[Synchronous schwannoma of the vagus nerve and the cervical sympathetic chain]. / Synchrones Schwannom des N. vagus und des zervikalen sympathischen Grenzstrangs.

Schwannomas are rare neural sheath tumors which are generally benign. Up to 45% of all schwannomas originate in the head and neck region. In the parapharyngeal space (PPS) they may arise from any of the lower cranial nerves IX, X, XI and XII or from the cervical sympathetic chain. We report a unique case of a synchronous schwannoma of the vagal nerve and the cervical sympathetic chain in a patient without neurofibromatosis.

[Genetics of pheochromocytoma]. / Genetik der Phäochromozytome.

About one third of all patients with a pheochromocytoma are carriers of germ line mutations of 1 of the 10 susceptibility genes. Thus, these patients can be diagnosed and classified with specific tumor syndromes. This group is composed of the entities of multiple endocrine neoplasia type 2 (MEN2) due to mutations in the RET gene, von Hippel-Lindau disease (VHL, VHL gene), the paraganglioma syndromes types 1-4 (PGL1-4) due to mutations of the genes SDHD, SDHAF2, SDHC, SDHB, neurofibromatosis type 1 (NF1) due to mutations of the NF1 gene and familial pheochromocytoma syndromes due to mutations of the SDHA, TMEM127 and MAX genes. Patients with hereditary pheochromocytomas run a lifelong risk of relapse of pheochromocytoma. In addition extraparaganglial tumors are frequent and include medullary thyroid carcinoma in MEN2 or renal cancer or neuroendocrine pancreatic cancer as well as hemangioblastomas of the retina and the central nervous system in VHL. Furthermore, renal cancer may be associated with PGL4 and PGL3. In conclusion, molecular genetic screening is essential for the diagnosis of pheochromocytoma-associated cancer syndromes and is thus the cornerstone for successful lifelong preventive medicine of such patients and their relatives.

[Large cystic tumor of the right neck]. / Große zystische Raumforderung zervikal rechts.

A 32-year-old patient presented to our department with a large cystic lesion in the right lateral neck (diameter 6 cm). After complete resection, the histopathological examination revealed a lymph node metastasis of a papillary thyroid carcinoma. We performed total thyroidectomy and bilateral selective neck dissection. There was a papillary microcarcinoma in the right lobe of the thyroid. The postoperative course was uneventful. Therapy with radioiodine was conducted. At 30 months after surgery the patient is free of disease.

Flat panel CT following stapes prosthesis insertion: an experimental and clinical study.

OBJECTIVES: Anatomical information of the middle and inner ear is becoming increasingly important in post-operative evaluation especially after stapesplasty with unsuccessful improvement of the air-bone gap (ABG). So far computed tomography (CT) has been the first choice for detection of reasons for recurrent hearing loss. CT has the disadvantage of metal-induced artefacts after insertion of middle ear implants and of a relatively high irradiation dose. METHODS: Flat panel CT (fpCT) was performed in three temporal bone specimen after experimental insertion of different stapes prostheses, aiming to validate the accuracy of fpCT of the middle and inner ear. Additionally, 28 consecutive patients, supplied with different stapes prostheses underwent post-operative fpCT to compare the pre- and post-operative hearing results with the determined prosthesis position in the middle and inner ear. RESULTS: In all cases, fpCT showed a statistically significant correlation between hearing improvement and prosthesis position. This technique provided detailed post-operative information of the implant position in patients and temporal bone specimen. CONCLUSIONS: The new imaging technique of fpCT allows the immediate and almost artefact-free evaluation of surgical results following stapesplasty. Further benefits are a lower irradiation dose and higher isovolumetric resolution compared with standard CT. KEY POINTS: Flat panel computed tomography (fpCT) helps otosurgeons design precise stapes protheses. fpCT allows a prediction of the postoperative hearing outcome in patients. fpCT is an adequate imaging technique for immediate postoperative quality control. Postoperative management of patients with prosthesis-related complications is more appropriate.

[Chondrosarcoma of the larynx. Experience gained in Freiburg since 1999]. / Chondrosarkome des Larynx. Freiburger Erfahrungen seit 1999.

BACKGROUND: Chondrosarcomas are rare tumors of the head and neck. Nevertheless, they display the most common non-epithelial malignancy of the larynx. MATERIALS AND METHODS: Between 1999 and February 2010 we treated six patients with laryngeal chondrosarcoma. The group included two female and four male patients ranging in age from 54 to 82 years. RESULTS: An 82-year-old female patient died 3 months after diagnosis and tracheostomy due to other underlying diseases. An 82-year-old male patient underwent primary radiation therapy. In the other patients, we performed a modified hemilaryngektomy in three cases and a laryngectomy in one. In those four cases, there were no signs of recurrent disease 50, 85, 87 and 95 months after surgery, respectively. There were no local or distant metastases. CONCLUSIONS: Chondrosarcomas of the larynx are slow growing neoplasms. Metastases occur in less than 3% of cases. Complete resection is the therapy of choice. Function-preserving surgical approaches should be favoured.

[Paragangliomas and paraganglioma syndromes]. / Paragangliome und Paragangliomsyndrome.

Paragangliomas are rare tumors of neural crest origin. They are benign in the majority of cases and are characterized by a strong vascularisation. In the head and neck region they most commonly occur as carotid body tumors. Jugulotympanic and especially vagal paragangliomas are seen less frequently. Complete surgical resection represents the only curative treatment option even though resection of locally advanced tumors regularly results in lesions of the lower cranial nerves and major vessels. Approximately 30% of all head and neck paragangliomas (HNPs) are hereditary and associated with different tumor syndromes. The paraganglioma syndromes 1, 3 and 4 (PGL 1, 3 and 4) make up the majority of those familial cases. PGL 1 is associated with mutations of the succinate dehydrogenase subunit D (SDHD) gene, PGL 3 is caused by SDHC and PGL 4 by SDHB gene mutations. Multiple HNPs and the occurrence of HNPs together with pheochromocytomas are seen in SDHD as well as SDHB mutation carriers. In patients with SDHB mutations the risk for the development of malignant paraganglial tumors is significantly higher compared to SDHC and SDHD patients as well as patients with sporadic tumors. SDHC mutation carriers almost exclusively present with benign HNPs that are unifocal in the majority of cases. The role of transmission is autosomal dominant for all 3 symptoms. Interestingly, there is a "parent-of-origin-dependent-inheritance" in subjects with SDHD gene mutations. This means that the disease phenotype may only become present if the mutation is inherited through the paternal line. We recommend screening for mutations of the genes SDHB, SDHC and SDHD in all patients with HNPs. Certain clinical parameters can help to set up the order in which the 3 genes should be tested.

[Cochlear implant device failure after cardioversion]. / Cochleaimplantatdysfunktion nach Kardioversion.

Cochlear implantation has become the standard procedure for the treatment of severe to profound hearing loss, even in patients with underlying diseases. We report the case of a CI patient who underwent cardiac defibrillation, following which he reported a reduction in sound quality, a worsening of tinnitus, as well as headaches. An integrity test showed multiple electrode anomalies which could not be directly attributed to the cardioversion. We performed explantation and reimplantation of the CI. During the course of rehabilitation, the patient showed good results in speech comprehension. Since cardioversion, like any electrical monopolar treatment, may damage CI, it should be avoided in CI patients wherever possible. If it cannot be avoided, we strongly recommend removing the sound processor during treatment sessions. When device-related problems occur, the treatment of choice is reimplantation.

[Exophytic tumor of the parotid gland]. / Exophytischer Parotistumor.

An 81-year-old male patient presented at our department with a large (diameter 10 cm), exophytic lesion in the right parotid region. Magnetic resonance imaging showed the tumor to derive from the lateral part of the parotid gland. We performed complete tumor resection with selective ipsilateral neck dissection. The facial nerve could be preserved. The postoperative course was uneventful. Histopathologic and immunohistochemical examinations revealed a malignant fibrous histiocytoma. We present the diagnostic and therapeutic options of this unusual malignancy of the parotid gland.

[Von Hippel-Lindau disease. Interdisciplinary patient care]. / Von-Hippel-Lindau-Erkrankung. Interdisziplinäre Patientenversorgung.

Von Hippel-Lindau disease is an important hereditary tumor syndrome with a clear option for effective treatment if diagnosed in time. Interdisciplinary cooperation is the key to successful management. Major components of the disease are retinal capillary hemangioblastomas, hemangioblastomas of cerebellum, brain stem and spine, renal clear cell carcinomas, pheochromocytomas, multiple pancreatic cysts and islet cell carcinomas, tumors of the endolymphatic sac of the inner ear, and cystadenomas of the epididymis and broad ligament. A well structured screening program should be performed at yearly intervals.

[Craniofacial fibrous dysplasia: scan policy or surgery?]. / Kraniofaziale Fibröse Dysplasie: Observieren oder Operieren?

BACKGROUND: The aetiologic correlations of fibrous dysplasia (FD) are more and more decoded by molecular biology, improved imaging procedures, and the use of computer assisted surgery--thus a review of present diagnostics and therapy methods is evaluated. METHOD: The valid methods of diagnostic and therapy procedures of craniofacial FD were retrospectively analysed in a collective of 9 patients in consideration of literature. The criteria of the decision for diagnosis and surgical procedures were evaluated. RESULTS: According to the literature, diagnosis was ascertained with modern CT and MRI scans. Bone scintigraphy was only used additionally in particular questions. In case of unclear radiological findings histomorphological procedures were used complementarily to distinguish FD from other bone tumors. The aim of surgical intervention was to reduce pain, to restore the function in compression symptoms, to recover original ostia, or to restore the natural geometry of the face. CONCLUSIONS: Current imaging procedures allow differential diagnosis from other benign bone tumors but also from malignancies. The therapy of FD is conservative (wait and scan) or operative in dependence on the localisation, the extension and the clinical manifestations of the disease. In the future molecular biological methods could function as supporting instrument for diagnosis if histomorphological results are not meaningful.

The free vastus lateralis flap for reconstruction in ablative oncologic head and neck surgery.

AIMS: The vastus lateralis muscle is an accessory extensor for the knee suitable as a free myocutaneous flap in reconstructive head and neck surgery. We report the use of this muscle as a flap. METHODS: We have used the free myocutaneous vastus lateralis flap for reconstruction following ablative head and neck tumour surgery in six patients. The clinical outcome, time of surgery for flap preparation and anatomosis, follow-up and functional outcome were analysed. RESULTS: Five of our patients showed a very satisfactory functional and cosmetic outcome. Post-operatively, there was no prolonged immobilisation and no limitation of movement to the hip and knee. No unfavourable side-effects at the donor side were noted. CONCLUSIONS: We find this flap a very useful addition to our free myocutaneous flap armamentarium. It has a specific suitability for replacing large defects.

Paragangliomas of the head and neck: diagnosis and treatment.

Paragangliomas of the head and neck (HNP) represent rare tumors of neural crest origin. They are highly vascular neoplasms that are benign in the majority of cases. The site of origin defines the name given those tumors. In the head and neck, they most commonly occur at the carotid bifurcation, where they are referred to as carotid body tumors (CBT). Other common sites of origin are the jugular bulb (jugular paraganglioma; JP), the tympanic plexus on the promontory (tympanic paraganglioma; TP) and the vagal nerve (vagal paraganglioma; VP). Patients with cervical paragangliomas frequently present with a painless, slowly enlarging mass in the lateral neck. In many patients with TP and JP, tinnitus and hearing loss are early symptoms. JP patients often suffer from lower cranial nerve deficits. Evaluation by an imaging modality is necessary to establish the diagnosis. Imaging procedures frequently used include B-mode sonography with color-coded Doppler sonography, computed tomography (CT), magnetic resonance imaging (MRI) and digital substraction angiography (DSA). Debate exists in the literature regarding the different treatment modalities for paragangliomas which include surgery, radiotherapy and stereotactic radiosurgery. The role of preoperative angiography and embolization has also been a matter of discussion. The diagnostic work up and the different treatment options for patients with head and neck paragangliomas will be presented and discussed.

[Etiology and therapy of the internal jugular vein thrombosis]. / Atiologie und Management von Thrombosen der Vena jugularis interna.

BACKGROUND: Thromboses of the upper limp and neck are rare in comparison with those of the lower extremities. Internal jugular vein thrombosis (IJVT) is a serious event with a potentially fatal outcome. Complications include pulmonary embolism, sepsis with septic emboli to different organs and tissues as well as intracranial propagation of the thrombus with cerebral edema. As any thrombosis, IJVT is precipitated by Virchow's triad: endothelial damage, alteration of blood flow and hypercoagulability. The history and examination in patients with an IJVT may be vague and misleading. Patients may present with a painful swelling of the neck but they may also be absolutely asymptomatic. Imaging procedures frequently used to diagnose an IJVT include sonography with color-coded duplex sonography, computed tomography, magnetic resonance imaging as well as magnetic resonance venography. Up to date, there is no standardized treatment regimen for patients with an IJVT. PATIENTS AND METHODS: This retrospective study includes all ten patients with an IJVT who were seen at our department between January 2000 and January 2004. There were six female and four male patients. The average age was 49.7 years, ranging from 28 to 79 years. RESULTS: In five cases, the thrombosis was associated with a malignant tumor. In four patients, it was caused by a deep neck space infection and in one case the IJVT was due to cervical, intravenous drug abuse. Two patients were found to be pregnant (one tumor patient and one patient with a deep neck space infection). In all cases, a ten day treatment regimen with intravenous antibiotics and anticoagulant therapy was initiated. Oral or subcutaneous anticoagulation was continued for six weeks to six months. No complications were seen in any patient. In three patients a revascularization of the affected vessel could be demonstrated with color-coded duplex sonography six months after the initial presentation. CONCLUSIONS: Thrombosis of the IJV is probably underdiagnosed. Since the clinical presentation may be vague or misleading, a high degree of suspicion is required to make the diagnosis. The potential complications such as pulmonary embolism or intracranial propagation of the thrombus may be fatal. Whenever the thrombosis is not caused by an inflammatory process, a malignant tumor should be excluded. We recommend a therapy with intravenous antibiotics as well as a systemic anticoagulation. Ligation or resection of the internal jugular vein is reserved for patients who develop complications despite adequate medical therapy.

[Diagnosis and management of cervical paragangliomas: the Freiburg experience]. / Diagnostik, Therapie und Behandlungsergebnisse zervikaler Paragangliome.

BACKGROUND: Paragangliomas of the head and neck represent rare tumors of neural crest origin that arise from chemoreceptors. They are highly vascular neoplasms that are benign in the majority of cases. There are controversial discussions concerning the different treatment options for cervical paragangliomas. PATIENTS: Between January 1992 and November 2003 a total of 13 patients with 15 cervical paragangliomas were treated at the Department of Otorhinolaryngology - Head and Neck Surgery, University of Freiburg. Our study includes eight female and five male patients with a mean age of 39.9 years (range: 20 - 79 years). There were 14 carotid body tumors and one vagal paraganglioma. RESULTS: 14 tumors were resected and one underwent primary radiation. In four patients we noted a total of 8 permanent, postoperative nerve deficits. During follow-up no recurrent tumors were seen. CONCLUSION: Complete surgical removal represents the therapy of choice for the majority of cervical paragangliomas. In cases of multiple head and neck paragangliomas or in patients with underlying diseases, primary radiation should be discussed as a treatment option.

[Paragangliomas of the head and neck. Part 2: Therapy and follow-up]. / Paragangliome im Kopf-/Halsbereich. Teil 2: Therapie und Nachsorge.

Paragangliomas of the head and neck are preferably treated surgically. Planning the surgical approach for temporal bone paragangliomas is performed according to the Fisch classification. Small temporal paragangliomas can be removed in a transtympanic or transmastoidal procedure. Locally advanced paragangliomas of the head and neck have to be embolized presurgically. An occlusion test is also recommended to check the possibility of a resection of the internal carotid artery. Type C and D temporal bone paragangliomas can be removed by different infratemporal approaches. Alternatively, some type C(1,2 )and De,i(1,2) temporal bone paragangliomas can be removed via variations of the juxtacondylar approach. Glomus caroticum tumors are resected transcervically. In cases of contraindications for surgery or in palliative situations radiotherapy is recommended.

[Paragangliomas in the head-/neck region. I: Classification and diagnosis]. / Paragangliome im Kopf-/HalsbereichTeil 1: Systematik und Diagnostik.

Paragangliomas of the head and neck occur sporadically or are hereditary. The hereditary phenotype characteristically occurs between the 2nd and 3rd decade of life; the sporadic phenotype beyond the 4th decade. The hereditary phenotype "paraganglioma syndrome type 1" frequently shows multilocular tumor manifestations and rarely cases of maltransformation. Therefore, paragangliomas should be considered a systemic disease. For diagnosis, an interdisciplinary, step by step procedure is mandatory comprising genetic screening, whole body medical check-up including catecholamine metabolism, and optionally an 18F-Dopa-PET and localised imaging procedures.

[Transvestibular closure with an autologous bone graft as surgical repair of a nasoseptal defect due to Le Fort-I osteotomy]. / Transvestibulärer Verschluss eines Nasenseptumdefekts durch freies Knochentransplantat nach Le-Fort-I-Osteotomie.

Defects of the nasal septum are a common complication after nasal surgery. Affected patients frequently suffer from bleeding, crusting and impaired nasal air flow. The surgical closure of septal defects remains a distinctive challenge. Though many different techniques have been described, the failure rate of this procedure remains high. In the case presented here, a large basal septum defect occurred after a prosthetic Le Fort-I osteotomy. The attempt to cover the distance between the bony nasal floor and the nasal septum with pedicled mucosal flaps failed due to extensive scar formation of the nasal mucosa. Therefore a nasal floor elevation by insertion of an autologous bone graft from the iliac crest was conducted. The bone graft was connected with the hard palate via two titanium screws. Other than with an autologous cartilage graft, no major resorption of the bone graft is to be expected. This indirect method for the closure of a basal nasal septum defect is new.