PPOD Case Report 16

PPOD Syndrome Diagnoses: bilateral inguinodynia (chronic inguinal pelvic pain), metrodynia (deep suprapubic/uterine pelvic pain), levator ani syndrome (bilateral para-anal pain), coccygodynia (coccygeal pain), proctalgia (rectal pain), vulvodynia (pain in the labia and clitoris), dyspareunia (pelvic pain with intercourse), vaginodynia (vaginal pain), recurring stress urinary incontinence (loss of bladder control with coughing, sneezing and straining), impaired vesicle sensory perception (loss of ability to perceive bladder filling), total urinary retention (complete loss of ability to empty the bladder), chronic lower urinary tract infections (recurring bladder infections), irritable bowel syndrome (painful chronic diarrhea), excessive flatulence (intestinal gas), mucorrhea (mucus discharge from the rectum), impaired rectal sensory perception (loss of ability to perceive rectal filling), fecal incontinence (loss of rectal control), dysmenorrhea (painful and irregular menstruation), dysfunctional uterine bleeding (persistent uterine bleeding), vaginitis (recurring vaginal infection), spontaneous miscarriage (precipitous loss of pregnancy), anorgasmia (loss of ability to achieve orgasm), loss of libido (loss of sexual desire/drive).

A 39-year-old woman presented with an extensive list of complaints accompanied by a long and complicated history covering a 21 year period. At 18 years of age while living in a convent, she had slipped and fallen down a flight of stairs. Although she had not sustained any broken bones or other apparently serious injuries, she was left with persistent pain localized to the left inguinal region of her pelvis. She was taken to the hospital and underwent evaluation for the injuries sustained in her fall, however nothing that could account for the presence of her pelvic pain could be identified. Over the next few months, for no apparent reason, she found that her left inguinal pain had gradually become more intense. During the same period of time, as her left inguinal pain had increased, she spontaneously notice the onset of sharp right-sided inguinal pain. Because of the severe nature and location of this complaint, she was taken to the emergency room for evaluation. After being examined she was admitted to the hospital and underwent an appendectomy. Inspection of the appendix at the time of her surgery revealed that it had been normal in all respects. However, despite its removal, her right inguinal pain continued to persist unabated. She had begun to menstruate at 15 years of age. Initially, and for the first few years, menstruation had been relatively pain free and regular. However, at about the time of her appendectomy, there was a clear change in her menstrual function. What initially had been relatively pain free had now become severely painful and, although pain was felt in the inguinal regions bilaterally, it was distinctly dominant on the left. In addition, persistent ongoing diarrhea had spontaneously developed. As a result, she was re-hospitalized for additional evaluation and treatment. After undergoing extensive testing her symptoms were attributed to an irritable bowel occurring as a result of stress, and she was released from the hospital with no improvement in her bowel dysfunction or ongoing pelvic and menstrual pain. Due to the deterioration of her physical health making it impossible to continue with her studies, she was forced to leave the convent. About 2-3 years later, persistent vaginal discharge and recurring bladder and vaginal infections had spontaneously begun to occur. Treatment for what was identified as local vaginal yeast and bladder infections provided only temporary relief. In addition, during this same period, she began to experience ongoing genital pain that radiated bilaterally into the labia and clitoris. This pain caused her outer genital region to become extremely sensitive and painful to touch or contact of any type. Menstruation, which had been intensely painful, became even more severe and was accompanied by irregularity and excessive bleeding. She was placed on estrogen in an attempt to regulate her menstrual dysfunction, however no significant improvement occurred. At 26 years of age, she married and became pregnant with her first child. Since the beginning, intercourse had been intensely painful with pain being experienced superficially in the outer genital region as well as deeply in the vaginal, inguinal, suprapubic, coccygeal and rectal areas. Additionally, as a result of her diminished genital sensitivity orgasm had never been possible. She did note however that if she could tolerate intercourse for a long enough period of time, she could reach a point of “culmination” at which time rather than experiencing a pleasurable sensation associated with normal orgasm, an intense pain would develop deep within the pelvis and spread upward through the abdominal and chest regions extending to the base of the skull and terminate in a headache which would persist for a couple of days. She stated that this same radiating pelvic pain, terminating in a headache, would occur each and every time intercourse could he tolerated to the point of culmination. During the later months of her pregnancy, she began to experience intermittent low back pain. This pregnancy ended in prolonged labor with a vaginal delivery of a normal, healthy boy. Two years later, with her ongoing pelvic symptoms unchanged, she became pregnant for the second time. At about three months of gestation breakthrough uterine bleeding began to occur. This pregnancy terminated in a spontaneous miscarriage at about 5 ½ months gestation. Although her pregnancy had been well attended to gynecologically, no explanation could be found for her miscarriage. A few months later, she became pregnant for the third time. This pregnancy was accompanied by the onset of uterine bleeding as before and ended two months prematurely with the birth of a girl. Following the birth of her daughter, because of continuous pelvic pain, chronic diarrhea (which had been present since 18 years of age) and ongoing uterine bleeding, a laparotomy was performed. Although the procedure had failed to reveal the presence of any abnormalities, the severe nature of her ongoing pain and persistent uterine bleeding resulted in a partial hysterectomy being performed. Upon awakening from her hysterectomy however, she found that she had suffered a complete loss of bladder sensory perception (awareness of bladder fullness or needing to void) which was accompanied by a complete loss of the ability to empty her bladder. Her loss of bladder function resulted in total urinary retention and she was told by her surgeon that this unfortunate state had probably occurred as a result of the inadvertent severing of the nerves controlling bladder function during her operation. As a result, the inability to empty her bladder was felt to be a permanent consequence of her surgery and required that she be trained in self catheterization techniques that she would have to perform every three hours in order to empty her bladder. At about the same period of time, bowel dysfunction which had been continuous since its onset, spontaneously worsened. In addition to experiencing chronic painful diarrhea, excessive flatus, rectal bleeding, mucorrhea, and nocturnal fecal incontinence also began to occur. Accompanying her fecal incontinence was an associated loss of rectal sensory perception which impaired her ability to distinguish between gas and fecal matter and alert her to the unexpected spontaneous passing of stool. She underwent further evaluation at that time and the symptoms were attributed to proctalgia fugax and rectal fissures. However, palliative treatment of various types, as well as three separate exploratory bowel surgeries over a period of a couple of years, provided no relief of her ongoing bowel dysfunction. Only eight weeks after her partial hysterectomy, left sided inguinal pain spontaneously increased in its severity. As a result, additional pelvic surgery was performed and the left ovary was removed. Examination of the ovary at that time revealed the presence of numerous small cysts. During this operation, the right ovary was inspected, however no evidence of any type of cystic or other abnormality could be found. Unfortunately, despite the removal of her left ovary her left inguinal pain continued to persist unchanged. Over the next year, right-sided inguinal pain gradually increased in its severity. As a result, she again submitted to surgery and the right ovary was removed. At that time, inspection of the right ovary revealed the presence of numerous cysts similar to those that have been identified on the left. However despite the removal of her right ovary, right-sided inguinal pain also continued to persist unchanged. About four years later, despite the continuation of total urinary retention, she began to experience loss of urinary control when she would cough, sneeze or strain. This paradoxical state, being unable to empty her bladder but at the same time being unable to maintain bladder control, was accompanied by the onset of recurring bladder infections. This was a cause for great concern because, being unable to empty her bladder in a normal fashion, she was at great risk of developing infections that could ascend to affect and potentially damage her kidneys. She was treated aggressively with various antibiotics. However, shortly after successfully resolving one bladder infection she would quickly contract another. Because of her continuing bladder infections and gradually worsening incontinence, she consulted a urologist. After a series of tests, she underwent an initial bladder suspensory surgery in an attempt to restore urinary control. This procedure was successful in providing improvement of her incontinence and bladder infections for about one year. During this period of time however, there were no changes in her loss of bladder sensory perception, inability to empty her bladder, or urinary retentive state. Approximately one year later, for unknown reasons, she experienced a recurrence of urinary incontinence and persistent bladder infections. In addition, she began wetting the bed several times a week. After additional testing had been performed she underwent a second suspensory surgery which, like the first, was successful in providing relief of her incontinence and infections. However, her improved urinary control was short lived, as about six months later, after having fallen twice within the same week, she again experience a return of urinary incontinence. A third suspensory surgery, this time with the addition of a supportive mesh, was attempted. However because she was unable to accept the implant an additional operation had to be performed to remove the mesh. A few months later, a second attempt at positioning the implant was made. Finally, after being able to accept the implant, her urinary continence was again restored. Several months before being seen by me she again began to experience spontaneously recurring urinary incontinence. However, because her surgeries had provided relief for only short periods of time she chose not to undergo any further urological evaluation or treatment. At the time that I first saw her, I wasn’t quite sure what to make of her condition. Never had I seen anyone so severely affected and never had I treated anyone whose condition had so defiantly worsened in the face of such exhaustive diagnostic evaluation and intensive therapeutic intervention, which had been performed by some of the best specialists at the most prestigious medical institutions in the Midwest. In fact, as I pondered the situation, I wasn’t even sure that she was a PPOD syndrome patient. Although she did exhibit the kinds of symptoms characteristic of the mechanically induced PPOD syndrome, at the time, I had not seen anyone who exhibited the extensiveness of involvement that she had. However, during her PPOD syndrome examination, to my surprise, she exhibited clear evidence of the mechanically induced pelvic pain and organic dysfunction syndrome. When I explained all of this to her, she didn’t seem overly surprised. Maybe with everything she had been through she just expected that there had to be more, or maybe she thought that by a process of elimination it had to finally come down to this. In any event, she stated that she couldn’t live with her pelvic pain any longer so accordingly, we made plans to begin her treatment a couple of days later. I had prepared her for the fact that she would probably experience a transient increase in her pelvic pain for several hours following her first few treatments. Although she wasn’t looking forward to it, she understood that this was a typical reaction during the initial phases of treatment. The day following her initial treatment she reported that there had indeed, been a distinct post treatment flare causing an increase in her pelvic and genital pain. However, by following her home therapeutic procedures and post treatment instructions, this pain increase began to subside before the evening. The first couple of weeks of treatment were entirely uneventful. Apart from the home therapeutic activities to minimize the pain following her treatments, she had little to report. Then, in the third week of treatment, she reported two notable changes. Although for several days she thought that the pain increase following her treatments was beginning to become less intense, she realized that her ongoing chronic pelvic pain seemed to be somewhat diminished as well. This caused her great excitement because, for over 20 years, this pain had done nothing but become more severe and had progressed to become more extensive involving more regions within the pelvis. The other change that she had noted was a fleeting sense of returning bladder sensory perception. She stated that this had occurred several times over the previous couple of days for only a few seconds at a time. She hadn’t made mention of this earlier because due to the short duration of this symptom she wasn’t sure that this was, in fact, what she was feeling. However, the previous afternoon following her treatment this sensation had returned for several minutes, and as it did, she became aware of the need of having to empty her bladder by the returning sense of bladder fullness and the normal urge or desire to void. Prompted by this urge she went to the bathroom and found that by straining as she attempted to void, she was able to pass a few drops of urine on her own. She was extremely excited about these events because it had been nearly 10 years, since having lost bladder function when she underwent her hysterectomy, that she had had any awareness of needing to empty her bladder or any ability to voluntarily pass even small amounts of urine. I was absolutely floored by the thought that she was experiencing returning bladder function. How could this possibly be if, in fact, the nerves controlling her bladder function had been cut during her hysterectomy. The complete loss of bladder function should be permanent. The next day, she reported continued brief periods of returning bladder sensory perception during which time she was still able to pass small amounts of urine on her own. Because of the changes that had begun to occur, my interest in her case had suddenly piqued. The following day, as I was finishing with the patient just ahead of her, I heard from down the hall the uncontrollable sobbing of a woman standing near the reception desk. I could tell that it was her, and from the sounds of it, it seemed like something had gone terribly wrong. As I thought about what might have happened I began to sense an uneasy feeling growing in the pit of my stomach. However, I didn’t have long to wait to find out what had occurred. When I entered the treatment room where she had been waiting, our eyes instantly met. Holding a tissue in her hand it was apparent that she was still crying. Without saying a word, she rose and walked over to me. She placed her arms around my back and quietly said “thank you” as she gave me a long warm hug. Not being one to understand even the not so subtle nuances of a woman, I was taken aback again. When I asked what had happened, she explained that the previous day after her treatment she continued to experience the brief fleeting episodes of returning bladder sensory awareness, however, they were occurring more often. By the evening, she was aware of bladder fullness and the urge to empty her bladder more often than not. As she prepared for bed she decided to try to void to empty her bladder rather than catheterizing herself as was customarily the case. To her absolute astonishment, as she began to void, she found that she was able to completely empty her bladder in a normal unimpeded fashion without needing to catheterize herself. She was beyond elation. She joked that she stayed up most of the night sitting on the toilet experiencing the overwhelming joy of just emptying her bladder. She went on to say that it seemed that with the return of normal bladder function she was able to eliminate excessive fluid that had apparently accumulated during the time that her bladder had not been functioning. This was made more apparent to her by the fact that by morning, long-standing swelling and puffiness affecting her ankles and feet and hands and fingers had significantly diminished. In fact, she demonstrated for me the looseness and the ease at which she could now remove her wedding band, where as before, this could only be done with difficulty and the aid of soap to lubricate her finger. As the realization of what had happened began to sink in, I suddenly became overwhelmed by a sense of gratification the likes of which I had not experienced before. As I thought about how this might have happened, the only thing I could surmise was at the nerves controlling bladder function had obviously not been cut during her operation. Why then had she been told that they were? Assuming that they hadn’t, what did happen to cause her to lose bladder function. It did seem odd that she could have sustained the kind of damage that she was told she had as a result of the type of procedure she had undergone. Given that unlikely scenario, it seemed more probable that the transfer from the gurney to the operating room table or the position in which she was placed to perform her surgery had probably strained her spine in a way to further injure the nerves controlling the function of her pelvic organs. Over the next few weeks, she continued to improve at a rapid pace. Her ongoing chronic pelvic pain continued to diminish and gradually faded altogether. During this time there was a simultaneous reduction in the intensity of her pelvic pain with intercourse as well. Gradually, pelvic pain with intercourse completely resolved altogether and there was a return of normal sexual desire. As these changes took place, her genital pain and hypersensitivity were also diminishing, and within a few more weeks these symptoms had completely resolved. She became aware of improved genital sensitivity and shortly thereafter she began to experience the pleasurable sense normal orgasm for the very first time in her life. Her bladder function which had already significantly improved fluctuated for several weeks before becoming consistently normal. Not only had she fully regained the ability to normally empty her bladder, but she also experienced the complete resolution of her persistently recurring stress incontinence. Bowel dysfunction, which had been present since nearly the beginning of her long ordeal, also rapidly improved. After several weeks of treatment, her frequent and painful diarrhea had resolved. Excessive flatulence, bleeding, mucus discharge and nocturnal fecal incontinence were all greatly improved and after a few more weeks of treatment these symptoms also disappeared. While her response to treatment was impressive, in some ways it seemed even more so when considering everything that she had been through to get to that point. In total, she had undergone 13 separate pelvic surgeries and had consulted more than 30 different doctors for the various problems she had developed over her 21 year ordeal. Despite these efforts however, none of the surgeries that she had had were effective at resolving the problem for which they were performed. The only exception is that of the hysterectomy which had been performed because of continued uterine bleeding and chronic pelvic pain. Although it did resolve the bleeding (as the uterus was removed), it had no effect at improving her pelvic pain. While her surgeries had not been helpful, it appeared that she was fortunate that the risks that she was subjected to and the damage caused by her multiple procedures did not obstruct or impair her ability to respond when her condition was properly diagnosed and appropriately treated.

Comment: It is of interest to note that despite being caused by a mechanical disorder of the spine, and in spite of the severe, extensive, progressive and longstanding nature of her condition, this woman had absolutely no history of back pain or any obvious (typical) accompanying spinal related complaints.