Intramedullary Lipoma Research Articles

Congenital spinal lipomatous malformations

To report this author's experience with patients with a congenital spinal lipomatous malformation with special emphasis on variations in clinical presentation, operative findings, and outcome based on the classification scheme proposed in the first part of this two part article. From January 1995 to July 2005, 80 patients with a congenital spinal lipomatous malformation were treated. All patients underwent routine neurological examination, plain radiographs of the spine and all but 10 patients underwent MRI. Ten patients underwent CT-myelography. Hoffman's functional grading scale was used for preoperative and postoperative clinical assessment. The operative findings, complications and outcome were assessed. Age ranged from 18 days to 19 years. The female: male ratio was 3:2. The malformations were divided into two groups: Group I: Lipomas without a dural defect and, Group II: Lipomas with a dural defect. Included in Group I were: 22 patients out of which there were Caudal lipomas: 10, Filum lipomas:11 and intramedullary lipoma: 1. In Group II there were 58 patients out of which there were Dorsal lipomas: 8, Caudal lipomas with dural defect: 8, Transitional lipomas: 10, lipomyelomeningoceles:28, lipomyeloceles: 4. Most of the group I patients were >5 years of age; cutaneous markers were absent in 60%, older children more often presented with sphincter disturbances. Surgery in group I was straight forward and consisted of sectioning of the filum in filum lipomas, debulking and untethering in caudal lipomas. Duroplasty was seldom required. CSF leak was rare. No patient deteriorated following surgery and no retethering was noted during follow-up. In Group II, all patients had cutaneous markers, most were <2 years of age, 19 were asymptomatic, older children had more severe neurological deficits. Duroplasty was required in most cases. A CSF leak occurred in 12%. Two patients deteriorated temporarily following surgery. Two patients presented with retethering 4 and 8 years after initial surgery. Improvement of more than one Hoffman's functional grade occurred when surgery was done <2 years of age. Congenital spinal lipomatous malformations do not constitute a single homogenous entity. They can be broadly classified into two groups depending on the presence or absence of a dural defect. These two groups are different from one another embryologically, clinically, surgically and prognostically.

Congenital spinal lipomatous malformations

Congenital spinal lipomatous malformations constitute a diverse group of lesions. There is considerable confusion in the literature regarding their terminology and a proper classification is long overdue. The first part of this two part report sets out a proposed classification scheme. On the basis of this author's experience with 80 patients with a congenital spinal lipomatous malformation treated over a 10 year period, a new classification is proposed. The proposed classification divides congenital spinal lipomatous malformations into two broad groups: 1. Lipomas without dural defect and, 2. Lipomas with dural defect. Within each group, there are several subtypes. These two broad groups differ from one another in their embryology, clinical presentation, operative findings, complications and prognosis Group I consists of Lipomas without dural defect. Included in this group are: Filum lipoma, caudal lipoma without dural defect, and intramedullary lipoma. Group II consists of lipomas with dural defect. Included in this group are: dorsal lipoma, caudal lipoma with dural defect, transitional lipoma, lipomyelocele, and lipomyelomeningocele. The definitions of the various subtypes and radiological and operative findings of all these lesions are described. Congenital spinal lipomatous malformations constitute a wide spectrum of lesions ranging from relatively simple lipomas of the filum terminale to complex malformations. These lesions differ from one another in their embryology, clinical presentation, operative strategies, complications and prognosis. Failure to differentiate between the different forms of congenital spinal lipomatous malformations may lead to inaccurate assumptions regarding prognosis and inappropriate management. The proposed classification seeks to address these issues.

Cervicobulbar intramedullary lipoma

Background context Spinal lipomas not associated with spinal dysraphism are rare entities. Further, large medullary lipomas with intracranial extension are occasionally described. Most of the intradural lipomas are subpial and not really intramedullary. Purpose Clinicians may be made aware of such a rare entity and its presentation. Study design/setting An extremely rare case of medullary lipoma extending into posterior fossa is described in an adult, not associated with spinal dysraphism. Methods A young male presented with high cord myelopathy in the form of spastic quadriparesis. Radiological investigations revealed dorsally placed intramedullary lipoma extending into posterior fossa. Results Surgical excision of tumor provided satisfactory resolution of symptoms. Conclusions Medullary lipomas may present with features of high cord compression and myelopathy. Magnetic resonance imaging remains the investigative and follow-up modality of choice. Even when total tumor excision is not feasible, subtotal removal and decompression provides long lasting symptom-free survival.

A massive cervicomedullary intramedullary spinal cord lipoma

We present a large cervicomedullary intramedullary lipoma in a 17-year-old boy, who presented with spastic quadriparesis and exaggerated reflexes. MRI revealed an intramedullary lipoma extending from the craniovertebral junction to the sixth cervical vertebra. There was no spinal dysraphism. Subtotal excision with primary dural closure was performed, which produced an improvement in the patient’s condition. The technique we used is discussed.

Spinal cord lipoma without dysraphism in the infancy that extends intracranially. Case report and review of the literature

Background Spinal intramedullary lipoma extending to the posterior fossa is very rare in children. We made a revision of the literature and report the diverse opinions and surgical procedures. Case Description We report the case of a 16-month-old male infant who was operated on in the Pediatric Neurosurgical Unit of our hospital; his clinical and surgical outcome are related. Conclusion Intramedullary lipoma of the spinal cord with intracranial extension in infancy is a very rare pathology reported in the literature. Our patient was treated with decompression and subtotal removal; he had a critical postoperative course but was recovering slowly after that. Most authors prefer incomplete resection because these lesions do not have a clear-cut margin. Another neurosurgeon performed a decompression only, without resection. Some authors performed the surgical treatment before symptom progression. Another surgeon reported that the surgical indication is debatable when the neurologic manifestations are poor or absent. The surgical indication and the strategy for treatment are controversial and they depend on the clinical situations of the patients.

Concomitant cervical and lumbar intradural intramedullary lipoma

Background Lipomas of the spinal cord are often a component of spinal dysraphic states. Nondysraphic intramedullary spinal cord lipomas are rare, and concomitant isolated cervical and lumbar intradural intramedullary lipomata are very rare. One patient with concomitant isolated nondysraphic cervical and lumbar spinal cord lipomata has been reported and management options discussed. Case Description A young girl presented with insidious-onset diffuse neck pain and early myelopathic signs. Conventional radiographs were normal. Magnetic resonance imaging of the whole neuraxis revealed concomitant cervical and lumbar intradural intramedullary lipoma. Subtotal resection of the lesion was performed at both levels, after which the patient improved symptomatically and was ambulant independently. Conclusion Concomitant intradural lipomas at 2 different locations unassociated with a dysraphic state is very rare. Magnetic resonance imaging with fat suppression study is the investigation of choice. Adequate decompression with subtotal removal is the treatment of choice.

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.

Presumed rupture of a conus medullaris dermoid cyst with cervical intramedullary fat and lipomatous infiltration of the cauda equina

J. Neurosurg: Spine / Volume 5 / August, 2006 Spinal dermoid cysts result from a congenital or acquired inclusion of ectodermic and mesodermic embryonic rests. They can be intramedullary, intradural extramedullary, or extradural lesions. Ruptured dermoid cysts of the lumbar spine in patients without spinal dysraphism are very rare. This 35-year-old man presented with ascending left-foot numbness after golfing. A T1-weighted magnetic resonance (MR) imaging sequence revealed an intramedullary lesion extending from T12 to L-1 and a C1–7 enhancing lesion (Figs. 1 and 2). A syrinx extended to T-11 and subarachnoid fat droplets were present (Fig. 1). Examination of computed tomography findings ruled out the diagnosis of spinal dysraphism. A combined L-1 laminectomy and cordotomy was performed. Pathological examination confirmed the diagnosis of a dermoid cyst. In addition, an intradural extramedullary lipomatous mass involving the cauda equina was found intraoperatively, consistent with a terminal filum lipoma. The patient’s symptoms resolved postoperatively, and MR images at 1 year are unchanged. Symptomatic lumbar spinal dermoid cysts can present as syndromes of the conus medullaris, cauda equina, or as radicular pain. Their rupture can be spontaneous, posttraumatic, or occur following surgery and can cause meningitis, seizures, hydrocephalus, and transient ischemic attacks. Fat droplets have been found in asymptomatic ruptured dermoid cysts located in the central canal in three cases1,2 and in the central cord in one case.2 The intramedullary migration of fat droplets is unexplained and the possible presence of a multifocal dermoid cyst cannot be excluded. A combination of a lipoma and a teratoma of the conus medullaris has been reported4 as well as a dermoid cyst with an intramedullary lipoma.3 In the present case the patient probably had a dermoid cyst with a terminal filum lipoma, which was not visible on MR images. Surgeons should be aware of these combinations because they can affect the surgical plan.

Intramedullary lipoma of the cervicodorsal spinal cord with intracranial extension: case report

Background An unusual case of an extensive intramedullary lipoma of the cervicodorsal spinal cord with intracranial extension causing compression of spinal cord and brainstem is described. Case Description A 3-year-old child with lack of limb movement since birth, the cause of which had remained obscure, developed opisthotonos and episodes of severe respiratory difficulty with brief periods of apnea. Craniospinal computed tomography and magnetic resonance imaging (MRI) were diagnostic of lipoma. Urgent occipitocervical decompression and subtotal removal of the posterior fossa and cervical spinal cord lipoma resulted in lasting resolution of symptoms. Conclusions Extensive spinal cord lipomas with intracranial extension can present with alarming spinal cord/medullary compression and respiratory symptoms. Computed tomographic scan and MRI are diagnostic. Total removal is not feasible, or necessary, because subtotal removal and decompression give long-lasting resolution of symptoms.

Dorsal intramedullary dermoids

We report a case of a 31-year-old woman with an intramedullary thoracic dermoid, who had progressive spastic paraparesis in her lower extremities over a period of three years, as well as loss of bowel and bladder control. She had been operated for "spina bifida" at three days of age but had no neurologic deficit. MRI of the thoracic spine showed an intramedullary hypointense lesion at T7-T8, associated with an intramedullary lipoma at a higher level. Intracapsular resection of an intramedullary dermoid cyst was performed. We review the literature on intramedullary dermoids differentiating them from the more common, but still rare, lumbosacral subdural juxtamedullary dermoids. We discuss the associated malformations with these lesions, as well as their pathophysiology and treatment. Although uncommon, dorsal intramedullary dermoids should be suspected and treated in a timely manner, especially in presence of other congenital anomalies.

Nondysraphic intramedullary spinal cord lipomas: a review

Lipomas of the spinal cord are often a component of spinal dysraphic states. Nondysraphic intramedullary spinal cord lipomas are rare, and their presentation, in the form of gradually worsening myelopathy, is nonspecific. The authors report on the methods used for diagnosis and treatment in patients presenting with these lesions at their institution, and they review the relevant literature. The authors treated 14 patients who presented with intramedullary lipomas over a period of 12 years. None of these patients had segmentation anomaly or dysraphism of the spine, or any hindbrain anomaly. Admission magnetic resonance images were diagnostic in all patients. All tumors were located dorsally in the spinal cord, the majority of them in the cervicodorsal cord. The lipomas were partially excised with the help of a carbon dioxide laser in all patients in an attempt to preserve neurological function. All patients attained improvement in their sensory and motor symptoms. Because these lesions do not have a clear-cut margin, it is vital to preserve neurological function at the time of surgery, even if it entails incomplete resection.

Surgical treatment of spinal lipomatous malformations

The value of early surgery in spinal lipomatous malformations has not been proved. This paper presents the results of surgical treatment performed immediately after detection in 11 patients presenting with three different groups of malformations: intramedullary lipomas—two patients, conus medullaris lipomas—seven patients, and filum lipomas—two patients. The diagnostic work-up included MRI, CT and spinal X-rays, and functionally the patients were investigated with SSEP and urodynamic tests. The surgical technique for the intramedullary lesions was partial debulking, and total microsurgical excision was performed on filum lipoma patients. Conus lipomas were managed by partial excision, with an emphasis on: (1) dissection of the malformative cord beginning from the normal area; (2) debulking of the lesion without approaching the conus–fat interface to make reconstruction of the conus with pial approximation possible; and (3) redundant dural sac reconstruction. The results are analyzed in view of the technique applied, and after a follow-up ranging from 9 months to 4 years, no significant early complications were observed in the conus medullaris lipomas group. The other two groups showed the predictable results, arrest of deterioration in the intramedullary lipomas and an excellent outcome for the filum lipoma patients.

Intramedullary lipoma and epidermoid—rare coincidence in elderly

Objectives: The incidence of intramedullary lipoma or epidermoid is less than 1% of all spinal tumors, particularly in adulthood. A variety of concurrent spinal anomalies can be found: from occult spinal dysraphism, dermal sinus tracts, hypertrichosis, spina bifida, to dural bands, tethered spinal cord with thick filum terminale to lipoma, epidermoid, diastematomyelia and lipomeningomyelocele. The typical manifestation in the elderly is low extremity pain, weakness, paresthesia, paraparesis, hyperreflexia and sphincter disorders. The onset of symptoms occurs often after sudden stretching or after low-intensity trauma, but relatively asymptomatic development can be found over a longtime period. Method: The authors present their experiences with three patients over a 20-year period. Intradural exploration was performed in two cases; microsurgical technique was used and all associated anomalies were operated on during the same operation. Results: MR was the imaging tool of choice. Diagnosis of lipoma is based on the T1 and T2 image with high-signal intensity, contrary to epidermoid (T1 low intensity, T2 very high-signal intensity and proton density differing from CSF, image possibly inhomogenous, sharply marginated or lobulated). Enhancement effect is not present in both lesions. The findings of the MRI were difficult to interpret. Microsurgical treatment differs in this. For epidermoid, we recommend intracapsular decompression and radical removal of the capsule, the only growing part of the tumor, with close adherence to the neurovascular structures, because the remnant can produce recurrency of the lesion. In spite of this fact, lipoma is the nongrowing part of this common lesion, and there is no cleavage space between the medulla and tumor. Therefore, only partial removal with a safe strip of tissue left is recommended. The treatment of concurrent anomalies is mandatory. Conclusion: The conventional explanation for clinical symptoms is tethering of the anchored spinal cord, stretching and low-intensity injury or, more often, involution changes. The authors also suggest that progressively slow linear growth with compression of the neurovascular structures can cause late clinical manifestations in the elderly. On the basis of their limited experiences, the authors do not recommend prophylactic surgery in asymptomatic or stabilized patients.

Non-dysraphic Intramedullary Spinal Cord Lipoma: Case Report

Spinal cord lipomas are rare tumors with a reported incidence of 1% of all intraspinal tumors. We recently experienced a case of intramedullary lipoma without spinal dysraphism in a 58-year-old woman. MRI, CT, myelography and CT myelography showed the characteristic findings of a lipoma.

A rare case of intramedullary lipoma associated with cyst

Intramedullary lipomas are benign tumours of the spinal cord corresponding to 1% of all primitive intramedullary tumours. We report a rare case of "true" intramedullary lipoma associated with cyst. The patient underwent subtotal resection and the diagnosis was made by histopathological examination. There was postoperative neurological improvement.

Spinal lipomatous malformations.

Spinal lipomatous malformations (SLM) include all the closed neural tube defects (NTD) with excessive lipomatous tissue in the spinal cord or filum terminale. We evaluated 65 cases of SLM seen & operated at our department in the last 7 years. Of these only 9 were asymptomatic and 8 were below 2 years of age. There were more males than females. In addition to subcutaneous lipoma many patients also had hypertrichiosis and dermal sinus as cutaneous makes. Twenty patients had foot deformity and 5 had unilateral limb shortening. Sixty-two patients had MRI and 3 had CT myelogram for evaluation. These revealed 7 patients with Chiari malformations, 10 with focal syrinx and 2 patients underwent VP shunt for hydrocephalus. Sacral agenesis was seen in 5 patients. Clinical features were similar to other cases of spinal dysraphism except that sensory loss and trophic ulcer were more frequent. Intradural lipoma and tethering was seen in 18 cases whereas intramedullary lipoma & conus lipoma was seen in 40 & 7 cases respectively. Additional tethering lesion was seen in 1/3 cases and was treated simultaneously. Preoperative deficits improved in 28 cases and stabilized in 33 cases. Three patients developed fresh deficits after surgery. We observed 8 CSF leaks and 4 wound infections in postoperative period. It is very clear from our data that a patient has about 95% chances that his neurological status may improve or stabilize following surgery and the risk of developing fresh deficits is about 5%. We, therefore, suggest that all patients of SLM should be treated with aggressive surgical management for best results.

Lipoma intramedular cervicodorsal de manifestación neonatal

We present the case of a ten day old boy with cervicodorsal intramedullary lipoma. The initial clinical presentation was of rapidly progressing unilateral brachial paralysis. Neurological examination at birth was completely normal apart from a cutaneous port wine stain on the shoulder homolateral to the paralysis thought to be due to an angiolipoma. On clinical suspicion of an intradural space-occupying lesion at cervical level, a magnetic resonance study was done which showed an intramedullary tumour at cervicodorsal level extending from C3 to T3, which disappeared with the fat suppression technique, a finding characteristic of lipomas. The anatomo-pathological report was that the mass was a tumour made up of mature fat cells in a connective tissue stroma, with no malignant formation or granulomatous components. Lipomas are the second most common spinal tumors in infancy after neuroblastoma. This type of tumour is usually found at lumbosacral level and is considered to be congenital. This lipoma was unusual in being cervicodorsal (a common site in adults but not in infancy), being intramedullary (most are extramedullary even when they are intradural), presentation being so early during the neonatal period, rapidly progressive and relapsing after surgical removal.

Intramedullary Holocord Lipoma in an Athlete: Case Report

This case report involves an intramedullary lipoma of the dorsum of the spinal cord, which essentially runs the full length of the cord. This occurred in an individual who had an active life, including involvement in athletics. The authors point out that the case presentation myelopathy as well as nonradicular pain should alert one to the possibility of this type of tumor. Although this is a very brief case report, it does address many of the problems of isolated intradural intramedullary lipomas. I have observed a number of large intradural intramedullary lipomas, but as the authors point out, this is a rare situation in which the lipoma is holocord. I think the management of this case was appropriate. David G. McLone Chicago, Illinois

Microsurgical treatment of intramedullary spinal cord tumors.

Eighty patients with intramedullary spinal cord tumors were treated by microsurgical methods between 1988 and 1996. Twenty-nine patients were diagnosed with astrocytoma, 36 with ependymoma, five with metastasis, four with lipoma, three with dermoid or epidermoid tumor, two with neurofibroma, and one with neuroma. Vascular and infectious lesions (such as abscesses and hemangioblastomas) were excluded. After laminectomy, total removal of the lesion was achieved in 68 of 80 patients and subtotal removal in 12. Postoperative radiation therapy was performed in 13 of 80 patients. The follow-up period ranged from 12 to 92 months (mean 42.2 months). All patients underwent preoperative and postoperative magnetic resonance imaging at intervals ranging from 3 months to 5 years postoperatively. Four patients showed clinical and radiological evidence of local tumor recurrence during the follow-up period. Four patients died 5 months to 15 months postoperatively from the re-expansion of their primary metastatic disease. The operative results at long term (after the 6th postoperative month) were better than the results at short term (before the 6th postoperative month) and revealed clinical improvement in 63, no change in 10, and deterioration in seven patients. We recommend early radical surgery, whenever possible, to be performed when the patient's neurological status is still good. Subtotal removal and irradiation are better for malignant or metastatic tumors. Partial decompressive removal is best for large intramedullary lipomas. Plastic laminotomy with preservation of the intervertebral joints is especially recommended in young or middle-aged patients.

Cervical intramedullary lipoma with unusual MRI features: case report

Cervical intramedullary lipoma with unusual MRI features: case report