Deforming lipoblastomatosis of the lower extremity.

OBJECTIVE: Lipoblastomatosis is a rare disorder in infants and children and nonexistent in adults. We discuss a case of a newborn child with lipoblastomatosis extensively involving the pelvis and lower extremities. The clinical and radiological characteristics of the disorder are discussed. CLINICAL FEATURES: A 2-month-old male had an enlarging deformity of the pelvis and lower extremities with progression of the condition from birth. There was no family history related to the disease. No other clinical abnormalities were present. INTERVENTION AND OUTCOME: Lipoblastomatosis is initially treated with surgical excision of the lipomatous neoplastic tissue. The postsurgical outcome is satisfactory; however, the likelihood of recurrence has been reported. CONCLUSION: We present the features of lipoblastomatosis, an uncommon disorder affecting infants and children. The clinical and radiologic manifestations of the disease are assessed with emphasis on magnetic resonance imaging.

[Pelvic lipomatosis. A case with ureteral and venous obstruction]. / Lipomatose pelvienne. A propos d'un cas avec obstruction urétérale et veineuse.

The authors report a case of pelvic lipomatosis in a 62 year old man associated with venous obstruction (third case reported in the literature), diagnosed after bilateral ureteric obstruction, and review of the literature on this subject. The diagnosis was suggested by the radiological triad of hyperlucency of the pelvis on plain abdominal X-ray, "hot air balloon" appearance of the bladder on IVU and a rigid and ascended rectosigmoid on barium enema, and was confirmed by CT and MRI. Treatment combining corticosteroids and urinary tract disinfection was partially effective. A double J ureteric stent resolved the problem of persistent right ureteric obstruction.

Lipomatosis and true lipomas of the ileocecal valve.

Two patients with lipomatosis and two patients with true lipoma of the ileocecal valve are described. The symptomatology, radiological appearances, pathological findings and treatment are discussed. True lipomas of the ileocecal valve should be differentiated from the more commonly occurring lipomatosis or lipohyperplasia. The rare true lipomas have a demarcating capsule around the fatty tissue and are confined to only one of the ileocecal lips. The lipomatosis, on the other hand, is characterized by a diffus fatty deposition in the submucosa of the valve with no encapsulation. Lipomatosis as well as true lipomas may appear as rounded, smoothly outlined and sharply demarcated masses on barium enema examination. Occasionally, these filling defects may present problems of differential diagnosis with respect to malignant involvement of the ileocecal region. A correct preoperative diagnosis, however, is important to prevent unnecessary or too radical surgical treatment.

[The enlarged retrorectal space--indicative of specific pathology or only unspecific symptom of disease in the true pelvis? (author's transl)]. / Der erweiterte Retrorectalraum--ein sicher pathologisches oder nur unspezifisches Zeichen einer Erkrankung im kleinen Becken?

The retrorectal space was measured on the lateral view of the rectum during double contrast barium enema examination in 300 patients in whom no abnormality of the rectum, sacrum or in the true pelvis was demonstrated clinically or radiographically. The measurement obtained is the distance between posterior wall of the rectum and the anterior surface of the sacrum between S2 and S5. The measurements at S5 are considered most accurate, since at this level the rectum will be positively in midline. The normal width at S5 is between 0.2 and 1.5 cm. In connection with other symptoms, a distance between 1.5 and 2 cm should be regarded as suspicious and above 1 cm as definitely abnormal, as was demonstrated in 95 cases with diffuse and localized enlargement of the retrorectal space. An abnormal measurement of greater than 2 cm however is an unspecific symptom and should not by itself be considered indicative of specific pathology, but rather should lend supporting evidence to other abnormal findings in the true pelvis.

Pelvic lipomatosis: diagnosis by computerized tomography scan.

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.

Multiple lipomas of the ileum with volvulus.

We describe a patient with symptomatic multiple lipomas with volvulus. He complained of gradually increased abdominal pain and distention. Preoperative roentgenograms showed many rounded filling defects of the small intestine. Operation was performed to reduce intestinal obstruction and ileal resection was performed. The dilated ileum was rotated 720 degree clockwise and it contained 115 submucosal and 26 subserosal lipomas. Histologically, the lipomas were benign and consisted of well-developed fat tissue. The patient's postoperative course was satisfactory, and neither residual lipoma nor recurrence were found at barium enema examination two years after operation.

Pelvic lipomatosis.

Three cases of pelvic lipomatosis are presented. Excretory urogram revealed characteristic elevation and elongation of urinary bladder base and relative hyperlucency of pelvic cavity. Associated varying degrees of hydronephrosis and hydroureters are seen secondary to distal ureteral obstruction. Barium enema showed elongation and elevation of rectosigmoid colon.