Chong-min Chin

Consultant Urologist, Department of Urology

Changi General Hospital

Singapore

Interstitial Cystitis (IC) and Urethral Syndrome (US) are entities based on the symptom complex of bladder pain and irritative voiding complaints, but with a negative urine culture. Together with Chronic pelvic Pain syndrome (CPPS), the diagnosis is a clinical one made through exclusion of known diseases because of the absence of pathognomonic findings. Needless to say, these syndromes are rife with many pathologic postulates and diverse treatment modalities.

IC is the most researched since Hunner described his cystoscopic findings back in 1915. Because of ambiguity in identifying such patients, the National Institute of Arthritis, Diabetes, Digestive & Kidney Diseases (NIADDK) laid down a consensus criteria to standardise the definition of IC An interstitial Cystitis database (ICDB) has even been set up to study the IC population. Essentially, epidemiologic data has shown a preponderance ratio of females by 10:1, with a median age onset of 40 years and median duration of symptoms of 8 years. Bladder ulcerations were seen in less than 20% of patients. Of interest are the low incidence of diabetes (4%) and a high hysterectomy incidence of 44% in this population. Associated diseases are allergies (41%), fibromyalgia, migraine, endometriosis, SLE, IBS and even Sjogren’s syndrome. The etiologic theories are: infection with fastidious organisms, detrusor mastocytosis, defective epithelial permeability, urine abnormalities, neuropeptide-induced inflammation, increased sympathetic discharge, autoimmunity, and hormonal milieu given the predominant female sufferer. As this disease is primarily based on exclusion, it is recommended the evaluation includes a thorough history, physical examination, urine culture & cytology, urodynamic evaluation (to determine compliance, capacity plus exclude detrusor instability) and cystoscopy-under-anaesthesia with hydrodistension up to 80 cm H₂0 for 1-2 minutes followed by re-distension & bladder biopsy. Glomerulations are the typical post-distension findings, but this is not specific for IC alone and must be taken in concordance to the clinical criteria. Treatment is usually empirical because of the nature of the symptomology in the absence of definite causative factors. It is often quoted that "there is no sure cure for IC". Nor is their single effective modality. The initial therapeutic approach is hydrodistension done during the first cystoscopy and subsequent "watchful-waiting". This is because there will be a group of patients who find their symptoms tolerable with just counselling and education. Medical therapy abounds and the drugs with reasonable success are: polysulfate (Elmiron), amitriptyline, hydroxyzine and nifedipine. The next line is intravesical therapy with DMSO, chlorpactin WCS 90, heparin, capsaicin, hyaluronic acid and BCG. Interestingly, up to 50% of patients go into spontaneous remission even with unrelated treatment. Surgery is reserved for the recalcitrant cases. However, bladder augmentation, supratrigonal substitution cystectomy and urinary diversion have all had their share of failures, leaving supravesical diversion with cystectomy as the ultimatum.

US was first coined in 1949. Again, it refers to the female patient with non-specific irritative symptoms but without demonstrable infection, much akin to prostatodynia in men. Because of the tendency to chronicity and its diagnosis by exclusion, it has been considered to be an overlap of IC. Before labelling a female to have US, local infectious processes like vaginitis and genital herpes must be excluded. A proper pelvic examination must be done to exclude definite urethral lesions like urethral caruncle, urethritis, and urethral stenosis. A thorough gynaecological examination is also crucial, as female hypospadias has been reported to be a cause. The problem with exclusion criteria becomes even more difficult when one includes pelvic floor hyperactivity, striated sphincter spasticity and estrogen deficiency as possible functional causes. Hence, this term is so ill defined that management is largely empirical and non-evidence based. Many give a short course of "best guess" antibiotics assuming that the "negative" cultures may not be reflective. Alpha-blockers and diazepam are given if a functional disorder is suspected. Urethral dilation is done if mechanical stenosis is suspected. However, because of lack of objectivity of definition, assessment and improvement criteria, it is hard to conclude what really works for US.

CPPS afflicts women in the reproductive age group and refers to non-menstrual pain of long-standing nature, severe enough to cause disability and distress. Like US, there is no standard definition for this condition and it is made through exclusion of known diseases like endometriosis, pelvic inflammatory disease, pelvic adhesions and irritable bowel syndrome. The current pathologic aetiologies are pelvic venous congestion, post-hysterectomy ovarian remnants, occult UV prolapse and pelvic joint syndrome and even sexual abuse. Investigations include transvaginal color Doppler ultrasonography and Laparoscopy. Relief has been reported with progestogen therapy, and embolotherapy for ovarian/pelvic varices

Because these pain syndromes are poorly understood, there is a strong suspicion of psychosomatic disorder. The sufferers often have a long history and seen many doctors. Counselling, psychotherapy and multi-disciplinary approach are an integral part of the treatment. There is a general opinion that the incidence of these syndromes, especially IC, is not so common here in Asia. Is it because of under diagnosis, the sociocultural factors, homeopathic medical practice or a true low occurrence related to the genetic make-up and diet of Asians? This may be the challenge for future research.