Lateral sinus thrombophlebitis

Lateral sinus thrombophlebitis TERRENCE R MURPHY, MD, FACS, and WILLIAM BOYDSTON, MD, PhD, Atlanta, Georgia

Lateral sinus thrombophlebitis (LST) occurs when infection causes (1) thrombophlebitis of small venules surrounding the outer dural wall of the sigmoid sinus, (2) erosion of the bone overlying the sigmoid sinus such as with cholesteatoma, or (3) infection that spreads to the sigmoid sinus through a dehiscence in the overlying bone. 1,2 Left untreated, the thrombophlebitis causes formation of an intrasinus thrombus that may embolize to the lungs or the brain.1 During the preantibiotic era, intracranial complications of otitis media caused 25 of 1000 deaths, and LST was the second most common cause of death from a complication of otitis media. 2 Antibiotic therapy has greatly reduced the overall incidence of death from otitis media by 90%; howevel, the incidence of mortality from LST today still remains nearly 16% to 35%.2-4 The decreased incidence and changing clinical presentations have made the early diagnosis of LST more difficult for physicians. Prompt diagnosis of LST requires a high index of suspicion and a firm understanding of the varied clinical presentations of LST. This article (1) reviews the varied clinical presentations of lateral sinus thrombophlebitis, (2) discusses diagnostic, laboratory, radiographic findings, and (3) outlines the management of lateral sinus thrombophlebitis. METHODS Four patients with LST were treated over the past 3-year period. Two girls aged 6 and 11 years and two boys aged 3 and 12 years form the basis for this study. Their charts were reviewed retrospectively for symptoms, laboratory data, radiographic findings, surgical findings, microbiologic data, operative findings, and postoperative course. CASE REPORTS Case I. A 6.5-year-old girl was seen by her pediatrician with an acute right otitis media, nausea, and vomiting. Antibiotics were prescribed, but she returned 3 days later with persistent fever and intermittent vomiting. After 2 days of continued antibiotic therapy, she returned with lethargy, low-grade fever, and intermittent vomiting. Her physical examination revealed postanricular tenderness without erythema or swelling. Her white blood cell (WBC) count was

Dr. Murphy is in private practice of otology and neurotology,and Dr. Boydson is a neurosurgeonwith Pediatric Neurosurgery,RC. Presented at the American Society of Pediatric Otolaryngology, Durango, Colo., May 1995. Reprint requests: Terrence R Murphy, MD, 5505 Peachtree Dunwoody Road, Suite 250, Atlanta, GA 30342. OtolaryngolHead Neck Surg 1997;117:S134-S137. Copyright © 1997 by the American Academy of OtolaryngologyHead and Neck Surgery Foundation,Inc. 0194-5998/97/$5.00 + 0 23/4/75143 $134

20,700/mm3 with 70% neutrophils, and her erythrocyte sedimentation rate (ESR) was 110 mm/hr. She was hospitalized and treated with parenteral antibiotics. During hospitalization her postauricular tenderness resolved within the first 48 hours, and she was discharged after 4 days of parenteral antibiotics and given oral amoxicillin with clavulanic acid (Augmentin). At the time of discharge, her WBC was 13,000, and her ESR was 66. She was rehospitalized 2 days later with continued headaches and intermittent vomiting. A computed tomography (CT) scan demonstrated opacification of the mastoid and sigmoid and partial transverse sinus thrombosis without evidence of intracranial abscess (Fig. 1). The patient continued to have headaches, nausea, and vomiting, and a sixth nerve palsy developed on the side of mastoiditis. The child had bilateral papilledema resulting from increased intracranial pressure from mastoiditis and sigmoid sinus thrombosis.5 She underwent a mastoidectomy and decompression of the sigmoid sinus. At the time of surgery, the mastoid was found to be full of granulation tissue, and there was a bony dehiscence over the sigmoid sinus, which was covered by infected fibrous tissue. The sigmoid sinus was thrombosed. All bone was removed from the sigmoid (lateral sinus), which allowed the infection of the sinus unrestricted drainage into the mastoid cavity. Microbiologic cultures of the mastoid tissue did not demonstrate an organism. Postoperatively the patient continued to have headaches with nausea and vomiting. A neurologic consultation was obtained on the fourth postoperative day for management and treatment of her persistent benign intracranial hypertension. A lumbar puncture demonstrated an opening pressure of 550 mm H20. Her headaches and vomiting rapidly resolved. She was discharged with acetazolamide (Diamox) and had a full recovery of her sixth nerve palsy and papilledema during the next 2 months. Cose 2. A 3-year-old boy was in good health until approximately i week before admission, a low-grade fever and left otitis media developed. The next day he was noted to have some postauricular swelling and erythema and forward protrusion of his pinna. ACT scan of the temporal bones and brain demonstrated mastoiditis and sigmoid sinus thrombosis (Fig. 2). On admission the WBC was 15,900 with 16% bands. The C-reactive protein (CRP) was 7.79. The patient was given parenteral antibiotics, and a mastoidectomy was performed. When the postauricular incision was made, pus oozed from the mastoid cortex, and the entire mastoid air cell system was full of friable, hypertrophic granulation tissue. As the sigmoid sinus was exposed, a dehiscence of the sinus was encountered. Pus emanated from the lumen of the sigmoid sinus in a pumping fashion, synchronous with the dural pulsations. A large thrombus that obliterated the lumen of the sigmoid sinus was partially removed. All bone covering the sinus and all disease in the mastoid cavity were removed. A Penrose drain

OtolaryngologyHead and Neck Surgery Volume 117 Number 6

Fig. 1. CT scan of the brain with thrombosis of the sigmoid and transverse sinus.

was placed and removed on the fifth postoperative day. Intraoperative cultures revealed Streptococcus pneumonia, and the patient's antibiotic was changed to penicillin G. He was discharged on the eighth postoperative day, and he recovered without sequela. C a s e 3. A 12-year-old boy was admitted to a neighboring hospital with fever, Gram-negative bacteremia, thrombocytopenia, hyponatremia, and hypoalbuminemia. The child had been ill 1 week before admission with right ear discharge and posterior head pain. An initial blood culture was positive for Proteus and Bacteroides. His temperature spiked to 104.4°F 2 to 3 times per day. His admission laboratory tests demonstrated a CRP of 10.1 mg/dl with an ESR of 87 mm/hr and a WBC of 16,000/ram 3, with 31% bands and 58% neutrophils. A C T scan of the mastoids demonstrated opaeification of the right mastoid and middle ear with absence of the ossicular chain. Right neck tenderness with adenopathy was noted, and a CT scan of the neck demonstrated significant adenopathy and a thrombosed internal jugular vein to the level of the clavicles. A C T scan of the chest demonstrated well-circumscribed cavitary lesions of the right middle and left midlung. These lesions were consistent with septic emboli. A diagnosis was made of sigmoid sinus thrombosis and internal jugular vein thrombosis resulting from mastoiditis. Ophthalmic fundnscopy revealed bilateral papilledema. The patient underwent an MRI of the brain and MRI angiogram (MRA) to assess the patency of the other intracranial sinuses. The MRA demonstrated thrombosis extending from the right transverse sinus to the right internal jugular vein (Fig. 3). The patient was taken to the operating room where the right internal jugular vein was ligated and a right radical mastoidectomy was performed. The child had a cholesteatoma filling the mastoid and middle ear. The sig-

MURPHYand BOYDSION $135

Fig. 2. CT scan demonstrating thrombosis of the sigmoid sinus.

mold sinus was completely dehiscent with pus pouring out of the sigmoid sinus, Infected granulation tissue covered the sigmoid sinus. Operative ear cultures grew the organism Morganella, sensitive to gentamicin and metronidazole. Postoperatively he showed a steady improvement, was discharged from the hospital on the ninth postoperal:ive day, and recovered without sequela. C a s e 4. An ll-year-old girl with severe psychomotor retardation was seen in the hospital with a 4-year history of intermittent malodorous otorrhea from the left ear. In the emergency department, her WBC was 33,000/mm3 and her ESR was 76 ram/hr. Her otologic examination demonstrated a perforation of pars flacida and cholesteatoma of the left ear. A C T scan of the temporal bones demonstrated opacification of the left mastoid and middle ear, whereas a CT scan of the brain demonstrated patent venous sinuses. A left canal-waltdown mastoidectomy was performed. Intraoperatively, a cholesteatoma of the mastoid and middle ear was found. The sigmoid sinus was dehiscent superiorly from the sinodural angle to the jugular bulb inferiorly. All of the bone covering the sigmoid sinus and anterior to the sigmoid sinus was eroded by infected granulation tissue. Cultures demo~strated light growth of Streptococcus. She was discharged on the eighth postoperative day after 8 days of intravenous antibiotics. Eight days later, she returned to the emergency department with severe headaches. Her temperature was 100.6°F with a WBC Of 11,000/mm 3 and an ESR of 70 mm/hr. ACT scan of the brain demonslxated a ring enhancing lesion of the cerebellum noncontiguous with the mastoid or sigmoid sinus (Fig. 4). The CT scan also demonstrated partial thrombosis of the lef~ transverse and superior sagittal sinus. In the operating room, a left occipital craniotomy was performed, and the abscess was aspirated. Cultures of the aspirate revealed Streptococcus intelwwdius. She recovered without sequela and was discharged on the eighth postoperative day.

$136 MURPHY and BOYDSTON

OtolaryngologyHead and Neck Surgery December 1997

Fig. 3. MRA demonstrating thrombosis extending from the right transverse sinus to the right internal jugular vein. Fig, 4. CT scan of the brain demonstrating an abscess of the cerebellum, noncontiguous with the sigmoid sinus.

DISCUSSION Lateral sinus thrombophlebitis may be the result of acute mastoiditis or of a chronic infective process such as a cholesteatoma.l-4 Length of symptoms neither eliminates nor confirms the possibility of LST. Patients may have a long history of otologic disease as seen in patients 3 and 4, or LST may occur rapidly in patients with no history of otologic disease, as with patients 1 and 2. Delay in diagnosing LST may lead to pulmonary emboli, benign intracranial hypertension, and brain abscess. 3,4 Classic LST is seen with a spiking fever reaching 104 ° to 105 °F, known as a "picket fence" fever. This pattern was seen in patient 3.1 Although this may be found, a more indolent course and pattern of presentation is more common since the advent of antibiotics, as seen in patients 1 and 2. 2,3,5 Patients who have been treated with antibiotics may be afebrile or have a low-grade fever, such as that found in patients 1 and 2. Progressive anemia and emaciation in patients with undiagnosed LST, as seen in patients 3 and 4, are typical of patients with chronic infectious disease. Otologic examination of the middle ear may be unremarkable or consistent with an acute or subacute otitis media. Although postauricular swelling and erythema may be absent, postauricular tenderness, neck tenderness, headache, nausea, and vomiting are often present. 3 Griesinger's sign, with postauricular edema and swelling over the mastoid tip, may be present in patients who have undergone thrombosis of the mastoid emissary vein. 1'6 Neurologic examination including ophthalmic funduscopy is essential in patients suspected of having LST. Special attention to cranial nerve VI and the optic discs is essential. Detailed inspection for papilledema is required. History of blurred vision, headache, nausea, and vomiting is suggestive of increased intracranial pressure. 5 Increased intracranial pressure resulting from obliteration of the sigmoid sinus is commonly seen in patients with LST. These symptoms were present in patients 1 and 3.

Children with otologic disease, headache, nausea, and vomiting need careful evaluation for LST with further laboratory and radiographic testing. Laboratory tests, including CBC with differential, CRR and ESR are performed in patients with suspected LST and are used to confirm the presence of an infectious process. Elevated CRP and ESR are found in patients with acute infection. CRP returns to normal rapidly with improvement of the infection. ESR remains elevated after an infection for 6 to 8 weeks and slowly returns to the normal range. Elevated WBC is normally found in patients with LST but may be only mildly elevated in patients who have been receiving antibiotics and may not give a true indication of the life-threatening nature of LST. Radiographic testing includes a fine-cut CT scan of temporal bones with special attention to the sigmoid sinus. Dehiscence of the bone overlying the sigmoid sinus should be noted. CT scan of the brain with contrast is used to assess the patency of the sigmoid and transverse sinuses. Gadolinium-enhanced MRI of the brain and MRA also may be used to assess the patency of the involved sinuses and the internal jugular vein. Treatment of LST requires both antibiotic therapy and surgical removal of the infective tissue adjacent to the sigmoid sinus. Antibiotic therapy should be given for both anaerobes and aerobes in patients with evidence of chronic ear disease and cholesteatomas. 7 Patients with LST caused by acute mastoid disease should be treated with antibiotics that penetrate the blood/brain barrier and cover the most common bacteria responsible for otitis media. Surgery includes removal of all disease in the mastoid and all bone adjacent to the sigmoid sinus. Frequently a fenestration of the sigmoid sinus will be encountered with pus pulsating from the sinus. The sinus should be opened adequately to allow a free flow of the purulent material from the sinus. Total removal of the infected thrombus is not required, and free bleeding from the sinus is not a goal of surgery. A

OtolaryngologyHead and Neck Surgery Volume 117 Number 6

Penrose drain should be secured during closure of the postauricular wound to allow drainage of purulent material and instillation of antibiotic drops into the mastoid cavity. Ligation of the sigmoid sinus or jugular vein is performed only in patients with evidence of pulmonary emboli. Heparinization is not performed. Repeated CT scan performed 1 week after surgery will demonstrate partial resolution of the thrombus. Complete recanalization of the obliterated sinus may take from 6 to 8 weeks.

SlLLERS et at. S137

2. 3. 4. 5. 6.

REFERENCES 1. Pallares R, Santamaria J, Ariza X, Gudiol F. Polymicrobial

7.

anaerobic septicemiadue to lateral sinus thrombophiebitis.Arch Intern Med I983;143:164-5. Holt GR, Gates GA. Masked mastoiditis. Laryngoscope 1983; 93:1034-7. Gower D, McGuirt WE Intracranialcomplicationof acute and chronic infectious ear disease: a problem stilI with us. Laryngoscope 1983;93:t028-33. Shoed WE Lateral sinus thrombosis.Am J Otol 1983;4:258-62. Sismanis A. Otologic manifestations of benign intracranial hypertension syndrome: diagnosis and management.Lapyngoscope 1987;97(Suppl42):1-17. NissanAJ. Intracranialcomplicationsof otogenic disease. ~rn J Otol 1980;2:164-7. TeichgraeberJF, Per-Lee JH, Turner JS. Lateral sinus thrombosis: a modem perspective. Laryngoscope 1982;92:744-51.

Transconjunctival endoscopic orbital decompression MICHAEL J. SILLERS, MD, CARLOS CUILTY-SILLER,MD, FREDERICK A. KUHN, MD, EDWARD S. PORUBSk'Y, Mr), and JAMES F. NIORPETH, MD, Augusta,Georgia

G r a v e s ' disease is a multisystem disorder characterized by thyroid glandular hyperplasia, infiltrative dermopathy, and ophthalmopathy. The orbital involvement is variable, and its natural history unpredictable] The pathophysiology of orbitopathy is believed to involve the deposition of immune complexes in the extraocular tissues. 2 This produces an inflammatory response and subsequent edema and fibrosis of the extraocular muscles and orbital fat. There is also mucopolysaccharide deposition by fibroblasts, which leads to further increases in orbital volume.3 As a result of increased orbital volume, malignant exophthalmos wiI1 develop in 2% to 4% of patients with Graves' disease, the manifestations of which include proptosis, exposure keratopathy, diplopia, and optic neuropathy resulting in decreased visual acuity or visual field defects. 4,5 Nonsurgical therapy has focused primarily on the use of high-dose steroids and radiotherapy. Prednisolone in dosages of 120 rag/day is given for several months, and often the side effects of therapy are unacceptablel6 Fractionated radiotherapy (20 Gy for 2 weeks) has been delivered to treat optic neuropathy but does not significantly reduce proptosis. 7 Plasmapheresis has been used to wash immunoglobulinsfrom the patient's system, but the improvement is temporary. 8,9 Cyclosporin A was used successfully in a single study) ° Orbital decompression for Graves' disease has been

From the Department of Surgery, Division of Otolaryngology-Head and Neck Surgery, Medical College of Georgia. Presented at the American Rhinologic Society, Palm Beach, Fla., May 7, 1994. Reprint requests: Michael J. Sillers, MD, 1501 5th Ave. South, Birmingham,AL 35233. Otolaryngol Head Neck Surg 1997;117:S137-S141. Copyright © 1997 by the American Academy of OtolaryngologyHead and Neck Surgery Foundation,Inc. 0194-5998/97/$5.00 + 0 23/4/75138

accomplished through a variety of surgical approaches. Each technique focuses on removing one or more of the four walls that make up the boundaries of the orbit, tn 19ll Dollinger decompressed the orbital contents into the infratemporal fossa through a lateral orbitotomy described by Kronlein in 1888. Naffziger performed a superior wall decompression through a frontal craniotomy in 1928o and Hirsh removed the orbital floor in 1930. Sewell proposed an external frontoethmoidectomy for medial decompression in 1936.11 Watsh and Ogura H introduced a two-wall inferior and medial decompression through a Caldwell-Luc maxillary antrostomy in 1948. This transantral approach has remained the most commonly used method for otolaryngologists since that Lime. However. in /990 Kennedy et al. t2 described a transnasaI endoscopic technique for removal of the medial wall and the medial orbital floor with reduction in proptosts comparable with that found

Fig. 1. Left eye demonstrating transconjunctival incision. Corneal protector is in place.