Trombosi della vena succlavia

Objective: To discuss the clinical aspects and management of internal jugular vein thrombosis associated with acute otitis media. Study Design: Case reports and review of the literature. Setting: University hospital, tertiary referral center.

Patient: The authors describe two cases of internal jugular vein thrombosis, without sigmoid sinus thrombosis, secondary to acute otomastoiditis.

Intervention: Jugular vein thrombosis was diagnosed in both cases by observation of filling defects of the involved jugular bulb on contrast-enhanced computed tomography and con­firmed by conventional magnetic resonance and magnetic res­onance venography.

Results: Both patients recovered after recanalization of the vessel concomitant to anticoagulation and antibiotic treatment associated with a simple mastoidectomy.

Conclusion: Internal jugular vein thrombosis may be a compli­cation of acute otitis media, without involvement of the sigmoid sinus and with a starting point in the jugular bulb. Anticoagula­tion associated with antibiotic therapy can be considered a safe and effective treatment. Surgery should only be performed to eliminate the source of infection from the middle ear and mas­toid. Key Words: Acute otitis media—Vein thrombosis.

Otol Neurotol 27:937– 944, 2006.

A well-known vascular complication of otitis media and/or mastoiditis is septic thrombosis of the sigmoid portion of the lateral venous sinus. Although it has be­come rare with the advent of antibiotics, it can still be fatal especially when associated with other intracranial complications (1,2). The thrombotic process can extend backwards to the superior sagittal sinus interfering with cerebrospinal fluid resorption by blockage of the arach­noid granulations, producing an otogenic hydrocephalus (3). It can extend through the petrosal sinuses to the cav­ernous sinus (4–6), the internal jugular and subclavian veins (7), and eventually to the superior vena cava (8). Starting from the jugular vein, bacteremia with lung embolization can develop into a clinical picture resem­bling Lemierre syndrome (9,10). A carotid artery spasm or arteritis (11) such as a carotid sheath abscess (12) can also be the consequence of an adjacent thrombophlebitis.

The observation of two unusual cases of jugular bulb thrombosis extending to the jugular vein but not the sigmoid sinus during acute otitis media prompted us to analyze this uncommon behavior and to focus on this

Address correspondence and reprint requests to Luca Oscar Redaelli de Zinis, M.D., Department of Otorhinolaryngology, University of Brescia, Piazzale Spedali Civili 1, 25123 Brescia, Italy; E-mail: redaelli@med.unibs.it

possible complication of simple acute otitis media with potentially severe general consequences.

CASE 1

A five-year-old boy was admitted after one-week treatment with nonsteroid anti-inflammatory drugs for left acute otitis media with worsening otalgia and fever (39.6°C) during the previous 48 hours. Clinical examination showed a thickened and bulging pars tensa with a reddish pars flaccida. Palpation of the mas­toid tip was painful. Antibiotic treatment with ceftazi­dime, 750 mg three times daily, was started. A blood cell count showed moderate neutrophilic granulocytosis, whereas Epstein-Barr viral tests were negative. The fol­lowing day, torticollis was evident followed by an epi­sode of vomiting. A cervical swelling appeared one day later, and a computed tomography (CT) of the temporal bone and the neck was performed (Fig. 1). High resolu­tion CT of the petrous bone demonstrated a coalescent left mastoiditis with mastoid septa erosion and complete opacification of the middle ear. After contrast adminis­tration, no opacification of the left jugular bulb was observed, indicating acute thrombosis, which involved the proximal end of the jugular vein together with multi­ple enlarged lymph nodes. Ultrasonographic examination

937

938                                                     L. O. REDAELLI DE ZINIS ET AL.

FIG. 1. Patient 1. Computed tomographic scan obtained after contrast media administration at the level of the jugular foramen (A) and neck (B). A filling defect of the left jugular bulb (arrow) indicating acute thrombosis is evident, whereas the left sigmoid sinus is patent (arrowheads). The jugular vein thrombosis extends to the neck (short arrow).

of the neck confirmed the thrombosis of the proximal part of the internal jugular vein. A simple mastoidectomy and a myringotomy with the insertion of a ventilation tube were performed. The surgical intervention revealed pu­rulent exudates without the presence of granulation tissue or bone erosion, and the puncture of the sigmoid sinus gave rise to blood flow. The day after the intervention body temperature was normal; the antibiotic regime was changed to ampicillin-sulbactam (1,300 mg three times daily) and netilmicin (60 mg twice daily). Subcutaneous low molecular weight heparin administration was started two days after surgery with 2,000 units twice daily. Coagulation tests showed a thrombophilic state caused by a deficit in factor V Leiden, the so-called activated protein C resistance. Oral anticoagulation was initiated on the sixth postoperative day with warfarin; subcuta­neous heparin was discontinued after eight days when therapeutic values of international normalized ratio by oral anticoagulant were obtained. On the fifth postoper­ative day, the results of intraoperative bacterial culture showed the presence of Streptococcus pyogenes; thus, netilmicin was discontinued. Ultrasonographic exami­nation, one and two weeks after operation, showed pro­gressive recanalization of the jugular vein. Amoxicillin clavulanate was administered (500 mg three times daily) instead of ampicillin sulbactam from the eighth postoperative day for 20 days. After two months, a so­nography showed that recanalization of the internal jug­ular vein was complete, and blood flow was normal. At that time, normal hearing was documented using pure tone audiometry. The following month, warfarin was dis­continued. The ventilation tube was extruded within the fifth postoperative month, with spontaneous eardrum clo‑

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sure; 30 months later, no further episodes of otitis media or venous thrombosis have been observed.

CASE 2

A seven-year-old boy was admitted for right acute otitis media with fever when, after three days of inef­fective treatment with an oral cephalosporin, diplopia, increasing ear pain, and headache occurred. Physical examination revealed that there was a bulging reddish right eardrum with a paresis of the right abducens nerve. Antibiotic treatment was started with ceftazidime (675 mg four times daily) and amikacin (185 mg twice daily), and CT of the brain and temporal bone was performed. A blood cell count showed moderate neutrophilic granulo­cytosis, and Epstein-Barr viral tests were negative. The computed tomographic scan excluded brain lesions and confirmed a right coalescent mastoiditis (Fig. 2A). The next day, a neck and a pharyngeal swelling together with a torticollis appeared, and the patient was evaluated using CT of the neck to rule out extension of the mastoid infec­tion. The computed tomographic scan revealed enlarged lymph nodes and occlusion of the proximal portion of the internal jugular vein involving the jugular bulb (Fig. 2B). Magnetic resonance (MR) and MR venography of the brain and skull base confirmed temporal bone inflam­mation and thrombosis of the jugular bulb extending ap­proximately 2 cm to the internal jugular vein (Fig. 2C and E). Ampicillin-sulbactam (1,500 mg three times daily) was started instead of ceftazidime, and amikacin was also administered (370 mg once a day). On the same day, the patient was submitted to a mastoidectomy with posterior tympanotomy that revealed purulent exudates and granulation tissue without bone erosion; puncture of the sigmoid sinus gave rise to normal blood flow. Co­agulation tests were normal. Subcutaneous low molecular weight heparin was started (3,000 units twice daily). Oral anticoagulation was associated in fourth postoperative day with warfarin, and subcutaneous heparin was discon­tinued after two days when therapeutic values of inter­national normalized ratio by oral anticoagulant were obtained. The intraoperative bacterial culture was nega­tive, and on the seventh postoperative day, amikacin was discontinued. The patient was discharged on the eighth postoperative day, with ampicillin-sulbactam (1,500 mg two times a day for five days); oral amoxicillin clavula­nate was also administered (500 mg three times daily for the next 15 days). MR examination including MR veno­graphy, performed three months later, documented com­plete recanalization of the internal jugular vein (Fig. 2D and F) when anticoagulation treatment was discontinued. Two years later, the child has not experienced any new episodes of otitis media.

DISCUSSION

Venous thrombosis may be associated with infection because of vascular damage. When the vein is affected

INTERNAL JUGULAR VEIN THROMBOSIS IN ACUTE MASTOIDITIS                      939

FIG. 2. Patient 2. A, Computed tomographic scan, bone algorithm, and opacification of the right mastoid (arrow). B, Contrast-enhanced computed tomographic scan. Absent opacification of the right jugular bulb (arrow), suggesting acute thrombosis. On the left side is a jugular bulb with a normal appearance, enhanced with contrast material. C, MR, T2-weighted axial section at the level of the jugular foramen. Hyperintense inflammatory tissue in the right mastoid. The flow void is absent in the right jugular bulb (arrow), which is hypoplastic as compared with the contralateral side. D, Follow-up MR at three months, T2-weighted axial section. Reappearance of the flow void in the right jugular bulb (arrow), indicating recanalization. E, MR venography. Right jugular vein thrombosis. Dominant left transverse sinus. F, MR venography, follow-up at three months. Complete recanalization of the right jugular vein.

by an adjacent septic focus, the initial site of involve­ment is the adventitia, which becomes congested and infiltrated by inflammatory cells. Next, all the layers of the vein become involved, and the inflammation reaches the intima where the adherence of fibrin, blood cells, and platelets causes thrombus development (13). Formation of the thrombus may be considered as a protective mechanism, attempting to localize the infec­tion (6). An infection within the temporal bone may cause inflammation of intracranial structures such as the dura over the lateral sinus by direct spread through dehiscent bone or through the round and the oval win­dows, through the endolymphatic sac, via a fistula in the otic capsule or even through the cochlear aqueduct or perineural spaces in the internal acoustic meatus (7). On the other hand, extension through intact bone can develop by retrograde thrombophlebitis via the small venules of the temporal bone, from the superior petrosal sinus, the cavernous sinus, the caroticotympanic canaliculi, the

pericarotid venous plexus, and other possible vascular anastomoses (6). Thrombosis may also be caused by osteothrombophlebitis in the early stage of acute otitis media, with an intact bony sinus plate (6). In many cases, dural venous thrombophlebitis is associated with an epidural abscess (11).

Not all infections are followed by venous thrombosis, but in most cases, venous thrombosis develops during an otitis media in a patient with a generalized hypercoagu­lable state that may be either inherited or acquired (14). Inherited hypercoagulable disorders include deficiency of antithrombin III (qualitative or quantitative), which usually binds and inactivates thrombin and factor Xa, and deficiency of protein C or S, which usually sup­presses the coagulation cascade by inhibiting factors Va and VIIIa (15,16). Other inherited hypercoagulable disorders are resistance to activated protein C caused by factor V Leiden mutation, elevated prothrombin levels caused by prothrombin gene 20210A mutation,

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increased levels of factors VII, XI, IX, VIII, von Will­ebrand factor, lipoprotein (a), and thrombin-activatable fibrinolysis inhibitor, dysfibrinogemia, hyperhomocys­teinemia, and plasminogen deficiency (15,17). Most thrombophilic genetic anomalies are autosomal domi­nant: heterozygous individuals are mildly or moderately symptomatic, but in combination, they significantly in­crease the risk of thrombosis (15,18). Furthermore, other poorly defined genetic alterations may be associated with hypercoagulability (19).

The classic type of acquired hypercoagulable disorder is antiphospholipid syndrome, which is an idiopathic dis­ease characterized by the production of different antibo­dies (lupus anticoagulant, anticardiolipin, etc.) against different phospholipid-protein complexes involved in the process of coagulation and anticoagulation (20). Other acquired hypercoagulable states are those associ­ated with myeloproliferative disease or other malignan­cies, the use of oral contraceptives, asparaginase, epoetin alfa and tamoxifen, pregnancy and puerperium, high al­titude, surgery, immobilization, splints or casts, central catheters, heparin-induced thrombocytopenia, trauma, renal diseases, pulmonary hypertension, congenital heart disease, sepsis, traumatic and nontraumatic intra­cranial hemorrhage in newborns, paroxysmal nocturnal hemoglobinuria, vasculitis such as that caused by Beh0et disease, thrombocytosis, sickle cell disease, altered cerebral hemodynamic states (i.e., congestive heart failure, dehydration, malnutrition, and diabetic keto­acidosis), and chronic debilitating diseases in general (14,16,18,21–25). When dealing with an infection, it is known that bacterial products can cause induction of cytokines via immune cells leading to coagulation distur­bances (26). Particularly, Fusobacterium necrophorum, the microorganism involved in Lemierre syndrome, is a specific inducer of hemagglutinin-mediated platelet aggregation (27). A complete work-up to find inherited or acquired coagulation disturbances including protein C level, AT activity, protein S free antigen, presence of factor V Leiden, factor II polymorphisms, methyltetrahy­drofolate reductase mutations, serum homocysteine levels, lupus anticoagulant, anticardiolipin antibodies, and lipoprotein (a) levels has been suggested for sinus thrombosis associated with otitis media (25).

The presenting signs and symptoms of an otogenic lateral sinus thrombosis are often vague; otologic symp­toms predominate, and only nonspecific symptoms like headaches or fever can be more strictly related to intra­cranial complications (14,21,28,29). Sometimes, it is only the finding of a prolonged course of otologic symp­toms of an acute otitis media that raises the suspicion of a complication (14,21,28,29). On the other hand, the appearance of a neck mass and/or torticollis, when an exteriorization of the otomastoiditis has been excluded, can be suggestive of internal jugular vein thrombosis. “Cord sign“, an induration corresponding to the course of the internal jugular vein beneath the anterior border of the sternocleidomastoid muscle, is considered typical but is rarely present (13). For our two patients, a diag‑

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nosis of vascular complication was preceded by neck swelling and torticollis in the first case and by diplopia and torticollis in the second. The occurrence of abdu­cens nerve palsy in our cases can be attributed to peri­neural inflammation caused by petrous apex infection or by extension of the thrombus to the inferior petrosal sinus that courses downward, near to the abducens nerve and not to an increased intracranial pressure as observed by other investigators (16,21). Those symp­toms prompted us to perform a computed tomographic scan, which showed the thrombosis of the jugular vein. Contrast-enhanced CT with thin slices is highly sensi­tive in the diagnosis of venous dural sinus thrombosis and therefore should be performed when there are clin­ical signs. The diagnosis consists of detecting hyper­density of the sigmoid and transverse sinus, which can be confirmed by observation of a filling defect after con­trast media injection. In the present cases, contrast­enhanced CT showed a filling defect in the jugular bulb and an absent opacification of the jugular vein in the neck (Figs. 1 and 2). Although the diagnosis of jugular vein thrombosis does not require MR, this examination is in­dicated to detect concomitant lesions in the cerebellum and to document with MR venography the extension of the entire process whenever extension of the thrombotic process in the lateral sinus is suspected. MR venography is a useful tool for the assessment of the extension of thrombosis and for the follow-up after anticoagulation to demonstrate recanalization (Fig. 2). In addition, MR is performed on patients with clinical symptoms or CT findings suggestive of intracranial complications because of its higher sensitivity in detecting extra-axial fluid col­lections and associated vascular problems. Conventional MR imaging revealed that flow-related enhancement and in-plane, turbulent, or slow flow can cause loss of the flow void and thus mimic thrombosis. Consequently, phase-contrast MR venography is the imaging modality of choice for the assessment of cerebral venous throm­bosis. Anatomic variations and atypical cerebral venous drainage must also be taken into account to avoid false-positive diagnosis of jugular vein thrombosis (30). In the second case, there was in fact a dominant left transverse sinus, and the right jugular bulb was smaller than the contralateral bulb (Fig. 2). MR venography is considered as the technique of choice for diagnosis and follow-up of cerebral vein thromboses, although in certain cases conventional MR may be superior because it shows the thrombus itself and not just the absence of a signal, as observed with MR venography. On the other hand, MR venography is noninvasive, does not involve ionizing radiation, and can be performed at the same time as MR imaging, using comparatively short acqui­sition times.

To our knowledge, this is the first time that internal jugular vein thrombosis has been observed from acute otitis media without sigmoid sinus thrombosis, although the explanation for this unexpected behavior remains unknown. One hypothesis is that the inflammation in the region of the hypotympanum caused the vascular

INTERNAL JUGULAR VEIN THROMBOSIS IN ACUTE MASTOIDITIS                            941

reaction at the level of the jugular bulb, with a propa­gation of the inflammation along the wall of the vessel in the neck. Internal jugular vein extension of a sigmoid sinus thrombosis during otitis media has been described in less than 30 cases during the last two decades (1,2,6–10,12,13,18,21,25–27,31–34). The most well­detailed cases are reported in Table 1 and are almost equally distributed between chronic and acute or sub-acute otitis media; most patients were adolescents or children (Table 1). The infectious agents, when detected by microbiological culture, included various groups with a high number of cases having mixed flora (Table 1). F. necrophorum, such as in Lemierre syndrome, was observed in only three patients (10,27,34). Septicemia by F. necrophorum during an otitis media without a thrombotic event has also been reported (35). A hyper­coagulable disorder was observed in two previously re­ported cases: antiphospholipid syndrome with anomalies in tissue thromboplastin inhibitor (18) in one case and APC resistance with a factor V Leiden mutation in the other (34). Associated intracranial complications (menin­gitis, epidural or cerebellar abscess, hydrocephalus, and cerebrospinal fluid otorrhea) have been reported in only a few patients (2,10,12,32). Septic pulmonary emboli have also been reported in two patients (8,9). Most patients had been treated by mastoidectomy, sigmoid sinus opening, and drainage (1,8,12,13,27); whereas some also under­went jugular vein ligation (2,6,7,9,10,31). Antibiotic treatment for periods varying from 2 to 8 weeks was used in all cases, usually in association with antibiotics that were tailored after the results of microbiological cultures were available. Anticoagulation therapy was used in less than half of the cases for a period of two weeks to six months (6–8,18,21,26,27,32–34). Only one patient needed lifelong anticoagulation for antiphospho­lipid syndrome (18). Recovery without important se­quelae was reported for most patients; only one death was reported that occurred in the early 1980s (12). Most patients that did not have opening of the sigmoid sinus or ligation of the jugular vein had recanalization of the vessel (21,26,32).

The observation of a progressive, more conservative approach of this clinical entity over time (Table 1) is undoubtedly caused by more effective medical treatment, which permits less aggressive surgery. Exposure and con­trol of the internal jugular vein and thrombus removal in cases having thrombosis of the sigmoid sinus, as sug­gested in the 1980s and early 1990s (Table 1), carry some risks, including septic emboli and violation of the dura with subarachnoid spread of infection (36). On the contrary, the aim of surgery should be to remove infection from middle ear and mastoid and restore ventilation, whereas management of thrombosed vessels does not seem to need surgery, as what was observed in our patients, and has been recently reported for sigmoid sinus thrombosis alone or associated with jugular vein thrombosis (21,26,32,36). At present, opening and drainage of the sinus and drainage or ligation of the jugular vein should be reserved for cases of empyema (37) or for rare

cases that develop septic embolism (27). Even transfe­moral or transjugular direct venous thrombolysis with urokinase infusion and balloon dilation if there is a ste­nosis, as what was proposed by some investigators (38–40), or combined intravenous heparin and direct intrathrombus administration of recombinant tissue plas­minogen activator (41) seem to be unnecessary in these cases (21,26,32). In general, endovascular thrombolysis has not yet been demonstrated to be more effective than systemic treatment (16).

General guidelines for the treatment of thrombo­embolic events, both in adults and in children, include intravenous heparin or subcutaneous low molecular weight heparin for several days and subsequent over­lapping with oral anticoagulants until the prothrombin time is prolonged to the therapeutic value with a target international normalized ratio of 2.5 (16). Anticoagulant therapy is stopped after three to six months when risk factors for thrombotic events are eliminated. In our patients, anticoagulants were discontinued after three months when the ear was completely recovered, and there was documentation of jugular vein recanalization. When a continuing risk factor or a second recurrence of thromboembolic event is present, indefinite oral antico­agulant therapy should be considered, although this pos­sibility is controversial because most thrombophilic individuals (those with low levels of baseline genetic hypercoagulability) never have venous thromboembolism throughout their lifetimes; if an episode does occur, it is unlikely to recur (15). Some investigators have reported recanalization of the sigmoid sinus with antibiotic treat­ment alone and dispute the usefulness of anticoagulants that are thought to carry potential risks (release of septic emboli, hemorrhage into the mastoid cavity, bleeding, drug interactions, thrombocytopenia, osteoporosis, and hemorrhagic skin necrosis) (2,14,33,36). In reality, in the case of otitis media thrombophlebitis, complications of anticoagulant therapy have not been reported. More­over, for children, anticoagulant therapy for dural sinus thromboses is considered safe and without sequelae (23).

Intravenous antibiotic treatment that is effective against infectious agents normally present in acute and chronic otitis media (Streptococcus pneumoniae, Haemo­philus influenzae, or Moraxella catarrhalis, Staphylo­coccus ureus, Pseudomonas aeruginosa, anaerobes) should be started and tailored after the results of bacterio­logic examination of middle ear and mastoid exudates are available. After approximately 2 weeks of intravenous antibiotics, when a favorable evolution is observed, oral administration can be started for a total period of four to six weeks (21,36).

In conclusion, we observed two unusual cases of in­ternal jugular vein thrombosis secondary to acute otitis media where the combination of antibiotic and anti­thrombotic therapy was found to be a safe and effective treatment with recanalization of the vein. Moreover, we have also analyzed cases of sigmoid sinus thrombosis with internal jugular vein extension reported in the liter­ature. The perception of a progressive trend toward more

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944                                                   L. O. REDAELLI DE ZINIS ET AL.

conservative treatment, where antibiotics and anticoag­ulants are the mainstay of therapy and surgery is reserved to eliminate the source of infection by draining the tym­panomastoid cavity, is deducible from the literature and confirmed by our experience. Surgical opening, drainage, and ligation of the jugular vein should be reserved for cases where purulent exudates within the vessel pose an impending risk of septicemia or diffusion of septic em­boli. Some controversies are still present about the choice and the duration of anticoagulant therapy (16).

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Otogenic Deep Neck Abscess: A Rare

Complication of Cholesteatoma with Acute

Mastoiditis

YAO-LIANG CHEN SHU-HANG NG HO-FAI WONG MUN-CHING WONG YAU-YAU WAI YUNG-LIANG WAN First Department of Diagnostic Radiology, Chang Gung Memorial Hospital, Chang Gung University

Deep neck abscess is a rare complication of acute mastoiditis in the era of antibiotics. In preantibiotic era, Bezold’s abscess was the most common cause of the otogenic deep neck abscess. We reported a 19- year-old male with cholesteatoma complicated by acute suppurative mastoiditis, lateral sinus throm­bosis, thrombophlebitis of the ipsilateral internal jugular vein and deep neck abscess formation. The pathway of the deep neck abscess formation is dif­ferent from the classic presentations of Bezold’s abscess. Computed tomography, magnetic resonance imaging and ultrasonography were complementary for the detailed demonstration of the origin and the extent of the disease. Segmental mural lysis of the infected internal jugular vein was considered to be responsible for the development of deep neck abscess formation.

Key words: mastoiditis; Bezold’s abscess; neck, abscess; sinus, thrombosis

Reprint requests to: Dr. Yao-Liang Chen

Address: First Department of Diagnostic Radiology, Chang Gung Memorial Hospital.

No. 5 Fu Hsing Street, Kwei Shan, Taoyuan 333, Taiwan, R.O.C.

Otitis media is a common disease of childhood. However, it has been estimated that 1.5% of all adults suffered from active chronic otitis media [1]. Complications of otitis media can be categorized into two groups: intracranial and extracranial. Intracranial complications occur in 0.24 % of the patients and include meningitis, encephalitis, brain abscess, epidur­al abscess and lateral sinus thrombosis. Extracranial complications occur in 0.45% of the patients and include facial nerve paralysis, labyrinthitis, perichon­dritis, coalescent mastoiditis and subperiosteal abscess [2]. Deep neck abscess is extremely rare in the era of antibiotics. Herein, we present a rare transcranial com­plication of cholesteatoma and acute mastoiditis with resultant deep neck abscess formation. Imaging studies with different modalities are valuable for tracing the pathway of disease extension.

CASE REPORT

A 19-year-old male came to our emergent unit with pale and ill looking. He suffered from progressive vertigo, vomiting, nystagmus, supervened chillness and fluctuating fever (the spike up to 40.3˚C) for five days. On physical examination, swelling, erythema, heatness and tenderness on his left retroauricular region were noticed. Otoscope displayed perforation of the left tympanic membrane with purulent discharge. Laboratory data showed elevated white blood cell count (23500/mm³) and C-reactive protein (180 mg/dl). Due to the past history of chronic otitis media, he was clinically impressed as acute mastoiditis complicated with Bezold’s abscess formation. High-resolution com­puted tomography (HRCT) of temporal bone showed soft tissue density filling the left tympanic cavity and cloudiness of the left mastoid air cells with bony erosion at the tympanic tegmen (Figure 1a). Magnetic resonance images (MRI) further displayed swelling of the left temporalis muscle, thrombosis of the sigmoid sinus, jugular bulb and upper internal jugular vein

252                                                                                  Otogenic deep neck abscess

(IJV) (Figure 1b, 2a). T1-based enhanced MR angiogram of the neck confirmed IJV thrombosis (Figure 2b). Enhanced CT of the neck disclosed deep abscess formation that was contiguous to the throm­botic left IJV and deep to the sternocleidomastoid muscle (SCM)(Figure 3a). High-resolution ultrasonog­raphy (HRUS) clearly depicted focal lysis at the wall of the infected left IJV (Figure 3b). The patient under­went left canal-down radical mastoidectomy, tym­panoplasty, meatoplasty, incision and drainage of the left neck abscess. Left suppurative mastoiditis compli­cated by attic cholesteatoma was explored. However, there was no continuation between the mastoiditis and deep neck abscess either by imaging findings or by surgical exploration. Pus culture showed moderate growth of anaerobic bacteria including Peptostre­ptococcus anaerobius and Peptostreptococcus asaccha­rolyticus. Treated by thorough open drainage of abscess and intravenous antibiotic administration, the patient was discharged three weeks later with the sequel of left hearing loss (over 100 decibel).

DISCUSSION

Acute mastoiditis can be the complication of pre­existing secretory otits media or other chronic disease of middle ear, including cholesteatoma. Before the use of antibiotics it was common and associated in some

instances with serious complications. The mucosa of the tympanic cavity and its extension into the mastoid cells has an inherent ability to overcome acute infec­tion. As a result, acute otitis media and mastoiditis may be self-limited. However, severe suppurative and necrotizing infections of the middle ear can cause sys­temic reaction [3].

The incidence of complications resulting from suppurative otitis media has significantly decreased in the era of antibiotics. In the beginning of the 20th century, 50% of all cases of otitis media developed coalescent mastoiditis. Recent studies suggested a current incidence of only 0.24% [2]. Complications of otitis media can be grouped into two broad categories: intracranial and extracranial. Intracranial complica­tions include meningitis, encephalitis and lateral sinus thrombosis. Prior to the widespread use of antibiotics, 2.3% of patients with otitis media developed intracra­nial complications. Nowadays, the rate has fallen nearly 10-fold to 0.24%. The contemporary risk for developing extracranial complications of otitis media is approximately twice of that for intracranial compli­cations, with 0.45% of patients experiencing problems such as facial nerve paralysis, labyrinthitis, perichon­dritis, coalescent mastoiditis, subperiosteal abscess or Bezold’s abscess [1].

Bezold’s abscess was considered clinically when mastoiditis coexisted with deep neck abscess. In 1908,

Figure 1. a. Coronal HRCT shows a focal break-through of the tympanic tegmen (double arrows) indicating the possibility of intracranial complications. b. Axial enhanced T1-weighted MRI displays abnormal enhancement (double arrowheads) in left sigmoid sinus and jugular bulb suggesting lateral sinus thrombosis.

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Bezold was the first to describe abscess in the neck arising from mastoiditis. Inflammation and infection may result in necrosis of the mastoid tip, allowing the pus to track from the medial side of the mastoid process through the incisura digastrica (digastric groove). The pus is prevented from reaching the surface by neck musculature, but can track along the fascial planes of the digastic muscle or SCM [1]. Bezold’s abscess usually spreads into the substance of the SCM and confines to the posterior cervical and perivertebral spaces by the pharyngobasilar fascia and the deep layer of deep cervical fascia [4,5]. It may extend into the carotid, prevertebral, danger, and retropharyngeal spaces. By gaining the access into the danger space, an abscess may extend into the medi­astinum or into the base of the skull [4]. Due to the depth of their location, Bezold’s abscess may be diffi­cult to be palpated. Pneumatization of the mastoid process leads to thinning of the bone and is considered an important factor in the development of Bezold’s abscess. Therefore, Bezold’s abscesses are more common in adults than in children because well pneumatization of mastoid tip often occurs in adults.

Mastoiditis contributes to most of the lateral (sigmoid) sinus thrombosis. In a series of 22 cases of

lateral sinus thrombosis, 19 were associated with sup­purative otitis media and mastoiditis [6]. Suppuration in the middle ear, mastoid, or both may spread to the adjacent intracranial structures through progressive thrombophlebitis, bony erosion, or direct extension, resulting in meningitis, extradural abscess, subdural empyema, focal encephalitis, brain abscess, lateral sinus thrombosis, and otic hydrocephalus. The clinical pictures of lateral sinus thrombosis are well document­ed: an ill patient with high swinging pyrexia (‘picket fence’ pattern), headache, neck pain and progressive anemia with, less commonly, occipital edema [6].

The occurrence of deep neck abscess and lateral sinus thrombosis may be coincidental, since both are complications of mastoiditis. There is also a possibili­ty that lateral sinus thrombosis may be arisen by retro­grade spread from the infected internal jugular vein which had already been involved by Bezold’s abscess. It is however possible that the neck abscesses may be formed by direct regional spread from the infected internal jugular vein that had autolysed [6], as in our case.

Radiological study is indicated in cases of acute mastoiditis or otitis media when there is clinical sug­gestion of coalescent mastoiditis, which signifies the

Figure 2. a. Coronal enhanced T1-weighted MRI depicts thrombosis in the left upper internal jugular vein (IJV). A low­signal gap and enhanced extramural tissue (double black arrows) imply thrombophlebitis and possibly segmental mural lysis of the left IJV. As compared with the signal void in right IJV(open large arrow), the thrombosis of left IJV shows slight low signal intensity (double white arrowheads) with thick mural enhancement. b. Venous phase of T1-based MR angiogram shows obvious enhanced flow signal in the right IJV but absence of flow signal in the left IJV, confirming left IJV thrombosis. (RIJV: right internal jugular vein, RCCA: right common carotid artery, RVA: right vertebral artery, LCCA: left common carotid artery, LVA: left vertebral artery)

254                                                                                  Otogenic deep neck abscess

3a                                                                                                       3b

Figure 3. a. Axial enhanced CT displays filling defects in the left IJV and a neighboring abscess (arrow) deep to the SCM. Note increased soft tissue density in the left posterior cervical space due to cellulitis. b. Longitudinal scans of HRUS clearly discloses a segmental mural lysis at the left IJV (arrowheads). Loss of compressibility and low echoic thrmobosis within the IJV are also found.

transition from mucoperiosteal disease to bone disease and even to intracranial complications. CT can show stages of disease progression when infection spreads by way of soft tissue and bone pathways into dural venous sinuses, meninges, labyrinth, facial nerve, epidural and other intracranial spaces [3]. CT is also valuable in early diagnosis of cholesteatoma in the mastoid cavity and exact delineation of abscess forma­tion [5]. MRI is more useful than CT for evaluation of the complications of acute mastoiditis in some aspects. Septic thrombosis of the lateral sinus and jugular bulb is a highly lethal condition. Enhanced CT may reveal evidence of sinus thrombosis, manifested as a filling defect in the vessel (empty delta sign). However, false-positive diagnosis up to 30% of cases has been reported [7]. In contrast, MRI can distinguish between flowing blood and thrombus. Enhanced MRI with gadolinium-DTPA is a valuable adjunct to achieve the diagnosis and to delineate the extent of the pathology [8], whereas magnetic resonance angiography is the procedure of choice for the confirmation of venous thrombosis. CT and MRI can detect concomitant cere­bral infection or infarction as well as infection in the epidural or subdural spaces [7].

For jugular vein thrombosis, HRUS can show an intraluminal mass of low or mild amplitude echoes, loss of respiratory rhythmicity and venous pulsation,

absence of venous response to respiratory maneuvers (Valsalva and sniff test) and incompressibility of the thrombotic jugular vein [9]. Because HRUS can depict both transverse and longitudinal planes, it can provide an accurate delineation of luminal compromise by thrombus [10]. For the patient we reported, only HRUS could depict the exact location of segmental autolysis of the jugular wall. Based on the imaging findings of CT, MRI and HRUS, we believe that the deep neck abscess arose from the septic throm­bophlebitis of the internal jugular vein rather than direct extension of mastoiditis from the mastoid tip, which is the typical pathway of Bezold’s abscess.

Early surgery is mandatory to treat severe mas­toiditis such as coalescent mastoiditis and subpe­riosteal abscess. The infected neck or abscess forma­tion needs thorough drainage. Given the low incidence and lack of consistent signs and symptoms, Spiegel et al suggested contemporary practitioner must be rely on radiological images to determine the presence and pathway of mastoid abscess [1].

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