Otogenic lateral sinus thrombosis in children: a review of 7 cases.
Otogenic lateral sinus thrombosis (LST) is a rare but serious intracranial complication of acute or chronic otitis media. Reported mortality rates have ranged from 8 to 25%; the pediatric mortality rate might be as low as 5%. Controversy still exists over the medical and surgical management of this condition. We conducted a retrospective chart review of 7 cases of pediatric otogenic LST that were treated at our institution over a period of 8 years. We hypothesized that good outcomes in very sick patients can beachievedby aggressively managing the mastoid cavity and without the need for a thrombectomy. Our study group was made up of 4 boys and 3 girls, aged 6 to 15 years (mean: 11.1). All patients received intravenous antibiotics and underwent mastoidectomy with unroofing of the sigmoid sinus and placement of a tympanostomy tube. Sinus exploration with thrombectomy was not performed in any patient. Anticoagulation was used perioperatively in 5 patients (71%) without complication. All patients recovered well without major sequelae, which supports our hypothesis. We also describe the case of a patient with multiple concomitant intracranial comorbidities associated with this rare condition.
Otogenic lateral sinus thrombosis (LST) is a rare and serious intracranial complication of acute or chronic otitis media. Reported mortality rates in most larger case series have ranged from 8 to 25%,14 but the pediatric mortality rate may be as low as 5%. (5)
Controversy still exists over the medical and surgical management of this condition. Management has traditionally included intravenous antibiotic therapy and mastoidectomy with thrombus removal. (6-10) Anticoagulation is used perioperatively in some cases, and its use continues to be an area of debate. Internal jugular vein ligation, historically a common part of surgical intervention, is no longer routinely employed. (11-13)
In this article, we present our small, descriptive case series of children with otogenic LST. We hypothesized that good outcomes in very sick patients can be achieved by aggressively managing the mastoid cavity and not performing thrombectomy. We also describe a case that involved multiple concomitant intracranial comorbidities associated with this rare condition.
Patients and methods
We conducted a retrospective chart review of all cases of pediatric otogenic LST that had been treated at our institution over a period of 8 years. We compiled information on presentation, coexisting intracranial complications, treatment, cultured organisms, and outcomes.
Our study group was made up of 4 boys and 3 girls, aged 6 to 15 years (mean: 11.1) (table 1). The most common presenting signs and symptoms were fever in 5 patients, otalgia in 5, and mastoid tenderness, nuchal rigidity, and otorrhea in 3 (table 2). Coexisting intracranial complications were present in 4 patients (57%), the most common of which were epidural abscess in 2 patients and otitic hydrocephalus in 2 (table 3).
All patients had been treated with intravenous antibiotics, and all had undergone mastoidectomy with unroofing of the sigmoid sinus and placement of a tympanostomy tube. Sinus exploration with thrombectomy was not performed in any patient. Anticoagulation was used perioperatively in 5 patients (71%) without complication. The most common organisms isolated were Streptococcus spp (table 1).
Institutional review board approval was obtained for the study protocol.
After treatment, all patients recovered well, and none experienced a major sequela. One patient with cavernous sinus thrombosis and otitic hydrocephalus experienced a persistent right visual-field deficit.
A10-year old girl (patient 6) presented to the emergency room with a history of blurred vision and ataxia. On physical examination, she was noted to have papilledema and right-sided otorrhea. Computed tomography (CT) demonstrated mastoid cavity and ethmoid sinus opacification on the right. A triangle of contrast-enhanced dura was seen at the level of the sigmoid sinus, which represents the so-called delta sign (figure 1). Magnetic resonance venography (MRV) demonstrated right sigmoid and transverse sinus thrombosis, right proximal internal jugular vein thrombosis, and bilateral cavernous sinus thrombosis (figure 2).
The patient underwent an urgent right tympanomastoidectomy with unroofing of the sigmoid sinus, rightsided endoscopic sinus surgery, and a bilateral myringotomy with tube placement. In addition to intravenous antibiotics, she was anticoagulated postoperatively.
The patient recovered well, but she was left with a persistent right visual-field deficit. Repeat MRV 2 months postoperatively showed a persistent thrombosis. An MRV obtained 9 months after surgery demonstrated improved flow on the right side, but the thrombosis had not fully resolved.
Otogenic LST as a complication of acute and chronic otitis media is believed to occur via one of two mechanisms. (1,4,11,14,15)
* Acute or chronic otitis media can cause erosion of the bone covering the sigmoid sinus, which then results in inflammation of the outer and inner walls of the sinus, which in turn eventually leads to thrombus formation.
* Thrombus formation occurs as a result of osteothrombophlebitic extension via small venules.
The clinical signs and symptoms of otogenic LST include high-grade fever, otalgia, mastoid tenderness, neck stiffness, otorrhea, papilledema, and headache." (14,16,17) "Picket fence" fevers are classically described but rarely seen.
A previous report of a case of LST in a child with congenital aural atresia illustrated the difficulties one can encounter in diagnosing this entity. (15) The diagnosis is based on a high degree of clinical suspicion, and it is generally confirmed with imaging studies. CT with contrast demonstrates temporal bone pathology, as well as any associated bony erosion. An empty triangle at the level of the sigmoid sinus surrounded by contrast-enhanced dura (the delta sign) is diagnostic.
Magnetic resonance imaging (MRI) is considered to be more sensitive than CT in detecting sigmoid sinus thrombosis. The clot appears initially as hypointense on T2-weighted imaging, and then it transitions to hyperintense on T1 and T2 weighting, which reflects the characteristics of methemoglobin. MRV demonstrates an absence of flow in the affected sinus.
MRI allows for noninvasive evaluation of the brain for detecting associated swelling, ischemia, hemorrhage, or inflammation. Improvements in imaging techniques and the increased availability of MRI have dramatically reduced the need for invasive cerebral angiography. (5,18,19)
Management of LST has traditionally included broad-spectrum intravenous antibiotics, tympanostomy tube placement, and mastoid surgery with opening of the sinus and removal of the thrombus after needle aspiration to confirm the diagnosis. (6-10) Perioperative anticoagulation is used in selected cases.
Few reports have specifically addressed the role of anticoagulation. Bradley et al suggested that patients with a thrombus confined to the sigmoid sinus may be considered for treatment without anticoagulation in order to avoid its associated risks, but they advised that patients with evidence of thrombus progression or extension may benefit from systemic anticoagulation; the decision should be made on a case-by-case basis. (20)
Shah et al urged caution regarding the use of perioperative anticoagulation in view of the increased risk of bleeding-related complications. (21) In a recent review of case series in the literature over a 20-year period, Au et al recommended management with a broad-spectrum antibiotic and surgical removal of all perisinus infection. (22) The role of anticoagulation was unclear.
Internal jugular vein ligation was originally recommended in the preantibiotic era as a way to prevent septic emboli and thrombus extension, but it is now reserved for rare cases of thrombus extension, infection and thrombosis of the internal jugular vein, and persistent septicemia and pulmonary complications despite initial medical and surgical management. (11-13)
In recent years there has been a trend toward more conservative surgical management of LST, and several other authors have reported successful outcomes without performing thrombectomy as part of the surgical intervention. Kutluhan et al described 4 cases of LST in which 2 patients were successfully managed with sinus aspiration during surgery to confirm thrombosis; no further intervention was required. (17) Agarwal et al presented the case of a 3-year-old girl with otogenic LST who was treated with mastoidectomy. (23) In that case, the sinus was neither aspirated nor opened. The thrombus was monitored by ultrasonography via a window in the parieto-occipital bone, and recannulation of the transverse and sigmoid sinuses was demonstrated at week 5. Anticoagulation was not used.
In the report by Bradley et al, the authors reviewed their experience with 9 patients with otogenic LST; 7 of those patients underwent mastoidectomy, while only 2 had the sigmoid sinus opened. (20) Ooi et al reported on the management of 4 cases of otogenic LST in patients between the ages of 13 and 25 years. (12) All of them underwent surgical intervention with mastoidectomy. Thrombectomy was performed in 2 of 4 patients, and anticoagulation was used in 1. The authors recommended always removing the sinus plate, and they concluded that conservative surgical treatment of the sinus did not seem to affect the overall prognosis in their small series.
Syms et al reported 6 cases of otogenic LST in which all patients underwent surgical intervention with mastoidectomy, exposure of the lateral sinus, and needle aspiration to confirm the diagnosis. (16) No sinus exploration or thrombectomy was performed, and no therapeutic anticoagulation was used.
Singh described the management of 36 cases of otogenic LST in South Africa; 33 of these patients were younger than 20 years. (24) All 36 patients were treated with intravenous antibiotics and exploratory mastoidectomy. The diagnosis was confirmed at the time of surgery by direct observation or sinus aspiration. Thrombectomy was not performed in any patient. Nine patients did not respond to initial therapy because of resistant bacteria, but all of them responded to a change in antibiotic regimen. No deaths were reported in this series.
Christensen et al (25) and Bales et al (26) reported their respective institution's experience with otogenic LST. Christensen et al obtained good results in 7 patients treated with intravenous antibiotics, simple mastoidectomy with unroofing of the sigmoid sinus, and tympanostomy tube placement. (25) Bales et al reported good outcomes in 13 patients with intravenous antibiotics, simple mastoidectomy, tympanostomy tube placement and, in most patients, anticoagulation. (26)
There have been additional recent reports of successful nonsurgical management in highly selected cases. Tov et al reported the case of a 5-year-old boy with otogenic LST who demonstrated a prompt response to intravenous antibiotics, and who therefore was successfully treated without surgical intervention. (18) Wong et al described 3 cases in which patients were managed conservatively with tympanostomy tube placement and antibiotics, thereby avoiding mastoidectomy. (5) They recommended intravenous antibiotics, drainage of the middle ear with tympanostomy tube placement, and close monitoring, with mastoidectomy being reserved for refractory cases only.
Finally, Garcia et al presented a case of otogenic TST that was successfully treated with intravenous antibiotics, tympanostomy tube placement, and anticoagulation. (11) Gradual resolution of the thrombus was observed on serial imaging studies. Garcia et al also reviewed the literature on pediatric otogenic TST from 1960 to 1995 and found that 95% of 58 patients had undergone surgical intervention. They suggested that the medical therapy they described can be sufficient in highly selected cases.
In conclusion, our study demonstrated good outcomes with emergent treatment of otogenic TST with mastoidectomy and unroofing of the sigmoid sinus, tympanostomy tube placement, intravenous antibiotics, and selective anticoagulation. We did not find throm bectomy necessary, which supports the trend toward more conservative surgical management.
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Jesse T. Ryan, MD; Maria Pena, MD; George H. Zalzal, MD; Diego A. Preciado, MD, PhD
From the Department of Otolaryngology and Communication Sciences, Upstate University Hospital, State University of New York, Syracuse (Dr. Ryan); and the Division of Pediatric Otolaryngology-Head and Neck Surgery, Childrens National Medical Center, Washington, D.C. (Dr. Pena, Dr. Zalzal, and Dr. Preciado). The study described in this article was conducted at the Children's National Medical Center.
Corresponding author: Jesse T. Ryan, MD, Department of Otolaryngology and Communication Sciences, Upstate University Hospital, Campus West Bldg., 750 E. Adams St., Room 241, Syracuse, NY 13210. Email: email@example.com Results
Table 1. Summary of presenting symptoms, intracranial complications, treatments, organisms cultured, and outcomes Pt. Age/sex Presentation Intracranial complications 1 11/M Fever, otalgia, right Right lateral, transverse, nuchal rigidity, and internal jugular vein frontal headache thrombosis, left trans- verse sinus thrombosis 2 8/M Fever, otalgia, Left lateral sinus mastoid thrombosis, left perisinus tenderness epidural abscess 3 15/M Fever, otalgia, Right lateral and transverse mastoid sinus thrombosis, internal tenderness jugular vein thrombosis 4 15/M Fever, otalgia, nuchal Right sigmoid sinus pain and rigidity, thrombosis, meningitis otorrhea headache 5 6/F Ophthalmoplegia Bilateral lateral sinus and papilledema thrombosis, right internal present for 4 days jugular vein thrombosis, after discharge from bilateral posterior fossae initial hospitalization epidural abscesses, right middle fossa epidural abscess, otitic hydrocephalus 6 10/F Blurred vision, Right sigmoid and transverse ataxia, papilledema, sinus thrombosis, right right-sided otorrhea proximal internal jugular vein thrombosis, bilateral cavernous sinus thrombosis, otitic hydrocephalus 7 13/F Fever, otalgia, Right sigmoid and mastoid tenderness, transverse sinus thrombosis, nuchal rigidity, right internal jugular otorrhea, altered vein thrombosis with mental status, nausea, extension into the right vomiting, diarrhea innominate vein Pt. Treatment Organism Outcome 1 Right simple mastoidectomy, -- No sequelae right myringotomy with tube placement, right maxillary sinus irrigation, anticoagulation 2 Left complete mastoidectomy No growth No sequelae with drainage of perisinus epidural abscess, left myring- otomy with tube placement 3 Right simple mastoidectomy, Streptococcus No sequelae right myringotomy with tube pneumoniae placement 4 Right simple mastoidectomy, Group A No sequelae right myringotomy with tube streptococci placement, anticoagulation 5 Bilateral complete mastoid- No growth No sequelae ectomies with drainage of abscesses, anticoagulation, bilateral myringotomy with tube placement 6 Right tympanomastoidectomy, No growth Right visual- right endoscopic sinus field deficit surgery, bilateral myringotomy with tube placement, anticoagulation 7 Bilateral myringotomy with Proteus No sequelae tube placement, incision mirabilis, and drainage of subperiosteal Corynebacterium abscess, staged tympano- spp mastoidectomy (delayed due to low hematocrit), anticoagulation Table 2. Most common presenting signs and symptoms (N = 7) Fever 5 Otalgia 5 Mastoid tenderness 3 Nuchal rigidity 3 Otorrhea 3 Papilledema 2 Headache 2 Table 3. Coexisting intracranial complications (N = 7) Epidural abscess 2 Otitic hydrocephalus 2 Meningitis 1 Cavernous sinus thrombosis 1
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|Title Annotation:||ORIGINAL ARTICLE|
|Author:||Ryan, Jesse T.; Pena, Maria; Zalzal, George H.; Preciado, Diego A.|
|Publication:||Ear, Nose and Throat Journal|
|Article Type:||Clinical report|
|Date:||Mar 1, 2016|
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