Cervical neurenteric fistula causing recurrent meningitis in Klippel-Feil sequence

Gumerlock, Mary K.; Spollen, Linda E.; Nelson, Martin J.; Bishop, Randall C.; Cooperstock, Michael

doi:10.1097/00006454-199107000-00010

Cited by 25 publications

(8 citation statements)

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“…The literature search identified four cases of recurrent bacterial meningitis associated with this condition (2,70,115,131). In three cases, the neurenteric cyst was located in the suboccipital region or cervicomedullary junction, and in one case, the lesion was found in the presacral area.…”

Section: Congenital Anatomical Defectsmentioning

confidence: 99%

“…After multiple diagnostic and surgical procedures, a coexisting small fistulous duct between the posterior cranial fossa and the left Eustachian tube was identified as being the cause. The difficulty of diagnosing these patients is also illustrated by the case of a 10-year-old girl, who experienced a total of 59 episodes of meningitis (32 culture-confirmed episodes) and had undergone eight surgical explorations before the diagnosis was eventually made (70). The most common causative organism in this group of patients was again S. pneumoniae (37%), closely followed by H. influenzae (34%) and S. aureus (23%).…”

Section: Congenital Anatomical Defectsmentioning

confidence: 99%

“…All episodes of recurrent meningitis associated with S. aureus occurred in connection with anatomical lesions, which included two cases of intranasal meningoencephaloceles (75,196), cases with dermal sinus tracts (103,115,198), and a patient who was subsequently found to have a neurenteric cyst (70). Strikingly, S. aureus infection was not reported for any patient with immunodeficiency.…”

Section: Microbiological Aspectsmentioning

confidence: 99%

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Epidemiology, Etiology, Pathogenesis, and Diagnosis of Recurrent Bacterial Meningitis

2008

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SUMMARY Recurrent bacterial meningitis is a rare phenomenon and generally poses a considerable diagnostic challenge to the clinician. Ultimately, a structured approach and early diagnosis of any underlying pathology are crucial to prevent further episodes and improve the overall outcome for the affected individual. In this article, we are reviewing the existing literature on this topic over the last two decades, encompassing 363 cases of recurrent bacterial meningitis described in 144 publications. Of these cases, 214 (59%) were related to anatomical problems, 132 (36%) were related to immunodeficiencies, and 17 (5%) were related to parameningeal infections. The review includes a detailed discussion of the underlying pathologies and microbiological aspects as well as recommendations for appropriate diagnostic pathways for investigating this unusual entity.

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Section: Congenital Anatomical Defectsmentioning

confidence: 99%

Section: Congenital Anatomical Defectsmentioning

confidence: 99%

Section: Microbiological Aspectsmentioning

confidence: 99%

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Epidemiology, Etiology, Pathogenesis, and Diagnosis of Recurrent Bacterial Meningitis

2008

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“…After a 13 day course, the fever continued and the patient became irritable. Bacterial meningitis was diagnosed on the basis of the remarkable number of polymorphonuclear leukocytes/ mm 3 in the CSF and normal brain computerized tomography (CT). No microoganisms were identified by anaerobic or aerobic cultures.…”

Section: Case Reportmentioning

confidence: 99%

Recurrent meningitis in a case of congenital anterior sacral meningocele and agenesis of sacral and coccygeal vertebrae

Funayama

Turcato

Moura-Ribeiro

et al. 1995

Arq. Neuro-Psiquiatr.

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SUMMARY -A rare case of recurrent meningitis due to congenital anterior sacral meningocele and agenesis of the sacral and coccygeal vertebrae is described. An autosomal dominant inheritance is demonstrated for lower cord malformation, and environmental factors (chromic acid or fumes) are discussed.KEY WORDS: recurrent meningitis, neural tube defects, inherited diseases.Meningite recorrente em um paciente com meningocele sacral anterior e agenesia sacral e coccigea Recurrent meningitis is a condition due to, among other causes, cerebrospinal fluid (CSF) fistulae through structures like the cribriform plate, stapedial footplate and spinal cord, which develops from congenital defects.infections, infiltrative tumors, traumas or surgeries. We report a rare case of recurrent meningitis related to congenital anterior sacral meningocele and agenesis of the sacral and coccygeal vertebrae. CASE REPORTSS, a one year old healthy white male infant, became feverish 35 days before admission. Otitis was diagnosed and amoxicillin prescribed. After a 13 day course, the fever continued and the patient became irritable. Bacterial meningitis was diagnosed on the basis of the remarkable number of polymorphonuclear leukocytes/ mm 3 in the CSF and normal brain computerized tomography (CT). No microoganisms were identified by anaerobic or aerobic cultures. Treatment with ampicillin was then started. This was his second episode of bacterial meningitis. The first occurred at 4 months of age, when the patient was seen at another hospital, and improved within two weeks. No germ was detected in the first episode, probably due to the administration of a systemic antibiotic for *São Paulo University, Ribeirão Preto, Brazil; ** Campinas University, Brazil Aceite: 9-agosto-1995. RESUMO -Um caso raro de meningite recorrente devido a meningocele sacral anterior e agenesia das vértebras sacras coccígeas é descrito. Herança autossômica dominante para malformação medular caudal é demonstrada e, possíveis fatores ambientais (ligados ao cromo), são discutidos. PALAVRAS -CHAVE: meningite recorrente, malformação do tubo neural, doenças hereditárias.

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“…The most common abnormalities associated with KFS are skeletal anomalies (such as Sprengel's deformity, basilar invagination, skull asymmetry, and scoliosis), hearing impairment, congenital heart diseases, ocular malformations, cranial and facial asymmetry, cleft palate, [21,37,38] genitourinary malformations, [45] and mirror movements. [22,50,62] Other rare associations reported to occur in conjunction with KFS include partially or completely split cervical cord, [9] anomalous rib, [48] neurenteric cyst, [61] neurenteric fistula that causes recurrent meningitis, [19] and dermoid cyst. [12,47] The exact causes of KFS are unknown, although genetic factors for C2-3 (autosomal dominant) and C5-6 (autosomal recessive) fusions have been suggested, [20] and environmental factors such as alcohol consumption may also have a role.…”

mentioning

confidence: 99%

The dysmorphic cervical spine in Klippel-Feil syndrome: interpretations from developmental biology

David¹,

Thorogood²,

Stevens³

et al. 1999

FOC

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The authors conducted a study to identify radiological patterns of Klippel-Feil syndrome (KFS), and they present a new interpretation of the origin of these patterns based on recent advances in understanding of embryonic development of the spine and its molecular genetic control. The authors studied radiographs and computerized tomography (CT) scans as well as magnetic resonance images or CT myelograms obtained in 30 patients with KFS who were referred for treatment between 1982 and 1996; the patients had complained of various neuroorthopedic complications. Homeotic transformation due to mutations or disturbed expression of Hox genes is a possible mechanism responsible for C-1 assimilation, which was found to have occurred in 19 cases (63%). Notochordal defects and/or signaling problems, which result in reduced or impaired Pax-1 gene expression, may underlie vertebral fusions. This, together with asymmetrical distribution of paraxial mesoderm cells and a possible lack of communication across the embryonic midline, could cause asymmetrical fusion patterns, which were present in 17 cases (57%). The wide and flattened shape of the fused vertebral bodies and their resemblance to the embryonic cartilaginous vertebrae as well as the process of progressive bone fusion with age suggest that the fusions occur before or, at the latest, during chondrification of vertebrae. The authors suggest that the aforementioned mechanisms are likely to be, at least in part, responsible for the observed patterns in KFS that affect the craniovertebral junction and the cervical spine.

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Cervical neurenteric fistula causing recurrent meningitis in Klippel-Feil sequence

Cited by 25 publications

References 0 publications

Epidemiology, Etiology, Pathogenesis, and Diagnosis of Recurrent Bacterial Meningitis

Epidemiology, Etiology, Pathogenesis, and Diagnosis of Recurrent Bacterial Meningitis

Recurrent meningitis in a case of congenital anterior sacral meningocele and agenesis of sacral and coccygeal vertebrae

The dysmorphic cervical spine in Klippel-Feil syndrome: interpretations from developmental biology

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