Treating an ulcerated hemangioma: a case report

Abstracts

O hemangioma é o tumor vascular mais presente em crianças até 1 ano de idade. Geralmente, sua involução é espontânea, sendo por isso recomendada uma conduta expectante. Ulceração e infecção secundária, seguidas de hemorragia, são as complicações mais frequentes dos hemangiomas. Estima-se que apenas 10 a 20% dos hemangiomas precisem ser tratados. A cirurgia é indicada nos casos de emergência, naqueles em que não há resposta aos tratamentos sistêmicos ou por razões estéticas. O objetivo deste estudo é relatar um caso de hemangioma combinado ulcerado do pavilhão auricular, em paciente de 8 meses de idade submetida a exérese total.

Hemangioma; neoplasias de tecido vascular; cirurgia; complicações

CASE REPORT

Treatment of ulcerated hemangioma: case report

Guilherme Benjamin Brandão Pitta^I; Rosamaria Rodrigues Gomes^II

^IDoutor. Professor adjunto, Universidade Estadual de Ciências da Saúde de Alagoas (UNCISAL), Maceió, AL, Brazil

^IIAcadêmica de Medicina, UNCISAL, Maceió, AL, Brazil

Correspondence

ABSTRACT

Hemangioma is the most common vascular tumor in children younger than 12 months. Because its regression is usually spontaneous, an expectant management is recommended in most cases. Ulceration and secondary infection followed by bleeding are the most frequent complications of hemangiomas. It is estimated that only 10 to 20% of hemangiomas require treatment. Surgery is usually indicated in emergency cases, when there is no response to systemic treatments, or for cosmetic reasons. The objective of this study was to report a case of ulcerated compound hemangioma on the external ear of an 8-month-old patient who underwent a complete surgical excision.

Keywords: Hemangioma, vascular tissue neoplasms, surgery, complications.

Introduction

Hemangiomas are vascular abnormalities in the group of vascular dysplasias.¹ They are the most common vascular tumor during childhood, with an incidence rate of 10 to 12% in children younger than 12 months of age.²

Virchow, in 1863, first classified vascular abnormalities based on their microscopic histologic features into the following categories: angioma simplex, angioma cavernosum, and angioma racemosum.³ In 1982, a new classification for vascular lesions was proposed based on clinical course, histopathological features, and natural history, distinguishing hemangiomas from vascular malformations. In 1996, the International Society for the Study of Vascular Anomalies reviewed that classification and adopted it as official, distinguishing vascular lesions into two groups: vascular tumors and vascular malformations.⁴

Vascular tumors are vascular neoplasm with proliferation of endothelial cells. Among them are included infantile hemangioma, rapidly involuting congenital hemangioma, noninvoluting congenital hemangioma, tufted angioma, kaposiform hemangioendothelioma, and pyogenic granuloma. Vascular malformations are considered to be defects of vascular morphogenesis and are classified according to the predominant anomalous vessel.

In clinical terms, hemangiomas can be classified into two categories:

1) Flat hemangiomas: superficial or deep hemangiomas, known as Port-wine stains, are the most frequent lesions among vascular malformations; they are observable at birth and can become raised and nodular during the second decade of life.

2) Tumorous hemangiomas: strawberry and cavernous hemangiomas. Strawberry hemangiomas are proliferating hemangiomas that may be observed at birth. Their involution usually starts spontaneously in the second year of life, but they can evolve to alarming hemangiomas. Cavernous hemangiomas are caused by defects in vascular morphogenesis. They are venous malformations with artery involvement, and they do not involute spontaneously. In these cases, indication for a surgical procedure should be considered early.⁵

In any phase of its natural history, ulceration is the most common complication, with an incidence rate of 23%,⁶ and may require surgical intervention.⁷ Other complications, such as visual and cardiac impairment, airway and auditory canal obstruction, amounted to 10.2% of all complications.⁶

Case report

An 8 month-old female patient living in Recife, state of Pernambuco, Brazil, with an ulcerated lesion on the left ear, was admitted to the hospital with complaints of bleeding ear. Assessment by a vascular surgeon was requested. On examination, the patient presented with well-demarcated, intense red nodules with telangiectasias on the ulcerated, bleeding surface on the posterior side of the left ear, on the earlobe, with a lump on the inside of the ear (Figure 1). Local abscess formation was also observed.

No complementary exams were performed, because of a clear-cut clinical diagnosis. Surgical excision of the vascular tumor was indicated. Anesthesia was induced with sevoflurane through orotracheal intubation, with the patient lying in dorsal decubitus position. Antisepsis was performed and the surgical site was prepared; incision in the posterior side of the left ear and partial exeresis of the cavernous hemangioma were performed. After that, an incision inside the ear and total exeresis of the hemangioma were performed. Finally, hemostasis was achieved, closure was performed and the surgical wound was closed. The postoperative course was uneventful (Figure 2). Anatomopathological examination revealed a proliferation of vascular channels lined by a layer of endothelial cells in a milieu of smooth connective tissue, interspersed in mononuclear infiltrate, thus confirming the diagnosis of cavernous hemangioma.

Discussion

Hemangiomas are characterized by rapid growth after birth followed by slow spontaneous involution; it has a 5:1 female predominance.² In 90% of the cases, the lesion is detected during the first month of life and, in 73% of the cases, it affects the head and neck region.¹

The first months of life are a critical time in hemangioma growth; children with this tumor require close observation and should be referred to a physician as early as complications appear.⁸ In almost all cases, diagnosis can be established exclusively on the basis of physical findings and clinical history, and an expectant management is preferred.⁹

Tumor growth is caused by proliferation of endothelial cells due to stimulators of angiogenesis.¹ In the proliferative phase, aggregates of proliferating endothelial cells arranged in a line or mass are observed, with lumen formation at times; thrombosis and hemosiderin deposits are rare and limited to areas of ulceration and inflammation; in the involution phase, endothelial cells are flattened and fibrofatty tissue gradually replaces the tumor area.²

About 24% of hemangioma patients experience complications.⁶ Ulceration is the most frequent complication (5 to 13% of cases) in the rapidly proliferating phase⁷ and usually occurs around the 4th month of life; bleeding occurs in 41% of ulcerated lesions, and the incidence of infections in these lesions is of 16%.¹⁰ Secondary infection is common and is usually restricted to skin, but it can also affect deep structures. Moderate bleeding may occur, which respond well to direct pressure. More severe cases, which demand surgical intervention, are rare.¹¹

The incidence of complications and need for treatment depend on the anatomical location of the hemangioma; 43% of complications occur in face and neck tumors.⁶ Congestive heart failure is a rare complication that may be associated with large or multiple lesions; treatment is focused on reducing blood volume in order to induce regression of the lesion Hypothyroidism can also be associated with giant hemangiomas that, due to high vascularization and type 3 iodothyronine deiodinase activity, can cause inactivation of thyroid hormone. Visual impairment can occur when a periorbital hemangioma provokes occlusion of the visual axis, compression of the eye globe or when the hemangioma invades the retrobulbar space. Respiratory compromise can result from airway obstruction by hemangiomas located in the nasal passages, oropharynx or trachea/larynx. Hearing impairment may result from obstruction of the external auditory canal. Finally, disfigurement can be a consequence of large hemangiomas in the face and neck; their residual scars can leave deformities that are difficult to correct, because regression is slow and incomplete in these areas.²

It is estimated that only 10 to 20% of hemangiomas require treatment.⁹ These include all complications mentioned above. For the management of hemangiomas, the following aspects should be considered: patient age, size, number and location of lesions, their current stage and presence of other associated symptoms.¹²

For most hemangiomas, indication is for conservative management, which includes corticosteroid therapy (systemic, topical, and local injection), interferon-alpha, laser, embolization, cryotherapy, and radiation therapy.¹³ Corticosteroids and interferon can cause adrenal failure; laser therapy can result in scars, skin atrophy, transient or permanent hyperpigmentation, as well as increased costs for the patient; cryotherapy can also cause scars and hyperpigmentation.¹³ Sclerotherapy is also used; among its side effects are thrombophlebitis, pulmonary thromboembolism, skin necrosis, and anaphylaxis.¹⁴

Surgical excision is one of the fundamental resources for hemangioma management and has specific indications, mainly during the proliferative phase. It is the treatment of choice in cases of cosmetic disfigurement and the consequent negative impact on the child's personality and self-esteem.¹⁵ Surgery is usually indicated in emergency cases, when there is no response to systemic forms of treatment, or for cosmetic reasons. It can be performed as embolization, selective arterial ligation, or excision, with or without reconstructive plastic surgery.⁹

References

• 1. Campos HGA, Curado JH. Angiodisplasias. In: Pitta GBB, Castro AA, Burihan E, editores. Angiologia e cirurgia vascular: guia ilustrado. Maceió: UNCISAL/ECMAL & LAVA; 2003. http://www.lava.med.br/livro
• 2. Gontijo B, Silva CMR, Pereira LB. Hemangioma da infância. An Bras Dermatol. 2003;78:651-73.
• 3. Mulliken JB, Fishman SJ, Burrows PE. Vascular anomalies. Curr Probl Surg. 2000;37:518-84.
• 4. Enjolras O, Mulliken JB. Vascular tumors and vascular malformations (new issues). Adv Dermatol. 1997;13:375-422.
• 5. Campos HGA, Curado JH. Angiodisplasias. In: Maffei FHA, Lastória S, Yoshida WB, Rollo HA, Giannini M, Moura R. Doenças vasculares periféricas. 4Ş ed. Rio de Janeiro: Guanabara Koogan; 2008. Vol. 2, p. 1949-77.
• 6. Haggstrom AN, Drolet BA, Baselga E, et al. Prospective study of infantile hemangiomas: clinical characteristics predicting complications and treatment. Pediatrics. 2006;118:882-7.
• 7. Garzon MC, Frieden IJ. Hemangiomas: when to worry. Pediatr Ann. 2000;29:58-67.
• 8. Chang LC, Haggstrom AN, Drolet BA, et al. Growth characteristics of infantile hemangiomas: implications for management. Pediatrics. 2008;122:360-7.
• 9. Bruckner AL, Frieden IJ. Hemangiomas of infancy. J Am Acad Dermatol. 2003;48:477-93.
• 10. Chamlin SL, Haggstrom AN, Drolet BA, et al. Multicenter prospective study of ulcerated hemangiomas. J Pediatr. 2007;151:684-9.
• 11. Esterly NB. Haemangiomas. In: Harper J, Oranje A, Prose N, editors. Textbook of pediatric dermatology. Oxford: Blackwell Science; 2000. p. 997-1016.
• 12. Frieden IJ. Management of hemangiomas. Special symposium. Pediatr Dermatol. 1997;14:57-83.
• 13. Selim H, Selim A, Khachemoune A, Metwally SA. Use of sclerosing agent in the management of oral and perioral hemangiomas: review and case reports. Med Sci Monit. 2007;13:114-9.
• 14. Belzunce A, Casellas M. Complications in the evolution of haemangiomas and vascular malformations. An Sist Sanit Navar. 2004;27 Suppl 1:57-69.
• 15. Waner M, Murillo J, Aubá C, Hontanilla B. Surgical treatment of haemangiomas. An Sist Sanit Navar. 2004;27 Suppl 1:93-101.

Publication Dates

• Publication in this collection
  04 Sept 2009
• Date of issue
  Sept 2009