The value of color doppler ultrasonography combined with computed tomography angiography and magnetic resonance angiography in the preoperative quantification and classification of carotid body tumors: a retrospective analysis.

BACKGROUND: Computed tomography angiography (CTA) and magnetic resonance angiography (MRA) provide accurate vascular imaging information, but their use may be contraindicated. Color Doppler ultrasonography (CDU) provides simple, safe, noninvasive, and reproducible imaging. We therefore investigated the role of preoperative CDU combined with CTA and MRA in the quantification, typing, and diagnosis of carotid body tumors (CBTs). METHODS: We retrospectively analyzed patients with CBTs categorized into group A (type I [n = 1] and type II [n = 10]) or group B (type III [n = 56]) per the intraoperative Shamblin classification. CDU, CTA, and MRA characteristics of CBTs were observed, surgical results were correlated, and the diagnostic threshold of the CBT classification was calculated. RESULTS: CBTs were usually located at the common carotid artery bifurcation, encircling the carotid artery. An increased angle was found between the internal and external carotid arteries. On CDU, CBTs primarily presented as homogeneous hypoechoic masses with clear boundaries, rich flow signals, and a high-speed, low-resistance artery-like flow spectrum. CTA showed uniform or heterogeneous marked enhancement. MRA showed mixed T1 and slightly longer T2 signals and uniform or uneven obvious enhancement. With increases in the lesion size, amount of blood transfused, and operation time, the intraoperative classification level and possibility of skull-base invasion increased. When the maximum diameter of the lesion, the volume of the tumor, the distance between the upper margin of the tumor to the mastoid and the mandibular angle were 3.10 cm, 10.15 cm3, - 3.26 cm, and 0.57 cm, respectively, the largest Youden index was the best diagnostic boundary value for Shamblin type III tumors. CONCLUSIONS: CDU combined with CTA and MRA can accurately evaluate the size and classification of CBTs.

The Double Mandibular Osteotomy for Vascular and Tumor Surgery of the Parapharyngeal Space.

PURPOSE: The purposes of this study are to describe our experience using a double mandibular osteotomy for access to the parapharyngeal space in vascular and tumor surgery and to report on the outcomes and complications of this procedure. PATIENTS AND METHODS: We designed and implemented a case series to review the medical records of all patients treated with a double mandibular osteotomy for parapharyngeal space access from 1994 to 2016. Patient demographic characteristics, indications for the procedure, outcomes, and complications were recorded. RESULTS: A total of 17 patients underwent a double mandibular osteotomy procedure for access to the parapharyngeal space during the study period. There were 7 men (41%) and 10 women (59%) comprising the cohort. The average age was 57 years (range, 29 to 75 years). The follow-up period ranged from 6 to 98 months (mean, 40 months), and 7 patients (41%) were tobacco users at the time of surgery. The most common indication was high internal carotid artery stenosis (n = 6) followed by carotid body paraganglioma (n = 3). Average blood loss was 186 mL, and there were no deaths during the study period. Eight postoperative complications were noted in 7 patients (41%). No procedures were aborted or compromised because of inadequate parapharyngeal space access. All patients showed clinical and radiographic signs of healing of the osteotomy sites. CONCLUSION: The double mandibular osteotomy provides adequate access to the parapharyngeal space for effective tumor removal and high carotid surgical intervention with acceptable patient morbidity and complications.

[Percutaneous Phil™-Embolization for Preoperative Therapy of Carotid Body Paragangliomas]. / Perkutane Phil™-Embolisation zur präoperativen Therapie von Karotis-Paragangliomen.

Introduction: Paragangliomas are rare tumors representing a therapeutic challenge. In particular, the surgical removal may lead to life-threatening bleeding. The preoperative percutaneous embolization is an alternative that allows a high closure rate of tumor-feeding vessels in a short intervention time and thus significantly reduces intraoperative bleeding probability. Complete tumor resection is facilitated thereby. The use of a new non-adhesive liquid embolic agent is presented here. Methods: A 50-year old patient presented with 4 cm large paraganglioma of the carotid body (Shamblin II). A percutaneous embolization with 7 ml PHIL™ (injectable precipitating hydrophobic liquid) was performed preoperatively. 24 h later the complete surgical resection of the tumor was performed. Results: A good distribution of the liquid embolic agent could be achieved over the entire tumor. Intraoperative resection of the tumor was much easier and faster due to low bleeding tendency over the entire surface of the tumor. Total blood loss was less than 50 ml. All adjacent nerve and arterial structures could be spared. Postoperative nerve function was normal and the patient was discharged on the 4th postoperative day. Conclusion: The combination of percutaneous embolization and surgical resection provides a safe combination in the treatment of advanced carotid body paragangliomas. The use of a novel liquid embolic agent may possibly further optimize the therapy.

Carotid Paraganglioma - Classical Surgical Technique.

INTRODUCTION: A carotid body tumor is a rare neoplasm, generally benign, that predominantly affects people between their fourth and fith decades of life. It manifests as a pulsatile and generally painless cervical mass with firm consistency, located below the angle of the jaw. Clinically it can cause localized pain, dysphagia, hiccups, hoarseness and hypersensitive carotid body syndrome. Surgery is the treatment of choice, bearing in mind the possibility of malignant transformation, peritumoral invasion and metastasis. The most widely-used technique is surgical resection, with or without concomitant preoperative endovascular embolisation. Overall complication rates, stroke rates between 0 and 8% and cranial nerve palsy less than 1% to 49%. Mortality rates vary from 0 to 3%. METHODS: Clinical case of a 69 years old male patient diagnosed with a carotid body tumor in a routine ultrasound exam. The patient was asyntomatic. Complementary exams were then conducted - CT scan and MRI supported the diagnosis. Neck CT scan: Well defined, nodular formation, enhanced after intravenous contrast, localized on the jugular-carotid region, with an aproximate diameter of 36 mm. Neck MRI: Expansive heterogenous solid lesion, localized on carotid bulb, well defined, enhanced after intravenous contrast, compatible with carotid paraganglioma - Shamblin's II. RESULTS: Patient was submited to a complete surgical classic ressection of the tumor, without any previously procedure. Proximal dissection was made with a help of a nose and ear surgeon. No post-surgery complications, except wound infection at week 3. No nerve damage. CONCLUSION: Follow up to 1 year without any complain and no lesions. In an era of multiple techniques there should always be a place for classic, well planned surgeries.

Recurrent carotid body tumors: embolization as a treatment option.

INTRODUCTION: We report a clinical case where transcatheter embolization was selected as the primary treatment for a large recurrent carotid body tumor CLINICAL CASE: A 55 year-old female presented with a painful left cervical mass, with progressive growth, for the past 12 months. She complained of jaw stiffness, odynophagia and dysphonia. She had a former history of bilateral carotid body tumor resection. The patient underwent carotid ultrasound examination that showed a recurrent left carotid body tumor with 7x5 cm in dimension, and occlusion of the left internal carotid artery. Magnetic resonance imaging confirmed the presence of a Shamblin type III tumor. The patient underwent transcatheter embolization of the tumor with 300-500 µm and 500-700 µm Bead-Block®. At 1 year of follow-up, the patient was found asymptomatic. DISCUSSION: The carotid body is located at the bifurcation of the common carotid artery. With increased size, carotid body tumors can induce significant symptoms and are usually detected by clinical examination. Confirmation of diagnosis is usually given by vascular ultrasound. For highly symptomatic, recurrent and frequently unresectable tumors--in patients unfit for surgery--transcatheter embolization can also be used as an effective palliative treatment.

Evaluating the Potential of Preembolization Using Ethylene-Vinyl Alcohol (Onyx and Squid) in Carotid Body Tumor Paraganglioma.

OBJECTIVE: This study aims to evaluate the efficacy and safety of preoperative embolization, used 48 hours before surgery to reduce tumor size and surgical complications in carotid body paragangliomas. METHODS: This systematic review and meta-analysis followed Preferred Reporting Items for Systematic Reviews and Meta-Analysis and Cochrane Handbook. A comprehensive literature search was performed in Medline, Embase, Web of Science, and Cochrane databases. The inclusion criteria were: 1) observational studies, 2) reporting on diagnosed carotid body paragangliomas, 3) undergoing preoperative embolization procedures, and 4) with ethylene-vinyl alcohol as an embolic agent. RESULTS: The study analyzed 106 patients, aged 18-79, using primarily Onyx 18 for embolization, with treatment intervals ranging from 24 hours to 2 weeks. Efficacy outcomes showed near-total devascularization in 67% of cases (95% confidence interval [CI]: 0.47-0.87; I² = 74%), subtotal devascularization in 33% (95% CI: 0.12-0.54; I² = 43%), and total devascularization in 97% (95% CI: 0.88-1.00; I² = 41%), indicating significant heterogeneity across outcomes. The mean estimated blood loss was 184.46 ml (95% CI: 116.72-252.20 ml). Postembolization complication rate was exceptionally low at 1% (95% CI: 0.00-0.06; I² = 0%). CONCLUSIONS: In conclusion, preoperative embolization of carotid body tumors achieved high rates of devascularization with minimal blood loss and a very low incidence of complications, highlighting its effectiveness and safety as a treatment strategy.

How to do it: excision of carotid space tumours in proximity to skull base.

This article along with the descriptive video demonstrates the step-by-step surgical approach for excision of tumours located in carotid space in proximity to skull base. We have described the surgical steps without mandibular osteotomy and also demonstrated the technique to safeguard all neuro-vascular anatomy in the vicinity of the carotid space and skull base.

Double osteotomy of mandibula in the treatment of carotid body tumors with skull base extension.

We report two patients with a carotid body paraganglioma that extended to the skull base, a position that is surgically inaccessible by means of a traditional lateral cervical approach. In both patients we were able to remove the lesion by performing a double mandibular osteotomy. Both patients underwent preoperative embolization to reduce the mass. In our experience, this approach has allowed a safe radical excision of exceptionally high lesions with only minor permanent nerve damage. In our opinion this advantage definitely outweighs the consequences of the increased invasiveness of this technique.

A comparison of particulate and Onyx embolization in preoperative devascularization of carotid body tumors.

INTRODUCTION: Preoperative embolization of a carotid body tumor (CBT) is a useful adjunct prior to surgical excision because it decreases operative blood loss and improves surgical outcomes. Traditionally, this is performed by transarterial particulate embolization (TAPE). More recently, direct percutaneous embolization (DPE) with Onyx is recognized as a promising technique for preoperative embolization. We compared these two techniques in patients treated for CBTs at our institution. METHODS: We retrospectively reviewed cases of preoperative devascularization of CBT from 1 January 1995 through 1 September 2012. Patient cases were placed into two groups: TAPE and DPE. Operative blood loss, operative length, angiographic devascularization, embolization procedure complications, operative transfusion requirements, postoperative hospital stay, intensive care unit (ICU) stay, and procedure-related mortalities were compared. RESULTS: A total of 17 patients underwent preoperative devascularization of their CBT with TAPE technique and ten patients using the DPE technique with Onyx. Average operative blood loss was significantly higher in the TAPE group (Mann-Whitney U test, p = 0.04). Operative time was also higher, although this difference was not significant. Two patients required intraoperative blood transfusions in the TAPE group while none required transfusions in the DPE group. There was no significant difference in ICU stay or length of hospitalization. One serious embolization procedure complication occurred in the TAPE group and none in the DPE group. CONCLUSION: Operative blood loss in the DPE group was significantly less than the TAPE group. Blood transfusion requirement, operative time, and complications were less in the DPE group, although they did not reach statistical significance.

n-Butyl cyanoacrylate-induced multiple retinal arteriolar occlusions.

We report a case of multiple retinal arteriolar occlusions due to delayed embolisation of N-butylcyanoacrylate glue, initially injected in the external carotid artery for the management of Carotid body tumour. Ocular massage and anterior chamber paracentesis were unfruitful and patient suffered irreversible visual loss in the affected eye. Embolisation of N-butyl cyanoacrylate glue into the retinal vasculature after intratumoral injection should be kept in mind as a rare but possible complication by radiologists and ophthalmologists.

Intraoperative embolization with poloxamer 407 during surgical resection of a carotid body tumor.

Surgical excision is the preferred definitive treatment for carotid body tumors, although postoperative morbidity rate as quoted in the literature is rather high. Morbidity includes cranial nerve dysfunction, stroke, and the majority severe blood loss. Embolization of the feeding branches of the external carotid artery can be performed a few days prior to surgery with the intention to decrease blood loss during operation, facilitate surgical resection, and reduce operating time and morbidity. The special risk of embolization is migration into the intracranial circulation. Poloxamer 407, a reverse-thermal polymer, is a nontoxic compound that is a viscous liquid at room temperatures but instantly changes to a firm water-soluble gel when warmed to body temperature. It dissolves spontaneously or can be dissolved at will by cooling. We describe an intraoperative technique for complete devascularization of carotid body tumor by using an intraarterial temporary occlusion technique with a poloxamer 407.

Comparison of 3 techniques of surgical treatment of carotid body tumors.

OBJECTIVE: Carotid body tumors (CBTs) are benign but challenging. This study compared outcomes of 3 techniques of the surgical treatment of CBTs. STUDY DESIGN: This retrospective observational study was conducted from April 2013 to March 2019. The 38 patients enrolled in the study had primary tumors, including 1 with bilateral tumors and another with adrenal gland pheochromocytoma. We collected data on age, sex, size of tumor, Shamblin classification, treatment, blood loss, operative time, hospital stay, complications, and recurrence. Statistical analyses were performed using IBM SPSS Statistics version 20 software. RESULTS: Twenty-four patients were male, and 12 were female, and they ranged in age from 11 to 71 years. Cases were assigned to Shamblin groups I (n = 6), II (n = 19), and III (n = 14). Tumor size ranged from 2.0 × 2.0 cm to 5.0 × 6.0 cm. Eleven CBTs underwent blunt dissection (BD), 20 underwent BD plus resection of external carotid artery division plus vessels of encapsulation with allograft dermal matrix (BD + RECA + VE), and 8 tumors underwent surgical resection of tumors plus common carotid artery-internal carotid artery artificial vascular reconstruction (SR +C-IAVR). No perioperative death or stroke occurred. There was a significant difference between Shamblin groups I, II, and III in terms of the size of the tumor, type of treatment used, blood loss, operative time, hospital stay, and complications. Six patients had mandibular branch facial nerve transient paresis; 7 patients had hypoglossal nerve dysfunction; 3 patients had Horner syndrome; and dysphasia occurred in 2 patients. The patients were seen in follow-up for 16 to 45 months, and 1 recurrence was observed. CONCLUSIONS: Three surgical techniques-BD, BD + RECA + VE, and SR + C-IAVR-are safe and feasible for the treatment of CBTs according to Shamblin classifications.

Early side effects after embolization of a carotid body tumor using Onyx.

The case of a 20-year-old woman with a carotid body tumor of Shamblin class III is reported. Ten hours after preoperative direct intralesional embolization with 20 mL Onyx (ethylene-vinyl alcohol copolymer; Micro Therapeutics, Irvine, Calif), the patient showed symptoms of Horner syndrome and deficits of the hypoglossal and glossopharyngeal nerves. Intraoperative examination 12 hours after Onyx embolization revealed a massive swelling of the hypoglossal and glossopharyngeal nerves. The patient's tongue motility and glossopharyngeal function improved after surgery, but Horner syndrome was still present. Owing to the delayed occurrence of these adverse effects, the optimal time of surgical intervention after Onyx embolization should be discussed and perhaps expedited.

Direct percutaneous embolization of a carotid body tumor with Onyx.

Carotid body tumors (CBTs) are rare highly vascular lesions that frequently require preoperative embolization to minimize surgical morbidity secondary to blood loss. Embolization has typically been performed via a transarterial route. However, this frequently results in incomplete devascularization of the tumor due to the complex angioarchitecture of the feeding arteries. Direct intralesional embolization has been used to gain easier accesses to the tumor vasculature and thus increase the likelihood of complete embolization. Cyanoacrylate glue has been the most commonly used embolic agent. The authors present a case of CBT that underwent direct intralesional embolization using Onyx (ev3; ethylene vinyl alcohol copolymer). To their knowledge, there have been no previous reports of direct percutaneous embolization of a CBT with this agent.

[Direct percutaneous embolization of a carotid body tumor with Onyx]. / Perkutane Onyx(R)-Embolisation zur Therapie von Glomus-caroticum-Tumoren.

Carotid body tumors are highly vascularized lesions that require successful preoperative embolization to achieve favorable clinical results in terms of morbidity and complete tumor resection. The procedure of percutaneous embolization was performed using ethylene-vinyl alcohol copolymer (Onyx) in addition to balloon-catheter protection to prevent particle displacement into the internal carotid artery. The procedure resulted in nearly complete tumor embolization and facilitated the uneventful complete surgical resection. Percutaneous angiographic embolization of carotid body tumors in the head and neck was found to be safe and effective. This technique is likely to result in improved surgical outcomes and tumor resectability.

Successful Resection of a Large Carotid Body Tumor Masquerading Complete Encasement of the Internal Carotid Artery on Preoperative Imaging.

A 41-year-old woman presented with a large painful and tender mass of the left side of her neck located just below the angle of the mandible. She was also complaining of frequent attacks of symptoms ranging from dizziness upon resuming the erect position to frank syncope. Color-coded duplex showed a large well-vascularized vascular mass at the level of the carotid bifurcation, suggesting the diagnosis of a carotid body tumor (CBT). A computerized tomographic angiography confirmed the diagnosis of a CBT, which measured 5.7 cm in its craniocaudal axis. The tumor appeared to encase the internal carotid artery (ICA) at the level of its origin, indicating the presence of a Shamblin group 3 tumor. During surgery, the CBT seemingly encased the ICA; however, it was successfully taken off the ICA, by establishing an adventitial dissection place, obviating the need for arterial resection and replacement. A brief intraoperative episode of cardiac arrest was successfully managed. Postoperative course was uneventful, and all symptoms were cured. Pathology confirmed the clinical diagnosis and did not show malignancy. In conclusion, meticulous surgical techniques can spare the ICA from removal or inadvertent injury during CBT surgery and reduce the morbidity often associated with the resection of large or advanced tumors but also cure atypical patient symptoms.

Stroke from delayed embolization of polymerized glue following percutaneous direct injection of a carotid body tumor.

A 52-year-old male with right carotid body tumor underwent direct percutaneous glue (n-butylcyanoacrylate [NBCA]) embolization. Several hours later, he developed left hemiparesis from embolization of the polymerized glue cast. Migration of glue during percutaneous tumor embolization is presumed to occur only in the liquid state, which may lead to stroke or cranial nerve deficits. To the best of our knowledge, this is the first report of delayed glue embolization from a treated hypervascular tumor of the head and neck.

Mandibular Lytic Lesion in Familial Paraganglioma Syndrome Type I: A Clinical Conundrum.

OBJECTIVE: The entity of primary mandibular paraganglioma (PGL) is not well accepted within the head and neck. Mandibular PGLs hitherto reported in literature are malignant metastatic lesions, mostly from a pheochromocytoma. METHODS: We report a case of mandibular lytic lesion in a young female with multifocal PGLs but no family history of PGLs. We also performed a literature search to identify published cases of mandibular PGL. RESULTS: Lack of established criteria for malignancy in a PGL made diagnosis and treatment challenging. Testing was negative for a pheochromocytoma and positive for mutation of succinate dehydrogenase gene encoding subunit D (SDHD), thus rendering a diagnosis of familial PGL syndrome type I. Due to the absence of prior published reports of nonmalignant, primary mandibular PGL, patient was treated with surgery and postoperative radiotherapy. Our literature search revealed 4 published cases of mandibular PGL, all of which had an osteoblastic appearance and were malignant. CONCLUSIONS: Isolated mandibular PGL does not always indicate a malignant metastatic lesion. Genetic testing is recommended in patients with early onset of PGL and/or multifocality even without a positive family history. Surgical resection alone with surveillance can be offered for such isolated lesions in the presence of familial PGL syndrome type I.

A multidisciplinary approach to carotid paragangliomas.

The surgical management of carotid paragangliomas can be problematic. A multidisciplinary approach was used to include vascular surgery, otolaryngology, and neuroradiology to treat these patients over 9 years. From January 1992 to July 2001, a multidisciplinary team evaluated patients with carotid paragangliomas. Analyzed patient data included age, gender, diagnostic evaluation, tumor size, preoperative tumor embolization, operative exposure, need for extracranial arterial sacrifice/reconstruction, postoperative morbidity including cranial nerve dysfunction, and long-term follow-up. Twenty-five carotid paragangliomas in 20 patients underwent multidisciplinary evaluation and management. Average age was 51 years (range, 28-83 years), and 52% were male. Diagnostic evaluation included computed tomography in 76%, magnetic resonance imaging/magnetic resonance angiography in 52%, catheter angiography in 60%, and duplex ultrasonography in 16%. An extended neck exposure was required in 11 cases (44%), mandibulotomy was used once (4%), and mandibular subluxation was never required. The external carotid artery (ECA) was sacrificed in 8 cases (32%). The carotid bifurcation was resected in 1 patient (4%) requiring interposition reconstruction of the internal carotid artery. Preoperative tumor embolization was performed for 13 tumors (52%). Operative blood loss for patients undergoing preoperative embolization (Group I) was comparable to the nonembolized group (group II): group I lost 365 +/-180 mL versus 360 +/- 101 mL for group II (P = .48). This occurred despite larger tumors (group I - 4.2 cm versus group II - 2.1 cm, P = .03) and a higher mean Shamblin class (group I - 2.5 versus group II - 1.45, P = .001) for group I. There were no perioperative mortalities. Transient cranial nerve dysfunction occurred in 13 CBTs (52%), 2 (8%) of which remained present after 4 months. Patients with carotid paragangliomas benefit from a multidisciplinary team approach. Neuroradiology has been used for selective preoperative embolization, which has decreased estimated blood loss during excision of larger complex tumors. A combined surgical team of otolaryngology and vascular surgery provides for exposure of the distal internal carotid artery as high as the skull base, limited permanent cranial nerve dysfunction, and selective early division and excision of the external carotid artery for complete tumor resection.

Avoiding pitfalls in surgery of the neck, parapharyngeal space, and infratemporal fossa.

The region of the upper neck, parapharyngeal space, and infratemporal fossa contains many vital structures in a confined area. Access is often limited, making surgical treatment challenging. Preoperative assessment is critical in determining the optimal surgical approach. In spite of improvements in preoperative evaluation and surgical techniques, sequelae from surgical therapy are sometimes unavoidable. Awareness of the potential problems is necessary to counsel patients appropriately regarding treatment options and all potential risks.