|Year : 2014 | Volume
| Issue : 2 | Page : 56-60
Vascular malformation of upper lip and left eye: A case report with nomenclature review
Parita K Chitroda, Girish Katti, Bhuvaneshwari Kalmath, Irfan Baba
Department of Oral Medicine and Radiology, Al-Badar Dental College and Hospital, Gulbarga, Karnataka, India
|Date of Web Publication||13-Aug-2014|
Department of Oral Medicine and Radiology, Al-Badar Dental College and Hospital, SY. No. 12, Daryapur Village, Nagan Halli Road, Gulbarga - 585 105, Karnataka,
Source of Support: None, Conflict of Interest: None
The term "Hemangioma" though traditional yet until date is the most frequently misused terminology by many radiologists for reporting all variety of vascular anomalies. Mulliken in 1982 developed a thorough study and classification, based on the cellular kinetics of anomalous vessels, and includes two entities:
- Hemangioma/vascular tumor and
- Vascular malformations (VM).
However, the continuous use of an older nomenclature causes confusion resulting in inaccurate diagnoses and hence potential mismanagement. The management of these abnormalities presents both a diagnostic and therapeutic challenges and they expand to include the preservation of both functional concerns and cosmetic appearance of the affected structures. Further the application of more advanced diagnostic techniques, all gives the precise localization of the source vessel allowing for making excision and reconstructive planning more accurate and satisfactory. Here we present a case of a 16-year-boy diagnosed with low flow VM affecting upper lip causing and left eye.
Keywords: Hemangioma, vascular anomalies, venous malformation and capillary malformation
|How to cite this article:|
Chitroda PK, Katti G, Kalmath B, Baba I. Vascular malformation of upper lip and left eye: A case report with nomenclature review. J Oral Maxillofac Radiol 2014;2:56-60
|How to cite this URL:|
Chitroda PK, Katti G, Kalmath B, Baba I. Vascular malformation of upper lip and left eye: A case report with nomenclature review. J Oral Maxillofac Radiol [serial online] 2014 [cited 2021 Jan 19];2:56-60. Available from: https://www.joomr.org/text.asp?2014/2/2/56/138647
| Introduction|| |
Vascular anomalies are congenital errors in vascular development and include hemangioma and vascular malformations (VM). They are very different entities, best distinguished by their history mainly relating to the time of onset and examination. They frequently involve the head, neck, and oral cavity. Uses of modern technology such as ultrasound (flow rate and tissue architecture), magnetic resonance imaging (extent of the lesion) and finally computer angiography in high flow lesions (identify feeding vessels) lead to treatment modalities much sophisticated and of cosmetic approach.  However, "hemangioma" a common nomenclature is still used by many radiologists causing confusion, inaccurate diagnoses, and potential mismanagement.
| Case Report|| |
A 16-year-old boy reported with the chief complaint of upper lip and left eye swelling since 4 years and 6 months, respectively. The upper lip swelling occurred spontaneously and was a size of peanut that gradually increased to reach the present day size in 4 years span [Figure 1]. Patient noticed another swelling that started 6 months ago in the left eye that was small and gradually increased in size [Figure 1]. In both situations, he denied any significant history of injury or pain. Apart from facial asymmetry no other functional abnormality, including vision disturbance was reported.
There was no other significant dental, medical or familial history and general physical examination showed all normal vital signs.
The upper lip swelling measured about 3 cm × 4 cm in its longest dimension and encompassed the upper lip (right to left lip commissure) and extended onto the cutaneous portion as well as onto the intra-oral aspect that appeared purplish blue in color. Inspection revealed mild pigmentation of skin over the swelling. Swelling was nonpulsatile, nontender, but soft in consistency. On palpation, pulsation were felt near the left ala of nose, auscultation revealed bruit sounds and Diascopy test showed blanching of lesion that indicated fluid displacement into the adjacent tissue compartment.
The left eye swelling was present towards the medial canthus, ovoid in shape and measured about 1 cm × 0.5 cm with hyper-pigmented skin (purplish color). Upon reflecting the lower eyelid the swelling appeared attached to the lower conjunctiva and was purplish red in color that gave an appearance of strawberry [Figure 2].
Provisional diagnosis-VM was considered.
Vascular tumor, arterio-venous malformation (AVM) and lymphangioma were considered as differentials.
Following investigations were made:
• Doppler test reported
- Soft tissue swelling of 4.4 cm × 2 cm in upper lip
- Low velocity flow seen in vessel
- Feeding vessel seen at the right margin of the lip from maxillary artery
- Swelling is in subcutaneous location with No evidence of deeper extension.
• Finial impression
- Soft tissue hemangioma in right upper and left lower eye lid
• Computed tomography (CT) - scan and CT angiogram study showed [Figure 3], [Figure 4], [Figure 5]
|Figure 3: Computed tomography-angiogram shows the external carotid|
artery and its branches are normal in caliber and no abnormal vessels seen
in upper lip and left peri-orbital region
Click here to view
|Figure 4: Computed tomography scan image shows well-defi ned hyper|
intense lesion in the upper lip
Click here to view
|Figure 5: Computed tomography scan image shows well-defi ned hyper|
intense lesion beneath the inner canthus of left eye with no evidence of
intra orbital extension
Click here to view
- The external carotid artery and its branches are normal in caliber
- No abnormal vessels seen in upper lip and left peri-orbital region.
- Well defined hyper intense lesion in the upper lip in the midline and beneath the inner canthus of left eye with no evidence of intra orbital extension
• Final report
- Suggestive of low flow hemangioma in the upper lip and inner canthus of left eye and no obvious feeding arteries identified.
Considering the history mainly relating to the time of onset, clinical presentation and above-mentioned investigation a final diagnosis of low flow VM of soft tissue was made.
| Discussion|| |
A confusion always existed regarding the use of older terminology such as "hemangioma or angiomas or birthmark" that were used to describe the vascular lesion and hence to avoid such confusion, The International Society for the Study of Vascular Anomalies (ISSVA) was born in 1992 with a primary goal of understanding and management of these lesions and to discard the old terms. 
A very basic classification system was Adopted 1996 workshop, to give us a common universal language.
Two main types of vascular anomalies are distinguished based on their biological features as shown in [Table 1].
Is a vascular tumor that grows by cellular (mainly endothelial) hyperplasia: Commonly presented as infantile hemangioma. A benign vascular tumor that are characterized by a rapid growth phase followed by gradual involution.
Is a structural anomaly of blood vessels but without endothelial proliferation, there is localized defect in the vascular morphogenesis, most likely caused by dysfunction in pathways regulating embryogenesis and vasculogenesis. VM persist throughout life without regression; most of them have commensurate growth during childhood, while some worsen over time if not treated. 
In our case, the lip swelling started around 12 years of age and continued to grow with along with patient's age. Another swelling was reported in the left eye that began 6 months ago.
Differentiating between vascular tumors and malformations is essential as not only their clinical, radiological and pathologic features and their morbidity, but also their management is quite different. Based on hemodynamics and on predominant anomalous channels, VM are either slow-flow or fast-flow. 
Low flow VM present with features such as soft, bluish with no arterio-venous fistulas. They do not involute and may rather get worse with age. Long-term deformity can cause depression and feeling of isolation.  The large number of venous lesions tends to be more evident at the vermilion border and intraorally on the mucosal side. The mucosa offers little resistance and becomes ballooned with the lesion.  The eye lesion may be cutaneous or may extend to subcutaneous tissue. The subcutaneous portion may extend into the orbit causing painless, nonpulsating proptosis of the involved eye. 
With our case most of the above-mentioned lip features were present with minimal eye features and no syndrome association was noted.
Syndromes associated with vascular malformations
Klippel-Trenaunay syndrome sometimes known as angio-osteo-hypertrophy syndrome and hemangiectatic hypertrophy is a rare congenital condition in which blood vessels and/or lymph vessels fail to form properly. The three main features are nevus flammeus (port-wine stain), venous and lymphatic malformations and softtissue hypertrophy of the affected limb. 
Sturge- Weber syndrome More Details More Details
Sometimes also referred as encephalo-trigeminal angiomatosis, is a rare congenital neurological and skin disorder and is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation and ipsilateral leptomeningeal angioma. It is characterized by abnormal blood vessels on the brain surface. 
Furthermore known as hereditary hemorrhagic telangiectasia - is a hereditary disorder leading to easily bleeding telangiectases on skin and mucosal surfaces, and it is associated with the presence of AVMs in multiple organ systems such as lungs, liver, and brain. 
Treatments for low flow VM: Treatment modalities various with each type and sub-type of malformation.
- Lymphatic malformations - Can be divided into two types: Macrocystic and microcystic.
- Microcystic lymphatic malformations can be treated with intra-lesion injection of pinyangmycin (1.0 mg/mL) and laser therapy. 
- Macrocystic lymphatic malformations, Sclerotherapy are the mainstay of treatment, with excellent effect. 
- Venous malformations - treatments for venous malformations are more complicated. In the past years, many attempts for treating venous malformations have been made that include irradiation, electrocoagulation, cryotherapy, intravascular magnesium or copper needles, laser and compression.  However the mainstay remains sclerotherapy and surgery for lip lesion, in particular, that involves surgical reduction. Debulking of small lesions can usually be achieved by a variety of excisional techniques such as horizontal and vertical wedge resections and elliptical excisions followed by lip reconstruction due to important functional and aesthetics. 
- Capillary malformations - the main treatment option is laser therapy further depending on the type of lesion, various laser devices and methods are available that can be selected.  In cases of eyelid occlusion leading to significant astigmatism, prompt treatment in the form of occlusion and refractive correction should be initiated with use of corticosteroids, surgery, laser photocoagulation and immunomodulators. 
Though capillary hemangioma of eye, in particular presents usually at birth and up to 1-year of age, spontaneous involution is the rule and only observation may be appropriate for such cases, however in few instances it is known to occur as late as 7 years in which case meticulous surgical excision gives good results; thou recurrent is observed. 
With our case sclerotherapy and surgical debulking for lip swelling was planned. However the capillary VM of left eye that started around 16 years, laser/surgery was considered as the best treatment option for which the patient was referred to specialty eye center.
| Conclusion|| |
Till today radiologist still continue to use the terminology hemangioma for reporting all forms of vascular anomalies, so it is very important that the radiologist should get enrolled in the continued education program and follow the recent classification and nomenclature as given by the ISSVA. Their correct classification and diagnosis is imperative to accurately ascertain prognosis and direct treatment. Multimodal therapy is frequently indicated, and in complex patients a referral to a multidisciplinary vascular anomalies team should be considered.
| References|| |
|1.||Ng BC, San CY, Lau EY, Yu SC, Burd A. Multidisciplinary vascular malformations clinic in Hong Kong. Hong Kong Med J 2013;19:116-23. |
|2.||Aelvoet GE, Jorens PG, Roelen LM. Genetic aspects of the Klippel-Trenaunay syndrome. Br J Dermatol 1992;126:603-7. |
|3.||Young AE. Arteriovenous malformations. In: Mulliken JB, Young AE, editors. Vascular Birthmarks: Hemangiomas and Malformation. Philadelphia: WB Saunders; 1988. p. 228-45. |
|4.||Bradley JP, Zide BM, Berenstein A, Longaker MT. Large arteriovenous malformations of the face: Aesthetic results with recurrence control. Plast Reconstr Surg 1999;103:351-61. |
|5.||Low DW. Management of adult facial vascular anomalies. Facial Plast Surg 2003;19:113-30. |
|6.||Graff JM, de la Garza AG, Carter KD. Capillary hemangioma: 4 month-old female with occlusive vascular lesion of right upper eyelid EyeRounds.org. Available from: http://www.EyeRounds.org/cases/57-capillary-hemangioma-eyelid.htm. [Cited on 2007 Jan 31]. |
|7.||Shirley MD, Tang H, Gallione CJ, Baugher JD, Frelin LP, Cohen B, et al. Sturge-Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N Engl J Med 2013;368:1971-9. |
|8.||Haitjema T, Westermann CJ, Overtoom TT, Timmer R, Disch F, Mauser H, et al. Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease): New insights in pathogenesis, complications, and treatment. Arch Intern Med 1996;156:714-9. |
|9.||Bai Y, Jia J, Huang XX, Alsharif MJ, Zhao JH, Zhao YF. Sclerotherapy of microcystic lymphatic malformations in oral and facial regions. J Oral Maxillofac Surg 2009;67:251-6. |
|10.||Zhong PQ, Zhi FX, Li R, Xue JL, Shu GY. Long-term results of intratumorous bleomycin-A5 injection for head and neck lymphangioma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998;86:139-44. |
|11.||Berenguer B, Burrows PE, Zurakowski D, Mulliken JB. Sclerotherapy of craniofacial venous malformations: Complications and results. Plast Reconstr Surg 1999;104:1-11. |
|12.||Zide BM, Glat PM, Stile FL, Longaker MT. Vascular lip enlargement: Part I. Hemangiomas - Tenets of therapy. Plast Reconstr Surg 1997;100:1664-73. |
|13.||Peled IJ. Aesthetics and function in lip reconstruction. Aesthetic Plast Surg 1999;23:275-8. |
|14.||Yanoff M, editor. Text Book of Ophthalmology. 3 rd ed. China: Mosby Elsevier Health Sciences; 2009. p. 1462. |
|15.||Nigwekar SP, Nigwekar PV. Atypical presentation of capillary hemangioma of upper eyelid: A case report. Pravara Med Rev 2011;3:34-6. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]