耳鼻喉科教学课件：Nasal and sinus disease.ppt

Nasal and sinus diseaseNasal and sinus diseaseContentsContents•nasal septal abnormalities•trauma•epistaxis •congenital choanal atresia •benign tumors of nose•malignant tumors of noseNasal septal abnormalitiesNasal septal abnormalitiesthe nasal septal cartilageand boneAnatomy Anatomy •Anteriorly--quadrangular cartilage•posteroinferiorly—vomer•posterosuperiorly--the perpendicular plate of the ethmoid •inferiorly-- the maxillary crest•The anterior portion is flexible, whereas the posterior portion is bony and fixed Nasal septumNasal septumNasal septal deviationNasal septal deviation•At birth: normally straight•Infancy and early childhood: remain straight•Age progresses: deviated to one side or the other or projection to develop— septal spur ,shelf ,hump•During the growth lose its midline position•Few adults have a septum that is altogether in the midline•Birth trauma---bent septum, twisted nose right itself after a few days (often)Persist deformity—correction (sometimes)Childhood or adult life trauma--correctionphysiology physiology The nasal septum serves many functions•separation of the nasal airway into two distinct chambers•support of the nasal dorsum• maintenance of the shape of the columella and tipphysiology physiology •Traumatic deviation or developmental abnormalities nasal airway obstruction and cosmetic deformity•Poor airflow impaired olfaction impaired humidification filtering of the passing air reduced oxygen inflow•Anatomic septal deviation chronic sinus disease an external nasal deformitysymptomssymptoms•Obstruction—look alike, the least important •Severely deviated septum may not obstruction cause any discomfort at all•Deviated—obstruction some no complaint others are in distresssymptomssymptoms•Headache—septal spur impinging on the inferior turbinate or a septal deviation when it causes nasal obstruction•Both headache— septal deformity is great•Deviated septum cause sinusitis—head pain , carefully before operationsymptomssymptoms Nosebleeds---air currents drying the mucosa that covers a deflected septum—mucosa trauma—crusts form –bleeding Anterior part of nose•The septoplasty operation combined with surgery on the turbinates and valves•Septoplasty corrects structural deformities of the nasal septum to relieve nasal obstruction•When septoplasty is combined with rhinoplasty, the procedure may help to straighten the deviated nose and provide cartilage graftsA, Deviated nose prior to septoplasty B, The significant correction of the deviated nose with septoplasty aloneDeviation of the nasal septumDeviation of the nasal septum•may be congenital (present at birth) or occur later from trauma.•cause nasal obstruction , headache,epistaxis,sneezing, postnasal drip, snoring, , anosmia .•Treatment : septoplasty.  Septal perforationSeptal perforationSeptal perforation•Cause trauma nose picking surgery perichondritis from chromium exposure cocaine abuse•Posterior perforations involving the vomer and perpendicular plate of the ethmoid may be the result of surgery or syphilisSeptal perforationSeptal perforation Examination: usually obvious overlooked when crusted Large septal perforation: see opposite side nose mucosa Perforation usually in cartilaginous septum may involve the bony septumSeptal perforationSeptal perforation•Surgical closure is possible but not always successful•Use ointment to control crusting•Bleeding require packing or cauterySeptal abscessSeptal abscess•A septal abscess is a collection of purulent material beneath the mucoperichondrium of the septal cartilage either unilateral or bilateral•Patients with this condition typically febrile complain of pain nasal obstruction Septal abscessSeptal abscess•Important: painful serious consequencesNecrosis of cartilage—dorsum of nose may fallThrombosis of cavernous sinus: infection of nose drain through the angular vein—inferior ophthalmic vein—cavernous sinus—death in a few days Septal abscessSeptal abscess•trauma or nasal surgery a septal hematoma becomes infected•The most common organism isolated Staphylococcus aureus•Signs: septum balloons out on both side and red widening just behind the columella and extend posteriorly pressure cause pitting and increase pain incision releases pusSeptal abscessSeptal abscess•The patient is usually admitted and started on intravenous antibiotics targeted at S. aureus• Incision and drainage is done as soon as possible• Cultures and Gram stain are obtained to guide antimicrobial therapy Septal abscessSeptal abscess•Complications for untreated: Both cosmetic and functional sequelae may result in•These include saddle-nose deformity, nasal airway obstruction, nasal septal perforation, extension of infection to paranasal sinuses, and extension to intracranial cavity through the venous systemSeptal haematomaSeptal haematoma•Injury or a nasal operation haematoma may develop•Blood between the cartilage and perichondrium•Treatment: incision drainage packing help to prevent addotional bleeding•Antibiotics to prevent abscess even no infection presentSeptal haematomaSeptal haematoma•Untreated septal hematomas may result in destruction of the septal cartilage secondary to infection or vascular necrosis•The destruction of cartilage then may cause a loss of dorsal nasal support, with a resultant external cosmetic deformity, termed a "saddle-nose deformity" Trauma Trauma Evaluating nasal trauma :•When did the trauma occur? •Is there a new external nasal deformity? •Is there new persistent nasal obstruction? •Is there loss of visual acuity or diplopia? TraumaTraumakey features are evaluated on the physical examination in facial traumaExternal nose and face •Check for cosmetic deformity (compare to pre-trauma picture)•Palpate nasal bones, bony step-offs Internal nose •Note any septal deviation, mucosal tear, or hematoma (palpate with cotton-tipped applicator; aspirate with needle if suspicous) •Clear rhinorrhea from nose (may indicate cerebrospinal fluid [CSF] leak)TraumaTraumaOral cavity •Check palate mobility (mobility indicates Le Fort's fracture)•Identify any malocclusion (may indicate mandible fracture)TraumaTrauma•Nasal bone fracture is diagnosed by palpation, not x-ray. If other facial fractures are suspected, the most useful study is a thin-cut (1-3 mm) axial CT of the face with coronal reconstructions •Nasal fracture is quite common during birth—greenstick type simple treat adult fracture—smash both to one side -- frontal blow depress bone --various other injures --facial fracture inferior orbital rim or zygomaThe two nasal bones together with the two upper and lower lateral cartilages form the externalframework of the noseTraumaTrauma•External nose lacerated compound fracture may exist•Usually simple nasal fracture•Cartilage of septum injure but not nasal bone•Both Cartilage of septum and nasal bone•Occasionally the tip of nose injure independently•The intercanthal line demarcates the transition point between thicker nasal bone superiorly and thinner bone inferiorly. Most nasal fractures will occur below this levelExamples of nasal fracture patternsTraumaTrauma•Treatment is not difficult•Aim of ruduction are to obtain a satisfactory airway and to restore the original appearance of the nose •Lateral type of fracture– smash inward and other outward simple thumb press back on the convex side•Depressed fracture—reduction under local or general anesthesia an elevator or a hemostat cover with a thin rubber tubing is used to pry up the nasal boneClosed nasal reduction.•After marking the distance of the intercanthal line on the elevator with a thumb, the tip of the instrument is used to reduce the medialized fragment. The opposite thumb may simultaneously reduce a contralateral outfractured nasal bone•Closed septal reduction using Asch forceps.TraumaTraumaComom errors•Set nasal fracture many years previously a new trauma•X-ray reneal no fracture but actually there is little pracal value clinic judgement is much more important•An easy-to-reduce fracture too seriously or a severe fracture too lightly •Wait too long to reduce the fracture within 1 weekDangerous triangle of the face•From corners of the mouth to nasion of the nose•It is possible (although very rare) for retrograde infections from the nasal area to spread to the brain•It is a common misconception that the veins of the head do not contain one way valves like other veins of the circulatory system. In fact, it is not the absence of venous valves but the existence of communications between the facial vein and cavernous sinus and the direction of blood flow that is important in the spread of infection from the face. http://www.ncbi.nlm.nih.gov/pubmed/20491800TraumaTrauma•The term maxillofacial trauma or craniomaxillofacial trauma might be better described the anterior wall and floor of the anterior cranial fossa •a significant impact on critical functions such as vision and mastication• Positioning of incisions and the extent of various surgical exposures can influence the final appearance of the face and the function of facial structures like the eyelids, lips, and noseTraumaTrauma•the proper management requires a comprehensive approach• familiar with the various ramifications of skull base, orbital, facial, sinus, dentoalveolar, and airway injuries and, most importantly, by those willing to collaborate when necessary with other specialists who may have overlapping areas of expertise•combined facial and anterior skull base injuries are frequently best approached jointly by a neurosurgeon and a craniomaxillofacial surgeonTraumaTraumaLateral view of a patient with a depressed central frontal fractureTraumaTraumaAxial CT demonstrates markedly displaced anterior and posterior walls of the frontal sinusTraumaTrauma•Severe direct trauma—a blow a fall or an automobile accident•So-call middle third or Le Fort III fracture entire middle third face is driven backward resulting deformity is a very flat face mandibular teeth protruding beyond teeth•Usually occur when auto is stopped suddenly right front seat is thrown forward face strike the hard dashboardTraumaTrauma•In all cases of severe facial injury it is important to determine where there has been any intracranial or cervical damage before correcting a major facial fracture•Immediate reduction of any of facial bones is not imperative•Results just as satisfactory can be obtained 2-3 days laterTraumaTraumaTraumaTrauma•Le Fort I fracture•horizontal fracture •that separates the bone •containing the maxillary •dentition from the •remainder of the •craniofacial skeleton The Le Fort II fracture "parametal" fracture that extends across the maxilla through the infraorbital rim and orbital floor up through the medial orbital wall and across the nasal root area, then across similarly the other side•The LeFort III fracture• the true craniofacial •separation that includes• fractures of the zygomatic •arches and frontozygomatic •areas, then crosses the •lateral inferior and medial• orbits and is completed •across the nasal root. •Note that all Le Fort fractures •cross the nasal septum and• pterygoid platesTrauma(Le Fort’s fracture)Trauma(Le Fort’s fracture)The key physical finding in Le Fort's fractures •Mobile palate (common to all Le Fort's fractures) •Facial edema, ecchymosis, and malocclusion •Epistaxis, bony step-offs •Midface elongation or compression •Severe manifestations of Le Fort's fracture may include blindness, CSF rhinorrhea, and airway obstruction (more common in Le Fort II and III) TraumaTraumaKEY POINTS of FACIAL FRACTURES•Rule out septal hematoma in every case of nasal bone fracture. •Thin-cut (1-3 mm) facial CT with coronal reformatting is the most useful and cost-effective radiologic examination in facial trauma. •Rule out open globe trauma prior to exploring/repairing a blow-out fracture. •All patients with Le Fort's fractures have a mobile palate.Blow-out fracture •an out-fracture of the orbital wall caused by a blunt, nonpenetrating object that impacts the globe and suddenly increases intraorbital pressure•The missile must be less than 5 cm in maximum diameter at the point of impact (typical objects include baseballs, fists, hockey pucks, and champagne corks) to clear the orbital rim and impact the globe Blow-out fracture findings •The orbital floor outfractures into the maxillary sinus and the medial orbital wall fractures into the ethmoid sinus•Orbital fat, which serves to protect the ocular globe, may extrude through the orbital floor defect and into the maxillary sinus additionally, the inferior rectus muscle may extrude and become entrapped•Small blow-out fractures often cause entrapment large fractures cause enophthalmosA coronal scan clearly demonstratinga complete blowout fracture of the right orbital floorAn axial scan demonstrating a medial orbital blowout fractureTraumaTrauma•After the herniated orbital contents are reduced into the intraorbital space, the orbital floor is reconstructed• A thin piece of autogenous or exogenous implant material is inserted between the orbital floor bone and orbital floor periosteum to prevent further herniationTraumaTraumaA tripod fracture, also called a zygomaticomaxillary complex (ZMC) fracture, is the most common fracture of the zygomatic bone and usually involves: •Zygomaticofrontal suture (or may involve the frontal process of the zygoma) •Zygomaticomaxillary suture •Zygomatic arch TraumaTraumaThe indications for repairing tripod fractures •Visual compromise •Extraocular muscle dysfunction •Globe displacement •Significant orbital floor disruption •Displaced or comminuted fractures EpistaxisEpistaxis•Most episodes of epistaxis are minor •The incidence of an episode of epistaxis during one's lifetime approximately 60% with less than 10% of these requiring medical attention •Males are slightly more affected than females over the age of 50 years, the ratio is close to 1:1•children and adolescents are more often afflicted with minor episodes of anterior nasal bleeding •severe posterior nasal bleeding is greater in those who are more than 50 years EpistaxisEpistaxis•Blood supply of the lateral nasal wallEpistaxisEpistaxis•Blood supply of the nasal septum.EpistaxisEpistaxis•The nasal mucosa is supplied by both the internal and external carotid arteries• The internal carotid artery gives rise to the ophthalmic artery, which branches into the anterior and posterior ethmoid arteries supply the anterior and posterior superior nasal cavity and septum EpistaxisEpistaxis•The external carotid artery contributes blood via the internal maxillary and facial arteries. •The internal maxillary artery divides into several branches, including the greater palatine and sphenopalatine arteries. The greater palatine artery contributes to Kiesselbach's plexus, whereas the sphenopalatine artery supplies the posterolateral nasal wall and is a main source of posterior epistaxis•The facial artery gives rise to the superior labial artery. The nasal branch of the superior labial artery contributes to Kiesselbach's plexus EpistaxisEpistaxisLOCAL CAUSES OF EPISTAXIS•Traumatic•••Nasal fracture with disruption of the nasal mucosa•••Nasal surgical procedures•••Nasal intubation•••Digital trauma (consider obsessive-compulsive disorder in adults)•••Antihistamine and steroid nasal sprays•••Cocaine, snuff, and heroin sniffing•••Nasal oxygen or continuous positive airway pressure•••Nasal foreign bodiesEpistaxisEpistaxisStructural•••Nasal septal deformity (deflections and spurs)•••septal perforationsInflammatory disease•••Viral upper respiratory infections•••Bacterial sinusitis•••Allergic rhinitis•••pyogenic granuloma ••Granulomatous diseases (Wegener's granulomatosis, tuberculosis, sarcoidosis, syphilis) ••Environmental irritants (cigarette smoking, chemicals, pollution)EpistaxisEpistaxisTumors/vascular malformations•••Angiofibroma•••Aneurysms•••Epidermoid carcinomas•••Nasal papilloma•••Adenocarcinoma•••Encephalocele meningoceles encephaloceles, or gliomas •••Esthesioneuroblastoma•••HemangiomaEpistaxisEpistaxisSYSTEMIC CAUSES OF EPISTAXISCoagulation deficits•••Thrombocytopenia•••Acquired coagulopathies•••Congenital coagulopathies•••Vitamin A, D, C, E, or K deficiency•••Liver disease•••Renal failure•••Chronic alcohol abuse•••Malnutrition•••Polycythemia vera•••Multiple myeloma•••Anticoagulant drugs (aspirin, nonsteroidal antiinflammatory drugs, heparin, Coumadin)•••LeukemiaEpistaxisEpistaxisSYSTEMIC CAUSES OF EPISTAXISVascular disease•••Arteriosclerotic•••Collagen abnormalities•••Hereditary hemorrhagic telangiectasiaCardiovascular conditions that increase venous pressure (congestive heart failure, mitral valve stenosis)Hypertension (unproven relationship)environmental factors Cold, dry air and temperature EpistaxisEpistaxisMANAGEMENT OF EPISTAXIS•Medical management••• Nasal hydration with saline mist, nasal gels, and ointment •Nasal packing•••Traditional anterior pack•••Nasal sponges•••Gelfoam•••Traditional posterior pack•••Nasal balloonMANAGEMENT OF EPISTAXISMANAGEMENT OF EPISTAXIS•Cautery•••Silver nitrate•••Endoscopic electrocautery•••Laser cautery•Embolization•Ligation•••Transantral ligation of the internal maxillary artery•••External ligation of the ethmoid arteries•••Endoscopic ligation of the sphenopalatine artery•Surgery•••Septoplasty•••Septal dermoplasty•Management protocols for acute epistaxisManagement protocols for chronic or Management protocols for chronic or recurrent epistaxisrecurrent epistaxisPlacement of traditional anterior nasal Placement of traditional anterior nasal packingpackingPlacement of a nasal spongePlacement of a nasal sponge•Posterior nasal packs•Placement of a nasal balloon•Positioning of a balloon•Incision used in intraoral approach to maxillary arteryTransantral ligation of maxillary artery Transantral ligation of maxillary artery showing anatomic landmarksshowing anatomic landmarksCongenital choanal atresiaCongenital choanal atresia•First reported in 1830 occurs in 1 in 5000 to 8000 live births •50% have other associated congenital anomalies •twice as common in females as in males •About 65 to 75% of these anomalies are unilateral the rest are bilateral •Up to 75% of the bilateral cases have other associated anomalies Congenital choanal atresiaCongenital choanal atresia•About 30% are pure bony; whereas 70% are mixed bony-membranous •four parts of the anatomic deformity a narrow nasal cavity, • lateral bony obstruction• medial obstruction caused by • thickening of the vomer• membranous obstructionCongenital choanal atresiaCongenital choanal atresia•the thickened posterior septum connected to abnormal lateral pterygoid bone by a thin fibroepithelial membraneCongenital choanal atresiaCongenital choanal atresia•Computed tomography of a patient with bilateral mixed bony-membranous choanal atresiaCongenital choanal atresiaCongenital choanal atresia•Computed tomography of a patient with pyriform aperture stenosis. The anterior nasal valve is narrowed by abnormal boneTumors of external noseTumors of external nose•Squamous cell carcinoma and basal cell carcinoma attack the external nose•Squamous cell carcinoma usually grow faster metastasize to the neck at any tage•Basal cell remain very small gradually spead locally•Early stage treatment is very successful wide local excision is necessary•Other malignant are unusualTumors of external noseTumors of external nose•Rhinophyma is benign caused by an overgrowth of sebaceous glands of the tip nose•“Potato nose” growth marked and produces lobulated tumors•Treatment:excess tissue is shaved of until a normal configuration skin graft is appliedTumors of the internalTumors of the internalnosenose•Squamous papilloma occur in the nasal vestibule cause irritation easily removed by excision or diathermy•Nasal polyps most common tumor from sinus or ostia and hang into nose Mucocele of sinus •A sinus mucocele is defined as a mucous collection lined by the mucous-secreting epithelium of a paranasal sinus. It occurs when a sinus ostium or a compartment of a septated sinus becomes obstructed, thus causing the sinus cavity to be mucous-filled and airless•Sixty to 65% of mucoceles reside in the frontal sinuses 20 to 25% in the ethmoid sinuses 5 to 10% in the maxillary sinuses 5 to 10% in the sphenoid sinusesMucocele of ethmoid sinus N Engl J Med 358;23 www.nejm.org june 5, 2008•A:Left exophthalmos and outward displacement of the orbit•B:On CT, a cystic lesion was observed in the left ethmoid sinus (Panel B), where it was compressing theglobe (white arrow) and eroding the lamina papyracea •Frontal mucocele•Pyomucocele in the left posterior nasal cavity and ethmoids, which appears to extend into the sphenoid sinus•mucocele in the ethmoids and sphenoid sinusCyst in the floor of maxillary sinusinverted papilloma inverted papilloma •From 3% to 24% of inverted papillomas contain or transform into carcinomas•The tumor is benign but aggressive, with frequent multicentricity. If the inverted papilloma is simply excised, it may recur with bony destruction and intracranial extension •Overall recurrence rates vary from 27% to 73%. inverted papilloma inverted papilloma •These tumors are less translucent than polyps. They are usually unilateral and more commonly present with epistaxis• As a rule, you should biopsy all unilateral nasal polyps in the clinic before surgery unless they are obviously vascularinverted papilloma inverted papilloma •As with genital condylomas, skin warts, and laryngeal papillomas, sinonasal papillomas often contain human papilloma virus (HPV)• Epstein-Barr virus recently has been found in 65% of inverted papillomasinverted papilloma inverted papilloma •When HPV is present, there is a higher likelihood of both malignancy and recurrence•HPV types 6 and 11 are associated with benign papillary tumors• types 16 and 18 are weakly associated with malignant degeneration•However, the presence of any HPV strongly predicts recurrence•In one study, 13 of 15 patients with recurrent papilloma were HPV positive, and 10 of 10 patients without recurrence were HPV negativeinverted papillomainverted papilloma•Histologically benign but clinically malignant•Continuity involve adjucent structures •Strong tendency toward recurrence and transform into carcinomas•Adequate surgery usually cure Squamous cell carcinomaSquamous cell carcinoma•the most common malignancy of the nose and paranasal sinusesSquamous cell carcinomaSquamous cell carcinoma•Squamous cell carcinoma accounts for 70% of these cancers•followed by adenocarcinoma (5-10%) and adenoid cystic carcinoma (5-10%)•Less common tumors include undifferentiated transitional cell carcinoma, olfactory esthesioneuroblastoma, lymphoma, and malignant melanomaSquamous cell carcinomaSquamous cell carcinoma•nose and sinus cancers typically originate• The maxillary sinus is the most common site (55%), followed by the nasal cavity (35%), ethmoid sinus (9%), and, rarely, sphenoid sinus. Septal cancers are exceedingly rare. Sinus carcinama signsSinus carcinama signs•Usually, patients experience prolonged sinusitis, especially unilateral•Patients may develop epistaxis, numbness, swelling, or nasal congestion•Patients with maxillary sinus tumors may have loose upper teeth or suddenly poor-fitting dentures•Pain is often a late and therefore ominous symptom•Ethmoid sinus tumors usually spread into vital structures before causing symptoms. They may invade the anterior cranial fossa, orbits, maxillary sinuses, or sphenoid sinuses• Usual symptoms include unilateral nasal obstruction, severe headache, and/or diplopiaSquamous cell carcinomaSquamous cell carcinoma•Carcinima of the maxillary sinus cause no symptomas at early•Erode into nose cause obstruction bloody discharge•Erode posterior or lateral wall of the antrum—persistent pain in face•Erosion of superior wall—orbitalcontents displaced upward produce proptosis double vision•Superior alveolar ridge involved –teeth looseSquamous cell carcinomaSquamous cell carcinoma•Diagnosis is by biopsy•Biopsy can be obtain from nose•Open the sinus through Caldwell-Luc incision to obtain a specimen when no erode the noseSquamous cell carcinomaSquamous cell carcinoma•Complete surgical excision of the maxilla•Necessary to resect the contents of the orbit when superior wall is invaded•Radiation alone is usually palliative•A combination of irradiation and maxillectomy may be more effective than either procedure alone•Early diagnosis greatly improve the surgical resultSquamous cell carcinomaSquamous cell carcinoma•Malignancy in the frontal sinus is more unusual than in maxilla •No early symptoms nasal bleeding, pain, or even proptosis may be first symptom•Treatment usually a combination of surgery and radiation•The prognosis is poorSquamous cell carcinomaSquamous cell carcinoma•Carcinoma in the ethmoid sinus is more common•Nose is full of tumors the eye is pushed outward the skin near the inner canthus breaks down•Cure is occasionally brought about external ethmoidectomy and late radiation therapySquamous cell carcinomaSquamous cell carcinoma•Malignancy in the sphenoid sinus is rare Squamous cell carcinomaSquamous cell carcinoma•Ohngren's line an imaginary line extending from the medial canthus of the eye to the angle of the mandible •Tumors above the line have a poorer prognosis because of their tendency to metastasize superiorly and posteriorly. •Tumors below the line are more easily resected and carry a better prognosis Squamous cell carcinomaSquamous cell carcinoma•nose and sinus tumors metastasize to cervical or retropharyngeal nodes•The incidence of cervical metastases on presentation is about 10% although up to 44% of cases will eventually metastasize to the cervical area Only 10% of patients ever develop distant metastasesSquamous cell carcinomaSquamous cell carcinoma•Surgery is the mainstay therapy and can be curative if resection is complete•surgery is more easily performed on small tumors Options for resection include inferior maxillectomy medial maxillectomy and total maxillectomy•Most centers use postoperative radiation therapy for large tumors positive margins perineural or perivascular invasion or lymph node metastasesSquamous cell carcinomaSquamous cell carcinomasummary•The most common cancer is squamous cell carcinoma•It typically presents in a late stage of development •Surgery is the mainstay of therapy •The prognosis for 5-year survival is typically 50-70% •Because surgery is potentially disfiguring, these patients require careful operative planning consideration for dental function, psychological preparation, and social support  Wegener's GranulomatosisWegener's GranulomatosisWegener's Granulomatosis•Wegener's granulomatosis (WG) is now considered a relatively common disease of the upper airway There was a tendency for it to be overlooked in the past, but now its detection occurs much earlier and management is much better•named for Friedrich Wegener in 1939 first described necrotizing granulomas and vasculitis the upper and lower respiratory tract occurring either together or as separate components•It should be classified not only as a granulomatous disease but also viewed as a vasculitis and an autoimmune diseaseWegener's GranulomatosisWegener's Granulomatosis•several diseases : lethal midline granuloma WG lymphomas carcinomas destructive infectious disorders such as fungal infections and tuberculosisThis is a clinical term and should be abandoned•Specific identification is vital because the management strategies are different for each disorderClinical Aspects of Wegener's Clinical Aspects of Wegener's GranulomatosisGranulomatosis•Three main forms of WG: types 1, 2, and 3•Type 1 is the limited form of WG. Typically, patients with this form have with symptoms of an upper respiratory tract infection persisting for several weeks•unresponsive to antibiotics and associated with nasal drainage and pain. The pain is especially severe over the dorsum of the nose the expression of very large nasal crusts in both sides of the nose•There is no disease other than WG with such severe crusting•Some patients with the limited form have systemic vasculitis characterized by night sweats, migratory arthralgia, generalized weakness, and moderately profound malaise•Nasal examination shows diffuse crusting of the nose and nasopharynx bilaterally• When the crusts are removed, the mucosa is very friable. Septal perforations are less common because the disease is diagnosed earlier•Flexible or rigid endoscopic examination is invaluable in determining the extent of the intranasal lesions•Type 2 indicates a sicker patient with more systemic symptoms•Nasal involvement is similar to that of a patient with type 1 disease but other organs are also involved•Pulmonary involvement is typified by hemoptysis and the finding of cavitating lesions on chest radiographs•Type 3 is widely involvement of multiple organs including airway, pulmonary, renal, and sometimes cutaneous lesions•Cutaneous involvement is typified by tick-bite-like lesions of the lower limbs•Some patients have moderate-sized cutaneous ulcerations over the back or chest•Renal involvement in the early stage is typified by hematuria and abnormal urinary sediment leading to progressive renal failureDiagnosis of Wegener's Diagnosis of Wegener's GranulomatosisGranulomatosis•The typical history and characteristic findings on clinical evaluation and endoscopy, as well as the presence of mild to moderate anemia, are all suggestive of the clinical diagnosis of WG. •The importance of a careful biopsy cannot be overstressed•This tissue is sent for stains and culture, including those for acid-fast and fungal organismsTreatment of Wegener's Treatment of Wegener's GranulomatosisGranulomatosis•oral cyclophosphamide (2 mg/kg per day) •prednisone (1 mg/kg per day) for 1 month then tapered to alternate days throughout the following 2 months discontinued once a complete response is determined• Cyclophosphamide administration is continued for 6 months to 1 year After the disappearance of symptoms tapered gradually over several months•If relapse occurs, the standard protocol is reinitiatedPlasma exchange and intravenous Plasma exchange and intravenous immunoglobulinimmunoglobulin•.•Patients with generalized disease who do not respond to immunosuppressive therapy provide challenging cases• Intravenous immunoglobulin (IVIG) has been tried in several patients with some positive results•Plasma exchange has also been used, but its benefits seem to be limited to the dialysis-dependent patient populationSurgical reconstructionSurgical reconstruction•Contrary to some published statements, surgical intervention may be used to restore function when the disease is in remission• This includes tympanoplasty or correction of saddle nose deformity, as well as upper tracheal reconstruction Thank you。