[Surgical treatment of focal drug-resistant epilepsy associated with temporal lobe encephalocele]. / Khirurgicheskoe lechenie patsientov so strukturnoi fokal'noi farmakorezistentnoi epilepsiei, assotsiirovannoi s visochnymi entsefalotsele.

BACKGROUND: In recent years, temporal lobe encephalocele has become more common in patients with focal drug-resistant epilepsy. Despite available experience, there are still no clear recommendations for choosing the extent of surgery in these patients. OBJECTIVE: To evaluate the effectiveness of diagnosis and surgical treatment of focal drug-resistant epilepsy associated with temporal lobe encephalocele. MATERIAL AND METHODS: The study included 21 patients with focal temporal lobe epilepsy and temporal lobe encephalocele. All patients underwent continuous video-EEG monitoring and MRI of the brain. There were 12 (57.4%) selective encephalocele resections and 9 (42.6%) anterior temporal lobectomies. The median follow-up period was 31 months. RESULTS: The overall effectiveness of surgical treatment with postoperative Engel class I was 76% (16 cases). Selective encephalocele resection was followed by postoperative Engel class I in 10 patients (83%). There were 6 (67%) patients with similar outcomes after temporal lobectomy. Mean volume of resected tissue adjacent to encephalocele was 8.3 cm3. CONCLUSION: Surgery is a highly effective treatment for patients with epileptic seizures following temporal lobe encephalocele. In our sample, favorable postoperative outcomes were achieved in 76% of patients (Engel class I). There were no significant differences in effectiveness between selective resection and temporal lobectomy. Further research is necessary for a clear protocol of surgical treatment of focal drug-resistant epilepsy associated with encephalocele.

Carolyn's window approach for spontaneous frontal sinus meningoencephalocele.

Spontaneous meningoencephaloceles (MECs) are sparsely documented in the literature. Those occurring in the frontal sinus are an exceedingly rare entity. MECs are commonly associated with cerebrospinal fluid (CSF) rhinorrhoea. CSF rhinorrhoea is frequently misdiagnosed, causing delays in diagnosis and management. The subsequently increased risk of bacterial meningitis can be life-threatening to patients. We report the case of a woman in her late 70s with a spontaneous frontal sinus MEC, presenting with a 6-month history of CSF rhinorrhoea. The patient was successfully treated using the novel Carolyn's window approach endoscopically; 9-month follow-up revealed no skull-base breach. Our case emphasises the importance of considering MEC as a differential diagnosis for clear rhinorrhoea and demonstrates successful repair through a novel surgical approach.

Semiautomated electric source imaging determines epileptogenicity of encephaloceles in temporal lobe epilepsy.

OBJECTIVE: We aimed to assess the ability of semiautomated electric source imaging (ESI) from long-term video-electroencephalographic (EEG) monitoring (LTM) to determine the epileptogenicity of temporopolar encephaloceles (TEs) in patients with temporal lobe epilepsy. METHODS: We conducted a retrospective study involving 32 temporal lobe epilepsy patients with TEs as potentially epileptogenic lesions in structural magnetic resonance imaging scans. Findings were validated through invasive intracerebral stereo-EEG in six of 32 patients and postsurgical outcome after tailored resection of the TE in 17 of 32 patients. LTM (mean duration = 6 days) was performed using the 10/20 system with additional T1/T2 for all patients and sphenoidal electrodes in 23 of 32 patients. Semiautomated detection and clustering of interictal epileptiform discharges (IEDs) were carried out to create IED types. ESI was performed on the averages of the two most frequent IED types per patient, utilizing individual head models, and two independent inverse methods (sLORETA [standardized low-resolution brain electromagnetic tomography], MUSIC [multiple signal classification]). ESI maxima concordance and propagation in spatial relation to TEs were quantified for sources with good signal quality (signal-to-noise ratio > 2, explained signal > 60%). RESULTS: ESI maxima correctly colocalized with a TE in 20 of 32 patients (62.5%) either at the onset or half-rising flank of at least one IED type per patient. ESI maxima showed propagation from the temporal pole to other temporal or extratemporal regions in 14 of 32 patients (44%), confirming propagation originating in the area of the TE. The findings from both inverse methods validated each other in 14 of 20 patients (70%), and sphenoidal electrodes exhibited the highest signal amplitudes in 17 of 23 patients (74%). The concordance of ESI with the TE predicted a seizure-free postsurgical outcome (Engel I vs. >I) with a diagnostic odds ratio of 2.1. SIGNIFICANCE: Semiautomated ESI from LTM often successfully identifies the epileptogenicity of TEs and the IED onset zone within the area of the TEs. Additionally, it shows potential predictive power for postsurgical outcomes in these patients.

Endoscopic endonasal repair of encephaloceles of the lateral sphenoid sinus: multiinstitution confirmation of a new classification.

OBJECTIVE: Encephaloceles of the lateral sphenoid sinus are rare. Originally believed to be due to defects in a patent lateral craniopharyngeal canal (Sternberg canal), they are now thought to originate more commonly from idiopathic intracranial hypertension, not unlike encephaloceles elsewhere in the skull base. A new classification of these encephaloceles was recently introduced, which divided them in relation to the foramen rotundum. Whether this classification can be applied to a larger cohort from multiple institutions and whether it might be useful in predicting outcome is unknown. Thus, the authors' goal was to divide a multiinstitutional cohort of patients with lateral sphenoid encephaloceles into four subtypes to determine their incidence and any correlation with surgical outcome. METHODS: A multicenter retrospective review of prospectively acquired databases was carried out across three institutions. Cases were categorized into one of four subtypes (type I, Sternberg canal; type II, medial to rotundum; type III, lateral to rotundum; and type IV, both medial and lateral with rotundum enlargement). Demographic and outcome metrics were collected. Kaplan-Meyer curves were used to determine the rate of recurrence after surgical repair. RESULTS: A total of 49 patients (71% female) were included. The average BMI was 32.8. All encephaloceles fell within the classification scheme. Type III was the most common (71.4%), followed by type IV (16.3%), type II (10.2%), and type I (2%). Cases were repaired endonasally, via a transpterygoidal approach. Lumbar drains were placed in 78% of cases. A variety of materials was used for closure, with a nasoseptal flap used in 65%. After a mean follow-up of 47 months, there were 4 (8%) CSF leak recurrences, all in patients with type III or type IV leaks and all within 1 year of the first repair. Two leaks were fixed with ventriculoperitoneal shunt and reoperation, 1 with ventriculoperitoneal shunt only, and 1 with a lumbar drain only. Of 45 patients in whom detailed information was available, there were 12 (26.7%) with postoperative dry eye or facial numbness, with facial numbness occurring in type III or type IV defects only. CONCLUSIONS: Endoscopic endonasal repair of lateral sphenoid wing encephaloceles is highly successful, but repair may lead to dry eye or facial numbness. True Sternberg (type I) leaks were uncommon. Failures and facial numbness occurred only in patients with type III and type IV leaks.

Meckel-Gruber syndrome together with Dandy-Walker malformation: an atypical case report of a 2nd recurrence in a consanguine marriage.

Meckel-Gruber syndrome is a lethal disorder characterized by occipital encephalocele, polycystic kidneys, and polydactyly. In most cases, it is identified and terminated antenatally. In this report, the authors present a case of Meckel-Gruber syndrome together with Dandy-Walker malformation. A pregnant woman referred at the 28th week of gestation with an abnormal ultrasound scan showing posterior encephalocele and bilaterally enlarged kidneys. Further imaging also indicated communication between the 4th ventricle and posterior cerebellar cerebrospinal fluid space, after which the fetus was diagnosed with Meckel-Gruber syndrome and Dandy-Walker malformation. Pregnancy termination was refused by the parents and the offspring was prematurely born to be the 2nd recurrence of Meckel-Gruber syndrome in this consanguine family. Remarkably, at the 3 different pregnancies, ultrasound was inconclusive before the 7th month of gestation. Though up to date Meckel-Gruber syndrome is ultimately lethal, the lifespan of affected newborns varied greatly. We suggest developing a severity classification to estimate life expectancy in unterminated cases.

Atretic cephalocele associated with sinus pericranii: a single-center analysis.

PURPOSE: The purpose of this report is to investigate the association of atretic cephalocele (AC) with sinus pericranii (SP) in order to improve the management of AC. METHODS: Pediatric patients with AC who underwent repair surgeries were retrospectively analyzed. Anomalies including SP were evaluated using computed tomography angiography and venography (CTA/V) as well as magnetic resonance imaging. RESULTS: Fourteen patients were identified, comprising of 5 males and 9 females. The AC was located interparietally in 8 cases and occipitally in 6 cases. Seven cases (50%) were found to be associated with SP, all of which did not involve major intracranial venous circulation. Five out of 8 parietal ACs (63%) were accompanied by SP, while 2 out of 6 occipital ACs (33%) exhibited SP. All of the SP associated with AC only contributed to a small portion of the venous outflow, and the repair surgeries were successfully performed without excessive bleeding. CONCLUSION: More than half of the ACs were associated with SP, with a higher prevalence in the parietal region compared to the occipital region. For the appropriate management of AC, preoperative investigation of SP using CT venography is considered important.

A rare case of occipital encephalocele presenting as the largest congenital head mass in an infant.

BACKGROUND: The prevalence of encephalocele is estimated to be 0.8-5.0 per 10,000 live births. The most frequent encephalocele is the occipital encephalocele. It is a congenital neural tube defect characterized by the protrusion or herniation of intracranial contents through a cranial defect. The term "giant/massive/large encephalocele" is used to describe an encephalocele that is significantly larger than the size of the head. CASE DESCRIPTION: A 2-month-old male infant presented in the neurosurgery outpatient department with one of the largest head masses over the posterior aspect since birth. The swelling was gradually progressive and developed ulceration over the swelling with intermittent cerebrospinal fluid (CSF) discharge but no associated weakness in limbs. Magnetic Resonance Imaging (MRI) brain showed a large occipital meningoencephalocele containing predominantly cyst with part of the cerebellar and occipital lobe. The surgery was planned. The sac contained CSF with the gliotic occipital lobe. The sac and gliotic brain tissue was excised. He had an uneventful postoperative course. CONCLUSION: Surgery serves several functions, including reducing the torque and weight of the head to allow for more normal motor development, removal of the thin, leaking scalp and dural closure to prevent CSF leak and subsequent infection, and improving the cosmetic and social issues that the child and family may have to endure.

Pathogenesis and treatment of a giant occipital bone defect with meningoencephalocele in an NF1 child: case report and review of the literature.

Autosomal dominantly inherited neurofibromatosis type I (NF1) is a systemic disorder caused by a mutation of a gene on chromosome 17q11.2 and characterized by multiple café-au-lait spots, lentiginous macules, Lisch nodules of the iris, and tumors of the nervous system. Bony manifestations such as scoliosis, dysplasia of the greater sphenoidal wing, tibial pseudoarthrosis, short stature, and macrocephaly have been reported in approximately 50% of patients. However, calvarial bone defects are rare. After screening 324 articles, 23 cases (12 adult and 11 pediatric patients) of occipital bone defects in NF1 patients were selected. All patients had a single/multiple bone defect over the lambdoid suture. Adjacent benign plexiform neurofibromas were observed in 14 patients (60.8%, 7 adults and 7 children); one adult patient was diagnosed with neurofibrosarcoma. Meningoencephalocele over the occipital defect was noted in 8 cases (34.78%, all adults). Cranioplasty was performed in only 17.39% of patients. Histologic examination was performed in 7 of the 15 patients with associated neurofibromas/neurofibrosarcomas. Biopsy of the bone margins surrounding the defect was performed in only one case. Pathologic examination of the herniated parieto-occipital or cerebellar tissue was not performed in any of the patients studied. We report the case of a 9-year-old girl with NF1 and a significant occipital bone defect and performed a systematic review of the relevant literature to highlight the challenges in treating this condition and to investigate the underlying mechanisms contributing to bone defects or dysplasia in NF1.

How I do it? Endoscopic endonasal transpterygoid repair of sphenoid lateral recess cerebrospinal fluid leak after previous unsuccessful transcranial surgery.

BACKGROUND: Direct access to the sphenoid lateral recess offers the best chance of sealing spontaneous cerebrospinal fluid (CSF) rhinorrhea caused by lateral sphenoid encephaloceles of the Sternberg canal defect. METHOD: We present a case of spontaneous left-sided sphenoid lateral recess CSF leak after previous unsuccessful transcranial surgery managed with an endoscopic endonasal transpterygoid approach (EETA). An anatomical-based step-by-step illustration of the EETA was presented in the surgical video. CONCLUSION: This case demonstrates the value of endoscopic endonasal transpterygoid corridor in the exposure and manipulation of the sphenoid lateral recess.

Drug-resistant temporal lobe epilepsy with temporal encephaloceles: How far to resect.

PURPOSE: This study sought to evaluate the impact of surgical extent on seizure outcome in drug-resistant temporal lobe epilepsy (DR-TLE) with temporal encephaloceles (TE). METHODS: This was a single-institution retrospective study of patients who underwent surgery for DR-TLE with TE between January 2008 and December 2020. The impact of surgical extent on seizure outcome was evaluated. In a subset with dominant DR-TLE, the impact of surgical extent on neuropsychometric outcome was evaluated. RESULTS: Thirty-four patients were identified (female, 56%; median age at surgery, 43 years). TE were frequently overlooked on initial magnetic resonance imaging (MRI), with encephaloceles only detected after repeat or expert re-review of MRI, additional multi-modal imaging, or intra-operatively in 31 (91%). Sixteen (47%) underwent limited resections, including encephalocele resection only (n = 5) and encephalocele resection with more extensive temporal corticectomy sparing the amygdala and hippocampus (n = 11). The remainder (n = 18, 53%) underwent standard anterior temporal lobectomy and amygdalohippocampectomy (ATLAH). Limited resection was performed more frequently on the left (12/17 vs. 4/17, p = 0.015). Twenty-seven patients (79%) had a favourable outcome (Engel I/II), and 17 (50%) were seizure-free at the last follow-up (median seizure-free survival of 27.3 months). There was no statistically significant difference in seizure-free outcomes between limited resection and ATLAH. In dominant DR-TLE, verbal memory decline was more likely after ATLAH than limited resection (3/4 vs. 0/9, p = 0.014). CONCLUSION: Expert re-review of imaging and multi-modal advanced imaging improved TE identification. There was no statistical difference in seizure-free outcomes based on surgical extent. Preservation of verbal memory supports limited resection in dominant temporal cases.

Meningoencephalocele and supernumerary ectopic limb arising from the occipital bone in a Jacob sheep lamb.

This report describes the clinical, computed tomography, and magnetic resonance imaging findings for a Jacob sheep lamb diagnosed with meningoencephalocele and supernumerary ectopic limb. Key clinical message: This case demonstrates the utility of tomographic imaging in diagnosing congenital malformations in sheep and can be used to assess the extent of the lesion. This may help to determine any viable treatment, or, as in the case presented here, determine that the extent of the lesion precludes surgical intervention.

Méningoencéphalocèle et membre ectopique surnuméraire provenant de l'os occipital chez un agneau Jacob. Ce rapport décrit les résultats cliniques, de tomodensitométrie et d'imagerie par résonance magnétique d'un agneau de Jacob chez qui on a diagnostiqué une méningoencéphalocèle et un membre ectopique surnuméraire.Message clinique clé :Ce cas démontre l'utilité de l'imagerie tomographique dans le diagnostic des malformations congénitales chez le mouton et peut être utilisée pour évaluer l'étendue de la lésion. Cela peut aider à déterminer tout traitement viable ou, comme dans le cas présenté ici, à déterminer que l'étendue de la lésion exclut une intervention chirurgicale.(Traduit par Dr Serge Messier).

MRI features to aid the identification of lateral temporal bone cephaloceles.

OBJECTIVES: To evaluate ancillary MRI features which may aid the identification of lateral temporal bone cephaloceles (LTBCs). METHODS: A retrospective cohort study analysed patients with MRI evidence of surgically confirmed spontaneous LTBCs as defined by intracranial contents traversing the tegmen tympani or mastoideum. Cases were identified from radiology and surgical databases. Two observers analysed three-dimensional T 2W temporal bone and whole brain imaging according to a priori criteria by consensus, with emphasis on the relationship of any adjacent cerebrospinal fluid (CSF) cleft to the defect. The contents, location, and clinical features of the LTBCs were recorded. RESULTS: Eighteen patients (11 female, 7 male; mean age 59.3 years, age range 42-86 years) with 20 surgically confirmed spontaneous LTBCs (2 bilateral;16 unilateral) were evaluated. A temporal lobe sulcus or other CSF cleft extending to or traversing the defect was identified in 19/20 (95%) cases. Isointense CSF tympanomastoid signal was present in 41.2% cases, whilst superior semi-circular canal dehiscence was found in 40% of cephaloceles. At least two MRI features of idiopathic intracranial hypertension were seen in 38.9% patients. Cephaloceles were most commonly centred on the tegmen tympani (55%). Meningoencephaloceles were present in 95% cases. CONCLUSION: A temporal lobe sulcus or CSF cleft extending to or traversing the defect may aid the identification of LTBCs. Isointense CSF tympanomastoid signal, superior semi-circular canal dehiscence and MRI features of idiopathic intracranial hypertension are only present in under half of LTBCs. ADVANCES IN KNOWLEDGE: The study details novel ancillary MRI features of LTBCs which may aid their identification.

Distinctive electro-clinical, neuroimaging and histopathological features of temporal encephaloceles associated to epilepsy.

OBJECTIVE: Encephaloceles (ENCs) may cause clinical complications, including drug-resistant epilepsy that can be cured with epilepsy surgery. METHODS: We describe clinical, diagnostic, and neuropathological findings of 12 patients with temporal ENC and epilepsy evaluated for surgery and compare them with a control group of 26 temporal lobe epilepsy (TLE) patients. RESULTS: Six patients had unilateral and 6 bilateral temporal ENCs. Compared to TLEs, ENCs showed i) later epilepsy onset, ii) higher prevalence of psychiatric comorbidities, iii) no history of febrile convulsions, and iv) ictal semiology differences. Seven patients had MRI signs of gliosis, and 9 of intracranial hypertension. Interictal EEG analysis in ENCs demonstrated significant differences with controls: prominent activity in the beta/gamma frequency bands in frontal regions, interictal short sequences of low-voltage fast activity, and less frequent and more localized interictal epileptiform discharges. Ictal EEG patterns analyzed in 9 ENCs showed delayed and slower contralateral spread compared to TLEs. All ENCs that underwent surgery (7 lobectomies and 1 lesionectomy) are in Engel class I. Neuropathological examination revealed 4 patterns: herniated brain fragments, focal layer I distortion, white matter septa extending into the cortex, and altered gyral profile. CONCLUSIONS AND SIGNIFICANCE: The described peculiarities might help clinicians to suspect the presence of largely underdiagnosed ENCs.

Flocculus Herniation into the Internal Acoustic Canal in Incomplete Partition Type I: A Case Report.

In this study, we present the first case with cerebellar herniation into the internal acoustic canal in incomplete partition type I anomaly. Cerebellar herniation into the internal acoustic canal is very rare with only a few cases reported in the literature. Although it is a rare clinical situation, cerebellar herniation into the internal acoustic canal may be seen in patients with incomplete partition type I. We presented magnetic resonance imaging findings of a 3-year-old girl with a history of meningitis, middle ear effusion, and bilateral congenital sensorineural hearing loss. Magnetic resonance imaging showed bilateral incomplete partition type I malformation and an additional flocculus herniation into the right internal acoustic canal. In the presented case, predisposition to cerebrospinal fluid leak in incomplete partition type I anomaly may be the reason for cerebellar herniation into internal acoustic canal. Also, possible increased intracranial pressure due to meningitis may be a contributing factor.

Amniotic band syndrome with CNS involvement: a pediatric neurosurgeon's dilemma-a case series and literature review.

BACKGROUND: Amniotic band syndrome (ABS) is a rare congenital disease characterized by a broad spectrum of congenital anomalies resulting from the strangulated developing organ(s) by the detached fibrous amniotic band. The prevalence of CNS involvement in ABS is rare, but the mortality rate in these cases is high, while morbidity among the surviving patients is inevitable. CASE REPORT: Three-month-old male, 9-month-old female, and newborn female babies were presented with head lump(s), severe facial cleft, syndactyly, and finger amputation. The patient's head imaging confirmed meningoencephalocele as the cause of the head lump in 2 patients; meanwhile, a porencephalic cyst was identified as the origin of head lumps in the other patient. VP shunt placement surgery was performed as the initial management in 2 patients, while one patient directly underwent meningoencephalocele resection surgery. Craniofacial and limb reconstructions were planned as the follow-up management in all cases. Unfortunately, one patient died of complications from suspected aspiration, while another never returned for follow-up treatment. CONCLUSION: Here, we report 3 ABS cases with CNS involvement. Despite the severe disfigurement and disability, the inexistence of fatal malformation might lead to long-term survival. The treatment of malformation(s) that might predispose to another fatal condition and surgery(-ies) to improve functional outcomes and patient's social acceptability should be prioritized in managing the surviving ABS patients.

Torsion of giant occipital encephalocoele in a 2-day-old infant: emergent surgery and outcome.

INTRODUCTION: Torsion of a giant occipital encephalocele leading to necrosis is an extremely rare complication found in neonates with only two such cases reported in literature previously. Infection and ulceration of the necrosed skin may lead to meningitis or sepsis. We present here a neonate with giant occipital encephalocele showing progressive necrosis during the first day of his life. CASE REPORT: A new-born baby, delivered vaginally, with no antenatal imaging was found to have a huge mass in the occipital region, which was covered by normal pink-purplish skin. During the first day of his life, the sac started becoming ulcerated accompanied with a rapid colour change in the skin, gradually turning darker and then black. The pedicle of the encephalocele was twisted with progressive necrosis of the encephalocele. MRI showed a giant encephalocele with single vein draining into the torcula and dysplastic occipital lobe herniating into the defect. The neonate was taken up for urgent excision and repair of the encephalocele. The encephalocele was excised 'in-total' with 'figure-of-8' repair of the meninges. One year after the operation, she appears to be well-developed without any neurological problems. CONCLUSION: Necrosis may have resulted from arterial or venous compromise caused by torsion of the pedicle during delivery or after birth. The high pressure inside the sac associated with the thin skin of the encephalocele may be another predisposing factor. In view of the risk of meningitis and rupture, immediate surgery with repair with minimal blood loss is indicated.

Lateral Transorbital Approach for Repair of Lateral Sphenoid Sinus Meningoencephaloceles in Proximity to Foramen Rotundum: Cadaveric Study and Case Report.

BACKGROUND: The repair of lateral sphenoid sinus cerebrospinal fluid leaks is routinely accomplished through the use of the endonasal endoscopic approach (EEA) with a transpterygoidal extension. This approach can incur sinus morbidity, damage to the vidian, palatine and trigeminal nerves, and the contents of the pterygopalatine fossa, particularly if the encephalocele is lateral to the foramen rotundum (FR) and V2. OBJECTIVE: To investigate the use of the lateral transorbital approach (LTOA) as an alternative approach for repair of lateral sphenoid sinus encephaloceles that avoids the potential morbidity of EEA. METHODS: We performed cadaveric dissections of 2 specimens (4 sides) and present one of the first cases of a lateral sphenoid sinus encephalocele repair lateral to the FR in a patient through an ipsilateral LTOA. RESULTS: We find that the LTOA provides a shorter distance to target compared with the EEA (56 vs 89.5 mm, P = .002). The LTOA field of view also affords excellent visualization of both the medial and lateral aspects of V2, whereas the EEA is less effective at exposing lateral to V2, even after sacrifice of the vidian nerve and maximal pterygopalatine fossa content retraction. We report a case of LTOA to repair a meningoencephalocele lateral to V2 in the sphenoid sinus. CONCLUSION: The LTOA to the foramen rotundum is a more direct approach that minimizes the morbidity associated with EEA to repair meningoencephaloceles both medial and lateral to foramen rotundum.