Diagnosis: Lobular capillary hemangioma (LCH)
Lobular capillary hemangioma is a benign, rapidly growing lesion of unknown origin. In the past, various terms, such as pyogenic granuloma, telengiectasic granuloma, granuloma pedunculatum, and infected granuloma, have been used to describe this disorder. At present, LCHis the preferred term because it best describes the lesion's histologic features, which are characterized by a proliferation of capillaries arranged in lobules in an edematous and fibroblastic stroma.1
This uncommon vascular lesion occurs in the skin and mucous membranes, and the oral cavity represents the most frequently involved region in the head and neck area.2,3Although the nasal area has been reported as the second most frequently involved site of LCH,1this location is generally considered unusual.2,4The disease occurs most often in the third decade of life,3with a female predominance, whereas its occurrence in pediatric populations has been described only in case reports. To the best of our knowledge, only 9 children with nasal LCH have been described in the English-language literature.5To date, our patient represents the youngest reported case of nasal LCH.
The pathogenesis of LCH is unclear. Nasal trauma and hormonal imbalances have been described as favored causes,2,3but other etiologic hypotheses, such as arteriovenous malformations, viral oncogenes, and the production of angiogenic growth factors, have also been proposed.6Even though not reported by the parents in the present case, routine nasal toilette with flexible aspirators performed after delivery could have caused traumatic injury of the nasal septum. This mucosal damage may have favored the formation of an LCH that increased in the following months. Physiologic nasal breathing at birth and its successive worsening until complete nasal obstruction develops support this hypothesis.
Lobular capillary hemangioma usually manifests as recurrent unilateral epistaxis, nasal obstruction, purulent nasal discharge, facial pain, hyposmia, and, rarely, headache.3,6On nasal endoscopy, the lesion appears as a reddish polypoid mass that bleeds easily and is generally located at the anterior portion of the nasal septum. In 1997, Bhattacharyya et al7coined the term giant LCHto describe those lesions that completely occupy the nasal fossae. As evidenced by the admission MRIs, the LCH in the present case could be considered giant.
When a typical nasal LCH is small, diagnosis is not difficult. However, problems can arise when the macroscopic appearance is unclear and, above all, when the mass is large. In these situations, imaging is mandatory. Although the findings are not specific, computed tomography shows nasal masses with soft-tissue density, whereas MRI reveals lesions with an intermediate to hyperintense signal on T2-weighted images and a hypointense signal on T1-weighted images. Enhancement after contrast administration can be helpful.8However, these diagnostic assessments can reveal important characteristics of the lesion such as size, probable site of origin, and vascularization pattern. Furthermore, imaging studies can provide additional information (eg, bone erosion and connection to the roof of the nasal cavity) that can be suspected in other disorders such as meningocele and malignant neoplasms. The present patient underwent MRI of the sinuses, which revealed a nasal vascularized mass totally filling the left nasal cavity, with dislocation of the nasal septum and nasal lateral wall in the absence of bone erosion. Given their large size, the hypoenhancing areas within the lesion could represent fibrosis rather than vascular flow voids.
The differential diagnosis of LCH includes other unilateral intranasal masses such as nasal polyp, antrochoanal polyp, angiomatous polyp, cavernous hemangioma, juvenile angiofibroma, hemangiopericytoma, hemangioendothelioma, paraganglioma, schwannoma, meningocele, meningoencephalocele, glioma, angiosarcoma, lymphoma, Kaposi sarcoma, olfactory neuroblastoma, and vascularized metastases.2,3,8,9Because LCH does not regress spontaneously,3the treatment of choice is total excision. Even if surgery can be performed through an endoscopic or external approach, endoscopic surgery is currently considered the criterion standard for the treatment of both small and large LCHs.6This surgical technique is less invasive, does not alter skeletal growth, avoids aesthetic damage, and allows good control of bleeding. Our patient's LCH was successfully removed with a diode laser through a transnasal endoscopic approach, with no perioperative complications. In conclusion, nasal LCHs are rare in the pediatric population. To our knowledge, the present case represents the youngest child described to date. Imaging studies are mandatory in cases involving large lesions. Radical excision by endoscopic sinus surgery is the ideal treatment for nasal LCH.
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Radiology Quiz Case 2: Diagnosis. Arch Otolaryngol Head Neck Surg. 2010;136(11):1143. doi:10.1001/archoto.2010.190-b