CASO CLÍNICO

 

Surgical treatment of facial hemangiomas in children

Tratamiento quirúrgico de los hemangiomas faciales en niños^*

 

 

M. Estrada Sarmiento¹, I. Virelles Espinosa², L. Terán Quiñones³, A. Báez Arias⁴

¹ Consultante. Profesor Auxiliar. Especialista de II grado en Cirugía Máxilo Facial. Investigador Agregado Asesor de la Universidad virtual de Salud y revista Multimed. Profesor Principal De cirugía Buco-Máxilo-Facial. Miembro de la Sociedad Cubana de Cirugía. Presidente de la Filial Granma de Cirugía Máxilo Facial.
² Lic. En Enfermería. Instructora. Jefa del área Quirúrgica. Miembro de la Sociedad Cubana de Enfermería.
³ Especialista de II grado en cirugía Máxilo Facial. Instructor.
⁴ Especialista de I grado en Cardiología. Profesor instructor. Jefe de Servicio.
Hospital Provincial Universitario «Carlos Manuel de Cespedes» Bayamo Granma. Cuba

^* Trabajo Presentado en la XVII Jornada Científica del Hospital Carlos Manuel de Céspedes. 5 y 6 de octubre 2007.

Correspondence

 

 

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ABSTRACT

Hemangiomas are the most common soft-tissue tumors in childhood. They occur in approximately 5-10% of one-year-old children. Despite their frequency, the pathogenesis of these tumors is not completely clear. Conservative management usually is proposed for hemangiomas that occur in childhood, but the occurrence of these tumors on the face can result in severe complications and may be an indication for treatment. We report four patients who received surgical treatment before the age of seven years for severe complications, including repeated bleeding, distortion of adjacent structures, and vision disorders. The surgical techniques depended on the location and size of the lesion.

Key words: Hemangioma/surgery; Oral surgery/procedures.

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RESUMEN

Los hemangiomas son los tumores de tejido blando más comunes en la infancia. Ocurren aproximadamente en el 5-10% de los niños de un año de edad. A pesar de la frecuencia de estos tumores, su patogénesis no está completamente esclarecida. Aunque el manejo conservador es usualmente propuesto para los hemangiomas que ocurren en la infancia, la presencia de estos tumores en la cara puede resultar en complicaciones severas y proporcionar una indicación para el tratamiento. En este trabajo mostramos cuatros pacientes que recibieron tratamiento quirúrgico antes de los sietes años por las complicaciones severas que incluían sangramientos repetidos, distorsión de las estructuras adyacentes y trastornos visuales. Las técnicas quirúrgicas dependieron de la localización y tamaño de la lesión.

Palabras clave: Hemangioma/cirugía; Procedimientos quirúrgicos orales.

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Introduction

The term "hemangioma" has been applied traditionally to a variety of vascular lesions that range from life-long conditions to benign, self-limited tumors of childhood. Hemangiomas are benign proliferations of the angioblastic mesenchyme. The term "cavernous hemangioma" is particularly confusing because it has been used to describe hemangiomas with subcutaneous components, such as structural anomalies of venous origin. These two types of lesions seem similar in childhood, but differ in their growth pattern, response to treatment, and prognosis.¹

Mulliken² defined hemangiomas as vascular tumors with a growth phase characterized by endothelial proliferation, hypercellularity, and an involutional phase.

This author recognized that many entities referred to as hemangiomas in reality are structural malformations derived from capillaries, veins, lymphatics, arteries, or a combination of vascular structures.^2,3

Although hemangiomas and vascular malformations usually do not appear in the same context, they sometimes coexist.⁴

The precise mechanism that controls the growth and involution of hemangiomas is not completely clear, but recent advances in our knowledge of normal vascular development and angiogenesis provide some information. "Vasculogenesis" refers to the process by which the precursors of endothelial- lined vessels develop into blood vessels. "Angiogenesis" refers to the development of new blood vessels out of the existing vascularization.

The role of vasculogenesis is less clear, but the presence of arterial anomalies in some patients with extensive hemangiomas has been attributed to the development of deffect at approximately 8 to 10 weeks of gestational age.^1,5

The hemangioma life cycle differs from that of other tumors in that hemangiomas have a phase of rapid proliferation that is followed by spontaneous involution. The mechanism that controls the involution of hemangiomas also is little known.¹

Genetic techniques have been useful in showing that the regulation of angiogenesis and vasculogenesis is deficient in rare vascular hereditary diseases.⁶

In rare cases, hemangiomas are familial and diverse relationships with a presumably autosomal dominant pattern of heredity have been described recently.⁷

Hemangiomas are clinically heterogeneous, and their physical aspect is conditioned by their depth, location, and stage of evolution. Thirty-three percent are diagnosed at birth and the rest are diagnosed in the first six months of life,¹ coinciding with the maximum development of the tumor. After this initial period of expansion, they usually remit spontaneously and gradually, and may even resolve completely after a few years. Hemangiomas are the most frequent tumor in childhood, affecting 10-12% of children younger than one year.² They show a predilection for the female sex of approximately 66%.^1,3

In newborns, hemangiomas can originate as a pale macule with filiform telangiectasias. As the tumor proliferates, it assumes its more recognizable forms.

Hemangiomas that lie deep in the skin are warm soft masses with a slightly bluish color. Hemangiomas often have superficial and deep components.

They vary from several milliliters to several centimeters in diameter and generally occur alone, although 20% of the children affected have multiple lesions.

The behavior of hemangiomas is well known, but it is difficult to predict the duration of the growth and involutional phases of individual lesions.⁸

The onset of involution often is difficult to predict, but it usually is preceded by a change in color from brilliant red to purple or gray.

Approximately 20 to 40% of patients have residual changes in the skin, and hemangiomas of the tip of the nose, lip, and parotid gland show a slow involution.⁹

The diagnosis sometimes can be difficult to establish. Imaging studies help to differentiate vascular malformations from neoplastic processes.

The treatment of hemangiomas continues to be a topic of debate. Conservative management usually is proposed for hemangiomas in children, but the occurrence of facial hemangiomas can result in severe complications and establish an indication for treatment.

The surgical technique depends on the location and size of the lesion and focuses on tumor resection, with reconstruction of the adjacent structures when necessary.¹⁰

Early surgery is mandatory in large periocular hemangiomas, labial hemangiomas that are prone to bleeding and cause difficulties while eating, and hemangiomas of the tip of the nose, which regress slowly and distort the cartilaginous structures.

The objective of this study was to describe our experience in the surgical treatment of facial hemangiomas in children.

 

Clinical Cases

All patients were treated with intralesional corticoids, betamethasone, or triamcinolone. The fibrotic lesion was eliminated and adjacent structures were reconstructed surgically as necessary.

Case 1

A 3-month-old girl presented at birth an erythematous area with fine telangiectasia that occupied her right upper lip. Within a few days, the lesion grew, forming a bluish-colored mass that occupied more than two-thirds of the lip and interfered with feeding (Figs. 1A and B).

In the general examination, no other anomalies were observed. Five intralesional infiltrations of steroids at a dose of 4 mg administered at 4-week intervals induced fibrosis and reduced the hemangioma. The lesion was then resected surgically and the lip was reconstructed. At present, the girl’s development is normal (Figs. 2 A and B).

Case 2

This girl presented at birth a bluish-colored spot in the right nasoorbital region, which grew rapidly during the first month of life.

When we saw her at the age of 6 months, she had a pedunculated 4-cm mass that involved the nasoorbital furrow and nasal dorsum. The child had acquired the habit of looking at the lesion and she presented strabismus (Fig. 3). We began treatment five days later with intralesional injections of betamethasone 4 mg, followed by four more sessions at fourweek intervals. The fibrotic hemangioma was resected using a superciliary incision (Fig. 4).

The postoperative evolution was satisfactory (Fig. 5).

Case 3

A seven-month-old boy presented at 15 days of birth a violaceous-colored plaque with progressive growth that occupied a large part of tip of the nose. The plaque was evaluated by the pediatrician, who maintained a "wait-and-see" attitude for 6 months. Upon observing that the lesion continued to progress, causing facial deformity, the child was referred to our department (Fig. 6).

We detected cartilage deformity and discrete respiratory difficulty in the examination.

Sclerosing treatment was begun with intralesional injections of betamethasone 4 mg. When the lesion had fibrosed, the lesion was excised surgically using an inverted gull wingshaped incision.

Three months after surgery, the patient had this appearance (Fig. 7).

Case 4

A three-month-old girl presented at birth a small increase in the volume of the left lower lip that grew progressively, producing labial deformity, ulceration, and repeated bleeding (Fig. 8).

After three intralesional betamethasone infiltrations, the lesion was excised using a V-shaped technique.

Six months after surgery, this was her appearance (Fig. 9).

 

Discussion

Hemangiomas are benign proliferations of the angioblastic mesenchyme. Mulliken² defined hemangiomas as vascular tumors with a growth phase characterized by endothelial proliferation, hypervascularity, and an involutional phase.

The precise mechanism that controls the growth and involution of hemangiomas is not completely clear, but recent advances in our knowledge of normal vascular development and angiogenesis provide some information.

Hemangiomas are clinically heterogeneous, and their physical aspect is conditioned by their depth, location, and stage of evolution. Thirty-three percent are diagnosed at birth.

Hemangiomas constitute the most frequent tumor in childhood, affecting 10-12% of children younger than one year.² They exhibit a predilection for the female sex of approximately 66%.^1,3 Hemangiomas are solitary lesions, but in 20% of affected children, multiple lesions occur.

The onset of their involution is preceded by a change in color from brilliant red to purple or gray.

The treatment of hemangiomas continues to be a topic of debate.

Even though the choice of procedure may vary, the decision to proceed in these cases usually is well founded. Selection of the appropriate surgical technique depends on several factors. Although a "wait and watch" attitude usually is advised for hemangiomas in children, surgical resection in childhood usually is indicated for the removal of residual fibrosis after complete resolution of the hemangioma. Nevertheless, there are indications for surgical intervention at early ages, such as: pedunculated hemangiomas, hemangiomas that are life-threatening or interfere with function, and hemangiomas in which pharmacologic therapy is ineffective or poorly tolerated. Surgery is mandatory in large periocular hemangiomas, labial hemangiomas that are prone to bleeding and that cause feeding difficulties, and hemangiomas of the tip of the nose, which regress slowly and produce distortion of the cartilaginous structures.¹¹

Children who reach the age of 2-3 years with a hemangioma may exhibit evident psychological problems. This is the age at which a child first becomes self conscious about his or her facial image. The child with facial hemangioma generally is accepted by peers in the preschool years. Problems appear in elementary school, when the child is exposed to older peers. This is the time to consider hemangioma removal.

Hemangiomas of the lip and tip of the nose are psychological targets and their elimination may be beneficial in childhood.

Surgical removal is unnecessary in cases in which the success of the procedure is doubtful. The benefits and risks of a surgical approach must be weighed carefully.

 

Conclusions

The surgical treatment of facial hemangiomas in children under the age of 7 years must be performed early when vital orifices are threatened, patients present frequent bleeding, and the hemangioma causes functional, aesthetic, or psychological disturbances.

 

 

Correspondence:
Dr. Manuel Estrada Sarmiento
Máximo Gómez 6/ Maceo y Canducha Figueredo.
85100 Bayamo, Granma, Cuba.
Email: mesarmiento.grm@infomes.sld.cu

Recibido: 12.11.07
Aceptado: 16.06.08

 

 

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