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2.
Cureus ; 16(6): e61667, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38966478

ABSTRACT

A rare and locally aggressive vascular tumor, juvenile nasopharyngeal angiofibroma (JNA) mostly affects male teenagers. This paper describes a 14-year-old male patient who presented with lethargy and recurrent nasal bleeding, which are symptoms of JNA. CT and MRI scans confirmed a vascular mass with a significant local invasion originating from the sphenopalatine foramen. After a CT angiography, which revealed the tumor's large blood supply and helped with efficient excision, a focused surgical strategy was designed. Histopathology verified the benign nature of the tumor, and the operation was successful and the patient had a smooth recovery. This case adds to the little literature on JNA. It highlights the need for healthcare professionals to be aware of the requirement of early identification and careful presurgical preparation in managing the illness.

3.
Cancers (Basel) ; 16(11)2024 May 24.
Article in English | MEDLINE | ID: mdl-38893114

ABSTRACT

Helium ion therapy (HRT) is a promising modality for the treatment of pediatric tumors and those located close to critical structures due to the favorable biophysical properties of helium ions. This in silico study aimed to explore the potential benefits of HRT in advanced juvenile nasopharyngeal angiofibroma (JNA) compared to proton therapy (PRT). We assessed 11 consecutive patients previously treated with PRT for JNA in a definitive or postoperative setting with a relative biological effectiveness (RBE) weighted dose of 45 Gy (RBE) in 25 fractions at the Heidelberg Ion-Beam Therapy Center. HRT plans were designed retrospectively for dosimetric comparisons and risk assessments of radiation-induced complications. HRT led to enhanced target coverage in all patients, along with sparing of critical organs at risk, including a reduction in the brain integral dose by approximately 27%. In terms of estimated risks of radiation-induced complications, HRT led to a reduction in ocular toxicity, cataract development, xerostomia, tinnitus, alopecia and delayed recall. Similarly, HRT led to reduced estimated risks of radiation-induced secondary neoplasms, with a mean excess absolute risk reduction of approximately 30% for secondary CNS malignancies. HRT is a promising modality for advanced JNA, with the potential for enhanced sparing of healthy tissue and thus reduced radiation-induced acute and long-term complications.

4.
Cureus ; 16(5): e60747, 2024 May.
Article in English | MEDLINE | ID: mdl-38903296

ABSTRACT

Background Surgical excision is the primary treatment for juvenile nasopharyngeal angiofibroma (JNA), but this procedure is challenging due to its high vascularity and local aggressiveness. Moreover, preoperative embolization is a subject of debate. Objective The objective of this study is to assess the efficacy, safety, and feasibility of endoscope-assisted excision as a surgical intervention for non-embolized advanced JNA. Materials and methods This case series involved six male patients (mean age: 16 years) with JNA, classified as stages Ⅱc to Ⅲb according to the Radkowski classification. None underwent preoperative embolization. Results Two stage Ⅱc cases underwent total endoscopic endonasal excision. One patient with stage Ⅲa and another with stage Ⅲb underwent surgery via an endoscope-assisted sublabial approach. Two patients, one with stage Ⅱc JNA and another with Ⅲb, underwent a two-stage procedure. Postoperative CT scans showed no residual disease at the six-month mark. On average, each procedure required 1.5 units of blood transfusion. One patient experienced intraoperative bleeding, whereas the remaining patients were free of any major complications. The mean operation duration was 175 minutes per procedure. The mean length of stay at the hospital was 3.75 days per procedure. Conclusion Endoscope-assisted or purely endoscopic approaches can be safely and effectively employed for the complete excision of non-embolized advanced JNAs.

5.
Article in Chinese | MEDLINE | ID: mdl-38858116

ABSTRACT

Objective:To summarize the procedures and efficacy of surgical treatment for Andrew stage Ⅰ-Ⅲ juvenile nasopharyngeal angiofibroma(JNA). Methods:A total of 12 patients with JNA who underwent surgery from 2016 to 2021 were enrolled, including 1 case in stage Ⅰ, 3 cases in stage Ⅱ, and 8 cases in stage Ⅲ. JNA was resected by transnasal endoscopic approach alone, or combined with transoral approach or Caldwell-Luc approach was performed. Results:Eleven cases underwent complete resection without recurrence and 1 case had residual tumor. There were no serious complications. The median intraoperative blood loss was 200 mL, and 1 patient received blood transfusion. The median operative time was 110 minutes. Conclusion:JNA in Andrew stage Ⅰ-Ⅲ can be quickly and completely resected by standardized surgical procedures using endoscopy and coblation technology.


Subject(s)
Angiofibroma , Endoscopy , Nasopharyngeal Neoplasms , Humans , Angiofibroma/surgery , Nasopharyngeal Neoplasms/surgery , Male , Endoscopy/methods , Adolescent , Treatment Outcome , Female , Operative Time , Young Adult , Blood Loss, Surgical , Adult
6.
Laryngoscope ; 134(8): 3568-3571, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38747477

ABSTRACT

Onyx is a safe and effective embolic agent to utilize in the treatment paradigm of JNA. We present a tandem approach that combines trans-arterial embolization (TAE) with direct puncture embolization (DPE) with Onyx to limit blood loss and facilitate safe resection. Laryngoscope, 134:3568-3571, 2024.


Subject(s)
Dimethyl Sulfoxide , Embolization, Therapeutic , Polyvinyls , Humans , Embolization, Therapeutic/methods , Polyvinyls/therapeutic use , Dimethyl Sulfoxide/therapeutic use , Punctures/methods , Male , Middle Aged , Female , Treatment Outcome , Combined Modality Therapy
7.
Indian J Anaesth ; 68(4): 391-393, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38586254

ABSTRACT

Surgery for excision of juvenile nasopharyngeal angiofibroma (JNA) carries the possibility of massive life-threatening haemorrhage. Anaesthetic management aims to maintain haemodynamic stability and reduce blood loss. This case series describes the application of the bundled approach as a multimodal blood loss prevention bundle (MBLPB). Twenty patients underwent 23 surgeries with MBLPB. The blood loss and the number of units of blood transfused were recorded. The surgeon satisfaction score was assessed. The median [interquartile range (IQR)] estimated blood loss was 1300 (650-2350) ml. Patients with tumours in stages I and II had a median (IQR) blood loss of 550 (270-750) ml compared to patients with higher grades of tumours (stages III, IV) with a median (IQR) blood loss of 2100 (1300-2500) ml. Median (IQR) units of packed red cells transfused was 1 (0-3). The surgeon's satisfaction score was high when MBLPB was applied for JNA. However, it does not appear to reduce blood loss markedly.

8.
J Otolaryngol Head Neck Surg ; 52(1): 85, 2023 Dec 19.
Article in English | MEDLINE | ID: mdl-38115030

ABSTRACT

BACKGROUND: Juvenile Nasopharyngeal Angiofibroma (JNA) is a fibrovascular tumor of the nasopharynx that classically presents in adolescent males. The reported mean age of onset is between 13 and 22 years old [1-6]. Significant androgen stimulation is hypothesized to explain the strong predisposition for JNA to present in young adolescent males. However, considerable variability in age at diagnosis exists with rare involvement of very young patients incongruent with typical male pubertal growth patterns. OBJECTIVE: The purpose of this systematic review is to identify cases of early-onset JNA (EOJNA), (defined as age < 10 years) in the literature and to examine the disease characteristics and treatments used in this patient group. A case of a 7 year old boy with EOJNA at our institution is also described and presented. METHODS: We searched Embase, Cochrane database and MEDLINE from 1996 to February 2021 for studies that reported cases of EOJNA. Relevant clinico-demographic data, disease severity and treatment outcomes were recorded and analyzed using descriptive statistics. We compared our findings with reported means for JNA in all ages. RESULTS: We identified 29 studies containing a total of 34 cases of EOJNA. The vast majority (31/34) of patients were males and the mean age of diagnosis was 8.15 years old. The most common presenting symptoms were nasal obstruction (65.2%) and epistaxis (60.9%). Patients were most commonly Radkowski stage II (39.4%) and III (39.4%). Primary treatment modalities included open surgery (66.7%), endoscopic surgery (24.2%), and radiotherapy (9.1%). Recurrence was evident in 30%. Radkowski stage and type of treatment did not differ significantly within the EOJNA group (p = 0.440 and p = 0.659, respectively). CONCLUSION: This systematic review suggests that rare cases of EOJNA have distinct disease characteristics. Patients in this cohort appeared to have more advanced disease and higher recurrence rates when compared with reported averages. We hope that this review prompts increased clinical awareness of this potentially more aggressive subtype of JNA. As more cases of EOJNA are reported, a more powered statistical analysis of this cohort would be feasible.


Subject(s)
Angiofibroma , Nasal Obstruction , Nasopharyngeal Neoplasms , Adolescent , Humans , Male , Young Adult , Adult , Child , Female , Angiofibroma/diagnosis , Angiofibroma/surgery , Nasopharyngeal Neoplasms/diagnosis , Nasopharyngeal Neoplasms/surgery , Epistaxis , Treatment Outcome , Nasal Obstruction/etiology , Retrospective Studies
9.
Medicina (Kaunas) ; 59(9)2023 Sep 07.
Article in English | MEDLINE | ID: mdl-37763739

ABSTRACT

Background and Objectives: Juvenile nasopharyngeal angiofibroma (JNA) is an angiomatous hamartoma of the nasal cavity. It is a benign but locally aggressive vascular tumor of the nasopharynx affecting adolescent males. Many surgical procedures are in practice, but the extended endonasal endoscopic (EEE) approach for JNAs is a suitable and effective technique. Materials and Methods: Fifteen adolescent patients having JNA who underwent extended endonasal endoscopic (EEE) surgery from January 2010 to January 2022 were studied retrospectively. Patients having residual and recurrent JNAs and those who underwent surgery other than EEE were excluded. Results: The average age of the patients was 18.3 years of age. A total of six patients (40%) each had stage V and IV while three patients (20%) had stage III JNAs. Gross total removal was achieved in eight (53.3%) patients and seven (43.7%) had partial removal. There was no per or postoperative mortality. All the patients had at least 3 years of postoperative follow-up and during follow-ups, seven patients were found to have residual tumors, and two had recurrences. Discussion: During the last decades, the endoscopic approach for the resection of JNAs has gained increasing popularity due to its obvious advantages over transfacial approaches. The magnified and angled field of view "behind the corner" helping in a more complete inspection for the resection and shorter hospitalization time makes it a better choice than the other approaches. Conclusions: Endoscopy is an excellent approach for primary JNA. It allows well visualization and precise removal of the angiofibroma. An endoscopic multiangle, multicorridor skull base approach including Denker's anteromedial maxillotomy is suitable and preferable for the resection of extensive JNAs.


Subject(s)
Angiofibroma , Nasopharyngeal Neoplasms , Adolescent , Male , Humans , Angiofibroma/surgery , Radiotherapy, Adjuvant , Retrospective Studies , Endoscopy , Nasopharyngeal Neoplasms/radiotherapy , Nasopharyngeal Neoplasms/surgery
10.
J Appl Genet ; 64(4): 749-758, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37656292

ABSTRACT

Lifestyle factors, including smoking, have been linked to neoplastic diseases, and reports suggest an association between smoking and overexpression of FGFR (fibroblast growth factor receptor) in certain neoplasms. This study aims to assess the expression of FGFR3 and FGFR4 genes in patients with and without a history of smoking.A total of 118 participants were recruited, including 83 Juvenile Nasopharyngeal Angiofibroma (JNA) patients and 35 healthy participants, the JNA patients were further stratified as smokers and nonsmokers. Total RNA was extracted from the blood & saliva sample by using TRIzol reagent, and quantified using a Nanodrop, and then subjected to gene expression analysis of FGFR3/4 using RT-PCR. Immunohistochemistry analysis was employed using fresh biopsies of JNA to validate the findings. All experiments were performed in triplicates and analysed using the Chi-Square test (P < 0.05). Smokers exhibited significantly lower total RNA concentrations across all sample types (P < 0.001). The study revealed significant upregulation of both FGFR3/4 genes in JNA patients (P < 0.05). Moreover, FGFR3 expression was significantly higher among smokers 66% (95% CI: 53-79%) compared to non-smokers 22% (95% CI: 18-26%). Immunohistochemistry analysis demonstrated moderate to strong staining intensity for FGFR3 among smokers. The study highlights the overexpression of FGFR3/4 genes in JNA patients, with a stronger association observed among smokers. Furthermore, medical reports indicated higher rates of recurrence and bleeding intensity among smokers. These findings emphasize the potential role of FGFR3 as a key molecular factor in JNA, particularly in the context of smoking.


Subject(s)
Angiofibroma , Nasopharyngeal Neoplasms , Humans , Angiofibroma/genetics , Angiofibroma/metabolism , Angiofibroma/pathology , Nasopharyngeal Neoplasms/genetics , Nasopharyngeal Neoplasms/metabolism , Nasopharyngeal Neoplasms/pathology , Immunohistochemistry , Smoking/genetics , RNA , Receptor, Fibroblast Growth Factor, Type 3/genetics , Receptor, Fibroblast Growth Factor, Type 4/genetics
11.
Cureus ; 15(8): e43256, 2023 Aug.
Article in English | MEDLINE | ID: mdl-37692702

ABSTRACT

A 54-year-old female presented to the otolaryngology (ENT) outpatient department with an eight-month history of unilateral nasal obstruction and headache. There was no change in the sense of smell, rhinorrhoea, facial pain, or associated epistaxis. On examination, there was a large, erythematous mass in the superior aspect of the right nasal cavity, filling the space between the nasal septum, middle, and superior meatus. The rest of the ENT examination was normal. Vital signs were all within the normal range. There was no significant past medical history, and she had tried steroid nasal spray without any benefit. She had a complete resolution of symptoms from surgical intervention, and the mass was confirmed to be an angiofibroma through histopathology. This case report discusses the importance of considering nasopharyngeal angiofibroma as a differential diagnosis for patients presenting with unilateral nasal masses, including female patients, regardless of age.

12.
Cureus ; 15(8): e43922, 2023 Aug.
Article in English | MEDLINE | ID: mdl-37746363

ABSTRACT

Subglottic stenosis (SGS), the narrowing of the upper trachea, can be an acquired condition in pediatric patients. Presenting with varying degrees of dyspnea and stridor, acquired SGS is most commonly due to intubation. Airway stenosis is often not considered a surgical complication, and no literature on acquired SGS after endoscopic sinus surgery exists. We present a unique case of a 13-year-old male with juvenile nasopharyngeal angiofibroma (JNA), who developed SGS in the setting of progressive dyspnea six weeks after endonasal resection of his mass. He required urgent intubation prior to preoperative embolization and endonasal surgery, which prolonged his total intubation period. After the patient was found to have acquired SGS, he eventually required serial dilation to treat his stenosis. The presentation and operative course of this patient, along with images and pathologic findings, are discussed. Based on an extensive literature review of PubMed, Medline, and Google Scholar, there have been no cases discussing SGS development post-intubation after endonasal surgery or in association with JNA. Acquired SGS can present as a life-threatening airway obstruction in pediatric patients. With the rise of endoscopic skull base surgery and the prevalence of JNA, this case study sheds light on the detection and management of SGS post-operatively.

13.
Article in Chinese | MEDLINE | ID: mdl-37549948

ABSTRACT

Objective:To investigate the surgical approach for the resection of juvenile nasopharyngeal angiofibroma(JNA) under nasal endoscopy. Methods:The clinical data of 87 patients undergoing endoscopic resection of nasopharyngeal fibroangioma were retrospectively analyzed. We classified JNA according to tumor site, size, invasion scope and anatomic position relationship between tumor and midline of pupil. Three endoscopic surgical approaches were selected according to the classification, and the postoperative symptoms, complications and recurrence were investigated and analyzed. Results:The tumor resection rate of 87 cases by nasal endoscopic surgery was 100%. Thirty-five cases were approached through the middle nasal passage(small tumors located in the nasal sinuses and pterygopalatine fossa), forty-five cases were approached through the lateral wall of the nasal cavity(tumor invaded the pterygopalatine fossa but did not exceed the midline of the pupil) , and seven cases were approached via the lateral wall of nasal cavity + ipsilateral anterior wall of maxillary sinus(tumor invaded the infratemporal fossa beyond the midline of pupil or invaded the cavernous sinus and the middle cranial fossa epidural), Postoperative patients with nasal congestion, nasal bleeding, headache, dizziness, vision loss and other symptoms showed varying degrees of improvement. No surgical death or intracranial infection occurred. The postoperative follow-up was 6-78 months, and the recurrence rate was 3.44%. Conclusion:Endoscopic resection of nasopharyngeal fibroangioma is the main treatment method for JNA. Selecting suitable endoscopic approach to resect JNA, To maximize the advantage of nasal endoscopic equipment according to the inherent anatomical space of the human nasal cavity, In order to achieve the purpose of JNA resection, reduce intraoperative and postoperative complications, reduce the recurrence rate and improve the prognosis.


Subject(s)
Angiofibroma , Nasopharyngeal Neoplasms , Humans , Angiofibroma/surgery , Angiofibroma/diagnosis , Angiofibroma/pathology , Retrospective Studies , Nasopharyngeal Neoplasms/pathology , Endoscopy/methods , Prognosis
14.
Cardiovasc Intervent Radiol ; 46(8): 1038-1045, 2023 Aug.
Article in English | MEDLINE | ID: mdl-37430013

ABSTRACT

PURPOSE: To determine the effects of blood supply from internal carotid artery (ICA) on the surgical outcomes of primary juvenile nasopharyngeal angiofibroma (JNA) after transarterial embolization (TAE). METHODS: A retrospective analysis was performed on primary JNA patients who underwent TAE and endoscopic resection in our hospital between December 2020 and June 2022. The angiography images of these patients were reviewed, and then they were divided into ICA + external carotid artery (ECA) feeding group and ECA feeding group according to whether the ICA branches were part of the feeding arteries. Tumors in ICA + ECA feeding group were fed by both ICA and ECA branches, while tumors in ECA feeding group were fed by ECA branches alone. All patients underwent tumor resection immediately after ECA feeding branches embolization. None of the patients underwent ICA feeding branches embolization. Data on demographics, tumor characteristics, blood loss, adverse events, residual and recurrence were collected, and case-control analysis was performed for the two groups. Differences in characteristics between the groups were tested using Fisher's exact and Wilcoxon tests. RESULTS: Eighteen patients were included in this study: nine in ICA + ECA feeding group and nine in ECA feeding group. The median blood loss was 700 mL (IQR 550-1000 mL) in ICA + ECA feeding group versus 300 mL (IQR 200-1000 mL) in ECA feeding group, with no significant statistical difference (P = 0.306). Residual tumor was found in one patient (11.1%) in both groups. Recurrence was not observed in any patient. There were no adverse events from embolization and resection in either group. CONCLUSION: The results of this small series suggest that the presence of blood supply from ICA branches in primary JNA has no significant effect on intraoperative blood loss, adverse event, residual and postoperative recurrence. Therefore, we do not recommend routine preoperative embolization of ICA branches. LEVEL OF EVIDENCE: Level 4, Case-control.


Subject(s)
Angiofibroma , Embolization, Therapeutic , Nasopharyngeal Neoplasms , Humans , Carotid Artery, Internal/diagnostic imaging , Retrospective Studies , Angiofibroma/diagnostic imaging , Angiofibroma/surgery , Nasopharyngeal Neoplasms/diagnostic imaging , Nasopharyngeal Neoplasms/therapy , Nasopharyngeal Neoplasms/pathology , Embolization, Therapeutic/methods , Carotid Artery, External/diagnostic imaging , Carotid Artery, External/pathology
15.
Acta Neurochir (Wien) ; 165(7): 1773-1780, 2023 07.
Article in English | MEDLINE | ID: mdl-37280419

ABSTRACT

BACKGROUND: Juvenile nasopharyngeal angiofibromas (JNAs) are rare, benign, and locally invasive nasopharyngeal tumors. Endoscopic endonasal resection is effective, non-invasive, with low complication rates. Until recently, endoscopic resection was not suitable for intracranially invasive tumors. PURPOSE/METHOD: We describe resection steps of an intracranially extending JNA with a combined endoscopic endonasal and endoscopic-assisted sublabial transmaxillary approaches. Indications, advantages, and approach-specific complications are also discussed. The main surgical steps are shown in an operative video. CONCLUSION: Surgical excision of JNAs by a combined endoscopic endonasal and sublabial transmaxillary approaches represents a safe and effective treatment for selected intracranially invasive JNA.


Subject(s)
Angiofibroma , Nasopharyngeal Neoplasms , Humans , Angiofibroma/diagnostic imaging , Angiofibroma/surgery , Angiofibroma/pathology , Nasopharyngeal Neoplasms/diagnostic imaging , Nasopharyngeal Neoplasms/surgery , Nasopharyngeal Neoplasms/pathology , Endoscopy , Treatment Outcome
16.
J Neuroimaging ; 33(5): 675-687, 2023.
Article in English | MEDLINE | ID: mdl-37164909

ABSTRACT

BACKGROUND AND PURPOSE: To comprehensively summarize the radiological characteristics of sinonasal tract angiofibroma (STA) (commonly known as juvenile nasopharyngeal angiofibroma). METHODS: Forty-four lesions from 41 cases provided by 33 study articles identified through a systematic review and 13 lesions from 13 cases from our institution associated with patients with STA who underwent MRI were included in the review study, carried out by two board-certified experienced radiologists. RESULTS: The study participants were all male patients with a mean age of 15.6 years at the time of diagnosis. All of them presented with nasal cavity lesions (100%), predominantly in the nasopharynx (98.2%). The sphenopalatine foramen/pterygopalatine fossa was involved in 76.0%, and compressive shift of the posterolateral wall of the maxillary sinus was present in more than half (57.9%). T2-weighted imaging signal intensity was heterogeneous with mixed high and iso intensities as compared to skeletal muscle (100%). T1-weighted imaging showed partial high signal intensity in 61.1% of the cases. Flow void and intense enhancement were present in almost all cases. Cystic/nonenhancement changes on contrast-enhanced MRI were relatively common (40.8%). The mean apparent diffusion coefficient value (2.07 × 10-3  mm2 /second) and some quantitative dynamic contrast-enhanced MRI parameters were high. There was a significant difference in the frequency of residual/recurrent lesions based on the presence of MRI findings of skull base invasion (p = .017) and intracranial extension (p = .003). CONCLUSIONS: We summarized the MRI findings of STA that can facilitate timely diagnosis and appropriate management.


Subject(s)
Angiofibroma , Nasopharyngeal Neoplasms , Paranasal Sinuses , Humans , Male , Adolescent , Angiofibroma/diagnostic imaging , Angiofibroma/pathology , Nasopharyngeal Neoplasms/diagnosis , Nasopharynx/pathology , Paranasal Sinuses/pathology , Magnetic Resonance Imaging
17.
Radiol Case Rep ; 18(6): 2096-2102, 2023 Jun.
Article in English | MEDLINE | ID: mdl-37089965

ABSTRACT

Juvenile nasopharyngeal angiofibroma (JNA) is a histopathologically benign and highly vascular neoplasm of the nasopharynx. Surgery is the treatment of choice for this type of tumor, but its highly vascular nature could result in profuse and uncontrollable bleeding. Preoperative transcatheter arterial embolization offers an effective and minimally invasive modality for reducing intraoperative blood loss. We report 2 cases of JNA in a 17-year-old male and 14-year-old male who underwent preoperative embolization of external carotid artery branch using gelatin sponge slury and polyvinyl alcohol. Even without internal carotid artery branch embolization, both of our reported cases had intraoperative blood loss of 1100 mL and 1300 mL which are less than the previously reported mean blood loss of 1428 mL in patients who underwent both internal and external carotid artery branch embolization.

18.
Paediatr Anaesth ; 33(7): 510-519, 2023 07.
Article in English | MEDLINE | ID: mdl-36869694

ABSTRACT

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.


Subject(s)
Angiofibroma , Antifibrinolytic Agents , Nasopharyngeal Neoplasms , Male , Adolescent , Humans , Child , Angiofibroma/surgery , Angiofibroma/blood supply , Nasopharyngeal Neoplasms/surgery , Nasopharyngeal Neoplasms/blood supply , Nasopharyngeal Neoplasms/pathology , Endoscopy , Blood Transfusion
19.
Laryngoscope ; 133(11): 3216-3220, 2023 11.
Article in English | MEDLINE | ID: mdl-36896888

ABSTRACT

OBJECTIVE: To evaluate the management of juvenile nasopharyngeal angiofibroma (JNA) from a national perspective with outcomes comparison based on hospital volume. STUDY DESIGN: Ten-year Pediatric Health Information Systems (PHIS) data analysis. METHODS: The PHIS database was queried for the diagnosis of JNA. Data regarding demographics, surgical approach, embolization, length of stay, charges, readmission, and revision surgery was collected and analyzed. Hospitals were classified as low volume if fewer than 10 cases and high volume if greater than or equal to 10 cases during the study period. A random effects model compared outcomes based on hospital volume. RESULTS: A total of 287 JNA patients were identified with a mean age of 13.8 (± 2.7) years. Nine hospitals were classified as high volume, accounting for a total of 121 patients. The mean length of hospitalization, blood transfusion rate, and 30-day readmissions did not differ significantly by hospital volume. Patients cared for at high-volume institutions were less likely to require postoperative mechanical ventilation (8.3% vs. 25.0%; adjusted RR = 0.32; 95% CI 0.14-0.73; p < 0.01) or return to the operating room for residual disease than patients admitted to low-volume hospitals (7.4% vs. 20.5%; adjusted RR = 0.38; 95% CI 0.18-0.79; p = 0.01). CONCLUSIONS: The management of JNA is complex from both an operative and perioperative management standpoint. Over the past decade, nearly half (42.2%) of JNA patients have been managed at nine institutions in the United States. These centers have significantly lower rates of postoperative mechanical ventilation and the need for revision surgery. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:3216-3220, 2023.


Subject(s)
Angiofibroma , Nasopharyngeal Neoplasms , Humans , Child , Adolescent , Angiofibroma/surgery , Angiofibroma/diagnosis , Retrospective Studies , Hospitalization , Nasopharyngeal Neoplasms/surgery , Nasopharyngeal Neoplasms/diagnosis , Hospitals, Low-Volume
20.
Laryngoscope ; 133(7): 1529-1539, 2023 07.
Article in English | MEDLINE | ID: mdl-36789781

ABSTRACT

OBJECTIVE: To compare outcomes of juvenile nasopharyngeal angiofibroma (JNA) resection between embolized and non-embolized cohorts, and between transarterial embolization (TAE) and direct puncture embolization (DPE). DATA SOURCES: Per PRISMA guidelines, PubMed, Embase, Web of Science, Scopus, and Cochrane databases were searched for publications prior to or in 2021. MATERIALS AND METHODS: Original English manuscripts investigating the resection of JNA with and without preoperative embolization were included. Embolization type, recurrence rate, complication rates, blood loss, and transfusions were extracted. Risk of bias was assessed by the Risk of Bias in Non-randomized Studies-of Interventions method. RESULTS: There were 61 studies with 917 patients included. Preoperative embolization was performed in 79.3% of patients. Of those embolized, 75.8% (N = 551) underwent TAE and 15.8% (N = 115) underwent DPE. JNA recurrence in embolized patients was lower than in non-embolized patients (9.3% vs. 14.4%; odds ratio [OR]: 0.61, 95% confidence interval [CI]: 0.35, 1.06). DPE resulted in lower rates of disease recurrence (0% vs. 9.5%; OR: 0.066, 95% CI: 0.016, 0.272) and complications (1.8% vs. 21.9%; OR: 0.07, 95% CI: 0.02, 0.3) than TAE. A random effects Bayesian model was performed to analyze the difference in mean blood loss in 6 studies that included both embolized and non-embolized patients. This analysis showed a mean reduction in blood loss of 798 mL in the embolized group. CONCLUSIONS: We found embolization decreases blood loss in JNA resection. DPE led to improved recurrence and complication rates when compared to TAE, but future prospective studies are needed to further evaluate which embolization technique can optimize outcomes in JNA. LEVEL OF EVIDENCE: NA Laryngoscope, 133:1529-1539, 2023.


Subject(s)
Angiofibroma , Embolization, Therapeutic , Nasopharyngeal Neoplasms , Humans , Angiofibroma/surgery , Bayes Theorem , Neoplasm Recurrence, Local , Nasopharyngeal Neoplasms/surgery , Embolization, Therapeutic/methods
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