Síndrome de Austrian en endocarditis infecciosa: reporte de un caso y manejo médico quirúrgico exitoso / Austrian syndrome in infective endocarditis

Resumen: El Síndrome de Austrian, corresponde al cuadro clínico descrito por Robert Austrian en 1957, definido por la triada de Neumonía, Endocarditis Infecciosa (EI) y Meningitis, causado por Streptococcus pneumoniae. En la mayoría de los casos el vicio valvular presente, es la insuficiencia valvular aórtica, cuyo tratamiento médico y resolución quirúrgica de acuerdo con su gravedad, deben ser realizados precoz y oportunamente. Un paciente de 51 años, sin antecedentes de valvulopatía, con historia de poli consumo de alcohol y cocaína comenzó dos semanas previo a su ingreso hospitalario con síndrome febril, neumonía, y meningitis bacteriana por Streptococcus pneumoniae. Sus hemocultivos fueron negativos. El ecocardiograma transesofágico (ETE) fue compatible con EI valvular aórtica con insuficiencia moderada a severa. Se trató como EI a microorganismo desconocido y se efectuó un reemplazo valvular aórtico electivo con prótesis biológica a la 5° semana después de terminado el tratamiento médico antibiótico, cuyo resultado fue exitoso.

Abstract: A syndrome including Infective endocarditis, pneumonia and Meningitis caused by S pneumoniae was described by Robert Austrian in 1957. The aortic valve is affected in most cases. Medical followed by surgical treatment should be promptly implemented. The clinical case of a 51 year old man with a history of multiple drug consumption developing fever, pneumonia, and meningitis caused by S pneumoniae is presented. Blood cultures were negative and trans esophageal echocardiography showed aortic valve vegetations and moderate regurgitation. After multiple antibiotic treatment the patient underwent aortic valve replacement and recovered satisfactorily. Clinical and epidemiological characteristics of this syndrome are discussed.

Surgically Cured, Relapsed Pneumococcal Meningitis Due to Bone Defects, Non-invasively Identified by Three-dimensional Multi-detector Computed Tomography.

A 43-year-old Japanese man presented with a history of bacterial meningitis (BM). He was admitted to our department with a one-day history of headache and was diagnosed with relapse of BM based on the cerebrospinal fluid findings. The conventional imaging studies showed serial findings suggesting left otitis media, a temporal cephalocele, and meningitis. Three-dimensional multi-detector computed tomography (3D-MDCT) showed left petrous bone defects caused by the otitis media, and curative surgical treatment was performed. Skull bone structural abnormalities should be considered a cause of relapsed BM. 3D-MDCT was useful for revealing the causal minimal bone abnormality and performing pre-surgical mapping.

Meningitis complicated by subdural empyema and deafness caused by pneumoccoccal serotype 7F in a 17-month-old child: a case report.

Despite the availability of effective antibacterial agents and vaccines, pneumococcal meningitis and sepsis are still associated with high mortality rates and a high risk of neurological sequelae. We describe the case of a 17-month-old boy vaccinated with heptavalent pneumococcal conjugate vaccine (PCV7) who developed bacterial meningitis complicated by subdural empyema and deafness caused by Streptococcus pneumoniae serotype 7F. The 7F strain is not contained in PCV7 (the only vaccine on the market at the time of the onset of meningitis) but is included in the new pediatric 13-valent PCV, which may therefore prevent cases such as this in the future.

Grafted neuronal precursor cells differentiate and integrate in injured hippocampus in experimental pneumococcal meningitis.

Bacterial meningitis (BM) frequently causes persisting neurofunctional sequelae. Autopsy studies in patients dying from BM show characteristic apoptotic brain injury to the stem cell niche in the subgranular zone of the hippocampal dentate gyrus (DG), and this form of brain damage is associated with learning and memory deficits in experimental BM. With an eye to potential regenerative therapies, the survival, migration, and differentiation of neuronal precursor cells (NPCs) were evaluated after engraftment into the injured hippocampus in vitro and in vivo in an infant rat model of pneumococcal meningitis. Green fluorescent protein (GFP)-expressing NPCs were grafted into the DG of organotypic hippocampal slice cultures injured by challenge with live Streptococcus pneumoniae. Seven days after engraftment, NPCs had migrated from the site of injection into the injured granular layer of the DG and electro-functionally integrated into the hippocampal network. In vivo, GFP-expressing NPCs migrated within 1 week from the injection site in the hilus region to the injured granular layer of the hippocampal DG and showed neuronal differentiation at 2 and 4 weeks after transplantation. Hippocampal injury induced by BM guides grafted NPCs to the area of brain damage and provides a microenvironment for neuronal differentiation and functional integration.

Serial PCR genetic load determination in the surgical management of pneumococcal intracranial sepsis.

PURPOSE: Aspirated intracranial fluid, in the surgical management of intracranial sepsis, may not culture an organism due to the previous administration of antibiotics. We have sought to utilise polymerase chain reaction (PCR) to determine the cause of culture-negative sepsis and in monitoring response to therapy. METHODS: This was a retrospective review of five cases of Streptococcus pneumoniae intracranial sepsis. Samples were analysed using real-time quantitative PCR targeting the pneumococcal lytA gene and the number of genome copies per microlitre of sample determined. RESULTS: Streptococcus pneumoniae sepsis was diagnosed by PCR in five culture-negative cases comprising: ventriculitis (×3), subdural empyema and meningitis. Serial serum inflammatory markers (CRP and WBC) and number of genome copies were graphically plotted over the duration of inpatient stay for cases requiring surgical drainage of recurrent collections or external ventricular drainage. A correlation was demonstrated between change in bacterial genomic load and serum inflammatory markers, reflecting similar changes in clinical state. CONCLUSIONS: This is the first report of the use of serial quantitative PCR in monitoring the course of intracranial sepsis secondary to S. pneumoniae. Further work is required to determine the precise relationship between serum inflammatory markers, clinical state and bacterial load: do changes in one precede the other? Furthermore, a threshold value for number of genome copies in cerebrospinal fluid/aspirate samples has yet to be defined.

Endoscopic repair of a rare basioccipital meningocele associated with recurrent meningitis.

Anatomical variants of the basiocciput are uncommon and usually clinically benign. While the majority remain undetected, these anomalies rarely manifest as CSF rhinorrhea or recurrent meningitis associated with meningocele. Compromise of the leptomeninges provides an avenue of ingress for pathological organisms and can lead to recurrent meningitis, necessitating operative repair of the defect to prevent infection. A review of the literature reveals only 3 cases in which a congenital basioccipital defect has been associated with a meningocele requiring surgical repair. The authors present a case of recurrent meningitis in an infant with a congenital basioccipital meningocele treated with a minimally invasive endoscopic technique. At the 2-year follow-up the repair remained successful, with no evidence of recurrence of the meningocele or CSF infection. The literature regarding the etiology and treatment of these lesions was reviewed, with an emphasis on the safety and efficacy of the endoscopic approach. Note that recurrent meningitis in the setting of a skull base defect may indicate the presence of other congenital anomalies that will necessitate multidisciplinary care for a patient's long-term well-being.

The treatment and outcome of postmeningitic subdural empyema in infants.

OBJECT: The management of subdural empyema (SDE) has been debated in the literature for decades. Craniotomy and bur hole drainage have been shown to achieve a favorable outcome. However, there is a lack of comparative data for these modes of management of SDE subsequent to meningitis in infants. METHODS: The authors conducted a retrospective review of 33 infants identified with SDE due to meningitis at the Department of Neurosurgery, Chang Gung Memorial Hospital between 2000 and 2006. Preoperative clinical presentation, duration of symptoms, radiological investigations, CSF data, and postoperative outcome were analyzed and compared between these 2 surgical groups. RESULTS: At diagnosis, there were no differences between the groups in age, weight, degree of consciousness, CSF analysis, or duration of fever. The outcome data showed no difference in the number of days until afebrile, number of days of postsurgical antibiotic treatment, neurological outcome, recurrence rate, or complication rate. There was only 1 death in the series. CONCLUSIONS: Subdural empyema due to meningitis in infants is unique with respect to the pathophysiology, presentation, and treatment of SDE. Early detection and removal of SDE provide a favorable outcome in both surgical intervention groups. Bur hole drainage is less invasive, and it is possible to expect a clinical outcome as good as with craniotomy in postmeningitic SDE.

[Recurrent meningitis in inner ear malformations]. / Nawracajace zapalenia opon mózgowo-rdzeniowych w wadach wrodzonych ucha wewnetrznego.

Authors present two cases of children with reccurent meningitis and unilateral deafness. Implemented diagnostics (CT, NMR, ABR) revealed one side inner ear congenital malformation in one case and anterior fossa bony defect accompanied by labirynthine deformation in the other case. The presence of perilymphatic fistulae in oval and round windows and cerebrospinal fluid leakage has been confirmed in both cases during surgery. Carefull obliteration of the Eustachian tube and both windows has been performed. Non- complicated postoperative course (2 months and 6 years - respectively) has prooved the effectiveness of applied treatment.

Austrian syndrome: a clinical rarity.

We present a case of classical Austrian syndrome in a 55-year-old man with a history of alcoholism. The rapid destruction in aortic valve cusps caused perforations with severe aortic insufficiency, acute pulmonary edema, and determined an urgent aortic valve substitution with good postoperative evolution. The review of the literature suggests cardiac surgery in this syndrome to improve the poor survival with conservative treatment. We recommend early surgery in these cases because of the fast impairment of the patient and valvular tissue destruction detected in intraoperatory examination.

Endogenous pneumococcal endophthalmitis followed by pneumococcal-induced uveitis.

BACKGROUND: We describe the case of a fulminant bilateral endophthalmitis occurring in a patient, who had 9 years earlier a splenectomy for an idiopathic thrombocytopenic purpura. HISTORY AND SIGNS: A 40-year-old woman, back from a trip to Morocco, presented with high fever, rapid decrease in visual acuity and loss of consciousness. Medical examination revealed a pneumococcal meningitis and bilateral endophthalmitis. THERAPY AND OUTCOME: Endophthalmitis was treated with local and intravitreal antibiotics injections (vancomycin and amikacin). Repeated parabulbar betamethasone injections were performed. Intravenous (iv) methylprednisolone pulses were followed by oral steroid therapy while systemic antibiotics were given (ceftriaxone and vancomycin). In spite of this therapy, fundus examination was impossible because the anterior chamber was filled with fibrin. A cataract developed with severe vitritis and we could observe a progressive narrowing of the anterior chamber. The patient underwent a bilateral vitrectomy and lensectomy. The retina had no lesion. No bacteria were found in the vitreous culture. Evolution was characterized by an increased ocular pressure due to anterior synechiae. Visual acuity remained under 1/10. The severe ocular inflammation could be the result of a mixed mechanism including an infectious followed by a severe immune response against bacterial components. CONCLUSIONS: This case report is rare. To our knowledge, only 3 similar cases have been reported in the literature.

Discrepancies between brain CT imaging and severely raised intracranial pressure proven by ventriculostomy in adults with pneumococcal meningitis.

OBJECTIVES: Computed tomography (CT) of the brain is recommended for assessment of intracranial pressure (ICP) of patients with acute bacterial meningitis who are comatose or show focal neurological deficits. The aim of this report is to draw attention to the possibility of a discrepancy between CT findings and ICP values in some patients with pneumococcal meningitis. METHODS: We describe three adult patients with pneumococcal meningitis who had both successive CT examinations and ICP measurements at the time of clinically evident cerebral herniation (n = 2) and/or prolonged coma (n = 2). RESULTS: Although measurements with a ventriculostomy catheter indicated that all three patients had severely raised ICP values of 90, 44, and 45 mmHg, repeated cranial CT greatly underestimated true ICP values. Despite clinical evidence of acute cerebral herniation, it was not detected in the contemporary CT findings of two patients. Continuous ICP monitoring in the ICU helped to guide treatment for increased ICP; nevertheless, two patients died. CONCLUSIONS: The clinician must be aware that cranial CT may fail to rule out the possibility of severely raised ICP or cerebral herniation in a patient with pneumococcal meningitis. Therefore, ICP monitoring of patients with bacterial (especially pneumococcal) meningitis who are in prolonged coma should be considered early and regardless of the cranial CT appearances.

[Temporal bone fracture after head trauma causing rhinoliquorrhea and meningitis]. / Felsenbeinlängsfraktur im Bereich des Karotiskanals als Ursache für rezidivierende Rhinoliquorrhö und Meningitis.

We report our experience in managing a temporal bone fracture after head trauma that had no apparent clinical signs. Recurrent CSF rhinorrhea and meningitis lead to extensive diagnostic procedures. Operative exploration of the temporal bone demonstrated a fracture line along the horizontal part of the carotid artery. The location of the fracture did not cause such typical symptoms as hearing impairment, facial paralysis, vertigo or tinnitus. Only CSF liquorrhea through the Eustachian tube indicated a fracture at the lateral skull base.

[Basal meningoencephalocele: a case report]. / Meningoencefalocele basal. A propósito de un caso.

An 8-year-old boy who had been operated for craniosynostosis was remitted from the pediatric service for two bacterial meningitis episodes. The cause was a meningoencephalocele of the right nasal cavity.

[Cochlear implant in inner ear abnormalities and footplate malformation]. / Cochlear Implant bei Innenohrmissbildung und Fussplattenfehlbildung.

BACKGROUND: Malformations of the cochlea can cause deafness and otogenic meningitis. Cochlear implantation in children with such malformations, which are sometimes quite complex, require special attention before, during and after surgery. PATIENT: The concept developed for these patients at Hannover Medical School is illustrated in a patient with severe bilateral inner ear malformation and bilateral stapes defect. Implantation of a cochlear implant was performed in this 2-year-old girl. A large endostal sack originating from the inner ear, which had caused meningitis twice, was observed in the right middle ear. Both ears had to be obliterated to prevent recurrent meningitis. RESULTS: The child had no post-operative problems and the initial rehabilitation efforts have been successful. The immediate and differentiated reactions to stimulation are very encouraging. This article briefly summaries all diagnostic and therapeutic procedures established over the last three years at Hannover Medical School in cases of implantation into a malformated cochlea. In particular, we describe perioperative radiology (CT scans, MRI and plain radiographs), facial nerve monitoring and intraoperative screening for "hearing sensations", details of surgical technique, and possible postoperative complications. CONCLUSIONS: Based on experience with 13 of these cases, we discuss the different aspects of implantation into a malformated cochlea. Given certain preconditions implantation into a malformated cochlea may be recommended.

[Acute bilateral amaurosis in sphenoid empyema caused by Streptococcus pneumoniae]. / Akute bilaterale Amaurose bei Sphenoidalempyem durch Streptococcus pneumoniae.

PATIENT: A 14-year-old boy suffered from an acute bilateral blindness which occurred in 24-h time, accompanied by headache and raised temperature, with inconspicuous optic nerve head and fundus. After diagnosis of empyema with magnet resonance tomography (MRT) the sphenoid sinus was fenestrated and streptococcus pneumoniae isolated. Liquor and serology being inconspicuous, there was no evidence of leucaemic or autoimmune disease, intoxication or intracranial tumor. CLINICAL COURSE: The condition of the patient improved under systemic antibiotic therapy. The bilateral amaurosis remained and opticus atrophy developed. CONCLUSION: A bilateral amaurosis with descending opticus atrophy as a consequence of a sphenoiditis and spreading inflammation to the meninges and the optic nerve in the area of the chiasm is a rare event. The imaging technique of the MRT offers new opportunities for an early and more pointed diagnosis and therapy.

The subcranial approach to trauma of the anterior cranial base: preliminary report.

The subcranial approach to the cranio-orbito-frontal junction allows direct access to the central anterior cranial base for repair of fractures, dural tears, and cerebrospinal fluid fistulae. It provides good visualization without brain retraction and is suitable in primary or delayed traumatic cases. For extended visualization, a portion of the frontal sinus may be removed and repositioned at the end of the procedure. Because the brain is not retracted, morbidity is low. Potential and active cerebrospinal fluid fistulae can be successfully managed with the use of free fascial grafts and often do not require a pericranial flap. Contraindications include parenchymal brain injury or bleeding that may require a more standard frontal craniotomy for management. The purpose of this report is to highlight the use of the subcranial approach to repair cerebrospinal fluid fistulae in immediate and delayed traumatic cases.

Delayed complications of ethmoid fractures: a "growing fracture" phenomenon.

Delayed complications of ethmoid fractures are considered relatively rare. However, meningitis, recurrence of previously ceased cerebrospinal fluid rhinorrhea and delayed onset of cerebrospinal fluid rhinorrhea are possible even years after trauma. We report 10 consecutive patients with delayed complications of ethmoid fractures, whom we treated over the past 11 years. All patients had previously sustained a closed head injury and had remained anosmic. Variously after trauma (ranging from 2 months to 31 years), these patients were re-admitted because of meningitis (6 cases), recurrence of previously ceased cerebrospinal fluid rhinorrhea (3 cases), and delayed onset of cerebrospinal fluid rhinorrhea (1 case). In all cases the delayed complications were associated with relatively large defects of the ethmoid bone. These bone lesions were now evident even in those patients whose radiological assessments had been normal after trauma. All patients underwent a successful surgical repair and remained well during the follow-up. We discuss the possibility that delayed complications of ethmoid fractures are due to a mechanism like that of "growing fractures" in children.

[Malformation of the internal ear and recurrent meningitis: radio-surgical correlations]. / Malformation de l'oreille interne et méningites à répétition: corrélations radio-chirurgicales.

Authors report one case of bilateral inner ear malformation in a 3 years-old child with congenital sensorineural hearing loss, recurrent meningitis and cerebro-spinal fluid leakage. This anomaly is a pseudo-Mondini's malformation with a meningocele through the oval window and a fistula of C.S.F. of the round window. A close radiological and surgical correlation support some physiopathological hypothesis about fistula between sub arachnoid space and middle ear. Authors discuss the place of high resolution computed tomography in case of congenital sensorineural learning loss.

Fractures of the middle third of the face and cerebrospinal fluid rhinorrhoea.

The incidence of cerebrospinal fluid (CSF) rhinorrhoea in patients with facial fractures is about 25%. Although the management of facial fractures is well documented, its timing and role in the presence of CSF leak is still open to debate. This study evaluates facial manipulation in 89 facial fractures associated with CSF rhinorrhoea, with a mean follow-up of 4 years. The facial fractures were reduced in 26 patients (29%) and the CSF fistula was repaired in 75 (84%). Twenty-three (25.8%) had both facial manipulation and dural repair with no deaths, post operative infection, failure or recurrence of CSF leak. On the other hand, when facial manipulation or dural repair was performed alone, the CSF rhinorrhoea either persisted or recurred in a significant number of patients requiring further intervention. Although this is a retrospective analysis of patients treated over several years and, there has been a change in the methods of investigation and treatment of these patients, one can conclude that manipulation of facial fractures and surgical dural repair can be carried out at the same sitting without increasing the surgical morbidity and mortality.