Dermoid cyst: A rare intramedullary inclusion cyst

Intramedullary dermoid cysts are rare tumors, especially those not associated with spinal dysraphism. Only six cases have been reported in the literature. Of these, only two cases have had magnetic resonance imaging studies. We report a case of an 18-year-old female patient, who presented with progressive weakness of both the lower limbs and wasting of both the upper limbs. Magnetic resonance imaging (MRI) showed an intramedullary lesion extending from C3 to D2 with peripheral enhancement on contrast. Decompression of the cystic contents with partial removal of cyst wall was done. Hair with oily cholesterol and keratin debris was encountered. Histopathology confirmed the diagnosis of dermoid cyst. This case adds to the previous reported cases of the rare and uncommon intramedullary space occupying lesions of the spinal cord.     

Intramedullary epidermoid cysts of the spinal cord. Case report.

Epidermoid cysts are tumors familiar to neurosurgeons, but intramedullary epidermoid cysts are rare. The authors report the case of a 6-year-old girl presenting with progressive paraparesis. A midthoracic intramedullary mass was revealed on myelography and magnetic resonance (MR) imaging and confirmed as an intramedullary epidermoid cyst at surgery, at which time the cyst was removed. This is the fourth report documenting a purely intramedullary epidermoid cyst occurring in a child. The pathology and etiology, epidemiology, clinical features, radiology (including MR image characteristics), and surgical treatment of such rare intramedullary benign tumors are discussed. Magnetic resonance imaging reduces the delay in diagnosis of spinal cord tumors but should be guided by clinical judgment.     

A rare case of intramedullary epidermoid cyst diagnosed by MRI]

Intramedullary epidermoid cysts are rare entities. A case recently seen on MRI led us to review the literature on the subject. A 9 year old boy developed in a four month-time a spastic paraparesis with a T7-T8 sensitive level. The MRI showed a widening of the spinal cord at the T6 level with an area of decreased signal on T1 weighted images, and a homogeneous round shaped area of increased signal on T2 weighted images. The authors discuss the contribution of MRI to the diagnosis of this lesion. The number of cases of intramedullar epidermoid cysts, like dermoid cysts, diagnosed with this neuroradiological method, is still very limited and the literature only mentions isolated cases.     

Intramedullary epidermoid cyst: preoperative diagnosis and surgical management after MRI introduction. Case report and updating of the literature

Many patients with spinal tumours of developmental origin do not receive preoperative diagnosis and the surgical management, especially as for capsule resection, is often unplanned. Like other uncommon tumours, the intramedullary epidermoid cyst is often an operative or histological finding. Since magnetic resonance imaging (MRI) introduction, evidence has accumulated that they may be preoperatively suspected. In reporting the case of a young patient with a T3-T4 intramedullary epidermoid cyst, the authors present an overview of the clinical, radiological and surgical aspects of such tumors and review the latest literature in which MRI and microsurgical excision were performed. Despite the introduction of new diagnostic and surgical instrumentation, the preoperative diagnosis and surgical management of such tumours need further discussion.     

脊髓内胆脂瘤的诊断和手术治疗

目的提高脊髓内胆脂瘤的诊断水平及选择治疗方法。方法总结１９７８～１９９５年收治的经手术和病理证实的１０例脊髓内胆脂瘤的经验。结果患者临床表现无特异性。本组ＣＴ检查８例，显示肿瘤呈现均匀低密度影像，边界清楚，强化扫描多无增强。ＭＲ检查４例，肿瘤呈现信号强度变化不定的特点，边界清楚。１０例患者均行手术治疗，肿瘤全切除１例，次全切除９例。８例术后得以随访，无复发。结论肿瘤全切是治疗本病的最好选择。     

Intramedullary epidermoid associated with an intramedullary spinal abscess secondary to a dermal sinus.

Intramedullary spinal abscesses are rare and potentially devastating lesions. A heightened clinical awareness of patients at risk is essential for an early diagnosis and intervention to improve the prognosis of these patients. The first case of spinal abscess was described in 1830, and only 63 cases have been reported subsequently. Intraspinal epidermoids also are unusual lesions. These tumors were reported first in 1829 and represent less than 1% of all intraspinal tumors. We present a case of a 2-year-old girl who had rapidly progressive paraplegia with urinary and bowel retention and was found to have an intramedullary epidermoid and an intramedullary abscess as a result of a dermal sinus. The association of an intramedullary abscess and epidermoid tumor, two rare lesions, has not been reported previously. We also review the literature related to both intramedullary epidermoids and intramedullary abscesses of the spinal cord. Advances in sectional imaging as described should help lead to an early and safe diagnosis of these rare but often debilitating lesions. This case illustrates the importance of a complete neurological assessment of all patients with a congenital dermal sinus because of the potential for intradural extension and the frequent association with other dysraphic abnormalities. Prophylactic surgery is indicated in many cases to prevent dangerous and recurrent infections of the central nervous system.     

Intramedullary epidermoid cyst presenting with abnormal urological manifestations

STUDY DESIGN: Report of an epidermoid cyst with intramedullary localization. OBJECTIVE: To describe an atypical presentation of intramedullary epidermoid cyst. SUMMARY OF BACKGROUND DATA: Intramedullary epidermoid cysts are rare entities with a marked variability in the clinical presentation, essentially of neurological pertinence. METHODS: Case report of a spinal epidermoid cyst in a 13-year-old girl presenting with urological symptoms: she had a 12-month history of recurrent low urinary tract infections, urinary frequency and nocturnal enuresis. A urodynamic evaluation was performed and showed the presence of involuntary bladder contractions with detrusor instability and low bladder compliance. Magnetic resonance imaging of the spine demonstrated an intramedullary lesion of the dorsal spinal cord. RESULTS: The mass was excised and 6 months after surgical excision, urological manifestations improved with decreased detrusor hyper-reflexia, increased bladder capacity and compliance and no later report of urinary tract infections. CONCLUSIONS: In our patient, unusual clinical manifestations of the tumor have delayed the diagnosis, but its complete removal has led to remission of symptoms. Detailed neurological examination and investigations are indicated in patients with clinical and urodynamic features, suggestive of neuropathic bladder.     

Intradural arachnoid cysts of the spinal canal associated with intramedullary cysts

Five patients had intradural arachnoid cysts of the thoracic spinal canal associated with syringomyelia or posttraumatic intramedullary spinal cord cysts. Three cases were diagnosed 6 to 18 years after spinal surgery and two 14 to 17 years after spinal cord trauma. In each case, delayed progression of symptoms led to the identification of the lesions. The diagnosis was assisted by the use of myelography and delayed computerized tomography scanning in two cases and by magnetic resonance imaging in all five. In each case, the arachnoid cyst appeared to compress the spinal cord or nerve roots; in three cases, the syrinx cavities appeared to exert a significant mass effect. In the two trauma-related cases, the intramedullary cysts were small and may have represented areas of cystic myelomalacia. In four cases, intraoperative real-time ultrasonography helped to localize the arachnoid and intramedullary cavities. All five patients were treated by fenestration of the arachnoid cyst; additional peritoneal shunting of the cyst was performed in one case and of the intramedullary cavity in three. In one patient, the two lesions appeared to have a balancing effect; after drainage of the arachnoid cyst, the syrinx cavity expanded and had to be treated separately. The neurological deficits were reduced in four patients and stabilized in one. Intradural arachnoid cysts and intramedullary cysts may occur together as a late complication of spinal surgery or spinal cord trauma, and either or both lesions may cause delayed neurological deterioration.     

Multiple intramedullary lipomas with conal intramedullary dermoid: magnetic resonance appearances.

BACKGROUND CONTEXT: Intramedullary fat-containing benign childhood tumors of the cord include lipomas, dermoid cysts, and teratomas. These are embryonal tumors. Most intramedullary fat-containing tumors are solitary. Multiple intramedullary lipomas are rare and may represent a spinal lipomatous malformation. The presence of another intramedullary dermoid tumor in the same case is rare. PURPOSE: The intent of this case report is to look at magnetic resonance features and possible mechanisms of association of these fat-containing intramedullary tumors. STUDY DESIGN/SETTING: A 3-year-old male child presented with spastic quadriplegia. METHODS: Magnetic resonance imaging (MRI) of spine was done on a 1.5-T scanner in different planes. RESULTS: MRI showed multiple intramedullary spinal lipomas with an intramedullary dermoid involving the conus, cord atrophy, and subarachnoid fat droplets. CONCLUSION: Multiple intramedullary lipomas with an intramedullary dermoid represent a form of spinal lipomatous malformation. Both may represent embryogenic mesenchymal inclusions and hamartomatous growth, which can be accurately diagnosed with MRI.     

Large intramedullary abscess of the spinal cord associated with an epidermoid cyst without dermal sinus. Case report

An intramedullary abscess of the spinal cord (IASC) represents a rare disease associated with a potentially devastating outcome. Few cases involving children suffering from an IASC have been reported in the neurosurgical literature. In the majority of the reported pediatric cases there were either congenital abnormalities, such as a dermal sinus, or signs of local infections leading to a secondary hemopoietic spread. The authors report the case of an 18-month-old girl with an extensive IASC associated with an epidermoid cyst extending from T-11 to S-2 without evidence of a dermal sinus or history of clinically apparent systemic infection. To their knowledge, this is the first case report of an IASC without a condition facilitating either direct contamination via a dermal sinus or hemopoietic spread from an infectious focus outside the central nervous system. Signs and symptoms, the clinical course, and imaging features are discussed and the relevant literature is reviewed.     

Spinal intramedullary ependymal cyst: a case report.

BACKGROUND: Spinal intramedullary ependymal cysts are extremely rare. Only seven pathologically proven cases have been reported in the literature. METHOD: We present an 18-month-old female with thoracic spinal intramedullary ependymal cyst that was diagnosed pathologically. RESULTS: Histological diagnosis was made by light microscopy after immunostaining. After partially removing the cyst wall and establishing communication between the cyst and the subarachnoid space, the patient improved neurologically. CONCLUSIONS: For spinal intramedullary ependymal cyst we recommend diagnosis by MR imaging without myelography, then enucleation of the cyst, if possible. Otherwise, we remove the cyst wall as much as possible and create adequate communication between the cyst and the subarachnoid space.     

Epidermoid cysts associated with thoracic meningocele

Summary 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. Correspondence: Chih-Jen Wang, M.D., Ph.D., Department of Neurosurgery, Kaohsiung Medical University, 100, Tzyou 1st Road, Kaohsiung 807, Taiwan.     

The association of hydrocephalus with intramedullary spinal cord tumors: a series of 25 patients

171 patients with intramedullary spinal cord tumors were operated on, of which 25 patients (15%), mostly children, developed symptomatic hydrocephalus. Twenty patients (12%) had malignant tumors, with 13 of the 20 cases (63%) complicated by increased intracranial pressure and ventriculomegaly. Of the remaining 151 patients with benign tumors (89%), only 12 (8%) developed symptomatic hydrocephalus. In an effort to understand the relationship between hydrocephalus and intramedullary spinal cord tumor, the authors analyze the level and histology of the neoplasm, as well as its association with spinal cysts. A review of the neurosurgical literature reveals that 34 similar cases of hydrocephalus associated with intramedullary spinal cord tumors have been reported to date. The authors note that the presence of hydrocephalus in patients with malignant intramedullary astrocytomas is associated with a shorter rate of survival than in those patients with high-grade lesions but without hydrocephalus, apparently due to rapid tumor progression. The ventriculomegaly seen with benign spinal cord gliomas has no statistically significant effect upon long-term prognosis.     

Spinal intramedullary arachnoid cyst: case report and literature review

Background contextIntramedullary arachnoid cysts are extremely rare; only 14 cases have been reported in the literature so far.PurposeWe report on the case of a 31-year-old woman who presented with back pain and progressive paraparesis secondary to a dorsal intramedullary arachnoid cyst detected on magnetic resonance imaging (MRI): the surgical planning and clinico-radiological outcome are discussed along with a review of the relevant literature.Study designCase report and literature review.Patient sampleOne patient affected by intramedullary arachnoid cyst.Outcome measuresMagnetic resonance imaging and pathological findings from operative specimens were used to confirm the diagnosis.MethodsA 31-year-old woman presented with a 7-year history of back pain that had worsened 3 months before admission to our department; for this reason, the patient had undergone a spinal MRI revealing the presence of a 1-cm cystic intramedullary lesion at the level T11–T12, with no contrast enhancement. After 2 months, the patient presented with a worsening of clinical symptoms complaining of severe back pain radiating to the lower extremities associated with a progressive paraparesis, urinary incontinence, and abdominal pain. Referred to our department, at the time of admission the patient was bedridden because of the impossibility of maintaining a standing position. The patient underwent a T11–T12 laminectomy with fenestration of the cyst.ResultsShe experienced an immediate relief of pain symptoms, and by the seventh postoperative day she was able to stand without help and walk a few meters with assistance. By the sixth postoperative month, the patient had significantly improved, having gained the ability to walk alone without assistance with complete resolution of the bladder dysfunctions, with no cyst recurrence after approximately 2 years of follow-up.ConclusionsIntramedullary arachnoid cysts should be considered in the differential diagnosis for intramedullary cystic lesions. A particular consideration deserves their occurrence in asymptomatic patients, who should be adequately informed on the possible natural evolution: when symptomatic, surgical therapy should be promptly offered, considering that a postoperative complete recovery is usually observed, regardless of the surgical technique.     

Intramedullary epidermoid cyst of the cervical spinal cord associated with an extraspinal neuroenteric cyst: case report.

A 4-year-old girl presented with an intramedullary epidermoid cyst of the cervical spinal cord. The clinical, radiological, and surgical features and a brief critical review of the literature are included in this report. The cyst contents were removed totally in two operations. The child had a coexisting neuroenteric cyst in the posterior mediastinum. To our knowledge, this coexistence has not been previously reported. Contemporary imaging modes and prospects of the surgical treatment are discussed.     

Dysembryogenetic spinal tumours in adults without dysraphism

The authors report 15 patients with spinal intradural dysembryogenetic tumours with clinical onset in adult age in the absence of clinical and radiological signs of dysraphism. The series includes seven lipomas, four epidermoid cysts, three dermoid cysts and one teratoma. The tumour site was the thoracic region in three cases, the lumbar cord and conus in six, the cauda equina in four and the filum terminale in two. Among 14 patients operated upon, the surgical removal was complete in eight cases, subtotal in two and partial in four. Recurrence was noticed only in one subtotally resected thoracic epidermoid cyst. Magnetic resonance imaging allows a precise diagnosis of these lesions, mainly of small lipomas and dermoids of the conus and filum, where a tethered conus is responsible for clinical symptoms. Dysembryogenetic spinal rumours that become symptomatic in adult age may require surgical treatment. Reduction of the mass and release of any associated tethered neural elements are the goal of surgery for spinal lipomas, whereas epidermoid and dermoid cysts require a more radical treatment. However, even partial resections to avoid neural damage result in a good clinical outcome and very low risk of recurrence.     

Spinal intramedullary ependymal cyst and tethered cord in an adult. Case report

Spinal intramedullary ependymal cysts are extremely rare. Fourteen cases have been reported in the literature, and only one was associated with another congenital lesion. The authors describe the case of a 33-year-old man in whom an intramedullary ependymal cyst and filar lipoma were present. These lesions were diagnosed using magnetic resonance imaging and were treated surgically. Pathological examination confirmed the diagnosis. At 6 months postsurgery, there was no evidence of recurrence.     

Long segment spinal intramedullary dermoid cyst: A case report

IntroductionSpinal intramedullary dermoid is very rare, accounting for <1% of intraspinal tumors. It can be congenital or acquired. They usually present in 2nd or 3rd decade of life in adults. It may or may not associated with spinal dysraphism. It is asymptomatic in most cases, manifest acutely if it ruptures.Long segment involvement of spinal intramedullary tumor in adult without the history of trauma makes this case unique. Fat suppression imaging helps to distinguish adipose tissue from lesions causing hematomyelia in patients presented with intramedullary hyperintensity in both T1 & T2 sequences of MRI spine.Case presentationWe report here a rare case of 30 years old male who presented to us with sudden urinary retention followed by rapidly progressive quadriparesis and paresthesia in his right arm. In MRI, spinal intramedullary tumor was noted from medulla till D5 vertebra. We performed subtotal excision of tumor and sample sent for histopathology which proved it to be intramedullary spinal dermoid cyst.ConclusionLong segment involvement of spinal intramedullary dermoid cyst in adult without history of trauma makes this case different. Fat suppression imaging must be done in patients with intramedullary hyperintensity in both T1 and T2 sequences of MRI spine. Early diagnosis and appropriate management will be helpful in reducing morbidity.     

Magnetic resonance imaging in cases of spinal dural arteriovenous malformation

Two patients with spinal dural arteriovenous malformations associated with intramedullary changes confirmed by the T2-weighted magnetic resonance imaging (MRI) scans are reported. The characteristics of the MRI findings for these 2 patients were as follows. 1) In the T2-weighted spin-echo image, intramedullary changes observed by MRI were visualized as a high signal intensity area at the level where delay in venous circulation of the spinal cord was revealed by the angiography of the spinal cord. 2) After the obliteration of the arteriovenous shunt by surgical management, intramedullary changes remarkably decreased and disappeared with the disappearance of swelling of the spinal cord observed preoperatively. After that, the atrophy of the spinal cord was shown. 3) The level, extent, and severity of intramedullary changes were decided by the condition of the level where the radiculospinal vein, as the flowing vein, refluxes into the coronal venous plexus and venous flow occurs through the radiculospinal vein into the epidural veins. We suspect that intramedullary changes shown by the T2-weighted MRI scan chiefly represent edema of the spinal cord, caused by an increase in venous pressure due to venous congestion of the spinal cord. MRI is a very useful diagnostic aid to detect intramedullary changes associated with a spinal dural arteriovenous malformation and to evaluate therapeutic results after surgery.     

Neurenteric cyst: surgery and diagnostic imaging

Neurenteric cysts are rare, with fewer than 30 cases noted in the literature. We report the case of a newborn infant with respiratory distress caused by a large neurenteric cyst that was identified by prenatal ultrasound. Treatment consisted of excision of the mass through a right posterolateral thoracotomy. The cyst adhered to the spine at the level of the first thoracic vertebra and communicated with the jejunum through a posterior diaphragmatic defect. Postoperative studies with magnetic resonance imaging (MRI) and computed tomography (CT) disclosed an anterior meningocele and tethering of the spinal column. This is the second reported case of a neurenteric cyst demonstrated by prenatal ultrasound. The presence of an intrathoracic cyst associated with spinal abnormalities is characteristic of this anomaly. With imaging techniques such as MRI and CT, we may detect residual intraspinal disease associated with neurenteric cysts.