Indian J Otolaryngol Head Neck Surg (Oct–Dec 2015) 67(4):417–421; DOI 10.1007/s12070-015-0822-y


Capillary Haemangioma: A Rare Vascular Tumour of the External Auditory Canal Saurabh Varshney • Manu Malhotra Navjot Kaur • Priyanka Gairola

Received: 27 October 2014 / Accepted: 5 January 2015 / Published online: 9 January 2015 Ó Association of Otolaryngologists of India 2015

Abstract Haemangiomas are relatively common in the head and neck region, but cases in the temporal bone are rare. Hemangioma of the external auditory canal (EAC) is a rare otologic entity. Up till now (till 2013), only 18 cases of hemangioma EAC have been reported in the English literature. It is commonly classified as capillary or cavernous hemangioma. According to the literature, this case represents the fourth patient with capillary hemangioma of the EAC. A 22-year-old male presented to our department with a 3 years history of right-sided aural fullness, mild pain and decreased hearing. There was no history of ear discharge, pulsatile tinnitus, vertigo and local trauma. Otomicroscopic and otoendoscopic examination revealed a smooth surface, reddish pulsatile mass arising from the right postero–superior portion of bony canal wall, which measured about 1 cm in diameter, obstructing 2/3rd of the EAC. The tympanic membrane was not seen. No facial nerve dysfunction was observed. Patient had moderate conductive hearing loss in right ear on audiology. HRCT temporal bone showed soft tissue mass involving the right EAC and middle ear. The lesion was excised via a postaural approach under general anesthesia. The tumor was a reddish, soft mass and measured 1.6 9 1.7 9 2.8 cm. The histopathologic assessment indicated a capillary hemangioma. Keywords Capillary hemangioma  Vascular tumour  External auditory canal

Introduction Hemangiomas of the head and neck region have been wellreported and are usually seen as benign childhood lesions that regress with age. Hemangiomatous lesions involving the external auditory canal (EAC), on the other hand, are rarely seen [1]. Only 18 such cases have been reported in English literature and out of which, only one with hemangioma of middle ear was extended to the mastoid cavity. In eight cases, the hemangioma was confined to middle ear while, in other nine cases there were isolate involvement of the EAC and/or tympanic membrane [2]. Haemangiomas are rare benign vascular tumours. They are relatively common in head and neck and can be classified as either capillary haemangioma, cavernous haemangioma or mixed. They are common in childhood and are commonly located on the cutaneous or mucosal surfaces of the oral and nasal cavities. Although rare, intratemporal hemangiomas may involve the EAC, tympanic membrane, facial nerve, middle ear space, and internal auditory canal. The common clinical manifestations of a hemangioma in the middle ear are hearing loss, pulsatile tinnitus and a reddish or purple mass seen on otoscopy [3]. Haemangiomas generally occur in the fifth decade, and are twice as common in males than in females [4]. EAC hemangiomas are rare. To the best of our knowledge, there have been only 18 cases presented in the world literature till now [5].

Case Report S. Varshney (&)  M. Malhotra  N. Kaur  P. Gairola Department of Otorhinolaryngology & Head Neck Surgery, All India Institute of Medical Sciences, Rishikesh 249201, Uttarakhand, India e-mail: [email protected]

We report a case of 22-year-old male, who presented to the out-patient department of All India Institute of Medical sciences, Rishikesh; with complaints of aural fullness, mild



pain and decreased hearing in the right ear, for the last 3 years with no history of ear discharge, pulsatile tinnitus, vertigo or aural trauma and had no evidence of facial palsy. Oto endoscopic examination of the right ear revealed a smooth-surfaced, oval-shaped, well-circumscribed, reddish, pulsatile, non-tender mass, about 1 cm in diameter, occupying two-thirds of the EAC, soft in consistency, compressible, and appeared to be arising from the right postero-superior portion of the bony canal wall (Fig. 1). The tympanic membrane could not be seen. There was no discharge. There was no facial nerve dysfunction. The left ear was normal on examination. Vestibular examination was normal and head and neck examination findings were not significant. Weber test at 512 Hz was lateralized to the right side, and bone conduction was greater than air conduction in the same ear. Pure-tone audiometry, showed moderate conductive hearing loss right ear with an average air-bone gap of 33 dB (pure conductive type at 250–2,000 Hz) (Fig. 2). High resolution contrast enhanced computed tomographic scanning (HR CECT) temporal bone showed poorly enhancing soft tissue mass involving the right EAC and middle ear. The ossicular chain was partially embedded in vascular tissue and there was no sign of bony erosion. The inner ear structures were normal and there were no intracranial extension noted (Fig. 3). The lesion was excised under general anaesthesia. Retroauricular incision was given and after meatotomy, mass was visualized. Intra canalicular incisions were made at 6 O’clock and 1 O’ Clock positions, mass was smoothly excised by elevating the tympanomeatal flap from the canal. Tympanic cavity was entered and remnants of the

Fig. 1 Otoendoscopic image, Right ear-smooth-surfaced, reddish, soft, pulsatile mass (arrow) occupying 2/3rd of the EAC


Indian J Otolaryngol Head Neck Surg (Oct–Dec 2015) 67(4):417–421

mass were seen occupying epitympanum. In order to obtain better exposure of the mass, atticotomy was done and mass excised along with malleus and incus as they were engulfed in the mass. The Eustachian tube opening was obliterated. Tympanic facial nerve was intact. The tumour was a reddish, soft mass and measured 1.6 9 1.7 9 2.8 cm. Type III tympanoplasty was done with temporalis fascia and splitskin graft (Fig. 4). Histopathological examination of the mass concluded that it was a capillary hemangioma, composed of numerous irregular thin walled vascular spaces of varying calibre, most of them closely arranged capillary-like channels. The intervening stroma showed fibrosis (Fig. 5). Post-operative period was uneventful. There was no evidence of residual disease or recurrence 3 months after the surgery.

Discussion Tumoral lesions of external ear canal are uncommon findings in ENT practice [6]. The most common localized benign vascular tumor of the head and neck is hemangioma, which can present in a number of forms. Arteriovenous malformations (AVMs) are lesions related to errors of vascular morphogenesis. These are almost always present at birth, manifest late in life and require treatment. There is no sex predilection. These must be differentiated from haemangiomas, which are neoplastic, affect females more than males, and may regress spontaneously. Histologically, haemangiomas show endothelial proliferation in contrast to AVM, which have none [7]. In 1982, Mulliken and Glowacki developed a system of classification that divided these, on the basis of their histological and natural history characteristics, into two categories: haemangiomas and vascular malformations. Haemangiomas, correctly defined as ‘‘capillary haemangiomas’’, are made up of closed arranged capillary-like channels secondary to a proliferative endothelial process that more frequently occurs in infancy and progressively disappears before the age of 5–6 years. In contrast, ‘‘cavernous haemangiomas’’ are made up of large cavernous vascular spaces and more frequently appear after the sixth decade of life; being vascular malformations, their clinical presentation, anatomical changes and progression are strongly correlated to infection, trauma, ligation, attempted excision or changes in serum hormone levels [8]. Mulliken and Glowacki classification was adopted at the 1996 meeting of the international society for study of vascular anomalies [9]. Hemangioma is a vascular lesion usually found in the dermis. It is more common in young children and it progressively disappears before the ages of 5–6 years. They occur in two developmental stages (1): a quick growth

Indian J Otolaryngol Head Neck Surg (Oct–Dec 2015) 67(4):417–421


Fig. 2 Pure-tone audiometry-Right ear moderate conductive hearing loss (average 33 dB)

Fig. 3 HR CECT temporal bone showing soft-tissue mass involving right EAC and middle

stage (proliferative stage), followed by a decline one. In histological terms, an endothelial hyperplasia and an increase of mastocytes is noticed in the proliferative stage. Fibrosis, fatty infiltration, cell reduction and number normalization of mastocytes are noticed in the decline stage (2). Hemangioma can be either capillary type or cavernous type when presenting large vascular spaces, which is more common in the skin and in the mucosa. In children, during the proliferative phase, it has been observed that the urine and serum levels of the basic fibroblasts growth factor (bFGF) have increased. Freedman (1972) was the first to report the presence of hemangioma in the outer ear, describing two male patients at their 60’s with lesion that started from the posterior wall of the EAC affecting TM [10]. Capillary haemangioma in the middle ear was first described by Jones in 1930 [6]. Hemangioma of the EAC is a rare otologic entity.

Temporal bone haemangiomas common presenting features, in the order of decreasing frequency, are conductive hearing loss, pulsatile tinnitus, bloody otorrhoea, otalgia, otitis media or facial palsy. It can be easily mistaken for other pathologies, due to the non specific symptoms and also its rarity. Differential diagnosis to be considered includes other vascular lesions (glomus tumour being the most common vascular tumour in the middle ear, high jugular bulb, aberrant intratympanic tympanic vessel, arteriovenous malformation), neoplastic lesions (meningioma, rhabdomyosarcoma, carcinoma, melanoma, pyogenic granuloma) or inflammatory lesions which are far more prevalent (cholesterol granuloma, ear polyp) [6]. Temporal bone hemangiomas have a predilection for three sites, which are in decreasing order of frequency, the geniculate ganglion, internal auditory meatus, and the origin of the chorda tympani [11].



Indian J Otolaryngol Head Neck Surg (Oct–Dec 2015) 67(4):417–421

Fig. 4 Per operative; a Mass in right EAC;b Mass being excised; c Excised mass (with atticotomy and removal of malleus and incus); d Type III tympanoplasty with temporalis fascia graft

Fig. 5 Histopathological image showing features of capillary hemangioma (H&E stained, 910)

Diagnosis is based on clinical evaluation, otoscopic examination, diagnostic tests include a pure tone audiometry, HRCT and Magnetic resonance imaging of temporal


bone, which are essential to delineate the extent of the tumour, the possible presence of osseous erosions and to perform differential diagnosis, which is better clarified by a histopathological examination. If retrotympanic, the differential diagnosis is the paraganglioma of the middle ear [9, 10]. Literature review revealed that almost all reported cases suggest that high resolution. CT scan and MRI as useful in evaluating this vascular tumour. CT scan provide a good assessment of the extent and bone MRI angiography the nature of the mass more definitely. However, one should bear in mind that MRI features of glomus tumours may resemble those of haemangioma [12]. The natural history of haemangioma in other head and neck region involves spontaneous involution. The age of onset may predict the possibility of spontaneous regression. However, haemangioma in the temporal bone is slightly different from other regions. It occupies a small, enclosed cavity and cause symptoms at early stage and one may not want to wait until complications arise. Generally, most advocate early resection. All the 18 reported cases were treated with surgery except for two cases. One had a spontaneous involution and the other was treated with radiotherapy. Complete surgical removal is the standard treatment for these lesions [3, 4]. The surgical approach depends on the

Indian J Otolaryngol Head Neck Surg (Oct–Dec 2015) 67(4):417–421

site and extent of the lesion. Small lesions confined to the external auditory meatus can be removed following transcanal approach [12] and lesions extending to middle ear cleft and mastoid require mastoidectomy with tympanoplasty and sometimes temporal bone resection [14]. The recurrence rate is relatively high and depends on the adequacy of surgical resection. Recurrence has been reported due to inadequate excision [14]. Apart from surgery, there are interesting development in alternative treatment modality. Kostrzewa et al. described the use of CO2 laser-assisted excision of tumour which reduced the risk of bleeding and allowed better visualisation [15]. Pavamani et al. reported the first time use of radiotherapy as the primary treatment of an inoperable capillary haemangioma of external and middle ear with a 5 years recurrence-free period [16]. Recent, propranol has been used extensively in treating haemangiomas in other body regions. However, its use in intratemporal haemangioma is limited by the low prevalence of such disease. All these alternatives are still relatively new and lack of good evidence to support their usage. The risk of irradiation induced malignancy has to be considered if primary radiotherapy were to be offered. These modalities might have a role to treat this condition conservatively, but further research and study would be required to establish their efficacy. Since surgery has given a good success rate with minimal morbidity, alternative modalities should only be applied to selected cases [17, 18]. Pre-operative embolization or embolization as treatment has been tried in some cases but is not essential for all the cases [13]. Carbon dioxide laser can be an alternative to the conventional surgery as it allows better visualisation of middle ear structures by reducing bleeding [19]. Watchful waiting is recommended for small asymptomatic lesions.

Conclusion Haemangiomas of EAC are rare benign vascular neoplasms. Their diagnosis and management is important because of their propensity for proliferation and osseous erosion. Definitive diagnosis is by histopathological examination. Treatment is excision and follow-up is essential.


References ¨ zkul H, Somay A (2009) A rare vascular tumor of the 1. Yas¸ ar H, O external auditory canal: the capillary hemangioma. Kulak BurunBogazIhtisDerg 19(4):212–215 2. Singh RK, Bhandary S, Tiwary A, Karki S (2008) Capillary hemangioma of tympanic cleft. Online J Health Allied Sci 7(4):8 3. Bovo R, Ciorba A, Castiglione A (2010) Cavernous hemangioma of external ear: case report and literature review. B-ENT 6:127–130 4. Blanco MA, Vazquez Barro JC, Amado ML, Santiago Freijanes MP, Vidal JM (2011) Capillary haemangioma of the middle ear: a case report. Acta Otorhinolaringol Espanola 62(1):74–76 5. Voon Hoong F, Fadzilah I, Iskandar H, Majid A, Goh BS (2013) Capillary haemangioma of external and middle ear. Brunei Int Med J 9(6):401–404 6. Mulliken JB, Glowacki J (1982) Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg 69:412–422 7. Meher R, Varshney S, Pant HC (2008) Arteriovenous malformation related to the pinna. Hong Kong Med J 14(2):157–159 8. Martines F, Bentivegna D, Maira E, Marasa` S, Ferrara S (2012) Cavernous haemangioma of the external auditory canal: clinical case and review of the literature. Acta Otorhinolaryngol Italica 32:54–57 9. Neto JFL, Miura, Saleh C, de Andrade M, Assmann M (2007) Hemangioma as mass of external ear canal. Int Arch Otorhinolaryngol 11(4):498–500 10. Yeo A, Majithia A, Kalan A (2008) Haemangioma or vascular malformation of the tympanic membrane? a case report and review of literature. Indian J Otolaryngol Head Neck Surg 60:59–61 11. Kojima H, Yaguchi Y, Moriyama H (2008) Middle ear hemangioma: a case report. Aurisnasus larynx 35(2):255–259 12. Bhat V, Salins PC, Bhat V (2014) Imaging spectrum of hemangioma and vascular malformations of the head and neck in children and adolescents. J Clin Imaging Sci 4:31 13. Hsueh PJ, Chen WY, Chiang YC, Lee FP (2007) Capillary hemangioma of the middle ear. Otolaryngology 136(4):666–667 14. Limb CJ, Mabrie DC, Carey JP et al (2002) Hemangioma of the external auditory canal. Otolaryngol Head Neck Surg 126:74–75 15. Kostrzewa JP, Bowman MK, Woolley AL (2010) Middle ear hemangioma: a novel treatment for a rare problem. Int J Pediatr Otorhinolaryngol 5:50–52 16. Pavamani SP, Surendrababu NRS, Ram TS, Thomas M, Viswanathan PN, Viswanathan FR (2007) Capillary haemangioma involving the middle and external ear: radiotherapy as a treatment method. Australas Rad 51:394–397 17. Jackson CG, Levine SC, McKennan KX (1990) Recurrent hemangioma of the external auditory canal. Am J Otol 11(2):117–118 18. Tau DH, Zhao SQ, Guo JZ (2000) Hemangioma of external ear; an analysis of 8 cases. Lin chuangEr Bi Yan HouKeZaZhi 14(1):5–6 19. Kumbhar S, Saraf R, Limaye U (2013) Middle ear and mastoid hemangioma treated by neurointerventionaltechniques. Indian J Otol 19:85–87


Capillary Haemangioma: A Rare Vascular Tumour of the External Auditory Canal.

Haemangiomas are relatively common in the head and neck region, but cases in the temporal bone are rare. Hemangioma of the external auditory canal (EA...
NAN Sizes 1 Downloads 10 Views