Recurrent meningitis associated with a petrous apex cephalocele

We present the case of a 6-year-old girl with recurrent bacterial meningitis and cerebrospinal fluid (CSF) rhinorrhea associated with a petrous apex cephalocele (PAC). We diagnosed her by means of three-dimensional computed tomography (CT) and heavily T2-weighted magnetic resonance imaging (MRI). Petrous apex cephaloceles are usually an asymptomatic incidental finding in adults; however, they should be considered as a possible cause of CSF rhinorrhea, otorrhea, and recurrent meningitis in children.     

前颅底骨折伴脑脊液鼻漏的颅底修复与重建治疗

目的分析颅底骨性重建和膜性重建治疗前颅底骨折伴脑脊液鼻漏患者的安全性及临床疗效。 方法收集西南医科大学附属中医医院神经外科自2013年6月至2017年10月收治的前颅底骨折伴脑脊液鼻漏患者40例,均行前颅底修复与重建手术治疗,对其临床诊断、手术治疗及临床预后进行分析。 结果初次手术后36例患者脑脊液鼻漏停止,4例患者术后仍然有脑脊液鼻漏,后再次接受开颅手术修复重建治疗,术后脑脊液鼻漏全部停止。3例患者发生脑膜炎,静脉使用抗生素后2例治愈,1例死亡。术后随访3~36个月,预后良好者33例（82.5%）,预后不良者6例（15.0%）,死亡1例（2.5%）。 结论开颅颅底骨性重建和膜性重建修复是前颅底骨折伴随的脑脊液鼻漏的有效治疗方法,手术并发症发生率低,患者恢复效果好。     

Recurrent bacterial meningitis in a case of Crouzon syndrome after craniofacial surgery]

A 29-year-old woman, who was diagnosed as Crouzon syndrome for which two cranio-facial surgeries had been performed as a child and at the age of 19, developed high fever, headache, and confusion for two days. She was admitted to our hospital. She was diagnosed as bacterial meningitis by cerebrospinal fluid examinations, and her condition was immediately improved by antibiotics. At the age of 23, she also suffered from bacterial meningitis caused by otitis media and sinusitis, and recovered by antibiotics with no sequela. Her cranial computed tomography showed sphenoid and ethmoid sinusitis, and bone deformation and hypertrophy with no fistula connecting intracranial space and sinus. Dead space by cranio-facial surgeries might cause the development of chronic or recurrent sinusitis leading to bacterial meningitis. Our patient is the second case of recurrent bacterial meningitis with Crouzon syndrome to our knowledge. We should recognize that recurrent sinusitis with Crouzon syndrome after cranio-facial surgery is a risk of recurrent meningitis.     

Extraordinarily Long-Term Posttraumatic Cerebrospinal Fluid Fistula

Most posttraumatic cerebrospinal fluid (CSF) leakage is noticed by the patients with the first symptom, rhinorrhea. A 38-year-old woman presented with frequent clear continuous rhinorrhea and otorrhea for 5 years after basilar skull fracture. After this, meningitis was developed with subsequent CSF fistula. Her clinical symptom was improved by medical treatment. The dural defect and CSF leakage were not detected by computerized tomography (CT) cisternography. We report a rare case of persistent posttraumatic CSF fistula that continued for five years.     

Recurrent Streptococcus Pneumoniae Meningoencephalitis in a Patient With a Transethmoidal eningoencephalocele

We report a case of recurrent Streptococcus Pneumoniae meningoencephalitis with a transethmoidal meningoencephalocele (TEME) but without cerebrospinal fluid (CSF) leakage.A 35-year-old man was admitted with S. pneumoniae meningitis. He had suffered from four episodes of recurrent pneumococcal meningitis during the previous 4 years. A computed tomography scan of the paranasal sinus showed the TEME protruding through a bony defect of the right frontal base. However, the patient did not have symptoms that could be attributable to CSF leakage, and radioisotope cisternography did not identify a leak. Brain magnetic resonance imaging revealed cortical lesions overlying the TEME, and electroencephalography revealed epileptiform discharges in frontal regions. Appropriate antibiotics therapy without steroids was given to improve his condition.The presented case suggests that even in the absence of clinically demonstrable CSF leakage, an occult skullbase defect and its associated meningoencephalocele should be considered in patients with recurrent bacterial meningitis.     

经鼻入路神经内镜下手术修补脑脊液鼻漏

目的 探讨经鼻入路神经内镜下脑脊液漏修补术的方法及相关问题。方法 回顾性分析2011年12月至2017年12月经鼻入路神经内镜下手术修补治疗的51例脑脊液鼻漏的临床资料,其中外伤性47例,自发性3例,医源性1例。根据漏口大小选择不同方法修补。结果 51例中,修补成功49例,2例术后发生颅内感染、脑脊液鼻漏,经保守治疗治愈。所有病例术后随访2~5年,均未再发生脑脊液漏。结论 经鼻入路神经内镜下脑脊液鼻漏修补术是脑脊液鼻漏的有效治疗手段。     

Verzögertes Auftreten posttraumatischer Liquorfisteln als Ursache rezidivierender Meningitiden

Immediate post-traumatic fistulas with rhinorrhea are well known after head injury with frontobasal trauma. Less attention is paid to the possibility of delayed onset of rhinorrhea after severe head injury. Unrecognised frontobasal injury may cause recurrent meningitis due to communication of CSF with the outside world. In the last 5 years, seven patients who developed rhinorrhea 2-25 years after trauma were treated in our department. All patients had been through at least two episodes of meningitis. Intermittent rhinorrhea was reported by four. In all cases, coronal bone window CT scan disclosed a bony defect of the anterior skull base, and in three cases an encephalocele was revealed on MRI. Treatment consisted of reconstruction of the anterior skull base with a pedicled galeal-pericranial flap via bifrontal craniotomy. Delayed traumatic CSF fistulas are not rare but must be considered in the differential diagnosis of recurrent meningitis.     

A case with bacterial meningitis caused by cerebrospinal fluid rhinorrhea 22 years after head trauma]

A 38-year-old man with past history of head injury at the age of 16 years was admitted to our hospital in April, 2000 because of bacterial meningitis. At the end of March 2000, he experienced massive cerebrospinal fluid (CSF) rhinorrhea when he rested in bed, and subsequently he developed high-grade fever, headache, and nuchal stiffness in 2 weeks. His symptoms and signs improved with the antibiotic therapy alone. He had no recurrence of meningitis since then. A defect of his right skull base was clarified by 3D-CT, and multiplanar reconstruction (MPR)-CT. MRI brain scan showed the dislocation of the right frontal lobe into the ipsilateral anterior ethmoidal sinus. The diagnosis of CSF rhinorrhea and bacterial meningoencephalitis were made according to his clinical manifestations and neuroradiological studies including 3D-CT, MPR-CT and MRI brain scans. These imaging modalities, which described his head anatomy precisely, helped us to clarify the cause of his illness. When CSF rhinorrhea is present, it is important to take detailed past history of head trauma, even if the event occurred more than 20 years previously.     

An 11-year-old girl with recurrent bacterial meningitis due to liquorrhea caused by bone malformation of the skull base

The patient is a healthy 11-year-old girl with no history of trauma or hearing impairment. She developed pneumococcal meningitis three times, at ages 7, 10, and 11. Intracranial examination revealed, pore expansion and cerebrospinal fluid leakage in the right internal acoustic foramen, which were attributed to a bone malformation of the base of the skull. A procedure was performed to close the cerebrospinal fluid leakage; no relapse has been observed thus far. Previous case reports indicate that repetitive bacterial meningitis is often caused by internal ear malformation, trauma, tumors, or surgical operation. This case suggests the possibility that underlying disorders may not be apparent in cases of repetitive bacterial meningitis and, more proactive investigations are required to prevent further recurrence of meningitis.     

伴脑脊液耳鼻漏的Common cavity畸形的临床特征分析(附一例报告)

目的 掌握common cavity畸形的临床特征,提高对伴有脑脊液耳鼻漏的Common cavity 畸形在临床诊断中的认识水平.方法 对1例伴有脑脊液耳鼻漏的Common cavity畸形患者的临床特征、影像学表现及诊疗过程等临床资料进行整理并结合文献分析.结果 Common cavity畸形自幼听力下降,可伴有脑脊液耳鼻漏或脑脊液耳漏,自幼常反复发作脑膜炎;常误诊为分泌性中耳炎、化脓性中耳炎、脑脊液鼻漏、化脓性脑膜炎,因此容易导致漏诊.结论 自幼进行性听力下降或全聋,反复发作不明原因的脑脊液耳鼻漏,伴反复发作脑膜炎者应疑及此病,颞骨CT或MRI检查可以确诊.     

Pathogenesis of non-traumatic cerebrospinal fluid rhinorrhea

15 consecutive patients with non-traumatic cerebrospinal fluid rhinorrhea were studied. 13 operations were performed on 10 patients. In 8 transcranial operations, an assumed defect in the anterior fossa was plugged with muscle, but only 3 operations were successful. In 4 operations, either transcranial or transsphenoidal, the sella was packed with muscle and rhinorrhea ceased immediately.
Based on radiological and operative findings, 3 groups of patients appeared (1) 9 patients had pathology related to the pituitary gland or the sella turcica: enlarged sella, empty sella, pituitary tumour, intrasellar cyst or erosion of the sellar osseous border. (2) 2 patients had rhinorrhea from extrasellar origin. (3) In 4 patients no abnormality could be found.
Prior to the rhinorrhea, 6 patients (5 from group 1 and 1 from group 3) had experienced episodes of neurological symptoms, compatible with a pituitary apoplexy.
It is suggested that non-traumatic cerebrospinal fluid rhinorrhea in most cases is the result of a spontaneous necrosis in a pituitary adenoma, which has caused sellar bony erosion.     

外伤性迁延性脑脊液鼻漏的病因及定位诊治研究

目的探讨脑脊液鼻漏的病理机制、定位诊断及治疗方法。方法总结分析21例外伤性迁延性脑脊液鼻漏的术前CT、CT脑池造影(CTC),与术中所见相比较,评估其诊断价值,从而术前准确定位漏口。结果术中见17例患者神经组织疝入鼻安,16例CT扫描显示骨缺损,所有病例CTC所显示漏口与术中所见完全一致。所有病例术后无复发,无手术并发症。结论神经组织经颅底缺损疝入鼻窦为外伤后脑脊液鼻漏不能自愈的重要病理机制,南于神经组织的疝入,导致黏膜、硬膜、蛛网膜难以修复。CTC可准确确定瘘口部位,4周内不能自愈的脑脊液鼻漏应积极早期手术治疗。     

Spontaneous cerebrospinal fluid rhinorrhea through clival defect

Spontaneous cerebrospinal fluid fistula secondary to clival defect is a very rare condition. Only 2 cases are reported previously in the literature. We report a case of clival defect in a young male patient presenting with spontaneous cerebrospinal fluid rhinorrhea which failed detection by conventional CT and MRI. The patient was referred to our center after failed anterior cranial fossa repair. Thorough radiological investigations successfully detected the defect in the posterior wall of the sphenoid sinus 5 mm distal to the posterior limit of the floor of the sella just on the midline. The fistula was managed successfully through an endonasal transsphenoidal approach. We present the case reviewing the literature concerning the pathophysiology of spontaneous cerebrospinal fluid fistula through the sphenoid sinus.     

Cerebrospinal fluid otorrhea and pseudomonal meningitis in a child with Mondini dysplasia: case report

Purpose

Mondini dysplasia is a rare congenital inner ear malformation that presents with abnormal cochlear development with accompanied vestibular dilation and vestibular aqueduct enlargement. This dysfunctional anatomy provides the potential for sensorineural hearing deficits, cerebrospinal fluid leaks, and severe cases of recurrent meningitis.

Methods

We present the case of a child with Mondini dysplasia who presented with unilateral hearing loss and cerebrospinal fluid (CSF) otorrhea that was surgically repaired through a combined middle fossa/transmeatal middle ear approach to alleviate any recurrence of infection and cerebrospinal fluid otorrhea.

Results

Postoperatively, the patient remained neurologically stable without any further CSF leakage. CSF cultures revealed a Pseudomonas aeruginosa infection, a rare occurrence within the context of Mondini dysplasia. Retrograde bacterial spread from the external ear canal into the CSF space has been theorized as the possible pathogenesis of the resulting meningitis. The patient was successfully treated with intravenous antibiotics without any neurologic complications.

Conclusions

Although Mondini dysplasia is a rare malformation, the life-threatening sequelae of meningitis that can result from the dysfunctional anatomy makes it a condition that requires elevated clinical vigilance, especially when considering children with hearing loss associated with recurrent meningitis, otorrhea, or rhinorrhea.

     

Cerebrospinal Fluid Rhinorrhea and Seizure Caused by Temporo-Sphenoidal Encephalocele

This case report describes the symptoms and clinical course of a 35-year-old female patient who was diagnosed with a temporo-sphenoidal encephalocele. It is characterized by herniation of cerebral tissue of the temporal lobe through a defect of the skull base localized in the middle fossa. At the time of first presentation the patient complained about recurrent nasal discharge of clear fluid which had begun some weeks earlier. She also reported that three months earlier she had for the first time suffered from a generalized seizure. In a first therapeutic attempt an endoscopic endonasal approach to the sphenoid sinus was performed. An attempt to randomly seal the suspicious area failed. After frontotemporal craniotomy, it was possible to localize the encephalocele and the underlying bone defect. The herniated brain tissue was resected and the dural defect was closed with fascia of the temporalis muscle. In summary, the combination of recurrent rhinorrhea and a first-time seizure should alert specialists of otolaryngology, neurology and neurosurgery of a temporo-sphenoidal encephalocele as a possible cause. Treatment is likely to require a neurosurgical approach.     

Endoscope-Assisted Trans-Sphenoidal Approach for Treatment of Sternberg's Canal

We report an uncommon case of a 45-year-old woman who presented with spontaneous rhinorrhea. A computed tomography (CT) scan of the head revealed an abnormally large sphenoid sinus associated with a parasellar bony defect (Sternberg''s canal) through which magnetic resonance imaging could detect an encephalocele of the right temporal lobe. An endoscope-assisted trans-sphenoidal approach was performed and, with the aid of image guided surgery, reduction of the encephalocele was obtained and followed by surgical repair of the dural and bony defects. The postoperative course was uneventful and the cerebrospinal fluid fistula was closed as confirmed by the postoperative CT scan and by the absence of rhinorrhea. After three years of monitoring the patient remained asymptomatic.     

Delayed traumatic CSF-fistulas: a retrospective analysis

Immediate posttraumatic CSF-fistulas are a well known entity after severe head injury. Delayed onset of rhinorrhea is considered to be rare. In the last 5 years 7 patients were treated in our department, who developed rhinorrhea 2-25 years after trauma. All patients went through episodes of meningitis. In 4 cases intermittent rhinorrhea was reported. In all cases a bony defect of the anterior skull base was detected by coronal bone window CT-scan. In three of them an encephalocele was revealed by MR-scanning. Treatment consisted in reconstruction of anterior skull base with a pedicled galeal-pericranial flap via a bifrontal craniotomy and went out without any complications. Delayed rhinorrhea after severe head injury is not a rare curiosity. In cases of bony defects after head injury reconstruction of anterior skull base is recommended to prevent episodes of recurrent meningitis.     

Allogeneic cartilage used for skull base plasty in children with primary intranasal encephalomeningocele associated with cerebrospinal fluid rhinorrhea

Three children with primary intranasal encephalomeningocele associated with cerebrospinal fluid rhinorrhea were operated on at the Department of Neurosurgery, Hradec Králové. In two children, aged 4 and 9.5 years, freeze-dried allogeneic costal cartilage was glued into the skull base defect. This plugging was covered up with deepfrozen allogeneic fascia lata. In the third child, an only 1-year-old boy, after transection of the neck of the encephalomeningocele freeze-dried allogeneic dura mater was glued on extradurally and deep-frozen allogeneic fascia lata applied intradurally. The cerebrospinal fluid rhinorrhea ceased immediately after surgery. Spontaneous atrophy of the intranasal portion of the encephalomeningocele was demonstrated respectively 11, 1, and 7 years postoperatively on computed tomography. To evaluate cartilage healing histologically, the extracted allogeneic cartilage used for orbital roof plasty after 4 months was examined. The extent of spotty regressions represented about 7% of the tissue volume. It is stressed that, once diagnosed, intranasal encephalomeningocele associated with cerebrospinal fluid rhinorrhea should be operated on for prevention of meningitis as soon as possible.     

Recurrent meningitis, hydrocephalus and tegmen tympani dehiscence

Cerebrospinal fluid (CSF) otorrhea is the usual presenting symptom of spontaneous tegmen tympani defects. A case associated with recurrent meningitis and CSF rhinorrhea without otorrhea is described. The coexistence of an hydrocephalus had led initially to ventriculoperitoneal shunting, which did not prevent meningitis recurrence. Previously reported in cases of anterior fossa and sella defects, an aqueduct stenosis was here associated with the tegmen tympani defect.     

A case of recurrent bacterial meningitis by delayed cerebrospinal fluid (CSF) leakage due to a head trauma]

A 42-year-old man was admitted due to recurrent bacterial meningitis, as he had been treated here for bacterial meningitis three years prior to the current event. He had a remote history of head injury that he had almost forgotten, and his laboratory data showed no immunodeficiency state. 111In-DTPA cisternography showed an abnormal radioactive accumulation in the frontal lobe adjacent to the left frontal sinus at 23 hours after intrathecal injection, and MPR CT images revealed the left frontal sinus bone fracture. These findings indicated that he had a head injury by which a delayed CSF fistula has been formed. He was surgically treated for a CSF leakage. Although a combination therapy of ABPC and CTRX was efficacious for this patient, this regimen may not be ideal, as meningitis by PRSP has been increasing in incidence. Pneumococcal meningitis, once not a difficult infection to treat, could be a difficult one, as resistant strains to ABPC and CTRX have been more common.