Treatment Of Relapse Of Juvenile Angiofibroma

Follow-Up

There are two different types of persistent lesions: those left intentionally because their resection would require unacceptable neural damage, and those left due to intraoperative oversight (Figs. 8.37, 8.38). In both cases, proper management mandates a precise assessment of site, size, and extent of the lesion.

Recurrences are a peculiar characteristic of juvenile angiofibroma. In fact, most authors doubt this theory and consider more likely the hypothesis of incomplete excision leaving lesion remnants (Andrews et al. 1989; Chagnaud et al.1998; Lloyd et al. 2000b; ).

To support this hypothesis is the fact that most persistent lesions occur within months or a few years r

Sphenopalatine Artery

Fig. 8.36a,b. Juvenile angiofibroma. DSA, before (a) and after (b) embolization. The juvenile angiofibroma is fed by terminal branches of the sphenopalatine artery, it occupies the nasal fossa and the nasopharynx; upwards it extends into the sphenoid sinus, downwards it reaches the superior part of the oropharyngeal lumen. Embolization permits complete devas-cularization of the mass

Fig. 8.36a,b. Juvenile angiofibroma. DSA, before (a) and after (b) embolization. The juvenile angiofibroma is fed by terminal branches of the sphenopalatine artery, it occupies the nasal fossa and the nasopharynx; upwards it extends into the sphenoid sinus, downwards it reaches the superior part of the oropharyngeal lumen. Embolization permits complete devas-cularization of the mass of primary treatment and more commonly are found in anatomical areas difficult to reach at surgery. This presumption is consistent with Chagnaud et al. (1998) who demonstrated that lesion remnants were already detectable at the first follow-up with cross sectional imaging.

Persistent lesions are more frequent when the primary juvenile angiofibroma invades the infratemporal fossa, sphenoid sinus, pterygoid root, clivus, and cavernous sinus (Herman et al. 1999; Howard et al. 2001). According to McCombe et al. (1990), "recurrences" are also related to primary juvenile angiofi-broma size, being more frequent in large lesions.

Nasopharynx And Cavernous Sinus

Fig. 8.37a,b. Follow-up of juvenile angiofibroma. SE T1 before (a) and after (b) contrast administration, axial plane. At 4 years after incomplete surgical resection a residual submucosal lesion is detected within the greater wing of sphenoid bone (black arrows). b After contrast administration, the lesion shows the typical enhancement of juvenile angiofibroma. Posteriorly it is in close contact with the carotid canal (black arrowhead). Only a smooth re-epithelized mucosa is visible from the nasal fossa (white arrows)

Fig. 8.37a,b. Follow-up of juvenile angiofibroma. SE T1 before (a) and after (b) contrast administration, axial plane. At 4 years after incomplete surgical resection a residual submucosal lesion is detected within the greater wing of sphenoid bone (black arrows). b After contrast administration, the lesion shows the typical enhancement of juvenile angiofibroma. Posteriorly it is in close contact with the carotid canal (black arrowhead). Only a smooth re-epithelized mucosa is visible from the nasal fossa (white arrows)

Juvenile Angiofibroma

Fig. 8.38a-c. Juvenile angiofibroma. Same patient as in Fig. 8.37. Enhanced T1 sequences in the coronal plane. Pretreatment examination for planning sub-total resection of the residual juvenile angiofibroma (a). Follow-up study after 13 months (b) and 18 months (c) after surgery. During surgery, a part of the lesion was intentionally left within the greater wing of the sphenoid bone, due to its relevant lateral extension and due to middle cranial fossa extradural invasion. Both the superior (black arrows) and medial (white arrows) limits of the lesion appear rather concave on preoperative and first follow-up MR examination, whereas 18 months after surgery their surface results more or less convex. This change in shape indicates progression of the lesion

Fig. 8.38a-c. Juvenile angiofibroma. Same patient as in Fig. 8.37. Enhanced T1 sequences in the coronal plane. Pretreatment examination for planning sub-total resection of the residual juvenile angiofibroma (a). Follow-up study after 13 months (b) and 18 months (c) after surgery. During surgery, a part of the lesion was intentionally left within the greater wing of the sphenoid bone, due to its relevant lateral extension and due to middle cranial fossa extradural invasion. Both the superior (black arrows) and medial (white arrows) limits of the lesion appear rather concave on preoperative and first follow-up MR examination, whereas 18 months after surgery their surface results more or less convex. This change in shape indicates progression of the lesion

Depending on the clinical condition (known or high-risk remnant lesion vs low-risk), follow-up will be scheduled: every few months for known remnants/high risk patients and every 6 months during the first year, then yearly, for the others, respectively (Chagnaud et al. 1998; Roger et al. 2002). In the first case, the goal of imaging is to detect changes in size of known remnants in order to decide the proper treatment strategy or to identify lesions arising from potential sites of persistence. In the second case, follow-up should be extended until adulthood, even though date supporting this strategy have not been provided yet.

Of course, unexpected persistent lesions show the same imaging characteristics as the primitive ones (Fig. 8.39), but their detection may be hampered by postoperative changes. These consist of altered bony and soft structures, due to surgical resection, healing, and chronic inflammatory re

Juvenile Angiofibroma Endoscopic

Fig. 8.39a-d. Recurrent/persistent juvenile angiofibromas in two different patients. CT after contrast administration, axial plane (a); endoscopic view (b); enhanced VIBE sequence in sagittal and coronal planes (c,d). a A thin plaque of non-enhancing scar tissue is detected at the level of sphenopalatine foramen (white arrow). The submucosal relapse (black arrows) has its epicenter at the pterygoid root level where the medullary bone is replaced by the enhanced juvenile angiofibroma (asterisk). Erosion of pterygoid bony boundaries is seen (arrowheads). The encircled area corresponds to the endoscopic view (b), which does not show any abnormality. c,d In a different patient, a relapsing juvenile angiofibroma is detected 2 years after endonasal surgery (arrows) located submucosally within residual sphenoid sinus floor

Fig. 8.39a-d. Recurrent/persistent juvenile angiofibromas in two different patients. CT after contrast administration, axial plane (a); endoscopic view (b); enhanced VIBE sequence in sagittal and coronal planes (c,d). a A thin plaque of non-enhancing scar tissue is detected at the level of sphenopalatine foramen (white arrow). The submucosal relapse (black arrows) has its epicenter at the pterygoid root level where the medullary bone is replaced by the enhanced juvenile angiofibroma (asterisk). Erosion of pterygoid bony boundaries is seen (arrowheads). The encircled area corresponds to the endoscopic view (b), which does not show any abnormality. c,d In a different patient, a relapsing juvenile angiofibroma is detected 2 years after endonasal surgery (arrows) located submucosally within residual sphenoid sinus floor action of the sinonasal mucosa (Chagnaud et al. 1998).

Bony defects of the sinonasal framework and skull base mainly depend both on the specific surgical approach and on the extent of the erosion caused by juvenile angiofibroma.

However, some constant postoperative findings appear to be the partial or total excision of the posterior, and medial antral walls, of the pterygoid root and of the floor of the sphenoid sinus, detectable in almost half of the patients, regardless of the surgical approach (Fig. 8.40).

The scar tissue replacing the site previously occupied by juvenile angiofibroma - pterygopalatine fossa, inferior orbital fissure, infratemporal fossa - appears usually hypointense on both T1 and T2 and does not enhance on CT or MR after contrast agent administration. Conversely, enhancement is shown either by the thickened inflamed sinonasal mucosa or by lesion remnants. Of course, inflammatory mucosal changes appear hyperintense also on T2 sequences, whereas juvenile angiofibroma has intermediate signal intensity. In addition, signal voids may be observed, their detection probably depending on the overall size of the persistent juvenile angiofibroma.

Nevertheless, it is not infrequent to image enhancing submucosal areas filling previous site(s) of the lesion. Serial examinations appear necessary to effectively estimate their growth, a finding that should be consistent with residual lesions (Fig. 8.41). However, known lesion remnants - left in place during surgery - may not show increase in size (Deschler et al. 1992). Angiography has been advocated to obtain a definite diagnosis, but it is hampered by false positive results (Bremer et al. 1986).

Owing to this limitation, and because the surgical treatment of small residuals - particularly those in-tracranially located - is rather controversial, the role

Endoscopy Surgery Juvenile Angiofibroma

Fig. 8.40a-d. Juvenile angiofibroma: postsurgical changes after endonasal surgery, normal findings. Enhanced SE T1 in the coronal plane (a,b), TSE T2 in the axial plane (c), endoscopic view (d). Thickening of the mucosal layer of both maxillary and sphenoid sinuses and ballooning of the mucosa (with liquid content) is quite a common finding in early postoperative examinations. The encircled area corresponds to the endoscopic view. NS, nasal septum; SS, sphenoid sinus; P, mucosa investing the posterior maxillary sinus wall; M, inferior border of the widened maxillary ostium; IT, inferior turbinate

Fig. 8.40a-d. Juvenile angiofibroma: postsurgical changes after endonasal surgery, normal findings. Enhanced SE T1 in the coronal plane (a,b), TSE T2 in the axial plane (c), endoscopic view (d). Thickening of the mucosal layer of both maxillary and sphenoid sinuses and ballooning of the mucosa (with liquid content) is quite a common finding in early postoperative examinations. The encircled area corresponds to the endoscopic view. NS, nasal septum; SS, sphenoid sinus; P, mucosa investing the posterior maxillary sinus wall; M, inferior border of the widened maxillary ostium; IT, inferior turbinate of angiography remains unclear (Chagnaud et al. 1998).

In effect, small submucosal residuals may be demonstrated by imaging modalities in otherwise asymptomatic patients with negative endoscopy. To adopt a proper treatment strategy, it would be necessary to know more about their spontaneous evolution: recurrent symptoms, regression of residual lesion, or persistent asymptomatic, residual complications. Moreover, spontaneous regression of juvenile angio-fibroma residues has been reported (Stansbie and Phelps 1986; Dohar and Duvall 1992).

A systematic postoperative imaging follow-up is, consequently, suggested. The use of MR is preferable because it avoids further radiation exposure of the young patients.

Collagenomas And Angiofibromas Patients

Fig. 8.41a-c. Recurrent/persistent juvenile angiofibroma (JA). SE T1 after contrast administration, coronal plane (a,b); endoscopic view (c). a Normal postsurgical MR findings are demonstrated in the first follow-up examination. b Patient did not follow a regular imaging and clinical follow-up. He presented 3 years after surgery showing a recurrent/persistent lesion at the level of the pterygoid root (b) with a prevalently exophytic pattern of growth, detectable on endoscopic examination (c)

Fig. 8.41a-c. Recurrent/persistent juvenile angiofibroma (JA). SE T1 after contrast administration, coronal plane (a,b); endoscopic view (c). a Normal postsurgical MR findings are demonstrated in the first follow-up examination. b Patient did not follow a regular imaging and clinical follow-up. He presented 3 years after surgery showing a recurrent/persistent lesion at the level of the pterygoid root (b) with a prevalently exophytic pattern of growth, detectable on endoscopic examination (c)

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