Descending necrotizing mediastinitis: etiopathogenesis, diagnosis, treatment and long-term consequences-a retrospective follow-up study.

PURPOSE: The primary aim of this retrospective study was to analyze the progression of descending necrotizing mediastinitis (DNM), evaluate the impact of comorbidities on complications and mortality and to observe long-term consequences of DNM on dysphagia and measurements quality of life. DNM is a serious infectious disease that requires multimodal treatment. Current literature varies in conclusions of risk factors, management and outcome of DNM. In addition, little is known about persisting effects on quality of life. METHODS: Retrospective data analysis of 88 patients with DNM representing the largest single-center study. Recording data of patients and diseases as well as clinical progression from 1997 to 2018. Two questionnaires were sent to the participants to measure quality of life and to detect dysphagia. RESULTS: 88 patients were included. The most frequently found pathogen were Streptococcus spp. (52%). 75% of the patients underwent multiple surgeries, mean count of surgical procedures was 4.3 times. 84% received intensive care treatment. Median length of stay on the intensive care unit was 7 days. 51% had pre-existing comorbidities associated with reduced tissue oxygenation (e.g., diabetes). The most common complication was pleural effusion (45%). During the observation period, the mortality rate was 9%. 12 questionnaires could be evaluated. 67% of the participants were affected by dysphagia at the time of the survey. CONCLUSIONS: Descending necrotizing mediastinitis (DNM) is a severe disease requiring an immediate initiation of multimodal treatment. Although quality of life usually isn´t impaired permanently, dysphagia may often persist in patients after DNM.

Malignant head and neck paragangliomas in SDHB mutation carriers.

OBJECTIVE: Three of four paraganglioma syndromes (PGLs) have been characterized on a molecular genetic basis. PGL 1 is associated with mutations of the succinate dehydrogenase subunit D (SDHD) gene, PGL 3 is caused by SDHC gene mutations, and PGL 4 is caused by SDHB gene mutations. The objective of this study was to investigate whether PGLs are associated with malignant head and neck paragangliomas (HNPs). STUDY DESIGN AND SETTING: Through November 2005, we screened 195 HNP patients for mutations of the genes SDHB, SDHC, and SDHD. RESULTS: We detected 5 SDHC, 13 SDHB, and 45 SDHD gene mutations. In seven SDHB mutation carriers, there were distant metastases. No signs of metastases were found in SDHC and SDHD patients. One patient with a sporadic HNP presented with locally metastatic disease. CONCLUSIONS: SDHB mutations are associated with a high rate of malignant HNPs. SIGNIFICANCE: In SDHB patients, a three-body region imaging and scintigraphy or DOPA-PET must be performed to exclude metastases.

Solitary fibrous tumors in the head and neck: new insights and implications for diagnosis and treatment.

OBJECTIVES: The solitary fibrous tumor (SFT) is a potentially malignant spindle cell neoplasm of mesenchymal origin that was originally described as a thoracic lesion originating from pleural tissue. Recently, numerous extrapleural sites of origin have been described, frequently affecting the head and neck region. The purpose of this article is to focus on a formerly underestimated neoplasm and to highlight its appearance in the head and neck region. METHODS: The authors present 2 illustrative cases, including what is probably the first reported case of an SFT arising in the human tonsillar fossa, and give a clinical and pathological literature review. RESULTS: The clinical, histologic, and radiologic features are presented, and the surgical treatment is described. The international medical literature concerning SFTs in general and SFTs of the head and neck in particular is reviewed, and the changing concept of SFTs and hemangiopericytomas is discussed on the basis of the updated World Health Organization classification of soft tissue tumors. CONCLUSIONS: It is nowadays recognized that the large majority of lesions formerly classified as hemangiopericytomas are, in fact, variants of SFTs. Although still a rare occurrence, SFTs have become increasingly recognized, and clinicians should be aware of their presentation.

Laryngotracheal reconstruction using prefabricated and preconditioned composite radial forearm free flaps. A report of two cases.

OBJECTIVE: Total or subtotal laryngectomy performed as surgical treatment of locally advanced laryngeal tumors requires a permanent laryngostomy or tracheostomy, and are both associated with severe and lifelong disability. The hitherto published reconstructive methods for the rehabilitation of patients after subtotal anterior laryngotracheal resection of extended laryngotracheal malignancies have not been thoroughly convincing. METHODS: We present a successful method of extended laryngotracheal reconstruction that combines modern surgical and biomedical techniques. RESULTS: A stable three-dimensional anterior neolarynx was prefabricated, including the use of vacuum-assisted prelamination and preconditioning of a composite radial forearm free flap, which was then transferred to the neck and vascularized by microvascular anastomosis to the cervical vessels. This reconstructive procedure permitted the restoration of a stable laryngotracheal conduit in two cases, with the subsequent successful closure of the tracheo-laryngostomy. CONCLUSION: In the reported cases the pre- or post-operative irradiation did not compromise the success of reconstruction. Not only did our technique facilitate the rehabilitation of patients suffering from drawbacks of the laryngo-tracheofissure, but it also assisted in avoiding total laryngectomy in cases of advanced anterior laryngeal cancer by extending the indications for subtotal anterior laryngeal resections followed by reconstructive surgery.

Spectrum and management of deep neck space infections: an 8-year experience of 234 cases.

OBJECTIVE: To study the clinical course and outcome of deep neck infections (DNI), with special emphasis on microbiology and histopathology. STUDY DESIGN: Two hundred thirty-four patients with DNIs were included in this study. Patients with peritonsillar or dental infections, infections arising from salivary glands, as well as subjects with abscesses caused by neck trauma were excluded. METHODS: Clinical analysis of all patients with DNIs who were treated between January 1, 1997 and May 31, 2005 in a single center. RESULTS: In 13 patients, the DNI was the first manifestation of a malignant tumor. In 17 cases, the DNI was associated with cat-scratch disease (CSD). Six patients suffered from tuberculosis, and in another 7, an infected lateral cleft cyst was found. In 176 patients, the origin of DNI remained unclear. CONCLUSIONS: Our results demonstrate that CSD, tuberculosis, and malignant tumors must be considered as possible causes of DNIs. The current study represents one of the largest series of DNIs in the modern medical literature. EBM RATING: C.

Cat-scratch disease: Otolaryngologic manifestations and management.

OBJECTIVE: The bacteria Bartonella henselae has been known as the principal causative agent of cat-scratch disease (CSD) since 1992. It is an important cause of infectious lymphadenopathies in the head and neck. Nevertheless, CSD often remains unrecognized in cases of cervicofacial lymph node enlargement. STUDY DESIGN: Between January 1997 and May 2003, we conducted a prospective clinical study including 721 patients with primarily unclear masses in the head and neck. RESULTS: CSD was diagnosed by serology and molecular investigations in 99 patients (13.7%; median age 33 years). Cervicofacial lymphadenopathy was the most common manifestation. Atypical manifestation of CSD including Parinaud's oculoglandular syndrome, swelling of the parotid gland and erythema nodosum were diagnosed in 8.1%, 8.1%, and 2.0% of cases, respectively. CONCLUSIONS: Our results demonstrate that CSD is a major cause of enlarged cervicofacial lymph nodes and should therefore be included in the differential diagnosis of lymphadenopathy in the head and neck region.

Paragangliomas in patients with mutations of the SDHD gene.

OBJECTIVE: Paragangliomas represent neoplasms of neural crest origin that arise from paraganglia. Mutations in the gene encoding succinate dehydrogenase subunit D (SDHD) are responsible for a percentage of hereditary paragangliomas. We previously described a group of 271 pheochromocytoma patients, 11 of whom had mutations of the SDHD gene. The objective of this study was to find out whether those 11 patients had additional paragangliomas. STUDY DESIGN: Ten patients participated in our clinical screening program that included MRI of the skull base and neck, thorax, and abdomen, as well as an 18 Fluoro-DOPA positron emission tomography (DOPA-PET). RESULTS: Five patients presented with head and neck paragangliomas, 1 patient with a thoracic paraganglioma, and 2 patients with intraabdominal paragangliomas. CONCLUSIONS: The screening for paragangliomas in patients with mutations of the SDHD gene offers the chance to diagnose those tumors in an early stage. SIGNIFICANCE: Because morbidity after surgical resection increases with tumor size, early surgery will minimize the potential risks.

Role of cat-scratch disease in lymphadenopathy in the head and neck.

Bartonella henselae is the causative agent of cat-scratch disease (CSD), which usually manifests as acute regional lymphadenopathy. The causes of cervical lymphadenopathy, with special regard to CSD, were investigated in a study of 454 patients who presented with unclear masses in the head and neck from January 1997 through January 2001. Sixty-one patients (13.4%) experienced CSD; 54 (11.9%) had primary lymphadenopathy due to other infectious agents, and 41 (9.0%) had lymphadenopathy that occurred in association with primary infections of other organs. For 171 patients (37.7%), the cause of the cervical lymph node enlargement could not be found. B. henselae DNA was detected in extirpated lymph nodes only during the first 6 weeks of lymphadenopathy, which indicates that the results of polymerase chain reaction strongly depend on the duration of illness. CSD should be included in the differential diagnosis of adenopathy in the otorhinolaryngologic patient population, to avoid unnecessary treatment.

Recurrent syncope as presenting symptom of Burkitt's lymphoma at the carotid bifurcation.

Burkitt's lymphoma is rare outside of Africa, and head and neck manifestations of this disease are even more uncommon. We report a case of Burkitt's lymphoma localized at the bifurcation of the right common carotid artery in a 64-year-old man. The presenting symptoms were recurrent syncopes that necessitated a pacemaker, followed by a rapid onset of cranial nerve palsies. The tumor had infiltrated cranial nerves IX, X, XI, and XII and was resected by selective neck dissection. The patient was subsequently treated with a chemotherapeutic regimen consisting of a combination of cytosine arabinoside, vincristine, ifosfamide, prednisone, and an intrathecal application of methotrexate. He also underwent external-beam irradiation of the neck. The patient has been followed up for a period of 42 months and has no evidence of recurrent disease. Repeated diagnostic testing and a coordination of multiple disciplines can speed diagnosis and therapeutic management. The current literature is reviewed and discussed.

Cicatricial pemphigoid in the upper aerodigestive tract: diagnosis and management in severe laryngeal stenosis.

Pemphigoid is a group of rare, acquired, autoimmune subepithelial blistering diseases. The condition has been subclassified into bullous pemphigoid and cicatricial pemphigoid (CP). Diagnosis is based on clinical presentation, evidence of subepithelialvesicles or bullae on routine histologic analysis, and direct and indirect immunofluorescence studies. Cicatricial pemphigoid is characterized by linear deposition of immunoreactants, principally IgG and complement factor 3, along epithelial basement membranes. Cicatricial pemphigoid usually leads to mucosal scarring. We present a case of severe CP that led to laryngeal and subglottic stenosis and involvement of both eyes and the oral, nasal, and nasopharyngeal mucosae. Treatment with dapsone, corticosteroids, azathioprine sodium, cyclosporine A, cyclophosphamide, methotrexate sodium, and mycophenolate mofetil between 1997 and 2001 only resulted in temporary disease control. The patient has been treated with leflunomide for the past 8 months, and there have been no relapses. Treatment of CP with leflunomide has not been described in the literature until now.

Lateral upper arm free flap for primary reconstruction of pharyngeal defects in ablative oncological surgery. Report of six consecutive cases.

Free microvascular flaps are an established method for soft tissue reconstruction following ablative oncological surgery in the head and neck. Functional reconstructions of the hypopharynx and the pharyngoesophageal segment (PES) are of particular relevance, as they are highly demanding surgical procedures. So far, the radial forearm free flap (RFFF) and the free jejunal transfer have been the transplants predominantly used for this purpose. The lateral upper arm free flap (LUFF) presents an alternative method for the fasciocutaneous tissue transfer. We report on our experience with the LUFF in a 56-year-old male patient with a pT3pN0M0 squamous cell carcinoma of the hypopharynx. A pharyngocutaneous fistula developed 5 days after pharyngolaryngectomy with bilateral neck dissection. The fistula was localized between the pharyngeal constrictor muscle and the esophagus and was closed with an LUFF from the left arm. Excellent flap adaptation to the remaining pharyngeal mucosa was observed. Although the length of the vascular pedicle and the diameter of the vessels in the LUFF are smaller than those in the RFFF, neither pedicle length nor vessel diameter proved to be a problem. The LUFF can be recommended as a well-vascularized, relatively safe and reliable flap for reconstruction of tubular structures such as the hypopharynx and the PES after tumor ablation and as an alternative to the RFFF. The flexibility of the LUFF allows surgeons to reconstruct the anatomy of the lost soft tissues as adequately as possible.

Moyamoya disease complicated by life-threatening epistaxis: first report of a case.

OBJECTIVE: Subacute haemorrhage is a common emergency in otorhinolaryngology. Rapid evaluation of the aetiology and localization is a precondition for suitable treatment. We demonstrate a rare case of primarily intractable epistaxis associated with occlusion of the circle of Willis (moyamoya disease). PATIENT: A 38-year-old man presented with a 24-h history of recurrent epistaxis. Anamnesis revealed long-term anticoagulation after heart valve transplantation and arterial hypertension. RESULTS: As a result of several re-bleedings after anterior nasal packing, a re-packing was followed by surgical treatment under general anaesthesia. Four days after discharge the patient presented to the intensive care unit with severe re-bleeding. After removal of a temporary Bellocq packing, interdisciplinary treatment was necessary. Emergency angiography revealed advanced moyamoya disease, with occlusion of both internal carotid arteries. The cerebral blood supply was sustained by an excessive collateral network originating from external carotid anastomoses. This complicated the endovascular treatment, which consisted of embolization of the infraorbital and maxillar arteries with liquid material and coils flanked by Bellocq packing. The patient was doing well at follow-up after 12 months. CONCLUSION: Epistaxis complicating moyamoya disease is rare, and endovascular treatment is difficult due to the high risk of cerebral embolism. Malformations of the cerebral arteries should be considered in the differential diagnosis of intractable epistaxis.

Intracochlear insertion of electrodes using hyaluronic acid in cochlear implant surgery.

Hyaluronic acid has been proposed as a lubricant for improving the outcome of cochlear implant surgery. We describe a method which includes coating the electrodes with a thin layer of hyaluronic acid as well as using a droplet of the lubricant on the cochleostomy to provide additional magnification, like a lens, that facilitates the insertion of the electrode array. The use of hyaluronic acid reduces trauma to the cochlea and avoids contaminating perilymph by blood or by saline used during the operation. It is an efficacious and reliable method for cochlear implant surgery.

Navigation as a quality management tool in cochlear implant surgery.

This cadaver study assessed the value of navigation in cochlear implant surgery. Cochlear implantation was simulated on a cadaver using a Stryker-Leibinger navigation system and a Nucleus 24 Contour implant. A conventional surgical strategy consisting of mastoidectomy, posterior tympanotomy, and cochleostomy was performed. The navigated surgical procedure was evaluated for accuracy, reliability, reproducibility, and practicability. The technology of computer-assisted surgery is applicable in cochlear implantation and beneficial in as much as the navigation-controlled implantation constitutes a non-invasive instrument of quality management. Nevertheless, in order to keep the point accuracy below one millimeter, a referencing method using concealed bordering anatomical structures may be further needed to perform the cochleostomy reliably under the guidance of a navigation system. More reproducible reference systems are needed if navigated lateral skull base surgery is to be fully relied upon.

Technique and timing for closed reduction of isolated nasal fractures: a retrospective study.

Isolated fractures of the nasal pyramid are among the most common facial injuries. Nevertheless, studies of therapeutic results following closed reduction of nasal fractures are rare. We conducted a retrospective clinical review of 187 patients who were evaluated for nasal trauma (including nondislocated fractures, dislocated fractures, and contusions) at our otolaryngology department during 1997 and 1998. Of this group, 96 fractures were treated with closed reduction--either under local anesthesia (n = 68), under general anesthesia (n = 21), or with concomitant septoplasty under general anesthesia (n = 7). At follow-up, which ranged from 1 to 2 years, 91 of the 96 patients (94.8%) expressed satisfaction with their results. Prior to deciding on a course of action, the surgeon must conduct a careful physical examination because the decision as to whether treatment is required, which technique to use (open vs closed reduction), and which type of anesthesia is appropriate (local vs general) all depend on the clinical findings, such as the degree of deviation and airflow obstruction. We also suggest that all patients receive both a Waters' view and a lateral view x-ray. In our opinion, closed reduction is a safe procedure for isolated nasal fractures and can be performed with local anesthesia in most adult patients. Morbidity is minimal in the hands of an experienced ENT surgeon.

Giant-cell reparative granuloma of the temporal bone: a case report and review of the literature.

Giant-cell reparative granuloma (GCRG) is an unusual, non-neoplastic fibrous lesion that most often arises in the mandible and maxilla. GCRG of the temporal bone is exceedingly rare. To the best of our knowledge, only 17 cases have been previously reported in the international medical literature. Although no case of metastasis has been reported, this malignancy can be locally aggressive, and it often recurs following incomplete excision. We report the case of a young woman with a very large GCRG of the right temporal bone. We discuss the clinical picture, differential diagnosis, histologic evaluation, appearance on computed tomography and magnetic resonance imaging, and treatment options. We also review the cases of temporal bone GCRG that have been reported in the literature so far.

Severe deep neck space infections and mediastinitis of odontogenic origin: clinical relevance and implications for diagnosis and treatment.

CONCLUSIONS: Early diagnosis and aggressive antimicrobial and surgical treatment are essential to successfully treat extensive cervico-mediastinal abscesses of odontogenic origin. Patient management should be performed by experienced clinicians well trained in managing possible complications. We recommend close clinical and radiological postoperative follow-up investigations with early surgical re-intervention if necessary. OBJECTIVES: While neck infections affecting the perimandibular space have a high prevalence and their clinical aspects have repeatedly been discussed, further spread of the inflammation and life-threatening situations have rarely been described. The objective was to determine clinical, diagnostic, and therapeutic aspects of severe neck infections of odontogenic origin. Emphasis was placed on grave descending deep neck space infections, sometimes resulting in mediastinitis as a life-threatening complication. PATIENTS AND METHODS: We reviewed 10 patients with severe odontogenic abscesses treated during an 8-year interval in a single center. RESULTS: The submandibular space was the most frequently encountered location of deep neck space infections. Mediastinitis was found in five patients. The most frequent causative bacteria were Streptococcus and Bacteroides species. All patients underwent intravenous antibiotic treatment and surgical therapy. Mediastinotomy was inevitable in five cases and thoracotomy in one case. All patients survived.