Temporal Encephalocele Research Articles

Repair of Temporal Bone Encephalocele following Canal Wall Down Mastoidectomy.

We report a rare case of a temporal bone encephalocele after a canal wall down mastoidectomy performed to treat chronic otitis media with cholesteatoma. The patient was treated successfully via an intracranial approach. An enhanced layer-by-layer repair of the encephalocele and skull base deficit was achieved from intradurally to extradurally, using temporalis fascia, nasal septum cartilage, and artificial dural graft. After a 22-month follow-up period the patient remains symptom free and no recurrence is noted.

Use of Titanium Mesh for Middle Cranial Fossa Skull Base Reconstruction

Objectives Temporal bone encephaloceles are usually encountered in the setting of a congenital defect of the tegmen or as an acquired defect after mastoid surgery. A variety of methods have been described in the literature for rigid reconstruction of tegmen defects. We introduce a new method of repair using orbital floor titanium mesh reconstruction plates to reconstruct the floor of the middle cranial fossa, and evaluate the outcomes, complications, and recurrence rates of temporal bone encephaloceles with this technique. Design Retrospective chart review of consecutively treated patients. Setting Tertiary care academic center. Participants Eight patients with middle cranial fossa skull base defects from January 2007 to February 2011. Main Outcome Measures Outcome measures included resolution of cerebrospinal fluid leak (CSF) and development of postoperative infection. Results One of nine patients had a postoperative CSF (cerebrospinal fluid) leak. There were no long-term complications of CSF leak or infection. Conclusions Titanium mesh is a safe and effective substitute for bone grafts in reconstruction of the middle cranial fossa skull base when rigid reconstruction is required.

Tailored surgery for drug-resistant epilepsy due to temporal pole encephalocele and microdysgenesis

Marco Giulioni *, Laura Licchetta , Francesca Bisulli , Guido Rubboli , Barbara Mostacci , Gianluca Marucci , Matteo Martinoni , Lorenzo Ferri , Lilia Volpi , Fabio Calbucci , Agostino Baruzzi , Paolo Tinuper b,c a IRCCS Istituto delle Scienze Neurologiche di Bologna, Division of Neurosurgery, AUSL di Bologna, Italy b IRCCS Istituto delle Scienze Neurologiche di Bologna, AUSL di Bologna, Italy Department of Biomedical and Neuromotor Sciences, University of Bologna, Italy d IRCCS Istituto delle Scienze Neurologiche di Bologna, Division of Neurology, AUSL di Bologna, Italy Danish Epilepsy Center, Epilepsihospitalet, Dianalund, Denmark f Section of Pathology ‘‘M. Malpighi’’ at Bellaria Hospital, Department of Biomedical and Neuro Motor Sciences, University of Bologna, Bologna, Italy

Temporal bone encephalocele and cerebrospinal fluid fistula repair utilizing the middle cranial fossa or combined mastoid–middle cranial fossa approach

The goals of this study were to report the clinical presentation, radiographic findings, operative strategy, and outcomes among patients with temporal bone encephaloceles and cerebrospinal fluid fistulas (CSFFs) and to identify clinical variables associated with surgical outcome. A retrospective case series including all patients who underwent a middle fossa craniotomy or combined mastoid-middle cranial fossa repair of encephalocele and/or CSFF between 2000 and 2012 was accrued from 2 tertiary academic referral centers. Eighty-nine consecutive surgeries (86 patients, 59.3% women) were included. The mean age at time of surgery was 52.3 years, and the left side was affected in 53.9% of cases. The mean delay between symptom onset and diagnosis was 35.4 months, and the most common presenting symptoms were hearing loss (92.1%) and persistent ipsilateral otorrhea (73.0%). Few reported a history of intracranial infection (6.7%) or seizures (2.2%). Thirteen (14.6%) of 89 cases had a history of major head trauma, 23 (25.8%) were associated with chronic ear disease without prior operation, 17 (19.1%) occurred following tympanomastoidectomy, and 1 (1.1%) developed in a patient with a cerebral aqueduct cyst resulting in obstructive hydrocephalus. The remaining 35 cases (39.3%) were considered spontaneous. Among all patients, the mean body mass index (BMI) was 35.3 kg/m(2), and 46.4% exhibited empty sella syndrome. Patients with spontaneous lesions were statistically significantly older (p = 0.007) and were more commonly female (p = 0.048) compared with those with nonspontaneous pathology. Additionally, those with spontaneous lesions had a greater BMI than those with nonspontaneous disease (p = 0.102), although this difference did not achieve statistical significance. Thirty-two surgeries (36.0%) involved a middle fossa craniotomy alone, whereas 57 (64.0%) involved a combined mastoid-middle fossa repair. There were 7 recurrences (7.9%); 2 patients with recurrence developed meningitis. The use of artificial titanium mesh was statistically associated with the development of recurrent CSFF (p = 0.004), postoperative wound infection (p = 0.039), and meningitis (p = 0.014). Also notable, 6 of the 7 cases with recurrence had evidence of intracranial hypertension. When the 11 cases that involved using titanium mesh were excluded, 96.2% of patients whose lesions were reconstructed with an autologous multilayer repair had neither recurrent CSFF nor meningitis at the last follow-up. Patients with temporal bone encephalocele and CSFF commonly present with persistent otorrhea and conductive hearing loss mimicking chronic middle ear disease, which likely contributes to a delay in diagnosis. There is a high prevalence of obesity among this patient population, which may play a role in the pathogenesis of primary and recurrent disease. A middle fossa craniotomy or a combined mastoid-middle fossa approach incorporating a multilayer autologous tissue technique is a safe and reliable method of repair that may be particularly useful for large or multifocal defects. Defect reconstruction using artificial titanium mesh should generally be avoided given increased risks of recurrence and postoperative meningitis.

Meningoencephalocele of the temporal bone: Pictorial essay on transmastoid extradural-intracranial repair

PurposeA spontaneous meningoencephalocele of the temporal bone may present with effusion in the middle ear, a cerebrospinal fluid leak, hearing loss, or rarely otitic meningitis. Repair of spontaneous encephaloceles in the temporal bone has been performed using transmastoid and transcranial middle fossa approaches or a combination of the two with varied results. The authors present a technical paper on the transmastoid extradural intracranial approach for the management of temporal lobe encephaloceles. Materials/MethodsCase reports and cadaver dissections are used to provide a pictorial essay on the technique. Advantages and disadvantages compared with alternative surgical approaches are discussed. ResultsTraditional transmastoid approaches are less morbid compared with a transcranial repair as they avoid brain retraction. However, in the past, there has been a higher risk of graft failure and hearing loss due to downward graft migration and a potential need for ossicular disarticulation. For the appropriate lesion, the transmastoid extradural intracranial approach lesion offers a stable meningoencephalocele repair without the comorbidity of brain retraction. ConclusionThe authors describe a transmastoid extradural intracranial technique via case reports and cadaver dissections for the repair of spontaneous meningoencephalocele defects larger than 2 cm. This approach provides more support to the graft compared to the conventional transmastoid repair.

Unusual cause of dizziness: Occult temporal lobe encephalocele into transverse sinus

Encephaloceles are rare manifestations, and occur most freuently in the occipital region. Temporal lobe encephaloceles are efined as pathological herniations of brain parenchyma through ura mater and bone, and they involve the middle cranial fossa r lateral temporal cranial vault. The majority of cases of tempoal encephalocele reported in the literature to date have presented ith complaints of epilepsy or ear disturbance (e.g., hearing loss, torrhea, otitis media with or without rhinorrhea, and recurrent eningitis) [1]. We present herein a rare case of lateral temporal encephaloele presenting with dizziness and imbalance. Interestingly, the ncephalocele sac was bulging into the left transverse sinus. The atient’s complaints were considered to be due to the impact of he encephalocele sac on the shape and flow hemodynamics of he transverse sinus, which is also another unusual aspect of the ase.

Seizure outcome in surgically treated drug-resistant mesial temporal lobe epilepsy based on the recent histopathological classifications

The study was performed to investigate the relation between seizure outcome after surgical treatment of mesial temporal lobe epilepsy (MTLE) and pathological findings, classified according to the recently proposed classifications of mesial temporal sclerosis (MTS), granule cell pathology (GCP), focal cortical dysplasia (FCD) and epilepsy-associated low-grade tumors (ELGT). The authors analyzed data obtained in 120 consecutive cases involving patients presenting with drug-resistant MTLE, who underwent tailored anteromesial temporal lobe resection, and correlated seizure outcome with pathological findings. They identified 5 histopathological groups: Group 1-ELGT, alone or associated with other lesions (30 cases); Group 2-isolated FCD (17 cases); Group 3-MTS, with or without GCP (28 cases); Group 4-MTS associated with FCD, with or without GCP (37 cases); Group 5-other lesions (8 cases). Engel Class I outcome was observed in 83% of patients with ELGT (Class IA in 63%); in 59% of patients with isolated FCD, with FCD Type II showing a better prognosis than FCD Type I; in 82% of patients with isolated MTS (Class IA in 50%), with MTS Type 1a and MTS Type 1b showing a better prognosis than MTS Type 2 and patients with MTS and GCP having better postsurgical results than those with MTS without GCP. Engel Class I outcome was also achieved in 84% of patients with FCD associated with MTS (Engel Class IA in 62%); also in this group MTS 1a and MTS 1b associated with FCD showed a better prognosis than FCD associated with MTS 2. Finally, Engel Class I was also achieved in 2 patients with vascular malformation and in 1 with a temporal pole encephalocele. Patients with MTLE and ELGT, MTS, or MTS associated with FCD showed the best postsurgical seizure outcome (Engel Class I in more than 80% of cases), whereas only 63% of patients with isolated FCD achieved the same type of outcome. Interestingly, the analysis of seizure outcome in histopathological subtypes of FCD and of MTS showed different prognoses in the different pathological subgroups, with worse outcomes for atypical MTS, absence of GCP, and isolated FCD Type I.

Temporal Craniotomy for Management of Middle Cranial Fossa Encephaloceles with CSF Leaks

Introduction: Temporal encephaloceles are pathological herniations of brain parenchyma through congenital or acquired osseus-dural defects of the middle cranial fossa. Both open procedures and endoscopic techniques have been advocated for the treatment of such conditions. We describe the midterm follow-up of patients with spontaneous, iatrogenic, and traumatic temporal bone encephaloceles treated by temporal craniotomy and determine the effectiveness and safety of this procedure.

Trans-Mastoid Management of Temporal Bone Tegmen Defects, Encephaloceles and CSF Leaks

Trans-Mastoid Management of Temporal Bone Tegmen Defects, Encephaloceles and CSF Leaks A review of records revealed fifteen post-surgical patients who underwent tegmen defect repair from 2009-2012. We reviewed patients’ surgical logs, pre- and postoperative charts, imaging, and billing records. At six to nine months postoperatively, all patients underwent MRI with appropriate sequencing to evaluate the repair site and evidence of residual tegmen defect, encephalocele, or CSF leaks.

Spontaneous Lateral Temporal Encephalocele

A spontaneous encephalocele is one that develops either because of embryological maldevelopment or from a poorly understood postnatal process that permits brain herniation to occur. We here report a rare case of lateral temporal encephalocele extending to the infratemporal fossa under the zygomatic arch. At birth, the infant was noted to have a large cystic mass in the right side of the face. After being operated on initially in another center in the newborn period, the patient was referred to our clinic with a diagnosis of temporal encephalocele. He was 6 months old at the time of admission. Computerized tomography scan and magnetic resonance imaging studies revealed a 8 × 9 cm fluid-filled, multiloculated cystic mass at the right infratemporal fossa. No intracranial pathology or connection is seen. The patient was operated on to reduce the distortion effect of the growing mass. The histopathological examination of the sac revealed well-differentiated mature glial tissue stained with glial fibrillary acid protein. This rare clinical presentation of encephaloceles should be taken into consideration during the evaluation of the lateral facial masses in the infancy period, and possible intracranial connection should be ruled out before surgery to avoid complications.

The Role of Obesity in Spontaneous Temporal Bone Encephaloceles and CSF Leak

Temporal bone encephaloceles and cerebrospinal fluid (CSF) otorrhea can lead to life-threatening sequelae such as meningitis and cerebritis. Obesity has been associated with spontaneous CSF leaks. We wished to determine if there were differences in the body mass index (BMI) of patients with spontaneous temporal bone encephaloceles/CSF leaks and nonspontaneous temporal bone encephaloceles/CSF leaks to help determine if obesity may be associated with their development. Single institution retrospective comparison. Demographic and clinical information was obtained from 20 patients treated for surgically confirmed temporal bone dehiscence leading to encephalocele and/or CSF otorrhea. Statistical analysis was performed on patients with spontaneous and nonspontaneous encephaloceles/CSF leaks to determine if there were differences in the degree of obesity between these groups. Temporal bone dehiscence was spontaneous in 11 patients, related to chronic infection in 4 patients, posttraumatic in 3 patients, postsurgical in 1 patient, and because of massive hydrocephalus in a final patient. The average BMI for patients with spontaneous encephalocele was 33.4 kg/m. The average BMI for patients with nonspontaneous encephaloceles was 27.0 kg/m. This difference was statistically significant (p = 0.02). Although several previous studies have demonstrated an association of obesity with spontaneous encephaloceles or CSF leaks, this study is one of the first to demonstrate a statistically significant increase in BMI for patients with spontaneous encephaloceles and CSF leaks as compared with patients developing them for other reasons. This suggests a potential etiologic role of obesity in the development of spontaneous encephaloceles and CSF leaks. 4.

Extent of resection and early postoperative outcomes following removal of cystic vestibular schwannomas: surgical experience over a decade and review of the literature

Vestibular schwannomas (VSs) are benign tumors of the eighth cranial nerve sheath, representing approximately 6%-8% of all newly diagnosed brain tumors, with an annual incidence of 2000-2500 cases in the US. Although most of these lesions are solid, cystic vestibular schwannomas (CVSs) compose 4%-20% of all VSs and are commonly larger at the time of presentation. The authors present their experience with the operative management of CVSs, including surgical approach, extent of resection, and postoperative facial nerve outcomes. The literature pertaining to clinical and histopathological differences between CVSs and their solid counterparts is reviewed. The University of Southern California Department of Neurosurgery database was retrospectively reviewed to identify patients who had undergone resection of a VS between 2000 and 2010. One hundred seventy-nine patients with VS were identified. Patients with CVSs were the subject of the present analysis. Diagnosis of a CVS was made based on MRI findings. Clinical and neuroimaging data, including pre- and postoperative assessments and operative notes, were collected and reviewed. Twenty-three patients, 14 men (61%) and 9 women (39%), underwent 24 operations for CVSs. These patients composed 12.8% of all cases of VS. Patient ages ranged from 28 to 78 years (mean 55 years), and the mean maximal tumor diameter was 3.6 cm (range 2.0-4.0 cm). Patients most frequently presented with headache, hearing loss, vertigo, and dizziness. Preoperative facial numbness was reported in 44% of patients. Among the 24 cases, 13 were treated with retrosigmoid craniotomy and 11 via a translabyrinthine approach. Complete resection was achieved in 11 patients (48%), subtotal resection (STR) in 8 patients (35%), and near-total resection (NTR) in 4 patients (17%). Facial nerve outcomes were available in all except one case. Good facial nerve outcomes (House-Brackmann [HB] Grades I-III) were achieved in 82% of the patients who had undergone either NTR or STR, as compared with 73% of patients who had undergone gross-total resection (GTR; p > 0.05, Fisher exact test). In comparison, 83% of patients with solid VSs had a good HB grade (p = 0.38, Fisher exact test), although this finding did not reach statistical significance. Complications included wound infection (2 patients), delayed CSF leakage (1 patient), and a delayed temporal encephalocele following a translabyrinthine approach and requiring surgical repair (1 patient). Cystic vestibular schwannoma represents a clinical and surgical entity separate from its solid counterpart, as demonstrated by its more rapid clinical course and early surgical outcomes. Facial nerve grades may correlate with the degree of tumor resection, trending toward poorer grades with more significant resections. Although GTR is recommended whenever possible, performing an STR when facial nerve preservation is in jeopardy to improve facial nerve outcomes is the preferred strategy at the authors' institution.

Surgical nuances for the endoscopic endonasal transpterygoid approach to lateral sphenoid sinus encephaloceles

Lateral sphenoid encephaloceles of the Sternberg canal are rare entities and usually present with spontaneous CSF rhinorrhea. Traditionally, these were treated via transcranial approaches, which can be challenging given the deep location of these lesions. However, with advancements in endoscopic skull base surgery, including improved surgical exposures, angled endoscopes and instruments, and novel repair techniques, these encephaloceles can be resected and successfully repaired with purely endoscopic endonasal approaches. In this report, the authors review the endoscopic endonasal transpterygoid approach to the lateral recess of the sphenoid sinus for repair of temporal lobe encephaloceles, including an overview of the surgical anatomy from an endoscopic perspective, and describe the technical operative nuances and surgical pearls for these cases. The authors also present 4 new cases of lateral sphenoid recess encephaloceles that were successfully treated using this approach.

Two cases of spontaneous temporal encephalocele

This is a report of two cases of spontaneous temporal encephalocele: one was anteroinferior and presented with epilepsy; the other was posteroinferior and presented with facial neuritis and labyrinthitis. Spontaneous temporal encephalocele is relatively rare and apparently not familiar to a majority of primary physicians. It may present with a variety of symptoms according to its anatomical location, including cerebrospinal fluid fistulas, recurrent meningitis, chronic otitis media, hearing loss, facial nerve palsy and medically intractable epilepsy. Attention should be paid to this disease entity, as it is easily overlooked in imaging studies and can leave serious neurological deficits.

Transmastoid Repair of Temporal Tegmen Defects, Encephaloceles, and CSF Leaks

Transmastoid Repair of Temporal Tegmen Defects, Encephaloceles, and CSF Leaks

Middle Cranial Fossa Approach for Repair of Tegmen Tympani Defects and Temporal Encephalocele

Middle Cranial Fossa Approach for Repair of Tegmen Tympani Defects and Temporal Encephalocele

Poster 392 Temporal Encephalocele and Cholesteatoma Complicating Severe Pediatric Traumatic Brain Injury: A Case Report and Review of the Literature

Poster 392 Temporal Encephalocele and Cholesteatoma Complicating Severe Pediatric Traumatic Brain Injury: A Case Report and Review of the Literature

The surgical management of temporal encephalocele with middle ear infection

The surgical management of temporal encephalocele with middle ear infection

Transtemporal management of temporal bone encephaloceles and CSF leaks: Review of 56 consecutive patients

Conclusions: This report details our experience in the management of 56 consecutive patients with mastoid encephaloceles and cerebrospinal fluid (CSF) leaks. The majority were managed through the transmastoid route with temporalis fascia and calvarial bone graft. Among the patients operated on at least 18 months before analysis, there was a median follow-up of 54 months with no intracranial complications. The average body mass index (BMI) of patients with spontaneous CSF leaks was found to be statistically significantly greater than that of patients with CSF leaks with traumatic, infectious or iatrogenic etiologies. Objectives: To investigate the long-term follow-up of patients with spontaneous, iatrogenic, and traumatic temporal bone encephaloceles and CSF leaks to determine the effectiveness of the repair, late intracranial complications, and recurrent lesions. Methods: This is a retrospective review from a tertiary care center of 56 consecutive patients managed with CSF otorrhea, encephaloceles, and spontaneous pneumocephalus. All patients were repaired through transtemporal, middle cranial fossa or combined approaches for extradural repair and bone grafting. Results: Of the 56 consecutive cases, 12 had CSF otorrhea alone, 19 had encephaloceles, and 23 had both. Two patients presented with spontaneous pneumocephalus without CSF leak. Thirty-three patients had spontaneous onset of their lesion without any history of temporal bone surgery or trauma. The average BMI of patients with spontaneous CSF leaks was found to be greater than the average BMI of patients with CSF leaks due to traumatic, infectious or iatrogenic causes. This difference was found to be statistically significant. Six of these had either preceding or subsequent contralateral mastoid or anterior fossa (sphenoid, ethmoid) CSF leak. Intracranial pressure was evaluated in these cases and most underwent VP shunting to reduce their intracranial pressure. Sixteen cases followed previous mastoid surgery or chronic ear disease and seven were attributed to previous temporal bone trauma. Lesions were repaired with the transtemporal approach (42), middle fossa (4), and combined (5) approaches. Five cases required petrosectomy and fat obliteration. There was one recurrent CSF leak 4 years after initial repair; the leak was anterior to the previously placed bone graft.

Sternberg′s canal as a cause of encephalocele within the lateral recess of the sphenoid sinus: A report of two cases

Background:Intrasphenoidal encephaloceles are extremely rare findings. Sternberg's canal is a lateral craniopharyngeal canal resulting from incomplete fusion of the greater wings of the sphenoid bone with the basisphenoid. It acts as a weak spot of the skull base, which may lead to develop a temporal lobe encephalocele protruding into the lateral recess of the sphenoid sinus (SS).Case Description:We present two cases of intrasphenoidal encephalocele due to persistence of the lateral craniopharyngeal canal. The first case presented with cerebrospinal fluid (CSF) rhinorrhea and the second one was referred to the neurosurgical department with CSF rhinorrhea and meningitis. Radiological investigations consisted of computed tomography (CT) scan, CT cisternography and magnetic resonance images in both cases. These imaging studies identified a herniated temporal lobe through a bony defect which communicates the middle cranial fossa with the lateral recess of the SS. Both patients underwent a transcranial repair of the encephalocele because of the previous failure of the endoscopic surgery. There was no complication related to the surgical procedure and no recurrence of CSF leakage occurred 2 and 3 years after surgery, respectively.Conclusion:Encephalocele within the lateral recess of the SS is a rare entity which must be suspected in patients who present with spontaneous CSF rhinorrhea. Congenital intrasphenoidal encephaloceles, which are located medial to the foramen rotundum, seem to be due to persistence of the Sternberg's canal. Transcranial approach is a good option when a transnasal approach had failed previously.