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Followup of 5 cases presented in 1971 and report of 8 additional cases confirm the initial conclusions that pelvic lipomatosis is a benign but potentially progressive process leading to ureteral obstruction. Young black subjects are affected most commonly. More than 40 per cent of the patients have required permanent urinary diversion.
Inverted teardrop-shaped bladder deformity ofetn is caused by pelvic lipomatosis or perivesical accumulation of extravasated blood or urine, or both. A foreign body (toothpick) lodged in the bladder and resulting in urinary infection and perivesical inflammation also was found to be capable of causing this unusual bladder deformity. The bladder contour returned to normal after removal of the foreign body and treatment of infection.
BACKGROUND: Epidural lipomatosis can be idiopathic or associated with obesity and steroid excess. This disorder can be treated by weight control, reduction of steroid load, or operative decompression. We describe a non-obese patient with neurogenic claudication who had localized epidural fat from L4 to S1. Minimally invasive treatment consisted of a small laminotomy and endoscopically guided fat aspiration. METHODS: A small bilateral L5-S1 laminotomy was performed. Using a special malleable endoscopic aspirator, loose fat was removed both dorsal and ventral to the dural sac. RESULTS: At surgery, endoscopically guided suction aspiration was successful in removing the majority of the fat. Areas of fat that were vascularized and adherent to the dura required microsurgical dissection. Postoperatively, the patient has been asymptomatic. CONCLUSIONS: This minimally invasive technique was successful in treating epidural dual compression that had failed conservative measures. Further evaluation of this technology is warranted.
Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital disorder and was first described in 1970. The main clinical features of the syndrome include convulsions beginning in infancy, mental retardation, and unilateral cutaneous and ophthalmologic lesions with ipsilateral cerebral manifestations. A 14-year-old caucasian boy with ECCL associated with otolaryngologic manifestations is reported. To our knowledge, this is the first case of ECCL with otolaryngologic manifestation in the English literature.
First described by the end of the fifties, pelvic lipomatosis is an uncommon disease that develops as a result of an excessive proliferation of benign fat tissue within the perivesical and perirectal spaces. The compressive effect on the urinary, and to a lesser degree, the digestive and vascular structures result in the well-known symptoms. Diagnosis is reached through X-ray studies, primarily computerised tomography. Contribution of four new cases in young males diagnosed through imaging studies as well as biopsies in three of them. Evolution has been varying, with medical control of symptoms in two cases and renal function impairment due to upper obstructive uropathy in the other two.
We report the unusual case of a 48 year old woman with jejunal lipomatosis complicated by intestinal intussusception and volvulus. Showing fatty masses CT allowed accurate diagnosis.
Multiple symmetrical lipomatosis (Madelung's disease) is a rare disease with multiple symmetrical unencapsulated fatty accumulation diffusely involving the neck, the shoulders and the upper extremities (Kohan et al. Otolaryngol. Head Neck Surg. 1993;108:156-159). We describe a 48-year-old Japanese man with a history of alcoholism and liver cirrhosis who reported gradually enlarging masses in his cervical region for 4 years. MRI revealed large masses suggesting lipomas in the neck. The patient underwent a two-stage lipectomy. This patient is the 13th case reported in Japan since 1978, though over 200 cases have been reported since 1846 in Europe, most of them from the Mediterranean (Kitano et al. ORL 1994;56:177 180; Kaku et al. Endocrinol. Diabetol. 1997;4:103-106).
A 9-year-old boy with a history of lipoma excision and laminectomy at the Th10-11 levels, resulting in incomplete paraparesis and neurogenic bladder, was admitted for a comprehensive rehabilitation programme. Physical examination revealed an ipsilateral focal dermal hypoplastic defect within an area of alopecia and a subcutaneous lipomatous tissue on the left temporo-parietal region of the scalp. Iris coloboma and chorioretinitis were diagnosed on the left eye. He also had mild mental retardation and triparesis. Magnetic resonance imaging of the brain and the spine demonstrated hyperintense masses which were consistent with lipoma. Although in the literature three cases of encephalocraniocutaneous lipomatosis (ECCL) concomitant with spinal cord involvement have been reported, to our knowledge iris coloboma and chorioretinitis in ECCL have not been reported previously. In conclusion, we would like to stress that aside from known ophthalmological malformations, iris coloboma and chorioretinitis may also be observed in ECCL and that all patients who have been diagnosed as having ECCL should be examined for spinal cord involvement.
Congenital infiltrating lipomatosis of the face (CIL-F) is a rare lesion. Presented here is a review of the literature concerning this condition, with the report of a case with clinical signs indistinguishable from those of unilateral facial hyperplasia. Previously unreported clinical and radiographic features of regional bony hypertrophy and macrodontia associated with this case of CIL-F are described and the proposition raised that previously reported cases of unilateral facial hyperplasia may have been due to CIL-F. It is suggested that the clinical findings in this case of facial asymmetry associated with CIL-F, regional macrodontia and regional bony enlargement may constitute a previously undescribed syndrome.
Here, we report pathogenetic aspects of spinal epidural lipomatosis (SEL) based on a literature review. SEL is a rare entity but can cause significant morbidity. Its symptoms can be identical to those of more common disorders such as vertebral and disc disease, and cord lesions (for example, transverse myelitis, multiple sclerosis and syringomyelia). Therefore, it often goes undiagnosed. In addition, SEL occurs in patients on glucocorticoid therapy, which can lead to myopathy, thereby mimicking the motor symptoms of SEL. Glucocorticoids seem to play a major role in the development of SEL, although idiopathic SEL has also been reported. The latter occurs almost exclusively in obese individuals who may have concurrent hypercortisolism. Once clinically suspected, SEL is best diagnosed by magnetic resonance imaging (MRI). Treatment of SEL is directed at reducing body weight in patients with idiopathic SEL, and at decreasing glucocorticoid excess in patients with endogenous or exogenous hypercortisolism. In severe cases, decompressive laminectomy might become necessary to alleviate the neurological symptoms caused by spinal cord compression.
Lipomatosis of the tongue is an extremely rare condition. To date, only 5 cases have been reported in the literature. An additional example, confirmed by biopsy, is presented. This appears to represent only the second instance of a nonoriental person who has been described with this condition.
Multiple symmetric lipomatosis (Madelung's disease) is a rare disease characterized by non-encapsulated adipose deposits in the neck, the superior part of the trunk and, very rarely, in the limbs. It is common in middle-aged Caucasian Mediterranean males but very rare in Chinese people. We describe two cases of Madelung's disease: both patients presented complaining of a development of a painless neck mass that was increasing in size. Panendoscopy was normal. Both underwent incisional biopsies that revealed benign adipose tissue characterizing Madelung's disease. We discuss the clinical presentation, associated morbidity, and treatments for Madelung's disease.
A 21-year-old woman receiving prolonged high dosage glucocorticoids developed spinal cord compression due to excessive accumulation of epidural fat. Computerized tomographic scanning confirmed the diagnosis and revealed a peculiar pattern of spinal cord displacement which we believe to be unique to compression by fat. Laminectomy did not afford relief, possibly because of prolonged neural compression or because of compression at a higher spinal level. Although an unusual complication of Cushing's syndrome, epidural lipomatosis should be considered when such a patient develops symptoms of spinal cord or cauda equina compression.
The benign symmetric lipomatosis (Madelung's disease) is frequently associated with alcoholism. After 150 years of history, many questions about its physiopathological mechanism are still unresolved. Presentation of a patient with this rare condition is reported, followed by a review of the literature, where the pathogenesis, the differential diagnosis and the surgical approach of this syndrome are especially pointed out.
Cowden's syndrome is either familial or sporadic and is associated with the predominantly postpubertal development of a variety of cutaneous, stromal and visceral neoplasms. The syndrome is associated with mutations of the PTEN gene and is closely related to Bannayan's syndrome in which macrocephaly and benign tumors, especially lipomas and hemangiomas are pathognomic. In PTEN knockout mice testicular tumors have been reported and for this reason we felt it prudent to examine the testes of our patients with genetically proven Cowden's syndrome. Seven of eight patients who underwent testicular ultrasound were found to have diffuse bilateral hyperechoic lesions. Four patients consented to testicular biopsy and on histological examination multiple foci of adipocytes were found within the testicular interstitium, with no evidence of dysplasia or preclinical malignancy. Immunohistochemical assessment of adipocytes suggested a stromal derivation without evidence of metaplasia from Leydig cells. In one case there was focal atrophy of seminiferous tubules, while in two others there was nodular periorchitis of the tunica albuginea. Biochemical evaluation of testicular function (luteinizing hormone, follicle-stimulating hormone, testosterone, sex hormone binding globulin and free androgen index), prostate-specific antigen and testicular tumor markers were normal, while seminal fluid analysis showed only minor abnormalities. The high incidence of testicular lipomatosis in our adult subjects suggests this to be an important diagnostic criterion for Cowden's syndrome.
BACKGROUND: Multiple symmetric lipomatosis (MSL) is a rare disease characterised by the growth of uncapsulated masses of adipose tissue. MSL is associated with high ethanol intake and complicated by somatic and autonomic neuropathy and the infiltration of adipose tissue at the mediastinal level. To date, the disease is considered as largely more prevalent in men. OBJECTIVE: To provide a detailed description of the clinical aspects of MSL in women. PATIENTS: A total of 11 women and 58 men with MSL. MEASUREMENTS: Morphological aspect of patient, location of the lipomatous masses, alcohol intake, extension of lipomatous tissue to the mediastinum, association with somatic and autonomic neuropathy, and metabolic profile. RESULTS: All female patients had the obesity-like appearance of type II MSL and the most frequent locations of lipomatous masses in women were at the proximal arms (90.9%) and legs (54.5%). Very few female patients (27.3%) presented with the submental deposition of lipomatous tissue typical of both type I (97.3%; P<0.001) and type II (66.7%; P<0.05) male subjects. An extension of the lipomatus tissue around the upper airways, associated with compression or dislocation of deeply located mediastinal structures, was observed less frequently in women than in men. The presence of a high ethanol intake, the association with somatic and autonomic neuropathy and the occurrence of a characteristic metabolic pattern (high HDL-cholesterol, low LDL-cholesterol, high uric acid) were similar in men and in women. CONCLUSIONS: Female patients with MSL had a sex-specific morphological aspect, characterised by a low occurrence of the typical 'Madelung collar' and a usual obesity-like appearance.
Encephalocraniocutaneous lipomatosis, or Haberland syndrome, is a rare congenital neurocutaneous disease. It is characterized clinically by unilateral lipomatous hamartomata of the scalp, eyelid, and outer globe of the eye, ipsilateral porencephalic cysts with cortical atrophy, cranial asymmetry, marked developmental delay and mental retardation. This syndrome should be distinguished from other mosaic neurocutaneous phenotypes such as as Delleman syndrome, Schimmelpenning syndrome, Goltz syndrome, Goldenhar syndrome and Proteus syndrome. Here we report a case of Haberland syndrome with bilateral involvement which underscores the extreme heterogeneity of clinical presentation of this and related syndromes.
BACKGROUND: Multiple symmetric lipomatosis (MSL) is a rare disease characterized by enlarging, symmetric, nonencapsulated, fat deposits mainly on the neck and upper trunk. Liposuction and lipectomy, although palliative, are the treatments of choice, especially indicated when vital structures are compromised. OBJECTIVE: Our purpose was to evaluate the efficacy and safety of liposuction and lipectomy in the treatment of MSL. METHODS: We have examined two patients diagnosed with MSL who presented with symptoms derived from the compression of vascular, nervous, and/or respiratory tract structures. One was treated with lipectomy and the other with liposuction. RESULTS: A rapid resolution of the clinical symptoms was achieved with both therapies. The patient who was treated with lipectomy suffered from a compression of the left brachial plexus by scar tissue as an adverse effect, requiring a second surgical procedure. Liposuction only provoked a mild autoinvolutive hematoma in the other case. No clinical recurrences were observed at 3 and 2 years of follow-up respectively. CONCLUSIONS: We consider both lipectomy and liposuction as safe and effective techniques for the treatment of MSL patients. Although liposuction is usually associated with less adverse effects than lipectomy, location of the lipomas must be carefully considered before choosing one technique over another.