Cerebrospinal fluid otorrhea presenting in complicated chronic suppurative otitis media.
Since the introduction of antibiotics, the simultaneous occurrence of multiple intra- and extracranial complications in chronic suppurative otitis media (CSOM) has been uncommon. Such a combination is associated with high mortality and morbidity, especially in the pediatric age group. Cerebrospinal fluid (CSF) otorrhea presenting in CSOM is unusual and rare. To the best of our knowledge, only 19 cases of CSF otorrhea in complicated CSOM have been previously reported in the literature. Identifying the site of leak might require a high index of suspicion. Management of such a case could be challenging in terms of leak closure, disease clearance, and restoration of function. We report a new case of CSF leak in CSOM, and we describe our line of management. Even though CSOM is an uncommon indication for radical mastoidectomy today, such surgery might be necessary for these cases.
Patients with chronic suppurative otitis media (CSOM) seldom present with multiple concomitant fulminant complications. Cerebrospinal fluid (CSF) leak following injury to the temporal bone is common, but CSF leak in CSOM in addition to other complications is rare. To the best of our knowledge, only 19 cases of CSOM with CSF leak have been previously reported in the literature. (1-3) In this article, we report a new case of CSF otorrhea and CSOM in a patient with multiple intra- and extracranial complications.
A 12-year-old boy presented to us in serious condition with right-sided otorrhea of 11 years' duration. During the preceding 10 days, the discharge had suddenly become watery and profuse. The otorrhea had become so severe that it had soaked the patient's pillow when he slept with his head turned to the right. At the same time, the patient also had drowsiness, headache, fever, disorientation, and giddiness. He had also experienced seizures and vomiting during the preceding 2 days, and he reported swelling behind the right ear and in the right side of the neck of 1 week's duration. He had no history of ear surgery or ear trauma.
On examination, the watery fluid could be seen dripping from the ear. On closer inspection, the ear canal was filled with granulation tissue and discharge, and the tympanic membrane was completely perforated. A diffuse swelling was observed in the right mastoid region, and it extended downward to the neck along the sternocleidomastoid muscle. Kernig's sign was positive. Funduscopic examination revealed features of papilledema.
Laboratory investigations revealed that the patient's blood parameters were within normal limits and that he was not immunocompromised. Findings of glucose and protein on biochemical analysis of the ear fluid were consistent with CSF. A lumbar puncture was performed, and the CSF was sent for analysis; the pathologist reported pyogenic meningitis. Pure-tone audiometry revealed a severe mixed hearing loss (figure 1). Computed tomography (CT) of the head demonstrated a dehiscent sinus plate, sigmoid sinus thrombosis, a cerebellar abscess, and dilation of the lateral and third ventricles, which suggested obstructive hydrocephalus (figure 2). The diagnosis was atticoantral-type CSOM with mastoid abscess, Bezold's abscess, sigmoid sinus thrombosis, meningitis, cerebellar abscess, otitic hydrocephalus, and CSF otorrhea.
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With the patient under local anesthesia, the mastoid and Bezold's abscesses were drained, and the pus was sent for culture and sensitivity. Pseudomonas aeruginosa was identified in the pus, and the patient was started on intravenous ceftazidime at 1 gram twice daily. His general condition improved, and after a few days we were able to demonstrate the CSF leak from the right ear with the patient's head bent forward (figure 3).
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The patient was scheduled for temporal craniotomy and mastoid surgery under general anesthesia. In the operating room, the right cerebellar abscess was identified and excised completely by a neurosurgeon. The mastoid antrum was opened via a postauricular approach. Extensive cholesteatoma and granulation tissue were found in the mesotympanum, attic, hypotympanum, and mastoid antrum. All ossicles of the middle ear, including the stapes footplate, were found to be destroyed. The sinus plate had been destroyed, but the tegmen plate was intact. The sigmoid sinus was solid and filled with granulation tissue. The retrosigmoid dura was found to be involved and breached. Pulsating clear fluid was seen leaking from the site. Two fistulae were discovered, one in the horizontal semicircular canal and one in the superior semicircular canal, and both were filled with granulation tissue (as a result, labyrinthine fistula was added to the list of complications). The eustachian tube orifice, the oval window, and the round window were also filled with granulation tissue. No herniation of the brain was observed in any part of the mastoid antrum.
In view of such extensive disease and destruction, we were left with no choice but to perform a radical mastoidectomy. The disease was meticulously cleared from the middle ear and mastoid antrum. After the granulation tissue was cleared from the fistulae, they were sealed with bone wax. The eustachian tube orifice was sealed with temporalis muscle tissue. Curettage was performed on the sigmoid sinus until all disease was cleared and the dura exposed. The defect was then closed in three layers with temporalis fascia, temporalis muscle tissue, and homograft septal cartilage. The round window and the oval window were covered with temporalis fascia. A meatoplasty was performed in the cartilaginous external canal. A tube drain was placed to connect the cavity with the external auditory canal.
Postoperatively, the CSF leak stopped and the patient improved markedly. He was discharged after 10 days. At the 6-month follow-up, no evidence of recurrence of disease or CSF leak was seen.
Chronic otitis media is widespread in India and elsewhere in the developing world. Many patients present quite late in the course of their disease, and therefore complications can be quite serious. Despite advances in healthcare delivery, aggressive atticoantral disease with multiple complications is prevalent in many parts of India, primarily in children. One of the Figure 2. Axial CTs of the head she complications that is and the dilation of the ventricles (C not common is CSF leak. In fact, to the best of our knowledge, only 19 cases have been reported. In 1961, Alberti and Dawes reported a series of 6 cases of CSF otorrhea associated with chronic ear disease. (1) In 1980, Hicks et al reported 11 such cases, 7 of which included intracranial abscesses. (2) Finally, in 1983, Adkins and Osguthorpe described the mini-craniotomy procedure for the management of 2 cases of CSF otorrhea from tegmen defects caused by chronic ear disease. (3) To the best of our knowledge, our case represents the 20th such published report.
Very few cases of dural exposure with resultant herniation of brain tissue and CSF leak as a result of CSOM, with or without cholesteatoma, have been described in the literature.(1,4,5) In many cases, CSF otorrhea in CSOM has been found to occur in association with intracranial abscesses. (2) Cases of CSF otorrhea giving rise to recurrent meningitis have also been described in the literature. (6)
Spontaneous CSF otorrhea through the oval window as a cause of recurrent meningitis has been reported in the recent past. (7) The hallmark of this pathology was the dilation of the cochlea and the absence of the modiolus on temporal bone. (7) In the inner ear, high-resolution CT of the 7 the potential sites of CSF leak include the oval window, stapes footplate, eustachian tube, and promontory fistula. (7) Leaks from the round window or through defects in the promontory are uncommon. (7) In our patient, no perilymph gusher was observed from the windows or from any other site in the middle ear, even after the extensive disease was cleared from the area.
According to the literature, the most likely mechanism of CSF otorrhea in CSOM is either (1) the erosive action of the disease on bone, with involvement of the subarachnoid space, or (2) the dehiscence that occurs as a result of previous surgery for preexisting chronic ear disease. (8,9) CSF leak is further facilitated by the congenital dehiscence of tegmen, which has an incidence of 5.4%. (10)
The CSF leak in our patient could be attributed only to the CSOM because he had no history of ear surgery or ear trauma. Also, we did not find any brain tissue hemiation along with the CSF leak. Other than the erosive action of the local disease, the simultaneous occurrence of meningitis, cerebellar abscess, and hydrocephalus might have led to an increase in intracranial pressure and facilitated a breach of the inflamed meninges.
The pathologic prerequisites for CSF leak in patients with these intracranial complications need to be investigated further. It has been shown that disease in the area of the lateral sinus can destroy the bone of the sinodural angle. (1) Further erosion of the bone over Trautmann's triangle can lead to profuse CSF otorrhea from the lateral cistern. (9) CSF leak might also be made possible by the erosion of the bony plates covering the dura and the subsequent breach of dura through the track of intracranial abscesses. In our patient, no obvious pathway into the intracranial abscess existed, either from the mastoid antrum or from the middle ear. The diagnosis of CSF otorrhea with CSOM is not difficult when it is suspected. The presence of clear fluid in a chronically draining ear that has been cleaned under the microscope is almost diagnostic. (2)
The definitive surgical procedure for these cases is often dictated by the specific individual conditions at the time of surgery. (2) Mastoid surgery is usually the initial procedure, along with drainage of the abscesses. Radical mastoidectomy was performed in 3 of the 11 cases of CSF otorrhea with chronic ear disease and temporal lobe abscess reported by Hicks et al. (2)
In the absence of brain tissue hemiation, surgical repair of the leak site in the dura can be performed conveniently via a transmastoid approach. Repair is preferably done in layers after all disease has been cleared. If the defect is small, temporalis fascia will suffice, but larger defects require fascia lata reinforced with temporalis muscle and cartilage to hold the fascia in place. Certain synthetic biomaterials, such as Marlex mesh, have also been used to support the fascia in cases of brain herniation. (2) It is difficult--and unnecessary--to suture these layers to the dura in a transmastoid repair, but care should be taken to tuck the fascia deep to the edges of the bony defect and then to pack it well into the space. (2) A craniotomy approach has been described for middle fossa defects, especially in cases of CSF otorrhea with brain herniation. (1)
In conclusion, a number of cases of spontaneous, congenital, and traumatic CSF otorrhea have been reported in the literature, but few have been reported with complicated CSOM. The possibility of CSF otorrhea must be kept in mind in a case of CSOM when the ear discharge suddenly turns watery, especially in the presence of intracranial complications. If the diagnosis of CSF otorrhea is missed and thus not adequately addressed by surgery, both recurrent CSF leaks and meningitis could ensue. Our case was unique in that it was managed successfully despite the fact that the patient presented at an advanced stage with multiple complications. CSF otorrhea in CSOM is one of the few non-neoplastic indications for a radical mastoidectomy today.
The authors thank Dr. Khaja Naseeruddin, professor and head of the Department of Otolaryngology-Head and Neck Surgery at the Kamataka Institute of Medical Sciences in Hubli, India, for his support and permission to submit this paper for publication.
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(3.) Adldns WY, Osguthorpe JD. Mini-craniotomy for management of CSF otorrhea from tegmen defects. Laryngoscope 1983;93:1038-40.
(4.) Dedo GG, Sooy FA. Endaural encephalocele and cerebrospinal fluid otorrhea. Ann Otol Rhinol Laryngol 1970;79:168-77.
(5.) Lundy LB, Graham MD, Kartush JM, LaRouere MJ. Temporal bone encephalocele and cerebrospinal fluid leaks. Am J Otol 1996;17:461-9.
(6.) Harris HH. Cerebrospinal otorrhea and recurring meningitis: Report of three cases. Laryngoscope 1978;88:1577-85.
(7.) Teo DT, Tan TY, Eng SP, Chan YM. Spontaneous cerebrospinal fluid otorrhoea via oval window: An obscure cause of recurrent meningitis. J Laryngol Otol 2004;118:717-20.
(8.) Cawthorne T. The surgery of the ear in the management of intracranial complications of chronic suppurative otitis media. J Laryngol Otol 1955;69:579-87.
(9.) Blatt IM. Surgical repair for cerebrospinal otorrhea due to middle ear and mastoid disease. Report of six cases. Laryngoscope 1963;73: 446-60.
(10.) Balance CA, Green CD, eds. Essays on the Surgery of the Temporal Bone. Vol. I. Plate XIII, figure 1. London: Macmillan & Co.; 1919.
Vikram Bhat, MS, DNB, MNAMS (ORL-HNS); Dandinarasaiah Manjunath, MBBS
From the Department of Otolaryngology-Head and Neck Surgery, Karnataka Institute of Medical Sciences, Hubli, Karnataka, India.
Reprint requests: Dr. Vikram Bhat, No: 4-64, Sagri Nole, Kunjibettu Post, Udupi-576102, Karnataka, India. Phone: 91-836-237-0579; cell: 91-944-865-8213; e-mail: firstname.lastname@example.org