Microsurgical Excision of the Craniocervical Neurenteric Cysts by the Far-Lateral Transcondylar Approach: Case Report and Review of the Literature

Neurenteric cysts in the anterior craniocervical junction (CCJ) region can be found in extremely rare cases. We report one case with craniocervical neurenteric cyst that was excised by the far-lateral transcondylar (FLT) approach. A 43-year-old man presented with a history of recurrent episodes of mild neck pain and dysesthesia in his bilateral hands of 2 years'' duration with rapid deterioration 3 weeks prior to admission. Magnetic resonance imaging (MRI) of the CCJ region revealed a well-defined intradural cystic lesion located ventral from the pontomedullary junction to C1 vertebra with medulla and C1 cord compression. This patient underwent total excision of the lesion via the FLT approach without any postoperative neurological deficits, and the histopathologic diagnosis was neurenteric cyst. Follow-up MRI has revealed no evidence of recurrence. The clinical features, imaging studies, and surgical approach options involved in resecting craniocervical neurenteric cysts are discussed, along with a review of the literature.     

True intraspinal neurenteric cyst in the lumbosacral region--case report

A 56-year-old man presented with a very rare true neurenteric cyst in the conus medullaris without evidence of vertebral or visceral anomaly manifesting as a 6-month history of mild low back and bilateral inguinal pain. No motor weakness was found in the bilateral lower extremities. He had also suffered dysesthesia in the bilateral feet for several weeks before admission. Lumbar spine magnetic resonance (MR) imaging demonstrated a cystic intradural extramedullary mass at the L1-2 levels without enhancement after gadolinium injection. MR imaging, computed tomography, and radiography detected no vertebral anomaly. Lumbar laminectomy at the L1-2 levels was performed and the lesion was incompletely removed. Histological examination showed the cystic wall lined with ciliated columnar epithelium. Neurenteric cyst should be considered in the diagnosis of isolated cystic mass lesion at the lumbosacral region even in the absence of vertebral or visceral abnormality.     

Primary spinal intradural extramedullary hydatid cyst in a child

BACKGROUND/OBJECTIVE: Spinal hydatid cyst is a serious form of hydatid disease affecting less than 1% of the total cases of hydatid disease. We present a case of pathologically confirmed primary intradural spinal cyst hydatid in an otherwise healthy patient who showed no other evidence of systemic hydatid cyst disease. CASE REPORT: An 8-year-old boy presented with back pain, left leg pain, and difficulty in walking. The patient had no other signs of systemic hydatid cyst disease. An intradural extramedullary cystic lesion was identified with magnetic resonance imaging and was shown to be a hydatid cyst by histopathologic examination after the surgical removal. CONCLUSION: Although extremely rare, primary intradural extramedullary hydatid cyst pathology might be the cause of leg pain and gait disturbance in children living in endemic areas.     

Neurenteric cyst of the craniocervical junction--case report

A 60-year-old female presented with occipital headache and limitation of neck movement. Neurological examination showed weakness of the right sternocleidomastoid muscle. Magnetic resonance imaging revealed a cystic lesion at the craniocervical junction and posterior compression of the brain stem. The lesion was totally removed through the transcondylar approach. The histological diagnosis was neurenteric cyst. The transcondylar approach provides a direct operative view of the clivus and anterior craniovertebral junction.     

A recurrent intradural cervical neurenteric cyst operated on using an anterior approach: a case report

The neurenteric cyst is an uncommon congenital lesion. In most reported cases, it has been operated on via a posterior approach using a laminectomy, despite the fact that the cyst is usually located ventral to the spinal cord. Reports have shown that early postoperative results have been good with the posterior approach, but very few studies of the long-term postoperative recurrence of neurenteric cysts have been conducted. Here, we report on a case of recurrent neurenteric cyst that was operated on using an anterior approach.A 42-year-old woman presented with a cervical neurenteric cyst that had recurred eight years after its partial removal via a posterior approach. The patient complained of pain on the lateral side of her upper arms, and an magnetic resonance imaging showed that the recurrent cyst was located ventral to the spinal cord and compressed the cord dorsally at the C4-6 level.The patient was operated on via an anterior approach using a vertebrotomy at the lower half of C5 and the upper half of C6. The cyst was attached to the spinal cord firmly and was subtotally removed, with the thickest portion adhering to the cord not being removed. The caudal end of the cyst was observed with the assistance of a rigid endoscope.A neurenteric cyst may recur after partial removal, and the patient's condition may deteriorate during postoperative follow-up. The anterior surgical approach provides good visualization and facilitates safe removal of the lesion.     

Primary Spinal Intradural Extramedullary Hydatid Cyst in a Child

Abstract

Background/Objective: Spinal hydatid cyst is a serious form of hydatid disease affecting less than 1% of the total cases of hydatid disease. We present a case of pathologically confirmed primary intradural spinal cyst hydatid in an otherwise healthy patient who showed no other evidence of systemic hydatid cyst disease.

Case Report: An 8-year-old boy presented with back pain, left leg pain, and difficulty in walking. The patient had no other signs of systemic hydatid cyst disease. An intradural extramedullary cystic lesion was identified with magnetic resonance imaging and was shown to be a hydatid cyst by histopathologic examination after the surgical removal.

Conclusion: Although extremely rare, primary intradural extramedullary hydatid cyst pathology might be the cause of leg pain and gait disturbance in children living in endemic areas.     

Intramedullary neurenteric cyst without an associated malformation. Case report

The authors report a case of an intramedullary neurenteric cyst without any associated dysraphic lesion. Unlike extramedullary intradural forms, this type of malformation remains rare. Our observation is one of the first to have been evaluated by magnetic resonance imaging. Theories concerning the embryogenesis of neurenteric cysts as well as their clinical characteristics and surgical treatment are discussed.     

Ventral intradural endodermal cyst in the cervical spine treated with anterior corpectomy--case report

A 14-year-old girl who presented with an endodermal cyst manifesting as severe neck and shoulder pain along with vesicorectal disturbances. Cervical magnetic resonance imaging showed a slightly enhanced intradural cyst at the C6-7 level in the ventral side of the spinal canal, with significant dorsal shortening and thinning of the spinal cord. Anterior corpectomy was chosen because of the dorsal effacement of the spinal cord. The cyst wall was subtotally removed to avoid damage to the normal spinal cord. After cyst removal, the iliac bone and an anterior cervical plate were used for anterior fusion. Postoperatively, her pain subsided without neurological deficits. The histological diagnosis was endodermal cyst. The cyst did not recur during a follow-up period of 18 months. Endodermal cysts are rare congenital lesions of the spine lined by endodermal epithelium. The natural history of this lesion is unclear, and the surgical strategy for the approach route and the extent of removal of the cyst wall remain controversial. We suggest that the anterior approach may allow a safer and more effective surgical route for the treatment of ventrally located endodermal cyst compared to the posterior approach.     

Excision of an asymptomatic cervical intradural neurenteric cyst through the anterior approach: a study of two cases and a review of the literature

BACKGROUND: Spinal neurenteric cysts are very rare lesions, especially after the second decade of life. They account for 0.3% to 0.5% of all spinal tumors and occur most commonly in ventral locations. The cysts are usually removed via a posterior approach. PURPOSE: To present the clinical and radiologic results of patients with spinal neurenteric cysts who were treated via anterior approach procedures. STUDY DESIGN: This report is composed of two cervical neurenteric cyst cases that are compared with published studies. METHODS: We present two patients, 41- and 39-year-old women, each with a cervical intradural neurenteric cyst. Both of these patients had apparent neck pain without neurological deficit. MRI revealed neurenteric cysts located at C7 and C7-T1 levels. After anterior corpectomy, the intradural cysts were removed, and then fusion was performed. RESULTS: The postoperative period went well. The follow-up cervical MRI studies were performed at 3, 6, and 18 months postoperatively, and there were no abnormalities found. CONCLUSIONS: This study has led to the conclusion that although neurenteric cysts are rare in adults, they can still be present with only persistent neck pain and without neurological deficits. This may lead to misdiagnosis. The importance of MRI is not controversial in the early diagnosis. An anterior approach may be considered the first preference for surgical technique in patients with ventrally located neurenteric cysts.     

Craniocervical junction synovial cyst associated with atlanto-axial dislocation--case report.

A 51-year-old female presented with a rare case of synovial cyst at the cruciate ligament of the odontoid process associated with atlanto-axial dislocation, manifesting as a history of headache and numbness in her left extremities for 5 months, and progressive motor weakness of her left leg. Neuroimaging studies revealed a small cystic lesion behind the dens, which severely compressed the upper cervical cord, and atlanto-axial dislocation. The cyst was successfully removed via the transcondylar approach. C-1 laminectomy and foramen magnum decompression were also performed. Posterior craniocervical fusion was carried out to stabilize the atlanto-axial dislocation. The cyst contained mucinous material. Histological examination detected synovial cells lining the fibrocartilaginous capsule. Synovial cysts of this region do not have typical symptoms or characteristic radiographic features. Careful preoperative evaluation of the symptoms and a less invasive strategy for removal of the cyst are recommended.     

Prenatal diagnosis of a large, cervical, intraspinal, neurenteric cyst and postnatal outcome

Neurenteric cysts are rare congenital anomalies derived from the notochord and located in front of the spinal column, mostly at the cervical level. They consist of an intraspinal cystic component that is connected to a mediastinal or thoracic cyst. A case of a cervical, intraspinal, neurenteric cyst, diagnosed at 28 weeks of gestation and operated on at 3 and half months after birth, is reported. Herein, we discuss important features of this case including its rare occurrence, intrauterine diagnosis, and early surgical intervention. In conclusion, the nature of prenatal intradural cysts should be fully evaluated and differentiated between neurenteric cyst and other types of cysts with fetal ultrasonography. Therefore, it is essential to be aware of this potential cyst in fetal ultrasonography in utero. Neurenteric cyst should be considered in the differential diagnosis of cervical intradural cystic lesions. In infants, successful surgery provides a cure in patients with cervical neurenteric cysts.     

A case of cervical endodermal cyst

A very rare case involving an endodermal cyst of the cervical spinal canal was documented. In 1999, a 28-year-old male presented with mild tetraplegia due to a traffic accident and consequently, he was admitted to another hospital. Magnetic resonance imaging (MRI) performed at that time demonstrated a cervical cord cyst. He was treated conservatively and as a result, complete resolution of symptoms was achieved. Five years later, he presented with progressive right hemiparesis and was referred to our institute. MRI at the time of admission exhibited an intradural extramedullary cystic lesion on the ventral side of the spinal cord at the C5-6 levels, which was characterized by low intensity on T1-weighted, and by high intensity on T2-weighted images. The cyst, which had increased in size, compressed the spinal cord remarkably backward. The anterior central vertebrectomy approach was performed. Subtotal resection of the cyst wall was conducted due to its tight partical adhesion to the spinal cord. The vertebral defect was reconstructed with an autogenous iliac graft. According to histological findings the cyst wall consisted of a single layer of columnar epithelial cells with secretory granules and immunohistochemical examination revealed that the cyst wall was positive for cytokeratin 7. Symptoms improved immediately. Subsequently, the patient was discharged with good performance status. Endodermal cysts are very rare developmental cysts derived from the embryonic endodermal layer. Moreover, these lesions are usually located intradurally in the cervical and upper dorsal spine ventral to the spinal cord. Total removal of the cyst is recommended if it is possible. However, total resection is often difficult due to adhesion of the cyst wall to the neural tissue so invasive resection should be avoided. In such cases, follow-up MRI is necessary in order to exclude recurrence of the remnant lesion.     

Is total excision of spinal neurenteric cysts possible?

Spinal neurenteric cysts are intradural cystic lesions. These represent a part of the spectrum of developmental anomalies. These are rare lesions. Most of the data in literature is review of case reports. This made us evaluate our results of 23 cases and review the literature. This is a retrospective study of 23 patients managed at our institute over 20 years. The slides were retrieved and histopathological features studied. Twenty patients were less than 30 years old and 21 were males. Follow-up was available for 21 patients with mean duration of 71 months (range 2 months to 23 years). The typical presentation was backache with progressive neurological deficits pertaining to the level. Atypical presentations as acute onset, recurrent episodic events and aseptic meningitis were also noted. The cervicothoracic region was the most common site. 16 patients had cyst in intradural extramedullary plane and seven had intramedullary location. Associated vertebral anomalies as hemivertebrae, Klippel-Feil and spina bifida were noted in seven patients. There were two histological types of cysts with no correlation between the type of cysts and associated vertebral anomalies and extent of outcome. Partial excision though had higher risk of recurrence, was not associated with poorer outcome. Dorsal approach is an acceptable route with reasonably good results for this lesion. Spinal neurenteric cysts present at younger age with varied clinical presentations. These are commonly located intradurally ventral to the cord. Histological types have no effect on the outcome. Total excision is the choice of treatment. However, partial excision is a feasible option in intramedullary lesions and when significant adhesions occur. Although associated with higher risk of recurrence, the outcome is still good in these patients on re-excision.     

Dumbbell C2 schwannomas involving both sensory and motor rootlets: report of two cases

OBJECTIVE AND IMPORTANCE: Intradural-extradural dumbbell C2 schwannomas are rare. This report concerns two such cases with the intradural compartment located ventral to the spinal cord and involving both sensory and motor rootlets. CLINICAL PRESENTATION: One patient was a 57-year-old woman with sensory disturbances in the right extremities and hyperreflexia in the left extremities. The other patient was a 73-year-old man who presented with tetraparesis, walking disability, atrophy of the nuchal and bilateral shoulder muscles, and pain in the right C2 dermatome. INTERVENTION: The extradural component of the tumor was removed first; next, the intradural component was removed successfully via the posterior approach combined with a C1-C2 laminectomy. The patients experienced symptomatic improvement without further deficits except for sensory impairment of the C2 dermatome in one of the patients. CONCLUSION: Intradural-extradural dumbbell C2 schwannomas can be satisfactorily managed with a posterior approach. Removal of the extradural component and opening of the dural ring of the C2 nerve root are necessary for safe extraction of the intradural ventrally located component after debulking. These tumors may arise extradurally within the nerve sheath, extend intradurally and ventrally toward the spinal cord, and involve both sensory and motor rootlets.     

A case of idiopathic herniation of the spinal cord associated with duplicated dura mater and with an arachnoid cyst

Idiopathic herniation of the spinal cord is a very rare disease, only 2 cases being so far reported in the literature. A 61-year-old man with a gradual loss of power in his lower extremities was diagnosed as intradural arachnoid cyst, and underwent operation. During operation, not only an arachnoid cyst was observed dorsally, but was also duplicated the dura mater ventrally. In the latter lesion, a ventral part of the spinal cord was herniated from a defected area of the dural inner layer. Although this dural inner layer as well as the arachnoid cyst was resected, his clinical symptoms remained unchanged after operation. When a shift of the spinal cord is detected in an image, we should precisely determine the relationship between the spinal cord and the dura mater during operation with consideration of a possibility of the presence of idiopathic herniation of the spinal cord.     

An intradural arachnoid cyst of the craniovertebral junction; a case report]

An intradural arachnoid cyst of the craniovertebral junction possibly of traumatic origin is reported. A 59-year-old man was admitted to our hospital with a 10-month history of progressive gait disturbance. He had a history of head injury with a fracture of the occipital bone. Myelography revealed pooling of the contrast medium in the posterior fossa and on the dorsal sides of C1 and C2. Metrizamide-enhanced computed tomography also showed pooling at the same level. Magnetic resonance imaging indicated a large cystic lesion at the craniovertebral junction. Craniectomy of the posterior fossa and laminectomy of C1, C2 and C3 were performed, and an intradural cyst with thickened dura and arachnoid was found. The cyst wall was opened to communicate with the subarachnoid space. Histological findings of the specimen showed that the arachnoid was thickened. There are over 130 reports of intradural arachnoid cyst of the spine, but those of traumatic origin are rare, and cysts located in the intracranial to spinal region are extremely rare.     

Thoracolumbar intradural extramedullary bronchiogenic cyst

Summary Intradural extramedullary bronchiogenic cysts are rare findings. All five reported cases were located cervically or upper thoracically. To our knowledge, we describe the first case of an intraspinal bronchiogenic cyst in a thoracolumbar location.We present the case of a 41-year-old patient with a known spina bifida occulta who suffered from a continuous, sharp, and therapy-refractory pain in the left leg. Magnetic resonance imaging of the thoracic and lumbar vertebra revealed an intradural extramedullar mass at T12 to L1 level. After laminectomy T-12 through L-1/L-2 and longitudinal opening of the dura mater, the cystic mass was shown to be attached to the conus medullaris and the cauda equina, and therefore could be removed only partially. Histopathological examination revealed the diagnosis of bronchiogenic cyst. We therefore conclude that intradural extramedullary bronchiogenic cysts may appear also at thoracolumbar levels. Surgical resection can be achieved with good outcome.     

Custom-tailored minimally invasive partial C2-corpectomy for ventrally located intramedullary cavernous malformation

Approaches to ventrally located intramedullary lesions of the upper cervical spine can be extremely challenging. We present a custom-tailored, minimally invasive anterior approach to a ventrally located, intramedullary cavernous hemangioma with partial lateral corpectomy of C2, complete resection of the lesion and subsequent reconstruction. A 20-year-old woman presented with the history of progressive numbness of the left upper and lower extremities and some episodes of severe headaches was referred to magnetic resonance imaging: Here, an intramedullary lesion with typical radiological features for a cavernous malformation at the ventral surface of the spinal cord at the C2 level was detected. The surgical procedure was performed under general anesthesia and electrophysiological monitoring (somatosensory-evoked potentials (SEP), muscle motor-evoked potentials (MEP), and D-wave recording). Complete resection of the cavernous malformation was achieved and reconstruction of the cervical spine was performed using a custom-tailored cage. Intraoperative neuromonitoring during resection, revealed a transient MEP loss, but unchanged D-wave and SEP recordings indicated unchanged neurological outcome. Early clinical follow-up of the patient revealed no new neurological deficits. At 3-month follow-up, there was some improvement of the sensory function. This custom-tailored minimally invasive anterior approach to a ventrally located intramedullary cavernous malformation with partial C2-corpectomy describes a possible and successful approach to ventrally located intramedullary lesions of the upper cervical spinal cord. Additionally, the hereby-described approach is not related to cervical instability.     

Removal of an anterior spinal dermoid cyst with fenestra corpectomy in Klippel-Feil syndrome: technical case report

OBJECTIVE AND IMPORTANCE: A spinal cord tumor occurring in association with Klippel-Feil syndrome is quite rare. The removal of an anteriorly located spinal cord tumor at the level of block vertebrae creates a surgical challenge. CLINICAL PRESENTATION: A case of an intradural extramedullary dermoid cyst located anterior to the spinal cord and a syringomyelic cavity at the level of block vertebrae in a 43-year-old woman with Klippel-Feil syndrome is presented. She experienced pain and numbness in both shoulders and in her neck, and she had a slight weakness in both arms before the operation. Her weakness and the clinical symptoms completely disappeared after the operation, and the resolution of the syringomyelic cavity was observed at control magnetic resonance imaging. INTERVENTION: An anterior approach creating a fenestra corpectomy to the block vertebrae was performed, and the tumor was removed totally. No fusion or fixation was performed. CONCLUSION: To our knowledge, this is the first report of an anteriorly located intradural extramedullary cervical spine tumor in association with Klippel-Feil syndrome treated with this surgical technique. A three-dimensional computed tomographic control scan obtained 1 year after the operation did not show any instability.     

Thoracic Myelopathy Secondary to Intradural Extramedullary Bronchogenic Cyst

Abstract

Background/Objective: To report a case of thoracic myelopathy secondary to intradural extramedullary bronchogenic cyst.

Study Design: Case report.

Methods/Findings: A 20-year-old man presented to the emergency department with increasing back pain and lower-extremity weakness. Magnetic resonance imaging demonstrated a cystic lesion at the T4 level with mass effect on the spinal cord.

Results: The lesion was resected, and histopathologic evaluation showed a cyst lined by respiratory-type epithelium consistent with a bronchogenic cyst.

Conclusions: Intradural extramedullary bronchogenic cysts of the thoracic spine have been reported previously but are extremely rare. The treatment of choice is surgical resection.