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Intracranial Lipoma Embryologic Origin

Theories regarding the pathogenesis of intracranial lipomas have evolved since the first report of a chiasmatic lipoma by Meckel in 1818.23 A contemporary consensus holds that intracranial lipomas result from the abnormal persistence and maldifferentiation of the meninx primitiva during the development of the subarachnoid space.20 This theory, first proposed by Verga in 1929 and well summarized and further supported by Truwit and Barkovich in 1990,20 accounts for several of the distinctive features of intracranial lipomas the subarachnoid, cisternal locations of intracranial lipomas the commonly associated parenchymal malformations (see Table 37-1) and the incorporation of, and respect for, intracranial vessels and nerves. Anomalies Associated with Intracranial Lipomas Genetic Syndromes that Manifest Intracranial Lipomas Encephalocutaneous lipomatosis The meninx primitiva, first described by Salvi, is a mesenchymal derivative of the neural crest. Between days 32 and 44 of...

Intradural Intramedullary and Intradural Extramedullary Lipomas Unassociated with Spinal Dysraphism

Spinal cord intradural, intramedullary lipomas are rare entities, usually found along the dorsal aspect of the thoracic spine and in the midline.12,23 In one early report, they were found in decreasing frequency over the cervical-thoracic spine and over the cervical spine. In addition, there are eight reports of intramedullary spinal cord lipomas affecting nearly all levels of the spinal cord, known as holocord lipomas.12 Patients with spinal cord lipomas most often have slowly progressive signs of spinal cord compression. The most commonly reported presenting symptoms are those of an ascending spastic motor weakness in one or both legs.10,12,23 The clinical presentation and examination findings reflect the location and size of the spinal cord lipoma. Spinal cord lipomas are uncommon, with approximately 200 reported cases, predominantly of the intradural or extra-medullary type, having been identified in the cervical and upper thoracic spine since the first report by Gowers in 1876.12...

Lipomas of the Spinal Cord Associated with Spinal Dysraphism

The term lipomyelomeningocele refers to a congenital lesion of the meninges or spinal cord plus meninges and the presence of benign fat, both of which exist with a dysraphic spinal column. Depending on the classification system used, these lesions may be divided into as many as five subtypes as they appear on neuroimaging.2 In general, a lipomyelomeningocele is recognized on MRI as a caudally descended conus medullaris invested in fat. Arai et al, using conventional myelogram and MRI, define lipomyelomeningocele as a low lying tethered spinal cord that enters a subcutaneous cystic meningeal sac and terminates in the lipomatous mass on the wall of the meningeal sac.2 The lipoma and meningocele may appear together or on opposite sides of the spinal canal. The asymmetric variety is less common and is generally associated with more severe deformities of the sacrum and lower extremities.23 Lipomyelomeningoceles may Lipomyelomeningoceles are not uncommon, and there is a female-to-male...

Lipomas of the Filum Terminale without Spinal Dysraphism

Lipomas of the filum terminale occur at the caudal tip of the spinal canal. These malformations are more common than lipomyelomeningoceles and greater than 90 are recognizable Patients with lipomas of the filum terminale may have similar symptoms to patients with lipomyelomeningocele and other tethered cord syndromes, including motor weakness, sensory disturbances, bladder and bowel malfunction, and pain. Their imaging and histologic characteristics are as described for other lipomatous tumors. Surgical resection and untethering of the spinal cord are the mainstays of treatment for symptomatic lipomas of the filum terminale. Outcome data for these patients are similar to patients with spinal cord lipomas, with the majority showing improved motor, bowel, and bladder function and decreased pain.3 Recurrence of these lipomas has not been reported. However, recurrent tethering of the spinal cord may occur and should be suspected when recurrent or progressive symptoms are noted.

Lipoma

Lipomas, also known as lipofibromatous hamartomas or neural fibrolipomas, are benign tumors arising from the proliferation of adipose or fibrous tissues. Grossly, they are well circumscribed and encapsulated and have a fusiform and yellowish appearance in the subcutaneous layer.92 Histologically, lipomas can be intimately associated with functioning nerve fibers from which they cannot be easily separated. The median nerve is most commonly affected. Larger lipomas can envelop, infiltrate, or compress important nerves such as the brachial plexus when located in the supraclavicular fossa.61 Overall, lipomas are the most common soft-tissue tumors in adults, occurring in 1 out of 1000 individuals.131 They occur mostly in children or during early adulthood. Treatment includes biopsy of the tumor and decompression of the involved nerve when it becomes entrapped by surrounding structures such as the carpal tunnel. Complete resection of these tumors when involving a nerve is usually not...

Lipomas

Lipomas are mature adipose tissue arranged in a nodular formation surrounded by a capsule and commonly located in the subcutaneous tissues. Lipomas can be diagnosed by clinical examination and identification of their unique, well-circumscribed, round, mobile character with a doughy consistency. Although most do not require it, excision may be considered for large tumors, rapidly growing lipomas, and those causing pain. Lipomas can occur in isolation or with other systemic diseases, as a component of genetically inherited disorders, or in other connective tissue layers. A steroid injection or liposuction can be used to treat select lipomas. Steroid injections can be used to treat painful lipomas less than 1 inch (2.5 cm) in size and in which pathologic examination is likely unnecessary. An injection of 1 to 3 mL of triamcinolone diluted with 1 lidocaine to a 10 mg mL concentration can be performed into the center of the lipoma monthly to shrink the tumor to the desired size. Formal...

Angiolipoma

The presentation of angiolipomas both intracranially and within the spinal canal may be more acute than that of the spinal cord lipomas and intracranial lipomas. Most patients seek treatment less than 1 year after the onset of symptoms. Long and fluctuating courses, however, are not unheard of, making the diagnosis more difficult. The acute precipitation of symptoms, in the case of angiolipomas, is likely related to complications within their vascular component, such as hemorrhage, thrombosis, vascular engorgement, and vascular steal.1,13 Pregnancy has also been noted to precipitate symptoms from spinal and intracranial angiolipomas.1 Like other mass lesions of the CNS, the size and location of the angiolipoma will dictate neurologic signs and symptoms. The epidemiology of angiolipomas is difficult to trace because there are no clear-cut diagnostic criteria to define them. Some authors have suggested that if lipomas and heman-giomas truly represent the limits of the same pathologic...

Intraspinal Lipoma

It is imperative to clarify the nomenclature regarding lipo-matous tumors of the spinal cord. This becomes of utmost importance when discussing treatment options and outcomes data. The lipomatous tumors of the spinal cord may be subdivided into three types intradural or intramedullary spinal cord lipomas not associated with spinal dysraphism lipomyelo-meningoceles (often termed lumbosacral lipomas) associated with spinal dysraphism and filum terminale lipomas. Similar to their intracranial counterparts, intraspinal lipomas may be isolated lesions or associated with significant developmental abnormalities.

Clinical Presentation

More than 30 of patients with intracranial lipomas are asymptomatic.23 The vast majority of the remaining patients have signs and symptoms not attributable to the lipoma but secondary to the associated developmental anomalies. Approximately 30 of patients with supratentorial lipomas have a seizure disorder. The seizure disorder is reportedly often severe, is focal or generalized, and has an average onset age of 15 years. Recurrent headaches are a presenting symptom in 25 percent, and . . . 10 to 15 percent have behavioral problems and or mental retardation.23 Other reported signs and symptoms include fainting spells, vomiting, episodic leg weakness, blurred vision, sleepwalking, diencephalic disturbances such as adiposogenital dystrophy and hypothermia, and even transient ischemic attacks.6 Presenting signs and symptoms of patients with infratentorial lipomas may include hydrocephalus, cerebellar ataxia, motor weakness, sleep apnea, and cochleovestibular symptoms such as hearing loss,...

Gross and Microscopic Appearance

Intracranial lipomas appear as dark-yellow lesions on gross inspection. The meninx primitiva contains within it primitive perivascular reticuloendothelium,6 which may become specialized in the storage of fat, and this may account for the histology of intracranial lipomas. Early precapillary parenchyma may also differentiate into fat cells. Microscopic examination reveals that these lesions are composed of mature adipose cells with peripheral, sometimes indented, small bland nuclei. A col-lagenous capsule that is intimately associated with adjacent brain often surrounds the lipoma. In general, no cleavage plane exists between the tumor and surrounding brain tissue. Histo-logically, although mature fat is the predominant element, variable amounts of fibrovascular tissue, bone, and calcification can also be present. Adjacent parenchymal calcifications and bone formation may occur, although the exact percentage of lipomas showing these changes is not well documented. Bone formation within...

Neurological Applications in Diagnosis and Treatment Extradural Spinal Lesions

Intramedullary lesions causing enlargement of the cord or conus with concomitant narrowing of the adjacent subarachnoid space include primary cord neoplasms such as ependymomas, astrocytomas, and hemangioblastomas metastases inflammatory conditions such as sarcoidosis and abscess formation cord hematomas and infarcts vascular malformations and congenital lipomas, dermoids, and epidermoids. Although these groups of lesions will most certainly cause widening of the silhouette of the cord or conus, the intrinsic pathology and extent of these intramedullary lesions are far better imaged with MRI.

Neurogenic and Vascular Tumors of the Head and Neck

Substance of the tongue.15,16 These lesions usually present as soft, easily compressible, fluctuant, ill-defined posterior neck masses that may come to clinical attention following a recent upper respiratory infection (Figure 16-3). Other neck masses that should be considered in the differential diagnosis of cystic hygromas include lipomas, branchial cleft cysts, lymphomas, and thyroglossal duct cysts, however the clinical presentation and history, supplemented by radiographic imaging of the neck (CT with intravenous contrast), will usually make the diagnosis quite apparent.17

Miscellaneous Intramedullary Pathology

Other non-neoplastic entities can occur as intramedullary spinal lesions.33,38 Vascular malformations, particularly cavernous angiomas, may occur in the spinal cord. Inclusion tumors and cysts are rarely intramedullary. Lipomas are the most common dysembryogenic lesion and account for approximately 1 of intramedullary masses. These are not true neoplasms but probably arise from inclusion of mesenchymal tissue within the spinal cord itself. They typically enlarge and produce symptoms in early and middle adult years through increased fat disposition in metabolically normal fat cells. Lipomas are often considered juxtamedullary, because they occupy a subpial location.

Miscellaneous Extramedullary Pathology

Extramedullary masses may be neoplastic or non-neoplastic. Dermoids, epidermoids, lipomas, teratomas, and neurenteric cysts are inclusion lesions that result from disordered embryo-genesis.39 They may occur throughout the spinal canal but are more common in the thoracolumbar and lumbar spine. Intramedullary locations have also been reported. Associated anomalies such as cutaneous lesions, sinus tracts, occult anterior or posterior rachischisis, or split cord malformations

Chromosomal Anomalies

The incidence of this trisomic syndrome, which occurs primarily in the offspring of older mothers, is approximately 1 in 7000, and it affects mainly females. M It should be noted that translocation can also result in this syndrome, but mothers of these children are usually younger. The clinical characteristics of this syndrome include multiple congenital anomalies with varying degrees of mid-facial anomalies. Infants may have ophthalmic abnormalities including anophthalmia, cyclopia, microphthalmia, and colobomas. Facial defects, such as cleft palate and lip and micrognathia, can also occur. A variety of congenital cardiac abnormalities such as ventricular or atrial septal defects and patent ductus arteriosus may be present. Intestinal malrotation, Meckel's diverticulum, and a spectrum of urogenital structural abnormalities are commonly seen. Polydactyly, with the third and fifth fingers overlapping the fourth, occurs in most affected infants. Neurologically, patients have...

Other Peripheral Nerve Tumors Not Included In The Who Classification System

We have described benign peripheral nerve tumors arising primarily from Schwann cells, in the case of schwannomas and neurofibromas, and perineural cells in the case of perineuri-omas. Other tumors arising from cells normally found in peripheral nerves that will be discussed include mucosal neuroma, neurothekeoma, granule cell tumor, lipoma, vascular tumors, and neuromuscular hamartoma. Peripheral nerve tumors can also originate from cells not normally found in nerves by local invasion, as in the case of desmoid tumors (i.e., from adjacent muscle tissue) or by metastatic spread in the case of primary tumors located at a distance. For example, a variety of primary tumors, such as breast and lung tumors, can metas-tasize to peripheral nerves and their plexi. More rarely, thyroid tumors osteochondromas bladder tumors extracranial meningiomas and malignant Ewing's tumors, which include Ewing's sarcoma, extraosseous Ewing's sarcoma, primitive neuroecto-dermal tumors (PNETs) or peripheral...

Magnetic resonance imaging

The musculoskeletal system is ideally suited for evaluation by MRI since different tissues display different signal intensities on Tj- and T2-weighted images. The images displayed may have low signal intensity, intermediate signal intensity or high signal intensity. Low signal intensity may be subdivided into (i) signal void (black) and (ii) signal lower than that of normal muscle (dark). Intermediate signal intensity may be subdivided into (i) signal equal to that of normal muscle and (ii) signal higher than muscle but lower than subcutaneous fat (bright). High signal intensity may be subdivided into (i) signal equal to normal subcutaneous fat (bright) and (ii) signal higher than subcutaneous fat (extremely bright). High signal intensity of fat planes and differences in signal intensity of various structures allow separation of the different tissue components including muscles, tendons, ligaments, vessels, nerves, hyaline cartilage, fibrocartilage, cortical bone and trabecular bone....

Describe the clinical features of spina bifida occulta

In spina bifida occulta the underlying spinal and or neural defect is covered by intact skin and thus may not be grossly evident on examination. External signs may include a lumbosacral hair tuft (faun's tail), skin-covered lipoma, cutaneous hemangioma, or a lumbosacral skin dimple. If the defect is limited to failure of fusion of the vertebral arch, the finding has little clinical significance. However, more complex types are associated with neurologic, urologic, and or orthopaedic abnormalities. The majority of more complex cases will require surgical intervention to prevent progression of neurologic deficits.

What is a lipomyelomeningocele

A lipomyelomeningocele is a common congenital spinal anomaly in which herniation of a lipoma into the conus medullaris or the dorsal spinal cord occurs through an osseous defect and communicates with an adjacent subcutaneous fatty mass. It is a common cause of tethered cord syndrome. Symptoms may include constipation, urinary urgency, dyspareunia, lumbar pain, or cephalgia (headache) with defecation. The term lipomyelomeningocele is actually a misnomer, because abnormal neural tissue does not extend outside of the spinal canal. Surgical treatment of this anomaly is extremely challenging and should be referred to a regional center with extensive treatment of these lesions.

Epidemiology and Clinical Presentation

Less common, but overrepresented, manifestations in MEN 1 include adrenocortical tumors,1517 foregut carcinoid tumors,18 23 nonmedullary thyroid neoplasms (mostly follicular),24,25 and a host of unusual cutaneous mucosal or visceral abnormalities, including multiple subcutaneous and visceral lipomas, multiple facial angiofibromas, hypomelan-otic macules, gingival papules, and collagenomas.2527 Rare associations with MEN 1 also include meningiomas, ependymomas, pinealomas, renal cancers, rhabdomyosarcoma, leiomyosarcoma, and pancreatic ductal adenocarcinoma.25 28

Multiple Endocrine Neoplasia Type 1

Furthermore, growth factors have been identified in the plasma of patients with MEN 1. A circulating blood factor that was mitogenic for parathyroid cells in tissue culture has been identified,23 and a subsequent study demonstrated that the factor was similar to fibroblast growth factor.24 In addition, patients with MEN 1 are susceptible to other tumors, including bronchial, thymic, and intestinal carcinoid tumors thyroid adenomas adrenal adenomas and multiple lipomas.14 Thus, the complete pathogenesis of the multiple endocrine tumors in MEN 1 patients is not completely understood. In patients with MEN 1, primary hyperparathyroidism is the most common clinical condition, occurring in approximately 95 of individuals.14 Functional pancreatic neuroendocrine tumors are the next most common condition, occurring in approximately 80 of patients.14 Finally, approximately 35 of individuals with MEN 1 develop a pituitary adenoma, most commonly a prolactinoma.14 Gastrinoma and insulinoma are the...

Clinical Findings

This syndrome must be differentiated from an L5 radiculopathy, which may manifest similar symptoms. Neurodiagnostic studies can assist in differentiating the two entities. Electromyographic muscle sampling about and below the tarsal tunnel must be undertaken. Prolongation of distal motor and sensory latencies across the tarsal tunnel with diminished muscle amplitudes in the distribution of one or both plantar digital nerves confirms the diagnosis. Nerve entrapment may result for the presence of a space-occupying lesion (e.g., lipoma, ganglion, or neurilemoma) within the fibro-osseous tunnel.

Neoplastic Neck Masses

Benign lesions in the head and neck include lipomas and fibromas and require no treatment unless they cause significant functional or cosmetic deformity. Sebaceous cysts and epidermal inclusion cysts are also common and usually need to be excised because of the high incidence of recurrent infection.

Median Neuropathy Carpal Tunnel Syndrome

Carpal tunnel syndrome (CTS) is one of the most common mononeuropathies. It typically occurs within the confines of the carpal tunnel in the wrist. The median nerve can also be entrapped in the forearm as a pronator or interosseous syndrome. The entrapment can be caused by anything that causes a decrease in the size of the carpal tunnel (e.g., Colles' fracture, rheumatoid arthritis, congenital carpal tunnel stenosis), enlargement of the median nerve (e.g., diabetes, amyloidosis, thyroid disease, neuroma), or an increase in the volume of other structures within the carpal tunnel (e.g., tenosynovitis, ganglion, gout, urate deposits, lipoma, hematoma, fluid retention in pregnancy).

Modalities for Imaging the Adrenal Gland

MRI is increasingly used because it can reveal tissue-specific characteristics, which allows the examiner to differentiate metastases, adrenocortical carcinoma, and pheochromocytoma from adenoma, lipoma, myelolipoma, and cysts.4 Because MRI does not use ionizing radiation, it is an attractive modality for evaluating children and pregnant women.5 T1-weighted images allow relatively fast data acquisition, which may be accelerated by using paramagnetic contrast media such as acid (DTPA), resulting in a reduction of motion artifact and increasing the sensitivity for identifying adrenal lesions. T2-weighted sequences reveal characteristic signal intensities in certain conditions and help with the differential diagnosis.6 Some studies have suggested that MRI can differentiate nonfunctioning from malignant adrenal lesions,6 but because of similar characteristics of some tumors, the results are not reliable enough to use in selecting therapy.6,7

Vascular Tumors or Hemangioma

Hemangiomas of peripheral nerves are rare tumors arising from vascular endothelial cells. Histologically, hemangiomas show erythrocytes populated within thin-wall blood vessels within or surrounding the peripheral nerve.18,75,94 These tumors occur mostly in younger patients. Clinical features include tenderness, pain, and the presence of a mass along with sensory or motor neurologic deficits. The surgical treatment of these tumors is similar to that of lipomas. Local recurrence sometimes occurs.

Clinical Summary

Bartholin glands and ducts are located over the lower third of the introitus near the labia minora. A cyst or abscess can result from an obstructed duct, which usually occurs secondary to scarring from trauma, delivery, or episiotomy. Infection of the cyst is usually with mixed vaginal or fecal flora (Escherichia coli) but may also contain Neisseria gonorrhoeae and Chlamydia trachomatis. Progressive enlargement and infection lead to increasing pain, swelling, and dyspareunia. A tender, fluctuant cystic mass with surrounding labial edema is easily appreciated on examination. Epidermal inclusion cysts and sebaceous cysts of the labia majora, hidradenitis suppurativa, vulvar hematomas, leiomyomas, lipomas, and fibromas may be confused with a noninfected Bartholin cyst.

Recurrences

When performing a laparoscopic hernia repairs, it is imperative that all potential defect sites as well as cord lipomas be investigated. Failure to identify and remove a cord lipoma may let the patient believe a hernia still exists (14). A large piece of mesh is required to cover all potential sites including the direct, indirect, and femoral space. Missed lipoma

Pathology

Noninvasive imaging can reliably diagnose some soft-tissue lesions such as lipomas, benign vascular tumors, and fibromatosis. For most other tumors, histologic examination of a biopsy specimen is currently the only reliable technique that can lead to a definitive diagnosis. Several techniques are in common use fine-needle aspiration (FNA), core needle biopsy, incisional biopsy and excisional biopsy. FNA is the easiest to perform and can be safely undertaken at the first clinic visit of a patient presenting with an accessible mass that is clinically non-pulsatile. Although the tissue obtained is almost invariably inadequate for identification of tumor grade, its usefulness lies in its being able to identify other more common tumors like squamous cell carcinoma, thereby guiding further evaluation. Core needle biopsy, on the other hand, provides good tissue for diagnosis and grade can be determined in virtually all specimens.9 Lesions of the paranasal sinuses and nasal cavity may be...

Outcomes

The histologic subtype may be the most significant predictor of prognosis after treatment in cervicomedullary tumors.10 Bricola reports in his series of children and adults with cervi-comedullary tumors that 6 of 12 patients (one ependymoma, five benign astrocytomas) made excellent neurologic recoveries, although the remainder (four malignant gliomas, one angioreticuloma, and one lipoma) showed no appreciable improvement.2 In Epstein's series,7 there were 24 patients with cervicomedullary gliomas. Sixteen had grade I or II astrocytomas, and four had gangliogliomas. All 16 patients were alive

Masses

Masses are assessed by shape, margin and density. The shape may be round, oval, irregular or lobulated, and the margin (or surface) may be smooth, obscured (by surrounding tissue), indistinct or spiculated. Any of these may be hyper-, iso- or hypodense. Benign lesions tend to be round or oval and well-defined, whereas malignancies tend to be irregular in shape and outline and are often hyperdense. A low-density lesion suggests fat and is usually benign, e.g. oil cyst, lipoma, galactocoele. Lymph nodes often have a distinct appearance due to the fatty centre or hilum.

Radial Neuropathy

Radial nerve injuries are much less common than other upper extremity neuropathies. Proximally, the radial nerve is most vulnerable in the axilla, where it can be injured by hyperabduction of the arm, which puts traction on the nerve. This is the case with Saturday night palsy, when an intoxicated person sleeps with an arm draped over a chair. A similar circumstance occurs when the nerve is compressed against the humerus at the spiral groove. It is also seen with improper fit or use of crutches. Other sources of compression that can injure the radial nerve along its course include lipoma, fibroma, and new or previous (from callus) humerus fracture.

Sciatic Neuropathy

The sciatic nerve arises from the sacral portion of the plexus. It leaves the pelvis through the sciatic notch and divides into the tibial and peroneal nerves at the popliteal fossa. The sciatic nerve provides sensation to the perineum, posterior thigh, lateral calf, and foot. It innervates the thigh extensors, hamstrings, and all the muscles of the lower leg and foot. Pain, weakness, and sensory changes caused by injury of the sciatic nerve or one of its two branches can be caused by trauma from gunshots, hip fracture or dislocation, compression from surgery or prolonged sitting on a hard edge, tumor, endometriosis, lipoma, aneurysm of the gluteal artery, or improper intramuscular injection into the gluteus. Symptoms of sciatic nerve compression can mimic L5-S1 radiculopathy. Again, EMG is helpful in these clinical situations.

Tinea Cruris Cura

Fungal Infection Gluteal Cleft

Figure 8-41 Lipoma of the back. A lipoma is a benign growth of subcutaneous fat and has a rubbery appearance. The epidermis is normal. Frequently, an encapsulated lipoma may grow to a very large size and elevate the overlying dermis and epidermis, as shown in Figure 8-41 a cross section through a lipoma is illustrated in Figure 8-42. The examiner can easily push into the soft tissue tumor. Another example of a lipoma is shown on the arm of the patient in Figure 8-43. Figure 8-42 Cross section through a lipoma. Figure 8-43 Lipoma of the arm. Figure 8-42 Cross section through a lipoma. Figure 8-43 Lipoma of the arm.

Inspect the Tongue

White Tongue Normal Variant

Inspect the mucosa, and note any masses or ulceration. Is the tongue moist Ask the patient to stick out the tongue. A neuromuscular weakness may be present if the tongue cannot protrude in the midline or move rapidly in all directions. Are there any mass lesions on the sides or undersurface of the tongue Ask the patient to lift the tongue to the roof of the mouth so that the inferior aspect of the tongue can be inspected. In older individuals, the large veins on the ventral aspect of the tongue may be tortuous. These varicosities never bleed spontaneously and have no clinical significance. Figure 12-23 shows a patient with sublingual varices. Figure 12-24 shows a patient with a benign lipoma of the tongue. Figure 12-24 Benign lipoma of the tongue. Figure 12-24 Benign lipoma of the tongue.

Neural Tube Defects

Defined as a defect in the posterior bony components of the vertebral column without involvement of the cord or meninges. These defects are often found incidentally on radiographic studies or are picked up because of a subtle clinical finding such as a tuft of hair or a cutaneous angioma or lipoma in the midline of the back marking the location of the defect. On rare occasions, a sinus tract may communicate from the skin to the underlying dura. The clinical presentation is largely dependent on the level and content of the defect. Pure meningoceles may be asymptomatic. Neurological disability is greatest in patients with myelomeningoceles. Infants with defects at or above L2 are more likely to have skeletal deformities, including kyphosis and scoliosis, dislocated hips, and clubfeet (see later). The degree of motor paresis is equally dependent on the level of the neural tube closure defect and is discussed in more detail under management and prognosis. Involvement of the kidneys,...

Grade Description

Imaging of spinal abnormalities is useful in children up to 3 to 6 months of age as the posterior elements are membranous not bony. Beyond this age, these elements calcify and generally US would then need to be complemented with another imaging modality. Early evaluation and differentiation of neural tube defects, such as lipomas, meningoceles, myelomeningoceles, and tethered spinal cord, is possible. US can evaluate the subcutaneous structures and allow for recognition of abnormal spinal canal development, including spinal cord abnormalities. Even in older children, the presence of abnormal skull dimples, pores, or hair tufts can be evaluated for underlying spinal dysraphism. Rarely, US can be useful in evaluating spinal neoplasm or syrinx development.

Therapeutic Options

Therapeutic considerations for patients with intracranial lipomas must be made within a risk-benefit analysis. Although there are limited data on the treatment of intracranial lipomas, surgical resection versus observation alone has been the standard of care. There are no reports of attempts to treat intra-cranial lipomas with radiation or chemotherapy. Because intracranial lipomas are benign entities whose contribution to the patient's underlying symptomatology is often unclear, the indications for surgical removal must be strong. Although there are case reports of intracranial lipomas increasing in size and causing symptom progression,19,23 this appears to be the exception rather than the rule. Intracranial lipomas are congenital lesions and hence the associated malformations, often the source of the actual symptoms, occur early in development and are not amenable to treatment. In general, surgical resection of intracranial lipomas should be the last resort. Hydrocephalus, if...

Preoperative Tests

The principal differential diagnoses of MPNST on imaging are soft tissue sarcoma, neurofibroma, and schwannoma. The rare lipomas and ganglion cysts that can mimic a peripheral nerve tumor are quite readily distinguished by their signal characteristics and association with a joint capsule, respectively, on MRI (see Chapter 73).

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