|Year : 2019 | Volume
| Issue : 2 | Page : 209-213
The star-crossed curse of childhood: A case of infantile hemangioma
N Geon Pauly1, Nidhin Kurian2, Roopashri Rajesh Kashyap1, Raghavendra Kini1, Prasanna Kumar Rao1, Ann Thomas2
1 Department of Oral Medicine and Radiology, A.J. Institute of Dental Sciences, Mangalore, Karnataka, India
2 Department of Pedodontics, A.J. Institute of Dental Sciences, Mangalore, Karnataka, India
|Date of Web Publication||26-Jun-2019|
Dr. N Geon Pauly
Department of Oral Medicine and Radiology, A.J. Institute of Dental Sciences, Kuntikana, NH-66, Mangalore - 575 004, Karnataka
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Vascular lesions are indeed very widespread, with vascular tumors numbering the most common tumors in childhood. Researchers and authors frequently use the idiom “hemangioma” to portray or describe vascular malformations and a potpourri of vascular anomalies. Infantile hemangioma, a type of hemangioma, is one of the most common benign vascular tumors in infancy and childhood. As hemangioma could regress spontaneously, it generally does not require treatment unless proliferation interferes with normal function or gives rise to the risk of serious disfigurement and complications unlikely to resolve without treatment. Various methods for treating infant hemangiomas have been documented, including wait-and-see policy, laser therapy, drug therapy, sclerotherapy, radiotherapy, and surgery. However, none of these therapies can be used for all hemangiomas. Hence, to obtain the best treatment outcomes, the treatment protocol should be individualized as per the case.
Keywords: Infantile hemangioma, orbital tumor, palate, vascular malformation
|How to cite this article:|
Pauly N G, Kurian N, Kashyap RR, Kini R, Rao PK, Thomas A. The star-crossed curse of childhood: A case of infantile hemangioma. J Indian Soc Pedod Prev Dent 2019;37:209-13
|How to cite this URL:|
Pauly N G, Kurian N, Kashyap RR, Kini R, Rao PK, Thomas A. The star-crossed curse of childhood: A case of infantile hemangioma. J Indian Soc Pedod Prev Dent [serial online] 2019 [cited 2019 Nov 12];37:209-13. Available from: http://www.jisppd.com/text.asp?2019/37/2/209/261341
| Introduction|| |
Hemangioma is a kind of endothelial malformation and can cause significant morbidity and mortality in children and adults. It is a benign vascular abnormality characterized by rapid endothelial cell proliferation and does not undergo malignant transformation. Although a few cases are congenital, most develop in childhood with rarely older individuals been affected. Literature proves that they tend to have a higher female predilection.
Hereby, we present a case of a 3-year-old female patient who visited our department with an unusual extensive growth across the palate.
| Case Report|| |
A 3-year-old female patient was referred to our department with a chief complaint of a growth inside the mouth in the palatal region. Her parents gave a history of a having first noticed a growth of such nature when she was 3 months old. They also gave a history of the growth having phases of spontaneous remission and regression over time and were positive that it had never completely resolved. She was moderately built and nourished for her age and had no associated complaints or lack of appetite. However, the recurrence after previous remission which dated 1 week prior had resulted in profuse bleeding while having food and, to the best of their knowledge, had been the most sizeable form they had even seen.
On clinical examination, there was a visually evident diffuse asymptomatic swelling on the left side of the face resulting in asymptomatic narrowing of the left eye with no impairment in vision [Figure 1]a and [Figure 1]b. On intraoral examination, multiple solitary/coalescing bluish purple pinpoint lesions with overlying elevated yellowish slough having irregular margins and surrounded by erythematous borders were seen across the hard and soft palate, predominantly on the left side extending onto the left alveolus distal to the primary second molar and crossing the midline onto the right side of the palate [Figure 2]a and [Figure 2]b. It bled profusely to touch.
|Figure 1: (a and b) Facial asymmetry on the left side with narrowing of the left eye|
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|Figure 2: (a and b) Bluish purple pinpoint lesions on the palate and alveolar mucosa with overlying yellowish slough. (c) Slight regression of the lesion after 3 months|
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A provisional diagnosis of infantile hemangioma (IH) was given based on the patient's history and clinical presentation. An occlusal radiograph was not possible as it bled acutely on coming in contact with the film at the alveolus region. Hence, a magnetic resonance imaging (MRI) and a computed tomography (CT) scan, plain and contrast, were advised. MRI and CT images revealed alarmingly extensive ill-defined areas of altered intensity/density involving the hard palate, pterygopalatine fossa, infratemporal fossa, and left orbit, encasing the medial rectus muscle suggestive of hemangioma [Figure 3]a and [Figure 3]b. The patient was referred to a vascular surgeon where they were advised to wait and watch for possible spontaneous regression with age as she was only 3 years old on date. The first periodic visit after 3 months revealed slight regression of the lesion [Figure 2]c.
|Figure 3: (a) Axial slice view on magnetic resonance imaging. (b) Coronal slice view on magnetic resonance imaging|
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| Discussion|| |
The term “hemangioma” is a generic one used to describe congenital hamartomas and vascular malformations. Although Hemangiomas' are overwhelmingly benign, they still remain under the scanner on whether they represent a true neoplasm, malformation, or hamartomas, and thus have been the subject of continuous debate, and use of the terms hemangioma and malformation can be confusing, which could well be applied to our case., The etiopathogenesis of hemangioma of infancy had remained surrounded by speculations and beliefs. Galen, the Greek physician, thought emotions of the mother to be the cause of vascular birthmark. The word “hemangioma” is derived from the Greek word “haima,” meaning blood, while the term “angioma” was designated by Virchow to refer to all vascular anomalies.
The prevalence of hemangiomas is estimated to be around 2%–3% in neonates, 10%–12% below 1 year of age, and 22%–30% found among babies who at the time of birth weigh <1000 g. These lesions represent the most common growths in infancy and childhood, and may vary from small innocent birthmarks to large disfiguring tumors. IHs usually appear 2–4 weeks after birth; grow rapidly till the age of 6–8 months and then slowly develop. By the age of 5–8 years, they start to involute and spontaneously regress in 70% of the cases and so were advised to observe as in our case.
Historically, hemangiomas have been classified in a variety of ways. According to Enzinger and Weiss, hemangiomas are broadly classified into capillary, cavernous, and miscellaneous forms such as verrucous, venous, and arteriovenous hemangiomas. An important descriptive classification is related to the depth of soft-tissue involvement given as: superficial, deep, and mixed and as per location as focal or segmental. Our case would clearly fall under deep and segmental types based on the classification charts.
Although hemangiomas are the most common benign tumors of the head and neck in children, their occurrence on the palatal mucosa is extremely rare. In 80% of cases, hemangiomas are known to be seen as single lesions. They usually, but not invariably, follow a benign course. Although the exact cause is unknown, some authorities believe that this lesion is not a true lesion, but rather a developmental anomaly or hamartoma. It has also been hypothesized that angiogenesis likely plays a role in the vascular excess present. Cytokines, such as basic fibroblast growth factor and vascular endothelial growth factor, are known to stimulate angiogenesis. Excesses of these angiogenic factors or decreases of angiogenesis inhibitors have been implicated in the development of hemangiomas.
Hemangiomas have also been seen to be associated with cerebrovascular and facial arterial anomalies. It was first noticed by Pascual-Castroviejo. Facial hemangiomas were first described by Reese et al. The acronym PHACE, which stands for posterior fossa brain malformation, hemangioma, arterial lesion, cardiac abnormalities and eye abnormalities, was first proposed by Frieden et al. However, its occurrence is very rare. Since PHACE syndrome has a multisystem involvement, a thorough evaluation prior to surgery will help rule out the possibility of such a condition.
Hemangiomas may mimic other lesions clinically, radiographically, and histopathologically. The differential diagnosis of hemangiomas includes pyogenic granuloma, peripheral giant cell granuloma, peripheral ossifying fibroma, chronic inflammatory gingival hyperplasia (epulis), epulis granulomatosa, varicocele, telangiectasia, and squamous cell carcinoma.
In our case, the hemangioma was seen extending up to the medial rectus muscle of the left eye. Literatures have shown that of the entire eyelid and orbital tumors seen in childhood, IHs are the most common. The incidence of IH is referenced in the literature between 3% and 10%. Although they are considered benign lesions that have a generally self-limited course, in the periocular region, they have the potential to cause amblyopia, strabismus, and severe disfigurement.
As far as management is concerned, there is no “gold standard” treatment applicable to all patients, and multidisciplinary management is often needed for best efficacy. Based on the articles published by Zheng et al. in 2009 and 2013, we have charted an algorithm to devise an effective treatment plan for treating IH [Figure 4].,
Usually, only 10%–20% requires treatment because of their size, location, or their behavior since the vast majority undergo spontaneous regression. Although different therapeutic procedures including microembolization, radiation, cryotherapy, sclerosing agents, corticosteroids and, recently, laser therapy have been reported, complete surgical excision of these lesions, if possible, offers the best chance of cure. The advent of neodymium: yttrium aluminum garnet laser, in particular, which can be used in a contact mode with fiber-optic hand piece, makes it the most suitable surgical option. The principal advantages are immediate hemostasis and little damage to the surrounding tissues, and so no postoperative pain. In addition, a sterile field is obtained, so there is no risk of secondary infection and no need for postoperative periodontal dressing. The prognosis of hemangioma, in general, is excellent since it does not tend to recur or undergo malignant transformation following adequate treatment.
However, one of the great difficulties in the management of IH lies within the decision to initiate therapy. Since many IHs may resolve spontaneously and interventions carry risk, the need to intervene is not always obvious. Parents and physicians alike approach the decision to intervene with anxiety and a degree of uncertainty.
| Conclusion|| |
Benign tumors like IH are highly unpredictable in most cases. Although they tend to involute in time, so wait and watch would be the wise and advisable choice. But on the flip side, few isolated cases do not involute and may turn threatening based on the extent and sites of involvement. Thus, it indicates the need for timely and accurate diagnosis of cases of IH at an early age, followed by a tremendously important sequence of mandatory periodic recalls and finally an essential need for extensive precautions to be undertaken for surgery, for there is adequate chance of heavy bleeding which could turn life-threatening if not curtailed.
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.
| References|| |
Zarepur E, Moghimi M. Trismus resulting from infantile hemangioma of the parotid: A rare case report. Iran J Ped Hematol Oncol 2015;5:249-53.
Mhapuskar A, Shende P. Non involuting cavernous hemangioma of buccal mucosa: A report of two cases. Oral Surg Oral Med Oral Pathol Oral Radiol 2015;1:89-92.
Dilsiz A, Aydin T, Gursan N. Capillary hemangioma as a rare benign tumor of the oral cavity: A case report. Cases J 2009;2:8622.
Singh P, Parihar AS, Siddique SN, Khare P. Capillary haemangioma on the palate: A diagnostic conundrum. BMJ Case Rep 2016;2016. pii: bcr2015210948.
Kaira N, Mahapatra GK, Srivastava D, Vaid L, Taneja HC. Hemangioma of the oral cavity in a seven year old: A case report. J Indian Soc Pedod Prev Dent 2002;20:49-50.
Mendiratta V, Jabeen M. Infantile hemangioma: An update. Indian J Dermatol Venereol Leprol 2010;76:469-75.
] [Full text]
Rathod V, Verma C, Sharma S, Mala S. Hemangioma of left buccal mucosa: A case report. Arch Dent Med Res 2016;2:61-5.
Avila ED, Molon RS, Conte Neto N, Gabrielli MA, Hochuli-Vieira E. Lip cavernous hemangioma in a young child. Braz Dent J 2010;21:370-4.
Pranitha V, Puppala N, Deshmukh SN, Jagadesh B, Anuradha S. Cavernous hemangioma of tongue: Management of two cases. J Clin Diagn Res 2014;8:ZD15-7.
Bharti V, Singh J. Capillary hemangioma of palatal mucosa. J Indian Soc Periodontol 2012;16:475-8.
] [Full text]
Fernandes S, Kakade A, Jetpurwala AM, Patil B, Jain S, Kasar P, et al
. Dental management of PHACE syndrome under general anesthesia. J Indian Soc Pedod Prev Dent 2011;29:S66-9.
Handa R, Sheikh S, Shambulingapaa P, Aggarwal A, Mago J, Salaria S. Capillary hemangioma of palate: A case report. Pak Oral Dental J 2013;33:55-7.
Zheng JW, Zhou Q, Yang XJ, Wang YA, Fan XD, Zhou GY, et al
. Treatment guideline for hemangiomas and vascular malformations of the head and neck. Head Neck 2010;32:1088-98.
Zheng JW, Zhang L, Zhou Q, Mai HM, Wang YA, Fan XD, et al
. A practical guide to treatment of infantile hemangiomas of the head and neck. Int J Clin Exp Med 2013;6:851-60.
Suprabha BS, Baliga M. Total oral rehabilitation in a patient with portwine stains. J Indian Soc Pedod Prev Dent 2005;23:99-102.
] [Full text]
Callahan AB, Yoon MK. Infantile hemangiomas: A review. Saudi J Ophthalmol 2012;26:283-91.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]