Perioperative management of pediatric patients undergoing juvenile angiofibroma resection. A case series and educational review highlighting patient blood management.

BACKGROUND: Juvenile nasopharyngeal angiofibromas are one of the most enigmatic, bloody tumors encountered by otorhinolarygnologists, head and neck surgeons, neurosurgeons, and anesthesiologists. Juvenile nasopharyngeal angiofibromas are rare, benign, highly vascular tumors with a propensity towards aggressive local invasion. Surgery, open or endoscopic, to remove the growth is the primary treatment of choice for Juvenile nasopharyngeal angiofibromas. Historically, surgical resection was associated with massive, rapid blood loss, traditionally managed by blood product transfusion and deliberate hypotension. Preventative management employing multimodal blood conservation strategies should be an essential standard of perioperative care for patients with Juvenile nasopharyngeal angiofibromas. METHODS: We describe a contemporary and comprehensive approach in the management of patients with high grade Juvenile nasopharyngeal angiofibromas. This includes surgical strategies such as preemptive external carotid artery embolization, endoscopic surgical approach, and staged operations, as well as anesthetic strategies including antifibrinolytic therapy and acute normovolemic hemodilution. These surgeries, once synonymous with massive transfusion, may potentially be performed without allogeneic blood transfusion, or deliberate hypotension. AIMS: Using a case series, the authors introduce a contemporary approach to multimodal, multidisciplinary blood conservation strategies for Juvenile nasopharyngeal angiofibromas surgery. RESULTS: Here in the authors report on an updated contemporary perioperative clinical approach to patients with Juvenile nasopharyngeal angiofibromas. From an anesthetic perspective, we describe the successful use of normal hemodynamic goals, restrictive transfusion strategy, antifibrinolytic therapy, autologous normovolemic hemodilution, and early extubation in the care of three adolescent males with highly invasive tumors. We demonstrate that new surgical and anesthetic strategies have yielded a significant decrease in intraoperative blood loss and eliminated the need for transfusion of autologous red blood cells, which enable improved outcomes. CONCLUSIONS: The perioperative approach to elective surgery for Juvenile nasopharyngeal angiofibromas management is presented from a multidisciplinary patient blood management perspective.

Nasal septal angiofibroma: less vascular behaviour than observed in radiological imaging, a rare case in a 51-year-old woman.

Angiofibroma of the head and neck is classically found in the nasopharynx of an adolescent male. We present a rare case where an angiofibroma was diagnosed in the anterior nasal septum of a 51-year-old woman, the eighth nasal septal angiofibroma reported in a female. This case highlights how an angiofibroma in an older woman has a less vascular behaviour than one in an adolescent male, even though radiological enhancement may suggest that the lesion is highly vascularised. Nasal septal angiofibromas can be managed by biopsy and removed by simple endoscopic resection with minimal haemorrhage. Although very rare, extranasopharyngeal angiofibroma/nasal septal angiofibroma should be included in the differential diagnosis when patients of all ages present with nasal obstruction secondary to a nasal mass, with or without epistaxis.

Direct Tumoral Puncture Onyx Embolization for a Juvenile Nasopharyngeal Angiofibroma in a Hybrid Neurointerventional Suite.

This case video demonstrates a multidisciplinary approach to resection of a juvenile nasopharyngeal angiofibroma highlighting direct intratumoral onyx embolization. The patient is a 14-year-old boy who presented with a 1-month history of worsening epistaxis and nasal congestion. Preoperative magnetic resonance imaging demonstrated a 4.5 x 3 x 3 cm lobulated mass in the right pterygomaxillary space, sphenoid, and the nasopharynx adjacent to the cavernous carotid. Given the high vascularity of the lesion, intratumoral onyx embolization was undertaken, which significantly reduced intraoperative blood loss in this case. The present video demonstrates the technique for safe direct intratumoral onyx embolization and its role in significantly reducing intraoperative blood loss (Video 1). Postoperatively, the patient made an uncomplicated recovery. The patient consented to the procedure.

Efficacy and safety of preoperative internal maxillary arterial embolization with gelfoam for nasopharyngeal angiofibroma.

PURPOSE: To investigate the efficacy and safety of preoperative internal maxillary arterial embolization with gelfoam particles in patients with nasopharyngeal angiofibroma. MATERIALS AND METHODS: We retrospectively reviewed a total of 27 consecutive patients with pathologically confirmed nasopharyngeal angiofibroma from August 2006 to September 2018. Of the 27 enrolled patients, 10 patients received surgical excision alone; 17 patients received preoperative internal maxillary arterial embolization followed by surgical excision. Embolic agents were gelfoam particles. RESULTS: The mean volume of intro-operative blood loss was 385.3 ml in patients with preoperative arterial embolization, which was significantly lower than 1215.0 ml in the patients without preoperative arterial embolization (P < 0.001). The mean surgical time was shorter in patients with preoperative arterial embolization than in the patient without preoperative arterial embolization, but the difference had no statistical significance (205.0 vs 264.5 min, P = 0.064). Neurological complications such as facial palsy or vision loss or hemiplegia were not observed in patients with preoperative arterial embolization. CONCLUSION: Internal maxillary artery embolization with gelfoam particles suffices to provide an effective and safe adjuvant procedure for surgical excision of nasopharyngeal angiofibroma.

Endoscopic endonasal transmaxillary ligation of a feeding artery and coblation plasma technology enables en bloc resection of advanced juvenile nasopharyngeal angiofibroma without preoperative embolization.

Juvenile nasopharyngeal angiofibroma (JNA) is a hypervascular tumor and uncontrolled hemorrhage makes its removal very difficult. Although preoperative intravascular embolization of a feeding artery is recommended, serious complications such as iatrogenic thrombosis in the brain and insufficient decrease in blood flow to the tumor are concerns. Recently, coblation plasma technology has been reported to be useful for tumor removal with minimum hemorrhage under a clear surgical field. Here we report successful removal of advanced JNA without preoperative embolization, using intraoperative ligation of the maxillary artery and coblation plasma technology. The left nasal cavity of a 23-years-old man was closed by a JNA tumor at Radkowski stage IIC, which was 65mm in size and extended from the nasal cavity to the infratemporal fossa. MRA imaging showed the maxillary artery running along the posterior wall of the maxillary sinus. Therefore, the maxillary artery was first clipped using an endoscopic modified medial maxillectomy (EMMM) approach and endoscopic endonasal en bloc resection of the tumor was then completed using coblation technology with no need for blood transfusion. This case illustrates that preoperative embolization is dispensable in JNA surgery even at Stage IIC if the maxillary artery can be ligated during surgery and a coblation device can be utilized.

[Angiofibroma of soft tissue: a clinicopathologic analysis of 24 cases].

Objective: To investigate the clinicopathologic and genetic features, pathologic diagnosis and differential diagnosis of angiofibroma of soft tissue(AFST). Methods: The clinicopathologic characteristics of 24 cases diagnosed at Fudan University Shanghai Cancer Center from 2011 to 2017 were analyzed; immunohistochemical staining and interphase fluorescence in situ hybridization (FISH) were performed, and the literatures were also reviewed. Results: There were 15 male and 9 female (male∶female=1.7∶1.0) patients with age of onset ranging from 8 to 68 years (mean, 45 years). Fourteen cases occurred in extremities, including upper limbs (n=3) and lower limbs (n=11); seven cases were in the trunk, and 1 case each was in the temporal region, retroperitoneum and liver, respectively. Clinically, the tumors usually presented as a slowly growing painless mass. Tumor sizes ranged from 0.8 to 14 cm (mean 4.6 cm). Microscopically, most lesions were well-circumscribed, with fibrous capsules. Few cases infiltrated the surrounding fibrofatty tissue focally. The tumors were mainly composed of sparse short spindle cells and numerous small, branching, thin-walled blood vessels distributed in amyxoid, fibromyxoid or collagenous matrix, often accompanied by medium-sized, round or irregular and ecstatic vessels at the tumor periphery.By immunohistochemistry, all tested cases expressed vimentin (5/5), and showed variable positivity for EMA (2/4), ER (1/2), PR (2/3), α-SMA (1/18)and desmin (1/10). Ki-67 proliferation index were all less than 5%. CD34, CD31 and ERG staining clearly outlined the contours of blood vessels in the stroma. Four cases were tested for NCOA2 gene rearrangement by FISH, of which three were positive. Follow-up data was available in 17 patients (range, 3 to 69 months; mean, 30 months) were all free of disease. Conclusions: Soft tissue angiofibroma is a benign fibroblastic neoplasm characterized by a prominent and complex vasculature set in a myxoid-to-collagenous stroma, and cytogenetically a distinctive NCOA2 gene rearrangement. Caution should be exercised for the possibility of potentially misinterpretation of AFST as vascular tumors and other myxoid soft tissue tumors.

Nasopharyngeal angiofibroma in a 32-year-old man.

Nasopharyngeal angiofibroma (NA) is a benign, highly vascularised tumour of the nasopharynx, which typically occurs in young males aged 14-25 years. We report an interesting case of an NA arising de novo in a 32-year-old male. He was referred to our facility for severe nasal haemorrhage after biopsy of a left nasopharyngeal mass. In the operating room, extensive bleeding was noted, and an endoscopic medial maxillectomy was performed, and the left internal maxillary artery was ligated allowing for near total resection of the lesion. The pathological specimen confirmed the diagnosis of NA. To our knowledge, this is one of the oldest patients presenting with a de novo NA, in his fourth decade of life, confirming that this diagnosis must be considered in all those with large nasopharyngeal masses.

Patterns of vascularization and surgical morbidity in juvenile nasopharyngeal angiofibroma: A case series, systematic review, and meta-analysis.

BACKGROUND: Vascular patterns of juvenile nasopharyngeal angiofibroma (JNA) are poorly defined. We performed both institutional and systematic literature reviews to characterize the relationship between arterial supply patterns of JNA with intraoperative blood loss and tumor recurrence. METHODS: A retrospective review of 26 patients with JNA treated at our institution from 1995 to 2015 with available angiograms, and systematic reviews and meta-analyses of 828 JNA cases undergoing angiographic embolization published between 1995 and 2015 were completed per Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. RESULTS: The systematic review (828 cases) found internal carotid artery (ICA) supply in 35.6% of tumors, and 30.8% of tumors received bilateral vascular supply. Our institutional data (n = 26) indicated 69% had bilateral supply. Meta-analysis of data from 5 studies demonstrated ICA/bilateral arterial supply is predictive of increased operative blood loss (P < .01). CONCLUSION: Complex vascular contributions to JNA are frequent, underreported, and portends increased blood loss. This information can justifiably be included in staging systems to enhance prognostic counseling of patients.

Efficacy of Preoperative Transcatheter Arterial Embolization for Nasopharyngeal Angiofibroma: A Comparative Study.

OBJECTIVE: This study aimed to retrospectively evaluate the efficacy and safety of preoperative transcatheter arterial embolization (pTAE) for treating nasopharyngeal angiofibroma (NPAF). METHODS: Seventy-four NPAF patients were hospitalized for elective surgical treatment with pTAE (pTAE group, n = 32) or surgical treatment alone (non-pTAE group, n = 42) between January 1990 and December 2013. The following outcome measures were retrospectively analyzed and compared: intraoperative bleeding volume, surgery time (ST), duration of postoperative hospital stay (PHS), and disease recurrence. RESULTS: Among Radkowski stage I patients, those in pTAE group had a slightly higher but not significant bleeding volume than patients in non-pTAE group (344 ± 407 vs. 248 ± 219 mL, P = 0.899); among stage II/III patients, however, patients in pTAE group showed a significantly lower bleeding volume than patients in non-pTAE group (stage II, 829 ± 519 vs. 1339 ± 767 mL, P = 0.035; stage III, 1267 ± 592 vs. 2125  ± 479 mL, P = 0.024). The two groups presented comparable OTs, PHSs, and rates of frontal recurrence (all P>0.05). CONCLUSIONS: pTAE significantly reduces intraoperative bleeding in NPAF patients with Radkowski stage II/III disease, but offers no additional benefits regarding ST, PHS, or recurrence.

Association between vascular supply, stage and tumour size of juvenile nasopharyngeal angiofibroma.

Juvenile nasopharyngeal angiofibroma (JNA) is a highly vascular tumour seen in adolescent males. To study the vascular pattern of these tumours, we retrospectively reviewed the records of patients with JNA who underwent preoperative angiography. Most (82.2 %) of the 45 patients assessed were Radkowski stage III with a mean size of 5.29 cm. There was a significant association between tumour stage and size (p = 0.029). Ten different vessels were seen to supply these tumours. All tumours had primary supply from the distal third of the ipsilateral internal maxillary artery (IMA). Accessory vessel supply was chiefly from the Vidian branch of internal carotid artery (ICA) (55.6 %). Stage III tumours were supplied by a greater number of feeding vessels than earlier stage tumours (p < 0.01). Larger tumours were more likely to have ICA supply (p = 0.04). Bilateral supply was seen in 48.7 %. However, there was no predominance of bilateral over ipsilateral IMA supply even in advanced stage tumours. One patient in our series was found to have a caroticocavernous fistula. Residual or recurrent tumours were characterized by new vasculature (100 %) and greater accessory supply from the ipsilateral ICA (85.7 %). Our study highlights the fact that surgical planning cannot be dependent on staging alone and should include preoperative assessment of tumour vasculature by angiography.

Endoscopic Management of Vascular Sinonasal Tumors, Including Angiofibroma.

The greatest challenge in the surgical treatment of angiofibromas is dealing with the hypervascularity of these tumors. Staging systems that take into account the vascularity of the tumor may be more prognostic. A variety of treatment strategies are used to deal with the vascularity of angiofibromas, including preoperative embolization, segmentation of the tumor into vascular territories, use of hemostatic tools, and staging of surgery. Even large angiofibromas with intracranial extension and residual vascularity can be successfully managed by a skull base team using endoscopic techniques.

Combined percutaneous and transarterial devascularisation of juvenile nasopharyngeal angiofibroma with protection of internal carotid artery: A modification of the technique.

Juvenile nasal angiofibroma (JNA) is a hypervascularised, benign, but locally aggressive tumour that grows in the posterior, upper part of the nasal cavity and invades surrounding anatomical structures. The treatment of choice is surgical removal, but complete resection of the tumour can be hampered because of profuse perioperative bleeding. Preoperative embolisation of the tumour has been proposed as an effective method for prevention of perioperative bleeding, thereby shortening of the time of the operation. In this report of five cases, we describe successful preoperative devascularisation of the tumour by applying a modified method of direct intratumoural injection of the liquid embolic agent Onyx combined with protection of the internal carotid artery. The control of bleeding during the embolisation and occlusion of the maxillary or sphenopalatine artery was achieved by using a bi-luminal balloon catheter. Such use of the dual-lumen catheter in treatment of JNA has not been reported so far in the medical literature.

Surgery for juvenile nasopharyngeal angiofibroma with lateral extension to the infratemporal fossa.

OBJECTIVE: The study aimed to assess the usefulness of skull base surgery for large juvenile nasopharyngeal angiofibroma (JNA) with lateral extension to the infratemporal fossa. MATERIALS AND METHODS: Eleven cases were enrolled for this study, and the mean age was 17.7 years old (range: 8-32). Six out of 11 cases underwent surgery as an initial treatment, and the other five underwent secondary surgery after initial surgery or radiotherapy in other institutions. The range of extension of tumor, feeding arteries, surgical approach, and treatment outcome were estimated. RESULTS: All tumors originated from the sphenopalatine foramen. Based on the imaging study, there was extension to the cavernous sinus observed in eight cases, as well as to the middle cranial fossa (8), orbit (4), and anterior cranial fossa (1). These tumors were diagnosed as Andrews' Stage IVa (3) and IVb (8). However, infiltration into the cavernous sinus was observed in one case only during surgery. Ten tumors were separated carefully from the cavernous sinus or dura and were accurately diagnosed as Stage IIIb. In all cases, the main arterial feeders of the JNAs were branches of the external carotid artery, which were embolized prior to surgery. However, 10 cases were also fed by branches of the internal carotid artery (branches of the ophthalmic artery), in which these arteries could not be embolized. Coronal skin incision (1) and a facial dismasking flap (9) were used, and in one case, wide lateral skin incision with temporary incision of the facial nerve was applied. The orbito-zygomatic approach and its modification was applied to all the cases. Fronto-lateral craniotomy was applied in four cases and lateral craniotomy in seven cases. Total resection was achieved in 10 cases and subtotal resection in one case. No mortality was noted in this series. Temporal trismus was observed in all cases which subsided gradually. Cheek numbness and facial palsy were observed in three and two cases, respectively. CONCLUSION: Coupled with craniotomy, tumor removal was successfully carried out in 11 patients with JNAs, which showed large lateral extension. Our surgical strategy is a safe and effective approach for the removal of JNAs with infratemporal fossa extension.

Juvenile nasopharyngeal angiofibroma: vascular determinates for operative complications and tumor recurrence.

OBJECTIVES/HYPOTHESIS: Operative complications and tumor recurrence in juvenile nasopharyngeal angiofibroma (JNA) are measurable and meaningful outcomes. This study aimed to assess the association of these two outcomes to various clinical indices and in particular, vascular determinates. STUDY DESIGN: Retrospective cohort study. METHODS: An 18-year retrospective chart review of an academic tertiary center was undertaken. Data from clinical notes, imaging studies, and arteriograms were analyzed. RESULTS: Thirty-seven male (mean age, 14.4 years) patients were included in the study. Tumor stages included: IA (three), IB (three), IIA (14), IIB (three), IIC (five), IIIA (five), and IIIB (four). Four complications (cerebrospinal fluid leak, cerebral vascular accident, and two transient ocular defects) occurred. Eight recurrences occurred within 24 months following surgery. Complications were associated with estimated intraoperative blood loss (EBL) (P = .045). Tumor recurrence was associated with feeding vessels from the contralateral internal carotid artery (ICA) (P = .017). EBL was significantly associated with surgical technique used. EBL, tumor stage, and tumor vascular supply were significantly associated with each other. CONCLUSIONS: Vascular factors were associated with JNA complication and tumor recurrence. EBL might affect complications, and contralateral ICA as a feeding vessel might affect recurrence. EBL was influenced by procedure choice and was interrelated to size and vascular supply of the tumor. This study bolsters the need to decrease intraoperative blood loss by preoperative embolization and use of endoscopic removal techniques. Furthermore, when branches of the ICA are found to be feeding vessels, greater surgical attention for a dry surgical field is encouraged.

Preoperative angiography and external carotid artery embolization of juvenile nasopharyngeal angiofibromas in a tertiary referral paediatric centre.

AIM: To evaluate the relationship between intraoperative blood loss and juvenile nasopharyngeal angiofibroma (JNA) vascular supply and tumour stage in patients who underwent superselective external carotid artery (ECA) embolization. This series is unique in that all embolizations were performed by dedicated paediatric interventional radiologists at a tertiary referral paediatric centre. MATERIALS AND METHODS: Seventeen male patients treated from January 2002 to August 2009 underwent preoperative angiography and embolization using polyvinyl alcohol (PVA) particles. Tumours were graded using three different staging systems based on preoperative imaging and correlated to surgical blood loss. All patients underwent bilateral internal and external carotid angiography, with embolization of ECA tumour supply via microcatheter delivery of PVA particles. Particle size ranged from 150-500 µm with a mean size of 250-355 µm. Surgical resection was performed with either endoscopic or open techniques within 24 h and intraoperative blood loss was reported. RESULTS: Seven lesions were supplied strictly by the ECA circulation and had mean surgical blood loss of 336 ml. Twelve lesions had both ECA and internal carotid artery (ICA) supply and had mean surgical blood loss of 842 ml. The difference in blood loss in these two groups was statistically significant (p = 0.03). There was no case of inadvertent intracranial or ophthalmic embolization. There were statistically significant correlations between estimated surgical blood loss and the Andrews (p = 0.008), Radkowski (p = 0.015), and University of Pittsburgh Medical Center (UPMC; p = 0.015) preoperative tumour staging systems, respectively. CONCLUSION: Preoperative embolization of JNA tumours can be safely performed without neurological complications. The present study identified a statistically significant difference in intraoperative blood loss between those lesions with a purely ECA vascular supply and a combination of ECA and ICA vascular supply. Angiography is helpful in delineating ICA supply and can help guide surgical planning.

[Embolization of skull-base hypervascular tumors: description of a series of cases and proposal of a therapeutic algorithm]. / Embolizacion de tumores hipervasculares de la base del craneo: descripción de una serie de casos y propuesta de un algoritmo terapéutico.

UNLABELLED: Preoperative embolization allows reducing intraoperative blood loss caused by hypervascular intracranial tumors and its undesirable consequences. AIM: To describe the experience with preoperative endovascular embolization of hypervascular skull base tumors, and to develop a therapeutic algorithm. MATERIALS AND METHODS: A retrospective examination of preoperative neuroimaging and results of preoperative embolization was carried out. RESULTS: Fifteen cases were identified, with a median age of 36 years old, most of them harboring meningiomas, nasopharyngeal angiofibromas or paragangliomas. The external carotid artery was involved in 93% of cases and was the only afferent to 60%. In 27%, there were branches from the internal and external carotid arteries simultaneously. An extensive or complete occlusion grade was achieved in 95% of the branches of the external carotid artery. No branch of the internal carotid artery was treated, because poor contribution to tumor irrigation or was not possible to catheterize the pedicle. There were not recorded procedure-related complications. CONCLUSION: Based on the unification of the experiences described in this study and using data from published series, we present an algorithm for rational selection of skull base tumors that can benefit from preoperative embolization.

Endoglin (CD105) expression on microvessel endothelial cells in juvenile nasopharyngeal angiofibroma: tissue microarray analysis and association with prognostic significance.

BACKGROUND: The purpose of this study was to examine endoglin (CD105) expression on microvessel endothelial cells (ECs) in juvenile nasopharyngeal angiofibroma (JNA) and its relationship with recurrence. METHODS: Immunohistochemistry was performed to detect CD105 expression in a tissue microarray from 70 patients with JNA. Correlation between CD105 expression on microvessel ECs and clinicopathological features, as well as tumor recurrence, were analyzed. RESULTS: Immunohistochemistry revealed CD105 expression on ECs but not in stroma of patients with JNA. Chi-square analysis indicated CD105-based microvessel density (MVD) was correlated with JNA recurrence (p = .013). Univariate and multivariate analyses determined that MVD was a significant predictor of time to recurrence (p = .009). The CD105-based MVD was better for predicting disease recurrence (AUROC: 0.673; p = .036) than other clinicopathological features. CONCLUSIONS: MVD is a useful predictor for poor prognosis of patients with JNA after curative resection. Angiogenesis, which may play an important role in the occurrence and development of JNA, is therefore a potential therapeutic target for JNA.

Spontaneous ICA rupture: a severe late complication after giant nasopharyngeal angiofibroma resection.

Juvenile nasopharyngeal angiofibroma (JNA) is a benign vascular tumor occurring in young males. Surgery for giant JNA is a complex procedure, with a high risk of major complications. We report a rare case of massive epistaxis in a 15-year-old boy resulting from spontaneous rupture of the intracavernous tract of the internal carotid artery 20 days after resection of a giant JNA by midface degloving. The event was managed by an emergency arteriography with coils selectively deployed to occlude the vessel and to stop hemorrhage. This treatment has been shown to be effective in producing immediate hemostasis and stable long-term occlusion.

Bilateral non-superselective embolization with particles under transient occlusion of the internal carotid artery in the management of juvenile nasopharyngeal angiofibroma: technical note.

Juvenile nasopharyngeal angiofibroma (JNA) is a rare histologically benign tumor, highly vascularized, with usually aggressive behavior, and can extend from the nasal cavity to neighboring structures. We present the case of a 14-year-old male harboring a JNA, presenting with an active severe and persistent epistaxis. Two previous surgical attempts of removal were unsuccessful, because of profuse intraoperative bleeding. Angiography showed a highly vascularized neoplasm with multiple branches arising from both internal carotid arteries, with absence of branches from the external carotid due to previous surgical ligation. Direct puncture tumor embolization was not possible because removal of nasal packing triggered major hemorrhage. The only option for embolization was a technique of non-superselective embolization with particles under transient occlusion of the internal carotid artery. The procedure was performed uneventfully from either side, the tumor was subsequently removed, and the patient had no recurrence 2 years after the initial treatment.