Pelvic lipomatosis

Pelvic lipomatosis

Pelvic Lipomatosis Diagnosis by Computerized Tomography Scan E. DAVID CRAWFORD, MD Los Angeles, California IGOR DUMBADZE, M.D. DAVID L. KATZ, M.D. ...

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Pelvic Lipomatosis Diagnosis by Computerized Tomography Scan

E. DAVID CRAWFORD,

MD

Los Angeles, California IGOR DUMBADZE, M.D. DAVID L. KATZ, M.D. JOHN W. VESTER, M.D. Cincinnati. Ohio

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 addltion to rectal bleeding as a mode of presentation. The clinical and roentgenologic constellation known as pelvic lipomatosis was first reported in 1959 by Engels [ 11, who described the original symptom complex as occurring in middle-aged men and consisting of urinary frequency, backache and episodic suprapubic pain. Roentgenographic deformity of the rectosigmoid colon and urinary bladder, plus the clinical findings of low grade fever, hematuria, pyuria and occasional rectal bleeding, was also mentioned. Fogg and Smyth [2] subsequently reported five additional cases in which they coined the term “pelvic lipomatosis.” Becker et al. [3] extended the age range from nine to 80 years, and Bender and Kass [4] discussed a case in which a woman had this condition. Abbott and Skinner [5] demonstrated interference with the deep venous return in the pelvis, offering this as an explanation for the 10 per cent postoperative incidence of thrombophlebitis in these patients. Since 1959, more than 60 cases have been reported in the English-language literature on this subject. This report describes three patients with this condition who were treated during the past three years at the Good Samaritan Hospital in Cincinnati. Special emphasis was given to (1) the use of the computerized tomography (CT) scan as a noninvasive technic for establishing the diagnosis; (2) the important role played by partial compression of the pelvic venous system in the disorder, and (3) hemorrhoidal varices with bleeding as presenting symptoms.

From the Department of Surgery, Division of Urology,Universityof New Mexico,Albuquerque, New Mexico and the Departments of Internal Medicine,Surgery,and Radiology,GoodSamaritan Hospital, Cincinnati, Ohio 45220. Requestsfor reprints should be addressed to Dr. E. David Crawford, University of New Mexico, Divisionof Urology,2211 Lomas Blvd., N.E., Albuquerque, New Mexico 87131. ManuscriptacceptedMay 2, 1978.

CASE REPORTS Case 1. A 52 year old black man was admitted for elective hemorrhoidectomy to correct rectal bleeding which had persisted for four years. His past history revealed long-standing, moderate hypertension in addition to hyperuricemia and alcohol abuse. Physical examination showed a blood pressure of 160/100 mm Hg, weight 88 kg (190 pounds) and height 168 cm (67 inches). Barium enema and intravenous pyelogram suggested a large mass lesion interposed between the bladder and the rectosigmoid that produced a “pear-shaped” bladder deformity and straightened and elongated

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the rectosigmoid (Figure 1). Difficulty with cystoscopy was experienced because the prostatic urethra was elongated and the bladder base was elevated. Median lobe prostatic enlargement, small bilateral ureteroceles and cystitis glandularis involving the bladder base were observed cystostopically. Sigmoidoscopy disclosed external and internal hemorrhoidal varices. When hemorrhoidectomy and pelvic laparotomy were performed, the pelvis was found to be filled with adipose tissue, biopsy specimens of which showed benign lipomatous tissue (Figure 2). The postoperative course was uneventful and although the patient has since been clinically well, a CT scan two years later revealed encasement of the pelvic viscera by tissue with the attenuation coefficient of fat (Figure 3).

Figure 1. Case 1. Barium enema following excretory urogram. Single arrow demonstrates an elongated vertically rising rectosigmoid. Double arrow shows anterior displacement of the bladder.

Figure-2.

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Case 2. A 51 year old black man, admitted for evaluation of pain in the right flank and right lower quadrant, had an eight year history of mild hypertension and a five year history of hemochezia due to hemorrhoids. On physical examination his blood pressure was 158/90 mm Hg, weight 90.8 kg (200 pounds) and height 169 cm (68 inches). An emergency drip infusion pyelogram showed a 6 mm partially obstructing right distal ureteral calculus and a larger left renal pelvic calculus. Moreover, straightening and elongation of the distal ureters along with a “pear-shaped” bladder deformity were noted. Right ureterolithotomy was performed via a Gibson incision using an extraperitoneal approach. The operative report made no mention of excessive pelvic fat, and the diagnosis was made by retrospective review of the roentgenograms. A stormy postoperative course was punctuated by chest pain on the eighth postoperative day with the gstechnetium lung scan showing bilateral pulmonary emboli. Phlebography of the lower extremities revealed extrinsic compression of both

Case 1. Biopsy specimen reveals normal adipose tissue.

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Figure 3. Case 1. CTscan demonstrating encasement of the bladder (single arrow) and rectosigmoid by homogeneous material with the attentuation coefficient of fat. Iliac vessels (double arrows)

external iliac veins without any definite filling defects (Figure

4). Six weeks later pulmonary emboli recurred, necessitating the insertion of a vena caval umbrella. Still later, right ilial femoral phlebitis supervened for which the patient was treated during subsequent hospitalization. CT scan performed one year later disclosed bladder and rectosigmoid encasement in tissue with the attenuation coefficient of fat (Figure 5). On the basis of his chronic venous insufficiency, the patient is currently on full disability. Case 3. Admitted for evaluation of rectal bleeding of three years’ duration, a 55 year old black man described nocturia three times since childhood and a recent onset of hypertension. Physical examination showed a blood pressure 160194 mm Hg, height 181 cm (69 inches) and weight 113 kg (230 pounds). A barium enema indicated relative pelvic lucency in association with a fusiform narrowing of the rectosigmoid (Figure 6). A cystogram revealed a “pear-shaped” bladder (Figure 7). Sigmoidoscopy was negative except for the presence of external hemorrhoidal varices. CT scan performed two years later showed anterior displacement of the bladder with encasement of the rectosigmoid and bladder; the tissue found by CT scanning was adipose (Figure 8).

COMMENTS Pelvic lipomatosis is a rare, usually self-limited condition, characterized by pelvic overgrowth of mature, unencapsulated lipomatous tissue producing striking roentgenographic changes in the appearance of the pelvic viscera. Plain abdominal films reveal an in-

Figure 4. Case 2. Phlebogram consistent with external compression of both external iliac veins (arrows).

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Figure 5. Case 2. CT scan; single arrow represents area of fat anterior to the bladder. Prostate and seminal vesicles (double arrows).

Figure 6. Case 3. Barium enema showing narrowing of the rectum and distal sigmoid colon.

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creased radiolucency of the pelvis due to fat. On barium enema, the rectum is narrowed, and the sigmoid colon is straightened and elevated but not rigid or fixed. Static cystogram reveals a bladder silhouette that is “pearshaped” or “inverted tear drop” in configuration. There is elevation of the trigone and bladder base with elongation of the posterior urethra which may produce mechanical difficulties in performing cystoscopy as in Case 1. Cystitis glandularis, a premalignant disorder [6], has been reported to occur in 75 to 80 per cent of patients with pelvic lipomatosis, and it was present in Case 1

[71. Symptoms may include rectal bleeding, although gastrointestinal complaints are usually mild or absent. Mild constipation may occur. All three of our patients had rectal bleeding, and two of the three were admitted for evaluation of this condition. Engels [l] mentioned rectal bleeding in his original report; however, this fact has not been emphasized in the recent literature. The impairment of pelvic venous return produces increased collateral flow in the hemorrhoidal veins and pelvic venous hypertension. We suggest this is a possible pathophysiologic explanation for the association with hemorrhoidal disease. On sigmoidoscopy, mucosal changes are absent, although there may be some narrowing of the lumen and straightening of the valves of Houston [2]. Voiding difficulties are rare [ 1,3,8,9]. Ureteral obstruction occurred in 17 per cent of the

4-

Figure displacement

1 clearly of a “pear-shaped”

elineatir

bladder.

previously reported cases, and it may give rise to back pain and pyelonephritis [ 5, lo- 121. Pertinent physical findings are listed in Table I. Characteristic roentgenographic findings on intravenous pyelogram and barium enema strongly suggest,

Figure 8. Case 3. CT scan showing subcutaneous same attenuation coefficient as lipomatous tissue.

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I

Pertinent

Clinical

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Findings Cases Per cent No.

Hypertension [9] Ill-defined suprapubic mass [ 1,2,9] Superior displacement of prostate [9] Obesity [ 1,9] Ureteral obstruction [ 5,121

72 29 48 a0 17

18125 14148 10121 a/i0 5130

NOTE: Per cents and numbers refer to the cases in which the specific finding was mentioned.

but are not diagnostic of, pelvic lipomatosis. Multiple conditions can cause a pear-shaped bladder including pelvic trauma with hematoma, inferior vena caval occlusion, metastatic carcinoma, lymphocysts and enlarged lymph nodes [ 13,141. Lymphogranuloma venereum in its late stages can straighten and elongate the rectosigmoid; however, mucosal aberrations are usually present [ 151. Nussbaum [ 141 described a patient with metastatic prostatic carcinoma who demonstrated the typical roentgenographic features of pelvic lipomatosis, but the presenting symptoms were not consistent with pelvic lipomatosis. Displacement of the bladder and straightening of the rectosigmoid are characteristic roentgenographic findings of a pelvic mass. CT scanning is effective in the evaluation of patients with known or suspected pelvic masses [ 151. In our three cases, the tissues around the rectosigmoid and bladder had an attenuation coefficient of - 124 U (Delta Scanner), the same as that for the patients’ subcutaneous fat. The attenuation coefficient is, in effect, a measure of tissue density. Tissues are placed on a scale of - 1,000 to + 1,000, referred to as Delta or Hounsfield numbers. The attenuation coefficient in Delta units of bone ranges from +300 to +l,OOO, water 0, soft tissue tumors +20 to i-50, and gas

- 1000. Fat typically measures in the - 100 range. The radiation dose for the study varies between 2 and 5 rads, which is similar to that for a combined barium enema and intravenous pyelogram. In the past, pelvic laparotomy with tissue diagnosis was essential to the documentation of this disease, but this invasive approach, coupled with the predilection of pelvic lipomatosis to produce postoperative deep venous thrombosis, entails significant risk. CT scanning can recognize lipomatous tissue adjacent to the bladder and rectum, further confirming the roentgenographic appearance of pelvic lipomatosis. Other lipomatous infiltrations of the pelvis, such as liposarcoma, would be expected to produce nonhomogeneous attenuation coefficients and other stigmata of neoplasm on barium enema and intravenous pyelography; such as organ invasion. There appears to be general agreement that the renal function of patients with pelvic lipomatosis should be evaluated via radioisotope renal scans every six months. Deterioration indicates the need for considering such possible therapeutic modalities as urinary diversion or ureteral lysis. It is our opinion that the CT scan is a safe, accurate method of diagnosing this entity. We urge that the invasive surgical exploration and biopsy be replaced with the noninvasive CT scan to substantiate the diagnosis. The CT scans of our three patients clearly and conclusively delineated the extent of the lipomatous deposition, and this, coupled with other roentgenographic findings, established the diagnosis of pelvic lipomatosis. ACKNOWLEDGMENT

We wish to thank Richard G. Wendel, M.D. and Susan M. Bonomini for assistance in this study.

REFERENCES 1.

2. 3.

4. 5.

6. 7.

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Engels EP: Sigmoid colon and urinary bladder in high fixation: roentgen changes simulating pelvic tumor. Radiology 72: 419,1959. Fogg LB, Smyth JW: Pelvic lipomatosis. A condition simulating pelvic neoplasm. Radiology 90: 558, 1968. Becker JA, Weiss RM, Schiff M Jr, et al.: Pelvic lipomatosis. A consideration in the diagnosis of intrapelvic neoplasms. Arch Surg 100: 94, 1970. Bender LI, Kass M: Periureteral lipomatosis: case report. J Urol 103: 293.1970. Abbott DA, Skinner DG: Congenital venous anomalies associated with pelvic lipomatosis (a case report). J Urol 112: 739, 1974. Bell TE, Wendel RG: Cystitis glandularis: benign or malignant? J Urol 100: 462, 1968. Yalla SV, lvker M, Burros HM. et al.: Cystitis glandularis with perivesical lipomatosis. Urology 5: 383, 1975.

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a. Barry JM, Bilbao MK, Hodges CV: Pelvic lipomatosis: a rare 9. 10. 11. 12. 13. 14. 15.

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cause of suprapubic mass. J Urol 109: 592, 1973. Sacks SA, Drenick EJ: Pelvic lipomatosis: effect of diet. Urology 6: 609, 1975. Blau JS, Janson KL: Pelvic lipomatosis. Arch Surg 105: 498, 1972. Carpenter AA: Pelvic lipomatosis. Successful surgical treatment. J Urol 110: 397, 1973. Golding PL, Singh M, Worthington B: Short notes of rare or obscure cases. Br J Surg 59: 69, 1972. Ambos MA, Bosniak MA, Lefleur RS, et al.: The pear-shaped bladder. Radiology 122: 85, 1977. Nussbaum PS: Carcinoma of the prostate presenting as pelvic lipomatosis. Surg Clin North Am 52: 405, 1972. Carter BL, Kahn PC, Wolpert SM, et al.: Unusual pelvic masses. A comparison of computed tomographic scanning and ultrasonography. Radiology 121: 383, 1976.