Conundrum of chronic pelvic pain and a pelvic journey of unparalleled grit: The good, the bad, and the ugly

There is some logic that even if poverty and marginalization are cut across gender, still women face unique discrimination, which ranges from fighting to be born to being allowed to be mobile and to being allowed to play sport and to dress up, the way they want to. If one delves deep into their lives, especially in the context of chronic pelvic pain (CPP), it reveals a story of loneliness, rebellion, constant struggle, and unparalleled PELVIC journey of grit to overcome the pain and suffering in CPP. This is far more evident from a WHO systematic review of the prevalence of CPP, which highlighted a prevalence of 16.8% to 81% for dysmenorrhea, 8% to 21% for dyspareunia, and 21% to 24% for noncyclical pain.[1] In another review, based on ACOG definition (noncyclical pain typically of more than 6-month duration), the prevalence of CPP ranges from 5.7% to 26.6%.[2] It has been proved time and again that there is far more to CPP than what meets the eye. While it bares everything – the good, the bad, and the ugly. There seems to be certain absence of agreement on the definition of CPP. The most notable one is by obstetricians and gynecologists and ReVITALize data definitions initiative which defines “Chronic Pelvic Pain as pain symptoms perceived to originate from pelvic organs/structures typically lasting more than 6 months.” It is often associated with negative cognitive, behavioral, sexual, and emotional consequences as well as with symptoms suggestive of lower urinary tract dysfunction, sexual dysfunction, bowel dysfunction, pelvic floor dysfunction, myofascial dysfunction, or gynecological dysfunction.”[1] Unfortunately, this definition does not address cyclical pain such as dysmenorrhea and mittelschmerz. It has been repeatedly demonstrated that chronic pain is a biopsychosocial phenomenon, wherein CPP is no less an exception. There is evidence of ripple effect in CPP, where Learman et al., Ayorinde et al., Allaire et al., and ACOG practice bulletin have observed that 12%–33% of women, cutting across inclusion criteria of CPP, meet the criteria for major depression.[1345] Moreover, the ripple effect in CPP also manifests as high degree of anxiety, phobias, fearfulness, frustration, mood disturbances, sleep disorders, cutting off behavior in a social fabric of the community. In majority of cases, there are more than one underlying cause of CPP. At times, both interstitial cystitis and endometriosis are together the underlying cause of CPP.[2] Endometriosis is the most common cause of CPP, almost 24%–40% of all CPP patients and has a prevalence rate of 10%–12% in various countries. However, there is a paucity of work in the field of nonendometriosis-related CPP. Other causes of CPP include pelvic adhesions, irritable bowel syndrome, and CPP which is at times associated with posttraumatic stress disorder and major depressive disorder.[67] CPP is a kind of complex regional pain syndrome (CRPS) of the pelvis.[89] There is a strong possibility of central sensitization phenomenon operating in CPP. In the opinion of Engeler et al. and Potts et al., these patients with CPP manifest allodynia and hyperesthesia, as a consequence of pelvic floor dysfunction.[89] CPP patients experience increased incidence of anxiety, depression, and sleep deficits. Childhood sexual abuse is an important event in the history of CPP patients, and it pinpoints both the somatic symptoms of CPP and the associated posttraumatic stress.[1011] Warning symptoms and signs include postcoital bleeding, postmenopausal bleeding, hematuria, or even a pelvic mass. It calls for detailed clinical examination for enlarged uterus, absence of uterine mobility on bimanual examination, or assessment of adenexal mass. As per the guidelines of European Association of Urology, the sacroiliac joint and lumbar spine must be examined for any tenderness.[12] Furthermore, Carnett test needs to be performed for abdominal wall pain associated with pelvic pain. Hence, when the legs are raised off the examination table with patient in supine position, if the pain on the painful abdominal wall area increases, on flexion of legs, it indicates myofascial pain, and any improvement in the pain with the leg flexion reflects visceral pain. Moreover, Tu et al. observed up to five times more asymmetry of the heights of the iliac crests and levels of pubic symphysis, in women with CPP.[13] In addition, Nasr et al. confirmed the validity and reliability of cotton-tipped applicator test as 100% specific in CPP, for evidence of cutaneous allodynia.[14] As per the recommendations of latest ACOG practice bulletin, the elective use of laparoscopic adhesiolysis is not beneficial in the long-term alleviation of CPP, having excluded conditions such as adenomyosis, endometriosis, vestibulitis, vulvodynia, and adnexal disorders (Evidence level A).[1] Sometimes, adhesiolysis can help in special conditions like dense adhesions tightly adherent to the uterus and like stricture of the bowel. Furthermore, in an interesting recent systemic review and meta-analysis, van den Beukel et al. concluded that following lysis of adhesions, there is no improvement in comparison to the diagnostic laparoscopy.[15] This meta-analysis also included a long-term follow-up study of one of the RCTs by Molegraaf et al., which concluded that pain relief was poor in the adhesiolysis group (only 19% reported complete relief of abdominal pain) compared to the diagnostic laparoscopy group (42% had reported complete pain relief of abdominal pain) (relative risk, 1.3).[16] Level B evidence based on recommendations in CPP includes the use of gabapentin, pregabalin, and serotonin – norepinephrine reuptake inhibitors, for the management of neuropathic CPP.[1] Opioids are to be discouraged in the management of CPP. Furthermore, level B evidence-based recommendations in CPP are inclusive of expert referrals for cognitive behavioral therapy, or pelvic floor exercises, sex therapy, alone or in association with pharmacotherapy.[1] In patients with myofascial CPP, in an attempt to relieve pain and enhance the functional ability, there is level B evidence-based recommendation for trigger point injections of steroids or saline, alone or in association with other management strategies.[1] Level C evidence-based recommendations in CPP include abdominal and pelvic neuromusculoskeletal clinical examination and screening for irritable bowel syndrome, diverticulitis, interstitial cystitis, and associated anxiety, depression, and frustration.[1] Last but not the least, level c evidence-based recommendations are also inclusive of yoga and acupuncture for the treatment of CPP due to musculoskeletal causes.[1] In the continuum of care of CPP patients, various nerve and plexus blocks have potential limitations in their efficacy. As in the algorithm of management of chronic pain, Hunter et al. suggest that the next step of neuromodulation may be an alternative option for selective group of CPP patients, who are proving to be refractory to conventional treatment.[17] For pain mapping in CPP, blocks should still be encouraged, mainly to delineate the pathway of pain from the peripheral nervous system to the central nervous system. Hence, its more of a diagnostic detail provided by a block, rather than the limited therapeutic role of a block. Nerve blocks and radiofrequency may provide therapeutic benefit, especially in the early stages of CPP. Recently, Deer et al. concluded that dorsal root ganglion (DRG) stimulation may offer another option for pain management of patients with CPP.[18] It is also evident from the observations of Hunter et al.,[19] Janick,[20] Barnowski,[21] Plateau et al.,[22] and Deer et al.[18] in the patients with interstitial cystitis and nerve injury that CPP may be a kind of CRPS. The kind of interventions in CPP management includes basic trigger point injections and botulinum toxin injections in muscle of pelvic floor to improve dyschezia and dyspareunia.[23] It would be interesting to know, Casasola et al.[24] reported 70% pain relief by blockade of superior hypogastric plexus (SHP) in CPP due to malignancy. In the benign CPP, interestingly, Rosenberg et al.[25] observed pain relief by blockade of SHP, in severe chronic penile pain following TURP. In addition, Plancarte et al.[26] reported that blockade of ganglion of Impar (or ganglion of Walther) produces significant pain relief in anal, rectal, perineal, and genital pain. Furthermore, sacral nerve stimulation has delivered good results in patients of CPP who are not responding to conservative management.[1827] It would be interesting to know that, Bosch JL observed that pudendal nerve stimulation is an effective alternative in CPP patients, who are not getting relief with sacral nerve stimulation.[28] Heinze et al.[29] recently proposed the STAR technique (S-Ischial Spine, T-Ischial tuberosity, A-Acetabulum, R- anal Rim) for identifying pudendal nerve under C-arm fluoroscopy. Another pathway that has been adopted by Ozkan et al.[30] is through the sacral foramen for sacral nerve stimulation. This is also termed as sacral transforaminal neuromodulation, with the help of Inter-Stim neurostimulation (Medtronic). An upcoming modality of “Dorsal Root Ganglion Stimulation” was granted approval by US- Food and Drug Administration in 2016. Basically, DRG stimulation brings a change in the sympathetic outflow, and so obviously, it may be beneficial in sympathically mediated pain syndrome, such as CPP. Recently, Hunter et al.[31] working on DRG stimulation and observed mean reduction in pain of 76.7% on NRS scale, in 6 patients with CPP, being managed with DRG stimulation. They also noted success in CPP patients by targeting bilateral L1 and S2 DRGs.[31] In addition, the conventional spinal cord stimulation is a huge challenge in CPP, simply because dermatomal targeting is complex in the pelvic region for the purpose of neuromodulation. On a theoretical basis, the midline stimulation of midthoracic cord should give better coverage of pelvic pain than more lateral placements. Similarly, as regard to positioning of intrathecal drug delivery in the treatment algorithm of CPP, it has yet to find a place. Last but not the least, physical therapy and YOGA and meditation shall always remain an essential and integral part of the holistic management of patients with CPP. In this CPP editorial, what we intend to write is fiercely cerebral and moreover gastronomic temptations as well as therapeutic confabulations may be in abundant supply in CPP; but optimal effective management of CPP shall always remain a huge challenge. No doubt focused research in CPP shall provide special push to pain physician's quest for alleviating the pain and suffering in CPP and shall deserve a fulsome praise as a compliment. We need a strategy to ramp up over our actions to combat the CPP crisis. We need to understand that a CPP patient's pain shall not resolve till her underlying adenomyosis or endometriosis is treated, her painful pelvic floor myalgia is alleviated, and her central sensitization is brought under control with neuromodulation and her comorbid anxiety or depression is brought to remission phase. Hence, multicentric, randomized, placebo-controlled studies are to encouraged to assess the precise efficacy and safety of various interventional therapies including the neuromodulation approach in the holistic management of CPP patients. “Pain and depression go hand in hand, always pushing you to your limits. With help from outside and time to accept, our love of ourselves will most defiantly resurrect. There is something being around the nature, that makes everything so right. If I get see a sunset, there would not be a single reason to get upset. Pain and despair, anger and frustration take a back seat, when I see that reflection.”[32]