Chronic%20pelvic%20pain Diagnosis

• A subdivision of chronic pelvic pain (CPP), chronic pelvic pain syndrome (CPPS) is pain localized to >1 organ site
• It is the occurrence of CPP without an obvious ongoing disease process that can account for the pain, often associated with negative behavioral, cognitive, emotional or sexual consequences in addition to symptoms that are suggestive of a bowel, lower urinary tract, gynecological, or sexual dysfunction
• CPPS is also suggested by the following: Pain persistence >6 months, incomplete pain reduction from prior therapies, pain is not consistent with tissue damage, loss of function & signs of depression are present, changes in the relationship & family dynamics
• The term CPPS may also be used when pelvic pain is poorly localized, specific pathology is unidentified, or if pain is localized to multiple organs within the pelvic area
• CPPS mechanisms include neuroplasticity & neuropathic pain mechanisms
• CPPS should be subdivided according to its corresponding phenotype based on the classification proposed by the European Association of Urology (EAU) if with adequate evidences
• Based on the axis system recommended by the International Association for the Study of Pain (IASP)

Gastrointestinal Aspect

Chronic Anal Pain Syndrome (Chronic Proctalgia)

• The following criteria should be met for the past 3 months with onset of symptoms at least 6 months before diagnosis: Chronic or recurrent rectal pain or aching, episodes lasting at least 20 minutes, other causes of rectal pain are excluded (eg coccygodynia, cryptitis, hemorrhoids, inflammatory bowel disease, intramuscular abscess & fissure, ischemia, prostatitis)
• Also demonstrates severe tenderness on posterior traction of the puborectalis muscle
• Intermittent chronic anal pain syndrome (proctalgia fugax) may be considered a subgroup of the chronic anal pain syndromes
  • The following diagnostic criteria should be met for the past 3 months with onset of symptoms at least 6 months before diagnosis: Recurring episodes of pain localized to the anus or lower rectum, episodes lasting from several seconds to minutes, absence of anorectal pain between episodes
• Workup: Endoscopy, anorectal manometry, rectal balloon expulsion test, magnetic resonance imaging (MRI) defecography, pelvic floor muscle testing
• Treatment: Biofeedback is the preferred treatment, Botulinum toxin A injection in women, electrogalvanic stimulation (less effective than biofeedback), percutaneous tibial nerve stimulation
  • May consider sacral neuromodulation; inhaled Salbutamol for intermittent anal pain syndrome

Gynecological Aspect

• Pain that is predominantly cyclical is likely to have a gynecological cause
• Pain associated with gastrointestinal & urinary symptoms must be assessed carefully
  • Symptoms suggestive of irritable bowel syndrome (IBS) or interstitial cystitis are frequently seen in women with CPP & these may be a primary cause or a component of CPP or a secondary effect brought about by the efferent neurological dysfunction of CPP

Vaginal & Vulvar Pain Syndrome

• Pain persisting for >6 months in the vagina or the female external genitalia (vulva which includes the labia, clitoris, & vaginal entrance) with no known cause; also called vulvodynia
• Causes of vulvodynia include history of chronic antibiotic use or sexual abuse, hormonal changes, injury or irritation to the nerve or muscle, hypersensitivity to yeast infections or allergies to chemicals or other substances, abnormal inflammatory response to trauma or infection
• Workup: Complete gynecological examination, ultrasound, laparoscopy
• Treatment: Hormonal treatments (combined oral contraceptives, progestins, GnRH agonists, or Danazol) should be considered for pain that seems to be cyclical in nature
  • Laparoscopy for treatable causes
  • Other therapies include a multidisciplinary approach for persistent diseases & psychological treatment for refractory chronic vulvar pain

Endometriosis-associated Pain Syndrome

• Patients with laparoscopically-confirmed endometriosis with chronic or recurrent pain despite appropriate therapeutic strategies & associated with negative behavioral, cognitive, or emotional efects, or sexual, urological, or bowel dysfunction

Dysmenorrhea

• May be considered as a chronic pain syndrome if persistent & associated with negative behavioral, cognitive, emotional or sexual effects

Musculoskeletal Aspect

Coccyx Pain Syndrome (Coccygodynia)

• Pain at the coccyx elicited by its manipulation that is due to pelvic floor hypertonicity & reduced coccyx mobility
• Usually related to prolonged sitting or trauma & is worsened by arising, bending, sitting, defecation, or intercourse
• Workup: Local anesthetic injection produces pain relief
• Treatment: Anti-inflammatory drugs, physical therapy, massage, neuroablation
  • Consider local injection if coccyx is unstable

Pelvic Floor Muscle Pain Syndrome

• A dull pressure or ache that worsens with sitting or lying down & is frequently associated with incomplete evacuation
• CPP can be a form of myalgia due to misuse of the pelvic floor muscles; pelvic floor overactivity & myofascial trigger points may also be involved
• Workup: Test for pelvic floor function & myofascial trigger points, pelvic floor muscle electromyography (EMG) 
• Treatment: First-line treatment is pelvic floor muscle therapy in patients with CPPS
  • Use biofeedback as adjuvant to muscle exercises
  • Treatment by pressure or needling is recommended for myofascial trigger points
  • May consider Botulinum A toxin injection for trigger points & pelvic floor muscle overactivity
  • Management of pain will involve a physiotherapist, a pain doctor, & a psychologist

Neurological Aspect 

Pudendal Neuralgia

• Burning, crushing or electric pain perceived in the perineum from the anus to clitoris or penis
• Related features include allodynia (pain on light touch), dysesthesia (unpleasant sensory perceptions usually but not necessarily due to provocation), hyperalgesia (heightened pain perception after a painful stimulus), & paresthesia (pins & needles)
• Associated with history of pelvic surgery, prolonged sitting, & postmenopausal older women; rarely in trauma (eg birth trauma, fractures, falls), cancer, or cycling
• Patient’s immobility & disability develop muscular aches & pain that lead to involvement of the musculoskeletal system
• Workup: Imaging & neurophysiology may aid in the diagnosis, but investigations are often normal
  • Gold standard is an image & nerve locator-guided local anesthetic injection
• Treatment: Refer to a specialist if a peripheral nerve problem is suspected
  • Treat as for any other nerve injury; utilize available standard regimens to neuropathic pain management

Psychological Aspect

• Majority of patients meet the diagnostic criteria for a persistent somatoform pain disorder in which pain is associated with psychosocial problems or emotional conflict
• Evaluating the link between psychiatric diseases & CPP is difficult; while chronic pain can result in a psychopathology, an existing psychopathologic condition may also affect pain perception & encourage the development of chronic pain
• Women with CPP often have comorbid conditions (eg anxiety, depression, substance abuse, or sexual problems)
  • Anxiety may be related to the patient’s fears of an undiagnosed pathology as the cause of pain, (eg cancer), & to uncertainties regarding treatment possibilities & the probable prognosis if treated or not
  • Depression may be attributed to pain or its impact
• Workup: Assess patient’s psychological history & pain-related beliefs & behavior
  • Asking the patient’s perception about pain gives the chance for appropriate information & reassurance
• Treatment: Offer psychological interventions in combination with other modalities (eg medical & surgical treatments)
  • Psychologically-informed physical therapy results in improved pain & function
  • Exercise & cognitive behavioral therapy combined with Medroxyprogesterone acetate reduces pelvic pain in majority of women

Sexual Aspect

• Sexual dysfunction is prevalent in patients with pelvic pain syndrome, is multifactorial, & may be related to depression, use of antidepressants, relationship satisfaction, & many others
  • In men, the common complaints are erectile & ejaculatory dysfunctions
  • In women, dysfunctions that are most reported are sexual avoidance, dyspareunia, & vaginismus
• Patients with symptoms suggesting CPPS should be screened for sexual abuse
• Workup: Inquire regarding psychiatric history & history of relationship, sexual functioning, & negative experiences eg abuse
• May use the Female Sexual Function Index (FSFI) & the International Index of Erectile Function (IIEF) questionnaires
• Treatment: Refer to a specialist for management of sexual dysfunction
  • Utilize a biopsychosocial approach in pain treatment
  • Offer partner treatment & behavioral strategies for coping with sexual dysfunction
  • Refer for pelvic floor muscle physical therapy

Urological Aspect

Bladder Pain Syndrome

• Pain, discomfort, or pressure perceived to be related to the bladder with at least daytime &/or nighttime increase in urinary frequency, aggravated by food or drink, relieved by voiding but returns soon, & other well-defined pathologies excluded
• Workup: Urinalysis, urine culture, uroflowmetry, general anesthetic rigid cystoscopy with hydrodistention, bladder biopsy, pelvic floor muscle testing, potassium chloride (KCl) sensitivity test, micturition diary, Interstitial Cystitis Symptoms Index (ICSI) score list
• Treatment: Hydroxyzine, Amitriptyline, Nortriptyline, Pentosan polysulfate, Cimetidine, Cyclosporin A
  • Intravesical therapies: Pentosan polysulfate sodium (PPS), Dimethyl sulfoxide (DMSO), Botulinum toxin A plus hydrodistention, Hyaluronic acid, Chondroitin sulfate, Heparin, Lidocaine with Sodium bicarbonate
  • Other therapies include neuromodulation, bladder training, physical therapy, psychological therapy, sacral or pudendal nerve stimulation

Prostate Pain Syndrome

• Pain perceived in the region of the prostate without lower urinary tract pathology for at least 3 months out of the past 6 months
• Workup: Urine culture, uroflowmetry, transrectal ultrasound of the prostate, pelvic floor muscle testing, National Institutes of Health-Chronic Prostatitis Symptom Index (NIH-CPSI) scoring list
• Treatment: Recommended are alpha blockers (Alfuzosin, Doxazosin, Silodosin, Terazosin) & single-use antibiotics (quinolones or tetracyclines) if duration is <1 year, & high-dose Pentosan polysulfate, may consider nonsteroidal anti-inflammatory drugs (NSAIDs)
  • Other therapies include phytotherapy, perineal extracorporeal shock wave therapy, electroacupuncture, percutaneous tibial nerve stimulation, psychological treatment focusing on pain

Scrotal Pain Syndrome

• Episodic pain that is persistent or recurrent within the scrotum, has symptoms suggestive of a urinary tract or sexual dysfunction, & is without obvious local pathology
  • Pain is not in the scrotal skin but perceived to be in its contents eg testes, epididymis, vas deferens, or the inguinal nerves
  • Usually associated with the nerves in the spermatic cord
  • Includes testicular pain syndrome, epididymal pain syndrome, post vasectomy pain syndrome & pain after inguinal hernia repair
• Workup: Semen culture, uroflowmetry, scrotal ultrasound, pelvic floor muscle testing
  • Scrotal ultrasound has limited value in diagnosing cause of pain
• Treatment: Based on the principles of chronic pain syndrome therapy
  • Consider also physiotherapy for an overactive pelvic floor
  • Surgical therapies include spermatic cord microsurgical denervation, epididymectomy if denervation was not beneficial, orchiectomy if all other therapies have failed
  • There may be lower risk for pain with open inguinal hernia repair compared to laparoscopic repair

Urethral Pain Syndrome

• Episodic pain that is chronic or recurrent, perceived in the urethra, is without obvious local pathology, & may occur in both men & women
• May be a part of bladder pain syndrome; urethral pain may be a neuropathic hypersensitivity after a urinary tract infection
• Workup: Uroflowmetry, pelvic floor muscle testing, micturition diary
• Treatment: There is no specific treatment for urethral pain syndrome; patients are recommended to be treated in a multidisciplinary & multimodal program
  • For distressed patients, may consider a pain-relevant psychological therapy

• A complete & detailed history should include organ-specific inquiries on gastroenterologic, gynecologic, musculoskeletal, urologic & neurologic symptoms including a thorough review of systems of infectious, endocrine, psychiatric & sexual disorders
  • Ask for previous radiation or medical treatment, recent pregnancy, history of pelvic surgery or infections or use of intrauterine device (IUD), history of physical or sexual abuse, history of alcohol & drug abuse
    • A history of sexual abuse as a child may lead to the development of depression, anxiety or somatization which makes him or her vulnerable to developing chronic pelvic pain (CPP)
  • Rule out malignancy or other severe systemic diseases by noting “red flag” symptoms eg rectal bleeding, pelvic mass, weight loss, suicidal ideation, new bowel symptoms at >50 years of age, & in women postcoital bleeding, new pain after menopause, irregular vaginal bleeding >40 years of age
• Characterize pain according to its quality, duration, location, intensity, timing, aggravating or alleviating factors, trigger situation, relation to movement & posture, association with menses, sexual activity, urination, defection, effect on quality of life
  • Visceral pain is dull, crampy, or poorly localized, somatic pain may be dull or sharp, & neuropathic pain may be burning, stabbing, electrical, or paresthesia-like
• CPP often has more than one component & goal of assessment is to investigate contributing factors related to CPP including environmental, social (social isolation, family problems), cognitive (pain assessment, pain control perception), emotional (symptoms of anxiety or depression), behavioral (level of activity & medication use), & physical (tiredness, sexual dysfunction, sleep problems)
  • May make use of the history & physical examination forms from the International Pelvic Pain Society
  • Psychological comorbidities may also be detected using other validated symptom-based tools

• If pain is intermittent, patient is best examined while in pain in order to look for pathological conditions & to be able to reproduce the pain to identify physical contributors
• Observe for general demeanor, mobility, & posture when patient walks into the clinic
• Check the back for trigger points, pelvic asymmetry, sacroiliac tenderness
• Abdominal exam includes checking for skin lesions, hypersensitivity around scars, or trigger points (areas where light localized pressure elicits a vigorous pain response)
  • A positive Carnett’s sign (examiner’s finger on the tender area of the patient’s abdomen while patient raises both legs off the table) with an increase in pain indicates a myofascial cause of the pain
  • Pain lessening on the head-raise test indicates an intraperitoneal cause of pain
• Individual pelvic structures are then examined to differentiate sources of pain & localized areas of tenderness are identified to help guide specific therapy
  • Palpation of the back, outer pelvis, & pelvic floor may show trigger points indicating a myofascial component
    • Inspect also for enlargement, distortion, prolapse, discoloration, or infectious sequelae
  • External genitalia exam, pelvic examinations with a single-digit one-hand exam followed by a bimanual exam in women, & a rectal exam to check for nodularity, masses, or point tenderness

• Initial workup includes a complete blood count (CBC), erythrocyte sedimentation rate (ESR), urinalysis with urine culture, beta-human chorionic gonadotropin (hCG), vaginal & endocervical swabs, semen & stool culture
  • Offer screening for sexually transmitted infections to sexually active patients with chronic pelvic pain (CPP)
• Cancer screening appropriate to patient’s age & related risk factors should be done
  • A serum CA-125 should be measured if the following symptoms occur >12 times per month: Early satiety, bloating, pelvic pain, urinary urgency or frequency
• The history & physical examination may point toward a specific diagnosis for which special testing may be indicated
  • Other special diagnostic tools or methods are reserved for specific questions but should not be included in the routine diagnostic workup

• Eg Ultrasound, magnetic resonance imaging (MRI), computed tomography (CT) scan
• Should only be performed when clinically indicated
• Transvaginal pelvic ultrasound in women is part of the initial diagnostic workup if history & physical exam are unrevealing of a specific diagnosis
  • Useful in evaluating pelvic masses
• MRI & CT scan are not routinely done but can help evaluate ultrasound findings
  • MRI may be helpful if a clear cause for pain was not elicited on examination of patient
  • MRI may be used in assessing palpable nodules in the pelvis or rectovaginal disease