Multifetal Gestation

Multifetal gestations are on the rise: twins now represent 33/1000 birth, and triplets or higher order gestations represent 1.53/1000 births. This is likely due to increased maternal age at conception and increased use of assistive reproductive medications and technologies. Certainly a multiple gestation pregnancy is exciting for patients, but convey a multitude of other risks for both maternal and fetal morbidity in pregnancy. Higher rates of almost anything you can imagine, including PIH/preeclampsia (RR 2.6 twins compared to singletons), placental abruption, HELLP, gestational diabetes, hyperemesis, anemia, hemorrhage, CD, PPD, cholestasis, PUPPS are elevated.

Most commonly preterm birth and its associated neonatal morbidity are encountered with these higher order pregnancies. Women with multifetal gestation are 6x more likely to deliver preterm and 13x more likely to deliver before 32 weeks compared to women with singleton gestation. Twins deliver on average around 35-36w and triplets around 32-33w. Furthermore, compared to gestational age matched premature singleton neonates, those in multifetal gestation have worse outcomes, higher rates of stillbirth, neonatal death, intraventricular hemorrhage, periventricular leukomalacia, and cerebral palsy And unfortunately, there are no strategies that exist to reliably mitigate this risk, including progesterone, cerclage, or pessary. However, many ongoing trials seek to answer this question. Multifetal reduction can help to mitigate these risks in higher-order gestations.

Diagnosing Multiple Gestation

The components of chorionicity and amnioniticity are ideally noted in a first or early second trimester sonogram (best at 10-14 weeks).

  1. Super important as this dictates antepartum management and delivery recommendations! 

    1. Dating in spontaneously conceived twins: if there is a CRL discordance, most radiologists would date pregnancy with larger CRL in order to not underdiagnose FGR (ART use transfer/IUI dating).

  2. Chorionicity (fetal side of uteroplacental interface) and amnionicity (sac with amniotic fluid)

  • Fraternal twins: dizygous 70% different sperm and different egg, same genetic relationship as any other set of siblings born at different ages -> dichorionic gestation (which implies diamnionicity as well).

  • Monozygous 

    1. Split within 3 days: dichorionic (20% of monozygous)

    2. Split 4-8 days: monochorionic / diamniotic (70%)

    3. Split 9-12 days: monochorionic / monoamniotic (1% of all monochorionic twins) 

    4. Split 13(+): conjoined (unlucky 13)

  • Rule of thumb, monochorionic always higher risk than dichorionic.

  1. Diagnosis: going back to episode 68 OB ultrasound

    • Dichorionic gestation can be diagnosed by any of the following: two distinct placentas, different fetal sex, or twin peak/lambda sign vs T-sign.

“Lambda” sign for dichorionic gestation. The OBG Project / Nevit Dilmen Creative Commons Attribution-Share Alike 3.0 license https://creativecommons.org/licenses/by-sa/3.0/deed.en

“Lambda” sign for dichorionic gestation. The OBG Project / Nevit Dilmen Creative Commons Attribution-Share Alike 3.0 license https://creativecommons.org/licenses/by-sa/3.0/deed.en

“T” sign for monochorionic, diamniotic gestation. The OBG Project / Nevit Dilmen Creative Commons Attribution-Share Alike 3.0 license https://creativecommons.org/licenses/by-sa/3.0/deed.en

“T” sign for monochorionic, diamniotic gestation. The OBG Project / Nevit Dilmen Creative Commons Attribution-Share Alike 3.0 license https://creativecommons.org/licenses/by-sa/3.0/deed.en

Common Antepartum Considerations for Multiple Gestations

Recommended weight gain

  1. CO 548 weight gain in pregnancy 

    1. Normal weight: 37-54

    2. Overweight: 31-50

    3. Obese: 25-42

Aneuploidy screening

  1. Inherent increased risk with dizygous gestation - two babies, twice the risk!

  2. Serum screening is not as sensitive because analyte levels are estimated by mathematical modeling. In reading serum levels from a multiple gestation, they are essentially “averaged out,” so a genetically normal twin can mask the abnormal level of the twin affected by aneuploidy.

    1. Can add nuchal translucency for increased sensitivity since you can see which twin may have an anomaly.

  3. NIPT (cell free DNA, Mat21 esp) in small studies have high sensitivity and specificity similar to singleton gestation, but the reported cases are small and we likely need larger studies to better get at the true sensitivity and specificity.

  4. As always, diagnostic testing with CVS or amniocentesis most accurate and should be offered. Loss rate about 1-2% for both procedures and slightly higher than in singleton gestation.

    1. Amnio for dichorionic gestation: can sample one sac, inject indigo carmine, then sample second sac to ensure clear fluid and ensure sampling of two separate sacs.

    2. Amnio for monochorionic gestation - if chorionicity has been confidently established, unlikely to have discordance between the two fetuses so might only sample one; although its possible to have genetic discordance due to fetal mosaicism 

Twin growth

  1. Used to have twin growth rates curves, but didn’t pan out in follow up studies. So now we use a singleton growth curve, and note a slow down after 30 weeks.

  2. Should obtain anatomy ultrasound 18-22w, and given increased risk of congenital anomalies reasonable to start with L2 or specialized ultrasound.

    1. Monozygous twins have higher rates of anomalies (especially cardiac), so fetal echoes are recommended.

  3. Growth ultrasounds every 4 weeks measure % discordance. If discordance > 20% may warrant closer evaluation (EFW lg - EFW sm / EFW lg), could be a sign of developing FGR and associated with poor neonatal outcomes.

    1. Monochorionic every 2-3 weeks after 16w.

    2. Dichorionic every 4-6 weeks after anatomy scan in otherwise uncomplicated pregnancies.

  4. Twin gestation with one fetal growth restricted fetus associated with worse perinatal outcomes.

  5. Cord anomalies (basically moot, since they’re already getting monitored for growth):

    1. Velamentous cord insertion 

      • 7-12% twin pregnancies compared to 2% in singleton gestation 

    2. Marginal cord insertion 

    3. Placenta previa

      • Higher incidence likely due to more placental mass 

    4. Vasa previa

      • Systematic review of cohort and case control series found that of 438 cases of vasa previa, 11% in twins.

Specific Monochorionic Considerations 

  1. Diamniotic (US 16-18 weeks)

    • Twin Twin Transfusion Syndrome

      1. 10-15% of mono/di pregnancies due to arteriovenous connections → unequal vascular sharing; by contrast, in 6% of monochorionic pregnancies  

      2. Diagnosis by Quintero staging 

        1. Stage 1: oligo/poly DVP.

        2. Stage 2: absent bladder in donor twin.

        3. Stage 3: abnormal doppler findings 

          1. UA, DV, UV

        4. Stage 4: hydrops

        5. Stage 5: death of one or both twins  

      3. Stage can remain stable, regress, or progress (sometimes rapidly).

      4. Earlier the diagnosis more severe the disease

      5. Treatment is laser ablation of anastomoses or amnioreduction 

      6. Monitoring:

        1. Weekly AFI 

        2. Every 3-4 weeks growth scan.

        3. After 30 week weekly BPP.

    • TAPS twin anemia polycythemia sequence 

      1. Atypical chronic TTTS where the transfusions happens super slowly, through the smallest vessel connections, manifested in >MCA 1.5 MoM of one twin and < 1.0 MoM of the other twin 

        1. 2-13% due to post-TTTS laser (large anastomosis obliterated, smaller ones persist).

    • To distinguish TTTS vs single IUGR - guide is fluid volume.

    • Twin Reverse Arterial Perfusion (TRAP) Sequence

      • Acardiac twin 

        1. One twin absent head/heart but stays living, other pump twin can develop high output heart failure with 50% mortality rate.

      • Requires ultrasonic laser ablation of acardiac twin vasculature.

  2. Monoamniotic

    1. Cord entanglement/accident → IUFD

      • Antenatal management hard to agree upon, some do daily NST beginning in the late second or early third trimester, IP vs OP management, no consensus.

      • Rare, but can have TTTS in mono/mono.

What happens if a twin demises? 

  1. Spontaneous reduction or vanishing twin 36% in the first trimester and even higher for higher order multiples 

  2. Second trimester: up to 5% twins and 17% triplets undergo death of one of the fetuses 

    1. Monochorionic set higher rate of stillbirth and neurologic deficits in surviving twin than dichorionic set, recommendation to continue with pregnancy until at least 34 weeks. 

    2. Thought to be due to hemodynamic changes where intrauterine demise of one twin becomes a low pressure system, and the other twin can effectively exsanguinate or have hypotension/anemia from perfusion being directed towards the demised twin, subsequently causing death or neurologic injury of surviving twin.

Preterm labor 

  1. Asymptomatic

    1. Counseling patients they will be at a higher risk of PTL 

    2. No recommended screening for asymptomatic women, since no interventions have been proven to be helpful for women with multifetal gestation.

    3. One study showed prophylactic cerclage in women with twin gestation and short cervix on US actually increased the rate of sPTB with a RR of 2.2 

  2. Managing spontaneous PTL 

    1. Short term of tocolysis for administration of corticosteroids recommended, as those trials included some women with multiple gestation less than 34w.

      • Includes one course of rescue steroids (risk of delivering within the next 7 days, most recent course was at least 7 days ago) and extending to 23w after counseling on expectations and working closely with NICU to go over available resources.

    2. What about ALPS steroids for multiple gestation?

      • They were not included in the original trial, but we extrapolate their benefits and do recommend them at our institution.

      • Currently the ACTWIN trial, a multicenter RCT enrolling based out of Seoul, is attempting to answer this question.

    3. Magnesium sulfate for neuroprotection under 32 weeks regardless of fetal number.

Delivery planning 

  1. Dichorionic 

    1. In uncomplicated pregnancies, delivery no later than 38th week.

    2. Candidate for vaginal delivery if presenting twin vertex and someone with experience with internal podalic version and vaginal breech delivery available, including TOLAC candidates (one previous).

  2. Monochorionic 

    1. 34 to 37’6, with same principles as above for VD vs CS.

  3. mono/mono 

    1. 32-34w scheduled CS.

Fibroids

Fibroids, aka leiomyomas, are non-cancerous overgrowths of fibromuscular tissues within the uterine wall. They’re very common, affecting 20-80% of women by the age of 50 (depending on the population). Additionally, fibroids for 50% of women are completely asymptomatic.

Many patients will inquire about cancer risk with these benign “tumors,” particularly given the bad press around power morcellation. Fibroids in general do not increase one’s risk for malignancy; the risk of sarcomas in leiomyomas range between 0.05-0.28% (very rare).

Risk factors for fibroids include age (older > younger), black race, obesity, family history of fibroids, nulliparity, vitamin D deficiency, food additive consumption, and use of soy milk, strangely enough.

Fibroids are classified using a FIGO classification system based on their location:

So if fibroids half the time don’t cause problems, how and why do they cause problems?

The most common symptom of fibroids is heavy, prolonged menstrual bleeding. Fibroids do not necessarily rule out the risk of endometrial malignancy, so for those at risk, you should still do an endometrial cavity evaluation with EMB or hysteroscopy D&C. However, the bleeding from fibroids can be significant. The degree of bleeding is correlated often with the location of the fibroid, with size of secondary importance. Submucosal fibroids most frequently cause significantly heavy bleeding. Intramural fibroids can also cause this. Subserosal fibroids are rarely associated with heavy bleeding. How and why does this bleeding occur? It’s unclear, but may include abnormalities of uterine vasculature.

Another common symptom of fibroids are “bulk” symptoms. This is due to the size or location of a fibroid causing mass effect. This often manifests as pelvic pain/pressure that is chronic, intermittent, and dull; urinary tract or bowel issues such as frequency, difficulty emptying, or constipation; painful menses or intercourse; and venous compression rarely, similarly to a gravid uterus causing vena cava compression and increasing VTE risk. Clinically you might also see fibroid degeneration, where the fibroid has outstripped its blood supply and is necrosing, which leads to pain, leukocytosis, low grade fever, and uterine tenderness.

The final category of symptoms are reproductive issues. If a fibroid distorts the endometrial cavity, it may result in difficulty conceiving or increased risk of miscarriage. The links here are suspect as large, observational studies looking at these problems have many other confounding factors (i.e., increasing age). That said, fibroids have also been noted to have association with adverse pregnancy outcome as well, including placental abruption, fetal growth restriction, malpresentation, and preterm labor and birth.

Therapy options can be broken down into “expectant management,” medical treatment, and surgical treatment.  

For medical therapies, there are multiple options:

  • Hormone therapies - really just to treat bleeding; doesn’t really help that much with other symptoms like bulk-related or reproductive issues.

    1. Combined estrogen-progesterone contraceptives - first line in treatment of AUB, but there is a high conversion rate to surgery in 5 year period.

    2. Levonorgestrel IUD - no randomized trials evaluating IUD for HMB related to fibroids. 

      1. There is a decrease in bleeding and increase in hematocrit in observational studies; however, distortion of cavity due to fibroid is a relative contraindication .

    3. Progestin treatments (ie. implant, injection, pills) - conflicting info about whether or not they can increase size of fibroid 

      1. Can be considered for treatment of mild symptoms especially in women that desire contraception 

    4. PRMs (progesterone receptor modulators) - not currently available in most countries

      1. Ulipristal acetate has been used outside the US, but stopped because of rare cases of liver toxicity; has been shown to decrease HMB 

      2. Mifepristone - not currently approved for treatment of fibroid; has been shown to reduce uterine volume by 26-74%, which is comparable to GnRH agonists! However, no availability in the doses that are used to treat fibroids (ie. 5-50 mg/day compared to 200mg for abortion) 

    5. GnRH agonists - most effective medical therapy for uterine fibroids; only available as injection.

      1. Initially increases release of gonadotropins, but then there is desensitization and downregulation to hypogonadotropic, hypogonadal state; a “medical menopause” if you will.

      2. Most women will develop amenorrhea or betterment of bleeding, and there is significant reduction of uterine size 

      3. Effect is temporary, and symptoms quickly return after you stop using it

      4. Can lead to menopausal issues due to hypoestrogenic state = Vasomotor symptoms, but also BONE LOSS

        1. Should not use for >6 months without add-back therapy: combined estrogen-progesterone or just progesterone in the form of 0.625 mg of conjugated estrogen and 2.5mg of medroxyprogesterone acetate or 5 mg norethindrone acetate);

        2. Usually GnRH agonists are used pre-operatively to shrink fibroid, as a “bridge” to surgery.

    6. GnRH antagonists - pretty new for this stuff! 

      1. Also induces hypoestrogenic state, but they are oral, not injections! Can lead to all the bad things that GnRH agonists can.

      2. The one available in the USA is Elagolix (Orilissa) 

      3. Also need add-back therapy if you want to use it long term.

    7. Aromatase Inhibitors - small studies show decrease in size of fibroids; not FDA approved 

    • Antifibrinolytic agents such as TXA are not well studied in HMB related to fibroids specifically, but is used for heavy bleeding in general.

    • NSAIDs - not extensively studied for HMB in fibroids; doesn’t decrease bleeding much, but can help with pain.

Surgical therapy is an alternative choice, particularly if medical management fails, or for bulk or infertility-related symptoms.  

  • Myomectomy 

    1. Usually for people who aren’t done with childbearing or want to retain their uterus.

    2. Try and complete it minimally invasively if possible for decreased morbidity; this includes laparoscopically, robotically, or hysteroscopically if possible.

    3. Otherwise will need to do a laparotomy.

  • Endometrial ablation 

    1. Purely for bleeding symptoms.

    2. However, some devices are only designed to be used in a normal cavity and not.a distorted cavity. Also, will not help with bulk symptoms.

    3. Relatively high rate of re-intervention for treatment failure.

  • Uterine artery embolism 

    1. Can lead to shrinkage of fibroids 30-46%.

    2. However, those with larger uteri and/or fibroids are at higher risk of failure.

    3. Relatively high rate of re-intervention for treatment failure. 

  • Hysterectomy 

    1. Suggested for women who are have severe hemorrhage not responsive to other treatments, done with childbearing and have other issues (like EIN, endometriosis, etc) that could be eliminated by hysterectomy, failed prior minimally invasive therapies for fibroids, or done with childbearing and want definitive treatment of symptoms.

    2. Main advantage: eliminates symptoms and any recurrent problems from fibroids.

    3. Morbidity may outweigh benefits if there is a solitary subserosal fibroid, a pedunculated fibroid, or a submucosal fibroid that is easily removed by hysteroscopy.

    4. Minimally invasive hysts should be pursued when possible to decrease morbidity.

#MedEd: Wellness

So today we wanted to spend some time talking about wellness. Yes, it’s practically a four-letter word in medicine these days. However, we want to share a systems approach to thinking about improving wellness and well-being for those of you who are students, residents, or young faculty who may be approaching this problem at your own institutions. Especially with COVID-19, we are all feeling the stress and anxiety of the unknown. It is particularly important to reach out if you are someone going through this to get help, or alternatively, if you are in a position of leadership to reach out to your residents/trainees to discuss mental health. 

We’re supposed to be social distancing now, and traditional visits may not be occurring right now, but here are some ways to have these conversations/therapy sessions virtually (not sponsored by anything, just experience/anecdotes have suggested these are helpful): 

  • Betterhelp.com - virtual chat and online face-to-face sessions approximately $40-$70/week.

  • Talkspace.com - same thing, can get $100 off with 1004U, about $25/week with just text, and can cost more if you want face-to-face sessions.

  • Headspace - meditation app, offering premium versions free for healthcare employees with verification using your NPI number.

  • Be sure to look with your employees to see if your employee assistance programs cover online mental health services! 

So let’s ask some questions: 

What is burn out?

The term “burnout” was first coined in the 1970s by psychologist Herbert Freudenberger, it is defined as emotional exhaustion, depersonalization, and a decreased sense of personal accomplishment due to work-related stress. We know that physicians and residents can feel this particularly deeply. And there’s a lot of reasons for that: we work super long hours. We see stressful things. We have to numb ourselves to these stressful things, and we still are expected to be learning and studying throughout all of this. It’s really hard to achieve work-life balance in the environment where we spend most of our time.

Some studies have quoted that burnout is as high as 47-70% among surgical residents. This high prevalence is problematic, as it leads to a number of downstream consequences, including poor healthcare outcomes for our patients and ourselves.

So how do we stop burn out, and not just promote wellness, but, as SMFM has put it, THRIVE? 

In recognizing the problem, it’s important to acknowledge the beast that is the institution of medicine helped to create this. It’s in recognizing that, and as we all become empowered, to lead change in medicine in the future.

As we all know, ACGME has now placed work-hour limitations starting 7/2003, with change to the 80-hour work week. The literature suggests that because of this, resident burnout has decreased, and resident satisfaction and well-being has increased after the implementation of work-hour limitations. Prevalence of burnout decreased from 74 → 58% in one of these studies; while perhaps somewhat meaningful, is still an over 50% rate of burnout! But this is encouraging in that systematic/institutional changes are the things that can lead to a bigger difference in resident wellness.

In NEJM Catalyst, Bohman and colleagues proposed a really nice framework for approaching systems-levels issues with wellness: practice efficiency, a culture of wellness, and personal resilience:

(C) NEJM Catalyst

Culture of Wellness

Cultural change is derived from the institution and the policies it promotes. Besides work hour issues, what else is essential in a culture that promotes well-being? One study in JGME surveyed residents and fellows and suggested that in particular these things were important:

  • Effective mentorship with formalization of mentorship systems. W

    • Wellness activities within these mentorship systems.

  • Awareness and de-stigmatization of mental health issues.

    • Robust systems to prevent and respond to these issues.

    • A residency culture that supports and prioritizes this.

Efficiency of Practice

Efficiency can be defined as achieving productivity with minimal wasted efforts. This gives you more time to focus on other areas in your life that will make you happy, and provide time to develop personal resilience (the last cog in this wheel). We want to help patients, not computers.

Within institutions, EMR hangups and interoperability along with systems and communications problems, can be addressed and provide efficient approaches to minimize extra work. Additionally, some of the development of efficiency is personal: there is an element of this that comes with experience, whether that is with patient care or with computer usage!

Personal Resilience

This is a more challenging feature to define, and looks different for everyone, but it boils down to the ability to recover quickly from difficulties. Perhaps you define this as “toughness,” “grit,” or “resilience.” Dr. Angela Duckworth has an excellent book called “Grit: The Power of Passion and Perseverance,” and you can hear a preview through an NPR podcast here.

This is where taking care of yourself comes in; perhaps this is why people promote yoga, meditation, etc. - maybe that’s the thing for you that makes it so that you can persevere. It also suggests why pizza and ice cream parties may not be effective. They are one-time “breaks” but may be helpful for some people to develop their personal resilience. That time might be better spent with family, or getting healthcare or personal errands done, or just being able to be yourself.

 Some Examples of Institutional Major Changes

Harvard South Shore psychiatry residency identified areas of improvement in wellness and made concrete changes, which they published in 2019:

  • One thing they wanted to change was “on call” experience, and thus made an “on-call patient task force” to improve workflow while on call.

  • Also made a “food action team” to provide healthy food while on call (culture of wellness).

  • Increased social activities outside of work - collaborated with HSS leadership to find funding to promote resident participation in sports leagues (culture of wellness) .

  • Also emphasized importance of preventative care (provided residents with psychotherapists, nutritionists, dentists, and PCPs).

At Columbia OBGYN, we discussed changes to culture with Dr. Dena Goffman at SMFM:

  • Changed hours for certain physicians (ie. working 12-8 pm to allow parents to take children to school, etc.).

  • This cultural change and unique approach to work hours (the 8-5 isn’t for everyone!) helps to promote a culture of wellness.

AMA’s Steps Forward Campaign

A lot of this is using technology to help instead of hurt us. As one example featured in this campaign, changes in call management included creating a “one touch team” to resolved 50% or more of incoming calls with “one touch” instead of being bounced around from person-to-person; hiring of NPs to help with ‘in-basket management’ and coverage for out-of-office physicians; and dedication to decompress clinic schedules and provide administrative time to work on clerical work.

Things We’ve Done at Brown OB/GYN:

  • Sharing of dotphrases to promote efficiency and consistency of documentation.

  • We’ve compiled a list of therapists that accept our employer-sponsored insurance, and who have after-hours times available.

  • We have a “no questions asked” policy with respect to sick leave - or maybe better phrased, “only loving questions asked.”  

    1. If you’re sick, we will dedicate someone will cover you.

    2. If you need to go to a medical appointment, someone will cover you.  

    3. Not made to feel guilty for missing days.

  • Efficiency of call schedule - we constantly scrutinize the schedule to minimize people in house who don’t have to be there.

  • Mentorship - we have robust mentorship systems for individuals, classes, and specialty interests, with built in social time for these various mentorship opportunities.

Go forth and be well!

Mullerian Anomalies

Today we are rejoined by Dr. Emily Seidler of the Division of Reproductive Endocrinology and Infertility at Beth Israel Deaconess and Boston IVF, as well as Margie Thorsen, a PGY-1 at Brown & Women and Infants, to talk through congenital uterine anomalies (CUAs), also known as Mullerian anomllies.

CUAs can cause pelvic pain, AUB/dysmenorrhea, recurrent pregnancy loss (RPL), and/or preterm deliver, or can be asymptomatic . They are often found during an evaluation for primary amenorrhea and infertility. On their own, CUAs are associated with skeletal and renal abnormalities (20-30% of women with CUAs have concomitant renal anomalies), and can also be associated with inguinal hernias. Due to these generally being asymptomatic, true prevalence is hard to estimate; but it’s estimated around 5-6% amongst all women. This increases to 8-10% in infertile women, 12-15% in women with history of SAB, and 20-25% in women with both infertility and prior history of SAB.

Frequency of types of CUAs in affected women, in decreasing order:

  • Septate (most common)

  • Bicornuate

  • Unicornuate

  • Didelphys

  • Agenesis 

“Arcuate” uteri, which refers to an up to 1cm “dip” i the fundal contour of the cavity, is considered a normal variant.

Embryology of Mullerian Structures

  • Wolffian ducts = mesonephric =  “male” (need Y chromosome🡪 SRY gene🡪 AMH).

  • Mullerian ducts = paramesonephric = “female” (default, no AMH means Wolfiann ducts regress).

    • Includes the UTERUS, TUBES, CERVIX & UPPER VAGINA

      • Ovaries are totally separate (urogenital ridge)

  • Mullerian ducts start to elongate caudally at ~6 wks GA.

    • By 12 wks GA, the caudal portion of Mullerian ducts fuse to form the early uterus and vagina.

      • Initially, Mullerian ducts are like 2 solid cylinders laying side by side. Later, each cylinder undergoes canalization, and then fuses (like putting your elbows together and then slowly bringing the arms together until your wrists join).

    • By 20 wks GA, septum absorption is complete.

      • Top part forms the fallopian tubes and fimbria

      • Bottom part becomes the uterus and upper vagina

The Mullerian Anomalies

  • Genetics are not well understood; mostly thought to be polygenic/multifactorial. For affected patients, karyotype is usually normal 46, XX.

  • 3 main types: agenesis/hypoplasia, lateral fusion defects, vertical fusion defects.

  • Agenesis or hypoplasia

    • Classic test question involves complete agenesis of uterus/cervix/upper vagina termed MRKH syndrome, presenting as primary amenorrhea with otherwise normal pubertal development.

      • On exam: overall look totally normal; pelvic exam = “blind pouch” + shortened vagina; normal breasts, normal pubic & axillary hair.

      • Exams may try to confuse this presentation with androgen insensitivity syndrome (AIS).

        • Key labs to differentiate: karyotype is 46,XX, total T is female range in MRKH, whereas 46, XY and elevated T in AIS.

      • Treatment: dilators to become sexually active; can have biologic children later in life with the use of a GC (ovaries/eggs are normal!)

  • Lateral fusion defects

    • i.e., your two arms don’t come together “elbows to hands” normally

    • Longitudinal vaginal/uterine septum, bicornuate, didelphys, etc.

      • Can have 2 cervices = “bicollis.”

      • If abnormal/absent uterine horn, that side’s tube will also be affected.

        • Same concept with renal abnormalities (abnormal uterine side = abnormal renal side).

      • Treatment depends on the anomaly, symptoms,  and patient’s goals.

        • Generally with infertility patients with a septum, will be resected,

        • If not associated with infertility and not surgically corrected, just helpful to know going into pregnancy (SABs, preterm labor/delivery, etc.).

  • Vertical fusion defects

    • Leads to a TRANSVERSE vaginal septum, partial vaginal agenesis, and/or cervical agenesis.

  • Miscellaneous uterine anomalies include those related to in-utero exposure to diethylstilbestrol (DES).

    • Moms were ironically given it to prevent pregnancy loss from 1950s-1971

    • Female babies born with classic uterine anomalies: T shaped uterine cavity, hypoplastic uterus, endometrial cavity adhesions.

Diagnostics for Suspected Anomalies

  • 2D ultrasound = initial imaging modality of choice, as it is widely available, noninvasive, relatively inexpensive, and can also look at renal system.

    • 3D sonohysterogram especially helpful if available.

  • MRI only necessary if ultrasound isn’t definitive; can be helpful for surgical planning.

  • HSG might make the initial diagnosis (infertility patient eval.) but this only evaluates the uterine CAVITY, not the fundus!

    • I.e., an HSG with 2 “bunny ears” could be a large uterine septum (normal fundus) or a bicornuate or didelphys (heart-shaped fundus).

COVID-19 for the OB/GYN

Today’s episode is an audio summary of the CDC and SMFM guidelines for COVID-19, focusing on the virus, identification, containment, and considerations for pregnancy and postpartum care. We do not explore treatments or any intricacies in either outpatient or inpatient care.

Rather than posting those guidelines here verbatim, we have gone through and identified a lot of reputable sources with factual, up-to-date information for healthcare providers:

We also mention in the episode a registry being established for pregnant patients with COVID-19. Please listen to learn more about how you can be involved.