Thoracic Meningocele Research Articles

Isolated Large Lateral Thoracic Meningocele

Isolated Large Lateral Thoracic Meningocele

A Massive Thoracic Meningocele in a Patient with NF1.

A Massive Thoracic Meningocele in a Patient with NF1.

Intracranial Hypotension Due to a Large Thoracic Meningocele.

Intracranial Hypotension Due to a Large Thoracic Meningocele.

Multiple-site neural tube defects complicated by multiple-site split cord malformations and thickened filum terminale: experience at a pediatric neurosurgical teaching hospital in Ethiopia. Illustrative case.

BACKGROUNDMultiple-site open neural tube defects (MNTDs) and multiple-site split cord malformations (MSCMs) are extremely rare congenital anomalies that are defined by the simultaneous noncontiguous occurrence of more than one neural tube defect (NTD) and split cord malformation (SCM), respectively, in a single case with normal neural tissue in between. This work shows the cooccurrence of MNTDs and MSCMs, which has never been reported in the literature.OBSERVATIONSA single-stage repair for a 13-day-old female neonate with a preoperative diagnosis of MNTDs (thoracic meningocele and thoracolumbar myelomeningocele) plus an additional intraoperative diagnosis of MSCMs (type 3c) of thoracic and thoracolumbar spine, and thickened filum terminale was done with a favorable smooth postoperative course.LESSONSThe use of intraoperative meticulous surgical technique along with preoperative skin stigmata helped for anticipation, detection, and treatment of associated complex spinal MNTDs, especially in resource-limited settings, where preoperative magnetic resonance imaging is not routinely used. Whether to repair the MNTDs as a single- versus multiple-stage procedure is mainly a function of the patient’s tolerance to the duration of anesthesia and the anticipated blood loss for the patient’s age. The overall developmental biology and long-term clinical outcome of MNTDs compared to single NTD/SCM is poorly understood and needs further study.

Giant Lateral Thoracic Meningocele Incidentally Detected During Breast Cancer Screening in a Patient with Type 1 Neurofibromatosis

Lateral torasik meningosel, genişlemiş intervertebral foramenden meninkslerin sakküler protrüzyonudur. Genellikle Tip 1 Nörofibromatozis (NF-1) ile birlikte olan çok nadir karşılaşılan bir patolojidir. Bilinen invaziv duktal meme karsinomu ve NF-1 tanısı olan hastanın metastaz taraması için çekilen 18-Florodeoksiglukoz Pozitron Emisyon Tomografisinde (18-FDG PET BT) spinal kanala uzanan FDG tutulumu saptanmayan hipodens lezyonun ayırıcı tanısı yapılmak üzere radyolojik incelemeleri yapılmıştır. Olgunun Bilgisayarlı Tomografi ve Manyetik Rezonans Görüntüleme bulguları literatür eşliğinde sunulmuştur. Paraspinal kitlenin metastaz veya diğer benign ve malign kitlelerden ayırt edilmesi gereksiz izlem ve girişimsel işlemlerin önlenmesi bakımından önemlidir.

Chiari tip 1 malformasyonu, siringomiyeli, anterior torakal meningosel ve nörofibromatozis tip 1 birlikteliği: Çok nadir bir olgu sunumu

Background: Neurofibromatosis type 1 (NF-1) is an autosomal dominant hereditary disease associated with neurological and skeletal dysplasias. It is known that anterior thoracic meningocele that is a rare pathology may be associated with NF-1. Chiari type 1 malformation (CM-1) is a developmental disorder possibly caused by mesodermal deficiency and it frequently causes syringomyelia. CM-1 may be also associated with NF-1. However any case with all of these four pathologies together is not reported in literature. Case report: A 45 year old lady with known NF-1 without any neurological signs or symptoms was presented. In her thoracic imaging performed for multinodular goiter investigation, an anterior thoracic meningocele was seen. Her spinal and brain magnetic resonance imaging (MRI) investigations revealed presence of CM-1 and syringomyelia also. Despite there was not any neurological compromise of the patient, surgical treatment for CM-1 and syringomyelia was offered because of wideness of the syringomyelia. However, the patient did not accept operation. Conclusion: Although, NF-1 may be associated with CM-1/syringomyelia or anterior thoracic meningocele, there was not any reported case with all of these pathologies together in literature. This case reminded us that the NF-1 cases even the ones without any neurological complaints must be evaluated for presence of spinal and brain pathologies.

La neurofibromatose rend souvent essoufflé

Type 1 neurofibromatosis, also called "Recklinghausen's disease" is among the most frequent autosomal dominant genetic disorders, with an incidence of 1:3500 births. It mainly affects the skin and peripheral nervous system. However, in its less frequent manifestations, are tumors such as meningocele and skeletal dysplasias leading to severe clinical presentation. We report the case of a 55-year-old patient with type 1 neurofibromatosis and dyspnea due to a large left thoracic meningocele combined with a significant kyphoscoliosis, causing a severe restrictive ventilatory defect, complicated by chronic respiratory failure and pulmonary hypertension. Symptomatic treatment with non-invasive ventilation permitted an improvement of the clinical situation. Our observation shows the complexity of the therapeutic support of the neurofibromatosis of type 1. The contribution of non-invasive ventilation was illustrated by the arterial blood gas and clinical improvements as well as improved quality of life, with an acceptable level of inconvenience to the patient.

Mediastinal Schwannoma Masquerading as a Cystic Lesion: A Case Report and Review of the Literature

Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) is a safe and minimally invasive diagnostic technique for the analysis of mediastinal lesions. This approach, however, may result in life-threatening mediastinitis. Here we report a rare case of mediastinal schwannoma presenting as a cystic lesion and pose an important question of risk versus benefit of aspiration of cystic lesions. A 39-year-old man developed persistent non-productive cough and dysphasia to solids for several months. A computed tomography (CT) of the chest revealed a large posterior upper mediastinal mass measuring 6.3 x 4.5 x 8.0 cm displacing the esophagus to the right and the trachea anteriorly, with a standardized uptake value (SUV) of 2.8. The patient then had a bronchoscopy, which showed marked extrinsic compression of the mid trachea without endobronchial disease. An esophagoduodenoscopy (EGD) and EUS were performed and were notable for an 8 cm oval, well-demarcated anechoic lesion with both solid and cystic components adjacent to the esophagus. FNA was intentionally not performed due to the risk of mediastinitis. The patient had a subsequent magnetic resonance imaging (MRI) of the chest showing the 9.3 x 4.1 x 6.0 cm midline retrotracheal superior mediastinal mass. He ultimately underwent posterolateral thoracotomy with excision of the lesion. A frozen section was consistent with a neurogenic tumor. Final pathology revealed a schwannoma. Diagnosis of mediastinal masses poses a significant challenge. The differential diagnosis for a posterior mediastinal mass alone includes neurogenic tumor, neuroblastic tumor, non-neurogenic tumor, paraspinal abscess, hernia, lymphadenopathy or lymphoma, foreget duplication cysts, and thoracic meningocele. EUS-FNA is a minimally invasive and a safe procedure with a complication rate (i.e. infection, bleeding, acute pancreatitis) of approximately 1%. This rate, however, increases when the lesion contains a cystic component rather than a purely solid lesion. Several case reports have described cystic infection and mediastinitis despite accepted techniques including prophylactic antibiotics. Schwannomas generally appear as solid lesions when imaged by either CT or MRI. In 23-30% of cases, this subtype of neurofibromas may be cystic and performing a FNA may result in serious consequences. More data are needed to accurately provide the risks and benefits of EUS-FNA of mediastinal cystic lesions and to validate the use of prophylactic antibiotics for aspiration of cystic lesions.

Congenital Jejunal Tubular Duplication in a Patient with a Congenital Thoracic Meningocele

Congenital Jejunal Tubular Duplication in a Patient with a Congenital Thoracic Meningocele

Correction of Chiari malformation due to closure of a concomitant thoracic meningocele

Chiari malformations are characterized by hindbrain herniation. Historically, some types have been linked to neural tube defects, but the causal relationship between the two conditions is still unclear. We report on a full-term male neonate with a prenatally diagnosed posterior thoracic meningocele, whose MRI demonstrated Chiari malformation. The meningocele was closed in the second week of life. The Chiari-related symptoms rapidly improved following surgery. Postoperative MRI at 7 months of age showed ascent of the cerebellar tonsils to a normal position. This case suggests that the traction on the cerebellum by a fixed spinal cord may play a role in the pathogenesis of the multifaceted Chiari malformations.

A case of double meningomyelocoele with hydrocephalous in a four months′ infant

Meningomyelocoele is a developmental congenital disorder caused by failure of the neural tube to close during the first month of embryonic development thus presenting with herniation of neural elements along with meninges. Myelomeningocoeles most commonly occur in the lumbosacral region, but can also occur at any level in the neuraxis, including occipital/suboccipital areas or nasally (encephalocoele).We report a rare case of double meningomyelocoele in a 4-month-old infant (cervical as well as thoracic meningocele) with weakness in the left lower limb (power grade 0) posted for resection with difficult intubation because of obstructive hydrocephalus, fear of rupture of neuroplaque, positioning and care of blood loss and temperature. The infant was discharged within 15 days, and his hospital stay was uneventful.

Giant Thoracic Meningocele Causing Acute Respiratory Compromise

Objective: The authors report a rare case of giant thoracic meningocele causing acute respiratory compromise, treated with a ventriculoperitoneal shunt. Case Report: We report the case of a 36-year-old with severe scoliosis status post repair over a decade ago, neurofibromatosis type I, and a known large meningocele in the left thoracic cavity, presenting with new acute respiratory compromise. She was taken to the operating room for a lumboperitoneal shunt, but the operation was aborted due to her severe spinal deformity. Two days later, she successfully underwent a procedure for ventriculoperitoneal shunt placement. Upon discharge a week later, the patient was hemodynamically stable, able to move all extremities with good strength, and demonstrated improved oxygenation. In the following 7 months, the patient demonstrated continued minimal requirement on nasal cannula, and MRI showed a stable left thoracic giant meningocele. Conclusion: Ventriculoperitoneal shunting is a method of treating and stabilizing acutely symptomatic giant meningoceles.

P19.17: Rare case of multiple schisis associated defects in both twins in dichorionic pregnancy

and a large neural tube defect suggestive of a large thoracic myelomeningocele. A subsequent MRI suggested a meningocele containing no neural elements. At US, we confirmed a large thoracic meningocele with spinal cord tethering to the overlying skin. Fetal MRI confirmed the findings and a limited dorsal myeloschisis was suspected. Elective caesarean section was performed at 38 weeks. Postnatal examination confirmed a large dorsal midline fluid filled mass at the level of T7-T12 covered by a thin layer of normal skin. The structure transilluminated easily without evidence of neural tissue within. No neurological deficits were found on neonatal examination. Post partum MRI confirmed the diagnosis of limited dorsal myeloschisis. The infant underwent surgery on day 2 to remove the exophytic lesion and to release the tethered neural tissue. His post operative course was uncomplicated and he was discharged home well 10 days later. Despite the markedly abnormal prenatal appearance of LDM, with a large meningocele and a tethered fibroneural stalk, it is associated with a good prognosis. Prenatal differentiation between meningomyelocele and LDM is therefore essential for appropriate couselling. The role of fetal MRI is invaluable to aid in the diagnosis. Children born with such a lesion usually have no neurological deficit and a normal neurological outcome can be expected. However, neurological deterioration can occur in the absence of timely corrective surgery to release the tethered spinal cord and remove the exophytic skin lesion.

Three- and four-dimensional HDlive rendering images of normal and abnormal fetuses: pictorial essay

Our objective is to present our experience of normal embryonic development and fetal anatomy and fetal anomalies reconstructed employing the three-dimensional (3D) and four-dimensional (4D) HDlive rendering mode. A total of 18 normal embryos and fetuses and 21 abnormal fetuses (one case each of thoracic meningocele, thickened nuchal translucency, multicystic dysplastic kidney, gastroschisis, omphalocele, and ovarian cyst, five of hydrops fetalis, three of skeletal abnormality, three of chromosome abnormality, two of cystic hygroma, and two of amniotic band syndrome) at 7-36weeks' gestation were studied using the 3D/4D HDlive rendering mode. In normal fetuses, marked embryonic development with advancing gestation was clearly shown in the first trimester of pregnancy, and various realistic facial expressions were noted in the second and third trimesters. In abnormal fetuses, anatomically realistic features such as gross specimens were obtained. In particular, 3D/4D HDlive provides new, realistic sensations for the diagnosis of amniotic band syndrome, skeletal abnormalities, and facial abnormalities. 3D/4D HDlive rendering images seem to be more readily discernible than those obtained by conventional 3D/4D sonography. 3D/4D HDlive may be an important modality in future embryonic research, fetal neurobehavioral assessment, and the evaluation of fetal anomalies.

Surgical Treatment of Thoracic Menigocele Associated with Neurofibromatosis and Kyphoscoliosis

A 46-year-old man presented with a lateral thoracic meningocele associated with cutaneous neurofibromatosis type I and kyphoscoliosis of the thoracic spine upon medical examination. In the majority of such cases, these meningoceles remain asymptomatic, but surgery is indicated when giant or symptomatic cysts are present. The large thoracic meningocele was successfully extirpated through the transthoracic approach in combination with lumbar puncture and cerebrospinal drainage for decompression of the cyst.

Giant thoracic meningocele associated with neurofibromatosis 1

A 48-year-old man with neurofibromatosis 1 presented with progressive shortness of breath over the course of 4 years. There was prominent kyphoscoliosis. Imaging revealed a large thoracic meningocele compressing …

Cornelia de Lange syndrome associated with thoracic meningocele

Cornelia de Lange syndrome associated with thoracic meningocele

Spontaneous subarachnoid pleural fistula: A rare complication of lateral thoracic meningocele

A case of spontaneous subarachnoid pleural fistula following rupture of a thoracic meningocele into the pleural cavity is described in this article. The patient had symptoms of low-pressure headache and difficulty in breathing. The fistulous opening was closed near the foramina by rotating a vascularized muscle flap. After showing initial improvement the patient had a recurrence of symptoms after 6 weeks, with a small leak at the closure site. A lumbar thecoperitoneal shunt led to permanent cure. In this article we discuss the course of the disease, the symptoms, the diagnostic methods, and the various treatment modalities for subarachnoid pleural fistula.

Cystoperitoneal shunt as alternative treatment for a giant thoracic meningocele in a patient with neurofibromatosis

Background Thoracic meningoceles are associated with neurofibromatosis 1 in 60% to 85% of all cases. Usually, these meningoceles remain asymptomatic, but back pain, headache, cough, and dyspnea are possible manifestations. Often, there is an associated kyphoscoliotic deformity of the thoracic spine. Case Description A 60-year-old woman known in our department after a fossa posterior decompression for an Arnold-Chiari malformation was admitted through the emergency department because of progressive dyspnea. A giant intrathoracic meningocele was already diagnosed earlier but was left untreated because the patient was asymptomatic at that time. She now had dyspnea, and on chest x-ray and CT scan, there was an obvious shift of the mediastinum to the right. Because of the long-existing hemithoracic meningocele, we assumed that this patient actually had only 1 functional lung, and so, left-sided thoracotomy with resection of the meningocele and closure of the defect included a high operative mortality. Instead, we chose to obtain a permanent drainage of the meningocele by putting a shunt between the meningocele and the peritoneum. Postoperatively, the patient recovered well and became oxygen-independent. Conclusion Treatment of giant intrathoracic meningoceles in patients with progressive dyspnea can be challenging, and different options can be found in the literature. Treatment with a cystoperýtoneal shunt, as in our case, can be a less invasive alternative in patients with a high operative mortality risk. To our knowledge, this is the first report of a patient with neurofibromatosis 1 treated in this way.

Epidermoid cysts associated with thoracic meningocele

Intraspinal tumours of cutaneous origin associated with various spinal dysraphisms have been well documented in the literature. However, the metachronous development of intra- and extra-medullary tumours in conjunction with dorsal meningocele is rare. The authors report a patient with a thoracic dorsal meningocele and congenital intradural extramedullary epidermoid tumour. The patient developed an intramedullary epidermoid growth 12 years later. Subtotal resection of the tumour predisposed to a later recurrence. Meningocele is not always an isolated clinical entity but the concurrent occult lesions are usually veiled by the more conspicuous surface anomaly. Thorough magnetic resonance imaging of the whole neural axis helps to identify associated pathologies. Delicate intradural exploration by a microsurgical approach is necessary to achieve appropriate treatment.