Factors affecting clean intermittent catheterization compliance among children and adolescents with neurogenic bladder due to spina bifida and caudal regression syndrome.

INTRODUCTION: Children and adolescents with neurogenic bladder often need clean intermittent catheterization (CIC) over a long period. Our study aimed to identify factors that affect CIC compliance and to determine if CIC compliance affected short-term urological outcomes among patients in Malaysia. STUDY DESIGN: 50 patients aged 2-18 years who perform CIC were included in this cohort study. Patient compliance with CIC was evaluated using the validated Intermittent Catheterization Adherence Scale (ICAS). CIC difficulties were assessed using the validated Intermittent Catheterization Difficulty Questionnaire (ICDQ). Data was obtained on patients' co-morbidity, caregiver factors, socio-economic factors, CIC technique, access to catheters and facilities, urinary tract infections, incontinence, urology tests and treatment. Statistical analysis was performed. RESULTS: Mean age was 6.68 ± 4.34 years. 32 (64%) patients commenced CIC within the first month of life. Mean daily CIC frequency was 4.70 ± 1.33.30 (60%) participants showed strong adherence to CIC. 39 (78%) participants were able to catheterize with no or minor difficulties. Pain (6, 12%), transient blocking sensation (6.12%), and urinary incontinence (3, 6%) were the predominant difficulties encountered. CIC performed by caregiver was associated with improved adherence compared to patient self-catheterization (p = 0.039). The mean age of participants who self-catheterized was 10.7 ± 3.7 years. Strong adherence was also observed among patients who purchased their own CIC catheters (p = 0.007). Participants with lower ICDQ score were more likely to be compliant with CIC (p = 0.007). CIC adherence was not affected by patient's age, gender, co-morbidity, mobility, caregiver factors, socio-economic factors, and age at initiation of CIC. There was no significant association between CIC adherence and febrile urinary tract infections, upper tract deterioration, and bladder stones at 6 months follow-up. DISCUSSION: There is lower CIC adherence when a child begins to self-catheterize and healthcare providers should be alert during this period of transition. Though most patients with spina bifida have decreased urethral sensation, some patients do experience significant pain during CIC which may impact their compliance. These patients would need a review of their catheterization techniques to improve adherence. The limitations of our study are its modest sample size from a single center and short study period. Our study provides insights into the feasibility of instituting CIC in developing countries. CONCLUSION: Strong CIC adherence was observed among patients who were catheterized by their caregiver, purchased their own CIC catheters, and encountered minimal difficulties during catheterization. CIC adherence had no effect on short-term urological outcomes.

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.

Arthrogryposis in a Case of Chiari Malformation II: First Case Report in a Mediterranean Population.

BACKGROUND Arthrogryposis multiplex congenita is a multifactorial syndromic or non-syndromic group of conditions consisting of multiple congenital contractures of the body, of unknown etiology. It is associated with a heterogenous group of disorders that include but are not limited to processes such as myopathic and neuropathic. Neural tube defect is a neuropathic disorder that incorporates myelomeningocele that might be either isolated or within a spectrum of multiple diseases. CASE REPORT This is a case report of a 28-day-old male born with lower limb arthrogryposis with myelomeningocele and Chiari II malformation in a Mediterranean population. CONCLUSIONS Lower extremity arthrogryposis with myelomeningocele and Chiari II malformation is a prenatal diagnosis that requires high clinical suspicion, early multidisciplinary intervention, and genetic counselling. As long as new approaches are being explored in the management of such cases, babies born now with neural tube defects can expect better quality of life.

Rupture of Giant Anterior Sacral Meningocele in a Patient with Marfan Syndrome: Diagnosis and Management.

BACKGROUND: Marfan syndrome is a genetic disorder that results in the weakening of connective tissues. Dural ectasia has been defined as a feature of Marfan syndrome and is present in up to 92% of patients. Rarely, dural ectasia can erode through the sacrum expanding into an anterior sacral meningocele. CASE DESCRIPTION: Information for this case report was gathered from patient notes and imaging from the patient chart. This is a case of a 46-year-old woman who presented with urinary incontinence, early satiety, and back pain in the setting of a known anterior sacral meningocele. Before operative management, the anterior sacral meningocele ruptured with the patient presenting signs and symptoms of intracranial hypotension. Conservative management did not alleviate the pain. She was ultimately managed with posterior sacroplasty followed by anterior sacral meningocele resection and placement of a lumboperitoneal shunt. The patient did not have reaccumulation of the meningocele or recurrent symptoms at the latest follow-up. CONCLUSIONS: The progression of dural ectasia in Marfan syndrome to an anterior sacral meningocele is uncommon. It is important to identify the characteristics associated with an expanding dural ectasia as this patient's symptoms progressed over time and the meningocele grew large. Given its rarity, there are no guidelines in place regarding size at which repair of an anterior sacral meningocele should occur prophylactically. It is important to review these cases in order continue to learn about progression, management, and outcomes of patients with an anterior sacral meningocele.

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.

Orbital and nasal meningoencephaloceles secondary to chronic hydrocephalus: A rare cause of bilateral proptosis.

Introduction Orbital meningoencephalocele formation is primarily a result of congenital defects in the pediatric population and trauma of the anterior cranial fossa in adults. We present a unique case of nontraumatic nasal and orbital meningoencephaloceles presenting as bilateral proptosis with exotropia secondary to chronic hydrocephalus. Clinical presentation A 20-year-old male with a history of tuberous sclerosis, X-linked intellectual disability, and epilepsy presented to the emergency department with two days of nausea, emesis, seizures, and two months of progressive proptosis. Initial radiographs of the skull showed a "copper beaten" appearance, indicating chronically elevated intracranial pressure. Computed tomography imaging of the head demonstrated bilateral defects in the cribriform plate and anterior cranial fossa. Magnetic resonance imaging of the brain revealed triventricular hydrocephalus with meningoencephalocele extension into the nasal cavity and frontal horn herniation into the extraconal space of the orbits. The hydrocephalus was managed with ventriculoperitoneal shunt placement with rapid and complete resolution of the proptosis. Conclusion No reports have described bilateral proptosis as the presenting finding of orbital and nasal meningoencephaloceles in the absence of trauma or congenital defect. This case study demonstrates the management of meningoencephalocele formation secondary to chronic hydrocephalus.

¿Es el manejo conservador el mejor recurso para el tratamiento de un pseudomeningocele como complicación del implante de un dispositivo de liberación continua de fármacos intratecales para control del dolor? / Is conservative management, the best resource for treatment a pseudomeningocele as a complication of the implantation of a continuous release device for intrathecal drugs for a pain control?

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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.

Tratamiento mediante punción guiada por TC de un meningocele intrasacro oculto doloroso: caso clínico y revisión de la literatura / Treatment by CT-guided aspiration in a painful hidden intrasacral meningocele: case report and review of literature

Casos clínicos: Presentamos una paciente de 43 años con historia clínica de dolor sacro incapacitante 8/10 en la escala de EVA de 8 meses de evolución. No se apreciaban alteraciones sensitivas ni pérdida del control de esfínteres. Realizó consulta en múltiples hospitales. Se le practicaron una tomografía computadorizada (TC) lumbar que puso de relieve un hemangioma en L5, una resonancia magnética (RM) de pelvis en la que se evidenció rotura muscular grado I del glúteo mayor izquierdo y una lesión quística en el sacro que se interpretó como quiste de Tarlov. Recibió tratamiento analgésico y antiinflamatorio sin mejoría. Se le realizaron dos bloqueos facetarios e incluso un bloqueo del músculo piriforme izquierdo, todo ello sin resultados. En nuestro centro se practicó una RM lumbo-sacra que puso de relieve la lesión quística de localización central y lateral derecha en canal espinal sacro a la altura de la transición S2-S3. Era de alta señal en T2 y de baja señal en T1. Según la clasificación de Nabors correspondía a un meningocele intrasacro oculto. Discusión: El meningocele intrasacro oculto (quiste meníngeo extradural tipo lB de la clasificación de Nabors) es una entidad poco frecuente que se presenta generalmente entre la tercera y cuarta década de la vida, como un cuadro clínico insidioso de dolor en extremidades inferiores, con o sin síntomas radiculares, disfunción de esfínteres y alteraciones sensitivas. El tratamiento habitual de este tipo de lesiones es la intervención quirúrgica. En nuestro caso se realizó punción y evacuación del quiste sacro guiado por TC obteniéndose unos 2 cc de un líquido sero-hemático. La evolución posterior fue de mejoría notable del dolor sacro que paso a ser de 3/10 en la escala de EVA. En la revisión de la literatura que hemos efectuado este es el primer caso en que se trata un meningocele intrasacro oculto doloroso mediante punción evacuadora guiada por TC (AU)

Case reports: We report a 43-year old patient with a history of disabling pain sacrum 8/10 on the VAS of 8 months. No sensory disturbances were noted or loss of bowel control. Consultation conducted in multiple hospitals. She underwent a computed tomography (CT) lumbar that highlighted a hemangioma in L5, a magnetic resonance imaging (MRI) of the pelvis which showed muscle breakdown grade I of the left maximus gluteus, and a cystic lesion in the sacrum, which was interpreted as a Tarlov cyst. She received analgesic and anti-inflammatory treatment with no improvement. She also goes through two facet blocks and even a lock in the left piriformis, all without results. In our center we practised a lumbosacral MRI which highlighted a cystic lesion localized in central and right lateral sacral spinal canal at the level of S2-S3 transition. It was high signal on T2 and low signal on T1. Discussion: According to Nabors classification, this lesion corresponds to a hidden intrasacro meningocele. The hidden intrasacro meningocele (extradural meningeal cyst type classification LB Nabors) is a rare entity that usually develops between the third and fourth decade of life, as an insidious clinical lower extremity pain, with or without radicular symptoms, bowel dysfunction and sensory disturbances. The usual treatment of these lesions is surgical intervention. In our case puncture and evacuation guided by CT of the sacral cyst was performed, yielding about 2 cc of a sero-hematic fluid. The postoperative course was a remarkable improvement, and the sacral pain happened to be 3/10 on the VAS. In the literature that we have reviewed, this is the first case in which a hidden painful intrasacro meningocele is evacuated by CT-guided puncture (AU)

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.

Anterior sacral pseudomeningocele following minimal trauma: case report.

Sacral fractures are rare and seldom result in formation of a sacral pseudomeningocele. Treatment of these pseudomeningoceles usually consists of conservative management with flat bedrest or open operative management. The authors describe the case of a 55-year-old woman with an anterior sacral pseudomeningocele that was successfully treated using a lumbar drain for temporary continuous CSF drainage. The patient first presented to an outside institution several days after sacral trauma from an ice skating fall. Initial symptoms included throbbing headaches relieved by lying flat. Head and cervical spine CT demonstrated no abnormality. As symptoms worsened, she presented to another institution where MRI of the lumbar spine indicated sacral fracture with pseudomeningocele. The patient subsequently transferred to the authors' facility, where symptoms included headaches and occasional mild sacral pain. Given her headaches and the authors' concern for CSF leak, another head CT scan was performed. This revealed no subdural hematoma or other abnormality. A subsequent CT myelogram revealed an anterior sacral pseudomeningocele at S3-4 with an anterior irregular linear filling defect, likely representing torn dura. Treatment included placement of a lumbar drain (10 ml/hr) and flat bedrest. Resolution of the CSF leak occurred on postprocedure Day 9. At the 4-week follow-up visit, the patient had no clinical symptoms of CSF leak and no neurological complaints. To our knowledge, this is the first description of temporary continuous CSF drainage used to treat a posttraumatic sacral pseudomeningocele. This technique may reduce the need for potentially complicated surgical repair of sacral fractures associated with CSF leak in select patients.

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.

Anterior sacral meningocele.

Anterior sacral meningocele is a rare condition presenting as a lower abdominal mass. One such case was seen in a baby girl aged 2 months, who was admitted with abdominal distension and urinary difficulty for one week. She had a lower abdominal mass which investigated by MRI, turned out to be an anterior sacral meningocele causing her symptoms. The meningocele was excised successfully via an open abdominal approach. Postoperative recovery and follow-ups remained uneventful. Presentation, various diagnostic modalities and treatment options for an anterior sacral meningocele are briefly discussed.

[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).