CERVICAL PATHOLOGY

Tratamentul chirurgical al hemangioamelor cervico-faciale la copil – unde, când şi cum?

 Surgical treatment for hemangiomas of face and neck in children – where, when and how?

First published: 06 martie 2020

Editorial Group: MEDICHUB MEDIA

DOI: 10.26416/Orl.46.1.2020.2838

Abstract

Introduction. Hemangiomas are among the most common benign tumors in children, with an incidence of 2-3% in newborns and up to 10% in the first year of life. The pathology at the level of head and neck accounts for 60% of the total number of cases with hemangiomas. In pediatric cases, most of them resolve spontaneously. Therefore, the the­ra­peutic modalities should be applied in cases with an im­por­tant esthetic deficit, with functional disturbances or with recurring bleeding. In children, the treatment should be preemptive in nature and requires the parents’ in­formed consent. Materials and method. We describe the case of a 9-year-old girl with hemangioma at the level of the right nasogenial groove. We applied our current al­go­rithm for diagnosis and treatment and we performed the oral radical ablation of the hemangioma. Discussion. In pediatric cases, many of them have a spontaneous re­so­lu­tion. The indication for undergoing a treatment mo­da­li­ty derives from major esthetic impact, functional dis­tur­bances or recurrent bleeding. Conclusions. The children with face and neck hemangiomas require a personalized ap­proach tailored in a complex team meeting. Selective em­bo­li­za­tion should be performed in selected cases before sur­gi­cal ablation by a trained interdisciplinary team.

Keywords
hemangioma, children, surgery

Rezumat

Introducere. Hemangioamele sunt cele mai întâlnite tu­mori benigne ale copilului, având o incidenţă de 2-3% la nou-născut şi ajungând până la 10% în apropierea vârstei de 1 an. Lo­ca­li­za­rea la cap şi gât reprezintă 60% dintre toate ca­zu­ri­le de he­man­gioa­me. Indicaţia terapeutică în cazul he­man­gioa­me­­lor la copil trebuie să ţină cont de faptul că cele mai mul­te re­gre­sea­ză spontan. Astfel, indicaţie terapeutică vor avea he­man­gioa­me­le cu impact estetic semnificativ, cele care induc tul­bu­rări func­ţio­nale sau cele cu sângerări recurente. Tra­ta­men­­tul he­­man­gioamelor la copil este considerat preventiv şi ne­­ce­si­tă con­sim­ţă­mân­tul informat al părinţilor. Materiale şi me­to­dă. Pre­zen­tăm cazul clinic al unei fetiţe de 9 ani, cu un he­man­giom al regiunii geniene drepte, la care s-a practicat abla­ţie ra­di­ca­lă prin abord oral. Prezentăm algoritmul de di­ag­­nos­tic şi tratament. Discuţie. Cele mai multe cazuri pediatrice au o rezoluţie spontană. Indicaţia de tratament se impune în cazul unui impact estetic semnificativ, al tulburărilor funcţionale şi al sângerărilor recurente. Concluzii. În he­man­gioa­mele cervico-faciale la copil, protocolul terapeutic tre­bu­ie individualizat. Discutarea acestui protocol trebuie fă­cu­tă într-o echipă mixtă şi antrenată în această patologie. Em­bo­li­za­rea selectivă se poate face doar preoperatoriu, în cazuri selec­ţionate. 

Cuvinte cheie

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).
 

Figure 1. Clinical aspect of right genial mass
Figure 1. Clinical aspect of right genial mass
Figure 2. Presence of the tumor at the level of right oral mucosa under light external pressure
Figure 2. Presence of the tumor at the level of right oral mucosa under light external pressure
Figure 3. MRI revealing the extent of the right genial tumor with T1 isosignal and T2 hyper signal
Figure 3. MRI revealing the extent of the right genial tumor with T1 isosignal and T2 hyper signal

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.
 

Figure 4. Doppler ultrasound of the soft tissues ena­bling the analysis of the contact between the mass and facial vessels and masseter muscle
Figure 4. Doppler ultrasound of the soft tissues ena­bling the analysis of the contact between the mass and facial vessels and masseter muscle


 

Figure 5. Bilateral carotid angiography followed by selective embolization of the right internal maxillary artery with gel foam
Figure 5. Bilateral carotid angiography followed by selective embolization of the right internal maxillary artery with gel foam


 

Figure 6. Transoral surgical approach with radical ablation of the mass
Figure 6. Transoral surgical approach with radical ablation of the mass


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. 

Figure 7. Clinical result 10 days after surgery
Figure 7. Clinical result 10 days after surgery

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.

Bibliografie

  1. Jacobs AH, Walton RG. The incidence of birthmarks in the neonate. Pediatrics. 1976; 58:218–222. 
  2. Waner M, Suen JY. Management of congenital vascular lesions of the head and neck. Oncology. 1995; 9:989–994. 997. 
  3. Finn MC, Glowacki J, Mulliken JB. Congenital vascular lesions: clinical application of a new classification. J Pediatr Surg. 1983; 18:894–900. 
  4. Zheng JW, Zhou Q, Yang XJ, Wang YA, Fan XD, Zhou GY, Zhang ZY, Suen JY. Treatment guideline for hemangiomas and vascular malformations of the head and neck. Head Neck. 2010; 32:1088–1098. 
  5. van Rijswijk CS, van der Linden E, van der Woude HJ, van Baalen JM, Bloem JL. Value of dynamic contrast-enhanced MR imaging in diagnosing and classifying peripheral vascular malformations. Am J Roentgenol. 2002; 178:1181–1187. 
  6. Guidelines of care for cryosurgery. American Academy of Dermatology Committee on Guidelines of Care. J Am Acad Dermatol. 1994; 31:648–653. 
  7. Al Buainian H, Verhaeghe E, Dierckxsens L, Naeyaert JM. Early treatment of hemangiomas with lasers. A review. Dermatology. 2003; 206:370–373. 
  8. Frieden IJ, Haggstrom AN, Drolet BA, Mancini AJ, Friedlander SF, Boon L, Chamlin SL, Baselga E, Garzon MC, Nopper AJ, Siegel DH, Mathes EW, Goddard DS, Bischoff J, North PE, Esterly NB. Infantile hemangiomas: current knowledge, future directions. Proceedings of a research workshop on infantile hemangiomas, April 7-9, 2005, Bethesda, Maryland, USA. Pediatr Dermatol. 2005; 22:383–406. 
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  11. Ho NT, Lansang P, Pope E. Topical imiquimod in the treatment of infantile hemangiomas: a retrospective study. J Am Acad Dermatol. 2007; 56:63–68. 
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