Accepted Manuscript Cerebrospinal fluid otorrhea secondary to non-otogenic meningitis in a child with Mondini dysplasia Mariah E. Small, Matthew D. Cox, Adam Johnson, John L. Dornhoffer PII:
S2468-5488(17)30015-2
DOI:
10.1016/j.xocr.2017.02.001
Reference:
XOCR 10
To appear in:
Otolaryngology Case Reports
Received Date: 19 January 2017 Revised Date:
30 January 2017
Accepted Date: 1 February 2017
Please cite this article as: Small ME, Cox MD, Johnson A, Dornhoffer JL, Cerebrospinal fluid otorrhea secondary to non-otogenic meningitis in a child with Mondini dysplasia, Otolaryngology Case Reports (2017), doi: 10.1016/j.xocr.2017.02.001. 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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Cerebrospinal Fluid Otorrhea Secondary to Non-Otogenic Meningitis in a Child with Mondini Dysplasia
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Mariah E. Small, MS41, Matthew D. Cox, MD2,3, Adam Johnson, MD PhD,2,3 and John L. Dornhoffer, MD2,3
University of Arkansas for Medical Sciences, College of Medicine, Little Rock, Arkansas, United States of America.
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Division of Otology and Neurotology, Department of Otolaryngology – Head and Neck Surgery, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States of America.
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Division of Pediatric Otolaryngology, Arkansas Children’s Hospital, Little Rock, Arkansas, United States of America.
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Short Running Title CSF Otorrhea and Repair in Mondini Dysplasia
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Disclosures No conflicts of interest exist with the authors of this study. John L. Dornhoffer, M.D. designed a series of ossiculoplasty prostheses that are produced by Grace Medical (Memphis, TN), but does not collect any royalties or other financial incentives as the result of sales.
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Corresponding Author Mariah E. Small, MS4, University of Arkansas for Medical Sciences, College of Medicine, Little Rock, Arkansas, USA. 4301 West Markham Street, Little Rock, Arkansas 72205.
[email protected]. (479)970-0975. No sources of funding were used for this study.
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INTRODUCTION
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The first reported congenital inner ear malformation was described by Carlo Mondini in 1791. Mondini dysplasia, as originally described, consists of a cochlea with 1-1.5 turns and a cystic apex, a hypoplastic modiolus, and an incompletely-formed interscalar septum. Modern classification schemes for these anomalies were proposed by Jackler et al. in 1987 1 and Sennaroğlu2 in 2002. According to Sennaroğlu’s classification system, Mondini dysplasia is known as incomplete partition type II.
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The internal auditory canal is the most commonly identified conduit for cerebrospinal fluid (CSF) to flow into the perilymphatic space, though others have been described.3 While several anatomic sites of communication between the perilymphatic space and the middle ear have been described, the most common involves a defect at the stapes footplate.4 It is not uncommon for the eponymous ‘Mondini dysplasia’ to be incorrectly applied to any radiographically detectable congenital malformation of the osseous labyrinth.5 To this point, it is well-described that severe cochlear dysplasia places a patient at increased risk for CSF fistula, but this phenomenon is uncommon in cases of true Mondini dysplasia.6 We present an unusual case in which a patient with true Mondini dysplasia first developed meningitis, which was secondarily complicated by development of a CSF fistula (presumably the result of increased intracranial pressure) and managed using a novel modification of the traditional surgical technique. CASE PRESENTATION
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A 3-year-old female with a known history of true Mondini dysplasia (see Figure 1) with rightsided profound hearing loss and hypoplastic cochlear nerve and left-sided severe-to-profound hearing loss presented to our institution with altered mental status, lethargy, and fever. At time of admission, informed consent was signed for medical treatment and use of personal data. Two days prior to presentation, she was prescribed cephalexin for a urinary tract infection. Despite empiric treatment, she remained febrile to 40°C and became progressively lethargic until having two generalized seizures, which prompted presentation to the emergency department. Imaging demonstrated possible developing hydrocephalus, small areas of ischemia, and possible purulent material in the interhemispheric area, and a lumbar puncture confirmed meningitis. She was admitted to the intensive care unit for meningitis, mechanically ventilated, and treated with empiric broad-spectrum intravenous antibiotics. Polymerase chain reaction confirmed Streptococcus pneumoniae as the causative agent. This patient did not have a prior history of acute otitis media, but was noted to have erythematous tympanic membranes with middle ear effusions at the time of presentation with meningitis. The otolaryngology service was consulted and myringotomy with tympanostomy tube placement was offered. Intraoperatively, bilateral serous effusions were noted, and cultures were negative. There was no clear fluid leak identified at the time of or during the days following tube placement.
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Her clinical status slowly improved until the seventh postoperative day, at which time the patient began to have clear, left-sided otorrhea, which tested positive for beta-2 transferrin. Shortly thereafter, the patient became febrile and had further seizure activity. A lumbar drain was placed, which lead to improvement, but not resolution, of the leak. The patient remained febrile and ultimately required surgery to repair the CSF leak.
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The patient was ultimately taken to the operating room for definitive management. Despite previously declining cochlear implantation, her parents were offered a cochlear implant to be placed concurrently with CSF leak repair, but they declined this portion of the intervention. At the time of exploratory tympanotomy, the leak was found to originate from a bleb of the stapes footplate (see Figure 2). The stapes and incus were removed, which left a patent vestibule with a CSF gusher. After the vestibule was packed with temporalis fascia to stop the flow of CSF, a cartilage cap was placed at the oval window and the native stapes was placed atop this cap. In order to secure the stapes in this position, a strut was created from another cartilage graft and was wedged between the stapes footplate and the malleus (see Figure 3). Fibrin glue was used to temporarily obliterate the middle ear, and a final cartilage graft was used to reinforce the tympanic membrane and close the perforation from previous tympanostomy tube placement. The patient tolerated the procedure well and had no recurrence of CSF leak. DISCUSSION
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The incidence of Mondini dysplasia is not well-described due to variations in the application of this eponymous term. In our patient, the temporal relationship between onset of the second phase of meningitic symptoms and the onset of CSF leak leads us to suspect that increased intracranial pressure associated with meningitis and seizure activity played a causative role. While we were not able to confirm the anatomic site of communication between the subarachnoid space and the inner ear, we did identify a bleb on the stapes footplate as the site of communication between the inner and middle ear.
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Surgical management of a CSF fistula in a congenitally-malformed cochlea typically involves obliteration of the vestibule, which serves as the conduit for communication between the subarachnoid space and middle ear. Upon exploration, the rate of CSF flow may be slow in some cases, whereas a brisk “gusher” is encountered in others. Most surgeons employ a lumbar drain intraoperatively to temper this flow rate and slowly taper its use post-operatively to encourage successful healing. Recurrence after surgical repair has been reported to occur in 30-60 percent of patients.4,7 Traditional obliteration materials consist of fat grafts or temporalis fascia and/or muscle, but the use of vascularized temporalis muscle flaps and reinforcement with fibrin glue have been described as well.5 Due to the increased risk for immediate or delayed recurrence of CSF leak when vestibular obliteration is accomplished by simple packing with temporalis fascia or fat grafts, several techniques for layered and reinforced closure have been described.3-5,7 In our case, layered closure with temporalis soft tissue and cartilage cap at the oval window provided a seal for the CSF gusher, and further reinforcement was provided by the native stapes, which was seated atop the cartilage cap. The native stapes was then secured by another cartilage graft, which acted much like a doorstopper (see Figure 3). The use of fibrin glue and cartilage grafting at the
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tympanic membrane provided further layers of protection. This novel modification of the traditional repair technique proved successful as there was no recurrence of CSF leak.
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While uncommon, CSF fistula may occur in patients with true Mondini dysplasia, or type II incomplete partition. The increased risk for meningitis in patients with CSF fistula is welldescribed. However, it is less common to secondarily develop CSF otorrhea after an initial episode of meningitis in this patient population. The native stapes and cartilage grafting techniques may be useful for reinforcement after vestibular obliteration using temporalis fascia during surgical repair of a CSF fistula in a congenital inner ear malformation. FIGURE LEGENDS
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Figure 1: CT radiograph demonstrates true Mondini dysplasia, or incomplete partition type II with decreased number of cochlear turns, partial bony partition and enlarged vestibular aqueduct.
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Figure 2: Intraoperative photograph demonstrates the bleb of the stapes footplate (arrow), which is the site of CSF leak in this case.
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Figure 3: Intraoperative photograph demonstrates replacement of the native stapes (short arrow) and cartilage graft placed as a reinforcing “doorstopper” (long arrow) to wedge the native stapes in position.
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REFERENCES Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: A classification based on embryogenesis. The Laryngoscope. 1987;97(S40):2-14.
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Sennaroğlu L, Saatci I. A New Classification for Cochleovestibular Malformations. The Laryngoscope. 2002;112(12):2230-2241.
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da Cruz MJ, Ahmed SM, Moffat DA. An alternative method for dealing with cerebrospinal fluid fistulae in inner ear deformities. Am J Otol. 1998;19(3):288-291.
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Ohlms LA, Edwards MS, Mason EO, Igarashi M, Alford BR, Smith RJ. Recurrent meningitis and Mondini dysplasia. Arch Otolaryngol Head Neck Surg. 1990;116(5):608612.
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Syal R, Tyagi I, Goyal A. Cerebrospinal fluid otorhinorrhea due to cochlear dysplasias. International Journal of Pediatric Otorhinolaryngology. 2005;69(7):983-988.
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Phelps PD, Lloyd G. Mondini dysplasia is not associated with meningitis and cerebrospinal fluid fistula. Arch Otolaryngol Head Neck Surg. 1991;117:931.
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Park TS, Hoffman HJ, Humphreys RP, Chuang SH. Spontaneous cerebrospinal fluid otorrhea in association with a congenital defect of the cochlear aqueduct and Mondini dysplasia. Neurosurgery. 1982;11(3):356-362.
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