28 Juvenile Angiofibroma with Intracranial Extension

Philippe Lavigne, Carl H. Snyderman, Paul A. Gardner


Advanced juvenile angiofibromas (JAs) are challenging tumors to treat surgically due to the involvement of skull base structures and vascularity derived from the intracranial circulation. Tumor infiltration of the pterygoid base and the pterygoid canal allows development of blood supply from branches of the internal carotid artery (ICA). Large tumors may surround the petrous and cavernous segments of the ICA and increase the risk of vascular injury during surgery.

With proper planning, large JAs with intracranial extension can be managed using endoscopic techniques. The biggest challenges are bleeding from tumor vessels derived from the intracranial circulation. The The University of Pittsburgh Medical Center (UPMC) staging system is useful in planning a surgical strategy based on the degree of residual vascularity and the route of intracranial extension. A staged approach with excision of vascular territories of the tumor allows safe resection with minimal morbidity. There is a higher risk of residual tumor with advanced JA with skull base involvement, but not all patients require further surgery. Radiation therapy can be avoided with a comprehensive surgical strategy.

28 Juvenile Angiofibroma with Intracranial Extension

28.1 Introduction

Juvenile angiofibromas (JA) are challenging tumors to treat surgically because of their vascularity and anatomical location. They likely originate from remnants of the first branchial arch artery and some authors consider them to be vascular malformations. 1 , 2 Traditionally, JAs are classified as benign tumors with locally aggressive features. They arise from the lateral basisphenoid and extend through foramina and fissures: (1) the sphenopalatine foramen into the nasopharynx and paranasal sinuses, (2) the pterygomaxillary fissure into the infratemporal fossa, and (3) the infraorbital fissure into the orbit. JAs have the potential for skull base erosion and intracranial extension, as seen in 10 to 20% of cases. 3 True dural invasion is rare and the tumor will usually have a well-circumscribed pushing border. Tumors that extend medial to the orbital apex can erode the bone of the planum sphenoidale to gain access to the anterior cranial fossa. JA can also extend to the middle cranial fossa through the inferior orbital fissure, orbital apex, and superior orbital fissure.

Recent advances in endoscopic endonasal surgery (EES) now allow surgeons to resect advanced JAs. A growing body of evidence supports purely endoscopic and endoscopic-assisted approaches. Case series and systematic reviews demonstrate favorable rates of residual and recurrent disease with reduced morbidity (▶ Table 28.1). 4 6

Table 28.1 Available multicenter and systematic reviews on the endoscopic approach for JAs




Study groups


Langdon et al 4


Multicenter retrospective review

Endoscopic approach (74) for stage IIIA and IIIB

Average blood loss 1279.7 mL

Residual: 33.3% (18/54) (16 observed and stable, 1 adjuvant radiation therapy, 1 revision surgery and radiation therapy)

Khoueir et al


Systematic review

821 cases, all endoscopic

Average blood loss 564 mL

Complication rate 9.3%

Residual 7.7%

Recurrence 10%

Boghani et al 6


Systematic review

Individual patient data: 158 endoscopic, 15 combined, 172 open

Average blood loss: Endoscopic 544.0 mL (20–2000 mL) vs. open 1579.5 (350–10,000 mL) When controlled for tumor stage, recurrence rates in endoscopic group are at least as good as open

28.2 Preoperative Evaluation and Anesthesia

Evaluation of tumor extension and staging is mandatory for optimal surgical planning. Computed tomography (CT) and magnetic resonance imaging (MRI) are both essential to assess tumor extension and can be used for intraoperative image guidance. Imaging should be obtained prior to angiographic embolization as embolized tumor segments are more challenging to delineate. CT is superior for identification of bony landmarks and remodeling and destruction of bone. Characteristic findings include anterior bowing of the posterior wall of the maxillary sinus (Holman–Miller sign) and tumor infiltration of the pterygoid base. 7 , 8 CT angiogram is useful for intraoperative navigation of the internal carotid artery (ICA). MRI is better for assessing soft tissues and intracranial extension. On MRI, JAs appear isointense to hyperintense on T1- and T2-weighted sequences. Flow voids related to small high-flow vessels supplying the tumor can be seen. There is intense enhancement following administration of gadolinium contrast.

Angiography is used to identify vascular contributions to the tumor. Combined with embolization, it reduces blood loss that improves visualization and tumor resection. It is commonly performed 24 to 48 h before the surgery. 8 In advanced JAs, both external carotid artery (ECA) and ICA can supply feeding vessels to the tumor. Bilateral vascular supply is also common in advanced tumors. 9 Residual vascularization from the ICA following embolization of ECA branches is predictive of increased operative blood loss. 10 Although feeders from the ICA can be embolized, the risk of complications (stroke, visual loss, facial paralysis, carotid dissection) is significant and its routine use is not recommended. 11 14 Preoperative balloon occlusion testing of the ICA may be considered with extensive tumors that encase the ICA, especially in surgical or radiation failures where risk of vessel injury is higher. This will assess the adequacy of the collateral circulation and the feasibility of carotid sacrifice, if necessary.

UPMC staging system (▶ Table 28.2 and ▶ Fig. 28.1) accounts for tumor vascularity derived from intracranial circulation and the route of intracranial extension. 10 , 15 Compared to previous staging systems, the UPMC staging system demonstrates stronger correlation with intraoperative blood loss, need for multiple surgeries (staged), and residual or recurrent tumor.

Fig. 28.1 The University of Pittsburgh Medical Center (UPMC) staging system: Axial computed tomographic (CT) scans showing tumor confined to the left nasal cavity and medial pterygopalatine fossa (UPMC stage I) (a) and tumor involving left lateral pterygopalatine fossa (UPMC stage II) (b). Figure panels c and d are from the same patient in whom the axial CT scan (c) shows tumor involving the left infratemporal fossa and an angiogram (d) shows no residual vascularity after embolization of the external carotid artery tributaries (UPMC stage III). Figure panels e and f are from the same patient in whom the axial CT scan (e) shows tumor involving the infratemporal fossa and angiogram (f) with residual vascularity from the internal carotid artery after embolization of the external carotid artery tributaries (UPMC stage IV). Figure panel (g) is an axial CT scan showing tumor lateral to the cavernous internal carotid artery (UPMC stage VL). (Reproduced with permission of Snyderman CH, Pant H, Carrau RL, et al. A new endoscopic staging system for angiofibromas. Arch Otolaryngol Head Neck Surg. 2010;136:588–594.)

Table 28.2 UPMC staging system


Staging criteria


Nasal cavity, medial PPF No residual vascularity


Paranasal sinuses, lateral PPF No residual vascularity


Skull base, orbit, infratemporal fossa No residual vascularity


Skull base, orbit, infratemporal fossa With residual vascularity


Intracranial extension: medial; lateral With residual vascularity

Advanced JAs demand special anesthetic consideration. Prior adjuvant radiation therapy, angiographic embolization, or surgical dissection of the masticatory space can result in difficult intubation due to trismus. 16 Large tumors may displace the soft palate inferiorly or extend to the oropharynx. With intracranial extension, increased intracranial pressure (ICP) may be present. 17 Measures to be considered in this setting are: smooth induction of anesthesia, invasive monitoring of blood pressure, and promotion of moderate hyperventilation. Invasive hemodynamic monitoring will provide safe hypotensive anesthesia and guide intravascular replacement strategies, if needed. In EES for advanced JAs, the average blood loss is more than 1000 mL. 4 In a patient population with low intravascular volume, excessive blood loss with consumption, dilution, and dysfunction of clotting factors and platelets can result in intraoperative coagulopathy. 18 Monitoring with thromboelastometry and early replacement of clotting factors are part of the management. 19 Restrictive transfusion strategies have been proposed to reduce the potential for transfusion-related complications (at Hb of 7–8 g/dL). 20 However, a strict transfusion threshold is difficult to recommend as this decision depends on many factors such as patient comorbidities and ongoing surgical variables. 18 Staging of surgery should be considered for large UPMC stage IV/V tumors if blood loss is excessive (50–100% of total blood volume), especially if significant tumor remains.

In patients where there is risk of ICA injury, neurophysiologic monitoring of cortical function (somatosensory-evoked potentials) is performed to detect global brain ischemia. Cranial nerves with motor function (III, IV, VI, and V3) can be monitored with electromyography (EMG). EMG aids in detection and protection of nerves with dissection and provides prognostic information for recovery. 21

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Feb 8, 2021 | Posted by in HEAD AND NECK SURGERY | Comments Off on 28 Juvenile Angiofibroma with Intracranial Extension

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