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Nasal surgery is frequently performed. Predictors of surgical outcome in terms of chemosensory function are not clear. A total of 64 patients were included in this prospective study (41 men, 23 women; age range 22–67 years). Prior to surgery, patients received a detailed otorhinolaryngological examination including nasal endoscopy, and CT scans used to establish the Lund–Mackay score. Olfactory function was analyzed using a custom-built odor identification test which allowed separation of chemosensory function into “olfactory” and “trigeminal” portions. Retest was performed 6 months after surgery. In terms of the sense of smell, nasal surgery produced the highest success rates in women, high degree of polyposis, and aspirin-intolerance. Neither age, presence of asthma, nor the number of preoperative surgical interventions had a significant impact on the outcome of surgery in terms of chemosensory function. Although “trigeminal scores” changed to a lesser degree than “olfactory scores”, the present results indicated that nasal surgery may also improve trigeminal function, although this needs further corroboration. Improvement of olfactory function following nasal surgery appears to last, on average, for at least 6 months. While the present results may be seen as an encouraging step towards the description of more detailed prognostic factors related to nasal surgery, results from the present investigation also point towards the idea that nasal polyposis is due to a multifactorial process that, so far, is not adequately addressed by current research. Future work is needed to identify further predictors of postoperative outcome in terms of olfactory function. [ABSTRACT FROM AUTHOR]

Transsphenoidal pituitary adenoma surgery is related to a low morbidity rate. The complications that can occur are classified as intra- and extracranial. The aim of the study is to discuss one group of these complications involving the sphenoid sinus: mucocele and its possible transformation into pyocele. We evaluate clinical presentation, management strategy and the outcome after long-term follow-up presenting an explicative case and a review of the literature. A patient presented to our outpatient clinic 8 months after transsphenoidal surgery for selective removal of a pituitary adenoma because of an acute onset of frontal headache during an airplane travel, fever and pulsating sensation in left eye and ear. MRI revealed a contrast-enhancing lesion in the left inferior portion of the sphenoid sinus. An endonasal endoscopic revision of the sphenoid sinus was performed. After opening of the scar to enter in the left sinus a pyocele was found and treated with drainage and marsupialisation. Development of sphenoid sinus pyocele is an extremely rare postoperative complication of transsphenoidal surgery. This lesion should be taken in consideration in patients presenting with retroorbital headache of acute onset and fever after pituitary surgery. Diagnosis can be suspected on the MRI studies and confirmed by a targeted flexible endoscope examination. Endoscopic drainage with wide opening of the sphenoid sinus and marsupialisation is the treatment of choice to avoid recurrences.

Tumours affecting the nose, paranasal sinuses and adjacent skull base are fortunately rare. However, they pose significant problems of management due their late presentation and juxtaposition to important anatomical structures such eye and brain. The increasing application of endonasal endoscopic techniques to their excision offers potentially similar scales of resection but with reduced morbidity. The present document is intended to be a state-of-the art review for any specialist with an interest in this area 1. to update their knowledge of neoplasia affecting the nose, paranasal sinuses and adjacent skull base; 2. to provide an evidence-based review of the diagnostic methods; 3. to provide an evidence-based review of endoscopic techniques in the context of other available treatments; 4. to propose algorithms for the management of the disease; 5. to propose guidance for outcome measurements for research and encourage prospective collection of data. The importance of a multidisciplinary approach, adherence to oncologic principles with intent to cure and need for long-term follow-up is emphasised.

Chordomas are rare malignant tumors arising from embryonic remnants of the primitive notochord, around which the skull base and vertebral column develop. They are locally aggressive but metastasize rarely. To our knowledge, this is the first reported case of synchronous intraosseous chordomas. A 32-year-old man presented with intermittent double vision secondary to a right-side abducent nerve palsy. Imaging revealed a clivus chordoma and an asymptomatic synchronous second primary chordoma in the fifth lumbar vertebra. Both chordomas were surgically excised: the clivus using the endonasal, endoscopic route and the L5 vertebra by total vertebral excision and replacement with a titanium prosthesis. The patient made an uneventful and complete recovery. We have modified our departmental practice as we believe that all patients diagnosed with chordoma should have magnetic resonance imaging of their entire spinal tract to exclude a second primary chordoma.

Multiple genes and their variants that lend susceptibility to many diseases will play a major role in clinical routine. Genetics-based cost reduction strategies in diagnostic processes are important in the setting of multiple susceptibility genes for a single disease. Head and neck paraganglioma (HNP) is caused by germline mutations of at least three succinate dehydrogenase subunit genes (SDHx). Mutation analysis for all 3 costs approximately US$2,700 per patient. Genetic classification is essential for downstream management of the patient and preemptive management of family members. Utilizing HNP as a model, we wanted to determine predictors to prioritize the most heritable clinical presentations and which gene to begin testing in HNP presentations, to reduce costs of genetic screening. Patients were tested for SDHB, SDHC, and SDHD intragenic mutations and large deletions. Clinical parameters were analyzed as potential predictors for finding germline mutations. Cost reduction was calculated between prioritized gene testing compared with that for all genes. Of 598 patients, 30.6% had SDHx germline mutations: 34.4% in SDHB, 14.2% SDHC, and 51.4% SDHD. Predictors for an SDHx mutation are family history [odds ratio (OR), 37.9], previous pheochromocytoma (OR, 10.9), multiple HNP (OR, 10.6), age

Objective: To perform the anterior-to-posterior-to-anterior technique in revision endoscopic sinus surgery, and to assess the effects and safety of this technique in treating recurrent chronic sinusitis and nasal polyps., Methods: One hundred and thirteen patients with recurrent chronic sinusitis and nasal polyps received anterior-to-posterior-to-anterior revision endoscopic sinus surgery. This surgical technique has 5 important steps, including exposing the choana, enlarging the maxillary sinus ostium, opening the sphenoid sinus and posterior ethmoid sinus anteriorly to posteriorly, resecting the ethmoid sinus posteriorly to anteriorly, and opening the frontal sinus posteriorly to anteriorly. The choana, maxillary sinus ostium, middle turbinate remnant, and the roof and lateral wall of the sphenoid sinus are 4 stable anatomic reference points., Results: Twelve months postoperatively, the mean SNOT-22 score had improved by 18.48 and 100 (88.50%) patients had an improvement in nasal obstruction; thick nasal discharge, loss of smell or taste, and facial pain/pressure improvements were noted in 102 (90.27%), 64 (56.64%) and 76 (67.26%) patients, respectively. Postoperative endoscopic evaluation after 12 months indicated that 29 (25.66%), 12 (10.62%), 5 (4.42%), 6 (5.31%), and 2 (1.77%) patients had mucosal edema, nasal discharge, synechiae, polyps, and crusting, respectively. No severe complications were observed., Conclusion: The anterior-to-posterior-to-anterior technique is an effective and safe approach to revision endoscopic sinus surgery. The 4 stable anatomic reference points are helpful for preventing severe complications in total ethmoidectomy., (Copyright 2009 S. Karger AG, Basel.)

Background: Standardized surgical technique, the use of ultrasonic dissection and neuro- monitoring, have reduced morbidity in thyroid surgery, so that aesthetic aspects and endoscopic or minimally-invasive thyroid surgery have become more important for the patients., Methods: We report on minimally-invasive and endoscopic procedures with either a cervical or extracervical access. Besides current literature we discuss our own results., Results: The critical evaluation of these procedures shows that the standards of endocrine surgery are not at all times entirely observed., Conclusions: We therefore suggest requirements for endoscopic thyroid resection: The access trauma must be minimal, the aesthetic result must be optimal--without visible scars--and the size of the resected thyroid tissue should be the same as in open surgery. It has to be possible to resect the tissue en bloc. Only if the quality of the endoscopic thyroid resection is ensured aesthetic aspects may begin to play a role. If all these demands are fulfilled, the advantages of endoscopic resections do not only cover aesthetic aspects. The first results show less perioperative pain and a shorter stay in hospital. However, to prove this, further studies are necessary.

Objective: To introduce endonasal drainage procedures to frontal sinus in inflammatory sinus disease and its indications, methods and efficacy., Method: One hundred thirty two patients undergoing Draf I-III frontal sinus drainage procedures with 1-12 years follow-up were reviewed retrospectively., Result: Forty two patients underwent type I frontal sinusotomy, 43 type II sinusotomy and 47 type III sinusotomy. A successful result was seen in these groups, 83.4%, 83.7%, and 89.4% respectively. Best effect was gained by type III sinusotomy. There was no significant difference in efficacy between the different Draf frontal sinus drainage procedures (P > 0.05)., Conclusion: Endonasal microscopic-endoscopic frontal drainage treatment of refractory, polypoid and recurrent frontal sinusitis can yield successful results.

Objectives: The aim of the study was to evaluate the frequency and the causes of the intra- and postoperative cerebrospinal fluid (CSF) leaks and to discuss the sella closure methods., Methods: During the period from 1995 to 2005, 313 patients underwent 356 transsphenoidal operations for pituitary adenoma. Microadenoma was found in 80 (22.5%) cases, and in 276 (77.5%) cases, macroadenoma was removed. Two different methods to close the sella were used. The first one consisted packing the sella turcica and sphenoidal sinus with autologous fat and restoring the defect of sella turcica with autologous bone. In more resent practice, the regenerated oxidized cellulose (Surgicel) and collagen sponge with human fibrin (TachoSil) were used to cover the sella membrane defect, followed by packing the sella with autologous fat and covering the dural defect with Surgicel and TachoSil., Results: Adenoma was totally removed in 198 (55.6%) cases out of 356. Microadenoma was totally removed in 91.3% and macroadenoma in 45.3% of cases, respectively. Postoperative complications were noted in 40 (11.2%) patients. Two (0.6%) patients died after surgery. Intraoperative CSF leakage was observed in 58 (16.3%) cases. Postoperative CSF leakages were observed in 3 cases, when the method of packing the sella with just autologous fat was used, whereas in 29 cases when the sella fat packing was used together with Surgicel and TachoSil to cover the sella membrane and dural defects, no postoperative CSF leakages were observed., Conclusions: The technique of covering the sella membrane and dural defects with Surgicel and TachoSil in the presence of intraoperative CSF leakage appeared to be the most reliable one, as no postoperative CSF leakage applying this technique has been observed.

Background: This study analyzes the management and outcomes of a series of 10 malignant peripheral nerve sheath tumors (MPNST) of the head and neck., Methods: From 1984 to 2004, 10 patients underwent surgical treatment of a MPNST. We retrospectively reviewed presenting symptoms, radiological findings, surgical management, and follow-up status and performed a literature review., Results: Eight tumors were located at the lateral skull base; 2 involved the vagus nerve in isolation. Two lesions were growing within the sinonasal tract. The most common presenting symptom was a rapidly enlarging cervical mass. Seventy percent of the tumors could be resected completely. Long-term follow-up showed a 2-year disease-specific survival rate of 50% and 5-year survival rate of 20%. Negative prognostic indicators were advanced tumor stage, early recurrence, and presumably also the presence of von Recklinghausen's disease. Postoperative adjuvant radiotherapy was found to make no difference in outcome., Conclusions: Although rare, MPNST is one of the most aggressive tumors in the head and neck area. Complete tumor removal is the mainstay of treatment and most important prognostic factor of MPNST. Adjuvant radiotherapy should be used to assist surgical excision in local control. The role of adjuvant chemotherapy remains controversial., ((c) 2006 Wiley Periodicals, Inc.)

Reconstruction of the anterior skull base must be secure and watertight. Failure to achieve this places the patient at risk of the development of cerebral sepsis. We have developed the technique of endonasal duraplasty and have achieved a 90% long-term success rate. In this article we described the key elements of our technique starting with radiographic and fluorescein localization of a skull base defect. The main steps in reconstruction and materials used are detailed, together with modifications of our technique for certain difficult situations and tips for success. Attention is drawn to potential pitfalls that have been identified over 25 years of clinical practice.

Objectives: This study was designed to compare the effectiveness of positron emission tomography (PET) and magnetic resonance imaging (MRI) in the pretherapeutic staging of squamous cell carcinoma (SCC) of the head and neck., Patients and Methods: The study included 34 consecutive patients (27 males, 7 females; mean age 61 years; range 42 to 82 years) with SCC of the head and neck. All the patients underwent whole body [18F]fluorodeoxyglucose (FDG)-PET and MRI scans for pretherapeutic evaluation. Diagnoses were confirmed by histopathologic examination of endoscopic biopsy specimens., Results: The sites of the primary tumors were the oropharynx (n=15, 44%), larynx (n=10, 29%), hypopharynx (n=8, 24%), and nasopharynx (n=1, 3%). Surgery was the treatment of choice in 20 patients (59%), including 23 neck dissections. Fourteen patients (41%) were treated with radiochemotherapy. Both PET and MRI were able to detect the primary tumor in 33 cases (97%). In two patients (6%), PET was able to detect distant metastases in the lung and iliac bone, all of which were confirmed by biopsies. Seven neck specimens (30%) showed lymph node metastasis. Sensitivity and specificity rates for detection of lymph node metastasis were 100% and 87.5% for PET, and 85.7% and 87.5% for MRI, respectively., Conclusion: Although PET seems to be superior to MRI in detecting nodal disease and distant metastases, it is still early to recommend it as a primary tool for pretherapeutic evaluation of head and neck cancers due to its limited availability and higher cost.

Objective: To demonstrate the functional long-term results after reconstruction of the lower lip with the tongue flap., Methods: We describe the surgical technique and long-term results of lower lip reconstruction with the tongue flap and review five cases in which this technique was used to reconstruct defects of the lower lip, particularly the lip vermilion., Results: Between 1993 and 2003 we performed reconstruction of the lower lip using the tongue flap in five patients. All patients were followed for 2 to 10 years (mean 3.4 years). The procedure achieved good functional and aesthetic results, with no major complications, in particular no flap necrosis. One patient complained of paresthesias of the tongue which resolved within 24 months. Speech was unaffected by use of the tongue flap, although eating and drinking were temporarily impaired prior to the flap separation at the second and final stage of surgery., Conclusion: The tongue flap is a simple and reliable technique for reconstruction of part or all of the lip vermilion. The technique is easy to perform and provides good aesthetic and functional results.

Objective: To report long-term functional results of the surgical treatment of cervical paragangliomas., Patients and Methods: A retrospective review of 22 patients with 34 head and neck paragangliomas of which 27 were resected between 1981 and 2004. Of these, 16 were carotid body tumors and 11 were vagal paragangliomas. There were 13 women and 9 men with an average age of 48.6 years (range, 26 to 75 years; median, 49 years) and the mean follow-up period was 82 months (range, 3 to 184 months; median, 61 months)., Results: There were 13 solitary tumors of which 5 were carotid body tumors and 8 vagal paragangliomas. Multiple head and neck paragangliomas were seen in 9 patients (41%). The incidence of associated multiple tumors was 64.3% for carotid body tumors and 38.5% for vagal paragangliomas. Complete tumor resection was achieved in all but 1 patient in whom a small intradural residual vagal paraganglioma had to be left. The internal carotid artery was preserved in all carotid body tumor resections. Lower cranial nerve deficits were sustained in 1 carotid body tumor resection only, but in all cases with multiple tumors. All patients with vagal paragangliomas had or developed a vagal nerve paralysis. In 4 cases minor complications developed postoperatively. No recurrent tumors were seen during the follow-up period., Conclusions: Even in large head and neck paragangliomas surgical treatment provides excellent tumor control with low postoperative morbidity. A wait-and-scan policy may be more appropriate for those patients with multiple tumors, advanced age, or high operative risk and for those whose tumors have recurred following radiotherapy.

Background: Surgical therapy for paragangliomas (PG) of the head and neck is, due to the alternatives of radiation therapy and wait-and-scan strategy and because of postoperative morbidity, under ongoing discussion., Material and Methods: Between 1981 and 2004, 79 patients with 94 PG of the head and neck were treated at our department. These patients had follow-up examination within a clinical trial considering tumor control, functional results and for the first time neuropsychologically evaluated postoperative quality of life. Mean follow-up time was 65 months (1 to 228 months)., Results: Among the 94 PG there were 19 carotid body (GCP), 12 vagal nerve (GVP) and 63 jugular-tympanal paragangliomas (JTP). Of these, 87 tumors underwent surgery. In 68 patients (78.1 %), the tumor could be removed completely. In particular, complete resection of GCP was achieved in 100 %, of GVP in 90.9 %, of JTP type A in 100 %, of JTP type B in 83.3 %, of JTP type C in 66.6 % and of JTP type D in 61.5 %. During the follow-up period, residual or recurrent tumors were diagnosed in 17 patients (19.5 %). Six of the seven residual PG were observed by magnetic resonance tomography and did not show growth. One residual PG and 6 recurrencies were resected completely. One recurrent tumor was radiated and 3 others are under observation without showing growth tendencies. Two patients died postoperatively due to borderline operations of extended tumors. The quality of life after PG surgery showed a SIP of 4.8, which is comparably much better than after acoustic neuroma surgery (SIP 10.3)., Conclusion: Whereas complete tumor resection of GCP and JTP types A and B is almost ever possible without cranial nerve palsies, surgery of GVP and advanced JTP causes often severe functional deficits. However, postoperative quality of life is mostly good. Nevertheless, advanced PG require an individualized therapeutic regime also including radiation and observation of tumor growth.

Background: The aim of this retrospective study was to assess the potentials and limitations of endonasal micro-endoscopic sinus surgery in the management of sinonasal inverted papilloma (IP) and to demonstrate long-term results., Methods: Eighty-seven patients underwent resection of an IP either via an endonasal, an osteoplastic maxillary or frontal sinus or a combined approach. Charts were reviewed for presenting symptoms, tumour stage according to the Krouse classification, surgical management and follow-up status., Results: Most tumours were staged as T2 or T3 (42.5% each). Sixty-eight (78.2%) patients were referred for primary surgery. Nineteen (21.8%) patients presented with recurrent disease. The majority of IP (70%) were removed via an endonasal micro-endoscopic procedure. In 20 (23%) patients a combined approach was performed. The overall recurrence rate was 10.3%. Referring to endonasal surgery the incidence of recurrent IP was 10% in contrast to 15% after a combined procedure., Conclusion: Our data show that endonasal micro-endoscopic surgery offers an effective and safe treatment modality of IP with insignificant morbidity. Strict application of selection criteria, wide removal of the tumour origin along the subperiosteal plane as well as drilling the underlying bone and close follow-up of patients are mandatory for success.

Background: The goal of this study was to assess the potentials and limitations of endonasal micro-endoscopic surgery in the treatment of sinonasal inverted papilloma (IP) and to demonstrate long-term results., Patients and Methods: From 1989 to 2005, 64 patients underwent resection of IP via an endonasal approach using either the endoscope or microscope. Charts were reviewed retrospectively for presenting symptoms, radiological and intraoperative data. All patients were followed by endoscopic and MRI control during a period of up to 174 months, median follow-up was 78 months., Results: Our study group consisted of 26 male and 38 female patients with an average age of 54.3 years. The majority of the patients (67 %) complained of unilateral nasal obstruction. 52 patients (81 %) were referred for primary surgery. In 12 cases (19 %) recurrent tumors were operated. According to the Krouse classification for IP the tumors were staged as T1 = 11 (17 %) cases, T2 = 37 (58 %) and T3 = 14 (22 %). In two patients a squamous cell carcinoma was associated with an IP ( = T4 stage). Most tumors were localized within the nasal cavity (72 %) or the anterior ethmoid (62 %). In 10 patients an infiltration of the bony skull base was present. During the follow-up period 6 patients developed recurrencies corresponding to an overall recurrence rate of 9.4 %., Conclusions: The advances in endonasal micro-endoscopic surgery allow both safe and effective removal of IP with low morbidity, and therefore it should be the approach of the first choice. The osteoplastic approach combined with endonasal surgery is suitable in far lateral located IP. Close follow-up is mandatory to ensure the surgical success.

This study evaluates the most extensive long-term treatment outcome of paranasal sinus mucocoeles with particular emphasis on the efficacy of endonasal micro-endoscopic management. It is a retrospective, consecutive case review of 255 patients with 290 mucocoeles including 125 frontal sinus, 23 frontoethmoid, 41 ethmoid, 72 maxillary sinus and 26 sphenoid mucocoeles. The median follow-up of the patients is 12 years (range 1 - 19 years). Sixtysix percent of the mucocoeles resulted after previous sinus surgery, whereas only 1.5% developed after endonasal micro-endoscopic surgery. The median period until mucocoele appearence was 10.8 years. Two hundred one mucocoeles (69.3%) were managed endonasally micro-endoscopically, 18.6% via the osteoplastic approach, 10% endoscopically in combination with an osteoplastic procedure, and 2% according to Lynch/Howarth. Thereafter, recurrence was found in 4 patients only (2.2%). In relation to the endonasal approach the recurrence rate was 1.6%. None of the patients treated endonasally had any complication. In view of these results this paper verifies endonasal micro-endoscopic surgery as a reliable treatment with favourable long-term outcome for paranasal sinus mucocoele management, but also describes contraindications for an endonasal procedure.

Background: The aim of the present study was to establish the efficacy of endonasal micro-endoscopic surgery for removal of benign and malignant neoplastic lesions of the paranasal sinuses and the anterior skull base., Material and Methods: This retrospective study evaluated 350 patients with tumors of the paranasal sinuses and the anterior skull base (215 benign and 135 malignant tumors) that were treated surgically at the ENT-Department of the Hospital Fulda gAG between 1993 and 2003. Median follow-up was 65 months., Results: Most frequent entities of benign tumors were osteomas, inverted papillomas and juvenile angiofibromas. Adenocarcinomas, squamous cell carcinomas and esthesioneuroblastomas were most frequently treated in the group of malignomas. 54 % of the benign (n = 118) and 41 % of the malignant tumors (n = 54) were resected exclusively via the endonasal micro-endoscopic approach. Within the follow-up period 3 recurrencies were observed, two inverted papillomas (one was operated endonasally) and one juvenile angiofibroma. In the malignoma group 34 patients died because of tumor disease (16 cases due to recurrencies, 18 cases due to metastases formation). The 5 year survival rate was 66.4 % with respect to the disease-specific survival. The Kaplan-Meier analysis revealed statistically significant differences for the pT stage: pT2 and pT3 tumors have had a 5-year disease-specific survival of 92.3 % and 83.8 %, respectively, compared to 61.5 % of the pT4 tumors. Disease-specific survival also showed differences dependent on histology, tumor site and occurrence, but was without proven significancy. In the patient subgroup who suffered from adenocarcinoma, squamous cell carcinoma or esthesioneuroblastoma the 5-year disease-specific survival was 78.4 % of 29 patients after endonasal resection compared to 66.4 % in 51 patients operated via an external approach., Conclusion: The advances in endonasal micro-endoscopic surgery also allow a safe and effective removal of benign and malignant anterior skull base tumors with low morbidity. Thereby, indication is dependent on tumor site and size as well as histology.

Background: Occult malformations of the lateral skull base are rare anomalies, but can cause severe complications such as recurrent meningitis. Therefore, they need to be precisely delineated and sufficient surgical closure is mandatory., Patients and Methods: Between 1986 and 2004 twenty patients (10 children and 10 adults) with occult malformations at the lateral skull base were treated surgically at the ENT-Department of the Hospital Fulda gAG. Of these 3 Mondini-malformations, 11 defects of the tegmen tympani or the mastoidal roof, 2 dural lesions to the posterior fossa and 4 malformations within the pyramidal apex have been found. Four patients have had multiple anomalies. Routing symptom was in all cases at least one previous meningitis. Radiological diagnostics included high-resolution computed tomography (CT) and magnetic resonance imaging (MRI) as well as CT- or MR-cisternography. Depending on type and localisation of the defect the following surgical algorithm was carried out: The trans-mastoidal approach was used in all cases of Mondini-malformation (including obliteration of the ear), in case of lesions to the posterior fossa as well as partly in anomalies at the tegmen tympani and mastoidal roof, respectively. Defects of the pyramidal apex should be explored via the trans-mastoidal way if the lesion is located caudally to the inner auditory canal (IAC), whereas the trans-temporal approach should be used if the lesion is situated ventral to the IAC and dorso-medially to the internal carotid artery (ICA). The trans-temporal approach was also performed in large defects of the tegmen tympani and mastoidal roof as well as in recurrences., Conclusions: In all cases of recurrent meningitis caused by agents of the upper airway tract the basic principle should be to search for occult skull base malformations radiologically as well as by sodium fluorescein endoscopy as long as the anomaly is detected.

Background: The aim of this study was to determine the best surgical approach in the treatment of paranasal sinus mucoceles according to their localization., Material and Methods: A retrospective analysis was carried out in 255 patients with 290 sinus mucoceles who were treated surgically at the ENT-Department of the Hospital Fulda gAG between 1983 and 2001. This series include 125 frontal sinus, 23 frontoethmoid, 41 ethmoid, 72 maxillary sinus and 26 sphenoid mucoceles. The patients' history, presenting symptoms, radiological findings, and surgical management were reviewed. Of them, 185 patients were followed by endoscopic and CT or MRI control during a period of up to 19 years, median follow-up was 12 years., Results: In 168 out of 255 patients (66 %) the mucoceles were arisen due to previous sinus surgery, in 37 cases (14 %) after traumatic lesions, in 5 patients (2 %) due to chronic sinusitis and in 2 cases (<1 %) according to tumors. In 43 cases (17 %) no causes were found. In 78.8 % the previous operation was performed via an external approach, either according to Jansen/Ritter or Caldwell-Luc, contrary to 1.5 % after endonasal surgery. The median period until mucocele appearance was 15 years for maxillary sinus, 13 years for frontoethmoid, 10 years for ethmoid, and 8 years for frontal and sphenoid sinus celes, respectively. 201 mucoceles (69.3 %) have been operated endonasal micro-endoscopically, 18.6 % via the osteoplastic approach, 10 % endoscopically combined with an osteoplastic procedure and only 2 % according to Jansen/Ritter. Thereafter, recurrence of mucoceles was found in 4 patients only ( = 2.2 %; related to the endonasal approach = 1.6 %)., Conclusion: Frontoethmoidal, ethmoidal, sphenoidal and maxillary sinus mucoceles are excellent indication for exclusively endonasal micro-endoscopic surgery. The osteoplastic approach combined with endonasal surgery is suitable in far lateral located frontal or maxillary sinus mucoceles.

Background: Congenital nasal fistulas and cysts are uncommon craniofacial malformations. Mostly their symptoms appear already during childhood. In general, they are superficially located but they can reach the skull base or extend intracranially., Patients and Methods: Between 1979 and 2004 fifteen patients (11 children and 4 adults) with nasal fistulas and cysts were treated surgically at the ENT-Department of the Hospital Fulda gAG. In 7 patients (46.6 %) the skull base was involved. An intracranial intradural extension was found in 1 child and therefore needed a combined ENT and neurosurgical procedure. In the other 6 cases the nasal fistulas or cysts were located extradurally. They were resected by removing the Christa galli and strenghten the dura. An additional septorhinoplasty was carried out in 4 of the 15 patients. Within the follow-up period of up to 25 years no recurrences were observed., Conclusions: The adequate therapy of nasal fistulas and cysts consists of complete resection. Magnetic resonance tomography (MRI) and computed tomography (CT) are essential to diagnose intracranial extension and to choose the proper surgical approach including a potential interdisciplinary procedure.

Background: In latero-basal, central or spheno-fronto-orbital skull base fractures the internal carotid artery is more frequently involved in severe lesions than expected., Patients and Methods: Between 1996 and 2003 we examined 684 patients with Glasgow Coma Scales (GCS) between 2 and 15, median 7.2, using computed tomography (CT). In suspicion of a latero-basal, central or spheno-fronto-orbital fractur they got an additional high resolution skull base CT. If the bony canal of the internal carotid artery (ICA) was involved the patient underwent digital subtraction angiography (DSA). The ICA lesion was treated either interventional neuroradiologically, by surgery or only conservatively., Results: Of the 684 patients 33 (4.8 %) had fractures of the ICA bony canal and therefore underwent DSA. Among them were 25 men and 8 women (mean age 35.3 years). Lesions of the ICA were seen in 1.9 % of the patients. A traumatic cavernous-carotid fistula was found in 7 patients (1 %) and in 6 patients (0.9 %) a dissection and/or an aneurysm of the ICA was diagnosed. Six of the patients had clinical symptoms. The lesions were treated primarily interventional neuroradiologically (n = 5) as well as surgically in two cases by clipping the aneurysm and closing the sphenoid sinus, respectively., Conclusions: Vessel lesions of the ICA in skull base fractures and involvement of the bony carotid canal are more frequent than mentioned in current literature. A solid diagnosis can only be achieved by DSA. Early diagnosis and treatment is important for improving the prognosis of these often multiply injured patients.

Background: The aim of the study was to evaluate postoperative hearing, facial nerve function, quality of life (QOL), affective status and neuropsychological performance after trans-temporal removal of acoustic neuromas (AN)., Patients and Methods: A retrospective analysis was carried out in 89 patients with AN who were operated at the ENT-Department of the Hospital Fulda between 1988 and 2004. Median follow up was 57 months. Of these 41 patients were additionally examined by a psychologist for evaluation of QOL, neuropsychological functions and affective disorders using several questionnaires., Results: The AN were classified as follows: Type A = 53 %, type B = 35 % and type C = 12 %. One year postoperatively facial nerve function was excellent in 93.3 % of the patients (grade I and II). In 53 % of cases hearing could be preserved. The subjective QOL was expressed through depression and social withdrawal associated with deafness. However, facial nerve dysfunction did not lead to QOL effect. Objective QOL (functional level) was reduced because of verbal memory disturbances and symptoms like dizziness. In 47.5 % of the patients affective and/or neuropsychological dysfunctions were diagnosed. Subsequent MRI evaluation showed lesions of the temporal lobe in 40.5 % of the cases. Of these 80 % suffered from cognitive and/or affective disturbances., Conclusions: The cognitive and affective disturbances after trans-temporal removal of AN could be to a certain degree due to the elevation of the temporal lobe during surgery. Further research, especially pre- and postoperative examination of QOL and psychological state as well as the comparison between different approaches, particularly trans-temporal vs suboccipital have to clear up specific morbidity of the different approaches.

Background: There are many autogenous and allogenous grafts as well as alloplastic materials available for the reconstruction of craniofacial bony frame. We review our techniques and describe the advantages of using calvarial bone, especially split-thickness grafts for bone reconstruction in this area., Patients and Methods: Between 1996 and 2003 the orbitocranial bony frames of 15 patients were reconstructed using split calvarial bone grafts at the ENT-Department of the Hospital Fulda gAG. In 12 patients the anterior frontal sinus wall or the entire Os frontale were affected. In 1 patient each the lateral wall, roof and floor of the orbit had to be reconstructed. The causes of the bone defects were trauma (n = 6), recurrent frontal sinusitis partly with osteomyelitis (n = 4), benigne tumors (n = 2) and malignancies (n = 3). Twelve patients have had multiple previous operations. In 3 patients the bone reconstruction was performed in the same operation as the tumor removal. Within the follow-up period between 2 and 8 years the split calvarial bone grafts remained stable in size and shape. Graft rejection, osteomyelitis or bone resorption did not occur. Furthermore, we have not experienced significant complications in harvesting cranial bone and have not seen major donor site morbidity., Conclusions: Our results demonstrate that split-thickness calvarial bone is an excellent graft not only for facial and forehead contouring but also for orbital and complex craniofacial reconstruction.

We describe a 71-year-old woman who complained of a 1-year history of double vision when looking to the left, numbness over the right cheek, intermittent tinnitus, and gradually increasing unsteadiness when walking. Computed tomography and magnetic resonance imaging revealed a cholesterol granuloma at the right pyramidal apex anterior to the internal auditory canal and a slight compression of the brainstem on the ipsilateral side. For surgical removal we used the transtemporal approach instead of the trans-sphenoidal approach to obtain better control over the internal carotid artery. To avoid the problems of stenting, the resulting dead space was obliterated with fat. We discuss the essential preoperative imaging, controversies in choosing the appropriate surgical approach, and developments in treatment.

Forty-two patients with recurrent respiratory papillomatosis (RRP) were accepted into a multicenter prospective study in 1983 to 1990, treated with alfa-IFN 3 MU/m 2 3 times a week and then followed-up until August 1, 2003. All the patients who had disease progression with pulmonary spread were characterized by insufficient response to IFN-therapy and detection of HPV type 11. Five patients (4/5 smokers) presented malignant transformation in lungs or nasopharynx (mean RRP duration was 27.2 +/- 8 years from RRP onset and 14.6 +/- 6.3 years from pulmonary spread until malignant transformation) with persistent RRP in larynx. The results of long-term follow-up in RRP patients with HPV 11 underline the necessity of reanalyzing the current therapy.

Objective: To characterize the cytogenetic alterations of esthesioneuroblastoma (ENB)., Methods: Comparative genomic hybridization (CGH) was performed on genomic DNA extracted from 12 patients with primary ENB, 4 patients with tumor recurrence and 7 with metastasis. Equal amounts of biotin-labeled tumor DNA and digoxigenin-labeled normal reference DNA were hybridized to normal meta phase chromosomes. Tumor DNA was visualized by fluorescein (FITC) and normal DNA by rhodamin (TRITC ) and detected by fluorescence microscopy. The signal intensities of the different fluorochromes were quantitated as gray levels along the single chromosomes. The over-and under-represented DNA segments were determined by computation of FITC/TRITC ratio images and average ratio profiles., Results: Consensus deletion regions were most frequently observed on chromosomes 1p, 2q, 3p/q, 4p/q, 5p/q, 6q, 8p/q, 9p, 10p/q, 11p, 12q, 13q, 18q, and 21q. DNA over-representations were identified on chromosomes 1p, 7q, 9q, 11q, 14q, 16p/q, 17p/q, 19p/q, 20p/q and 22p/q. The genetic pattern of ENB was distinct from that of other small round-cell tumor types and neuroblastomas. The deletion on chromosome band 1p21-p31 was associated with bad prognosis. In particular, all patients died whose tumors had combined 1p21-p31 deletion, with tumors in clinical stage C or D, and of low differentiation (grade III or IV). Clonality analysis revealed a high concordance between pairs of primaries and metastases., Conclusion: CGH analysis identifies characteristic cytogenetic aberrations of esthesioneuroblastoma associated with its malignant phenotype.

Background: Carcinomas of the external auditory canal (EAC) and the middle ear are rare and considered to have a poor prognosis. The recommended therapeutic strategy consists of surgical excision and postoperative radiotherapy. However, there are different opinions about the extend of the primary operation., Patients and Methods: A series of 21 patients with carcinoma of the EAC and middle ear were treated at the ENT-Department of the Hospital Fulda from 1985 to 2003. Their records and radiologic findings were reviewed retrospectively with particular reference to tumor type and size, its relation to surrounding tissues, surgical procedures and radiation techniques. The tumors were staged according to the modified Pittburgh staging system for temporal bone carcinomas. The average follow-up time was 6.2 years (range 0.2 - 18.75)., Results: 17 patients suffered from carcinoma of the EAC, 4 carcinomas were primarily located in the middle ear. There were 15 squamous cell carcinomas, 3 adenoidcystic carcinomas, 2 adenocarcinomas and one mucoepidermoid carcinoma. 12 patients came primarily to our institution and were staged as follows: pT1 (n = 2), pT3 (n = 2), pT4 (n = 8). 8 patients showed up with recurrent or residual tumors (all of T3 or T4 stage). One patient could not be classified. In 5 cases the tumor was inoperable. These patients underwent combined chemoradiation therapy. All other 16 patients were operated and most of them received adjuvant radiation therapy. In the group of patients who were primarily operated overall 5-year survival rate was 100 %. In contrast, patients who's recurrent or residual tumors were resected had a 5-year survival rate of only 33 %. Patients who received combined chemoradiation therapy showed a 2-year survival rate of 75 %., Conclusion: Carcinoma of the EAC and middle ear should be treated primarily by a lateral or subtotal temporal bone resection stage dependent combined with a parotidectomy as well as a neck dissection. Local resection of the EAC is not sufficient, not even in T1 tumors. As from stage T2, in cases of recurrent tumor removal and questionable free margins as well as in cases with lymph node metastases an adjuvant radiation therapy should be added. The most important survival factor is removal of the primary tumor with histologically clear margins.

This study reviewed the management and outcomes of 11 facial nerve neuromas treated in our institution during the past two decades with particular emphasis on surgical concepts and functional outcomes. All patients underwent complete surgical resection of their tumor. Eight patients (73%) were followed on an outpatient basis. A retrospective chart review for pre- and postoperative clinical and radiological data was performed. All facial neuromas were multi-segment tumors. All segments of the facial nerve were represented, but 54% involved the geniculate ganglion and 45% involved the labyrinthine or tympanic portions of the nerve, or both. Depending on the extent of sensorineural hearing loss, surgical removal was performed through the middle cranial fossa or translabyrinthine approach. To obtain adequate nerve reconstruction, we combined intra- and extracranial approaches (e.g., the transmastoidal and transtemporal routes). Regardless of the type of nerve reconstruction, the best recovery achieved was moderate facial weakness (House-Brackmann Grade III) in 75% of the patients, even in a patient who was Grade IV preoperatively. The choice of treatment for facial neuromas and surgical approach depends on the extent of tumor, grade of facial palsy, and hearing function. When facial palsy is present, complete resection is clearly indicated. In patients without facial dysfunction, a conservative strategy consisting of clinical and radiological observation should be considered as a treatment option.

We report on an unusual case of Frey's syndrome. A 45 year old male patient presented with gustatory sweating 14 years after parotidectomy. The sialography showed a post-parotidectomy status without any pathologic findings. The MRI also showed a normal rest-parenchyma of the parotid gland without any pathologic findings. Minor's starch iodine test [14], confirmed the gustatory sweating in the left preauricular area. Frey's syndrome can appear with a latency of 14 years postoperative or post-trauma. The pathogenic mechanism and the curative therapy for this auriculotemporal-syndrome are still unsolved.

Comparative genomic hybridization (CGH) was used to screen 22 esthesioneuroblastomas (ENB) from 12 patients including 12 primary tumors and 10 metastasis/recurrent lesions for chromosomal imbalances being the most extensive study so far. The analysis revealed a characteristic pattern consisting of deletions on chromosomes 3p and overrepresentations on 17q in up to 100% of cases. Other important alterations being detectable in more than 80% of cases were deletions on 1p, 3p/q, 9p, 10p/q along with overrepresentation on 17p13, 20p and 22q. Particularly striking was the pattern for chromosomes 3, 10 and 17q and 20 being affected almost exclusively by deletions or overrepresentations, respectively. Pronounced overrepresentations suggestive for high copy amplifications were seen on 1p34, 1q23-q31, 7p21, 7q31, 9p23-p24, 17q11-q22, 17q24-q25, 19, 20p, 20q13 and 22q13. Comparing tumor pairs from the same patient revealed a high concordance indicating clonality and confirming the genetic homogeneity of the tumor entity. The analysis of metastatic/recurrent lesions indicated a higher percentage of pronounced alterations, e.g., high copy DNA gains at 1q34-qter, 7q11, 9p23-p24, 9q34, 13q33-q34, 16p13.3, 16p11, 16q23-q24 and 17p13. The analysis furthermore suggested specific alterations, e.g., deletions of chromosome 11 and gains of 1p to be associated with metastasis formation and/or worse prognosis. Our results indicate that ENB is a distinct entity and provides criteria for its genetic distinction from other small round cell tumor types.

Background: The detection of cerebrospinal fluid fistulas in the region of the anterior or lateral skull base can be difficult. The fluorescein test with lumbar administration of 5% sodium fluorescein solution can be used to detect cerebrospinal fluid leakage, identify weak points in the dura, achieve precise localisation of cerebrospinal fluid fistulas and to check intraoperatively that watertight dural closure has been achieved. However, use of the test is problematic as the fluorescein solution used is not licensed for this indication in Germany and severe neurological complications are described in the literature. In order to clarify the legal situation regarding use of the test, we therefore analysed the complications occurring in a sizeable patient sample., Method: The records of all patients in whom a fluorescein test had been performed between 1979 and June 2000 were analysed retrospectively for the occurrence of complications., Results: The most frequent complication in the 368 fluorescein tests performed was headache, followed by nausea and vomiting, temperature elevation, dizziness and nuchal pain. These side-effects were no more frequent than described for lumbar puncture alone. Twenty-six patients experienced side-effects on the day of the operation, 65 on the first postoperative day, 36 on the second day, 34 on the third day and 13 patients after the third day. There were two cases of grand mal seizures following concomitant intrathecal contrast medium administration. None of the patients had side effects persisting longer than 4 weeks., Conclusions: Intrathecal administration of a 5 % fluorescein solution is a safe procedure provided that the maximum dosages are not exceeded and the solution is prepared and administered correctly and in accordance with the specified indications and contraindications. In view of its great diagnostic benefit and low risk when properly used, the dictates of therapeutic freedom allow use of this drug despite the fact that it is not licensed for this purpose in Germany. It is necessary to obtain written informed consent from the patient.

Occult malformations of the skull base are rare anomalies, but can cause severe complications such as meningitis. Detailed skull base investigations for detecting cerebrospinal fluid fistulas or celes are often not initiated until after a history of recurrent meningitis. We present a child first seen at the age of 12 with recurrent episodes of bacterial meningitis since early childhood, requiring antibiotic prophylaxis for years. High-resolution computed tomography revealed a chronic sinusitis and a bony defect on the right olfactory groove, while magnetic resonance imaging and CT-cisternography indicated no cerebrospinal fluid fistula or cele at that time. Endonasal surgery for chronic sinusitis was performed with a confirmed bony defect on the right olfactory groove and an olfactory fibre without its sleeve-like dura prolongation running into an adjacent ethmoidal cell, necessitating that it be covered. In the absence of any antibiotics a new episode of meningitis occurred 5 years after surgery. CT-cisternography and magnetic resonance imaging were repeated, now indicating a transclival bony defect with a meningocele in its proximal part, most probably presenting a canalis basilaris medianus. Endonasal surgery confirmed this bony defect after adenoidectomy, and closure was accomplished. No further meningitis has been observed for 2 years. Congenital skull base defects may be difficult to detect, but sufficient surgical closure after their precise delineation is mandatory to prevent infectious endocranial complications. The presence of more than one developmental skull base defect should be considered during careful radiological skull base evaluation, which has to include the clivus in order not to overlook rare basilar malformations.

Case Report: Granulomatous infectious processes have a wide differential diagnosis. This report describes the case of a 73-year-old woman who had gone through an 8-year ordeal involving several paranasal sinus operations, development of chronic facial pain, orbital exenteration of the left eye, and now threatening loss of the remaining right eye on account of progression of the chronic inflammation. Despite repeated histologic examination of ENT material by various pathologic institutes, neither the histology nor laboratory parameters were able to point us in the right direction. In the end, it was the clinical course which led to the diagnosis of Wegener's granulomatosis., Conclusion: In chronic necrotizing granulomatous inflammatory processes in the region of the paranasal sinuses with involvement of the surrounding anatomic structures (orbit, skull base), Wegener's granulomatosis should be included in the differential diagnosis at an early stage, even if the typical signs of vasculitis and the typical antibodies are absent. The clinical course calls for an interdisciplinary treatment approach in conjunction with internists experienced in immunosuppressant therapy.

Objective: To summaries the treatment strategy of esthesioneuroblastoma (ENB)., Methods: Between 1988 and 2001, 17 patients with ENB were treated at the Department of Otorhinolaryngology of the Klinikum Fulda. All patients were monitored on an outpatient basis after completed therapy with a median follow-up of 44 months. In a retrospective review, the patients' charts, the computed tomography, and magnetic resonance imaging scans, the operation reports, and the follow-up data were analyzed, particularly with respect to the surgical approaches., Results: All tumors were staged according to Morita. One patient was classified as stage A, six stage B, nine stage C, and one stage D. All patients received surgical resection. Ten patients were disease free for at least 2 years, whereas 6 patients died because of ENB and one due to other disease. Of 10 patients who were free of disease, the tumors were removed via a transnasal approach in 6 patients using the microscope in combination with the endoscope. These tumors resected endonasally were staged as A (1 case) and B (5 cases). In ENB of stage C a craniofacial resection was performed using a subfrontal approach or the midfacial degloving. The lateral rhinotomy was applied only in cases in which an exenteration orbitae had to be carried out., Conclusion: ENB is best managed by complete surgical resection followed by adjuvant stereotactic radiation therapy. The Fulda surgical concept in management of anterior skull base tumors is also forwarded to resection of ENB. It classifies the following indications: 1) Endonasal approach in cases without tumor infiltration of the orbit and/or the brain; 2) Subfrontal approach in cases with extended tumor infiltration of the intradural space or the brain; 3) Midfacial degloving in cases with far lateral tumor spread, particularly fossa pterygoidea or pterygopalatina; 4) Lateral rhinotomy in all cases where an exenterative orbita is needed.

Objectives/hypothesis: Magnetic resonance imaging (MRI) has become the investigation of choice to follow up patients after vestibular schwannoma resection., Study Design: Retrospective., Methods: Postoperative MRI findings of 70 patients after vestibular schwannoma resection through a transtemporal (n = 48) and a translabyrinthine (n = 22) approach were reviewed. Time-dependent changes in intensity, size, and shape of enhancement in the internal auditory canal before and after contrast administration, postoperative temporal lobe gliosis, and changes of fat grafts were evaluated., Results: After vestibular schwannoma resection, all patients showed signal enhancements in the internal auditory canal ranging from a faint to high signal intensity in the first postoperative MRI, 3 to 6 months after surgery. In the next MRI at 12 to 24 months after surgery, 30 patients (43%) showed a decreased signal, 35 patients (50%) a stable enhancement, and 5 patients (7%) an increased enhancement in the internal auditory canal depicted as an intense nodular or mass-like pattern. In patients with decreased or stable enhancement, a residual tumor could be excluded in the following MRI scans, whereas in all patients with increased enhancements after 12 to 24 months, signal enhancement further increased and residual tumors were detected. Different degrees of temporal lobe gliosis were found in 15 of 48 cases (31%) after transtemporal tumor removal. Enhancement of fat grafts used in 22 cases decreased to different degrees in 14 cases (64%)., Conclusions: Differentiation of residual tumor from scar tissue in the internal auditory canal after vestibular schwannoma resection requires close, long-term follow-up. Nodular and progressive enhancements in the internal auditory canal indicate residual tumor. Linear enhancement in the internal auditory canal has been found to be a common finding after vestibular schwannoma resection not associated with residual tumor.