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A pearl in the ear: Intracranial complications of pediatric cholesteatomas
Accepted Manuscript A pearl in the ear: Intracranial complications of pediatric cholesteatomas Krystyne Basa, Jessica R. Levi, Erin Field, Robert C. O'Reilly PII:
S0165-5876(16)30407-4
DOI:
10.1016/j.ijporl.2016.11.009
Reference:
PEDOT 8316
To appear in:
International Journal of Pediatric Otorhinolaryngology
Received Date: 22 September 2016 Revised Date:
7 November 2016
Accepted Date: 9 November 2016
Please cite this article as: K. Basa, J.R. Levi, E. Field, R.C. O'Reilly, A pearl in the ear: Intracranial complications of pediatric cholesteatomas, International Journal of Pediatric Otorhinolaryngology (2016), doi: 10.1016/j.ijporl.2016.11.009. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
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A Pearl in the Ear: Intracranial Complications of Pediatric Cholesteatomas
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Krystyne Basa, BAa,b; Jessica R. Levi, MDa,b; Erin Field, MMS, PA-Cc; Robert C. O’Reilly,
Boston Medical Center, 1 Boston Medical Pl, Boston, MA 02118; bBoston University School of
Children, 1600 Rockland Rd, Wilmington, DE 19803
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Correspondence:
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Medicine, 72 E. Concord St, Boston, MA 02118; cNemours/Alfred I. duPont Hospital for
Robert C. O’Reilly, MD
Nemours/Alfred I. duPont Hospital for Children PO Box 269
Tel: 302-651-5829 Fax: 302-651-6410
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[email protected]
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Wilmington, DE 19899
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Conflicts of Interest: None.
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
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A Pearl in the Ear: Intracranial Complications of Pediatric Cholesteatomas
Abstract
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A nine-year-old male had a cholesteatoma of the mastoid and middle ear found incidentally after myringotomy tube placement. Associated asymptomatic sigmoid plate dehiscence with sinus invasion or thrombosis and ossicular chain destruction complicated his case. He had canal wall
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down tympanomastoidectomy and was followed for 4.5 years. Disease recurrence necessitated revision. Our case highlights an unusual clinical presentation, possible complications, and the
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aggressive quality of a benign lesion common in the pediatric population. To our knowledge, this is the first report of an asymptomatic lateral sinus obstruction secondary to an invasive cholesteatoma in this population.
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thrombosis
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Key words: sigmoid sinus, dural sinus thombus, cholesteatoma, pediatric, lateral sinus
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1.1 Introduction Cholesteatomas are keratinized lesions found within the external ear canal, middle ear, mastoid and petrous bone. The annual worldwide incidence of cholesteatomas in the pediatric population
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is three per 100,000 [1]. There are two types: congenital and acquired [1-4]. Congenital
cholesteatomas are pearly white, usually asymptomatic, masses found in the anterior superior quadrant of the tympanic membrane [2]. Acquired cholesteatomas present with tympanic
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membrane abnormalities, typically a retraction pocket, associated otorrhea, and recurrent otitis
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media [2].
Regardless of origin, cholesteatomas are typically slow growing and can be destructive. Pediatric cholesteatomas are considered more aggressive than those found in adults [3,4]. Presenting
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symptoms can include conductive hearing loss, facial nerve weakness, and imbalance [5].
Here, we present a case of an extensive right-sided cholesteatoma extending into the middle ear with associated ossicular chain destruction, sigmoid plate dehiscence, and sigmoid sinus
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extension. To our knowledge, this is the first pediatric case of an asymptomatic sigmoid sinus
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obstruction secondary to an invasive cholesteatoma.
2.1 Case Report
A nine-year-old male with a history of recurrent bilateral otitis media presented to our clinic for evaluation of a known right-sided cholesteatoma. At an outside hospital, the child underwent bilateral myringotomy and tympanostomy tube placement. At the postoperative visit, the child had an abnormal right tympanic membrane. Computed tomography showed the middle ear was
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completely opacified with soft tissue, concerning for a cholesteatoma. There was erosion of the mastoid air cells as well as much of the bone over the sigmoid. He subsequently was referred to
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our institution.
On presentation to our institution, his symptoms included intermittent otorrhea from his right tube. He denied vertigo and facial weakness. On audiologic evaluation, he had a right-sided
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conductive hearing loss. Physical exam was notable for a smoothly marginated area of debris adjacent to the right tympanic membrane, which was completely opaque. A tympanostomy tube
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was visible and in place; however, there were no visible middle-ear landmarks. We obtained an MRI (Figures 1, 2) to determine the extent of the disease and to rule-out an erosive process beginning from the posterior fossa since there was evidence of sigmoid plate erosion on CT.
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Initial MRI/MRV showed a soft-tissue mass in right middle ear and within the mastoid air cells. This mass was T2 hyperintense, and it measured approximately 2.4 x 0.8 x 2.5 cm. There was also noted extension into the medial aspect of the right external auditory canal and the
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anterosuperior inner ear structures. There were similar soft-tissue findings in the distal right transverse and proximal sigmoid sinuses and a notable filling defect in this region on MRV, but
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there was no complete occlusion of the sinuses. This most likely reflected cholesteatoma extension into the venous sinuses. Interestingly, there was no clear connection between the middle ear mass and the venous sinus mass present on imaging.
He subsequently was referred to neurosurgery due to concerns about the extension of the mass into the dural sinuses. Since our patient’s presentation was unconventional, a biopsy was
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performed first. Pathology came back as a right-sided cholesteatoma and a tympanomastoidectomy was scheduled for a later date.
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2.2 Surgical Technique
The patient’s first procedure was a right middle ear exploration to obtain a tissue biopsy with removal of the incus. The patient had a large vascular granuloma at the 6-o’clock position, which
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was removed. The tympanic membrane was completely featureless and thickened. The previously inserted tympanostomy tube was seen in the anterior inferior quadrant. A
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tympanomeatal flap was elevated, and the middle ear space was entered inferiorly. In the middle ear space, there was balloon mucosa, and a cholesteatoma sac filled the middle ear cleft. Biopsies were taken of the middle ear mucosa over the promontory. The chorda tympani was wrapped entirely in the cholesteatoma sac. There was erosion of the lenticular process of the incus and
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head of the malleus and lack of the superstructure of the stapes. The chorda tympani and body of the incus were subsequently removed during this procedure. The chorda tympani was sent for
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specimen with the biopsied mucosa.
The postoperative pathology report was consistent with a cholesteatoma. Therefore,
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tympanomastoidectomy with neurosurgery available was planned for a later date.
For the second procedure, removal of the cholesteatoma, a canal wall down (CWD) tympanomastoidectomy approach was chosen. The tegmen and the dura posterior to the sigmoid sinus were carefully skeletonized, and the mastoid air cells were completely dissected to expose the cholesteatoma sac. The sac was entered and the matrix was carefully removed. Matrix found
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in the mastoid was carefully removed as well, along with the mastoid mucosa. The canal wall was then taken down, and all visible cholesteatoma was removed from the middle ear cleft.
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Intraoperatively, there was destruction of the mastoid air cells with extension into the subarcuate space and a largely dehiscent sigmoid, measuring up to 2.5 cm in length. The sigmoid was
rubbery to palpation, thought to be thrombosis within the dural sinus; however, there was no
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obvious direct extension of the cholesteatoma into the sigmoid itself. In addition, the middle ear space and mastoid were noted to be acutely infected. At this point, the neurosurgeon came in to
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visualize the sigmoid. Both he and the senior otolaryngologist mutually agreed that resection of the sigmoid would not be prudent, as the patient was asymptomatic. In addition, complete removal of the diseased area would have required a large resection.
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A fascial graft was taken, and a meatoplasty was performed. The superior aspect of the remaining tympanic membrane was resected, and the fascial graft was placed to create a “cavum minor.” Gelfoam (Pfizer, New York, NY) squares were used as support, and the remainder of the
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fascial graft was used to line the mastoid cavity. Surgiflo (Ethicon, Somerville, NJ) was used to fill the mastoid cavity in the middle ear space. The meatoplasty was then sutured opened.
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Postauricular wound closure was achieved in three layers.
2.3 Results
The patient was followed by Otolaryngology and Neurosurgery for 4.5 years after his initial CWD tympanomastoidectomy. A planned MRI was obtained 3.5 months after surgery, which showed stable soft tissue/filling defect of the sigmoid. Nine months after the procedure, he began
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to complain of short-term memory problems, intermittent headaches, visual disturbances, and imbalance. Neurosurgery continued to follow him for these complaints and diagnosed him with migraines. However, given the history of sigmoid sinus thrombosis, an MRI/MRV (Figures 3, 4)
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was obtained nine months after his CWD tympanomastoidectomy. Clinically, he had developed some narrowing of the meatoplasty and adhesions over the mastoid bowl. His images showed growth of a recurrent mastoid cholesteatoma but stable sigmoid findings with no interval growth
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intracranially. Because of evidence of disease recurrence, he underwent a revision of the rightsided meatoplasty and cleaning of the mastoid bowl 17 months after his initial
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tympanomastoidectomy. During the time of the revision surgery, cholesteatoma was found filling the mastoid. There was also notable new bone growth over the sigmoid sinus. All visible cholesteatoma again was removed. Serial MRI/MRVs were continued to monitor for recurrence. The patient was followed for 38 months from the date of his revision and 4.5 years from his
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initial tympanomastoidectomy. He had a benign postoperative course. His last MRV, which was 13 months after his revision, showed a stable “5-mm residual soft-tissue component noted in the mesotympanum of the right middle ear cavity and persistent cholesteatoma in the right
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transverse/sigmoid sinuses, resulting in partial filing defect, not significantly changed from the prior study.” His most recent visit showed no right-sided otologic symptoms or complications.
sinus.
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His physical exam showed a clean canal wall down bowel and no further changes to the sigmoid
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3.1 Discussion Derlacki and Clemis characterized the modern description of a cholesteatoma in 1965: they described a pearly white mass in the middle ear, behind the tympanic membrane [6]. Congenital
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cholesteatomas appear as “pearls” in the middle ear space, usually in the anterosuperior quadrant. They are usually asymptomatic and develop from embryonic rest of epithelium [7]. Acquired cholesteatomas usually develop from a history of recurrent middle ear disease [2]. They are
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associated with a retraction of the pars flaccida into Prussak’s space and are thought to be more erosive [7]. While exact classification of our patient’s cholesteatoma is difficult to assign, our
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patient’s long history of bilateral otitis media, the presence of otorrhea in clinic, and presence of debris adjacent to the tympanic membrane on exam makes an acquired cholesteatoma the more likely diagnosis. It is also possible that epithelial cells were introduced into the middle ear at the time of tube placement, leading to formation of a cholesteatoma. His case was complicated by
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the unique finding of a sigmoid sinus thrombosis or likely extension of soft tissue from the middle ear into the sigmoid and transverse sinuses.
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3.2 Diagnosis
Our patient’s cholesteatoma was identified after a myringotomy at an outside institution, making
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it unclear whether or not typical tympanic membrane characteristics (a perforation or retraction with squamous debris) were present at the time of diagnosis. There is also evidence in the literature that tympanostomy tube placement may cause secondary cholesteatomas in approximately 1% of cases [8-10]. Our patient’s history of prior tube placement makes delineating the etiology of his cholesteatoma difficult.
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His delay in diagnosis and presenting symptomatology differed from the typical cholesteatoma case. Clinical suspicion of a cholesteatoma and work-up of the mass was not pursued until after a postoperative follow-up visit. Asymptomatic cases of acquired cholesteatoma typically have
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tympanic membrane retractions, termed attic retractions, on exam [2]; our patient’s tympanic abnormalities were complete opacification, inflammation, and adjacent debris. He did present with otorrhea, which is the most common presenting symptom of acquired cholesteatomas [2];
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however, this may be postsurgical otorrhea rather than a result of his cholesteatoma.
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His delay in diagnosis likely allowed for extensive local bony destruction, including ossicular damage and violation of the dural space. Our patient’s case highlights the aggressive growth associated with acquired cholesteatomas, particularly pediatric cholesteatomas. Dornelles et al discuss the controversies regarding the behavioral differences between adult and pediatric
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acquired cholesteatomas [1]. They found that presence of metalloproteinases, associated with tissue destruction, were greater in the pediatric study population, indicating a more aggressive process. The literature also describes higher recidivism rates in pediatric cholesteatomas
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[1,11,12]; our patient’s mass recurred in less than one year. We determined two likely etiologies for our patient’s asymptomatic sigmoid sinus occlusion: a lateral sinus thrombosis or, more
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likely, direct dural extension of the cholesteatoma.
3.3 Lateral Sinus Thrombosis Smith and Danner [13] characterized the various complications associated with chronic otitis media and cholesteatomas, including lateral sinus thrombosis (LST). They found that, in cases of secondary LSTs, infectious etiologies tend to be the causative agents in young children; whereas,
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thrombosis due to cholesteatomas was more likely in adults. There is a paucity of literature regarding the relationship between pediatric cholesteatomas and LSTs. To our knowledge, this is the first report of an asymptomatic sinus thrombosis secondary to a cholesteatoma in the
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pediatric literature.
In children, the literature describing LSTs focuses almost exclusively on its association with
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otogenic infections [14,15]. LSTs also have been generally symptomatic in the literature. A review of 104 pediatric patients describes the presence of at least one sign or symptom of
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increased cranial pressure (such as headache or vomiting) on presentation [16]. This was consistent with other case series in the pediatric literature [15,17,18], all attributing etiology to current or recent otogenic infections.
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There are no reports of asymptomatic LST in pediatric patients due to cholesteatoma, though there are some in the adult literature. Neto et al [19] described a pediatric lateral sinus thrombosis where a cholesteatoma was the causative agent, but the patient had symptoms of
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increased cranial pressure (altered mental status, nausea, vomiting, and fever), similar to cases
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involving acute or recent infections.
In our case, serial MRI/MRVs showed a significant filling defect in the right sigmoid and transverse venous sinuses, but our patient never showed symptoms of increased intracranial pressure secondary to venous congestion, despite it being the dominant sinus. This is likely due to slow occlusion of the sinus over time, likely by direct extension. Interestingly, there was no clear evidence of this on imaging or intraoperative exam. It was not until disease recurrence nine
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months after initial presentation that our patient complained of any neurologic symptoms. Our patient’s initial presentation was isolated to middle ear symptoms—otorrhea and conductive hearing loss—despite evidence of disease extension into the dural sinuses on imaging. The
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asymptomatic nature of our patient’s thrombus shows that a complication with potential for high morbidity and mortality can remain clinically undetected. In our case, imaging was crucial in
clinical follow-up were important in managing our patient.
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detecting an uncommon complication. Initial imaging, serial follow-up imaging, and close
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Our patient’s young age at the time of presentation was also unusual for a case involving an LST secondary to a cholesteatoma. In a retrospective case series of 12 patients performed by Manolidis and Kutz Jr [14], 50% (6/12) of the LSTs were secondary to acute otitis media; the remaining were caused by chronic otitis media. Of the acute otitis media cases, all were under
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age 14 years, further confirming the association between acute infectious etiologies and LST in the pediatric population [14]. There is a greater prevalence of LST cases associated with chronic otitis media and subsequent cholesteatoma formation in the adult population than in the pediatric
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population; however, due to the small number of total cases and unclear distinctions of what ages are pediatric, this was not statistically significant and was difficult to interpret [14,15,20,21]. The
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youngest age of a patient with a cholesteatoma as the etiology of LST in these studies was 13 years [20]. Our patient was younger—nine years—at initial presentation.
3.4 Direct Dural Extension Pediatric cholesteatomas are usually associated with local extension to the mastoid or ossicles [22]. In a study by Farah et al [22], 57% of cases extended to the mastoid, and 50% of 26 cases
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showed ossicular destruction; they did not, however, describe any cases involving intracranial spread [22]. Scales developed to objectively quantify the extent of destruction caused by a growing cholesteatoma—such as the Austin-Kartush Classification Score—use only middle ear
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structures as benchmarks of disease severity [23,24]. Our patient had evidence of sigmoid sinus dehiscence and occlusion in addition to mastoid extension on imaging without intracranial
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symptoms.
Other case series looking at the direct intracranial invasion of cholesteatomas seem to link those
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originating in the mastoid with a greater likelihood of intracranial spread [25,26]. Our patient’s cholesteatoma likely originated in the middle ear secondary to his recurrent acute otitis media; his lack of symptoms allowed for extensive local growth, leading to sigmoid invasion despite his
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cholesteatoma originating outside of the mastoid cavity.
Mathews et al [27] found that, in cases of acute mastoiditis requiring surgery, there was a peak incidence of lateral sinus pathology in children aged 11–15 years, with all cases being secondary
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to invasive cholesteatomas. This represented 86% of all lateral sinus pathology [27]. All of the patients at least presented with pyrexia and otalgia, and 34% had some neurological symptom,
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such as cranial nerve palsy, ataxia, or altered mental status [27]. Our patient denied any similar symptoms and reported only intermittent otorrhea (possibly due to ear tubes).
In an adult case of a large cholesteatoma presenting with isolated hearing loss, the patient’s mass extended from the skull base to the upper part of the neck, with radiological evidence of vascular invasion of the jugular bulb and the sigmoid sinus [28]. His history showed progressive hearing
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loss since childhood, which was exacerbated with recurrent episodes of acute otitis media; he did not present for evaluation of his cholesteatoma until his seventh decade of life [28]. Given the time between the start of his symptoms and his presentation, he was able to acclimate to the
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growing mass such that he did not experience mass effect sequelae. Our patient did not have this length of time, but nonetheless was asymptomatic. His clinical picture and initial exam raised no
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concerns about an invasive mass until after imaging.
4.1 Conclusion
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Our case shows that extensive disease may occur with relatively minimal signs and symptoms, making the diagnosis of these lesions difficult. Two components made diagnosing this patient complex: his young age at presentation and his lack of intracranial symptoms. In addition, his prior history of tympanostomy tubes makes it difficult to fully determine the etiology of his
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cholesteatoma and to classify it as acquired or congenital.
Regardless of classification, early diagnosis is important for both types because the aggressive
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growth of pediatric cholesteatomas and their destruction of local structures can lead to significant morbidity and mortality. This case also describes how extensive and severe complications of
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cholesteatomas—LSTs and direct invasion of the dural space—can present, particularly in an asymptomatic patient.
This case, to our knowledge, is the first case of an asymptomatic sigmoid sinus obstruction secondary to an acquired cholesteatoma in the pediatric population. This report demonstrates that a severe complication may be clinically silent and present at an earlier age than previously
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described. This highlights the importance of imaging and high clinical suspicion in the diagnosis
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and management of invasive cholesteatomas in the pediatric population.
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[25] F.M. Warren, M.L. Bennett, R.H. Wiggins 3rd, K.L. Saltzman, K.S. Blevins, C. Shelton, H.R. Harnsberger, Congenital cholesteatoma of the mastoid temporal bone, Laryngoscope 117
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[26] J.H. Lee, S.J. Hong, C.H. Park, S.H. Jung , Congenital cholesteatoma of mastoid origin, J. Laryngol. Otol. 121 (2007) e20. [27] T.J. Mathews, Lateral sinus pathology (22 cases managed at Groote Schuur Hospital), J. Laryngol. Otol. 102 (1988) 118–120.
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[28] D. Cazzador, N. Favaretto, E. Zanoletti, A. Martini, Combined surgical approach to giant cholesteatoma: a case report and literature review, Ann. Otol. Rhinol. Laryngol. 125 (2016) 687–
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Figure Legends Figure 1. T2-weighted axial non-contrast MRI with hyperintense right-sided mass Figure 2. T2-weighted coronal MRI with contrast showing right-sided hyperintense mass
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Figure 3. T2-weighted coronal MRI with contrast showing recurrence of right-sided mass
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Figure 4. T2-weighted axial MRI without contrast showing right-sided soft-tissue mass
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S0165-5876(16)30407-4
DOI:
10.1016/j.ijporl.2016.11.009
Reference:
PEDOT 8316
To appear in:
International Journal of Pediatric Otorhinolaryngology
Received Date: 22 September 2016 Revised Date:
7 November 2016
Accepted Date: 9 November 2016
Please cite this article as: K. Basa, J.R. Levi, E. Field, R.C. O'Reilly, A pearl in the ear: Intracranial complications of pediatric cholesteatomas, International Journal of Pediatric Otorhinolaryngology (2016), doi: 10.1016/j.ijporl.2016.11.009. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
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A Pearl in the Ear: Intracranial Complications of Pediatric Cholesteatomas
MDc a
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Krystyne Basa, BAa,b; Jessica R. Levi, MDa,b; Erin Field, MMS, PA-Cc; Robert C. O’Reilly,
Boston Medical Center, 1 Boston Medical Pl, Boston, MA 02118; bBoston University School of
Children, 1600 Rockland Rd, Wilmington, DE 19803
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Correspondence:
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Medicine, 72 E. Concord St, Boston, MA 02118; cNemours/Alfred I. duPont Hospital for
Robert C. O’Reilly, MD
Nemours/Alfred I. duPont Hospital for Children PO Box 269
Tel: 302-651-5829 Fax: 302-651-6410
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[email protected]
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Wilmington, DE 19899
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Conflicts of Interest: None.
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
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A Pearl in the Ear: Intracranial Complications of Pediatric Cholesteatomas
Abstract
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A nine-year-old male had a cholesteatoma of the mastoid and middle ear found incidentally after myringotomy tube placement. Associated asymptomatic sigmoid plate dehiscence with sinus invasion or thrombosis and ossicular chain destruction complicated his case. He had canal wall
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down tympanomastoidectomy and was followed for 4.5 years. Disease recurrence necessitated revision. Our case highlights an unusual clinical presentation, possible complications, and the
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aggressive quality of a benign lesion common in the pediatric population. To our knowledge, this is the first report of an asymptomatic lateral sinus obstruction secondary to an invasive cholesteatoma in this population.
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thrombosis
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Key words: sigmoid sinus, dural sinus thombus, cholesteatoma, pediatric, lateral sinus
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1.1 Introduction Cholesteatomas are keratinized lesions found within the external ear canal, middle ear, mastoid and petrous bone. The annual worldwide incidence of cholesteatomas in the pediatric population
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is three per 100,000 [1]. There are two types: congenital and acquired [1-4]. Congenital
cholesteatomas are pearly white, usually asymptomatic, masses found in the anterior superior quadrant of the tympanic membrane [2]. Acquired cholesteatomas present with tympanic
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membrane abnormalities, typically a retraction pocket, associated otorrhea, and recurrent otitis
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media [2].
Regardless of origin, cholesteatomas are typically slow growing and can be destructive. Pediatric cholesteatomas are considered more aggressive than those found in adults [3,4]. Presenting
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symptoms can include conductive hearing loss, facial nerve weakness, and imbalance [5].
Here, we present a case of an extensive right-sided cholesteatoma extending into the middle ear with associated ossicular chain destruction, sigmoid plate dehiscence, and sigmoid sinus
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extension. To our knowledge, this is the first pediatric case of an asymptomatic sigmoid sinus
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obstruction secondary to an invasive cholesteatoma.
2.1 Case Report
A nine-year-old male with a history of recurrent bilateral otitis media presented to our clinic for evaluation of a known right-sided cholesteatoma. At an outside hospital, the child underwent bilateral myringotomy and tympanostomy tube placement. At the postoperative visit, the child had an abnormal right tympanic membrane. Computed tomography showed the middle ear was
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completely opacified with soft tissue, concerning for a cholesteatoma. There was erosion of the mastoid air cells as well as much of the bone over the sigmoid. He subsequently was referred to
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our institution.
On presentation to our institution, his symptoms included intermittent otorrhea from his right tube. He denied vertigo and facial weakness. On audiologic evaluation, he had a right-sided
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conductive hearing loss. Physical exam was notable for a smoothly marginated area of debris adjacent to the right tympanic membrane, which was completely opaque. A tympanostomy tube
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was visible and in place; however, there were no visible middle-ear landmarks. We obtained an MRI (Figures 1, 2) to determine the extent of the disease and to rule-out an erosive process beginning from the posterior fossa since there was evidence of sigmoid plate erosion on CT.
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Initial MRI/MRV showed a soft-tissue mass in right middle ear and within the mastoid air cells. This mass was T2 hyperintense, and it measured approximately 2.4 x 0.8 x 2.5 cm. There was also noted extension into the medial aspect of the right external auditory canal and the
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anterosuperior inner ear structures. There were similar soft-tissue findings in the distal right transverse and proximal sigmoid sinuses and a notable filling defect in this region on MRV, but
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there was no complete occlusion of the sinuses. This most likely reflected cholesteatoma extension into the venous sinuses. Interestingly, there was no clear connection between the middle ear mass and the venous sinus mass present on imaging.
He subsequently was referred to neurosurgery due to concerns about the extension of the mass into the dural sinuses. Since our patient’s presentation was unconventional, a biopsy was
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performed first. Pathology came back as a right-sided cholesteatoma and a tympanomastoidectomy was scheduled for a later date.
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2.2 Surgical Technique
The patient’s first procedure was a right middle ear exploration to obtain a tissue biopsy with removal of the incus. The patient had a large vascular granuloma at the 6-o’clock position, which
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was removed. The tympanic membrane was completely featureless and thickened. The previously inserted tympanostomy tube was seen in the anterior inferior quadrant. A
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tympanomeatal flap was elevated, and the middle ear space was entered inferiorly. In the middle ear space, there was balloon mucosa, and a cholesteatoma sac filled the middle ear cleft. Biopsies were taken of the middle ear mucosa over the promontory. The chorda tympani was wrapped entirely in the cholesteatoma sac. There was erosion of the lenticular process of the incus and
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head of the malleus and lack of the superstructure of the stapes. The chorda tympani and body of the incus were subsequently removed during this procedure. The chorda tympani was sent for
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specimen with the biopsied mucosa.
The postoperative pathology report was consistent with a cholesteatoma. Therefore,
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tympanomastoidectomy with neurosurgery available was planned for a later date.
For the second procedure, removal of the cholesteatoma, a canal wall down (CWD) tympanomastoidectomy approach was chosen. The tegmen and the dura posterior to the sigmoid sinus were carefully skeletonized, and the mastoid air cells were completely dissected to expose the cholesteatoma sac. The sac was entered and the matrix was carefully removed. Matrix found
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in the mastoid was carefully removed as well, along with the mastoid mucosa. The canal wall was then taken down, and all visible cholesteatoma was removed from the middle ear cleft.
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Intraoperatively, there was destruction of the mastoid air cells with extension into the subarcuate space and a largely dehiscent sigmoid, measuring up to 2.5 cm in length. The sigmoid was
rubbery to palpation, thought to be thrombosis within the dural sinus; however, there was no
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obvious direct extension of the cholesteatoma into the sigmoid itself. In addition, the middle ear space and mastoid were noted to be acutely infected. At this point, the neurosurgeon came in to
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visualize the sigmoid. Both he and the senior otolaryngologist mutually agreed that resection of the sigmoid would not be prudent, as the patient was asymptomatic. In addition, complete removal of the diseased area would have required a large resection.
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A fascial graft was taken, and a meatoplasty was performed. The superior aspect of the remaining tympanic membrane was resected, and the fascial graft was placed to create a “cavum minor.” Gelfoam (Pfizer, New York, NY) squares were used as support, and the remainder of the
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fascial graft was used to line the mastoid cavity. Surgiflo (Ethicon, Somerville, NJ) was used to fill the mastoid cavity in the middle ear space. The meatoplasty was then sutured opened.
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Postauricular wound closure was achieved in three layers.
2.3 Results
The patient was followed by Otolaryngology and Neurosurgery for 4.5 years after his initial CWD tympanomastoidectomy. A planned MRI was obtained 3.5 months after surgery, which showed stable soft tissue/filling defect of the sigmoid. Nine months after the procedure, he began
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to complain of short-term memory problems, intermittent headaches, visual disturbances, and imbalance. Neurosurgery continued to follow him for these complaints and diagnosed him with migraines. However, given the history of sigmoid sinus thrombosis, an MRI/MRV (Figures 3, 4)
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was obtained nine months after his CWD tympanomastoidectomy. Clinically, he had developed some narrowing of the meatoplasty and adhesions over the mastoid bowl. His images showed growth of a recurrent mastoid cholesteatoma but stable sigmoid findings with no interval growth
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intracranially. Because of evidence of disease recurrence, he underwent a revision of the rightsided meatoplasty and cleaning of the mastoid bowl 17 months after his initial
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tympanomastoidectomy. During the time of the revision surgery, cholesteatoma was found filling the mastoid. There was also notable new bone growth over the sigmoid sinus. All visible cholesteatoma again was removed. Serial MRI/MRVs were continued to monitor for recurrence. The patient was followed for 38 months from the date of his revision and 4.5 years from his
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initial tympanomastoidectomy. He had a benign postoperative course. His last MRV, which was 13 months after his revision, showed a stable “5-mm residual soft-tissue component noted in the mesotympanum of the right middle ear cavity and persistent cholesteatoma in the right
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transverse/sigmoid sinuses, resulting in partial filing defect, not significantly changed from the prior study.” His most recent visit showed no right-sided otologic symptoms or complications.
sinus.
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His physical exam showed a clean canal wall down bowel and no further changes to the sigmoid
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3.1 Discussion Derlacki and Clemis characterized the modern description of a cholesteatoma in 1965: they described a pearly white mass in the middle ear, behind the tympanic membrane [6]. Congenital
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cholesteatomas appear as “pearls” in the middle ear space, usually in the anterosuperior quadrant. They are usually asymptomatic and develop from embryonic rest of epithelium [7]. Acquired cholesteatomas usually develop from a history of recurrent middle ear disease [2]. They are
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associated with a retraction of the pars flaccida into Prussak’s space and are thought to be more erosive [7]. While exact classification of our patient’s cholesteatoma is difficult to assign, our
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patient’s long history of bilateral otitis media, the presence of otorrhea in clinic, and presence of debris adjacent to the tympanic membrane on exam makes an acquired cholesteatoma the more likely diagnosis. It is also possible that epithelial cells were introduced into the middle ear at the time of tube placement, leading to formation of a cholesteatoma. His case was complicated by
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the unique finding of a sigmoid sinus thrombosis or likely extension of soft tissue from the middle ear into the sigmoid and transverse sinuses.
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3.2 Diagnosis
Our patient’s cholesteatoma was identified after a myringotomy at an outside institution, making
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it unclear whether or not typical tympanic membrane characteristics (a perforation or retraction with squamous debris) were present at the time of diagnosis. There is also evidence in the literature that tympanostomy tube placement may cause secondary cholesteatomas in approximately 1% of cases [8-10]. Our patient’s history of prior tube placement makes delineating the etiology of his cholesteatoma difficult.
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His delay in diagnosis and presenting symptomatology differed from the typical cholesteatoma case. Clinical suspicion of a cholesteatoma and work-up of the mass was not pursued until after a postoperative follow-up visit. Asymptomatic cases of acquired cholesteatoma typically have
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tympanic membrane retractions, termed attic retractions, on exam [2]; our patient’s tympanic abnormalities were complete opacification, inflammation, and adjacent debris. He did present with otorrhea, which is the most common presenting symptom of acquired cholesteatomas [2];
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however, this may be postsurgical otorrhea rather than a result of his cholesteatoma.
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His delay in diagnosis likely allowed for extensive local bony destruction, including ossicular damage and violation of the dural space. Our patient’s case highlights the aggressive growth associated with acquired cholesteatomas, particularly pediatric cholesteatomas. Dornelles et al discuss the controversies regarding the behavioral differences between adult and pediatric
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acquired cholesteatomas [1]. They found that presence of metalloproteinases, associated with tissue destruction, were greater in the pediatric study population, indicating a more aggressive process. The literature also describes higher recidivism rates in pediatric cholesteatomas
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[1,11,12]; our patient’s mass recurred in less than one year. We determined two likely etiologies for our patient’s asymptomatic sigmoid sinus occlusion: a lateral sinus thrombosis or, more
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likely, direct dural extension of the cholesteatoma.
3.3 Lateral Sinus Thrombosis Smith and Danner [13] characterized the various complications associated with chronic otitis media and cholesteatomas, including lateral sinus thrombosis (LST). They found that, in cases of secondary LSTs, infectious etiologies tend to be the causative agents in young children; whereas,
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thrombosis due to cholesteatomas was more likely in adults. There is a paucity of literature regarding the relationship between pediatric cholesteatomas and LSTs. To our knowledge, this is the first report of an asymptomatic sinus thrombosis secondary to a cholesteatoma in the
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pediatric literature.
In children, the literature describing LSTs focuses almost exclusively on its association with
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otogenic infections [14,15]. LSTs also have been generally symptomatic in the literature. A review of 104 pediatric patients describes the presence of at least one sign or symptom of
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increased cranial pressure (such as headache or vomiting) on presentation [16]. This was consistent with other case series in the pediatric literature [15,17,18], all attributing etiology to current or recent otogenic infections.
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There are no reports of asymptomatic LST in pediatric patients due to cholesteatoma, though there are some in the adult literature. Neto et al [19] described a pediatric lateral sinus thrombosis where a cholesteatoma was the causative agent, but the patient had symptoms of
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increased cranial pressure (altered mental status, nausea, vomiting, and fever), similar to cases
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involving acute or recent infections.
In our case, serial MRI/MRVs showed a significant filling defect in the right sigmoid and transverse venous sinuses, but our patient never showed symptoms of increased intracranial pressure secondary to venous congestion, despite it being the dominant sinus. This is likely due to slow occlusion of the sinus over time, likely by direct extension. Interestingly, there was no clear evidence of this on imaging or intraoperative exam. It was not until disease recurrence nine
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months after initial presentation that our patient complained of any neurologic symptoms. Our patient’s initial presentation was isolated to middle ear symptoms—otorrhea and conductive hearing loss—despite evidence of disease extension into the dural sinuses on imaging. The
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asymptomatic nature of our patient’s thrombus shows that a complication with potential for high morbidity and mortality can remain clinically undetected. In our case, imaging was crucial in
clinical follow-up were important in managing our patient.
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detecting an uncommon complication. Initial imaging, serial follow-up imaging, and close
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Our patient’s young age at the time of presentation was also unusual for a case involving an LST secondary to a cholesteatoma. In a retrospective case series of 12 patients performed by Manolidis and Kutz Jr [14], 50% (6/12) of the LSTs were secondary to acute otitis media; the remaining were caused by chronic otitis media. Of the acute otitis media cases, all were under
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age 14 years, further confirming the association between acute infectious etiologies and LST in the pediatric population [14]. There is a greater prevalence of LST cases associated with chronic otitis media and subsequent cholesteatoma formation in the adult population than in the pediatric
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population; however, due to the small number of total cases and unclear distinctions of what ages are pediatric, this was not statistically significant and was difficult to interpret [14,15,20,21]. The
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youngest age of a patient with a cholesteatoma as the etiology of LST in these studies was 13 years [20]. Our patient was younger—nine years—at initial presentation.
3.4 Direct Dural Extension Pediatric cholesteatomas are usually associated with local extension to the mastoid or ossicles [22]. In a study by Farah et al [22], 57% of cases extended to the mastoid, and 50% of 26 cases
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showed ossicular destruction; they did not, however, describe any cases involving intracranial spread [22]. Scales developed to objectively quantify the extent of destruction caused by a growing cholesteatoma—such as the Austin-Kartush Classification Score—use only middle ear
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structures as benchmarks of disease severity [23,24]. Our patient had evidence of sigmoid sinus dehiscence and occlusion in addition to mastoid extension on imaging without intracranial
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symptoms.
Other case series looking at the direct intracranial invasion of cholesteatomas seem to link those
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originating in the mastoid with a greater likelihood of intracranial spread [25,26]. Our patient’s cholesteatoma likely originated in the middle ear secondary to his recurrent acute otitis media; his lack of symptoms allowed for extensive local growth, leading to sigmoid invasion despite his
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cholesteatoma originating outside of the mastoid cavity.
Mathews et al [27] found that, in cases of acute mastoiditis requiring surgery, there was a peak incidence of lateral sinus pathology in children aged 11–15 years, with all cases being secondary
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to invasive cholesteatomas. This represented 86% of all lateral sinus pathology [27]. All of the patients at least presented with pyrexia and otalgia, and 34% had some neurological symptom,
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such as cranial nerve palsy, ataxia, or altered mental status [27]. Our patient denied any similar symptoms and reported only intermittent otorrhea (possibly due to ear tubes).
In an adult case of a large cholesteatoma presenting with isolated hearing loss, the patient’s mass extended from the skull base to the upper part of the neck, with radiological evidence of vascular invasion of the jugular bulb and the sigmoid sinus [28]. His history showed progressive hearing
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loss since childhood, which was exacerbated with recurrent episodes of acute otitis media; he did not present for evaluation of his cholesteatoma until his seventh decade of life [28]. Given the time between the start of his symptoms and his presentation, he was able to acclimate to the
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growing mass such that he did not experience mass effect sequelae. Our patient did not have this length of time, but nonetheless was asymptomatic. His clinical picture and initial exam raised no
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concerns about an invasive mass until after imaging.
4.1 Conclusion
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Our case shows that extensive disease may occur with relatively minimal signs and symptoms, making the diagnosis of these lesions difficult. Two components made diagnosing this patient complex: his young age at presentation and his lack of intracranial symptoms. In addition, his prior history of tympanostomy tubes makes it difficult to fully determine the etiology of his
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cholesteatoma and to classify it as acquired or congenital.
Regardless of classification, early diagnosis is important for both types because the aggressive
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growth of pediatric cholesteatomas and their destruction of local structures can lead to significant morbidity and mortality. This case also describes how extensive and severe complications of
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cholesteatomas—LSTs and direct invasion of the dural space—can present, particularly in an asymptomatic patient.
This case, to our knowledge, is the first case of an asymptomatic sigmoid sinus obstruction secondary to an acquired cholesteatoma in the pediatric population. This report demonstrates that a severe complication may be clinically silent and present at an earlier age than previously
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described. This highlights the importance of imaging and high clinical suspicion in the diagnosis
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and management of invasive cholesteatomas in the pediatric population.
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Figure Legends Figure 1. T2-weighted axial non-contrast MRI with hyperintense right-sided mass Figure 2. T2-weighted coronal MRI with contrast showing right-sided hyperintense mass
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Figure 3. T2-weighted coronal MRI with contrast showing recurrence of right-sided mass
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Figure 4. T2-weighted axial MRI without contrast showing right-sided soft-tissue mass
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