This study aimed to present the clinical features and surgical outcomes of juvenile nasopharyngeal angiofibroma patients who were surgically treated.

The medical records of 48 male patients histologically confirmed as having juvenile nasopharyngeal angiofibroma, who underwent transnasal endoscopic surgery between 2005 and 2016, were retrospectively reviewed.

The overall recurrence rate was 20.8 per cent; however, the recurrence rate differed significantly between patients diagnosed aged less than 14 years (34.7 per cent) and more than 14 years (8 per cent) (p < 0.05). Advanced-stage tumours (Radkowski stage of IIC or more, and Önerci stage of III or more) were more aggressive than earlier stage tumours (p < 0.05 and p < 0.01, respectively). Pre-operative embolisation significantly prolonged mean hospitalisation duration, but had no effect on intra-operative blood loss in patients with advanced-stage tumours (p < 0.001 and p = 0.09, respectively).

The findings show that transnasal endoscopic surgery could be considered the treatment of choice for juvenile nasopharyngeal angiofibroma. Patients diagnosed when aged less than 14 years and those with advanced-stage tumours are at risk of recurrence, and should be monitored with extreme care.

Juvenile nasopharyngeal angiofibroma often attaches firmly to the adjoining bony region around the sphenopalatine foramina–sphenopalatine fossa–pterygomaxillary fissure. This can result in hourglass-shaped constriction and predispose to incomplete resection (residual disease) with a transpalatal approach. This paper describes attempts to address this ‘inaccessible’ area with a novel instrument, used since 2012.

Measurements of the sphenopalatine foramen, nasal septum, posterior nasopharyngeal wall and hard palate were undertaken in 20 skulls and 10 computed tomography scans (lateral extension). A device was designed (in terms of angulation and length) following several trials with malleable wire. A search of patents was also undertaken. Recurrence rates were compared in cases of device use and non-use.

The novelty of the sphenopalatine fossa dissector was established and the device was patented. This device has significantly improved our 17.59 per cent recurrence rate of the past 4 decades; of 63 cases over 3 years, there were only 3 recurrences and 2 residual disease cases. Findings of our previous studies with or without the device are compared.

Existing evidence supports the incorporation of this inexpensive instrument in the armamentarium for resecting lateral extension of juvenile nasopharyngeal angiofibroma during a transpalatal approach.

Extra-nasopharyngeal angiofibroma is a rare but distinct clinical entity, different from juvenile angiofibroma.

This clinical record elucidates the only case of extra-nasopharyngeal angiofibroma arising from the septum in a female child, who presented with epistaxis.

The histopathological diagnosis was confirmed by immunohistochemistry, and the case was managed surgically with no recurrence.

In a female paediatric patient presenting with epistaxis, extra-nasopharyngeal angiofibroma (of the inferior turbinate) is a rare albeit important differential diagnosis, as it challenges the hormonal theory of angiofibroma aetiopathogenesis.

Molecular categorisation may explain the wide variation in the clinical characteristics of juvenile nasopharyngeal angiofibroma.

Variations in molecular markers in juvenile nasopharyngeal angiofibroma in an Indian population were investigated and compared with global reports.

Variable molecular marker expression was demonstrated at the regional and global levels. A wide variation in molecular characteristics is evident. Molecular data have been reported for only 11 countries, indicating a clear geographical bias. Only 58 markers have been studied, and most are yet to be validated.

Research into the molecular epidemiology of juvenile nasopharyngeal angiofibroma is still in its infancy. Although the molecular variation is not well understood, data obtained so far have prompted important research questions. Hence, multicentre collaborative molecular studies are needed to establish the aetiopathogenesis and establish molecular surrogates for clinical characteristics.

Epistaxis is the most common ENT emergency encountered in the Emergency Department. Most cases can be managed by simple anterior nasal packing. This is usually a safe and very effective option in an emergency situation, requiring minimal expertise and infrastructure. This paper describes a rare instance of a serious complication following anterior nasal packing in a case of nasopharyngeal angiofibroma.

A 27-year-old man diagnosed with nasopharyngeal angiofibroma presented to the Emergency Department with bilateral epistaxis. The patient was stabilised and anterior nasal packing was performed, which controlled the bleeding. Three hours later, the patient developed complete blindness in both eyes. Aggressive medical management was initiated immediately, but failed to restore the patient's vision.

Anterior nasal packing is a simple and minimally invasive procedure practised regularly in an Emergency Department setting. However, it can occasionally lead to serious complications such as blindness. Thus, obtaining informed consent is essential to avoid medico-legal consequences in high-risk cases.

Juvenile nasopharyngeal angiofibroma often presents with lateral extensions. In countries with limited resources, selection of a cost-effective and least morbid surgical approach for complete excision is challenging.

Sixty-three patients with juvenile nasopharyngeal angiofibroma, with lateral extensions, underwent transpalatal, transpalatal-circumaxillary (transpterygopalatine) or transpalatal-circumaxillary-sublabial approaches for resection. Clinico-radiological characteristics, tumour volume and intra-operative bleeding were recorded.

The transpalatal approach was suitable for extensions involving medial part of pterygopalatine fossa; transpalatal-circumaxillary for extensions involving complete pterygopalatine fossa, with or without partial infratemporal fossa; and transpalatal-circumaxillary-sublabial for extensions involving complete infratemporal fossa, even cheek or temporal fossa up to zygomatic arch. Haemorrhage was greatest with the transpalatal-circumaxillary-sublabial approach, followed by transpalatal approach and transpalatal-circumaxillary approach (1212, 950 and 777 ml respectively). Tumour size (volume) was greatest with the transpalatal-circumaxillary approach, followed by transpalatal-circumaxillary-sublabial approach and transpalatal approach (40, 34 and 29 mm3). There was recurrence in three cases and residual disease in two cases. Long-term morbidity included small palatal perforation (n = 1), trismus (n = 1) and atrophic rhinitis (n = 2).

These modified techniques, performed with endoscopic assistance under hypotensive anaesthesia, without embolisation, offer a superior option over other open procedures with regard to morbidity and recurrences.

The occurrence of juvenile nasopharyngeal angiofibroma is reportedly higher in India than in some other parts of the world, and our centre has seen a four-fold increase in its occurrence across seven decades.

This paper reports a retrospective archival analysis of 701 juvenile nasopharyngeal angiofibroma cases from 1958 to 2013, and considers probable environmental factors in an Indian context that may affect its biology and the global distribution, as reported in the literature.

A continuously progressive increase in occurrence was evident, but the rapid rise observed in the current decade was alarming. The world map of juvenile nasopharyngeal angiofibroma incidence does not reflect true global distribution given the paucity of reporting. Our centre has dealt with approximately 400 cases in the last 24 years.

With the alarming increase in juvenile nasopharyngeal angiofibroma incidence, there is a need for a registry to define its epidemiology. The world literature needs to reflect the status of juvenile nasopharyngeal angiofibroma incidence in the third world as well. Environmental factors known for hormone disruptive actions may influence its occurrence. Such aspects need to be considered to plan specific prevention policies.

Juvenile nasopharyngeal angiofibroma is a benign, vascular tumour that primarily occurs in adolescent males. Despite its benign nature, aggressive growth patterns can cause potential life-threatening complications. Juvenile nasopharyngeal angiofibroma is normally unilateral, originating from the sphenopalatine artery, but bilateral symptoms can occur if a large tumour extends to the contralateral side of the nasopharynx. This paper presents the first reported case of true bilateral extensive juvenile nasopharyngeal angiofibroma involving clinically challenging pre-surgical planning and surgical strategy.

A 21-year-old male presented with increasing bilateral nasal obstruction and discharge. Examination revealed tumours bilaterally and imaging demonstrated non-contiguous tumours. Pre-operative angiography showed strictly ipsilateral vascular supplies requiring bilateral embolisation. Radical removal performed as one-step, computer-assisted functional endoscopic sinus surgery was performed. The follow-up period was uncomplicated.

This case illustrates the importance of suspecting bilateral juvenile nasopharyngeal angiofibroma in patients presenting with bilateral symptoms. Our management, including successful pre-operative planning, enabled one-step total removal of both tumours and rapid patient recovery.

To discuss the minimally invasive treatment and prognosis of juvenile nasopharyngeal angiofibroma involving the eye and optic nerve.

Retrospective analysis of clinical data for 18 large juvenile nasopharyngeal angiofibromas, with reports of three typical cases.

The tumour invaded the orbit, eye, optic nerve and optic chiasm in 18, 9, 8 and 5 patients, respectively. Twelve patients were cured after surgery, with the affected eye and vision essentially returning to normal. In six patients, tumour residue was found in the middle cranial fossa; two of these six did not return for follow up. Four patients underwent radiotherapy; all four regained placement of the eye in a normal or near-normal position. One of these four patients regained normal visual acuity, two experienced no improvement in visual acuity, and one became blind.

In patients with this tumour, surgery may return the eye to the normal position and may restore visual acuity if the optic fundus, macula, retina and optic nerve are only mildly affected. Modern radiotherapy can treat the intracranial residue effectively. A combined approach via the antrum, infratemporal fossa and nasal cavity, using a Caldwell–Luc incision, provides minimally invasive surgical access.

To provide an update on the ever-increasing role that embolisation plays in the practice of otolaryngology.

A literature search was performed during November 2008. The Medline, Embase, PubMed and Cochrane databases were searched. This resulted in 285 papers relevant for review.

The role of embolisation has expanded greatly to include the management of refractory epistaxis, pre-operative preparation of vascular tumours, vascular injuries and as an adjunct in skull base surgery.

Juvenile nasopharyngeal angiofibroma may be successfully resected using endoscopic techniques. However, the use of coblation technology for such resection has not been described. This study aimed to document cases of Fisch class I juvenile nasopharyngeal angiofibroma with limited nasopharyngeal and nasal cavity extension, which were completely resected using an endoscopic coblation technique.

We retrospectively studied 23 patients with juvenile nasopharyngeal angiofibroma who underwent resection with either traditional endoscopic instruments (n = 12) or coblation (n = 11). Intra-operative blood loss and overall operative time were recorded.

The mean tumour resection time for coblation and traditional endoscopic instruments was 87 and 136 minutes, respectively (t = 9.962, p < 0.001). Mean intra-operative blood loss was 121 and 420 ml, respectively (t = 28.944, p < 0.001), a significant difference. Both techniques achieved complete tumour resection with minimal damage to adjacent tissues, and no recurrence in any patient.

Coblation successfully achieves transnasal endoscopic resection of juvenile nasopharyngeal angiofibroma (Fisch class I), with good surgical margins and minimal blood loss.

Surgical approaches to the pterygopalatine and infratemporal fossae are complex and cause significant morbidity. The commonest benign tumour to extend to the pterygopalatine and infratemporal fossae is angiofibroma.

This prospective study included 15 male patients aged 12–27 years with recurrent, severe epistaxis. After computed tomography and magnetic resonance imaging, a modified Wormald and Robinson's two-surgeon approach was used. Follow up, with endoscopy and magnetic resonance imaging, ranged from two to five years.

Twelve patients were cured (endoscopically and radiologically). Three patients suffered recurrence, one each in the lateral sphenoid wall, pterygoid canal and infratemporal fossa. Revision surgery was performed, but one patient suffered another recurrence (lateral sphenoid wall with cavernous sinus infiltration) and was referred for gamma knife surgery.

This endoscopic two-surgeon technique is an excellent approach for managing angiofibroma extending to the pterygopalatine and infratemporal fossae. Our modification markedly decreased morbidity by avoiding septum opening and sublabial incision, and by enabling better haemostasis (via maxillary artery control). Recurrence may be minimised by careful examination of the lateral sphenoid wall, pterygoid canal and infratemporal fossa pterygoid muscles.

To determine the effect of embolisation on endoscopic resection of angiofibroma.

A partially blinded trial was undertaken. Twenty-three patients with angiofibroma (nine embolised and 14 not embolised) underwent endoscopic resection between January 2007 and August 2008 in two tertiary referral centres. Demographic data were collected, the pre-operative tumour extent was assessed by computed tomography, and tumours were staged according to their computed tomography appearance (Radkowski scale). In addition, we evaluated the duration of surgery, amount of haemorrhage, blood pressure during surgery, duration of hospitalisation, complications of surgery and embolisation, cost of treatment, and number of post-operative recurrences, as well as the angiographic characteristics in the embolisation group.

There was no significant difference between the general characteristics of both groups. At the end of the study period, we could find no significant difference between the two groups regarding haemorrhage, number of recurrences or complications. The only significant difference was cost of treatment, which was significantly higher in the embolisation group.

Endoscopic resection is a feasible and safe method for angiofibroma surgery. The current evidence does not support obligatory embolisation in every case of endoscopic angiofibroma resection.

To evaluate the efficacy of endoscopic exposure and excision for juvenile nasopharyngeal angiofibroma.

Prospective analysis of the outcomes of endoscopic removal of the angiofibroma at a tertiary care centre, using endoscopic and radiological parameters.

Twenty-eight consecutive patients with angiofibroma were included. These patients underwent endonasal endoscopic excision of the lesion, following pre-operative computed tomography and digital subtraction angiography. Post-operative endoscopy and radiology were performed at six weeks to detect any residual lesion, and thereafter at six-monthly intervals to detect recurrence.

Twenty-one patients (75 per cent) were new cases and seven patients (25 per cent) had recurrent tumour. Pre-operative embolisation was performed in 21 patients (75 per cent). An endoscopic endonasal approach was used to access the pterygopalatine fossa. The average blood loss was 228 ml. Residual lesion was noted in one patient (initially with recurrent stage II C pathology). The remaining 27 patients (initially with stage I, II A and II B pathology) were free of residual pathology or recurrence after a minimum follow-up period of 12 months.

Endoscopic exposure and excision of angiofibroma was found to be effective.