Permanent Sterilization with Dr. Aparna Sridhar

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Here’s the RoshReview Question of the Week:

​​A 38-year-old woman presents to your office seeking counseling. She has four children, and she would like to have a tubal sterilization procedure. You explain to your patient the risks and benefits of bilateral salpingectomy compared to tubal ligation. Which of the following is this patient at risk for if she undergoes this procedure?

Check out the correct answer by following the links above!

Today we welcome back Dr. Aparna Sridhar, associate professor at UCLA Health, to talk about permanent sterilization counseling. You may remember her from our previous episode about combined hormonal contraceptives.

Dr. Sridhar gives us an awesome overview of all forms of permanent sterilization, including male permanent sterilization (vasectomy).

Thrombocytopenia in Pregnancy

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Here’s the RoshReview Question of the Week!

A 23-year-old primigravid woman at 25 weeks gestation presents to the prenatal appointment to follow up on lab results. Her platelet count is noted to be 77,000/μL, decreased from 205,000/μL in the first trimester. She reports no abnormal bruising or bleeding. What is the most likely reason for her thrombocytopenia?

Check your answer and enter the QE Exam Giveaway at the links above!

Practice Bulletin 207 is our companion reading this week!

Introduction 

  • Evaluation of thrombocytopenia in pregnancy can be difficult because there are many things that could cause it - not all of them are related to pregnancy 

  • Definition: when platelets are <150,000/microL 

  • So what is normal? 

    • Recall in our very first few episodes that pregnancy can lead to increased plasma volume and not necessarily an equal increase in blood components 

    • This can lead to what appears to be some dilutional effects 

    • In the National Health and Nutrition Examination Survey (NHANES), the mean plt count in pregnancy is as follows: 

      • Non pregnant: 273k 

      • 1st trimester: 251k

      • Second trimester 230k

      • Third trimester 225k 

      • Delivery 217 k 

      • Postpartum (7 weeks) 264k  

  • Definitely suggests that some decrease in platelet count could be expected in pregnancy

What are the causes of thrombocytopenia in pregnancy?

  • Gestational thrombocytopenia - benign and self limited; may even occur in the first trimester, but usually is more common as pregnancy continues; frequency is 5-10% at the time of delivery

    • But remember this is a diagnosis of exclusion! It’s acceptable if there is mild thromboctyopenia, but again, you should look if Plt <100k  

  •  Immune Thrombocytopenia (ITP) - 1-3/10,000 pregnancies, but really low plts only affect a small portion of these

    • ITP is more frequent in pregnancy than in the general adult population but may be because of recognition (checking more frequent CBCs in preg) 

    • Autoimmune condition where antiplatelet antibodies interfere with platelet production and causes destruction of circulating platelets

      • Diagnosis based on exclusion of other causes of thrombocytopenia  

  • Preeclampsia/HELLP Syndrome

    • Remember that it is very rare <24 weeks, and there are usually other signs and symptoms as well like elevated BP, headache, vision changes, etc. As well as other hematologic abnormalities like anemia due to hemolysis (high LDH), and may have abnormal LFTs 

    • We won’t go too much into preeclampsia since we discuss it elsewhere, but always something to keep in mind 

  • Less likely things, but still things to keep in mind 

    • DIC - Disseminated intravascular coagulation

      • There is usually an underlying cause of this such as placental abruption, sepsis, etc 

      • Patients will have bleeding and oozing at IV sites for example 

      • Usually will have low fibrinogen and elevated PT and PTT labs as well  

    • Acute fatty liver of pregnancy 

    • Thrombotic thrombocytopenic purpura (either immune or hereditary)

      • Will usually present with purpura, can have neurological changes, fever, kidney injury, can have elevated BPs and may be confused with PEC 

      • Will have reduced activity of ADMATS13, but this may be a send out lab in most places and will not come back for some time 

      • Will have schistocytes on smear 

    • Lupus 

    • Infection

    • Inherited platelet disorders

So… when should we start to worry?

  • When platelets are: 

    • If platelets are between 100k-150k if there are risk factors present (but usually, do not need work up and can be attributed to gestational thrombocytopenia) 

    • At any point <100k (usually this is beyond the lower end of gestational thrombocytopenia)

      • Plts of <100k only occur in 1% of uncomplicated pregnancies 

      • Remember that there is increased risk of spontaneous bleeding if Plt <20k 

      • Some institutions have cut offs for platelets for ability to give neuraxial analgesia - some places are 70-80k, some places are 100k. Please check with your institution and your patients, because this may require treatment or your patient won’t get an epidural! 

Ok, so that’s a lot of causes… how do I go about figuring out what to do? 

  • Evaluation of the patient

    • Get a good history and physical - this can sometimes help you determine what it is not

      • If patient is well appearing, with no pain, vaginal bleeding, elevated blood pressure or other complaints, it’s usually not going to be something like AFLP, DIC, TTP, or preeclampsia  

      • Look through the patient’s chart: what were their platelets before? What other medical problems? What about new medications? 

      • Any history of lupus, TTP, liver disease, anemia

      • You have the CBC - did the lab do a smear? Are there abnormalities on the smear, like schistocytes? 

      • Most of the time, if you have someone who is sitting in the clinic and appears well, you may have some time  

  • The asymptomatic patient with Plt >100k

    • Usually, plts between 100-150k without other cytopenis or other major clinical findings can be attributed to gestational thrombocytopenia 

    • The other major cause could be ITP, but minor ITP with plt >100k also does not need treatment 

    • Our practice is to check plts monthly to make sure they do not drop below 100k, or below threshold for neuraxial analgesia 

  • The asymptomatic patient with Plt <100k 

    • Review the CBC - make sure there are no other cytopenias; ask for a smear 

    • Evaluate for HIV (usually already done in pregnant patients), as this could also lead to thrombocytopenias 

    • Obtain other coagulation panel like PT, PTT, fibrinogen level 

    • Also obtain CMP - evaluate kidney and liver function 

    • If no obvious signs, it is ok to get hematology involved early

    • More likely to be ITP if <100k, and patients can be treated with steroids or even IVIG in refractory cases if needed 

    • This is to make sure that the platelets do not drop further so that they cannot get neuraxial analgesia or so that they don’t drop too low as to cause issues with bleeding 

  • What if they are symptomatic and <100k?

    • A lot of this is going to depend on their history and physical again - we are usually pretty good at evaluating for preeclampsia - get their vital signs and do your exam

      • Do they have fever? Purpura? 

    • Labs: CBC, CMP, coag panel, HIV, LDH, urinalysis, bilirubin 

    • If you are suspicious of TTP (ie. fevers, kidney injury, neurological changes) 

      • Make sure to get CT head to rule out bleed 

      • ADAMTS13 

      • Hematology consult 

    • Ok to get MFM and hematology involved early 

Management 

  • Treatment for bleeding or severe thrombocytopenia 

    • If Plt are <10k or <20k, there is increased risk of spontaneous bleed 

    • If Plt <20k and severe bleed (ie. intracranial), you should give platelets regardless of the underlying cause of thrombocytopenia (yes, even in ITP if it will get consume)

  • Some platelet thresholds to consider in delivery 

    • Vaginal delivery: 20-30k 

    • C/S: 50k 

    • Neuraxial anesthesia: institution based; ours is 80k; most institutions will have a count between 50-80k 

  • Other considerations 

    • Operative vaginal deliveries are relatively contraindicated if there is severe maternal thrombocytopenia 

    • This is because there is concern that there could also be fetal thrombocytopenia (ie. immune mediated or hereditary) 

    • However, if you must perform an operative delivery, forceps is favored over vacuum 

    • Remember: Just because someone has ITP does not mean they can’t have an operative delivery 

  • Treatment of specific disorders 

    • ITP - steroids or IVIG 

      • Dosing of steroids: prednisone 1mg/kg/day for two weeks followed by gradual taper; may need 2 weeks to see peak effect (usually 1-4 weeks for peak) 

      • IVIG should be given at least 1 week in advance to allow for maximal efficacy and platelet count retesting if trying to raise platelet counts for epidural 

      • If refractory, other methods in pregnancy are not well studied, and you should have a conversation with your MFM and hematology colleagues 

    • TTP - plasma exchange

    • Preeclampsia or DIC due to abruption: delivery! 

  • What about fetal testing of platelets?

    • There isn’t really evidence to suggest we should test fetal platelets (ie. either via PUBS or from fetal scalp during labor

    •  We won’t discuss fetal/neonatal thrombocytopenia here, but just some brief reasons to test neonatal platelets: 

      • Maternal ITP 

      • Neonatal thrombocytopenia in previous pregnancy (concern for NAIT, though this is usually not associated with maternal platelet issues) 

      • Congenital anomalies associated with thrombocytopenia 

      • Bleeding or petechia on the infant 

      • Neonatal infections (ie. CMV, rubella) 

Soft Markers for Aneuploidy

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Here’s this week’s RoshReview Question of the Week!

A 38-year-old G1P0 woman at 20 weeks gestation presents to the clinic for her anatomy ultrasound examination. She underwent a first-trimester screen, which showed a borderline nuchal translucency of 3.1 mm. Which one of the following isolated ultrasound findings confers the greatest risk for trisomy 21?

Check out the links above to see if you answered correctly. Also, you can enter for a chance to win a Rosh Review Qualifying Exam (“written boards”) QBank!

Check out the SMFM Consult Series 57 for excellent companion reading!

What are the ultrasound soft markers, and why do we care? 

  • In the era of cell-free DNA, you might ask: what is the utility of soft markers? Aren’t they poor predictors of aneuploidy?

    • Originally introduced to improve the detection of Down syndrome over that of just age-based or serum-based screening 

    • While it is true that each isolated soft marker may be poor predictors, if we see multiple soft markers, that does improve sensitivity  

    • There may also be some misunderstanding of soft markers seen on ultrasound, and so the purpose here is to review some of these soft markers in the setting of cfDNA and discuss next steps 

  • Remember: patient’s baseline risk should not limit screening options, and cfDNA should be offered to all per ACOG and SMFM 

What are the first steps when you see a soft marker?

  • Make sure that the soft marker is truly isolated - look for other soft markers, fetal growth restriction, or other anomalies 

    • If you feel that your office is not equipped to do this, can refer to MFM to have a level II ultrasound performed - this is of course a discussion with the patient, and not all patients will want further evaluation 

  • Look at the patient’s history: 

    • What is their baseline risk? (age, family history, history of aneuploidy) 

    • What are their previous aneuploidy screening results? Did they have any? 

  • Ok, so I see one of the soft markers, what do I do next?

    • First of all, have they had cfDNA?

      • Most of the time, there is not much to do after that (again: ISOLATED soft marker) 

      • This is because with cfDNA, the posttest probability of a common aneuploidy (ie. Trisomy 21) of negative cfDNA is very low - it is lowered by 300x for trisomy 21

        • Per the consult series, the residual risk of a 35-yo woman, whose age related risk of Down syndrome is 1/356 is reduced to <1/50,000 after a negative cfDNA result  

    • But what if they didn’t have cfDNA? 

      • If they have had negative serum screening, also ok, no need to do further testing at this time 

        • The detection rate of serum screening test for Down is still high, about 81%-99% depending on the test 

      • If no screening at all, counsel about noninvasive aneuploidy testing - not all patients will want screening 

    • Remember: there isn’t an established cut off residual risk when there is recommendation to do diagnostic testing 

      • Many labs will establish a cutoff of 1:250 or 1:300 

    • SMFM does not recommend diagnostic testing for aneuploidy only for evaluation of isolated soft marker following negative serum or cfDNA screening result 

The Soft Markers (all photo credit to Radiopedia)

Tubal Ectopic Pregnancy Management

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Here’s the RoshReview Question of the Week!

A 24-year-old G2P1 woman presents to the emergency department with right-sided pelvic pain and vaginal spotting. She has been trying to conceive and her last menstrual period was 8 weeks ago. The patient reports her left fallopian tube was removed 3 years ago due to hydrosalpinx. Her beta-human chorionic gonadotropin is 6,700 mIU/mL. On ultrasound, there is no intrauterine pregnancy identified. Fetal heart tones are detected in the right fallopian tube. There is a minimal amount of free fluid noted in the posterior cul-de-sac. What is the most indicated intervention at this time?

Check to see if you got it right at the links above!

While we have reviewed the workup of the early unlocated pregnancy and diagnosis of ectopic pregnancy previously with Dr. Cleary, and talked about the unusual problem of cesarean ectopic pregnancy before on the show, somehow we missed the management of the regular tubal ectopic! 

ACOG PB 191 is a great resource for all things ectopic pregnancy and important companion reading for the podcast today.

Background Info

  • Ectopic pregnancy represents about 2% of reported pregnancies, but this is likely an undercall as not all ectopic pregnancies are reported.

  • Ruptured ectopic accounts for a significant cause of maternal morbidity and mortality - 2.7% of maternal deaths in 2011-2013 were attributable to ruptured ectopics. 

  • Fallopian tube is the most common location for an ectopic (90%), but as we’ve talked about before, these can be anywhere – abdomen (1%), cervix (1%), ovary (1-3%), and cesarean scar (1-3%). 

    • Can also co-occur with an intrauterine pregnancy – heterotopic pregnancy.

      • Naturally conceived: 1 in 4,000 to 1 in 30,000

      • IVF: as high as 1 in 100

Risk Factors for Ectopic Pregnancy

  • 50% of those who receive a diagnosis don’t have any known risk factor. 

  • Risk factors that can be present include:

    • Prior ectopic - recurrence risk is about 10% after 1 prior, 25% after 2 prior

    • Prior fallopian tube surgery / damage

    • History of PID or ascending pelvic infection

    • ART - tubal infertility, multiple embryo transfer, infertility in general

    • Cigarette smoking

    • AMA > 35yo

  • Contraception and ectopic risk:

    • Those using IUDs are at lower risk overall of ectopic because IUDs are highly effective at preventing pregnancy in general.

      • However, in those who do become pregnant with an IUD in place, up to 53% of these pregnancies are ectopic.

    • OCP use, emergency contraceptive failure, previous pregnancy termination, pregnancy loss, and cesarean delivery have not been associated with increased risk of ectopic pregnancy. 

Confirming a Diagnosis of Ectopic Pregnancy

  • We covered this pretty extensively in our episode with Dr. Cleary - there we do a great job of talking you through the “pregnancy of unknown location” workup, especially when you see a patient in ED/triage with bleeding/pain and early pregnancy. 

  • We won’t go through it all again today, as we want to focus primarily on management, but a few big points:

    • Trending bHCG every 48 hours helps to determine if the pregnancy is normal or abnormal.

      • If a bHCG is higher than the DZ and you don’t see anything - that’s a good indicator of an abnormal pregnancy, with 50-70% being ectopic. 

    • Transvaginal ultrasound to assess the uterus and adnexae will help you identify any unusual mass that might be an ectopic.

  • So let’s start from the point of abnormally rising bHCG, so we know our suspicion is for an abnormal IUP versus ectopic. What options are available?

    • Expectant Management

      • We can continue to trend bHCG in a stable patient, particularly in the case of highly desired pregnancy or low bHCG values that may need more time to declare itself.

      • These patients should be counseled strongly about presenting for care should they experience significant bleeding, severe pain, or other symptoms worrisome for ectopic rupture. 

    • Uterine Aspiration

      • If we are reasonably certain the pregnancy is abnormal, a uterine aspiration can be done to determine if the pregnancy is intrauterine or not.

        • The aspirate can be sent to pathology or floated to quickly identify chorionic villi – if found, then you know it was an IUP.

        • If villi are not found, then hCG should be measured again at 12-24 hours after aspiration.

          • If the hCG drops at least 10-15%, it was likely successful aspiration of a failed IUP; however, drops of 50% or greater are more indicative. 

            • Serial hCG should be followed to zero in these patients since no pathology was identified.

          • If the hCG is plateaued or rising, then the pregnancy is ectopic, and the patient will need additional treatment. 

    • Proceeding Directly to Treatment

      • The PB mentions there is debate whether aspiration is necessary before treating an abnormal pregnancy with methotrexate.

        • On one hand, confirmation of the diagnosis with the procedure helps avoid unnecessary exposure to MTX.

        • On the other hand though, the procedure adds at least 12-24 hours of additional time (and potential ectopic rupture) before giving treatment.

      • ACOG notes that the risk of rupture during this time period overall is low, and that presumptive treatment with MTX doesn’t confer cost savings

        • However, it reserves the choice for patients and their physicians after discussion of risks and benefits.

Medical Treatment of Ectopic Pregnancy

  • The standard, as we’ve mentioned, is methotrexate.

    • Folate antagonist binding to catalytic site of dihydrofolate reductase → inhibits synthesis of nucleotides and amino acids, thus inhibiting DNA synthesis, cell repair, and cell replication.

    • MTX affects all rapidly-proliferating cells because of it – marrow, mucosa, cancers, and trophoblasts. 

      • This is helpful to keep in mind to thinking about side effects of MTX:

        • Nausea, vomiting

        • Stomatitis 

        • Abdominal pain

        • Alopecia (rare)

        • Pneumonitis (rare)

      • There are no recommended alternatives to MTX for medical therapy.

  • Contraindications to MTX:

    • Absolute:

      • Intrauterine pregnancy

      • Chronic liver or kidney disease

      • Bone marrow dysfunction (anemia, blood dyscrasia, thrombocytopenia, leukopenia).

      • Active GI disease (i.e., PUD) or respiratory disease.

      • Breastfeeding

      • Hemodynamically unstable patient.

      • Inability to participate in follow up. 

    • Relative:

      • Cardiac activity in the ectopic pregnancy

      • High hCG concentration (>5000 mIU/mL)

        • Reviews demonstrate a failure rate of 14.3% or higher at this concentration (vs 3.7% when under 5000 mIU/mL)

      • Ectopic size greater than 4cm on TVUS

      • Refusal to accept blood transfusion

  • MTX Regimens:

    • ACOG in the PB 191 mentions three primary regimens: single-dose, two-dose, and fixed multi-dose.

  • Single-dose is the simplest but may require additional dose in up to 25% of patients.

  • Two-dose has high success rate with similar monitoring to single-dose regimen.

    • A recent review article suggested the two-dose protocol was more successful while also exposing patients to only minimal, transient side effects versus single dose, and has higher success rates with higher hCG levels.

  • Multi-dose fixed regimen requires up to 8 days of treatment with alternating MTX and folinic acid for rescue and minimization of MTX side effects.

  • What about surveillance / labs for MTX?

    • Before administration (day 1), you should obtain:

      • bHCG

      • CBC

      • CMP

    • Patients should be counseled about side effects of MTX, and should avoid medications, foods, and supplements that may worsen efficacy

      • Have them stop prenatal vitamins at this time, so the folate doesn’t counteract the MTX!

        • Folate-rich foods and NSAIDs may also decrease the efficacy of MTX.

        • Narcotics, alcohol, and gas-producing foods should also be avoided so as not to mask or be confused with signs of rupture.

        • Patients should also avoid vigorous activity and sex until confirmation of resolution so as not to induce ectopic rupture. 

    • With single and two-dose protocols, you’ll evaluate bHCG again on days 4 and day 7.

      • Success in these protocols is noted with a 15% or more decline between days 4 and 7. 

        • If the decline is less than that, or bHCG increases, then an additional dose of MTX should be administered on day 7. 

        • With repeat doses of MTX, it’s reasonable to consider repeat laboratories to evaluate for any toxicity. 

      • bHCG should continue to decline to zero, and should be followed at least weekly once the initial 15% decline is noted.

        • Resolution can take up to 8 weeks, though average:

          • Two dose: 25.7 +/- 13.6 days

          • One dose: 31.9 +/- 14.1 days

    • Finally, patients should consider avoiding pregnancy for at least 3 months after the last dose of MTX.

      • Studies have found MTX still detectable in cells up to 116 days past exposure. 

      • However, limited evidence also suggests that anomalies and pregnancy loss is not elevated in those who become pregnant shortly after MTX exposure.

    • MTX does not have a measurable effect on fertility.

Surgical Therapy

  • For patients who do not desire MTX or are not candidates, surgical therapy is the other option. Surgical therapy is also needed for the patient with hemodynamic instability or symptoms of rupture/intraperitoneal bleeding. 

    • Can also be reasonably considered in stable patients with an indication for another procedure, like salpingectomy for sterilization or hydrosalpinx removal. 

  • Surgeries available include salpingectomy (removal of the tube) or salpingostomy (opening the tube).

    • These are generally accomplished laparoscopically – laparotomy is reserved for unstable patients or patients with large bleeding and compromised laparoscopic visualization. 

  • Surgery may be more effective than medical therapy and requires less follow up, but does expose patient to surgical risk. 

  • Salpingectomy is technically easier to perform, and that’s likely how most of us have trained.

    • Salpingostomy can be considered in patients with desired fertility and damage to the contralateral fallopian tube, and would require ART for future pregnancy.

    • To perform, typically you make an incision along the long axis of the tube over the ectopic, and resect the pregnancy tissue. 

      • Achieving hemostasis is rather tricky in these cases, and may additionally cause damage to the tube. The tube is usually left to heal on its own and not sutured as this may crimp the tube and cause further damage. 

      • Because you may not resect all of the pregnancy tissue at salpingostomy, bHCG monitoring after salpingostomy is needed to ensure complete resolution.

      • MTX may also be given prophylactically if incomplete resection is considered. 

Expectant Management

  • We bet you weren’t expecting this one… but ACOG does mention there may be a role for expectant management of ectopic.

  • They note that candidates for EM should be:

    • Asymptomatic

    • Objective evidence of resolution (i.e., plateau or decreasing bHCG)

    • Accepting of potential risks after counseling, including tubal rupture, hemorrhage, emergent surgery.

      • EM should be abandoned if hCG insufficiently decreases or begins to rise or with any suspicion for tubal rupture. 

  • If initial hCG is under 200 mIU/mL, 88% of patients will have spontaneous resolution.

  • In a single small RCT of patients with hCG < 2000 mIU/mL, EM was not associated with lower treatment success than single dose MTX (59% vs 76%).

Interstitial Cystitis, feat. Dr. Edward Kim

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Here’s the RoshReview Question of the Week!

A 41-year-old woman presents to your office for dysuria. She states that she has been having persistent urinary urgency and frequency for the past six months. She reports discomfort with bladder filling, pain with urination, and relief after voiding. A review of her history reveals normal fluid intake. A workup for pelvic pain performed by her primary care provider and gastroenterologist was negative. On physical exam, you note suprapubic tenderness. Her urinalysis and culture are negative. Which of the following is the best therapeutic option?

Check out if you answered correctly at the links above!

Today we welcome Dr. Edward Kim to the podcast. He is a urogynecology fellow at the University of Pennsylvania, performing research on a challenging topic: interstitial cystitis. Dr. Kim is looking to recruit more patients into a study on quality of life and patient education in IC — if you have questions or someone to refer, let us know by emailing us or contacting us with the form on the website!

Overview

  • IC: kind of a misnomer!

    • There is no conclusive evidence that there is an inflammation nor distinct pathology in the bladder interstitium.

    • Contemporary thinking: chronic pain condition related to or perceived to be originating from the bladder.

      • Newer terminology has been proposed: bladder pain syndrome.

      • In clinical practice, some patients seem to prefer ‘interstitial cystitis’ because to them is sounds more like a more medical diagnosis and they don’t want to be labeled as having a pain syndrome. So IC/BPS are used interchangeably.

Epidemiology

  • Can affect men and women but more common in women.

    • High prevalence in age 40s.

    • Don’t know the precise prevalence given complexity of syndrome.

Clinical Presentation

  • Variable

  • Persistent urinary urgency, urinary frequency, and pain or discomfort related to voiding.

    • Note that we say pain or discomfort. Some patients describe what they’re feeling as discomfort and not pain.

    • Classic: patient with these symptoms who had been treated multiple times for urinary tract infections despite having negative urine cultures. They also may report going to the bathroom frequently or spending a lot of their day on the toilet to relieve their urgency and discomfort or pain.

      • Many of these patients may also have associated conditions like irritable bowel syndrome, fibromyalgia, and pelvic floor muscle dysfunction.

      • They also may have concurrent psychiatric comorbidities such as depression or anxiety.

Diagnosis

  • American Urological Association: “An unpleasant sensation (pain, pressure, discomfort) perceived to be related to the urinary bladder, associated with lower urinary tract symptoms of more than six weeks duration, in the absence of infection or other identifiable causes.”

    • In other words, IC is a clinical diagnosis and diagnosis of exclusion.

    • Differential diagnosis should include:

      • Infection

      • Overactive bladder

      • Bladder or urethral cancer, gynecologic cancer

      • Uterine fibroids with compression effect on the bladder

      • Bladder stone

      • Bladder diverticulum

      • Foreign material such as synthetic mesh or suture

      • Neurologic conditions that may cause urinary retention in particular

      • Other chronic pelvic pain conditions such as endometriosis.

    • This is why it’s important to perform a thorough history and physical and obtain a post void residual and urine tests as an initial evaluation to rule out these other etiologies.

  • Hunner lesions can be seen on cystoscopy.

    • They are specific for IC, but they only are seen in about 10% of patients with IC.

  • Potassium sensitivity test KCl is instilled into the bladder.

    • This is not performed anymore due to its low sensitivity and specificity and also it’s very painful!

  • Urodynamics is not typically used to diagnose IC but it can be done to rule out other etiologies.

Treatment

  • Management strategy is multi-faceted.

  • First-line treatments are patient education, behavior and diet modification, and general stress management.

    • There is good evidence behind teaching patients bladder retraining where they learn to increase the interval between each voids.

    • Avoidance of things like artificial sweetener, caffeine, alcohol, spicy food, citrusy or acidic foods can help with symptoms.

      • Doesn’t require elimination, but helps to make informed decisions about diet.

    • Applying heat or ice packs to the suprapubic or perineal regions can also be helpful.

    • Pelvic floor muscle tenderness or dysfunction on exam —> consider pelvic floor PT.

  • Second-line treatments include oral medications and bladder instillation.

    • PRN medication is usually pyridium or over the counter AZO.

      • Warn patients that their urine will turn orange and may stain clothing.

    • Daily medications, the most commonly used ones are: amitriptyline, hydroxyzine and pentosan polysulfate sodium (Elmiron).

      • Note that Elmiron is the only FDA approved medication for IC. However, use of Elmiron has been associated with macular eye disease. Thus, in 2020, the FDA inserted a warning label to reflect this and use of Elmiron has been declining.

      • Between amitriptyline and hydroxyzine, currently there is more data on amitriptyline. Some patients find amitriptyline helpful in controlling their symptoms but some cannot tolerate its sedative and anticholinergic side effects.

    • If there is inadequate response to medications, then bladder instillation can be considered. This involves instilling a mixture of local anesthetic, heparin, DMSO, etc. via a catheter. Usually this involves repeated treatments.

  • Third-line treatment is hydrodistention.

    • Hyper-distention of the bladder under anesthesia for about 10 minutes and emptying the bladder.

      • The thought is that sensory nerves in the bladder are disrupted due to the hyper distention.

      • For patients who see prolonged and significant benefit, repeat treatments are considered.

    • If on cystoscopy Hunner lesions are found, they can be addressed with cautery, resection or injection with steroids.

  • Fourth-line treatments include neuromodulation using Botulinum toxin A injection into the bladder and sacral neuromodulation.

    • These techniques have been used for overactive bladder and neurogenic bladder but recent clinical trials have reported efficacy for interstitial cystits. 

  • Fifth-line treatment is cyclosporine A. Use is limited due to its side effects and paucity of convincing data.

  • Sixth-line and last resort is surgical diversion of the bladder with or without cystectomy.

    • Fortunately, patients seldom have to go past fourth-line treatments. As with any chronic pain condition, it is a difficult journey for many of them and it is critical for providers to listen and empathize with them.