypothesized that the deficient nerves are of sympathetic origin and that unchecked parasympathetic influence on blood flow through the postcapillary venules results in progressive vascular ectasia. They are graded according to the degree of ectasia of the vessels into Grades I–IV that correlate well with the clinical features and outcome of treatment. The dilated venules produce a discoloration or ''stain'' of the skin within the affected region that will be with the patient for life.,
CM lesions are observed more commonly among Caucasians than African Americans and Asians. There is no gender predilection. They usually begin as flat and pink macules becoming thicker and darker over time. In advanced lesions, nodules or “cobblestones” may be present. Over time, these lesions darken with 11% thickening and 24% developing nodules. Adjacent bone and soft tissue often become hyperplastic; this process is not well understood but is thought to result from the release of growth factors from skin affected by the PWS. Head and neck PWS usually follow the distribution of the branches of the trigeminal nerve, but more than one branch may be simultaneously involved as in the present case. When the maxillary and/or mandibular divisions are involved, the bone and soft tissue of the oral cavity are often affected. As a result, dentists and dental specialists often anticipate complications such as bleeding caused by the hypervascularity of the gingival and oral soft tissues, along with functional and cosmetic deformities from bony overgrowth involving the jaws and teeth.,,
CM can be isolated malformations or they may be associated with conditions that have systemic involvement. When PWS are located in a trigeminal distribution, Sturge–Weber syndrome, characterized by glaucoma, leptomeningeal venous angiomas, seizures, hemiparesis contralateral to the facial lesion, and intracranial calcifications must be considered. CM may also be associated with other syndromes such as proteus (with an overgrowth of the skeleton; skin, adipose, and central nervous system tissue; severe disfigurement; tumors; pulmonary complications; deep vein thrombosis; and pulmonary embolism), Beckwith–Wiedemann (with exomphalos, macroglossia, and gigantism), and Bonnet–Dechume–Blanc (with unilateral arterio-venous malformations involving the retinas, brain, and skin of the face). Our patient was investigated thoroughly to rule out any possible association with syndromes and was, therefore, a nonsyndromic or an isolated case of CM.
Patients with facial CM often demonstrate oral manifestations of their disorder. Familiarity with the orodental manifestations of CM may result in improved dental care for patients with PWS. Orodental manifestations of CM, such as staining of the oral soft tissues, hyperplasia of the gingiva, oral bleeding, overgrowth of the bony alveoli, and possible interruption in dental eruption sequence, have been reported. In the present case, staining of the buccal mucosa and palate was present. He also had significant gingival bleeding that regressed after a long time. As a result, opinions in the literature vary widely regarding the dental, periodontal, and surgical treatment of patients with CM. Although one author claims, Port wine stains or capillary malformations rarely present with major problems during surgery, some authors suggest to avoid Periodontal probing as even gentle probing may result in uncontrolled bleeding.
Color and texture changes in facial CM lesions occur in affected patients as they mature, but these processes can begin at any age. Darkening of the CM is suggestive of increased vascular ectasia. In our case, the CM had increased in color intensity resulting in a purplish hue and cobblestone appearance. Most of the patients do not report an unusually high rate of more serious bleeding after oral surgical procedures as CM affects small vessels, bleeding from which may be a nuisance but is not usually severe or life-threatening.
Dowling et al. in their study demonstrated that thickening or nodularity of the CM, statistically increased likelihood of gingival staining, and high-risk of orodental manifestations. In the present case, the CM was thicker, pebbled, and had increased propensity for gingival bleeding. CM involving the oral soft tissues is also a predictor of additional orodental complications including tissue hyperplasia and shifting of teeth. Dentists treating or manipulating the oral soft tissues in patients with oral CM should use vasoconstrictors and cautery or electrosurgery wherever possible, and prepare in advance with absorbable hemostatic dressings and extra time for adequate hemostasis.
Treatment is usually recommended for CM as they can cause significant morbidity, bleeding, and psychological and cosmetic concerns. The most effectual treatment is vascular-specific laser therapy, most notably pulsed dye laser (PDL). PDLs emanate wavelengths that explicitly target oxyhemoglobin, treating the vascular abnormality with minimal damage to the surrounding tissue. Effective treatment protocols include wavelengths of 585–600 nm, fluences of 6–12 J/cm 2, and pulse durations of 0.45–10 ms with concomitant epidermal cooling. Sequential treatment sessions every 4–8 weeks are recommended. Common side effects include transient erythema, edema, and mild purpura that spontaneously resolve in few days. Progressive lesional fading is seen after each treatment with multiple treatments typically necessary to achieve >75% improvement.
Laser treatment is most efficient when commenced in the first year of life, as infant skin is thinner, thus allowing better laser penetration. As the CM thickens and becomes nodular with increasing age. The long-pulse alexandrite (755 nm), long-pulse 1064-nm neodymium-doped yttrium aluminum garnet (Nd: YAG), and dual 595-nm PDL and 1064-nm Nd: YAG lasers have proven useful in such conditions, particularly when lesions have developed nodularity, because of the ability of these systems to achieve deeper dermal penetration.
Intense pulsed light (IPL) has also been used in patients' recalcitrant to PDL. A randomized study verified that PDL was generally more effective in initial regression, but in recalcitrant lesions, 6 of 15 patients had more than 75% clearance with IPL.
Although lasers continue to be used as the primary treatment for PWS, anti-angiogenic therapy is also in its nascent stage as an adjuvant therapy. Daily or thrice weekly application of topical imiquimod 5% between laser treatment sessions has been shown to reduce PWS color reduction more than PDL treatment alone. Few patients had minor skin irritation after imiquimod treatment.
| Conclusion|| |
Patients with CM often exhibit oral manifestations associated with this disorder. Patients with CM may exhibit physical findings involving the oral and perioral structures. We as oral physicians play an important role in diagnosing these conditions and identify patients at risk for future complications. Awareness of the orodental manifestations of PWS may result in improved dental care for patients with CM.
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.
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Conflicts of interest
The authors have obtained appropriate patient consent for the information published in this article.
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[Figure 1], [Figure 2], [Figure 3]