Management of a recurrent right juvenile nasopharyngeal angiofibroma using direct tumorous puncture embolisation and a subtemporal-preauricular infratemporal fossa surgical approach

Background

The term ‘juvenile nasopharyngeal angiofibroma (JNA)’, first coined by Friedberg1 in 1940, is a rare, benign vascular tumour arising from the pterygopalatine fossa at the aperture of the pterygoid (vidian) canal. They account for 0.05%–0.5% of all head and neck tumours.2 3 These tumours are seen to arise exclusively in men with a typical age of presentation between 14 and 25 years.4 It should be acknowledged that some tumours have been reported in the literature to have occurred in women, though very rarely.5 6 The predilection for the male sex has been explored, and strong evidence reveals a hormonal influence by the presence of androgen receptors on these tumours.7 8

Symptoms arising from JNAs are due to their high vascularity and mass effect. Common presenting symptoms include: persistent nasal obstruction (80%–90%), recurrent epistaxis (60%) and nasal discharge (23%).3 4 9 Though benign, JNAs can invade surrounding structures such as the orbit, skull base and paranasal sinuses with resultant symptoms such as diplopia, headaches and facial deformity. Diagnosis is made clinically and radiologically, given their characteristic features on imaging. Biopsy should be avoided due to the high vascular nature of these tumours.

The mainstay of treatment for JNAs is surgical resection with complete excision being the goal. Due to the high vascular nature of these tumours and potential for significant blood loss intraoperatively, preoperative angioembolisation has become standard practice as an adjunct to reduce intraoperative bleeding. If tumour feeding vessels cannot be safely ligated via transarterial (TA) embolisation then direct tumorous puncture (DTP) embolisation should be considered.2

Surgical resection can either be carried out via an open/external approach or via an endonasal endoscopic or a combination of both depending on tumour size, extension as well as surgeon experience. Management of residual disease include further surgical resection, radiotherapy or simply close clinical monitoring, thereby reducing the risk of morbidity from multiple surgical procedures.10

This case report highlights the use of intratumorous embolisation and an open surgical approach using a subtemporal-preauricular infratemporal fossa approach for a recurrent JNA in an adult male.

Case presentation

An adult male in his 20s was admitted to the hospital for an elective resection of a recurrent right-sided skull base JNA. His previous operation was carried out 2.5 years prior and consisted of an endoscopic endonasal resection of the medial aspect of the skull base JNA after preoperative embolisation. The patient had previously underwent two prior endoscopic resections in Belgium. He was originally referred to the skull base team at our institution 3 years prior for further management of the recurrent JNA, after surveillance CT imaging showed interim growth of the tumour. At that time, he only complained of decrease sensation to the right side of his face in the distribution of the maxillary and mandibular division of the trigeminal nerve (V₂ and V₃). The patient had no perioperative or postoperative complications from the previous procedure. This operation would be open surgery and would consist of a lateral sub-temporal approach with orbital and zygomatic osteotomies as well as a coronoid osteotomy to excise the infratemporal and middle fossa component of the JNA (see figure 1A,B). Surgery specific risk factors were explained to the patient and included: facial nerve injury, trismus, facial numbness, diplopia, blindness, cerebrospinal fluid leak and stroke. Further embolisation of the tumour’s feeding vessels was planned preoperatively. Originally, this procedure was planned to occur 12 months after the previous operation; however, this was postponed due to the events of the COVID-19 pandemic. Specifically, as the patient would not be allowed visitors during his hospitalisation due to the government mandated restrictions,therefore, he chose to have this procedure done when restrictions were lifted.

In hospital, 5 days prior to his surgery, he underwent intratumorous embolisation using precipitating hydrophobic injectable liquid as well as coil embolisation of the superficial temporal artery. The ascending pharyngeal artery was also meant to be a target but was deemed too high risk for cranial nerve 9–11 deficits. Postembolisation, the patient remained well. In preparation for surgery, four units of packed blood cells were requested, and the cell salvage device was reserved for the operation. Preoperative blood investigations were normal.

Treatment

General anaesthesia was performed in the standard fashion. Monitoring included: blood pressure, pulse oximetry, ECG, arterial blood pressure as well as facial nerve monitoring. Incisions were marked, and the patient was prepped in the usual fashion. A right frontotemporal incision was made and extended inferiorly preauricularly into the cervical region two finger breadths below the angle of the mandible. The platysma and the superficial musculoaponeurotic system were raised in continuity with the subgaleal scalp flap. The main trunk of the facial nerve was identified and protected. The carotid bifurcation was located, and the external carotid artery was identified and protected with vascular loops. This allowed for vascular control inferiorly if excessive bleeding was encountered. Soft tissue and muscle layers were dissected off the orbital rim and zygomatic arch in preparation for the bony cuts. Orbitozygomatic osteotomies were then made. The temporalis and masseter muscles were reflected off their bony attachments allowing for a coronoidectomy. Now, with adequate exposure of the tumour, resection proceeded in an extracapsular fashion using bipolar dissection from a lateral to medial fashion. The tumour had eroded into the middle cranial fossa floor with extension medially to the foramen rotundum and ovale. Inferiomedially, the tumour extended beyond the lateral pterygoid plate up to the nasopharynx and sphenoid sinus. A pterional craniotomy was deemed necessary to develop a dissection plane along the middle cranial fossa, thereby allowing for safe resection of the tumour (see figure 2). V₂ and V₃ were sacrificed due to gross tumorous involvement. Resection was completed en bloc, and the specimen was sent for histopathological analysis. Surrounding tissue specimens were sent for histological analysis and included tissue from: middle cranial fossa floor, pterygopalatine fossa, right sphenoid and a level 2a lymph node. Medpor temporal fossa implant was inserted and secured with screws. Bone flaps were replaced and secured using miniplates and screws. Haemostasis was achieved, and a Jackson Pratt drain was inserted into the neck wound. The surgical wound was then closed in layers. A nasogastric tube (NGT) was placed prophylactically to supplement enteral diet in the event of postoperative swallowing dysfunction. The patient was extubated and transferred to the intensive care unit (ICU). Prophylactic antibiotics consisted of a 7-day course of ceftriaxone 1.5 g intravenously once a day and metronidazole 500 mg intravenously three times a day.

Outcome and follow-up

Postoperatively, the patient remained stable. His facial nerve was functioning normally; however, he did have weakness to the temporal branch supplying frontalis. This was expected due to traction on the nerve during the operation. He complained of hypoaesthesia to the right side of his face in the distribution of V₁–V₃. The patient remained on the ICU for 2 days before being transferred back to ward level care. During his ICU stay, he was noted on postoperative day 2 to have several temperature spikes; however, this was deemed as a result of postoperative surgical inflammation rather than a true postoperative infection as blood and urine cultures showed no bacterial growth. Postoperative contrast MRI of the head showed no gross tumour residuum (figure 2). However, due to postoperative tissue enhancement, it was difficult to assess for small volume tumour residuum. On the ward, he remained well and successfully had his NGT removed on postoperative day 8 after his swallowing and oral intake were deemed satisfactory by the speech and language and dietitian teams, respectively. He was discharged the same day from the hospital.

Histological analysis from the intraoperative specimens sent showed JNA tissue in all specimens except for the level 2 lymph node.

Five months postdischarge from hospital, the patient was admitted from nurse led wound clinic due to three small areas of wound dehiscence measuring (1.5×1.5 cm, 0.5×0.5 cm and 0.3 cm) along the temporal region of the wound. Exposed temporal fossa implant was noted at the deeper aspect of the dehiscence. Wound swabs taken at that time ruled out any evidence of infection. Blood laboratory investigations were normal. The rest of the clinical exam was normal aside from hypoaesthesia to the patient’s face along V₂ and V₃ distribution (long standing). MRI of the brain requested at this time did not show any evidence of infection within the intracranial cavity or gross tumour recurrence. After review by the neurosurgery and plastics’ team, the plan was for primary closure as a joint procedure on an elective basis. The wound was closed using a local rotational flap followed by primary closure after removal of the temporal fossa implant. He made a complete recovery and was discharged from the hospital 5 days after the operation. The temporal fossa implant cultured Staphylococcus aureus and Aspergillus fumigatus. The patient continues to be followed up at neurosurgery wound clinic.