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Introduction

Hemangiomas are some of the most common benign tumors in pediatric population, with an incidence of 3% at birth and reaching 10% till the age of 1 year old⁽¹⁾. Head and neck location represents 60% of the cases with hemangioma. There is a general female predominance⁽²⁾. During their evolution, it is described a proliferative stage of up to 18 months followed by an involution stage with color change to wine-like red⁽³⁾. Thus, 50% of the head and neck hemangiomas resolve by the age of 5, and up to 70% by the age of 7⁽⁴⁾. Their etiology is still debatable. There is some association with older age of the parents, high blood pressure during pregnancy, and increased weight at birth⁽⁵⁾.

Since 1982, Mulliken and Glowacki have classified vascular anomalies at birth in hemangiomas and vascular malformations⁽³⁾. Hemangiomas represent, from a histological point of view, a proliferation of endothelial cells leading to formation of small vascular tumors with capillaries, venules and small vein. Given the type of vessels proliferating inside, the hemangiomas can be cavernous or capillary^(1-3). Cutaneous hemangiomas may be superficial (inside papillary dermal layer), deep (inside reticular dermal layer or subcutaneous tissue) or mixt. This staging can be also applied to hemangiomas at the level of the mucosa. Furthermore, their clinical evolution undergoes three steps: proliferation in the first year, involution up to 5 years, and involuted up to 10 years^(1,2,4).

The current principles of diagnosis in hemangiomas begin with thorough ENT clinical exam, searching for pulsations and murmurs implying the evolution of an arteriovenous malformation. Flexible endoscopy searches for associated oral and pharyngeal pathology. Doppler ultrasound is a noninvasive imaging modality for clinical monitoring. The gold standard is represented by cervical and cerebral MRI. Hemangiomas show a T1 hypo signal and T2 hyper signal along with contrast media uptake⁽⁵⁾. Bilateral carotid angiography permits selective embolization before surgery in order to diminish bleeding. Hemangiomas without a surgical indication should not be embolized and benefit only from Doppler ultrasound and MRI follow-up. Selective embolization should be reserved only to those cases with active bleeding.

The differential diagnosis of hemangiomas focuses on solid tumors or other vascular tumors like lymphangiomas or arteriovenous malformations. The latter represent a direct connection between frail arteries and veins with the absence of capillaries and high-pressure blood passing between these two territories⁽⁴⁾.

While facing such a case, the ENT surgeon should take into consideration the fact that many of the hemangiomas disappear spontaneously. The treatment modalities should be applied in those cases with increased esthetic impact, functional disturbances and recurrent bleeding. In children, the surgical removal is considered preemptive and requires parents’ or legal guardians’ informed consent⁽⁴⁾.

Clinical case presentation

We encountered the case of a 9-year-old girl with a right genial tumor with debut two years before, painless but with increased dimensions during effort or mastication (Figure 1). The clinical exam revealed a soft mass, without pulsations and protruding through oral mucosa while pressing on the genial grove (Figure 2).
 

Cerebral MRI with contrast described a soft tissue mass in the right genial region with T1 hypo signal and T2 hyper signal (Figure 3). Doppler ultrasound both of facial soft tissues enabled further study of the relations between facial vessels and masseter muscle (Figure 4). Before surgery, we performed bilateral carotid angio­graphy and selective embolization of the right internal maxillary artery with gel foam (Figure 5). Surgery was performed transoral under general anesthesia (Figure 6). Surgical healing was uneventful, with complete removal of sutures at 10 days (Figure 7). The pathology result concluded to a cavernous hemangioma.
 

Discussion

We still lack a clear recipe for approaching hemangiomas. Every case requires a personal and sequential therapy protocol. One of the most recent is the Treatment Guideline for Hemangiomas and Vascular Malformations of the Head and Neck (Head and Neck 2010)⁽⁴⁾.

The available treatment principles range from watchful waiting to laser therapy, medicine therapy (topical steroids, betablockers), sclerotherapy, radiotherapy, or cryosurgery. Classic open surgery is not the first line of treatment^(6-14).

Surgery is indicated in cases with hemangiomas at the tip of the nose and on the lips which are not responding to other treatment modalities, upper eye lid hemangiomas with visual impairment, esthetic impact of hemangiomas of the forehead and scalp, and in cases with recurrent bleeding episodes^(4,8). Further desk reference should be sought in the Guidelines of Care for Hemangiomas of Infancy (American Academy of Dermatology)⁽¹⁵⁾. Unfortunately, in cases affecting the oral or pharyngeal mucosa, the guidelines require further data.

The general practitioner should refer such cases to dermatologists, pediatricians, plastic surgeons, OMF surgeons, or ENT surgeons. Ideally, such cases should benefit from the input of an interdisciplinary team reuniting also imaging specialists and interventional radiologists. The experience gathered with each case improves the outcome of the next child with hemangioma of face and neck. 

Conclusions

Regarding pediatric cases with hemangiomas at the level of face and neck, the therapy protocol should be custom tailored. All the steps should be approved by a complex team trained to approach such complex cases. Selective embolization should be performed only before surgery and in other instances postponed. All the necessary arguments to support surgical decision and close cooperation with the parents and their informed consent are necessary.

Conflicts of interests: The authors declare no conflict of interests.