The external nose consists of the nasal pyramid (frontal process of the maxilla) with the paired nasal bones forming the dorsum and meeting the frontal bone superiorly at the glabella. Inferiorly are the upper lateral cartilages and lower lateral (alar) cartilages, which contribute to the nasal tip and nasal valves (Fig. 3.1).
The internal nasal and sinus anatomy is complex and variable. The midline nasal septum is composed of the quadrangular cartilage, the perpendicular plate of the ethmoid bone, the vomer bone, and the palatine bone, with an overlying mucosal covering. There are four paired sinuses: the maxillary, frontal, ethmoid, and sphenoid (Fig. 3.2). The lateral nasal wall consists of the inferior, middle, and superior turbinates. The nasolacrimal duct opens in the inferior meatus. The frontal, anterior ethmoidal and maxillary sinuses drain via the middle meatus. The posterior ethmoids drain via the superior meatus. The sphenoid ostia are near the level of the superior meatus on the anterior wall of the sphenoid sinus. Clinically, the relation of the paranasal sinuses to adjacent anatomic structures is important as it relates to the potential for the spread of infection or an iatrogenic injury. Specifically, the ethmoid roof may be an extremely thin bone along the lateral lamella of the cribriform plate and may vary in its height considerably; intracranial contents lie superiorly. The lamina papyracea separates the orbit from the ethmoids; the orbital spread of infection is discussed in Chapter 3.1.2.
The sphenoid sinus is bounded by the internal carotid artery, optic nerves, and cavernous sinus and sella; an overriding posterior ethmoid (Onodi) cell may risk critical structures. Dehiscence of the bone covering the internal carotid within the sphenoid is relatively common and should be routinely assessed for on a preoperative computed tomography (CT) scan. The frontal sinus is bounded by the orbit and the anterior fossa, and also may be a source of spread of rhinogenic infection. A Haller cell is an anterior ethmoid cell that pneumatizes laterally at the orbital floor and can contribute to maxillary sinus drainage problems. Agar nasi cells are anterior ethmoid cells that pneumatize superiorly and can contribute to frontal sinus drainage problems.
There is abundant external and internal carotid supply (Fig. 3.3). Kiesselbach’s plexus (also known as Little’s area) of the anterior septum provides superficial anastomoses. External carotid branches supply the nose via the facial artery externally and the maxillary artery internally, including the spheno-palatine artery. Internal carotid branches are supplied via the ophthalmic artery to the anterior and posterior ethmoid arteries. Venous drainage occurs via facial veins as well as ophthalmic veins, which have valveless intracranial connections to the cavernous sinus and therefore relate to intracranial hematogenous spread of infection. Epistaxis is discussed in Chapter 3.1.5.
General sensory supply is via the first and second divisions of the trigeminal nerve. Importantly, the nasal tip is supplied via V1 (the first division if the trigeminal nerve). Thus, if possible herpetic lesions involve the nasal tip, ophthalmologic evaluation is indicated to rule out herpes zoster of the eye. Special sensory supply is via the olfactory (first cranial) nerve. Complex autonomic innervation is supplied to mucosa via the pterygopalatine ganglion regulating vasomotor tone and secretion. The vidian nerve contains preganglionic parasympathetic fibers from the greater superficial petrosal nerve (from cranial nerve [CN] VII) and sympathetic fibers from the deep petrosal nerve (from superior cervical ganglion) and synapses at the pterygopalatine ganglion prior to innervating the sinonasal mucosa.
Table 3.1 gives a summary of tests for the assessment of sinonasal physiology. Warming and humidification of inspired air, olfactory function, and immune function all are aspects of nasal physiology. The nose and paranasal sinuses play a role in host defenses. In general, mucociliary clearance is a key feature. Specific factors such as secretory immunoglobulin A (IgA), lactoferrin, lysozyme, cytokines, and the complex regulation of cells that mediate immunity are critical to the maintenance of normal sinus function. A detailed discussion of sinonasal physiology is beyond the scope of this book. The presence of infection, inflammation, allergy, neoplasm, or traumatic, iatrogenic, or congenital deformity may all perturb sinonasal physiology and must be considered in the evaluation of the patient with complaints related to the nose.
Utility (Tests for)
Cultures of sinus drainage
Specific infectious organism
University of Pennsylvania Smell Identification Test (SIT)
RAST (blood testing for allergen-specific IgE)
Skin testing (prick test; SET)
Nasal obstruction; not currently widely used outside of research settings
Ciliary beat frequency
Ciliary electron microscopy
Assess ciliary function and anatomy, i.e., for Kartagener syndrome. Typically tested on adenoid or mucosal biopsy.
Abbreviations: ACE, angiotensin-converting enzyme; ANA, antinuclear antibody test; c-ANCA, antineutrophil cytoplasmic antibody; ESR, erythrocyte sedimentation rate; FTA-ABS, fluorescent treponemal antibody absorbed; IgE, immunoglobulin E; RAST, radioallergosorbent test; SET, skin endpoint titration.
Bolger WE. Anatomy of the paranasal sinuses. In: Kennedy DW, Bolger WE, Zinreich SJ, eds. Diseases of the Sinuses: Diagnosis and Management. Hamilton, ON: BC Decker; 2001:1–12
Lang J. Clinical Anatomy of the Nose, Nasal Cavity, and Paranasal Sinuses. Stuttgart/New York: Thieme; 1989
Mehta D, Ralph WM. Surgical anatomy of the nose and paranasal sinuses. In: Van de Water TR, Staecker H, eds. Otolaryngology: Basic Science and Clinical Review. Stuttgart/New York: Thieme; 2006:455–471
Stierna PLE. Physiology, mucociliary clearance, and neural control. In: Kennedy DW, Bolger WE, Zinreich SJ, eds. Diseases of the Sinuses: Diagnosis and Management. Hamilton, ON: BC Decker; 2001:35–45
Voigt EP, Edelstein DR. Nasal and paranasal sinus physiology. In: Van de Water TR, Staecker H, eds. Otolaryngology: Basic Science and Clinical Review. Stuttgart/New York: Thieme; 2006:472–484
• A rapidly progressive sinonasal fungal infection can be fatal.
• Acute invasive fungal infections occur almost exclusively in immunocompromised or debilitated patients,
• Successful treatment requires early detection, wide surgical débridement, and correction of the underlying predisposing condition.
In the debilitated patient, certain fungal infections can become angioinvasive with tissue necrosis, cranial nerve involvement, and possible orbital or intracranial extension. The most common organisms are Mucor or Aspergillus species. High-risk patients include those with neutropenia from any cause (e.g., leukemia, bone marrow transplantation), other oncology patients undergoing chemotherapy, chronic immunosuppressive therapy or corticosteroid use, diabetes mellitus and diabetic ketoacidosis, or acquired immuno-deficiency syndrome (AIDS). Acute invasive fungal rhinosinusitis is a distinct and rapidly aggressive disease process that is distinguished by its fulminant course from other forms of fungal sinusitis, such as mycetoma, allergic fungal rhinosinusitis, or chronic invasive (indolent) fungal rhinosinusitis.
Also known as rhinocerebral mucormycosis, acute invasive fungal rhinosinusitis occurs in the at-risk populations described above: patients with hematologic malignancies, patients status post solid organ or bone marrow transplant, diabetics, those on chronic steroid therapy, neutropenic patients, and patients with AIDS.
Signs and Symptoms
A high index of suspicion in any at-risk patient is required, as early diagnosis improves prognosis. A fever of unknown origin should raise suspicion, as should any new sign or symptom of sinonasal disease. Facial edema, periorbital swelling, pain, or numbness are common findings. However, the leukopenic patient may be unable to mount a febrile response. Other findings may include epistaxis, headache, mental status change, or crusting/eschar at the naris that can be mistaken for dried blood. One should consider unilateral cranial neuropathy, acute visual change, or altered ocular motility in an immunocompromised patient to be acute invasive fungal rhinosinusitis until proven otherwise. A black intranasal eschar on exam is considered pathognomonic for invasive Mucor.
A noninvasive sinonasal infection, such as acute bacterial sinusitis, should be considered. An acute bacterial sinusitis complication, such as orbital cellulitis or intracranial suppurative spread may present similarly. Radiographically similar processes may include squamous cell carcinoma, sinonasal lymphoma, and Wegener granulomatosis.
See Fig. 3.4 for a diagnostic and treatment algorithm.
The patient suspected to have acute invasive fungal rhinosinusitis should be seen without delay. The head and neck examination should focus on cranial nerve function and should include nasal endoscopy. Avoid tetracaine spray or other topical anesthetics. Insensate mucosa noted during an endoscopic exam is consistent with invasive fungal infection. Dark ulcers or pale, insensate mucosa may appear on the septum, turbinates, palate, or nasopharynx. Early infection may appear as pale mucosa; the presence of dark eschar has been considered to be pathognomonic. Signs of cavernous sinus thrombosis include ophthalmoplegia, exophthalmos, and decreased papillary responses.
Biopsy of suspicious areas such as the middle turbinate or septal mucosa is required for diagnosis. It is important to obtain actual tissue at biopsy, not just overlying eschar or necrotic debris. These specimens should be sent fresh for immediate frozen section analysis as well as silver stain. Patients may be thrombocytopenic, and although a low platelet count may lead to profuse bleeding after biopsy, the risk of this must be balanced with the high mortality associated with a delay in diagnosis. If necessary, platelet transfusion should be ordered early. As a rule, a platelet count of 50,000 is desired. Acceptable hemostasis can usually be obtained with chemical cautery and Avitene (Davol, Inc., Cranston, RI), Gelfoam (Pfizer Pharmaceuticals, New York, NY), or other hemostatic packing.
CT findings may be nonspecific. However, the presence of bone erosion and adjacent soft tissue edema on contrast-enhanced maxillofacial CT strongly suggests the diagnosis if clinical correlation is present. Unilateral edema of the nasal mucosa has also been associated with invasive fungal sinusitis, as well as obliteration of the retroantral fat planes. Both soft tissue and bone windows, as well as high-resolution axial and coronal views are necessary. Magnetic resonance imaging (MRI) is useful to evaluate intracranial extension and extension beyond the paranasal sinuses.
Cultures are inadequate and play no role in the initial diagnosis and management of suspected acute invasive fungal rhinosinusitis. Positive culture results will most likely be available late in the course of the disease. Useful labs to assess risk factors include a complete blood count (CBC), absolute neutrophil count, chemistries, blood glucose and hemoglobin A1C (HbA1c) in the diabetic, and human immunodeficiency virus (HIV) testing with CD4 lymphocyte counts and viral load in AIDS patients.
Biopsy of the middle turbinate or other suspicious lesions with immediate frozen section analysis is the gold standard test to confirm the presence of tissue-invasive fungus. Mucor is identifiable within the mucosa as large, irregularly shaped nonseptate hyphae that branch at right angles. Aspergillus is identifiable as smaller hyphae that are septate and branch at 45-degree angles. Methenamine silver stain is performed to confirm the diagnosis; however, these results may not be available for several hours.
This is a surgical emergency: complete surgical resection and the reversal of underlying immune dysfunction are critical. The diabetic patient can be successfully treated with early diagnosis, insulin drip, and wide surgical resection. In the oncology patient, if neutropenia cannot be reversed, mortality is high. Granulocyte-macrophage colony-stimulating factor (GM-CSF) may improve survival. Surgical goal is resection of all involved tissue. This may be accomplished endoscopically in select cases. However, an extended total maxillectomy with orbital exenteration may be necessary in advanced disease. Systemic antifungals as well as intranasal nebulized amphotericin are administered, but should be considered adjuvant therapy.
Outcome and Follow-Up
Prognosis is very poor with intracranial involvement. A bone marrow transplant patient with uncorrectable neutropenia has a poor prognosis. Overall survival in diabetic patients may approach 80% if ketoacidosis is corrected.
Zygomycosis (phycomycosis or mucormycosis)
Dhong H, Lanza DC. Fungal rhinosinusitis. In: Kennedy DW, Bolger WE, Zinreich SJ, Eds. Diseases of the Sinuses: Diagnosis and Management. Hamilton, ON: BC Decker; 2001:187–193
Gillespie MB, O’Malley BW. An algorithmic approach to the diagnosis and management of invasive fungal rhinosinusitis in the immunocompromised patient. Otolaryngol Clin North Am 2000;33(2):323–334
• Sinusitis is a common cause of orbital infection.
• Significant morbidity and even mortality can result.
• Orbital extension of sinusitis is most common in pediatric patients.
• Combined otolaryngology and ophthalmology care is required.
Orbital extension of sinonasal disease requires immediate attention, as rapid progression and blindness may occur. Anatomically, the orbit is bounded by all paranasal sinuses and infection may spread to the orbit directly or via retrograde thrombophlebitis. The Chandler classification system is heuristically useful in staging and managing orbital complications of sinusitis (Table 3.2). Hospital admission and intravenous antibiotic therapy are required for treatment; surgical drainage is necessary for abscess formation, vision compromise, or lack of improvement with medical therapy.
Cavernous sinus septic thrombosis
Orbital complications occur in ~3% of sinusitis cases. This is most common in children but can occur at any age. Subperiosteal abscess is present in ~20% of cases of orbital extension of sinusitis. Cavernous sinus thrombosis is rare. Immunosuppressed patients are at increased risk and require aggressive treatment.
Signs and Symptoms
The most common findings are orbital edema, pain, proptosis, and fever. Orbital disease may be the first sign of sinusitis in children. In more advanced cases, there may be gaze restriction and visual acuity change.
In the pediatric age group, orbital pseudotumor consists of painful proptosis without a fever or leukocytosis. Orbital rhabdomyosarcoma may present with inflammatory changes in 25% of patients. An ethmoid mucocele may present with proptosis, and CT will reveal an expanded sinus in this instance. Other sinonasal causes of proptosis or orbital edema include allergic fungal rhinosinusitis and neoplasm, as well as iatrogenic injury. Abnormal thyroid state may cause ophthalmopathy.
Examination requires the combined input of the otolaryngologist and the ophthalmologist. In general, the patient will have a history of preceding sinusitis or current complaints consistent with acute sinusitis. Fever is usually present. Head and neck exam reveals lid or periorbital edema, erythema, and tenderness. In cases of preseptal (periorbital) cellulitis, the remainder of the eye exam is normal. The presence of proptosis, chemosis extraocular muscle limitation, diplopia, or decreased visual acuity suggests orbital cellulitis or subperiosteal abscess. With cavernous thrombosis or intracranial extension, findings may include a frozen globe (ophthalmoplegia), papilledema, blindness, meningeal signs, or neurologic deficits secondary to brain abscess or cerebritis. Superior orbital fissure syndrome is a symptom complex consisting of retroorbital pain, paralysis of extraocular muscles, and impairment of first trigeminal branches. This is most often a result of trauma involving fracture at the superior orbital fissure, but dysfunction of these structures can arise secondary to compression. Orbital apex syndrome adds involvement of the optic nerve.
Contrast-enhanced CT scanning with coronal and axial views is the imaging study of choice if there is any suspicion of postseptal involvement (i.e., other than simple periorbital cellulitis). A subperiosteal abscess is identifiable as a lentiform, rim-enhancing hypodense collection in the medial orbit with adjacent sinusitis. The medial rectus is displaced. In the absence of abscess formation, there may be orbital fat stranding, solid enhancing phlegmon, or swollen and enhancing extraocular muscles, consistent with orbital cellulitis. A lid abscess may be present less commonly. Suspicion of cavernous sinus thrombosis is better evaluated with MRI.
A CBC with differential may be useful. Preoperative labs should be ordered as indicated.
In younger children, microbiology is often single aerobes including alpha Streptococcus, Haemophilus influenzae, or coagulase-positive Staphylococcus. In those over 10 years old, organisms are often mixed and may include anaerobes.
All patients should be admitted and treated with serial ophthalmologic exams and intravenous (IV) antibiotics that have good cerebrospinal fluid (CSF) penetration. Generally, a third-generation cephalosporin is used. Oxacillin is often used in children. Older patients are often double-covered with clindamycin for anaerobes. Alternatives include ampicillin/sulbactam, vancomycin, or aztreonam. Antibiotics are adjusted according to cultures, if possible. Systemic steroids are not recommended. Topical nasal vasoconstrictors are useful (i.e., oxymetazoline).
A majority of patients with orbital complications require surgery. This is an area of some controversy. Clearly, surgical drainage is required urgently for abscess formation or decreased visual acuity. If the clinical setting allows for close follow-up (i.e., frequent serial ophthalmologic assessment), many clinicians will observe certain cases of preseptal or early postseptal cellulitis. If there is any progression or lack of resolution with medical therapy over 48 hours, surgery is recommended. Surgical drainage may be accomplished endoscopically by experienced surgeons; however, consent for an external ethmoidectomy approach is recommended. Regardless of approach, the abscess should be drained and the underlying sinus disease should be addressed. For cavernous thrombosis, involved sinuses including the sphenoid must be drained; systemic anticoagulation remains controversial.
Outcome and Follow-Up
The natural history of untreated disease (all stages) results in blindness in at least 10%. Most cases of Chandler stage I–IV disease recover well with treatment. There remains up to an 80% mortality rate with cavernous sinus involvement, although new literature reports suggest this figure is high.
Phlebitis and thrombophlebitis of intracranial venous sinuses
Chandler JR, Langenbrunner DJ, Stevens ER. The pathogenesis of orbital complications in acute sinusitis. Laryngoscope 1970;80(9):1414–1428
Choi SS, Grundfast KM. Complications in sinus disease. In: Kennedy DW, Bolger WE, Zinreich SJ, eds. Diseases of the Sinuses: Diagnosis and Management. Hamilton, ON: BC Decker; 2001:169–172
• Intracranial complications due to sinusitis are a life-threatening emergency.
• Management requires a multidisciplinary approach including neurosurgical consultation.
• Complications include meningitis, dural sinus thrombosis, and intracranial abscess.
Sinusitis may result in spread of infection intracranially. Extension may occur via osteomyelitic bone, trauma, or via venous channels. The frontal sinus is commonly the source, although ethmoid or sphenoid sinusitis can lead to intracranial spread. Complications include meningitis, epidural abscess, subdural abscess, parenchymal brain abscess, and cavernous sinus thrombophlebitis.
This is uncommon. Since the advent of antibiotics, the incidence has decreased dramatically. Currently, probably less than 1% of sinusitus cases are complicated by spread of infection.
Signs and Symptoms
The patient with meningitis of a rhinologic origin will manifest signs and symptoms typical of bacterial meningitis. These include high fever, photophobia, nausea and emesis, mental status change, and nuchal rigidity, pulse and blood pressure changes. However, epidural or subdural abscess without meningitis may be more subtle. Fever and headache may be present but are nonspecific. Usually focal signs are absent. Meningeal signs may develop gradually. A parenchymal brain abscess of rhinologic origin (frontal lobe abscess) may initially result in few signs or symptoms. However, this may progress from headache to signs of increased intracranial pressure, vomiting, papilledema, confusion, somnolence, bradycardia, and coma. Cavernous sinus thrombophlebitis results in spiking fevers, chills, proptosis, chemosis, decreased visual acuity and blindness, and extraocular muscle paresis. Infection can rapidly spread to the contralateral cavernous sinus via venous communications.
Meningitis of nonrhinologic origin must be considered. The incidental finding of paranasal sinus disease on imaging does not necessarily signify a causal relationship.
Pathogenesis may involve several mechanisms. Extension may occur via osteomyelitic bone. Also, traumatic bone disruption may allow communication of infected sinus contents with dura, for example, after posterior table fracture of the frontal sinus. Also, infection may propagate via venous channels in bone or retrograde venous circulation to the cavernous sinus. General hematogenous spread is possible, especially in a severely immuno-compromised host.
Complete head and neck exam is required with careful assessment of all cranial nerves. Nasal endoscopy may reveal active sinonasal disease and provide mucopus for culture and sensitivities. A neurologic exam including orientation to person, place, and date will reveal any focal deficits and serve as a useful baseline to monitor for any deterioration. The presence of ocular findings or neurologic deficit should prompt ophthalmologic and neurosurgical consultations.
Both CT and MRI are usually obtained. Contrast-enhanced CT scanning will provide detail regarding paranasal sinus disease and will reveal evidence of bony dehiscence that may underlie intracranial spread of infection. Intravenous contrast will reveal an enhancing abscess. Gadolinium-enhanced MRI will provide a more sensitive assessment of dural enhancement and intracranial disease but lacks the bony detail necessary for planning sinus surgery.
Obtain CSF via lumbar puncture (LP) in the patient with possible meningitis, after obtaining a CT scan. There is a risk of acute tonsillar herniation following LP, almost always seen in patients with a noninfectious intracranial process causing a mass effect with localizing signs and papilledema. See Table 3.3 for interpretation of CSF values. Cell count (tube 3), protein and glucose (tube 2), and Gram stain with culture and sensitivities (tube 1) are ordered. Cultures with sensitivity will guide antibiotic therapy.
In addition to CSF studies, other useful labs include blood cultures, CBC with differential, chemistries, and prothrombin time (PT), and partial thromboplastin time (PTT).
IV high-dose antibiotics are empirically started following LP, and adjusted based on cultures. Broad-spectrum agents with good CSF penetration are utilized, such as ceftriaxone or cefepime. Vancomycin is often added for double coverage and for possible methicillin-resistance. Peak and trough levels must be monitored with vancomycin. The role for anticoagulation for cavernous thrombophlebitis remains controversial. Anticoagulation may interfere with the ability to perform intracranial pressure monitoring or craniotomy.
The underlying sinus infection is drained either by endoscopic or open approach. If an intracranial abscess is present, this is drained by the neuro-surgeon in conjunction with sinus drainage.
Outcome and Follow-Up
Close observation in an intensive care unit (ICU) setting with serial neurologic exams is required postoperatively. Intracranial pressure monitoring may be required.
Meningitis due to unspecified bacterium
Phlebitis and thrombophlebitis of intracranial venous sinuses
Acute sinusitis, unspecified
Greenberg MS. Handbook of Neurosurgery. 7th ed. Stuttgart/New York: Thieme; 2010
• CSF rhinorrhea may occur due to iatrogenic injury to the ethmoid roof during sinus surgery.
• CT and MRI, β-2 transferrin assay, radioactive pledget scan, or intrathecal fluorescein with endoscopy can be useful for evaluation.
• The skull base can be repaired with endoscopic instruments from below, or via neurosurgical approach from above.
Anterior skull base disruption can result in drainage of CSF via the nose. The underlying problem may be the result of trauma, iatrogenic injury, or congenital anomaly, or it may arise spontaneously with no obvious specific cause. The management of CSF rhinorrhea related to skull base injury from endoscopic sinus surgery is highlighted. CSF leak from the temporal bone may present with rhinorrhea via the middle ear and eustachian tube.
This is uncommon. Although the exact incidence of complications is unclear currently, previous estimates suggest a ~0.5% rate of skull base injury with CSF leak following ethmoid surgery.
Signs and Symptoms
Patients present with watery rhinorrhea. Often, the drainage can be provoked by leaning the patient forward with the head lowered. Typically, the drainage has a salty or metallic taste. Headache may be reported. Anosmia and nasal congestion may accompany iatrogenic skull base injury with encephalocele.
The consideration for watery or clear rhinorrhea includes various forms of rhinitis versus CSF drainage. Vasomotor rhinitis typically is elicited by cold temperatures, physical activity, or other specific stimuli.
The approach to the patient typically begins with a thorough and complete history. A source of skull base damage may, of course, be quite obvious if there was a suspected iatrogenic injury during preceding endoscopic ethmoid surgery. One should note if the drainage is unilateral as well as its duration and severity. Associated complaints such as headache, visual disturbance, epistaxis, and anosmia should be noted. Details of any previous sinonasal surgery, neurosurgery, otologic surgery, or trauma are important. Any previous imaging studies of the head should be obtained, if possible. Previous history of meningitis should be sought. General past history, medication list, social and family history are obtained.
A complete head and neck exam is performed. CNs I to XII should be tested. A standard test for olfactory function such as the Smell Identification Test (SIT; Sensonics, Inc., Haddon Heights, NJ) is recommended, to document olfactory function prior to treatment. Nasal endoscopy is performed. A 4-mm rigid 30-degree endoscope is ideal. Drainage, masses, edema, purulence, and prior surgical changes are noted. If the injury is iatrogenic, it may be possible to assess the location and size of the skull base defect. Be suspicious of any mass arising medial to the middle turbinate, as encephalocele or esthesioneuroblastoma can arise in this location. Having the patient perform the Valsalva maneuver may result in visible enlargement of a meningocele or encephalocele.
Identification of the site of leak may be straightforward or may be difficult. For a known iatrogenic injury, thin-cut axial CT with coronal views will typically verify the site of injury along the ethmoid roof. This scan should be obtained using an image-guidance protocol so that computer-assisted surgical navigation can be used for endoscopic repair. For other situations, CT is useful to study the bony detail of the anterior skull base and sphenoids. A bony dehiscence may be evident. For a patient presenting with a soft tissue mass, MRI is the study of choice to assess for possible meningocele or encephalocele.
If rhinorrhea fluid can be collected, this should be sent for β-2 transferrin assay. Usually, at least 1 mL is required; the laboratory may require refrigeration or rapid handling of the specimen. The presence of β-2 transferrin suggests strongly that the fluid is indeed CSF.
See Table 3.4 for a summary of available studies.
Radioactive Pledget Scanning
This study can be done to help confirm and localize a leak site. Small cotton-oid 1 × 3 neuropledgets can be trimmed and placed within the nasal cavity in defined locations. Usually, two pledgets are placed per nostril, one ante-riorly and one posteriorly, with the string secured to the skin and labeled. Radioactive tracer is then injected intrathecally, typically technetium-99 (99mTc). After suitable time, the pledgets are removed and assayed for radioactivity counts. If there is an active CSF leak, the location of a “hot” pledget can guide exploration and repair.
β-2 transferrin assay
Confirm that drainage is CSF
Radioactive pledget scanning
Confirm presence of CSF leak, provide localizing information
Localize site of leak during nasal endoscopy/endoscopic repair
Assess bony detail of anterior skull base; assess for intracranial hematoma or pneumocephalus following iatrogenic injury
Assess for anterior skull base encephalocele, meningocele, neoplasm
Abbreviations: CT, computed tomography; MRI, magnetic resonance imaging.
The use of a dye to visualize endoscopically the CSF leakage is extremely helpful, both for endoscopic diagnosis and at the time of repair. For the procedure, a lumbar drain is placed and 10 mL of the patient’s CSF is collected, after which 0.1 mL of 10% IV fluorescein is diluted into the 10 mL CSF sample. This is reinjected intrathecally slowly over 5 minutes. The fluorescein causes active CSF leakage to appear greenish endoscopically. Intrathecal fluorescein can cause seizures at higher dosage; however, the protocol described here is widely accepted as safe.
Skull base disruption causing CSF rhinorrhea is managed with surgical repair. Repair of acute iatrogenic injury is discussed separately from other cases.
Repair of Acute Iatrogenic Injury
If the ethmoid roof is injured during sinus surgery, it may be possible to repair the injury. If there is extensive injury, severe bleeding, or obvious intradural injury, it is highly recommended that neurosurgical consultation be obtained, if possible. Concomitant injury to the anterior ethmoid artery can occur, so the orbit should be assessed for lid edema, ecchymosis, and proptosis. If this occurs, lateral canthotomy, IV mannitol, and IV steroids may be required. To repair the skull base defect, good visualization is required. Mucosa adjacent to the leak site is gently reflected away. If possible, a bone graft placed intracranially is used. This is recommended if the defect is larger than ~0.5 cm. For this graft, a portion of turbinate bone is ideal. After placing the bone on the intracranial side of the defect, fibrin glue (or similar material) and fascia or other soft tissue is layered on the nasal side of the defect, followed by several layers of absorbable packing material such as Gelfoam. It is helpful if the patient can emerge from anesthesia smoothly, without “bucking” and straining, and without the need for high-pressure bag-mask ventilation following extubation, to minimize chances of causing pneumocephalus. Postoperatively, once stable, a head CT is recommended, to evaluate for intracranial hematoma or pneumocephalus. If the scan reveals hematoma or significant pneumocephalus, neurosurgical consultation is necessary. Otherwise, postoperative management should include head of bed elevation, bed rest for 2 to 3 days, and stool softeners. Most surgeons recommend antibiotic prophylaxis with Ancef (GlaxoSmithKline, Brentford, Middlesex, UK) or clindamycin. The use of a lumbar drain is debated. For a large defect, lumbar drainage to reduce CSF pressure for 3 days can be helpful.
Elective Repair of Other Anterior Skull Base CSF Leaks
The management is individualized. Depending on surgeon experience, many ethmoid or sphenoid sinus leaks can be approached endoscopically from below. Intrathecal fluorescein used intraoperatively is very helpful; the lumbar drain may be used postoperatively to reduce CSF pressure. In other cases, neurosurgical colleagues may approach the skull base defect from above; a pericranial flap can be used to close the defect.
Outcome and Follow-Up
Repair of anterior skull base CSF leaks has a good success rate. Complications can include repeat leakage, infection including meningitis or abscess, encephalocele, anosmia, postoperative intracranial bleeding, or pneumocephalus.
Cerebrospinal fluid rhinorrhea
Bolger WE, Kennedy DW. Surgical complications and postoperative care. In: Kennedy DW, Bolger WE, Zinreich SJ, eds. Diseases of the Sinuses: Diagnosis and Management. Hamilton, ON: BC Decker; 2001
• Management, as for other emergency situations, should begin with ABCs (airway, breathing, circulation) and follow standard protocols for resuscitation and treatment.
• Address predisposing conditions.
• Accurate localizing of bleeding site is required for treatment.
The word “epistaxis” derives from the Greek epi, meaning on, and stazo, to fall in drops. A nosebleed may present as anterior (bleeding from the nostril), posterior (blood present in the posterior pharynx), or both. Accurate localization of the bleeding site is the key to treatment. It is estimated that >90% of epistaxis cases arise from the anterior nasal septum at Kiesselbach’s plexus, also known as Little’s area (Fig. 3.3). However, the nasal blood supply involves both the internal and external carotid systems and brisk bleeding can arise posteriorly. Major vessels include anterior ethmoid, posterior ethmoid, sphenopalatine, greater palatine, and superior labial arteries.
Most nosebleeds are self-limited, not requiring medical intervention. An estimated 45 million Americans suffer from at least minor epistaxis. In children nearly all cases are anterior, often due to digital trauma. Over age 40, the incidence of posterior bleeds rises.
Signs and Symptoms
Patients will report bleeding from the nares or the mouth. There may be an obvious antecedent nasal trauma, surgery, or foreign body reported. Hematemesis is common. On exam, blood may be fresh or clotted.
The existence of epistaxis is established on history and exam. A variety of underlying local and systemic conditions should be considered (Table 3.5). Most important to exclude is a neoplasm presenting with epistaxis. Consider juvenile nasopharyngeal angiofibroma in any teenage male with a unilateral sinonasal mass and epistaxis.
juvenile nasopharyngeal angiofibroma
Squamous cell carcinoma
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu)
Coumadin, aspirin, Plavix, etc.
Intranasal illicit drug use
Tuberculosis, syphilis, rhinoscleroma, viral
In most cases, the approach to the patient begins with a thorough history. The exception to this rule is the patient with severe active bleeding or hemodynamic instability that must first be corrected. Important historical information should include how long nosebleeds have been occurring, their frequency, whether bleeding is typically left or right-sided and anterior or posterior, how long nosebleeds last, and whether packing or cauterization has ever been required. Note any previous nasal or sinus surgeries. If recent sinonasal surgery has occurred, obtaining operative notes may be helpful. General information that is relevant includes a prior history of easy bruising or bleeding; a family history of such problems or known bleeding disorder; bleeding problems with previous surgeries or dental work; history of anemia, malignancy, leukemia, lymphoma, or chemotherapy; other systemic illnesses; or recent trauma. A complete accurate medication list is needed. Specific attention is directed at any recent use of medications that can promote bleeding, such as aspirin, other nonsteroidal antiinflammatory drugs (NSAIDs), coumadin, or Plavix (Bristol-Myers Squibb, New York, NY). Also consider vitamins such as vitamin E, and other supplements or herbs, many of which can promote bleeding. The social history is obtained. Chronic alcohol abuse can be related to coagulation disorders from impaired liver synthetic function as well as malnutrition and vitamin deficiencies; illicit intranasal drug use may be causative. In summary, awareness and treatment of systemic conditions may be required to obtain definitive effective management of epistaxis.
Priorities with any severe epistaxis must focus on the ABCs and on resuscitation. The key to effective treatment is localization of the bleeding source. The head and neck exam should therefore focus on this goal. It is essential to have a nasal speculum and headlight, Frazier tip suctions, and oxymetazoline (Afrin; Schering-Plough Healthcare Products Inc., Memphis, TN) or Neo-Synephrine (Bayer Consumer Health, Morristown, NJ) spray (for decongestion and hemostasis). Topical 2% pontocaine can be used for intranasal anesthetic, if needed. If topical 4% cocaine is available, this is a very effective decongestant and anesthetic, used sparingly. The availability of a 4-mm rigid nasal telescope (i.e., Karl Storz 7200B; Karl Storz, GmbH, Tuttlingen, Germany) and light source is ideal, especially to localize a posterior bleed. Often, the otolaryngologist will need to first remove improperly placed or ineffective packing materials. Removal of clots with suction will facilitate identification of bleeding sites, using the equipment described above.
Profuse bleeding in a maxillofacial trauma patient should be managed with emergent angiography. An injury to the extradural portion of the internal carotid cannot be controlled with nasal packs; most of these injuries probably exsanguinate in the field. Also, in recurrent or difficult-to-treat epistaxis, angiography may be diagnostic (to localize a bleeding source and guide definitive therapy such as ethmoid ligation) or therapeutic (to embolize an external carotid source).
Identification of an intranasal mass on exam should prompt CT or MRI scanning to fully evaluate a possible sinonasal neoplasm or inflammatory disease prior to definitive therapy. However, this should not delay acute treatment of active epistaxis.
Blood work should include a hemoglobin, hematocrit, platelet count, PT and PTT, as well as blood type and screening tests. Bear in mind that hemo-concentration in the underresuscitated patient may yield laboratory results that do not initially reflect the degree of blood loss. Platelet function studies may be helpful, although results may not be rapidly available. Platelet dysfunction is seen with NSAID use and von Willebrand disease.
A patient presenting to the emergency department with severe epistaxis or any suspicion of hypovolemia should have IV access, continuous cardiac monitoring, and aggressive fluid resuscitation.
Correction of severe clotting disturbances will facilitate the efficacy of local therapy for epistaxis (i.e., reversal of overcoumadinization with fresh frozen plasma [FFP] or IV vitamin K; platelet transfusion in the thrombocytopenic oncology patient). Spontaneous mucosal bleeding typically occurs in patients with platelet counts <20,000. Control of hypertension is essential. Patients with underlying renal failure may need DDAVP (desamino- D-arginine vasopressin) to correct coagulopathy from underlying uremic platelet dysfunction. Some forms of von Willebrand disease also respond to DDAVP or desmopressin.
Localize the source, as described above, using oxymetazoline spray, suction, a nasal speculum, and/or nasal endoscopy. Avoid removable packing unless other methods are ineffective. Most bleeds are anterior and can be treated effectively with topical vasoconstrictors and direct pressure and/or simple silver nitrate cautery sticks. A small piece of Gelfoam or Surgicel (Johnson & Johnson, New Brunswick, NJ) placed over the cauterized area is helpful. If bleeding will not respond to this, nasal packing to place pressure over the bleeding site may be required. Gelfoam, Surgicel, with or without topical thrombin, or Floseal are among the absorbable products available that will not require removal but will dissolve with nasal saline administration. Intranasal salt pork is a highly effective packing material.
For refractory bleeding, removable packing is placed. Many types are available, i.e., Merocel (Medtronic XOMED, Inc., Jacksonville, FL) sponges. Rapid Rhino (Brussels, Belgium) products, which are covered with a procoagulant and contain an inflatable balloon, are also effective. Experience has shown that an effective removable pack should be left in place for 4 days to enable healing prior to removal. Any patient with packing in the nose should be placed on systemic antibiotics with good gram-positive coverage, such as cephalexin or clindamycin, for prophylaxis against toxic shock. In the rare situation where properly placed nasal packing results in ongoing hemorrhage via the posterior nares, an Epistat (Medtronic XOMED, Inc., Jacksonville, FL) balloon pack is very effective. This device has a balloon that is inflated in the nasopharynx and a second balloon that provides pressure intranasally, and effectively tamponades a posterior bleeding vessel.
Other formal posterior packing techniques and devices are available as well. Any patient with a posterior pack requires hospital admission with continuous pulse oximetry. A patient failing or rebleeding following these maneuvers is usually sent for angiography and possible embolization. If unavailable, and/or the surgeon is confident that the feeding vessel is identifiable, clipping or cauterization of the sphenopalatine can be performed endoscopically; ligation of the internal maxillary system can be performed via a Caldwell-Luc approach and takedown of the posterior wall of the maxillary sinus; or anterior or posterior ethmoid artery ligation can be performed via an external incision. Recall the 24-12-6 mm rule to locate the anterior ethmoid, posterior ethmoid, and optic nerve along the orbital wall; however, there is substantial variation with these measurements.
Therapy for the Osler-Weber-Rendu patient is a challenge. One should rule out pulmonary or intracranial vascular malformations, which may be life-threatening, with appropriate imaging. Avoid packing. Laser [Nd:YAG (neodymium:yttrium-aluminum-garnet), Argon, KTP (potassium titanyl phosphate)] treatments at 1024 nm have been effective. Septodermoplasty remains an option in severe cases.
Outcome and Follow-Up
Ongoing management of underlying medical disorders may be preventive. Moisture with nasal saline and humidification is helpful. Avoid NSAIDs.
Cullen MM, Leopold DA. Nasal emergencies. In: Eisele DW, McQuone SJ, eds. Emergencies of the Head and Neck. St Louis, MO: Mosby; 2000:239–245
Wormald PJ. Endoscopic Sinus Surgery: Anatomy, Three-Dimensional Reconstruction, and Surgical Technique. 2nd ed. Stuttgart/New York: Thieme;2008
• Rhinosinusitis is an inflammatory condition of the nose and sinuses.
• By definition, signs and symptoms last less than one month.
• The condition resolves with treatment; inadequate treatment may lead to disabling chronic disease.