Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature.

Bacterial meningitis is a serious infection affecting mostly the pediatric age group. Ninety percent of cases of meningitis occur in patients under age five. The most common organisms are Hemophilus influenza Type B, Streptococcus pneumoniae, and Neisseria meningitidis. Mortality in modern series is quoted as between 1% and 5%. However, up to 50% of survivors may display some long-term neurological sequelae.

While bacterial meningitis is relatively common, recurrent meningitis is definitely uncommon. The precise epidemiology of this entity has not been adequately characterized. Any case of recurrent bacterial meningitis demands a search for an underlying abnormality. The abnormality may be anatomical, immunological, or a combination of the two. Immunological problems are suggested by recurrent episodes of infections in multiple sites. A family history of similar problems, cancer chemotherapy, immunosuppression after transplantation, or HIV infection also suggest immunodeficiency. Appropriate screening tests for immunodeficiency include quantitative determination of immunoglobulin levels and assessment of immunoglobulin function. This is done by checking pre- and post- immunization titers. Isohemagglutinin determination is also a simple way of checking for an intact immunoglobulin system. Compliment levels can also be easily determined. In cases of recurrent staphylococcal infection, chemotactic and phagocytic function of leukocytes should be investigated.

Anatomical abnormalities are those in which an abnormal connection exists between the CNS and either a mucosal surface or the skin.

Congenital connections between the CNS and nose or paranasal sinuses are usually of the midline type, such as encephaloceles. Acquired defects in this area are either traumatic, i.e., anterior skull base fractures; or iatrogenic, such as following endoscopic sinus surgery.

Congenital CSF fistulas to the ear exist and can be divided into translabyrinthine or perilabyrinthine types. Translabyrinthine types include an abnormal cochlea, and are usually accompanied by sensorineural hearing loss with or without vestibular symptoms. Perilabyrinthine types occur in temporal bones with normal inner ears. Of the translabyrinthine type, the Mondini malformation is most commonly associated with CSF leak and recurrent meningitis. Traumatic CSF fistulas to the temporal bone can occur following surgery or blunt head trauma.

Congenital skin - CNS fistulas should be suspected in patients with Klippel-Feil syndrome, who may have cervical or occipital dermal sinuses. In a ten-year review of 47 patients with well-documented recurrent bacterial meningitis, Kline was able to identify predisposing factors in 44. The most common underlying cause was a congenital CSF fistula, usually through the stapes footplate. Ten patients had immune deficiency. Eight of these were compliment deficiencies, and immunoglobulin deficiencies accounted for the other two.

Investigation of patients with recurrent bacterial meningitis should include a complete history, stressing any previous head trauma or surgery on the nose, paranasal sinuses, or ear. The previously mentioned immune screen should be carried out. To rule out a congenital abnormality of the ear resulting in sensorineural hearing loss, audiometry is important.

Imaging studies, while commonly performed, are often disappointing. CT scanning of the temporal bones and anterior skull base are recommended. This can be supplemented by contrast enhanced cisternography. Radioisotope cisternography can be used both as an imaging modality, and to localize the site of CSF leaks. Pledgets can be placed in various sites in the nose and ears and, after the installation of isotope labeled albumin intrathecally, these pledgets can be read for radiation levels.

Immunologic deficiencies should be corrected where possible. Anatomic abnormalities must also be repaired. Anterior skull base defects are best repaired via a frontal craniotomy. Congenital and acquired CSF through the temporal bone can be approached either via a transmastoid or middle cranial fossa approach, as the situation dictates.

Case Presentation

A 10-year-old Latin American male, presented with decreased level of consciousness, headache, photophobia, right otalgia, and fever. Physical examination revealed a temperature of 100.1°, positive Kernig's and Brudzinski's signs, and a dull, red right TM. Lumbar puncture revealed cloudy CSF with 255 white cells (94% PMN). Culture grew Strep pneumonia.

Previous medical history was remarkable for a right longitudinal temporal bone fracture sustained in a motor vehicle accident seven years earlier, an incus transposition, and one episode of meningitis two years previously. Immunological investigations were normal. A CT of the head revealed an opacified right mastoid air cell system. Temporal bone CT revealed a defect in the right tegmen with soft tissue herniation.

After two weeks on IV antibiotics (initially Cefotaxime, changed to Penicillin G after culture results) the patient was taken to the operating room. He was found to have a right temporal bone encephalocele, which was repaired using a combined transmastoid-middle fossa approach. The patient had an uneventful recovery, and is well at the one-year follow-up, with mild right conductive hearing loss.

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