Intrathoracic Meningocele Research Articles

Neurofibromatosis type 1 with huge intrathoracic meningoceles misdiagnosed as pleural effusion: A case report and literature review

BackgroundNeurofibromatosis type 1 is a genetic disease that affects multiple organs and systems, leading to various clinical manifestations. In Neurofibromatosis type 1, rare intrathoracic meningoceles often occur alongside bone dysplasia. These meningoceles contain cerebrospinal fluid and can be mistakenly diagnosed as ‘pleural effusion’.Case presentationIn this case report, we mistakenly identified ‘cerebrospinal fluid’ as ‘pleural effusion’ and proceeded with drainage. This error posed significant risks to the patient and holds valuable implications for the future diagnosis and treatment of similar patients.ConclusionsIn patients with Neurofibromatosis type 1 complicated by spinal deformity, there is a high incidence of intrathoracic meningoceles. Treatment strategies may differ based on the specific features of the lesions, and collaboration among multiple disciplines can significantly improve patient outcomes.

A Rare Case of an Isolated Intra-Thoracic Meningocele: Case Report and Review of the Literature

A spinal meningocele is a saccular protrusion of the meninges through a dilated intervertebral foramen or a bony defect of the vertebral column. Intrathoracic meningocele is a rare condition. Only a few cases were related in the literature. It is usually associated with neurofibromatosis type 1. Isolated intrathoracic meningoceles without neurofibromatosis is a very rare entity few cases have been reported in the medical literature. Regardless of the treatment plan cross-sectional imaging techniques such as CT and MRI are essential not only for the diagnosis but also to determine the relationship to the surrounding structures and the exclusion of other neuromas and any skeletal deformities. Surgical excision is the treatment of choice in symptomatic patients.

Anesthesia management for thoracoscopic resection of a huge intrathoracic meningocele: a case report

BackgroundDiagnosed intrathoracic meningocele is an uncommon complication of neurofibromatosis type 1. We report an anesthesia management for a rare case undergoing thoracoscopic resection of a huge intrathoracic meningocele.Case presentationA 51-year-old woman was scheduled for thoracoscopic meningectomy under general anesthesia. We monitored intrathecal pressure during anesthesia to prevent a decrease in intrathecal pressure. During surgery, the intrathecal pressure occasionally increased by around 5 cmH2O immediately after the insertion of the drainage tube and occasionally decreased by up to 10 cmH2O during the careful slow aspiration of the cerebrospinal fluid (CSF). The pressure rapidly recovered after the interruption of the procedures. She was discharged on postoperative day 4 without major complications.ConclusionsThe CSF pressure was fluctuated by procedures during thoracoscopic resection of a huge meningocele. A CSF pressure monitoring was useful to detect the sudden change of CSF pressure immediately, which can cause intracranial hemorrhage.

MON-322 Intrathoracic Meningocele Associated with Neurofibromatosis Type 1: A Case Report

Background: Intrathoracic meningocele is a relatively rare condition, with 60 to 85% of cases being associated with neurofibromatosis type 1. In most cases, meningoceles are small and asymptomatic, and regular follow-up with periodic imaging is recommended without a surgical approach. Most patients become symptomatic between 30 and 50 years of age. Surgical treatment is indicated only when there is an accelerated growth of the meningocele or when the patient presents intense symptoms due to the compression of adjacent structures. Clinical Case: CRSG, 39 years old, female, with non-consanguineous parents. Patient referred to Endocrinology at age 20 by dermatology with the diagnosis of neurofibromatosis made because of the presence of cutaneous neurofibromas and cafe au lait spots. Her father had neurofibromatosis and C-cell hyperplasia diagnosed after total thyroidectomy, and the genetic analysis of RET oncogene was negative. During follow-up, patient evolved with complaints of paresthesia in bilateral upper limbs and mechanical type cervicalgia. In cervical spine MRI, dural ectasia was observed from C4 to T2 levels, with enlargement of the vertebral canal and remodeling of the posterior wall of the vertebral bodies. In parallel, the patient had a diagnosis of primary hyperparathyroidism with serum calcium of 10.7mg/dL(8.6-10.2), vitamin D 29.8ng/dL(>20), PTH 115pg/ml(15-65) and serum phosphorus of 2.2mg/dL(2.8-4.1), and normal renal function. 99mTc-sestamibi scan showed an increased parathyroid gland posterior to the superior pole of the right lobe of the thyroid. After discussion of the case with Neurosurgery and Neuroradiology, it was then hypothesized as a cervical-thoracic meningocele and opted for conservative treatment followed by serial imaging, due to the high surgical risk and little clinical compromise of the patient at the time. Subsequently, she evolved with nausea, intermittent vomiting that made her need daily antiemetic medication. We considered the hypotheses of emesis secondary to hypercalcemia and/or secondary to cerebrospinal fluid hypotension caused by meningocele. Treatment with pamidronate was initiated and the patient and is still under evaluation for probable intracranial cerebrospinal fluid hypotension. Conclusion: Neurological complaints are common during the follow-up of patients with neurofibromatosis and their differential diagnosis is of great importance for adequate therapeutic management. Regarding the main tumor lesion, in addition to the mentioned meningocele in the case, it should be differentiated from neurofibroma, neuroblastoma, ganglioneuroma and cystic hygromas of the posterior mediastinum. Reference: Huang TW, Huang HP, Ye QY, et al. A neurofibromatosis type 1 patient with thoracic encapsulated fluid and intracranial hypotension syndrome: a case report. Neurologist 2011;17:167-171.

A Mature Cystic Teratoma that Arises in the Posterior Mediastinum and that is Associated with an Anterior Intrathoracic Meningocele

A Mature Cystic Teratoma that Arises in the Posterior Mediastinum and that is Associated with an Anterior Intrathoracic Meningocele

Spontaneous Extracranial Vertebral Artery Dissection in a Neurofibromatosis 1 Patient with Bilateral Intrathoracic Spinal Meningoceles around the Scoliosis: Report of an Autopsy Case

Neurofibromatosis 1 (NF1) is a common autosomal dominant disorder that causes several systemic diseases. Many studies have reported that NF1 is associated with intrathoracic meningoceles and scoliosis. The incidence of vertebral artery dissection is estimated to be 1 -1.5 per 100,000 population. We experienced an autopsy case of massive intrathoracic hemorrhage due to spontaneous vertebral artery dissection in a patient with NF1, who had intrathoracic spinal meningoceles and scoliosis. A 47-year-old man was found dead at his home in the morning. He had a history of NF1 including numerous cutaneous neurofibromas and hyperpigmented macules, scoliosis, and deformity of the leg. The autopsy revealed the dissection and rupture of the left vertebral artery, and a pseudocyst that had formed due to arterial leakage on the wall of the meningocele on the left side. The pseudocyst had eventually ruptured and leaked blood, resulting in a massive hemothorax on the left side. Thus, it was revealed that the patient had suffered from NF1-associated intrathoracic meningoceles and scoliosis, and we concluded that the cause of his death was a massive hemothorax on the left side, caused by the dissection and rupture of the left vertebral artery.

An Isolated Spinal Meningocele Discovered Fortuitously on a CT Scan: Case Report

A spinal meningocele is a saccular protrusion of the meninges through a dilated intervertebral foramen or a bony defect of the vertebral column. Intrathoracic meningocele is a rare condition. Only a few cases were related in the literature. It is usually associated with neurofibromatosis type 1. Isolated intrathoracic meningoceles without neurofibromatosis are more uncommon. We report a case of isolated paraspinal meningocele in a 54 old-year-man, for whom the diagnosis was established by computed tomography and magnetic resonance imaging features.

Large intrathoracic meningocele in a patient with neurofibromatosis type 1

Intrathoracic meningocele associated with neurofibromatosis type 1 is quite rare. We report the case of a Chinese women with a 20-day history of right-sided chest pain and sense of pressure on the chest. Computed tomography and magnetic resonance imaging indicated a homogeneous thin-walled cystic mass in the right rear mediastinum, ranging from the 1st to 5th thoracic segments. Surgery was performed to removal the meningomyelocele pouches and shunt the cyst to the subarachnoid region.

Large Intrathoracic Meningocele Associated With Neurofibromatosis Type 1

A 43-year-old man with neurofibromatosis type 1 (NF1), who presented for a routine physical examination, was found to have unilateral diminished breath sounds. He had kyphoscoliosis, for which he had undergone thoracic spinal fusion and instrumentation 32 years previously. Chest radiography revealed an extrathoracic “soft tissue mass” (9.5 × 8.5 cm). Computed tomography identified an extrapleural lesion (9 × 8 × 7 cm) suspicious for a spinal neurofibroma with cord invasion, as well as bony erosion of thoracic vertebrae and neural foramina. Concern of malignancy prompted surgical consultations. A computed tomographic myelogram revealed a meningocele at T7-T8 (measuring 7.5 cm maximally), communicating with cerebrospinal fluid and multiple smaller meningoceles at adjacent levels. Neurofibromatosis type 1 is an autosomal dominant disorder associated with a tumor suppressor gene defect on chromosome 17q. Its incidence is 1 in 3500 persons. Although NF1 has variable expression, it is usually recognized by cutaneous stigmas such as cafe-au-lait macules and intertriginous freckling and by development of neurofibromas. Plexiform neurofibromas are a distinct type of neurogenic tumor with malignant potential. About 10% of patients with NF1 develop malignant peripheral nerve sheath tumors with very poor prognosis. Bone dysplasia, scoliosis, and meningeal defects (spanning from dural ectasia to meningoceles) are common in patients with NF1. Intrathoracic meningoceles are rare but, when present, are usually seen in the constellation of NF1. The etiology of meningoceles is thought to be herniation of spinal meninges with cerebrospinal fluid extrusion through enlarged intervertebral foramina. Treatment ranges from observation to excision. Given the absence of symptoms in our patient, expectant management was recommended.

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hemithorax, severe scoliosis, and postoperative changes (Figure 1). Bedside sonographic images of the left upper thorax were consistent with the presence of intrathoracic fluid, which appeared loculated. Given the severe distortion of the thoracic anatomy, a thoracentesis was postponed, and computed tomography (CT) of the chest was ordered. CT scans of the chest and thoracolumbar spine revealed a 12.58.813.3-cm fluid collection in the left upper hemithorax exerting a mass effect over surrounding structures (Figures 2, 3); the left upper and left lower lung lobes exhibited compressive atelectasis, and the mediastinal structures were displaced to the right. Significant deformity of the spine was observed in the region of fluid collection, which appeared to be contiguous with the spinal canal, although the implanted rods hindered a detailed radiographic inspection of the spinal anatomy. At this point, the working diagnosis was giant intrathoracic meningocele. The differential diagnosis for this lesion included loculated pleural effusion, seroma secondary to the extensive thoracic and spinal surgery, and a large pleuralbased neurofibroma. To confirm our working diagnosis, magnetic resonance imaging (MRI) of the spine would have been indicated; however, given this patient’s history of metal hardware implants, an MRI scan was deemed unsafe, and an alternative confirmatory strategy had to be pursued.

Cystoperitoneal Shunt for a Giant Intrathoracic Meningocele Under Local Anesthesia

Giant intrathoracic meningoceles are extremely rare, and the standard treatment for giant intrathoracic meningoceles remains controversial. We present the case of a patient with giant intrathoracic meningoceles associated with neurofibromatosis type I. Our patient had poor respiratory function because of the giant intrathoracic meningocele, so we performed a cystoperitoneal shunt under local anesthesia. We describe our cystoperitoneal shunt technique using an adjustable-pressure valve. This simple, minimally invasive treatment is a valuable alternative treatment option in patients at high operative risk, especially those with low respiratory function.

Chest wall bleeding with giant intrathoracic meningocele in neurofibromatosis type 1

We report the case of a 66-year-old woman with neurofibromatosis type 1 who developed chest wall bleeding with severe scoliosis and a giant intrathoracic meningocele. She was brought to the emergency department with acute-onset of left-sided chest pain and clinical signs of hypovolemia. Bleeding control was difficult in the first operation because the tissue was friable and there were multiple subcutaneous bleeding points. During the first operation, the patient developed disseminated intravascular coagulation, which required immediate management; therefore, the surgery was aborted and a repeat surgery was performed later to stop the bleeding. The major cause of bleeding was presumed to be the mechanical stretching of the intercostal arteries and branches of the internal thoracic artery secondary to the severe deformity of the thoracic vertebra and ribs. The massive bleeding remained as a hematoma and did not lead to development of hemothorax. This was believed to be because the giant intrathoracic meningocele supported the expansion of the hematoma and prevented the perforation of the visceral pleura. After the second operation, the hematoma shrunk gradually; however, the patient required ventilatory support because the decrease in the size of the hematoma was accompanied by the expansion of the meningocele.

Congenital spinal intradural arachnoid cyst associated with intrathoracic meningocele in a child

Congenital spinal intradural arachnoid cyst associated with intrathoracic meningocele is very rare. We report a case in a 9-year-old Chinese boy who presented with a two-week history of progressive paraparesis and gait ataxia. Magnetic resonance imaging revealed that a dorsal intradural extramedullary cystic lesion extended from T1 to T5 and compressed the spinal cord. A left lateral intrathoracic meningocele pouch was found incidentally at the level of T1. The arachnoid cyst as well as meningocele was removed and the spinal cord compression was relieved. Arachnoid cyst was confirmed by histological examination. The patient recovered well postoperatively. This is the second report of such a case in the world according to the available literature. The take-home message for our case is that the surgical approach should be individualized, depending on the size and location.

A Large Intrathoracic Meningocele in a Patient with Neurofibromatosis-1

A large intrathoracic meningocele, a saccular protrusion of the meninges through a dilated intervertebral foramen or a bony defect of the vertebral column, was diagnosed in a 41-year-old female patient showing clinical features of neurofibromatosis-1 (NF-1), including café-au-lait spots, cutaneous neurofibromas, and axillary frecklings and Lisch nodules on the iris. Her daughter and son also had similar manifestations of NF-1. Regular follow-up with periodic imaging was recommended without surgical treatment because there were no signs or symptoms. Meningocele should be differentiated from posterior mediastinal tumors such as neurofibroma, neuroblastoma, and ganglioneuroma because NF-1 has a high risk of tumor formation. We report on this case with a brief review of the literature.

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.

Isolated giant intrathoracic meningocele associated with vertebral corpus deformity

Published reports of intrathoracic meningocele with vertebral corpus defects in the absence of neurofibromatosis are very rare. We report a 9-year-old male with intrathoracic meningocele. We believe that vertebral corpus defects may play a certain role in the etiology of intrathoracic meningocele.

A neurofibromatosis type 1 patient with severe kyphoscoliosis and intrathoracic meningocele

The patient presented with neurofibromatosis and a dystrophic kyphoscoliosis around the cervico–thoracic junction. When the patient was 59 years old, he started to suffer from dyspnea caused by an intrathoracic meningocele in the upper left thoracic cavity. A wide laminectomy from T2 to T5 was performed and the meningocele was resected. Although the dyspnoea disappeared postoperatively, the patient started to neurologically deteriorate. Laminectomy alone caused instability around the apex of the kyphosoliosis and spinal cord compression. Halo cast was applied and brought remarkable recovery of neurologic deficits. This result encouraged us to perform posterior fusion in situ from C3 to L2 with bone graft from the iliac crests and the Luque technique in conjunction with the Isola system. This resulted in the patient being able to walk again. The removal of the posterior element predisposes the patient to unstable postlaminectomy kyphosis and removes valuable bone stock required for posterior spinal fusion. For this reason, spinal fusion should have been conducted during surgery for the patient’s meningocele.

Intrathoracic giant meningocele associated with neurofibromatosis.

Intrathoracic giant meningocele associated with neurofibromatosis.

Paraparesis after excision of intrathoracic meningoceles in a patient with neurofibromatosis

Intrathoracic meningocele associated with neurofibromatosis is a rarity. We treated a 16-year-old boy with neurofibromatosis, marked kyphoscoliosis, and two rightsided intrathoracic meningoceles. Because his chief complaints of cough and chest pain were thought to be caused by the meningoceles, resection of these lesions was performed prior to correction of the spinal deformity. On the day after the resection, complete paraplegia developed, followed by recovery to paraparesis. Decompressive lumbar puncture was performed, but intraspinal pressure was normal. Postoperative spinal cord damage and consequent paresis may have resulted from a loss of pressure buffering by the meningocele, which rendered the cord vulnerable to injury. The possibility of a similar unusual complication should be borne in mind when treating patients with intrathoracic meningocele associated with neurofibromatosis.

Ｖｏｎ Ｒｅｃｋｌｉｎｇｈａｕｓｅｎ病に合併した胸腔内髄膜りゅうの２例

Ｖｏｎ Ｒｅｃｋｌｉｎｇｈａｕｓｅｎ病に合併した胸腔内髄膜りゅうの２例