Central skull base osteomyelitis: new insights and implications for diagnosis and treatment.

Central skull base osteomyelitis (SBO) is a life-threatening disease originating from ear and from sinonasal infections. The intention of this study was to evaluate contemporary trends in etiology, diagnosis, management, and outcome of SBO and to draw the clinician's attention on this probably underestimated disease. Over a 6-year period we performed this systematic study in an academic quaternary medical care and skull base center including 20 patients (mean age 63.7 years) with central SBO, which is one of the largest series from a single center. In contrast to previous studies we explicitly excluded infections limited to malignant external otitis only but did not restrict central SBO to conditions unrelated to aural pathology. Fifteen patients had otogenic and five sinugenic SBO; four patients had fungal or mixed fungal infections. Pre-existing illnesses altering bone vascularization were detected in 70 % of the patients and had a negative effect on the improvement of cranial nerve palsies that were found in 14 patients. In relation, patients with otogenic SBO more often had local and systemic predisposing factors. Contrary to previous studies 16 patients (80 %) underwent surgical therapy and none of our patients died. A meta-analysis of five recent studies was done and compared with our own data and two previous meta-analyses. The present study highlights several important aspects with major implications for diagnosis and treatment of SBO that have not been adequately addressed as yet. In contrast to the restrictive attitude towards surgery in literature we recommend early and radical operative treatment to reduce its mortality.

Transmucosal core needle biopsy: a novel diagnostic approach to oral and oropharyngeal lesions.

PURPOSE: Oral and oropharyngeal tumors have often been characterized by a deep submucosal growth pattern under an intact mucous membrane. This will be particularly true for lesions of the tongue and could be associated with relevant difficulties regarding diagnosis and treatment planning. We hypothesized that the transoral transmucosal application of a core needle biopsy (CNB) for lesions of the tongue, the base of the tongue, and the floor of the mouth could be of considerable clinical usefulness for many patients. It has only previously been described in single cases and has not yet been separately discussed. MATERIAL AND METHODS: We report on the novel transoral use of CNB during rigid endoscopy with the patient under general anesthesia, with an emphasis on lesions of the tongue. A series of cases and illustrative patients are presented. RESULTS: Transmucosal CNB was accurate and of considerable clinical usefulness in all cases. The relevant advantages of this technique compared with conventional biopsy using biopsy forceps and transmucosal fine needle aspiration are presented. CONCLUSIONS: The transoral transmucosal approach to the submucosal space of the oral cavity and the oropharynx has further extended the field of application of CNB. In selected patients, this CNB approach offers essential diagnostic benefits, in particular, for tumors of the tongue.

An elderly woman with increasing dyspnoea after a fall.

An otherwise healthy 92-year-old woman was admitted to our department with shortness of breath and dysphagia 10 h after a fall in her bathroom. Medical checkup at another institution had not uncovered the causation of the complaints. Clinical and radiological examinations at our department then revealed an expanding retropharyngeal and prevertebral haematoma. Because of increasing dyspnoea, a lateral cervical approach was used to remove the haematoma and to achieve haemostasis. The authors could demonstrate that the source of bleeding was a minor injury of the anterior longitudinal ligament. Retropharyngeal haematoma is a potentially life-threatening condition because it can rapidly progress to airway obstruction. Large retropharyngeal haematoma after minor blunt head and neck trauma is not a well-recognised condition. This case, however, illustrates that precarious retropharyngeal haematoma can occur after low-energy trauma even without anticoagulation therapy. A high index of suspicion for this airway collapse is advisable in older patients.

Descending necrotizing mediastinitis: contemporary trends in etiology, diagnosis, management, and outcome.

OBJECTIVE: To evaluate contemporary trends in etiology, diagnosis, management, and outcome of descending necrotizing mediastinitis (DNM) and to draw the clinician's attention on this probably underappreciated disease. SUMMARY OF BACKGROUND DATA: An uncommon but one of the most serious forms of mediastinitis is DNM which is caused by downward spread of deep neck infections and arises as a major complication of "banal" odontogenic, pharyngeal, or cervicofacial foci. As most studies are based on small patient populations, current data on risk factors, etiology, and outcome vary significantly. Also, the optimal form of treatment remains controversial. METHODS: This retrospective study, which is the largest single-center study since 1960, is based on the management of 45 patients with DNM treated over a period of 12 years. Additionally, a meta-analysis of 26 studies on DNM published between 1999 and 2008 was performed and compared with own data and 2 previous meta-analyses covering the interval from 1960 to 1998. RESULTS AND CONCLUSIONS: Today DNM most commonly arises from pharyngeal foci and mixed polymicrobial aerobic and anaerobic infections. Reduced tissue oxygenation and impaired immune function promotes its development. Most cases of DNM are limited to the upper mediastinum and can be adequately drained by a transcervical approach. Formal thoracotomy should be reserved for cases extending below the plane of the tracheal bifurcation. Although DNM remains an aggressive infection with high morbidity, a favorable outcome can now be obtained in 85% of patients, even with this selective approach. Early diagnosis and surgical intervention are crucial.

How safe is the use of ultrasound-guided cutting needle biopsy in the head and neck?

OBJECTIVES: To analyse the risks and potential complications of cutting needle biopsy (CNB) for head and neck lesions, which have not been fully discussed previously. MATERIALS AND METHODS: We performed a systematic clinicopathological quality assessment study of 200 patients with cervicofacial lesions who underwent 444 CNB procedures with a semiautomatic biopsy gun. RESULTS: Adequate target tissue was obtained in 181 patients and revealed malignancy in 127 patients (70.2%). Follow-up uncovered 2 (1.1%) false-negative results. Emphasis was placed on the analysis of 4 minor and 2 major complications that were recorded. One patient suffered from a cerebrovascular event and died after dissection of a distinctively kinked carotid artery. The risks of CNB are discussed and compared to fine-needle aspiration cytology (FNAC) and open biopsy and related to series of parameters. CONCLUSION: CNB is a safe and reliable technique in the majority of cases but its risks in the head and neck are not negligible due to the proximity of major neurovascular structures and airways. We recommend special attention after neck surgery and radiotherapy and when the target is not clearly circumscribed and is close to major vessels.

Minimal-invasive core needle biopsy of head and neck malignancies: clinical evaluation for radiation oncology.

BACKGROUND AND PURPOSE: Neck masses are common presentations of cancer and require tissue sampling to establish a diagnosis prior to the institution of adequate therapy. The purpose of this study was to evaluate the specific use and potential advantages of core needle biopsy (CNB) for radiation oncology in the head and neck. MATERIALS AND METHODS: We performed a retrospective analysis of 346 CNB procedures implemented in 165 patients with cervicofacial masses over a period of 56 months. Seventy-three patients had a history of malignancy, 43 had previously received radiation. RESULTS: High-quality tissue cores were obtained from all patients. The target lesion was correctly sampled in 92.1% of patients. Final diagnosis was malignant in 115 patients, of whom 78 received radiotherapy. One biopsy result was false-negative. CNB was equally successful in the pre-irradiated patients and enabled institution of therapy in 92.3% of lymphoma patients. CONCLUSIONS: CNB is an efficient tool for tissue sampling of head and neck masses. The potential advantages of CNB for radiation oncology over fine needle aspiration and open surgical biopsy are discussed. CNB enables clinicians to make optimal therapeutic strategies and facilitates prompt referral to the relevant clinical team, both at initial presentation and during follow-up.

Malignant peripheral nerve sheath tumour of the paranasal sinuses and the anterior skull base.

BACKGROUND: Malignant peripheral nerve sheath tumours (MPNSTs) are highly aggressive neoplasms with a marked propensity for local recurrence and metastatic spread. The management of MPNSTs continues to challenge pathologists and surgeons. As MPNSTs of the paranasal sinuses and the skull base are rare, prognostic factors and treatment modalities have not been consistently identified. PATIENTS AND METHODS: We present a case of MPNST of the anterior skull base and provide an overview of all MPNSTs reported since 1970, in which the tumour location was the anterior skull base or the paranasal sinuses. RESULTS: Literature review revealed 33 well-documented cases of MPNSTs in this anatomic location. These cases were analysed with emphasis on age, gender, affected site, therapy, outcome, presence of neurofibromatosis, local recurrence and metastases. CONCLUSIONS: Despite multimodal therapy and advances in surgical techniques, the prognosis of MPNST located in the paranasal sinuses and the anterior skull base remains dismal. Outcome is mainly a function of local control by surgical resection. Adjuvant radiochemotherapy has shown no benefit. It may therefore be advisable to abstain from radiochemotherapy in order to improve chances for surgical intervention in case of recurrent disease. Close follow-up investigations are indispensable.

Posttraumatic reactive fibrous bone neoformation of the anterior skull base mimicking osteosarcoma.

OBJECTIVES: Malignant bone tumors and fibro-osseous bone lesions of the skull base are uncommon, although fibrous dysplasia in this anatomic location is not a rare condition. In general, fibro-osseous lesions of the skull are often difficult to classify on either clinical presentation, radiological findings, or histological presentation alone. The objective of this article is to present a probably important differential in the management of bony neoformations of the skull and to highlight the diagnostic difficulties when dealing with osseous and fibro-osseous conditions affecting the craniofacial bones. DESIGN: We present here a novel case of posttraumatic reactive fibrous bone neoformation of the anterior skull base mimicking osteosarcoma in a 16-year-old boy. RESULTS: Diagnostic steps, clinical, histological, and radiological presentation, as well as surgical treatment are described in detail. The international medical literature concerning reactive fibrous bone neoformations is reviewed, and the problem of adjusting the correct differential diagnosis when dealing with fibro-osseous bone lesions of the skull base is discussed. CONCLUSIONS: The highlights of this case are an uncommon location of a rare pathological entity, which might constitute an important differential of fibro-osseous conditions affecting the craniofacial bones.

Aberrant extracranial internal carotid arteries: New insights, implications, and demand for a clinical grading system.

BACKGROUND: Aberrant internal carotid arteries (ICAs) are not rare anatomic variations. The purpose of this study was for us to highlight their relevance and underline the need for a clinical grading system in addition to the currently used anatomic classification. METHODS: This systematic quality assessment study over 7 years includes 125 adults with 231 aberrant ICAs. Digital imaging datasets were analyzed and ICAs classified according to the anatomic classification in tortuosity, kinking, and coiling. Risk potential for ICA injury was estimated from the vessel's relation to the pharynx and neck. RESULTS: The analysis revealed a high frequency of medial and lateral ICA aberrations that seem surgically relevant, but the risk potential for injury was not reflected by the anatomic classification. Our previously proposed clinical grading system was reviewed and updated. CONCLUSION: Aberrant ICAs are underestimated anatomic variations with high clinical impact for transoral and transcervical interventions. The introduced clinicoradiologic grading system could be of major benefit. © 2015 Wiley Periodicals, Inc. Head Neck 38: E687-E693, 2016.

Bilateral tinnitus due to middle-ear myoclonus.

Tinnitus is a common otological symptom. Usually it is subjective (perceived only by the patient); very rarely is it objective (heard by both the patient and the examiner) Objective tinnitus due to middle-ear myoclonus is extremely rare, with only a few case reports published in the literature. We present three cases of objective tinnitus caused by middle-ear myoclonus. All patients were cured by tympanotomy with stapedial and tensor tympani tendon section.

The clinical impact of aberrant internal carotid arteries in children.

OBJECTIVE: Variations in the course of the internal carotid artery are not rare and likely to be a congenital anomaly. The purpose of this study is to highlight the clinical impact of aberrant internal carotid arteries in children for surgical and interventional procedures in the head and neck. METHODS: Retrospective study of the vessel course in patients under the age of 18 years over a period of 4.5 years. Vessels were classified according to the anatomic classification by Weibel and Fields and related to a previously proposed clinicoradiological risk classification system. Clinical implications are pointed out. RESULTS: Nine patients with a total of 14 aberrations (4 tortuosities, 7 kinkings and 3 coilings) were included. All aberrations were incidental intraoperative or radiological findings; no patient suffered from distinct symptoms. The minimum distance to the pharyngeal wall ranged from 0.8 to 9.0 mm with a mean of 3.8 mm. Anatomic classification and clinical risk of injury did not correlate. CONCLUSIONS: Routine pharyngeal surgery and interventional procedures are usually performed without any preoperative imaging. However, variations in the course of the internal carotid artery may considerably increase the risk of vessel injury and should always be anticipated.

Lemierre syndrome associated with 12th cranial nerve palsy--a case report and review.

Since the widespread availability and use of antibiotics the prevalence of Lemierre syndrome (L.S.) has decreased. It is a well-described entity, consisting of postanginal septicaemia with thrombophlebitis of the internal jugular vein with metastatic infection, most commonly in the lungs. The most common causative agent is a gram-negative, non-spore-forming obligate anaerobic bacterium, Fusobacterium necrophorum (F.n.). We describe the unusual clinical features of a 12-year-old boy with Lemierre syndrome with isolated hypoglossal nerve palsy - the latter symptom is an extremely rare manifestation of this disease.

LOH-profiling by SNP-mapping in a case of multifocal head and neck cancer.

AIM: To introduce an approach for the detection of putative genetic host factors that predispose patients to develop head and neck squamous cell carcinomas (HNSCC). METHODS: HNSCC most often result from the accumulation of somatic gene alterations found in tumor cells. A cancer-predisposing genetic background must be expected in individuals who develop multiple cancers, starting at an unexpectedly young age or with little carcinogen exposure. Genome-wide loss of heterozygosity (LOH) profiling by single nucleotide polymorphism microarray mapping was performed in a patient with a remarkable history of multifocal HNSCC. RESULTS: Regions of genomic deletions in germline DNA were identified on several chromosomes with a remarkable size between 1.6 Mb and 8.1 Mb (mega base-pair). No LOH was detected at the genomic location of the tumor suppressor gene P53. CONCLUSION: Specific patterns of germline DNA deletions may be responsible for susceptibility to HNSCC and should be further analyzed.

An elderly woman with increasing dyspnoea after a fall.

An otherwise healthy 92-year-old woman was admitted to our department with shortness of breath and dysphagia 10 h after a fall in her bathroom. Medical checkup at another institution had not uncovered the causation of the complaints. Clinical and radiological examinations at our department then revealed an expanding retropharyngeal and prevertebral haematoma. Because of increasing dyspnoea, a lateral cervical approach was used to remove the haematoma and to achieve haemostasis. The authors could demonstrate that the source of bleeding was a minor injury of the anterior longitudinal ligament. Retropharyngeal haematoma is a potentially life-threatening condition because it can rapidly progress to airway obstruction. Large retropharyngeal haematoma after minor blunt head and neck trauma is not a well-recognised condition. This case, however, illustrates that precarious retropharyngeal haematoma can occur after low-energy trauma even without anticoagulation therapy. A high index of suspicion for this airway collapse is advisable in older patients.

Head and neck cancer in young adults and nonsmokers: study of cancer susceptibility by genome-wide high-density SNP microarray mapping.

CONCLUSION: Our results raise the question as to whether specific patterns of 'germline loss of heterozygosity (LOH)' could contribute to the genetic susceptibility for head and neck squamous cell carcinoma (HNSCC). OBJECTIVES: HNSCC usually occurs in older individuals with a history of smoking. However, about 5% of HNSCC patients have never used tobacco or develop this disease at an exceptionally young age. Therefore, genetic susceptibility must contribute significantly to HNSCC risk. The objective was to introduce a novel approach that might help to unveil candidate genes contributing to cancer predisposition and to identify individuals at risk for HNSCC, and to present our observations with this method in a specific group of patients. METHODS: High-resolution SNP (single-nucleotide polymorphism) microarray mapping for homozygous stretches in germline DNA was performed in 12 patients who appeared particularly susceptible to develop HNSCC, because they were exceptionally young or never users of tobacco. RESULTS: We could identify strings of consecutive homozygous SNPs that were much longer than would be expected to appear by chance alone, indicating regions of DNA deletions that we named germline LOH.

A minor wound with a fatal course.

The authors report on a fatal case of severe tetanus in a 74-year old woman. Despite comprehensive intensive care management they could not achieve a satisfying control of the autonomic dysfunction caused by tetanus. By now there is no established therapy for the treatment of the autonomic dysfunction. This report demonstrates the dismal prognosis of severe tetanus in the older people, which is often complicated by cardiovascular comorbidity and underlines the importance of tetanus prevention by sufficient vaccination.

Diagnostic effectiveness of sonography-assisted cutting needle biopsy in uncommon cervicofacial lesions.

OBJECTIVE: Persistent cervicofacial masses require tissue sampling to guide treatment. While open biopsy is invasive, fine-needle aspiration cytology is insufficient to establish a diagnosis in rare clinical or pathological conditions. The cutting needle biopsy (CNB) is not a widely used technique in the head and neck, and its diagnostic effectiveness in uncommon cervicofacial lesions has not been evaluated as yet. STUDY DESIGN: This was a systematic clinicopathologic quality assessment study. We performed 347 CNBs in 160 patients with unclear masses including 29 patients in whom final diagnosis revealed rare conditions. RESULTS: We experienced 100% success in obtaining high-quality tissue cores. CNB enabled accurate diagnosis even in highly exceptional lesions. One false negative result was recorded. CONCLUSION: Adequate assessment of rare lesions is one of the major benefits of CNB over fine-needle aspiration. CNB can therefore be recommended as the initial procedure in cervicofacial masses, even if an uncommon causation is presumed.

Sonography-assisted cutting needle biopsy in the head and neck for the diagnosis of lymphoma: can it replace lymph node extirpation?

OBJECTIVES: Malignant lymphoma frequently present with cervical lymph node enlargement. Before adequate therapy can be instituted, appropriate biopsy specimens must enable definite subclassification of the disease. While lymph node extirpation, traditionally considered the gold standard in the diagnostic evaluation of lymphoma, is invasive, diagnoses from fine needle aspiration cytology are often clinically insufficient on which to base treatment decisions. The aim of this study was to evaluate the diagnostic accuracy of cutting needle biopsy (CNB) in the management of patients in whom lymphoma was diagnosed or suspected in the head and neck. STUDY DESIGN: Prospective quality assessment study in an academic tertiary care medical center. METHODS: A systematic clinicopathological follow-up study was performed over a period of 54 months, including the results of 347 CNBs in 160 patients with cervico-facial masses. Ninety-seven CNBs were done in 45 patients with malignant lymphoma as the final or the tentative diagnosis. RESULTS: We experienced 100% success in obtaining high-quality tissue cores. The target lymph node was correctly sampled in 41 of the 45 patients. Within these 41 patients, no false positive or false negative results were recorded. Full subclassification of the disease with prompt institution of therapy was possible in 92.3% of the lymphoma patients. CONCLUSIONS: CNB proved to be a sufficient alternative to open biopsy with excellent diagnostic yield and clinical usefulness. We recommend this procedure as the initial diagnostic step in patients with suspected lymphoma, either at presentation or at recurrence, and advocate its use in peripheral lymphadenopathy of the neck.

A clinical classification system for aberrant internal carotid arteries.

OBJECTIVES/HYPOTHESIS: Pronounced anatomic variations of the extracranial internal carotid artery (ICA) can be found in 5% to 6% of the general population. An aberrant ICA is at risk of injury during routine pharyngeal procedures if the vessel is placed into close relationship with the pharyngeal wall. The objective was to highlight the impact of parapharyngeal ICA aberrations and to analyze the clinical usefulness of the current anatomic classification system of these anomalies. STUDY DESIGN: Prospective quality assessment study in an academic tertiary care medical center. METHODS: A systematic clinicoradiological follow-up study was performed over a period of 18 months. The ICA's course was classified according to Weibel and Fields in 21 consecutive patients with 35 pronounced parapharyngeal ICA aberrations. The vessels' minimum distance to the pharyngeal wall and the corresponding pharyngeal level was analyzed. RESULTS: Tortuosity was found in 13, kinking in 18, and coiling in 4 aberrant ICAs. The vessel's minimum distance to the pharyngeal wall ranged from 0.8 to 17.9 mm (mean, 7.0 mm), but the currently used anatomic classification was inadequate to determine the risk potential for ICA injury in the given cases. We propose a clinicoradiological classification system, which considers the vessel's relation to the pharyngeal wall. CONCLUSIONS: Parapharyngeal ICA aberrations are common and probably under-appreciated anatomic variations. The awareness of these anomalies is essential for clinicians. Since the current anatomic classification is insufficient to outline patients with an increased risk of ICA injury, a clinicoradiological graduation of cervical ICA aberrations may be of practical benefit.