A Giant Basal Meningoencephalocele Repair in a Neonate: A Case Report.

Primary encephaloceles are congenital mesodermal defects that result in brain tissue protruding through the skull. These defects most commonly occur occipitally but can be present anywhere in the calvarium. Meningoencephaloceles are a subclassification that includes herniation of the meninges. Basal meningoencephaloceles with cleft palate defects are the rarest form, with very few reports discussing anesthetic implications. We report a case of a giant basal meningoencephalocele that involves the nasal and oral cavities with a risk of thecal sac rupture.

Unruptured giant lateral thoracic meningocele: extremely rare cause of cerebrospinal fluid (CSF) hypotension in neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1) is a multisystem neurocutaneous disorder. Scoliosis and dural ectasia are features of the associated mesodermal dysplasia. Lateral thoracic meningoceles can develop in NF1 and progressively enlarge due to cerebrospinal fluid (CSF) pulsations. Large meningoceles can cause compressive symptoms in the thorax. We are reporting a case of a NF1 presenting with acute onset respiratory distress, who also had chronic orthostatic headaches. CT chest showed unruptured enlarging bilateral lateral thoracic meningoceles causing lung compression. MRI of the brain and spine showed features of CSF hypotension, explaining the headaches. CSF hypotension with unruptured meningoceles is extremely rare. Management of the condition is challenging since surgical removal is prone to complications due to underlying mesodermal abnormalities. Cystoperitoneal shunting to relieve lung compression may worsen CSF hypotension. A shunt with a programmable valve allowed controlled drainage and successfully relieved lung compression without worsening of orthostatic headaches in our case.

Pseudomeningocele Mimicking Sciatica.

We report on an elderly woman with sciatica due to disc herniation, experiencing complete pain resolution following surgery. Four weeks later, she developed refractory, excruciating pain with foot palsy, prompting a lumbar magnetic resonance imaging that revealed no recurrent disc herniation. Upon exploration, intermittent nerve entrapment through the dural tear and pseudomeningocele was identified. Following dural repair and nerve repositioning, the postoperative course and long-term outcome were uneventful.In contrast to recurrent disc herniation, sciatica resulting from nerve entrapment by a pseudomeningocele is exceedingly rare. Its differential diagnosis is crucial in cases with seemingly unspectacular magnetic resonance imaging findings, as paralysis can occur in symptomatic patients. The radiological presence of a postoperative pseudomeningocele could be overlooked, and the cause of sciatica might become apparent solely during surgical exploration.

Challenges in the Management of Symptomatic Fallopian Canal Meningoceles: A Multicenter Case Series and Literature Review.

OBJECTIVE: To describe the presentations, the diagnosis, our treatment approaches, and the outcomes for 11 patients with fallopian canal meningocele (FCM). STUDY DESIGN MULTICENTER: Retrospective case series. SETTING: Tertiary referral centers. PATIENTS: Patients (N = 11) with radiographically or intraoperatively identified, symptomatic FCM. INTERVENTIONS: Surgical repair of cerebrospinal fluid (CSF) leak and meningocele versus observation. MAIN OUTCOME MEASURES: Presentation (including symptoms, radiographic imaging, and comorbidities), management (including surgical approach, technique for packing, use of lumbar drain), clinical outcomes (control of CSF leak, meningitis, facial nerve function), and revision surgery. RESULTS: Patients presented with spontaneous CSF leak (n = 7), conductive (N = 11) and sensorineural hearing loss (n = 3), nonpositional intermittent vertigo (n = 3), headaches (n = 4), and recurrent meningitis (n = 1). Risk factors in our series included obesity (n = 4), Chiari 1 malformation (n = 1), and head trauma (n = 2). Noncontrast computed tomography of the temporal bone and magnetic resonance imaging were positive for FCM in 10 patients. Eight patients were managed surgically via a transmastoid approach (n = 4), combined transmastoid and middle fossa (N = 3), or middle fossa alone (n = 1); three were managed conservatively with observation. Postoperative complications included worsened facial nerve palsy (n = 1), recurrent meningitis (n = 1), and persistent CSF leak that necessitated revision (n = 1). CONCLUSIONS: Facial nerve meningoceles are rare with variable presentation, often including CSF otorrhea. Management can be challenging and guided by symptomatology and comorbidities. Risk factors for FCM include obesity and head trauma, and Chiari 1 malformation may present with nonspecific otologic symptoms, in some cases, meningitis and facial palsy. Layered surgical repair leads to high rates of success; however, this may be complicated by worsening facial palsy.

Endoscopic Transnasal Management of Meningoceles and Encephaloceles in Children: A Systematic Review.

OBJECTIVE: To review surgical techniques used in the endoscopic transnasal repair of pediatric basal meningoencephaloceles and compare perioperative outcomes in children <2 and ≥2 years old. DATA SOURCES: MEDLINE, EMBASE, and CENTRAL. REVIEW METHODS: Data sources were searched from inception to August 22, 2022, using search terms relevant to endoscopic transnasal meningoencephalocele repair in children. Reviews and Meta-analyses were excluded. Primary outcomes were the incidence of intraoperative and postoperative complications, including cerebrospinal fluid leak, recurrence, and reintervention. Quality assessments were performed using Newcastle-Ottawa Scale, ROBIN-I, and NIH. RESULTS: Overall, 217 patients across 61 studies were identified. The median age at surgery was 4 years (0-18 years). Fifty percent were female; 31% were <2 years. Most defects were meningoencephaloceles (56%), located transethmoidal (80%), and of congenital origin (83%). Seventy-five percent of repairs were multilayered. Children ≥2 years underwent multilayer repairs more frequently than those <2 years (P = 0.004). Children <2 years more frequently experienced postoperative cerebrospinal fluid leaks (P = 0.02), meningoencephalocele recurrence (P < 0.0001), and surgical reintervention (P = 0.005). Following multilayer repair, children <2 years were more likely to experience recurrence (P = 0.0001) and reintervention (P = 0.006). CONCLUSION: Younger children with basal meningoencephaloceles appear to be at greater risk of postoperative complications following endoscopic endonasal repair, although the quality of available evidence is weakened by incomplete reporting. In the absence of preoperative cerebrospinal fluid leak or meningitis, it may be preferable to delay surgery as access is more conducive to successful repair in older children.

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.

Posterior median surgical approach to anterior sacral meningocele complicated by rectothecal fistula.

In this case report, we aimed to describe the clinical presentation, surgical approach, and follow-up of a patient with rare anterior meningocele associated with rectothecal fistula. An 17-year-old female patient was admitted to the emergency department with meningitis. On further examinations, an anterior sacral meningocele accompanied by rectothecal fistula was detected. Appropriate antibiotic treatment was arranged and surgical plan was made with the pediatric surgery clinic. The patient underwent meningocele repair via posterior approach and colostomy operation. The patient did not experience any neurological issues after the surgery. The colostomy was reversed 3 months later, and third-month follow-up MRI showed complete regression of the meningocele sac with no neurological complications. Anterior meningocele accompanied by a rectothecal fistula is a rare and complicated case. Only seven cases of coexisting ASM and RTF have been reported in literature. Although both anterior and posterior approaches have been used for the treatment of ASM, the choice of treatment is essentially based on the patient's clinical and imaging findings.

Posterior Surgical Ligation and Cyst Decompression -via Needle Puncture- of a Large Anterior Sacral Pelvic Meningocele Through Posterior Sacral Laminectomy.

The first documented description of an anterior sacral meningocele was Bryant's in 1823. Anterior sacral meningocele patients have constipation as a universal symptom; urinary incontinence is also common. All the symptoms are directly related to the pressure from a pelvic mass on adjacent structures. When the patient stands, a headache often develops because the spinal fluid pressure decreases as the meningocele sac fills. Finally, a scimitar-shaped sacrum on a neuroradiological anteroposterior plain assessment is pathognomonic. The coccyx may be absent, and the lower sacral laminae may be absent or incomplete. The surgical options for this rare clinical condition are still matter of debate.Anterior sacral meningocele is a pathology that lacks a current classification and neurosurgical therapeutic standards, even though a similar dynamic has been shown by the related traumatic pseudomeningocele. Anterior approaches (retro- and transperitoneal meningocele neck occlusion with internal cerebrospinal fluid (CSF) cyst drainage) and posterior approaches (posterior sacral laminectomy, dural sac ligation, and CSF cyst drainage) are the available surgical strategies.We now report the case of an adult patient for whom a posterior approach was suggested and performed and report her postoperative surgical follow-up. The surgical rationale is also discussed.

Quiste aracnoideo sacro recidivante con meningocele anterior gigante: reporte de caso / Sacral arachnoid recurrent cyst with giant anterior meningocele: a case report

Los quistes aracnoideos corresponden a lesiones benignas expansivas del canal medular secundarias a defectos anatómicos durales, mientras que los meningoceles anteriores consisten en la herniación de la duramadre hacia la pelvis a través de forámenes dilatados o defectos óseos. Ambas entidades son infrecuentes y sus manifestaciones clínicas puede variar de acuerdo a estructuras anatómicas que comprimen. Una historia clínica completa, la pesquisa diagnóstica y la adecuada interpretación de imágenes orientan al diagnóstico y manejo de estos pacientes. Se presenta el caso de una paciente adulta mayor con historia de masa pélvica, dolor lumbar severo y monoparesia en quien se realizó el diagnóstico incidental de quiste aracnoideo sacro y meningioma sacro anterior.

Arachnoid cysts are benign expansive lesions of the spinal canal secondary to dural defects, whereas the anterior meningoceles consist of the herniation of the dura into the pelvis through dilated foramina or bone defects. Both pathologies are infrequent and its clinical manifestations vary according to compressed anatomical structures. A complete clinical history, the diagnostic investigation and the correct imaging studies interpretation guide the diagnosis and management of these patients. We present the case of an elderly adult patient with a history of pelvic mass, severe lumbar pain and monoparesis in whom the incidental diagnosis of sacral arachnoid cyst and anterior sacral meningioma was performed.

Manejo endoscópico del meningoencefalocele de base de cráneo anterior / Endoscopic management of anterior skull base meningoencephalocele

RESUMEN La fístula de líquido cefalorraquídeo (LCR) corresponde a una comunicación anormal entre el espacio subaracnoideo y la porción neumatizada de la base de cráneo anterior en relación con las cavidades paranasales. Fístulas persistentes requieren reparación quirúrgica por el riesgo de meningitis, abscesos cerebrales y neumoencéfalo asociado. El gold standard es el abordaje extracraneal endoscópico. Uno de los principales inconvenientes es dañar estructuras intracraneanas nobles. La ayuda de tecnologías como la cirugía guiada por imágenes, contribuye a disminuir este problema. A continuación se presentan dos casos clínicos de fístula de líquido cefalorraquídeo en base de cráneo anterior, asociado a meningoencefalocele, intervenidos por cirugía endonasal guiada por imágenes.

ABSTRACT Endoscopic management of anterior skull base meningoencephalocele. The cerebrospinal fluid leak (CSF) is an abnormal communication between the subaracnoid space and the pneumatic portion of the anterior cranial base which is related to the paranasal cavities. The persistent leak requires surgery due to the potential complications such as meningitis, cerebral abscess or pneumoencephalus. Extracranial endoscopic approach is the gold standard procedure. One of the most important risk of the surgery is to damage noble intracranial structures. This situation can be ameliorated by using image guided surgery. We present two cases of CSF in anterior cranial base associated with meningoencephalocele that were treated in our center using nasal image guided endoscopic surgery.

Síndrome de Currarino como enfermedad infrecuente dentro del diagnóstico diferencial de la patología anexial oncológica / Currarino Syndrome as a Rare Disease in the Differential Diagnosis of Oncological Adnexal Pathology

El Síndrome de Currarino se define como la presencia de una tríada característica que asocia: estenosis anal, malformación sacrococcígea y masa presacra. La escasa sintomatología, caracterizada además por su inespecificidad, provoca que en muchas ocasiones el diagnóstico se realice durante la edad adulta y pueda confundirse con patologías ginecológicas, de origen predominantemente oncológico. El diagnóstico mediante pruebas de imagen, se realiza fundamentalmente a través de la resonancia magnética nuclear. La extirpación quirúrgica de la masa presacra unida al tratamiento sintomático del resto de la triada son la base terapéutica de esta infrecuente patología. Presentamos el caso de una paciente de 20 años de edad con estreñimiento crónico y dolor abdominal inespecífico. Es enviada al Servicio de Ginecología por la presencia de una masa de gran tamaño que se supone de origen anexial. Tras el estudio exhaustivo de dicha paciente, se llegó a la conclusión diagnóstica de que dicha tumoración corresponde a un meningocele, asociado a agenesia parcial sacra y estenosis anal; tríada que define al Síndrome de Currarino.

Currarino Syndrome is defined as the presence of a characteristic triad that associates anal stricture, sacrococcygeal malformation and presacral mass defines this syndrome. The scarce symptomology, also characterized by nonspecific symptoms, conditions that in many cases the diagnosis is made in adulthood and can be confused with gynecological pathologies. The diagnosis is made by imaging tests, fundamentally the NMR (nuclear magnetic resonance). The main treatment is the surgical excision of the mass presacra together with the symptomatic treatment of the rest of symptoms. We present the case of a 20-year-old patient with chronic constipation and abdominal pain that is referred to Gynecology due to the presence of a mass that is supposed to be adnexal. After the study of this patient is diagnosed a meningocele, associated with partial sacral agenesis and anal stricture, triad that defines Currarino Syndrome.

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.

Retalho de rotação com cicatriz resultante em tridente para fechamento de meningomielocele / Closure of myelomeningocele defects using a rotation flap that resulted in a trident scar

Myelomeningocele is a defect of the spinal cord, spinal column, and skin covering these areas that results in the defective closure of the posterior portion of the neural tube during the fourth week of gestation. Its etiology is multifactorial and has yet to be clarified. The objective of this study was to demonstrate an addítional skin fiap alternative for the treatment of this defect. Closure with a flap that creates a trident-shaped scar has been shown to bea viable alternative that is easy to implement, reduces surgical time, minimizes blood loss, and requires no cutaneous grafts to cover the flap donor area.

A meningomielocele é um defeito da medula espinhal, da coluna vertebral e da pele sobre essa área, resultando em defeito no fechamento da porção posterior do tubo neural durantea quarta semana de gestação. Sua etiologia é multifatorial e ainda não está esclarecida. O objetivo deste trabalho é demonstrar mais uma opção de retalho de pele para tratamento desse defeito. O fechamento com retalho resultando em cicatriz em formato de tridente apresentou-se como alternativa viável, de fácil execução, com tempo cirúrgico reduzido e mínima perda sanguínea, não requerendo enxertos cutâneos para cobertura da área doadorados retalhos.

Diagnóstico clínico y tratamiento quirúrgico de un meningocele occipital / Clinical diagnosis and surgical treatment of an occipital meningocele

El meningocele occipital es una malformación congénita producida por un defecto en el cierre del tubo neural. El curso natural de esta enfermedad es hacia la muerte por infección del sistema nervioso central o hacia un grado de incapacidad motora y sensitiva avanzada. Se han descrito diversas formas clínicas de esta malformación. Su diagnóstico se basa en el examen físico y estudios imagenológicos y neurofisiológicos, indispensables para conocer el contenido del saco herniario y determinar el tipo de cirugía a realizar. Se presentó el caso de un paciente que tuvo que ser intervenido quirúrgicamente, solamente por el examen físico y el estudio radiográfico simple de cráneo, debido a la imposibilidad económica de los padres de realizar el resto de los exámenes complementarios. Se realizó apertura del saco herniario, con drenaje del líquido cefalorraquídeo y reparación de los planos musculares y cutáneos. El paciente evolucionó favorablemente.

The occipital meningocele is a congenital malformation produced by a defect in the neural tube closure. This disease's natural course is toward death caused by the infection of the central nervous system or toward some level of advanced motor or sensitive disability. There have been described several forms of this malformation. Its diagnosis is based in the physical examination and in neurophysiologic and imaging studies, indispensable to know the content of the hernia sac and to determine the kind of surgery to carry out. We presented the case of a male patient that had to be surgically treated only for the results of the physical examination and simple radiographic study, due to the economical family impossibility of paying the rest of the complementary examination. We opened the hernia sac, drained the cerebrospinal fluid and repaired the skin and muscular planes. The patient had a favorable evolution.

Obliteración del seno frontal para el tratamiento de fístulas de LCR y meningoencefaloceles / Frontal sinus obliteration for treatment of CSF leaks and meningoencephaloceles

El tratamiento endoscópico de las fístulas de LCR se ha convertido en el gold standard. No obstante, aquellas localizadas en seno frontal presentan serias dificultades para su abordaje endoscópico, especialmente si se asocia la existencia de un meningoencefalocele. En estos casos se debe valorar el abordaje externo con obliteración del seno frontal. Presentamos dos casos de fístulas de LCR cuyo origen fue debido a sendos meningoencefaloceles protruyendo a través de la pared posterior del seno frontal y que precisaron de un abordaje externo para su tratamiento.

Endoscopic treatment of CSF leak is the Gold Standard. Nevertheless, at frontal sinus it is very difficult the endoscopic approach, especially if there is a meningoencephalocele associated. In these cases, an external approach with frontal sinus obliteration should be considered. We present two cases of CSF leaks whose origin are meningoencephaloceles that protrude from posterior frontal sinus wall, treated with an external approach.

Retalho fasciocutâneo bipediculado para tratamento de meningomieloceles / Bipedicled fasciocutaneous flap in the meningomyeloceles treatment

Introdução: Os defeitos congênitos do tubo neural e tecidos associados são classificados como disrafismos espinhais, sendo a meningomielocele a forma mais grave. Objetivos: Avaliar a eficácia do retalho fasciocutâneo bipediculado bilateral no tratamento das meningomieloceles. Método: A técnica foi utilizada em 9 pacientes com diagnóstico de meningomielocele, no período de dezembro de 2006 a janeiro de 2009. Os pacientes foram submetidos à correção cirúrgica nas primeiras 36 horas de vida, com atuação conjunta das equipes de Neurocirurgia e de Cirurgia Plástica. Resultados: Observou-se que a principal localização do defeito foi a região lombossacra (77,78%), seguida da toracolombar (11,11%) e torácica (11,11%). A utilização do retalho fasciocutâneo bipediculado bilateral possibilitou o fechamento da lesão em todos os casos. O defeito apresentava, em média, 32,1 cm2. A única complicação observada nesta série foi a epiteliólise segmentar do retalho na linha média, observada em 1 (11,1%) paciente. Discussão: A escolha do retalho fasciocutâneo bipediculado bilateral utilizada nos casos apresentados deve-se à segurança em relação à vascularização, menor tempo cirúrgico quando comparado aos retalhos musculares, facilidadeda dissecção, aplicabilidade e baixos índices de complicação. Conclusão: O retalho fasciocutâneo bipediculado e bilateral é adequado para o tratamento das meningomieloceles.

Introduction: Neural tube defects and associated tissues are classified as spinal dysraphism, being the most severe one the meningomyelocele. Objectives: To evaluate the efficacy of the bilateral bipedicled fasciocutaneous flap in the meningomyeloceles treatment. Methods: Nine patients were operated on meningomyelocele from December 2006 to January 2009. The patients had a surgery correction performed in the first 36 hours of life, with the presence of Neurosurgery and Plastic Surgery teams. Results: The main location of the defect was in the lumbosacral region (77.78%), followed by the thoracolumbar (11.11%) and the thoracicone (11.11%). In all the cases, the bilateral bipedicled fasciocutaneous flap was performed to close the defect. Discussion: The bilateral bipedicled fasciocutaneous flap was chosen owing to the security regarding vascularization, the shorter surgical time compared to muscles flaps, dissection facility, applicability and low rate of complications. Conclusion: The bilateral bipedicled fasciocutaneous flap is an adequate treatment for meningomyelocele.

Meningocele manque dorso-lumbar: a propósito de un caso / Lumbar back meningocele manque: a purpose of a case

EL meningocele manque es una patología infrecuente que se caracteriza por una protrusión de meninges y liquido cefalorraquídeo, con una banda anclante de raíces nerviosas libres funcionantes o de la medula misma sin placoda neural. Se presenta este caso de un recién nacido masculino con diagnóstico de meningocele manqué, que fue operado en el Hospital Central “Dr. José María Vargas” San Cristóbal (HCJMV), evolucionando satisfactoriamente. Esta entidad es infrecuente, sin predilección de sexo y se reporta en menos del 4 por ciento con una tasa de 1 x 100000 nacidos vivos; en pacientes con localización dorsolumbar, el conocer esta entidad permitiría al neurocirujano, identificar claramente estos casos durante su práctica profesional.