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Epidural lipomatosis can be induced by systemic glucocorticoid treatment and may cause severe neurologic deficits. So far, 41 patients have been reported in the literature. Early recognition of this rare, relatively unknown, but extremely important side effect of exogenously administered glucocorticoids can help avoid persistent neurological sequelae.
Benign symmetric lipomatosis of the pseudoathletic type was identified in a woman with a positive family history for the disorder and a past history of alcohol abuse. She had an exceptionally high number of additional diseases such as arthropathy with degenerative osteoporosis, hyperuricemia, hyperlipidemia, psoriasis, neuropathy, muscular atrophy, arteriosclerosis and increased cardiovascular risk factors.
Epidural lipomatosis is a rare but severe complication of long-term corticoid treatment. A female who was treated with oral corticoids for more than 4 years developed progressive paraparesis over the course of 2 years. As causal for the clinical symptoms we found a massive epidural lipoma of the thoracic spine. Neurosurgical intervention was necessary.
A diffuse fatty infiltration of all the intrapelvic organs (bladder, rectum, sigmoid, colon) was present in a 67-year old-man. This led to the typical topographical and functional changes in the organs affected. Treatment has been very controversial. Surgical measures such as exeresis of the fat tissue with or without the intrapelvic organs affected by the abnormal fat deposition with subsequent drainage (colostomy/ileum conduit) were carried out. Conservative treatment with or without temporary cortisone administration was recommended. Pelvic lipomatosis is diagnosed radiologically. If there is no unequivocal restriction in the function of the affected organs, treatment should remain conservative.
We present a case of epidural lipomatosis in a non-obese patient with no history of steroid use. On MRI, the lipomatous mass had an unusual appearance and was centered over thoracic vertebrae with Paget's disease. Decompressive laminectomy was effective in promoting resolution of the neurological deficits.
An extremely rare case of congenital infiltrating lipomatosis of the face (CILF) associated with ipsilateral hemimegalencephaly is reported in a 3-month-old boy. MRI not only thoroughly evaluated the extent of the lesion, but also demonstrated the cerebral anomaly. MRI is indispensable in the evaluation of patients suspected of having CILF.
We report a case of symptomatic epidural lipomatosis in an 8-year-old girl with Cushing's syndrome secondary to longstanding high-dose steroid therapy for Crohn's disease. MR imaging of the spine revealed massive diffuse epidural fat compressing the entire spinal cord with T2 prolongation in the central gray matter of the cord suggesting ischemic myelopathy. This finding has not been previously demonstrated on imaging. A proposed mechanism underlying these findings is discussed.
Benign symmetric lipomatosis is a rare form of typical fat distribution in the shoulders, the arms, and the neck that can compromise the respiratory, nutritional, and psychological status of the patient. Alcoholism, malignant tumors of the upper airways, endocrine tumors, hypothyroidism, diabetes, and hypertriglyceridemia are often associated with its occurrence. Surgical removal via lipectomy or liposuction can give good cosmetic results, although recurrences often occur. Liposuction has become the first choice to treat this disorder in patients with smaller masses. We have operated on four such cases in which liposuction failed and surgical excision had to be performed. We present a summary of the clinical characteristics of all four patients and discuss the different treatment options.
Lumbosacral epidural lipomatosis (LEL) is characterized by excessive deposition of epidural fat (EF). The purpose of our retrospective study was to quantify normal and pathologic amounts of EF in order to develop a reproducible MRI grading of LEL. In this study of 2528 patients (1095 men and 1433 women; age range 18-84 years, mean age 47.3 years) we performed a retrospective analysis of MRI exams. We obtained four linear measurements at the axial plane parallel and tangent to the superior end plate of S1 vertebral body: antero-posterior diameter of dural sac (A-Pd DuS), A-Pd of EF, located ventrally and dorsally to the DuS, and A-Pd of the spinal canal (Spi C). We calculated (a) DuS/EF index and (b) EF/Spi C index. We developed the following MRI grading of LEL: normal, grade 0: DuS/EF index >or=1.5, EF/Spi C index or=75% (severe EF overgrowth). The MRI exams were evaluated independently by three readers. Intra- and interobserver reliabilities were obtained by calculating Kappa statistics. The MRI grading showed the following distribution: grade 0, 2003 patients (79.2%); LEL grade I, 308 patients (12.2%); LEL grade II, 165 patients (6.5%); and LEL grade III, 52 patients (2.1%). The kappa coefficients for intra- and interobserver agreement in a four-grade classification system were substantial to excellent: intraobserver, kappa range 0.79 [95% confidence interval (CI), 0.65-0.93] to 0.82 (95% CI, 0.70-0.95); interobserver, kappa range 0.76 (95% CI, 0.62-0.91) to 0.85 (95% CI, 0.73-0.97). In LEL grade I, there were no symptomatic cases due to fat hypertrophy. LEL grade II was symptomatic in only 24 cases (14.5%). In LEL grade III, all cases were symptomatic. A subgroup of 22 patients (42.3%) showed other substantial spinal pathologies (e.g., disk herniation). By means of simple reproducible measurements and indexes MRI grading enables a distinction between mild, moderate, and severe EF hypertrophy. Kappa statistics indicate that LEL can be reliably classified into a four-grade system by experienced observers.
Multiple symmetrical lipomatosis is a rare disorder characterized by progressive anomalous deposition of fat typically located in the neck and shoulders. Magnetic resonance imaging allows exact definition of the abnormal fatty tissue and the involvement of deep structures. We describe the MR findings in two patients with the typical fat deposition in the neck and upper thorax which also presented unusual location of abnormal fat. One patient had laryngeal involvement with fatty infiltration of true and false cords. The other patient had inguinal and scrotal large deposition of fat.
Using lymphocytes from nine unrelated patients with multiple symmetric lipomatosis we investigated a possible defect in the mitochondrial respiratory chain as the biochemical cause for the disease. A significant decrease in oxygen consumption of intact lymphocytes as well as a decreased activity of the individual components of the respiratory chain were detected. These findings are consistent with the recently described deletions and point mutations of mitochondrial DNA in patients suffering from this disease.
Spinal epidural lipomatosis (SEL) in patients on steroid therapy may be explained by two hypotheses: (1) steroids induce SEL and (2) steroids cause the growth of a pre-existing SEL, especially in obese children. Steroid-induced SEL (SSEL) is rarely described in children, with only six cases reported elsewhere. However, we have already reported four nephrotic children with SSEL, including one child in another hospital, in addition to the six cases. We investigated the frequency of SSEL in 125 children with renal diseases treated with glucocorticoids in a single hospital over 16 years, and examined the risk factors in 62 patients with SSEL reported in the literature. When patients complained of symptoms at the early stage of SSEL, i.e., back pain or numbness, and patients were obese, we performed spinal magnetic resonance imaging (MRI). SSEL was detected in 5 of 125 children (4.0%). Of the 5 patients with SSEL, 3 had vertebral compression fractures, and all 5 patients were on methylprednisolone pulse therapy. Our study suggests that many more patients can be detected at the early stage of SSEL by performing spinal MRI for patients with early symptoms, obesity, and those who have received methylprednisolone pulse therapy.
BACKGROUND: Encephalocraniocutaneous lipomatosis (ECCL) is a relatively new, nonhereditary, but congenital, neurocutaneous syndrome with unilateral cutaneous tumors and ipsilateral ophthalmologic and neurologic malformations. The syndrome is rare, with only 25 cases reported since first communication in 1970. The primary clinical features noted for almost all cases are as follows: (1) unilateral porencephalic cysts with cortical atrophy, (2) ipsilateral lipomatous hamartoma of the scalp, eyelids, and outer globe of the eye, (3) cranial asymmetry, (4) marked developmental delay and mental retardation, (5) seizures, and (6) spasticity of the contralateral limbs. OBJECTIVE: We discuss underlying pathophysiology, diagnostic difficulties, differential diagnosis, and therapeutic possibilities of the syndrome. CONCLUSIONS: The syndrome seems to be more frequent than it was thought. ECCL may remain unrecognized, as some patients may not represent the full clinical spectrum of the disease. Periodic neurologic and cardiologic assessment with echocardiography and electrocardiography should be carried out in all patients with ECCL because of a possible progressive disease course.
Being rare tumors of the heart, cardiac lipomas are usually discovered incidentally during non-cardiac-related examinations of the chest. Although they are reported to be typically solitary, multiplicity has been described in tuberosclerosis patients. Here we reported the multidedector-row computer tomography (MDCT) findings of a nontuberosclerosis case with multiple cardiac lipomas along with pericardial lipomatosis, who presented with symptoms of left heart failure after a hysterectomy surgery but otherwise healthy before that operation.
Pelvic lipomatosis is a rare, relatively self-limiting disease characterized by the overgrowth of unencapsulated lipomatous tissue within the pelvis. The diagnosis is suggested, but not substantiated, by the striking roentgenographic changes noted on barium enema and intravenous pyelogram. Previously, pelvic laparotomy with tissue diagnosis was essential for documenting the disease. We report three cases in which computerized tomography was utilized as a safe, noninvasive and accurate method of diagnosis. The role played by partial venous obstruction is discussed in addition to rectal bleeding as a mode of presentation.
A case of congenital infiltrating lipomatosis of the face is presented, associated with a congenital cytomegalovirus infection.
A rare association of sebaceous naevus of Jadassohn and primary mediastinal lipomatosis is presented. These are two benign conditions that can have serious complications.
An extreme form of hyperalphalipoproteinemia was studied in a patient affected by multiple symmetric lipomatosis (MSL); four relatives and three MSL controls were also evaluated. Plasma lipids and apolipoproteins were measured and overall lipoprotein profile was assessed by density gradient ultracentrifugation. The patient showed a plasma HDL-cholesterol of 138 mg/dl and an apo A-I of 218 mg/dl; moreover significantly high HDL levels were found in two unaffected relatives. The hypobetalipoproteinemia trait was also found both in the patient and in one of his daughters. We suggest that some pre-existing conditions may enhance lipoprotein metabolism alterations in this lipid storage disease.