Sphenoid sinus pyocele after transsphenoidal approach for pituitary adenoma.

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.

Clivus chordoma: is it enough to image the primary site?

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.

Technique and results of the anterior-to-posterior-to-anterior approach in revision endoscopic sinus surgery.

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.

Clinical predictors for germline mutations in head and neck paraganglioma patients: cost reduction strategy in genetic diagnostic process as fall-out.

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

Management of cerebrospinal fluid leak after surgical removal of pituitary adenomas.

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.

[Draf's endonasal drainage for inflammatory frontal sinus disease].

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.

Is PET superior to MRI in the pretherapeutic evaluation of head and neck squamous cell carcinoma?

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.

Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review.

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.

Inverted papilloma: feasibility of endonasal surgery and long-term results of 87 cases.

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.

Surgery for paranasal sinus mucocoeles: efficacy of endonasal micro-endoscopic management and long-term results of 185 patients.

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.

Cervical paragangliomas-tumor control and long-term functional results after surgery.

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.

Incidence, age at onset, and potential reasons of malignant transformation in recurrent respiratory papillomatosis patients: 20 years experience.

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.

[Cytogenetic aberrations of esthesioneuroblastoma studied by comparative genomic hybridization].

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.

Clinicoradiological and surgical considerations in the treatment of cholesterol granuloma of the petrous pyramid.

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.

CGH pattern of esthesioneuroblastoma and their metastases.

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.

Use of sodium fluorescein solution for detection of cerebrospinal fluid fistulas: an analysis of 420 administrations and reported complications in Europe and the United States.

BACKGROUND: Localization of dural fistulas in the region of the anterior or lateral skull base may be difficult. For many years, a sodium fluorescein solution of 0.5 to 5% (2.5-50 mg) has been administered intrathecally by way of the lumbar space. However, fluorescein is not commercially available for this stated purpose in either Germany or the United States. METHODS: Retrospectively, 420 fluorescein applications by the authors were retrospectively analyzed. Under the Freedom of Information Act, the United States Federal Drug Administration and the manufactures of fluorescein were queried for adverse reaction reports. RESULTS: Four hundred twenty fluorescein applications in 305 patients could be evaluated. Mean age of recipients was 46.9 years, ranging from 1 to 82 years. At a concentration of 5% fluorescein, 26 patients on the day of surgery and 69, 37, 34, and 14 patients on days 2 through 4 suffered from minor side effects that may or may not have been related to this drug. Two of these patients had grand mal seizures, which were attributable to simultaneous intrathecal application of contrast medium. All other side effects were thought to be the result of a postspinal headache and related lumbar puncture. At a concentration of 0.5%, the intraoperative intrathecal administration of 0.5 to 2 mL of fluorescein followed by 4 to 5 days of lumbar drainage resulted in some degree of spinal headache without other complications. In both groups, no patient had sequelae longer than 4 weeks. An additional seven complications were reported to the Federal Drug Administration and the fluorescein manufacturers at doses of 100 to 700 mg. CONCLUSIONS: Complications from intrathecal application of fluorescein appears to be dose dependent. At concentrations of 5%, or preferably lower, side effects are transient. A grand mal seizure can be minimized when following the general cautions of lumbar puncture and dose. The patient should be supervised for 24 hours. A written informed consent from patients for use of fluorescein is recommended.

Facial nerve neuroma: surgical concept and functional results.

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.

[Management of esthesioneuroblastoma of the Fulda surgical concept].

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.

[Chronic bilateral necrotizing and destructive granulomatous inflammatory process in the region of the paranasal sinuses and the orbita as manifestation of localized Wegener's granulomatosis]. / Chronisch granulomatöser nekrotisierender und destruierender Entzündungsprozess im Bereich der Nasennebenhöhlen und der Orbita beidseits als Ausdruck eines lokalisierten Morbus Wegener.

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.

Nasal correction in maxillonasal dysplasia (Binder's syndrome): a long term follow-up study.

Maxillonasal dysplasia or Binder's syndrome is an uncommon though easily recognizable congenital condition characterized by a retruded mid-face with an extremely flat nose. The facial deficiencies lead to functional as well as psychological problems. We report on 10 patients with maxillonasal dysplasia whose noses were corrected with onlay costal cartilage grafts using a combined oral vestibular and external rhinoplasty approach. The technique has been used in children as well as adults with promising results. Since the degree of malformation in Binder's syndrome varies significantly surgical correction needs to be tailored individually based on the principles demonstrated. In all patients, minor malocclusion was first treated by orthodontists. Over a follow up period up to 14 years the advancement of the nose was found to be stable, even in lateral cephalograms. The treatment of these patients is a challenge for every surgeon and needs interdisciplinary cooperation.