Pelvic Pain Without Pelvic Organs

0022-534 7 /92/1473-0683$03.00/0 Vol. 147, 683-686, March 1992

THE JOURNAL OF UROLOGY Copyright© 1992 by AMERICAN UROLOGICAL ASSOCIATION, INC.

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PELVIC PAIN WITHOUT PELVIC ORGANS LAURENCE S. BASKIN AND EMIL A. TANAGHO* From the Department of Urology, University of California School of Medicine, San Francisco, California

ABSTRACT

We report on 4 patients with persistent, severe pelvic pain unresponsive to removal of the bladder, uterus, ovaries and fallopian tubes. Of the patients 3 had a diagnosis of interstitial cystitis and 1 had voiding dysfunction. We conclude that severe pelvic pain may not be responsive to the elimination of pelvic organs and alternative organ-preserving therapies should be considered. KEY WORDS:

pain, intractable; bladder diseases; cystitis; urination disorders

Pelvic pain may arise from a number of sources. Urologists naturally implicate the bladder and urethra, whereas gynecologists look toward the female reproductive organs. Gastroenterologists concentrate on colonic and rectal problems, and orthopedists on the bony structures. Urologists and gynecologists are usually the first to treat these patients, since they commonly present with a urinary or genital complaint. With deep pelvic pain, especially if it is periodic or aggravated by the menstrual cycle, endometriosis is suspected. Laparoscopy is immediately considered. Not infrequently, advice for hysterectomy will follow. If pain is associated with frequency and urgency, interstitial cystitis becomes suspect and endoscopy is planned. If for any reason there are mucosa! hemorrhagic spots, decreased capacity or even simple pain during examination and bladder filling, the diagnosis might be confirmed. Once this is rendered and mentioned to the patient, most likely it will be reconfirmed by every subsequent evaluation. Treatment for interstitial cystitis will begin, including hydrostatic distension and intravesical irrigations with a variety of chemical agents. Some of these agents, because of their irritant nature, might aggravate the symptoms and in a short period the patients become depressed and desperate for effective treatment. Not infrequently, they will then readily accept the advice for partial or complete resection of the involved organ. We have encountered a number of patients in whom, despite careful evaluation and diagnosis, surgical removal of the responsible organ did not ameliorate the problem. We report on 4 such patients who continue to have debilitating pelvic pain.

pain and has required multiple hospitalizations for inflannnation of the pouch with pyelonephritis and control of pain. Case 2. A 19-year-old woman presented at age 16 years with pelvic pain, urinary frequency and nocturia. Findings on physical examination were normal. Multiple urine cultures were negative. Initial cystoscopic evaluation showed a capacity of 300 ml. without obvious pathology. The patient was treated conservatively with antibiotics, antihistamines and antispasmotics. The symptom complex progressed to urinary frequency every 10 minutes, nocturia every hour and continued pelvic pain. Results of cystoscopy with the patient under anesthesia and bladder biopsy were consistent with interstitial cystitis. During the next year the patient underwent 4 cystoscopic hydrodistensions of the bladder with instillation of dimethyl sulfoxide while under anesthesia. Alternative therapy with oral antihistamines, narcotics, anti-inflammatory agents, belladonna suppository and transcutaneous electrical nerve stimulation units was also unsuccessful. At age 16 years the patient underwent total cystectomy with ileal loop urinary diversion (fig. 2). Postoperatively she continued to suffer pelvic pain.

CASE REPORTS

Case 1. A 29-year-old woman initially had pelvic pain at age 22 years. Hysterectomy with lysis of pelvic adhesions was performed at age 23 years. Subsequent treatment with estrogen, progesterone, pyridium, anti-inflammatory agents, antifungal creams and antibiotics failed to alleviate the symptoms. Continued pelvic pain led to exploration at age 26 years with bilateral salpingo-oophorectomy and lysis of adhesions. Further evaluation at this point focused on the bladder. Persistent pain led to a diagnosis of interstitial cystitis. The pain was associated with severe urinary urgency, frequency and nocturia. After treatment with dimethyl sulfoxide, sodium oxychlorosene and bladder hydrodistension failed, the patient underwent total cystectomy, urethrectomy and creation of an ileocecal urinary reservoir. Revision of the reservoir with an ileo-pouch augmentation was necessary secondary to intermittent pouch contractions, which caused incontinence that required an external appliance (fig. 1). Subsequently, the reservoir was converted to an ileal loop because of pain and difficulty with catheterization. Presently, the patient is using narcotics for continued pelvic Accepted for publication August 9, 1991. *Requests for reprints: Department of Urology, U-575, University of California, San Francisco, California 94143-0738. 683

FIG. 1. Case 1. X-ray, urinary diversion with multiple surgical clips

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FIG. 3. Case 3. X-ray, multiple surgical clips

FIG. 2. Case 2. X-ray, ilea! loop diversion with bilateral reflux and multiple surgical clips.

During the next 2 years she had 6 operations, including revision of an ileal loop, lysis of adhesions causing small bowel obstruction, incision and drainage of wound seroma, revision of a peristomal hernia, presacral neurectomy and hysterectomy with bilateral oophorectomy, for a total of 16 general anesthetic episodes within a 4-year period. At age 19 years she had no reproductive potential and was addicted to narcotics for continued pelvic pain. She also had difficulty with stool continence secondary to the presacral neurectomy. Case 3. A 38-year-old woman had pelvic pain at the age of 25 years. She eventually was diagnosed as having interstitial cystitis based on associated urinary frequency and urgency. Early in the course hysterectomy with bilateral salpingo-oophorectomy was performed. Continued pelvic pain was unresponsive to antibiotics, bladder instillations, anti-inflammatory agents, narcotics and antispasmotics. The pain was associated with progressive symptoms of urinary frequency and nocturia. At age 36 years she had undergone total cystectomy, urethrectomy, and Kock pouch urinary diversion (fig. 3). The patient continues to have chronic pelvic pain requiring narcotics. Multiple emergency room visits as well as hospitalizations have been required, with the diagnosis always being inflammation of the pouch with pyelonephritis but the therapy being parenteral narcotics. The patient has contemplated suicide. Case 4. A 30-year-old woman presented with pelvic pain, dysuria and recurrent urinary tract infections. Radiological and

urodynamic evaluation revealed a large smooth-walled bladder and high post-void residuals (200 to 300 ml.) with no detrusor activity. The patient was initially treated with multiple courses of antibiotics and timed voiding. Persistent pain and urinary tract infections led to repeat evaluation, which revealed persistently elevated post-void residuals along with a large bladder capacity. During the next 3 years the patient had 7 major operations (partial cystectomy and Y-V plasty of the bladder neck, urethral dilation for stenosis and scarring, resection of the posterior bladder neck twice, augmentation ileocystoplasty, anterior exenteration of the bladder, urethra, uterus and ovaries, Kock pouch urinary diversion and revision of the Kock pouch), as well as 27 hospital visits and approximately 186 office visits. The first operation decreased the size of the bladder, resulting in urethral scarring and stenosis. Subsequently, she required bladder augmentation and then, for continued pelvic pain, eventual anterior exenteration (including urethrectomy) with Kock pouch urinary diversion (fig. 4). Presently, she continues to have difficulty with catheterization of the urinary diversion and the persistent pelvic pain requires chronic narcotics. DISCUSSION

Although many patients have the diagnosis of interstitial cystitis, an exact definition of this disease and its cause remain elusive. 1- 3 In a recent review from the University of Pennsylvania 21 of 55 patients (36%) who had been given the diagnosis of interstitial cystitis failed to meet the research criteria proposed at the National Institutes of Health Workshop. 1 Furthermore, detrusor mastocytosis proved to be nonspecific, occurring in 64% of the patients with interstitial cystitis and 80% of the control group (who had voiding dysfunctions secondary to other pathological conditions). Our patients with severe pelvic pain and interstitial cystitis did not have impressive mastocytosis (fig. 5), illustrating the lack of definitive histological findings in patients with chronic pelvic pain. This finding supports the concept that interstitial cystitis is a complex

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FIG. 4. Case 4. Cystography. A, preoperative. B, after reduction cystoplasty. C, after augmentation cystoplasty

FIG. 5. Histological sections from cystectomy specimens. A, case 1-intact urothelium with mild infiltrate of plasma cells and mast cells in submucosa, minimal vascular dilatation and fibrosis. Scattered mast cells are also observed between detrusor muscle fiber. B, case 2uroepithelium with chronic inflammatory cells, and moderate number of mastocytes and histiocytes in submucosa and muscularis. C, case 3epithelium without evidence of ulceration, plus no atypical features, scattered lymphocytes in submucosa and fibrous connective tissue throughout bladder wall. Moderate number of mast cells are present. Reduced from Xl50.

Characteristics of 4 patients with peivic pain without pelvic organs Pt. l

Pt. 2

Pt. 3

Pt. 4

Age at presentation (yrs.) 28 30 22 16 27 17 36 34 Age at cystectomy (yrs.) Urinary diversion Indiana pouch Ilea! loop Kock pouch Kock pouch No. major operations 7 7 5 16 No. hospital admissions* 14 27 31 35 All were women in whom the reproductive organs had been removed. * Pain control, pyelonephritis, inflammation of the pouch, operations and diagnostic procedures.

disease whose diagnosis is based on a symptom complex rather than objective histological criteria. Patients with interstitial cystitis can usually obtain relief from a number of conservative treatments. 4 Hydrodistension of the bladder with the patient under anesthesia, dimethyl sulfoxide, heparin and pentosanpolysulfate have all been successful to a certain extent. 5 •6 A small percentage of patients, however, will require some form of surgical therapy. 7 Attempts at denervation of the bladder by cystolysis initially were encouraging but long-term results have been disappointing. 8 Re-

moval of the majority of the bladder with supratrigonal cystectomy and ileocecocystoplasty has also proved to be disappointing.9 In hopes of completely removing the offending organ, thereby eliminating the source of pain, urinary diversion with cystectomy has been suggested as the definitive procedure. The outcome in this group, already severely debilitated, has been lacking. We report on 4 patients with a poor outcome. The pathophysiology of pain in patients with interstitial cystitis and/or pelvic pain is simply not understood. Our 4 patients continue to have severely debilitating pelvic pain without pelvic organs. Three patients had undergone urethrectomy. All 4 are women in their reproductive years and, interestingly, all had undergone hysterectomy. As is clear from the patient characteristics (see table), all 4 women also had multiple operations as well as hospitalizations. It is noteworthy that 3 of the 4 women had continent urinary diversion. All 3 patients have had difficulty with the reservoirs and 2 required revisions, 1 to a conduit diversion. One might speculate that these patients would do better with a conduit urinary diversion. Case 2, however, illustrates the point that despite a conduit diversion she too continued to have debilitating pelvic pain.

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Besides the pelvic visceral organs, the pelvic musculature constitutes an integral part of the pelvic cavity, guarding the outlet and controlling the orifices of the visceral organs. Coordination between the pelvic musculature and the pelvic visceral organs is essential for the proper functioning and integrity of the latter. We do not discuss the varieties of pelvic floor dysfunction, their symptoms and their impact on the integrity of the pelvic visceral organs. However, it must be stressed that before extirpation of pelvic visceral organs is considered, pathological findings should be documented. The pelvic muscular element, which could well be the source of pain, must be evaluated. Urodynamic studies, electromyelography, neurostimulation and neuromodulation are essential to complete the evaluation of pelvic pain before a major operation is considered/0012 (We have -been highly successf-ul with neuromodulation for treating a large number of these patients labeled as having interstitial cystitis and the results will be reported at a later date.) It also is possible that the origin of pelvic pain in these patients is central. At this time, however, this is a theoretical concern with no documentation of central pathology.

Although it would be useful to define a unique characteristic common to these 4 patients, unfortunately their common bond is the persistence of debilitating pain despite removal of the pelvic organs. Our study supports the findings that objective histological features are lacking in interstitial cystitis, and that pelvic pain can persist even after radical cystectomy and urinary diversion. Clearly, severe pelvic pain may not be responsive to the removal of pelvic organs and alternative organpreserving therapies should be considered. REFERENCES

1. Hanno, P., Levin, R. M., Monson, F. C., Teuscher, C., Zhou, Z. Z., Ruggieri, M., Whitmore, K. and Wein, A. J.: Diagnosis of interstitial cystitis. J. Urol., 143: 278, 1990. 2. Gillenwater, J. Y. and Wein, A. J.: Summary of the National Institute of Arthritis, Diabetes, Digestive and Kidney Diseases workshop on interstitial cystitis, National Institutes of Health, Bethesda, Maryland, August 28-29, 1987. J. Urol., 140: 203, 1988. 3. Messing, E. M.: The diagnosis of interstitial cystitis. Urology, suppl. 4, 29: 4, 1987. 4. Messing, E. M. and Stamey, T. A.: Interstitial cystitis. Early diagnosis, pathology and treatment. Urology, 12: 381, 1978. 5. Fowler, J. E., Jr.: Prospective study of intravesical dimethyl sulfoxide in treatment of suspected early interstitial cystitis. Urology, 18: 21, 1981. 6. Parsons, C. L.: Sodium pentosanpolysulfate treatment of interstitial cystitis: an update. Urology, suppl. 4, 29: 14, 1987. 7. Freiha, F. S., Faysal, M. H. and Stamey, T. A.: The surgical treatment of intractable interstitial cystitis. J. Urol., 123: 632, 1980. 8. Freiha, F. S. and Stamey, T. A.: Cystolysis: a procedure for the

selective denervation of the bladder. J. Urol., 123: 360, 1980. 9. Nielsen, K. K., Kromann-Andersen, B., Steven, K. and Hald, T.: Failure of combined supratrigonal cystectomy and Mainz ileocecocystoplasty in intractable interstitial cystitis: is histology and mast cell count a reliable predictor for the outcome of surgery? J. Urol., 144: 255, 1990. 10. Tanagho, E. A., Schmidt, R. A. and Orvis, B. R.: Neural stimulation for control of voiding dysfunction: a preliminary report in 22 patients with serious neuropathic voiding disorders. J. Urol., 142: 340, 1989. 11. Tanagho, E. A. and Schmidt, R. A.: Electrical stimulation in the clinical management of the neurogenic bladder. J. Urol., 140: 1331, 1988. 12. Schmidt, R. A.: Advances in genitourinary neurostimulation. Neurosurgery, 19: 1041, 1986. EDITORIAL COMMENTS The authors present 4 cases of persistent pelvic pain after cystectomy performed for interstitial cystitis in 3 and for voiding dysfunction of unknown etiology in 1. Many of us who treat interstitial cystitis have learned the hard way not to perform extirpative surgery on young women whose main complaint is pain. As was appropriately noted, these women should be evaluated carefully and treated with noninvasive means, and they should be repeatedly counseled on how to cope with the pain. However, in carefully selected patients, namely elderly women with a fibrotic, contracted bladder and severe frequency, cystectomy with total urethrectomy is a successful treatment. Fuad S. Freiha Division of Urology Stanford University Medical Center Stanford, California This is an interesting and intriguing article that serves to highlight the critical caveat that cystectomy and diversion may not cure all patients with pelvic pain presumably secondary to interstitial cystitis. It is noteworthy that 3 of the 4 patients had a continent diversion. More and more anecdotal reports of problems related to the development of pain in the pouch in interstitial cystitis patients with a continent diversion have been surfacing, and this is an issue that deserves careful analysis. I have seen 2 patients with unremitting chronic pain who are worth mention. One patient has a large capacity bladder and I have hesitated to recommend surgery. The other patient had classical interstitial cystitis and proceeded from diversion to cystectomy-urethrectomy with no relief of the symptoms. Both patients had signs of Parkinson's disease and the latter was definitively diagnosed with the condition. The question of whether the pain is central in origin in these patients and unrelated to the pelvis itself is worth considering. This group of patients is extremely difficult to manage and the article serves as a much needed warning to those who allow patients to guide their own care, and perform a major operation for a condition that is not lifethreatening. Philip M. Hanno Department of Urology Temple University Hospital Philadelphia, Pennsylvania