International Journal of Pediatric Otorhinolaryngology Extra
Volume 4, Issue 3 , Pages 105-110, September 2009

Idiopathic cerebrospinal fluid leakage from a patent fallopian canal involving arachnoid cyst in an infant: A case report

  • Kazuharu Yamazaki

      Affiliations

    • Department of Otorhinolaryngology, Iwate Medical University, Uchimaru 19-1, Morioka 020-8505, Iwate Pref., Japan
    • Corresponding Author InformationCorresponding author. Tel.: +81 19 651 5111; fax: +81 19 652 8642.
    • ,
  • Hiroaki Sato

      Affiliations

    • Department of Otorhinolaryngology, Iwate Medical University, Uchimaru 19-1, Morioka 020-8505, Iwate Pref., Japan
    • ,
  • Atsushi Kamei

      Affiliations

    • Department of Pediatric, Iwate Medical University, Japan
    • ,
  • Manami Akasaka

      Affiliations

    • Department of Pediatric, Iwate Medical University, Japan

Received 9 July 2008; accepted 9 September 2008. published online 03 November 2008.

Article Outline

Summary 

Objective: To describe idiopathic cerebrospinal fluid (CSF) otorrhea from a patent fallopian canal in an 11-month-old boy and to discuss the case with reference to the literature.

Study design: Case report. Patient, intervention and results: We present the case of an 11-month-old boy with idiopathic CSF otorrhea caused by congenital fallopian canal. The patient underwent attico-antrotomy and CSF leakage from the labyrinthine portion of the facial nerve canal was confirmed. Several pieces of cortical bone, temporalis fascia and a temporal muscle flap with fibrin glue were used to pack the fistula. Neither CSF otorrhea nor meningitis has been observed for 7 years postoperatively.

Conclusions: Idiopathic CSF otorrhea is extremely rare and only 10 other cases of leakage from the fallopian canal have been reported. The patient in this case is the youngest to have been reported.

Keywords: Spontaneous cerebrospinal fluid otorrhea, Patent fallopian canal, Infant, Cisternography

 

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1. Introduction 

Cerebrospinal fluid (CSF) otorrhea can be caused by bone destruction due to head trauma, surgery or tumor, and congenital temporal bone anomaly can cause idiopathic CSF otorrhea [1]. In children, idiopathic CSF otorrhea can be caused by inner ear malformations such as Mondini malformation, or congenital fistula unaccompanied by inner ear malformation [2], but CSF otorrhea unaccompanied by inner ear malformation is extremely rare. We report herein the case of an infant with idiopathic CSF otorrhea from the fallopian canal. To the best of our knowledge, only 10 cases have been reported previously [3], [4], [5], [6], [7], [8], [9], [10], [11]. Furthermore, the present patient was an 11-month-old infant, representing the youngest occurrence of this condition yet to have been reported. We discuss the therapy and long-term clinical course for this patient with reference to the literature.

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2. Case report 

On July 17, 2001, an 11-month-old boy developed a fever above 40°C. Left otorrhea developed the next day (July 18) and the patient was brought to an Ear Nose Throat clinic. The patient was initially treated for otorrhea due to acute otitis media, but symptoms had not improved by July 19. As otorrhea was transparent, CSF leakage was suspected and the patient was referred to our department. On presentation, colorless transparent otorrhea was evident accumulated in the external auditory canal, and the posterosuperior area of the external auditory canal was swollen. The eardrum was not red and no site of perforation could be identified. As CSF otorrhea was suspected, the glucose level in otorrhea was measured and was high, at 60mg/dl. CSF otorrhea was diagnosed and the patient was admitted the same day. No past history of head trauma or acute otitis media was apparent.

On admission, computed tomography (CT) of the temporal bones (Fig. 1) and magnetic resonance imaging (MRI) were performed. A lesion that appeared to represent CSF retention was seen from the tympanic cavity to the mastoid cells, and although no clear signs of inner ear malformation or damaged skull base were apparent, the area near the geniculate ganglion was more expanded on the left side than on the right. To prevent meningitis, 900mg of cefotaxime sodium was injected intravenously and the patient was monitored conservatively. Otorrhea stopped 3 days later and indium cisternography (Fig. 2a) performed 1 month after onset clearly showed CSF leakage in the middle ear cavity, and the patient underwent surgery. The patient did not have a fever exceeding 38°C.

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  • Fig. 1. 

    CT of the temporal bones: (a) right side and (b) left side. The labyrinthine portion of the left fallopian canal (arrow) was expanded as compared to the right side, and a lesion appearing to be CSF retention was seen in mastoid cells.

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  • Fig. 2. 

    Results of 111In cisternography (1h after injection) and SPECT (3h after injection). (a) One month after onset. Accumulation was seen in the left temporal region. (b) One month after surgery. Accumulation was seen in the left temporal region.

Auditory brainstem response examination was performed preoperatively, showing that threshold value on the affected side was 45dB, and latency of I waves was prolonged slightly. Threshold value on the healthy side was 15dB. No asymmetry was seen in the latency of I–V waves. Bacteriologic examination of otorrhea fluid was performed but yielded negative results.

Left-side mastoidectomy with middle ear exploration was performed on September 12. The tympanic cavity and mastoid cavity were filled with CSF. A bone defect was seen in the fallopian canal of the labyrinthine portion, and an arachnoid cyst and CSF leakage were confirmed (Fig. 3). The leakage was severe, and this area was closed using fascia, cortical bone fragments, fibrin glue and temporal muscle flap, and a bone fragment was placed in the Eustachian orifice to prevent infection.

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  • Fig. 3. 

    Intraoperative findings. Incus was removed. A fissure was seen in the labyrinthine portion of the fallopian canal, and an arachnoid cyst (AC) protruded through the fissure. CSF leakage was observed at the bottom of the AC (arrow). M: body of the malleus.

The patient was discharged on September 28. On October 23, the first postoperative indium cisternography was performed, revealing clear accumulation on the affected side (Fig. 2b), suggesting that leakage persisted. Neurosurgery was thus considered, but patching the internal auditory canal by performing craniotomy was considered difficult. Also, the patient did not display postoperative fever or any sign of meningitis, so monitoring was continued. As the patient was young and a risk of meningitis via upper respiratory tract infection existed, the parents were asked not to enroll the patient in nursery school. Cisternography was periodically performed every 6–12 months. Starting at 3 years 6 months old, the patient began attending preschool, and had developed acute otitis media four times before 4 years old. However, only redness of the eardrum was identified, with no otorrhea. The patient responded to antibiotics every time. However, rhinitis persisted and effusion was seen inside the tympanic cavity. CT of the temporal bones also showed soft tissue density, but whether this represented exudative otitis media or CSF retention was unclear. Tympanotomy was not performed. In 2005 at 5 years old (4 years after onset), scintigraphy showed reduced accumulation on the affected side and only slight leakage. In 2006, cisternography and single photon emission tomography (SPECT) were performed at the same time, and no significant accumulation was seen on the affected side. CT of the temporal bones performed during the same period showed no lesion in the tympanic cavity, suggesting that leakage had stopped (Fig. 4). For the past 7 years postoperatively, the patient has not developed meningitis.

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  • Fig. 4. 

    CT of the temporal bones at 5 years after surgery. Although postoperative changes were seen, no CSF accumulation was present and fistula was obliterated by ossification (arrow).

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3. Discussion 

Escat was the first to report CSF otorrhea in 1897 [12]. CSF otorrhea can be caused by various factors, and Dandy [1] classified these causes as (1) temporal bone fracture, (2) surgery-induced damage, (3) erosion due to tumor or inflammation and (4) congenital anomaly. CSF otorrhea is often caused by secondary surgical damage, and idiopathic CSF otorrhea is rare [3]. Pappas et al. [13] classified idiopathic CSF otorrhea into (1) leakage from a temporal bone defect and (2) labyrinthine segment defect or anomaly not involving the labyrinthine portion.

CSF otorrhea caused by a defect in the tegmen of the temporal bone is common in adults. In many cases, CSF otorrhea occurs without a clear inducer, and fluid retention in the tympanic cavity causes conductive hearing impairment. CSF otorrhea is often discovered by tympanotomy or tube placement. Two theories have been put forward to explain defects in the tegmen of the temporal bone. The first theory posits a congenital origin, with CSF leakage occurring when the tegmen becomes weakened due to aging or hernia induced by brain hypertension. Autopsy studies of normal individuals have found a 15–34% frequency of tegmental defects [14]. The second theory involves arachnoid granulation (AG), as proposed by Gacek [15]. AG represents a condition in which the arachnoid membrane invades the venous sinus and intracranial bone wall via a defect in the dura mater, and CSF pressure increases the size of the defect.

In children, CSF otorrhea is often caused by inner ear malformation, such as Mondini malformation, and meningitis recurs in more than 90% of such cases. CSF otorrhea often presents as meningitis, and frequently causes severe hearing impairment. In Mondini malformation, CSF enters the middle ear cavity through the subarachnoid cavity via an enlarged internal auditory canal. CSF then leaks into the middle ear cavity via a fistula, such as the oval window, stapes plate defect or round window.

Pediatric idiopathic CSF otorrhea unaccompanied by inner ear malformation is very rare. In 1979, Gacek and Leipzig [3] reported a 2-year-old patient who repeatedly developed meningitis. CSF otorrhea was after placement of a ventilation tube and mastoidectomy was performed. Near the labyrinthine portion, an enlarged fallopian canal and CSF leakage were confirmed, and surgery was performed to stop the leakage. Gacek and Leipzig [3] listed the following four potential areas for fistulation besides the labyrinthine portion: (1) cochlear aqueduct, (2) petromastoid canal, (3) Hyrtl’s fissure and (4) fallopian canal. Each of these pathways is closely examined below.

3.1. Cochlear aqueduct 

Spector et al. [16] conducted a thorough pathological study on the fetal development of human cochlear aqueduct. At a fetal age of 16–18 weeks, the primitive cochlear aqueduct is dilated and includes the periotic duct, Hyrtl’s fissure and inferior cochlear vein. With this route, a communication exists between the posterior cranial fossa and round window niche. Then, up to a fetal age of 20–24 weeks, tissue around the round window becomes ossified to close Hyrtl’s fissure. The inferior cochlear vein also separates from the cochlear aqueduct at a fetal age of 22 weeks and vascularizes through the canal of Cotugno. Then, at a fetal age of 26–40 weeks, the cochlear aqueduct narrows and extends. In adults, the cochlear aqueduct is filled with connective tissue and is mostly closed [17]. Park [18] reported 15 patients with CSF leakage inside the cochlear aqueduct, but based on their descriptions, Phelps [2] found that this passage was Hyrtl’s fissure, not the cochlear aqueduct. Other researchers have questioned the involvement of the cochlear aqueduct in CSF leakage [19].

3.2. Petromastoid canal 

Schuknecht [20] proposed that the petromastoid canal is a fissure accompanying meningitis. This small bony canal carrying the subarcuate vessels, which are branches of the anterior inferior artery, passes below the arch of the superior semicircular canal and connects the posterior cranial fossa and mastoid antrum. However, Migirov and Kronenberg [21] reported that the petromastoid canal is filled with connective tissue, and the petromastoid canal seems unlikely to serve as a pathway to disseminate infection into the cranium.

3.3. Hyrtl’s fissure 

As mentioned above, Hyrtl’s fissure generally closes before a fetal age of 20–24 weeks, but Spector et al. [16] reported a case in which Hyrtl’s fissure remained patent. Several reports have described CSF otorrhea via Hyrtl’s fissure [3], [4], [22], [23]. Gacek and Leipzig [3] reported a case of enlarged meningocele via Hyrtl’s fissure, while in Japan, Kawashiro et al. [4] reported a case involving a 9-year-old boy in 1986.

3.4. Fallopian canal 

The present patient displayed CSF leakage through the fallopian canal. Including the present case, a total of 11 cases have been reported [3], [4], [5], [6], [7], [8], [9], [10], [11]. Regarding the location of leakage, the most common area is the labyrinthine portion, and reports have also described involvement of the tympanic portion. CT often shows an enlarged labyrinthine portion, and this was also seen in the present patient. Facial palsy was seen in 3 of the 11 cases, and an arachnoid cyst was involved in 2 cases. Arachnoid cyst inside the internal auditory canal has been reported to cause facial palsy [24], while Isaacson et al. [9] found that CSF leakage was a complication of an arachnoid cyst. In the present case, arachnoid cyst was seen in the bone defect in the labyrinthine portion, and although CSF was seen from the arachnoid cyst, no facial palsy was present. Gacek examined the fallopian canal in 163 temporal bone samples and reported that the subarachnoid cavity extended to the geniculate ganglion in 6 samples (4%) [25]. Gacek stated that persistent CSF pressure expands the subarachnoid cavity to thin the surrounding bone and induce CSF otorrhea. Past reports with CT have confirmed circular enlargement of the labyrinthine portion, the same finding seen in the present patient. Intraoperative findings confirmed that the arachnoid cyst protruded from the bone defect, supporting the above theory.

High-resolution CT, MRI, cisternography and cisterno-SPECT have been used as diagnostic imaging tests for CSF otorrhea. In the presence of clear morphological abnormalities such as inner ear malformation, CT and MRI can identify the location of leakage, but in the absence of inner ear malformation, the location of leakage is often difficult to identify preoperatively. With respect to CSF leakage, the more severe the leakage, the more effective the cisternography (111In DTPA) and metrizamide CT utilizing a water-soluble agent [26]. However, mild CSF leakage may not be detected by preoperative diagnostic imaging. Several studies have found that the sensitivity of cisterno-SPECT is high [27], [28], [29].

Fortunately, the present patient did not develop meningitis postoperatively, but follow-up cisternography indicated that CSF leakage persisted. Cisternography performed 4 years postoperatively finally showed reduced accumulation, and cisternography and cisterno-SPECT performed 5 years after surgery showed no accumulation. CT did not show any lesion inside the tympanic cavity or mastoid cells, and these findings indicated that leakage had stopped.

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4. Conclusions 

We have described a very rare case of idiopathic CSF otorrhea unaccompanied by inner ear malformation. Leakage occurred through the labyrinthine portion of the fallopian canal, which was then closed using fascia, bone fragments, fibrin glue and muscle flap. Postoperative cisternography confirmed persistent leakage and the patient was monitored by diagnostic imaging. Leakage stopped 5 years after surgery, and the patient has not developed meningitis for 7 years postoperatively.

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References 

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PII: S1871-4048(08)00065-8

doi:10.1016/j.pedex.2008.09.003

International Journal of Pediatric Otorhinolaryngology Extra
Volume 4, Issue 3 , Pages 105-110, September 2009

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