A rare cause of paediatric epistaxis: lobular capillary haemangioma of the nasal cavity

Abstract

The authors describe a case of a 14-year-old male child presenting with massive anterior epistaxis on a background of recurrent episodes of epistaxis. Immediate management constituted anterior nasal packing. Endoscopic nasal examination revealed a 5 mm purple vascular lesion anterior to the right-middle turbinate. The initial working diagnosis of juvenile nasopharyngeal angiofibroma which is most common in this population was excluded following MRI. The lesion was excised via an endoscopic approach with no complications. Histological analysis confirmed the diagnosis of lobular capillary haemangioma. The patient made an uneventful recovery and remains on follow-up with no recurrence.

Background

One of the most important differential diagnoses in male teenagers is juvenile nasopharyngeal angiofibroma. This was the initial working diagnosis in this case. However, there are other rare vascular haemorrhagic lesions including lobular capillary haemangioma (LCH) as diagnosed in this case. These must be considered to prevent unnecessary exhaustive investigation and the possibility of making an erroneous diagnosis of idiopathic epistaxis, a diagnosis of exclusion.

Case presentation

A 14-year-old male teenager was transferred to our hospital following an episode of massive epistaxis. He had a prior several-month history of recurrent brief episodes of epistaxis. He had no other medical history and was not on medication. There was no family history of note.

Clinical examination was initially unremarkable. However, nasendoscopy revealed a dark purple lesion located just anterior to the right-middle turbinate (figure 1). No other lesions were identified.

Figure 1.

Lobular capillary haemangioma of the right nasal cavity.

Investigations

MRI was performed to assess the nasal cavity, in particular to exclude a diagnosis of juvenile nasopharyngeal angiofibroma.

Differential diagnosis

• ▶Juvenile nasopharyngeal angiofibroma
• ▶Idiopathic epistaxis.

Treatment

Initial epistaxis was managed conservatively with nasal packing. Subsequently, he underwent uneventful endoscopic excision of the lesion under general anaesthesia.

Outcome and follow-up

There has been no further episodes of epistaxis and no recurrence. He remains on long-term follow-up.

Discussion

LCH was first described as ‘botryomycosis humaine’ by Poncet and Dor in 1897.¹ It is a rare benign capillary haemangioma of unknown aetiology with a distinct microscopic lobular structure. LCH is made up of lobules of capillaries enclosed in fibrous tissue. It usually arises as a solitary lesion.²

In the paediatric population, there is a higher incidence in males, however, in adults it affects women more than men, particularly those in the third to fifth decade of life.^{3 4}

It affects the skin and mucosal lining of the oral cavity and nose.^{5 6} The nose is an unusual site of presentation. The most commonly affected sites within the nasal cavity are the anterior septum and the turbinates.

Trauma and pregnancy have been identified as predisposing factors in developing LCH, however, in the majority of reported cases an identifiable cause is not established. LCH is thought to be a benign neoplastic lesion of unknown aetiology rather than a reactive proliferative lesion, however, in pregnant patients there is marked regression of the lesion postpartum, suggesting hormonal influence.⁷

Epistaxis is the most common mode of presentation of LCH. However, as the lesion increases in size it may cause symptomatic unilateral nasal obstruction.⁶ Clinically, LCH presents as a friable pink-purple pedunculated or sessile lesion which bleeds easily.

CT imaging is the modality of choice in investigating LCH. It has the non-specific features of a soft tissue density mass with an iso- or hypoattenuating cap of variable thickness. There may be associated bony destruction or invasion of adjacent structures.⁸

Endonasal endoscopic excision is the standard treatment.² Recurrence of LCH in the reported literature is rare.

Learning points.

• ▶Epistaxis in the paediatric population is not always idiopathic.
• ▶Thorough clinical examination is important, including endoscopic examination in appropriate age groups.
• ▶Radiological investigation of the nasal cavity is important in assessing massive epistaxis in the teenage male population.
• ▶Histological analysis of specimens is important for establishing an accurate diagnosis in rare lesions.