An antibody to Notch3 reverses the skeletal phenotype of lateral meningocele syndrome in male mice.

Lateral meningocele syndrome (LMS), a genetic disorder characterized by meningoceles and skeletal abnormalities, is associated with NOTCH3 mutations. We created a mouse model of LMS (Notch3tm1.1Ecan ) by introducing a tandem termination codon in the Notch3 locus upstream of the proline (P), glutamic acid (E), serine (S) and threonine (T) domain. Microcomputed tomography demonstrated that Notch3tm1.1Ecan mice exhibit osteopenia. The cancellous bone osteopenia was no longer observed after the intraperitoneal administration of antibodies directed to the negative regulatory region (NRR) of Notch3. The anti-Notch3 NRR antibody suppressed the expression of Hes1, Hey1, and Hey2 (Notch target genes), and decreased Tnfsf11 (receptor activator of NF Kappa B ligand) messenger RNA in Notch3tm1.1Ecan osteoblast (OB) cultures. Bone marrow-derived macrophages (BMMs) from Notch3tm1.1Ecan mutants exhibited enhanced osteoclastogenesis in culture, and this was increased in cocultures with Notch3tm1.1Ecan OB. Osteoclastogenesis was suppressed by anti-Notch3 NRR antibodies in Notch3tm1.1Ecan OB/BMM cocultures. In conclusion, the cancellous bone osteopenia of Notch3tm1.1Ecan mutants is reversed by anti-Notch3 NRR antibodies.

Cerebrospinal Fluid Leak and Symptomatic Pseudomeningocele After Intradural Spine Surgery.

OBJECTIVE: To describe the incidence of and characterize risk factors for cerebrospinal fluid leak and symptomatic pseudomeningocele (CSFL/SP) after intradural spine surgery. METHODS: A retrospective analysis of a prospectively collected and consecutive series of patients undergoing intradural spine surgery by the senior author (A.D.L.) was conducted over a period of 20 years. RESULTS: Data on 460 surgeries performed on 430 consecutive patients were gathered. The incidence of CSFL/SP formation was 2.8% (n = 13). Of the 13 cases complicated by CSFL/SP, 4 were successfully managed nonoperatively (4/13, 31%); 9 postoperative CSFL/SP required surgical repair (9/13, 69%), making for an overall postoperative surgical repair rate of 1.9% (9/460). Factors significantly related to development of postoperative CSFL/SP on the Fisher exact test were surgery located at the craniocervical junction (risk ratio [RR] 2.7, P = 0.03) and use of any external cerebrospinal fluid drain (any drain: RR 2.5, P = 0.02; lumbar drain specifically: RR 2.6, P = 0.02), the latter finding most likely being attributable to selection bias. No significant difference was observed between primary dural closure and closure incorporating the use of one or more dural repair adjuncts. In addition, the total number of dural repair adjuncts used did not significantly influence the development of postoperative CSFL/SP. CONCLUSIONS: We present the largest series of intradural spine surgeries focusing specifically on the risk factors for and management of CSFL/SP. Although craniocervical junction surgery and use of external cerebrospinal fluid drain were associated with CSFL/SP, type of closure and type/number of dural substitutes were not.

Tailored Strategies to Manage Cerebrospinal Fluid Leaks or Pseudomeningocele After Surgery for Tethered Cord Syndrome.

BACKGROUND: Cerebrospinal fluid (CSF) leaks are a dreaded complication after surgery for tethered cord and are associated with significant patient morbidity. Although many strategies for managing postoperative CSF leaks exist, this problem is still daunting, especially in very young patients. In this study, we compared different management techniques for CSF leaks or significant pseudomeningocele in patients with tethered cord syndrome (TCS). METHODS: We analyzed a cohort of children who underwent surgery for TCS from January 2011 to March 2016 (n = 260) and postoperatively experienced either a CSF leak or significant pseudomeningocele. A subset of patients presented with CSF leak (n = 25). We analyzed patient age, sex, presentation, leak appearance, management, and outcome. The different techniques of management were compared for efficacy and morbidity. RESULTS: The diseases associated with leak formation included lipomyelomeningocele (n = 16), myelocystocele (n = 4), and myelomeningocele (n = 5). Three children also had hydrocephalus. Management techniques included cystoperitoneal shunt (CPS) (n = 15), primary resuturing with local rotation flap of muscle (n = 3), external ventricular drain placement (n = 1), ventriculoperitoneal shunt (n = 3), external ventricular drainage (n = 1), and a combination of techniques (rotation flap with external drain; n = 1). Five patients who underwent primary wound revision experienced a leak and required a secondary intervention, but none of the patients who underwent CPS had any complications. CONCLUSIONS: In carefully selected cases, CPS performed early after CSF leakage is highly successful with low morbidity. The primary closure can be attempted for low-pressure leaks without an associated pseudomeningocele.

Melanotic neuroectodermal tumour of infancy: A case report and differential diagnosis.

Melanotic neuroectodermal tumour of infancy is an uncommon pigmented neoplasm of neural crest origin. It was first described in 1918 by Krompecher, known as congenital melanocarcinoma at that time. Although it is generally agreed upon that it is a benign entity, it is locally aggressive and has a significant recurrent risk, reported to be between 10-15%. There have also been prior reports of malignant behaviour in these tumours, although extremely rare. The majority of cases of this tumour (about 70%) arise from the maxilla and its occurrence in the cranial vault represents approximately 15.6% of cases. We describe a rare case of melanotic neuroectodermal tumour of infancy, with simultaneous involvement of the cranial vault and petrous temporal bone, in a four-month-old child, complicated by post-surgical pseudo-meningocele. This case illustrates the diagnostic dilemma in differentiating reactive osseous sclerosis from direct tumour infiltration, both of which can occur in the context of melanotic neuroectodermal tumour of infancy. The discussion places emphasis on differential diagnoses and useful radiological features to assist in clinching the diagnosis of melanotic neuroectodermal tumour of infancy.

Pseudomeningocele Aspiration and Blood Patch Effectively Treats Positional Headache Associated With Postoperative Lumbosacral Pseudomeningocele.

STUDY DESIGN: Retrospective chart review with limited prospective follow-up survey. OBJECTIVE: To evaluate the efficacy and safety of blood patch injection for the treatment of positional headaches caused by postoperative lumbosacral pseudomeningoceles. SUMMARY OF BACKGROUND DATA: Pseudomeningocele is one of the most common complications after posterior lumbosacral spinal surgery. Common treatments include bedrest, abdominal binder use, subarachnoid lumbar drainage, and surgical re-exploration for durotomy closure. To date, only small case reports support the use of epidural blood patch injection for symptomatic pseudomeningocele treatment. METHODS: A retrospective chart review analyzed the outcomes and complications of 19 consecutive patients who underwent blood patch injection, with and without pseudomeningocele aspiration, for symptomatic postoperative lumbosacral pseudomeningoceles between 2009 and 2015. An attempt was made to survey patients by phone regarding satisfaction. RESULTS: As of last follow-up (average time = 22.3 months), 16 patients (84%) experienced headache resolution after blood patch injection and did not require further treatment of their pseudomeningocele. In addition to symptomatic improvement, 12 of the 16 successful patients had imaging, which demonstrated pseudomeningocele resolution. Persistent pseudomeningoceles were demonstrated on imaging among all three unsuccessful patients. CONCLUSION: Pseudomeningocele aspiration followed by blood patch is an effective treatment for symptomatic postoperative lumbosacral pseudomenigocele. This is a minimally invasive alternative to surgical re-exploration with durotomy closure. Injections are most effective when performed early after pseudomeningocele development. LEVEL OF EVIDENCE: 4.

CT-guided epidural blood patch for treatment of CSF leak and pseudomeningocele following tethered cord release in a 3-year-old.

An epidural blood patch (EBP) has become standard of care for management of postdural puncture headache, and in recent years, attempts have been made to expand its applicability. Its utility in the management of postsurgical pseudomeningocele remains poorly defined, and few reports describe its use in children. In this case, we report the successful management of lumbar pseudomeningocele via EBP in a 3-year-old after tethered cord release, thereby obviating the need for surgical repair and its potential morbidity.

Lateral temporal encephaloceles: case-based review.

PURPOSE: Lateral temporal encephalocele is an extremely rare clinical condition, with only 18 cases presented in the literature to date. No review articles have examined lateral temporal encephalocele in depth. We therefore reviewed past cases of lateral encephalocele to clarify the clinical characteristics of this extremely rare deformity. We also present a case of lateral encephalocele with arachnoid cyst which has never been reported in past reports. METHODS: We identified 8 reports describing 18 cases of lateral temporal encephalocele. We therefore reviewed 19 cases of lateral temporal encephalocele, including our own experience, and discussed the clinical characteristics of this pathology. RESULTS: All the cases with lateral temporal encephalocele were detected at birth except for an occult case. The majority occurred at the pterion, and occurrence at the asterion appears much rarer. Due to the preference for the pterion, the ipsilateral orbital wall was also distorted in some cases. Lateral temporal encephalocele seems to have fewer associated malformations: only 3 cases of lateral temporal encephalocele had associated malformations, including our case which was associated with intracranial arachnoid cyst. The only case of lateral temporal encephalocele to have shown hydrocephalus was our own case. Patients with this deformity have relatively good prognoses: only 3 of the 19 cases showed delayed psychomotor development during follow-up. CONCLUSIONS: Provision of adequate treatment is likely to achieve a good prognosis in patients with lateral temporal encephalocele, so we should keep in mind this deformity when encountering pediatric patients with mass lesions on the temporal cranium.

Lumbar pseudomeningocele following blunt trauma without spinal fractures / Pseudomeningocele lombar após trauma fechado sem fraturas vertebrais

The lumbar pseudomeningocele (PSM) is an uncommon condition, mainly, after a lumbar blunt trauma. The authors present a rare case of PSM following a lumbar blunt trauma which did not show any abnormalities in the magnetic resonance imaging (MRI) of the lumbar region. Firstly, the patient underwent to conservative treatment that fail and then it was performed a surgical approach of the lumbar area, however, the lumbar fluid collection appeared again and a lumboperitoneal shunt (LPS) was then performed with complete successful. One year and half afterwards the LPS the patient continues asymptomatic. The standard treatment of this condition remains uncertain, but the conservative treatment followed by LPS is a good option and can be done in several cases.

A pseudomeningocele lombar é uma condição incomum, principalmente após trauma lombar fechado. Os autores apresentam um caso raro de pseudomeningocele lombar após trauma lombar fechado que não apresentou alterações na ressonância magnética da região lombar. Primeiramente, o paciente foi submetido ao tratamento conservador que falhou, sendo submetido, em seguida, à abordagem cirúrgica da região lombar, entretanto, a coleção lombar fluida apareceu novamente, sendo, então, realizada uma derivação lomboperitoneal com remissão completa da coleção. Um ano e meio após a derivação lomboperitoneal, o paciente permanece assintomático. O tratamento padrão dessa patologia ainda permanece incerto, mas o tratamento conservador seguido de derivação lomboperitoneal é uma boa opção e pode ser feito em certos casos.

Spinal nerve root herniation into a pseudomeningocele associated with lumbar spondylolysis: a case report.

A 38-year-old man presented with low back pain and sciatica on the left side. Magnetic resonance imaging showed spinal nerve root herniation into a pseudomeningocele associated with lumbar spondylolysis. After closure of the dural defect, the sciatic pain was relieved and subsequently the patient was able to return to work.

[Meningocele, glioma and optic nerve meningioma: differential diagnosis and treatment]. / Meningocele, glioma y meningioma del nervio óptico: diagnóstico diferencial y tratamiento.

PURPOSE: After studying 3 clinical cases, we have reviewed the clinical and radiological characteristics of meningocele, meningioma and optic nerve glioma. The differential diagnosis and therapeutic management are also discussed. METHODS: Review of three clinical reports of three patients seen in our unit and a bibliographic search concerning the diagnosis and therapeutic management of these three entities at the present time. RESULTS: Differential diagnosis has to be based on a wide range of parameters: epidemiologic (age, race, sex, prevalence of the tumors), clinical (visual acuity, perimetry, Hertel exophthalmometry and funduscopy) and radiologic (computed tomography and magnetic resonance). Anatomopathologic study is required only rarely. The therapeutic options are: observation, surgery and radiotherapy. CONCLUSION: A correct differential diagnosis is mandatory to be able to individualize the treatment for each entity (Arch Soc Esp Oftalmol 2009; 84: 563-568).

Endoscopic endonasal repair of difficult-to-access cerebrospinal fluid leaks of the frontal sinus.

BACKGROUND: Cerebrospinal fluid (CSF) leaks located within the frontal sinus can be difficult to repair effectively. Additional challenges arise from the need to simultaneously repair the leak or defect and to ensure the future patency of the sinus itself. METHODS: The cases of two patients who underwent surgical repair of CSF leaks and skull base defects located in difficult to access locations within their frontal sinuses were reviewed. RESULTS: Two patients with CSF leaks in the frontal sinus underwent surgery to repair the leaks. The leaks were in the superomedial and superolateral locations within the sinus. One leak (in the superolateral location) was secondary to iatrogenic surgical trauma during craniotomy. The second leak (in the superomedial location) was secondary to congenital encephalocele. Surgical approach to ensure adequate exposure was via endoscopic modified Lothrop procedure. Successful repair was undertaken via underlay technique. At follow-up (27 and 7 months, respectively), frontal sinuses remained patent without evidence of CSF leak. CONCLUSION: CSF leaks located in medial and lateral areas of the frontal sinus may be successfully repaired via endonasal approach with endoscopic modified Lothrop. Repair in this manner requires that attention be paid both to achieving a complete repair and to maintaining a patent sinus drainage pathway.

Diagnosis and treatment of isolated sphenoid sinus disease: a review of 109 cases.

CONCLUSION: Endoscopic transethmoidal sphenoidotomy performed mainly in the early stages of the pathology and by expert hands is [WX1]very effective in treating isolated sphenoid sinus disease. OBJECTIVE: This study aimed to investigate the causes of isolated sphenoid sinus disease identified in 109 patients and report on the most appropriate diagnostic and therapeutic patterns for an earlier diagnosis and a successful treatment of the disease. SUBJECTS AND METHODS: A total of 109 subjects with various isolated sphenoid pathologies were first examined by general objective examination, nasal sinus endoscopy, CT scan of paranasal sinuses, and in some cases with MRI. Then, they underwent medical and/or surgical treatment. RESULTS: Nineteen patients (17.43%) had isolated sphenoiditis, 6 (5.5%) fungal sinusitis, 30 (27.52%) mucocele, 6 (5.5%) fibrous dysplasia, 6 (5.5%) meningoencephalocele, 5 (4.58%) inverted papilloma, 4 (3.66%) epidermoid carcinoma, 10 (9.17%) liquor fistula, 1 (0.9%) rhabdomyosarcoma, 1 (0.9%) chordoma, and 1 (0.9%) had carotid pseudoaneurysm. Evidence of definitive diagnosis by endoscopy was obtained in less than half of the cases. CT scan, however, sometimes in combination with MRI, determined the pathology in all the cases. A follow-up of at least 4 years post-surgery showed good results in all the patients who underwent endoscopic transethmoidal sphenoidotomy.

Meningoencephalocele formation after nasal septoplasty and management of this complication.

OBJECTIVE: Patients undergoing nasal septoplasty can face many complications. Some of these complications are rare but their results are life threatening. Being aware of this complication could prevent further problems such as enlargement of the bone and dural defect, herniation of the meninges and brain tissue through the defect by pulsation of the brain and ascending infection. With early diagnosis, a less aggressive method could be used to treat this complication. CASE DESCRIPTION: A 50-year-old woman was admitted our hospital with the complaint of loss of consciousness. Her Glasgow coma score was 7 on admission. She had no lateralizing signs, but had nuchal rigidity. Blood pressure was 200/110mm Hg, the respiratory pattern was apneic, complete blood count revealed 12000 leucocytes/mm3 and arterial blood gases showed respiratory acidosis and other biochemical parameters were within normal limits. Computerized cranial tomography (CCT) showed diffuse brain edema without evidence of other signs. Lumbar puncture was performed revealing purulent and highly viscous cerebrospinal fluid (CSF). CONCLUSION: Late diagnosis and late repair of arachnoidodural tearing could lead to life-threatening complications, and cases with meningitis and larger defects may require more extensive surgery instead of transnasal endoscopic repair.

Spinal cord compression from traumatic anterior cervical pseudomeningoceles. Report of three cases.

Pseudomeningoceles rarely develop after cervical trauma; in all reported cases the lesions have extended outside the spinal canal. The authors report the first known cases of anterior cervical pseudomeningoceles contained entirely within the spinal canal and causing cord compression and neurological injury. The authors retrospectively reviewed the cases of three patients with traumatic cervical spine injuries and concomitant compressive anterior pseudomeningoceles. The lesion was recognized in the first case when the patient's neurological status declined after he sustained a severe atlantoaxial injury; the pseudomeningocele was identified intraoperatively and decompressed. After the decompressive surgery, the patient's severe tetraparesis partially resolved. In the other two patients diagnoses of similar pseudomeningoceles were established by magnetic resonance imaging. Both patients were treated conservatively, and their mild to moderate hemiparesis due to the pseudomeningocele-induced compression abated. The high incidence of anterior cervical pseudomeningoceles seen at the authors' institution within a relatively brief period suggests that this lesion is not rare. The authors believe that it is important to recognize the compressive nature of these lesions and their potential to cause devastating neurological injury.

Maternal anterior sacral meningocele in pregnancy.

Anterior sacral meningocele is a rare condition resulting from herniation of the meninges through a sacral defect. Women with this condition may undergo inappropriate surgery because the correct diagnosis is missed, resulting in serious complications. In labor, the cyst may rupture, with consequent meningitis and high maternal mortality. We report the case of a 31 year-old pregnant woman with an anterior sacral meningocele. We describe the role of magnetic resonance imaging in the evaluation and management planning in this condition. She was delivered by cesarean section at 34 weeks without complications. Accurate diagnosis and delivery by cesarean prior to labor are important in assuring good outcomes in women with anterior sacral meningoceles.

Management of pseudomeningocele following neurotologic procedures.

OBJECTIVES: A pseudomeningocele results from the escape of cerebrospinal fluid through a dural defect with trapping of subarachnoid fluid in surrounding soft tissue. The present study evaluates the incidence of pseudomeningocele following neurotologic procedures and delineates an algorithm for management. METHODS: A retrospective review of 375 consecutive patients undergoing neurotologic procedures at a single institution identified 17 patients with the postoperative complication of pseudomeningocele. RESULTS: The incidence of pseudomeningocele formation was 4.5% in the present study. Fourteen pseudomeningoceles resolved with nonoperative management including pressure dressing, bed rest, and lumbar spinal drainage. Three patients failed nonoperative management and required surgical procedures for resolution. All patients ultimately had resolution of their pseudomeningocele. CONCLUSION: Skull-base pseudomeningoceles occur as a complication following neurotologic procedures and can cause complications as they enlarge. The majority of these cases can be dealt with in a nonsurgical manner, but those failing to respond to conservative management should be considered for surgical intervention.