Lateral sinus thrombosis as a complication of acute mastoiditis

ACTA otorhinolaryngologica italica 2008;28:30-33
Case report
Lateral sinus thrombosis as a complication of acute mastoiditis
Trombosi del seno laterale come complicanza di mastoidite acuta
C. BIANCHINI, C. AIMONI, S. CERUTI, D.L. GRASSO, A. MARTINI Department of Medical and Surgical Specialities, Audiological Unit, University of Ferrara, Italy
Summary Lateral sinus thrombosis is a rare complication of middle ear diseases: in children, it is usually related to acute otitis media, but it is also found in adults with chronic otitis. It was more frequent in the pre-antibiotic era and mortality was high. The Authors present a paediatric case of lateral sinus thrombosis in which they describe the clinical approach and related literature. Key words: Acute otitis media • Complications • Lateral sinus thrombosis • Diagnosis • Angio-MR • Anticoagulant therapy
Riassunto La trombosi del seno laterale è una complicanza rara delle patologie dell’orecchio medio: in età pediatrica si correla solitamente ad un quadro di otite media acuta, mentre nell’adulto può associarsi anche ad un’otite media cronica. La trombosi del seno laterale era di più frequente riscontro in era pre-antibiotica, con mortalità elevata. Gli Autori descrivono un caso di trombosi del seno laterale in età pediatrica, presentando una revisione della letteratura. Parole chiave: Otite media acuta • Complicazioni • Trombosi del seno laterale • Diagnosi • Angio-RM • Terapia anti30 coagulante
Acta Otorhinolaryngol Ital 2008;28:30-33

Introduction
Lateral Sinus Thrombosis (LST) was considered a frequent complication of middle ear infection at the beginning of the last century and mortality reached up to 100% in untreated cases 1. Over the last fifty years, its incidence has greatly decreased due to the widespread availability of antibiotic drugs and mortality has dropped below 10% 2. Morbidity, related to LST, is approximately 30% and is associated with septic cardiomyopathy, acute respiratory distress syndrome, anacusis and seizures 3. Antibiotic resistance has now been recognized as the main factor of the increasing incidence of LST as a complication of acute and chronic otitis 4. Especially in young adults, LST is now more often seen in association with a generalized hypercoagulable state, inherited or acquired 5 6. The Authors present a paediatric case of LST in which they describe the clinical approach and the related literature review.
Case report
BB, a 4-year-old girl, was admitted to the ENT Department in November 2001, with a history of a left acute otitis media which had built up over the past 4 days and with a temperature fluctuating between 38.5 °C-40 °C. On examination, a

purulent otorrhea, as well as a significant left post-auricular swelling, were present. Computed tomography (CT) scan of the brain and temporal bones without contrast was immediately performed revealing diffuse clouding of left tympanic and mastoid cavities, without signs of coalescent mastoiditis or bone erosion. Laboratory investigations showed an inflammatory syndrome (WBC 10400/µl, PCR 17.00 g/dl, VES 112 mm). During paediatric hospitalization, the child received antibiotic therapy intravenously (iv) (Ceftriaxone 1 g/day). An auricular specimen, collected for microbiologic test, resulted negative. Over the following days, the local and general conditions of the patient improved and after 6 days the patient was discharged with antibiotic therapy (Amoxicillin and Clavulanate 1 g perorally/day). Upon examination, 2 days later, swelling in the left retroauricular area and a fever were observed (37.5°); microtoscopy indicated persistence of the acute involvement of the middle ear. The patient was hospitalized. Myringotomy and enlarged mastoidectomy were performed; there was evidence of partial erosion of the bone covering the lateral sigmoid sinus area. A retroauricular drainage was inserted and removed after 4 days. The intra-operatory microbiological sample was negative. Antibiotic treatment iv was changed (Ceftazidime 600 mg/ day, Amikacin 250 mg twice a day, Teicoplanin 180 mg/ day). After surgery, the clinical status suddenly improved

Lateral sinus thrombosis

and no fever was present. A post-operative (CT) scan of the brain and temporal bones, without contrast, was performed 5 days after surgery, to clearly define the extension of the bone erosion and to exclude cerebral involvement. Imaging showed a wide erosion involving the left mastoid and sinus plate with no evidence of cerebral lesions. Magnetic resonance imaging (MRI) revealed thrombosis in the left lateral and transverse sinuses and meningeal inflammation, with thickening and contrast enhancement (Figs. 1A, 2A). Magnetic resonance angiography (MRA) confirmed the lack of the normal high flow in the left lateral and transverse sinuses (Fig. 3A). The study of the hypercoagulability status was normal. The patient received low molecular weight heparin (0.3 ml per day). After 20 days, MRI showed the persistence of thrombosis in the lateral and transverse sinuses. Anticoaugulant therapy was then administered for the following 5 months (low molecular weight heparin 0.3 ml per day), in accordance with haematologic consultation. After a further 6 and 12 months, MRI and MRA were performed and showed partial recanalization of the involved venous sinuses and normalization of the meninges (Figs. 1B, 2B, 3B).

Fig. 2A. T1 weighted MR coronal images after contrast administration. MR performed a few days after surgery displays meningeal thickening and contrast enhancement in left middle cranial fossa, without lesions involving temporal lobe; jugular vein bulb is enhanced after contrast.

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Fig. 1A. T1 weighted MR axial images after contrast administration. MR performed a few days after surgery displays constrast enhancement of left lateral sinus, that has lost normal slow flow signal.

Fig. 2B. T1 weighted MR coronal images after contrast administration. MR performed displays no meningeal enhancement in middle cranial fossa and normal flow related signal in jugular bulb.

Fig. 1B. T1 weighted MR axial images after contrast administration. MR performed one year later displays normal flow-related signal in central part of left lateral sinus.

Fig. 3A. MRA supero-inferior view. A few days after surgery, no flow is displayed in left transverse and lateral sinuses and internal jugular vein bulb.

C. Bianchini et al.

ningeal signs. Papilloedema and the Griesinger sign,

consisting of oedema and tenderness over the mastoid

process (due to the septic thrombosis of the mastoid emis-

sary veins) are often present 1. LST can be also associ-

ated with sixth and seventh nerve palsy, hydrocephalus

or signs of thrombus propagation, that can also cause,

proximally. jugular bulb and, distally, occlusion of other

dural sinuses and then neurological symptoms due to in-

volvement of the lower cranial nerves (IX, X, XI pairs) 5 9.

When clinical suspicion of intracranial complications,

during middle ear infection, is present, imaging is consid-

ered essential to formulate diagnosis and planning man-

agement. Contrast-enhanced CT imaging of the brain and

Fig. 3B. MRA supero-inferior view. One year later, partial recanalization is observed.

temporal bones displays the sinus plate erosion and may show the “delta sign” (central non-enhancing clot surrounded by enhancing dural sinus wall), indicating sinus

thrombosis, and other intra-cranial complications, such as

Discussion

brain abscess and empyema 3. In the diagnosis of LST, MRI is considered to have a higher resolution than CT

Lateral sinus thrombosis is usually associated with acute or on account of its ability to show low or absent flow in the

chronic middle ear diseases. As far as concerns pathogen- venous sinuses, clot formation and the presence of inflam-

esis, it has been proposed that the propagation of infection mation in the brain and meninges 1. Using MRI, the pres-

from the small venules of the mastoid into the sigmoid si- ence of a thrombus appears as an increased signal inten-

nus could result from direct spreading of the inflammatory sity in T1 and T2 images. MRA is useful in confirming the

process through a coalescent or cholesteatomatous bone diagnosis of thrombosis in doubtful cases on MRI.

erosion, causing the formation of a perisinus absess 3 5 6. Treatment of LST has recently become more conservative 10.

Subsequently, adherence of fibrina, blood cells and platelets Most Authors agree that, in children, surgical treatment of

can produce a mural thrombus. The persistence of the in- septic LST combined with antibiotic therapy i.v. is stand-

flammation and the increase in thrombus volume might re- ard care, today 1 3-5 8 10. Mastoidectomy is performed in

32 sult in an obliterative LST. In the early course of acute otitis patients with AOM, while radical or radical modified mas-

media, LST can be caused also by an osteothrombophlebitis toidectomy is recommended in cases of cholesteatoma,

phenomen; if this is the case, the sigmoid bony sinus plate depending on the extension of the disease 9. A combined

will be intact at the time of surgical exploration 1.

neuro- and oto-surgical approach has been suggested, in-

Hypercoagulable states, inherited (antithrombin III, pro- stead, to drain the purulent discharge and to remove the

tein C or protein S deficiency, factor V Leiden mutation thrombus 3.

or prothrombin gene 20210A mutation) or acquired (neo- Internal jugular vein ligation using a cervical approach to

plasms, trauma, autoimmune disorders, neurosurgical in- prevent thrombus propagation, routinely performed in the

terventions, myeloproliferative disease, anti-phospholipid pre-antibiotic era, is now controversial 1. Today, current in-

syndrome, pregnancy or use of oral contraceptives) are dications to this treatment are persistent septicaemia even

considered as risk factors in LST 5.

after mastoidectomy, or septic pulmonary or extrapulmo-

Thus, once LST has been diagnosed, investigations to nary embolization 9. Since the risk of embolization is low,

identify all possible haematological predisposing risk fac- jugular vein ligation is rarely necessary 1 3 4 8 10.

tors are necessary 5.

Recently, anti-coagulant treatment has been proposed to

Several microbiological agents can be isolated and cul- prevent complications associated with thrombus persist-

tured in LST cases; Syms et al. reported anaerobic organ- ence or its possible propagation 11. If anti-coagulation use

isms in 100% and Proteus Spp in 66% of patients with is mandatory in all patients with a hypercoagulable state,

LST secondary to chronic otitis media 7. Seven et al. inherited or acquired, the role of anticoagulants in oto-

described Proteus Mirabilis followed by Pseudomonas genic LST cases is still not clear, be Bradley et al. pointed

Aeruginosa and Bacteroides Fragilis as the most common out that the risk of complications related to LST (such as

microrganisms detected, while in one third of patients no embolization and persistent sepsis) should be balanced

germs could be found 3. Also B-haemolytic Streptococcus with the possible complications associated with anti-co-

and Staphylococcus Aureus have been reported as causa- agulation, especially within the paediatric population, as

tive agents 4.

major risks of anti-coagulation treatments include throm-

At least two clinical pictures, related to LST, are described: bocytopenia, drug interactions, bleeding, haemorrhagic

the septic form and the aseptic form. The first is associated skin necrosis and bleeding 5. Nevertheless, if anti-coagu-

with clear signs of otomastoiditis and rarely complicated by lant therapy is necessary, then the type of drug used, the

cerebral abscess. The aseptic form is often associated with entire duration, the dosage, as well as the specific patient

endocranic hypertension and possible ocular signs 8. The characteristics, must be taken into consideration 5 12.

case described is a typical occurrence of the septic form.

In the case presented here, mastoidectomy associated with

The most common manifestation in patients with LST anti-coagulant treatment, represented the therapeutic ap-

is fever, which is usually sustained or occurs in a spik- proach correlating with extension of disease and local

ing pattern. Other symptoms are othorrea, post-auricular complications.

oedema, otalgia, headache, nausea, vomiting and me- No other treatment was necessary.

Lateral sinus thrombosis

Conclusions
LST in children is considered an unusual complication of otitis media requiring accurate recognition and treatment. Imaging is essential to formulate diagnosis and for plan-

ning strategy. Management of LST is controversial, but usually consists of conservative surgical treatment combined with antibiotic therapy iv. Recently, anti-coagulant treatment has also been proposed.

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Received: May 22, 2006 - Accepted: March 22, 2007

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Address for correspondence: Prof.ssa C. Aimoni, Clinica Audiologica, Università di Ferrara, Arcispedale “S. Anna”, corso Giovecca 203, 44100 Ferrara, Italy. Fax +39 0532 247709. E-mail: [email protected]