Popular Birthing Trends and the OB/GYN

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What are some birthing trends that we have seen rise in the United States?

  • Obviously, there are many, but unfortunately, we don’t have time to address all of them, and not all of them have robust scientific literature. Therefore, we will focus on some that are more controversial and may come up more often to help our colleagues navigate these trends with their patients that might desire them. 

  • Please see our other episode on limiting interventions in birth to look at some other trends that we fully support, like having a doula or other support person in labor and nonpharmacologic methods for coping. 

  • Today, we will discuss: 

    • Lotus Birth 

    • Vaginal seeding 

    • Placentophagy (ie. eating the placenta)

Lotus Birth 

  • What is a lotus birth? 

    • Practice of leaving the placenta attached to the umbilical cord and baby until the cord falls off on its own 

    • Anecdotally, this can take up to 3-10 days 

    • Conventional practice, as we know, is for delayed cord clamping for 30-60 seconds 

  • What are the perceived benefits? 

    • Modern resurgence is thought to be credited to Claire Lotus Day in 1974 

    • She observed that apes don’t sever their infants from placenta 

    • Delayed cord clamping does have many benefits, as we reviewed in previous episode Delayed Cord Clamping 

      • Increased hemoglobin levels, improved iron stores in first few months of live, increased red blood cell volume, decreased need for blood transfusion, and decreased risk of NEC and IVH 

    • There isn’t a lot of research about the benefits of lotus birth, but those that practice it believe it can: 

      • Increase blood and nourishment from the placenta 

      • Decrease injury to the belly button 

      • Be a gentle, less-invasive transition for the baby to the world 

      • Be a ritual to honor the placenta (though there does not appear to be written record of cultures that leave the cord uncut), and gives patients autonomy on their desires for delivery

    • The way it is done: 

      • The cord is not detached during birth and the placenta is usually kept in a cotton bag with a drawstring that contains herbs or salt to dry and preserve the placenta  

  • What are the risks? 

    • Qualitative studies show that many patients who practice lotus birth view the placenta as belonging to the baby and that it is something the baby should release when they are ready. They also discuss it in spiritual and ritualistic terms, but medical benefit and cleanliness were often secondary concerns 

    • Overall, very little data about lotus birth 

      • However, there is currently no evidence regarding effects on cognitive or emotional development of infants or possible benefit

      • There are case reports suggesting potential for infection, such as endocarditis from staphylococcus lugdenensis and omphalitis 

      • No data available regarding late-onset sepsis 

  • How should we counsel our patients regarding umbilical cord nonseverance? 

    • First of all, we should always respect the wishes and decision of patients 

    • It is important to review patient’s beliefs and why they desire lotus birth 

    • Discuss current evidence (very little) and society recommendations 

      • Important to realize that right now, there are no formal recommendations available from professional societies.

      • From the American Academy of Pediatrics:

        • Providers should conduct routine assessment and management of ill-appearing neonate 

        • Any placenta and umbilical cord attached to affected child should be immediately removed if child is ill appearing (esp if necrotic tissue is evident) 

        • Tissues should be cultured 

        • Antimicrobial coverage with anaerobic bacteria and vanc may be needed to be included to usual regimens 

    • Ultimately, the biggest risk is infection and patients should be counseled by us and pediatrics about signs of neonatal infection

    • Overall, there does not appear to be significant medical benefit to lotus birth and there are possible risks, but if it is highly desired by your patient, it is not unreasonable to achieve 

    • Things to consider: 

      • Cesarean delivery - it is possible to do lotus birth with cesarean 

      • Postpartum hemorrhage - if there is hemorrhage, in order to save the woman’s life, lotus birth may not be possible 

      • Non-vigorous infant or preterm infant - there is not a lot of data in these cases, but should review with patient that in order for expeditious pediatric evaluation, the cord may need to be clamped and cut

      • Placental pathology (ie. accreta, vasa previa) - lotus birth is likely not possible 

      • Review placenta disposal - placenta should not be flushed down the toilet or buried close to the surface of the ground; if it is buried, then it should be disposed of in a location that adheres to local laws and sanitation guidelines 

      • Be careful of buying placenta bags – not sure what the material is made from, not sure what the herbs that are included are, and realistically, not sure if what is included can actually harm babies

Vaginal Seeding 

  • What is vaginal seeding?

    • For babies who are born via C-section, inoculation using cotton gauze or swab with maternal vaginal fluid applied to the newborn’s mouth, nose, and/or skin 

  • What are the purported benefits? 

    • Thought is that it can restore the newborn’s microbiome that is more typical of vaginal delivery 

    • Epidemiologic studies show that there is a relationship between cesarean sections and increased risks for various conditions such as allergies 

    • Nonvaginal delivery may be associated wit changes in the infant’s microbiome (though changes do not appear to persist) 

  • What are the risks? 

    • Vaginal seeding has potential to transfer pathogens to newborns that are associated with vertical transmission (ie. GBS, HIV, HBV, syphilis, etc) 

    • There are other factors that may be related to initial colonization beyond the mode of delivery (ie. gestational age, transfer via breastfeeding) 

    • Of note, both AAP and ACOG recommend against vaginal seeding outside of research settings: 

      • Families should be counseled regarding the risk of exposure to pathogens that may occur despite negative screening because of possible false negative results or acquisition of the pathogen after the screening is done  

      • Concerns are compounded by increased risk of infections in preterm infants 

  • How do I counsel my patient? 

    • Again, it is important to discuss the patient’s beliefs and motivations 

    • We should review that currently, there is no data to suggest that vaginal seeding leads to benefits, but there is data about possible harm 

    • Of course, we can’t control what patients will do when we get home, but would recommend against vaginal seeding per ACOG and AAP 

Placentophagy 

  • What is placentophagy? 

    • Eating the placenta, usually prepared by steaming, followed by dehydration and then grinding to a powder and then encapsulated 

    • However, there are also practices of eating the placenta raw, cooked, or blended in liquid extracts

  • What are the purported benefits? 

    • For spiritual reasons 

    • Claims that it will increase milk supply or improve energy and decrease postpartum depression, though these results have not been substantiated 

  • What are the risks?

  • How do I counsel my patients?

    • As always, review the patient’s beliefs and motivations 

    • Discuss the current literature and data with the patient and that we ultimately don’t recommend eating the placenta 

    • If someone really wants to do it: 

      • Review that the placenta should be professional prepped if possible - do not do it at home 

      • The process should ensure that the placental tissue gets to high enough temperatures to kill viruses and bacterias 

      • Really recommend against it if there is infection of certain things that can be vertically transmitted (ie. GBS, HIV, Hep B, etc.) 

      • Monitor yourself and your baby closely - if either one gets sick, please seek professional help 

      • Similarly, if patient begins to have symptoms of PPD, don’t wait for the purported benefits of the placenta to kick in; should seek medical help 

The APGAR Score

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What is an APGAR?

  • 1952: Dr. Virginia Apgar devised scoring system to assess rapidly clinical status of a newborn to determine if prompt intervention is required to establish breathing.

  • Has subsequently stuck, and is reported at 1 minute and 5 minutes after birth for all infants

    • Continued at 5 minute intervals thereafter until 20 minutes for scores under 7.

  • The score is eponymous for Dr. Apgar as well as an acronym.

  • Each component of the score can receive 0, 1, or 2 points:

    • Appearance (color)

      • 2 points: normal over entire body

      • 1 point: normal except extremities (acrocyanosis)

      • 0 points: cyanotic/pale all over

    • Pulse

      • 2 points: HR >100

      • 1 point: HR < 100

      • 0 points: absent HR

    • Grimace (reflex irritability)

      • 2 points: sneeze, cough, or vigorous cry, active withdrawal of extremities to stimulus

      • 1 point: grimace, weak response to stimulus

      • 0 points: no response to stimulus

    • Activity (muscle tone)

      • 2 points: Active motion

      • 1 point: arms and legs flexed to some degree

      • 0 points: hypotonic, limp

    • Respirations

      • 2 points: Good effort, crying

      • 1 point: Gasping, irregular efforts, hypoventilation

      • 0 points: not breathing

  • While the Apgar score is useful for conveying information about the infant status quickly, resuscitation needs to be started before the Apgar calculator intervals.

  • The Apgar can be to some degree prognostic – an Apgar of 0 at 10 minutes have very few reports of infants surviving with normal neurologic outcomes, and it’s reasonable to consider discontinuing resuscitative efforts at that point.

  • The five minute score is generally considered more useful/prognostic:

    • A score of 7-10 is considered reassuring

    • 4-6 is moderately abnormal

    • Under 4 is abnormal, especially in term and late-preterm infants

Limitations of the Apgar Score

  • The score is one moment in time, and is subjective in some components

  • Multiple factors can influence the score and make it more “false positive” with low scores, such as:

    • Maternal sedation/anesthesia

    • Congenital malformations or genetic abnormalities

    • Gestational age

  • Biochemical disturbances such as fetal acidemia must be quite profound to affect the Apgar score

  • A low score doesn’t predict morbidity or mortality for any individual infant, and cannot be used alone to diagnose asphyxia.

    • Cord gases should be obtained to demonstrate poor gas exchange and metabolic acidemia in order to truly diagnose asphyxia.

  • Apgars are often continued to be assessed during resuscitation, but these are obviously not equivalent to scores assigned to spontaneously breathing infants

    • There is no standard for reporting Apgars after the start of postnatal resuscitation, because those interventions obviously affect the score. 

    • There are expanded Apgar score forms, where additional information regarding the infant’s resuscitation and response can be recorded to help assess the impact of interventions and the infant’s status in light of these.

      • Essentially like a “code sheet” for neonates

Outcomes after Apgar Scoring

  • 1 minute Apgar scores don’t predict outcomes well at all

  • 5 minute scores of 0-3 correlate with neonatal mortality in large populations, but are not individually good at predicting neurologic dysfunction for an infant.

    • Low Apgar scores do seem to correlate at population level with increased relative risk of cerebral palsy (20-100x higher risk with 0-3 versus 7-10 score).

    • Most infants with low Apgar scores do not go on to develop neurologic issues or CP.

  • ACOG does recommend that a cord gas be sent for any 5 minute Apgar of less than 5, and considering sending placenta to pathology as well. 

  • Apgars can also be useful to monitor for quality improvement programs to assess both obstetric and pediatric response and resuscitation.

Monkeypox for the OB/GYN

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Reading/sources:

What is monkeypox and how is it transmitted? 

  • Name and type of virus 

    • WHO is planning to rename the virus to reduce stigma and racist overtones - but this hasn’t happened yet! 

    • Orthopoxvirus (genus of Poxviridae family) and has features similar to smallpox or variola 

      • DNA genome

      • There are two different strains: the Congo basin clade and the west African clade 

        • Congo basin clade has historically caused more severe disease and is thought to be more transmissible 

        • The West African clade seems to be the dominant circulating strain → case fatality ratio of 3% to 6% 

  • Cases and outbreak 

    • First case was in 1970 in the Democratic Republic of Congo

    • First case in the US in this outbreak was on 5/17/2022 

    • Most recent reports from the CDC states (as of 8/31/2022) - there have been 18,989 total confirmed monkeypox cases in the US 

    • Demographics 

      • There is not complete demographics data for everyone

      • Most recent data from mid August from the CDC shows that only 1.5% of all cases were in women and transgender men

      • No deaths have been reported in this population  

  • Transmission 

    • Human to human transmission can occur from

      • Direct contact with infected rash, scab, or body fluid 

      • Respiratory secretions during prolonged or intimate physical contact 

      • Contact with contaminated items, such as clothing or bedding 

    • A person with monkeypox infection is considered contagious from initial viral prodrome and development of rash until lesions have full healed and new skin has formed over the scabs 

    • Unclear if transmission can also occur through vaginal or seminal fluids 

    • Perinatal infection can occur through transplacental transmission or during close contact during and after birth 

    • Zoonotic transmission can also occur following direct contact with blood, bodily fluids, or cutaneous/mucosal lesion of infected animals 

Clinical Presentation 

  • Current outbreak 

    • Many of the initial patients in this outbreak have shown painful genital and perianal lesions, oral lesions, and proctitis in the setting of mild or no prodromal symptoms 

  • Clinical course 

    • Average time between contact with monkeypox and symptoms is 5-13 days, with range of 4-17 days 

    • Classic features of infection: 

      • Fever, lymphadenopathy, malaise, headache, muscleaches 

      • Can have lymphadenopathy

      • Rash develops approximately 1-4 days after prodromal symptoms → deep-seated vesicular or pustular, often beginning centrally and spreading to the limbs 

      • Rash can last 2-4 weeks, progressing through stages includes macules, papules, vesicles, pustules, and even scabs and crusts 

      • Rash can leave scars

  • From the Green Journal article (not sure we can use?) 

Pregnancy Implications of Monkeypox 

  • Not very much is known: 

    • We reviewed that the monkeypox virus can be transmitted to the fetus during pregnancy or to the newborn by close contact during and after birth 

    • There has been an increased risk of maternal mortality and morbidity documented with other poxviruses, but it’s unknown if pregnant people are more susceptible to monkeypox or if disease is more severe in pregnancy 

    • One publication looked at 5 cases of documented perinatal outcomes 

      • 2 = SAB 

      • 1 = stillbirth 

      • 1 = preterm birth 

Evaluation for individual with suspected monkeypox 

  • Routine screening is not recommended for asymptomatic patients 

  • If suspicion of monkeypox virus infection:

    • Collect recent travel history, ask specifically about countries where monkeypox has been reported 

    • If rash or anogenital lesions, ask about close contact or sexual exposure to someone with monkeypox 

    • Full body skin exam, including oral mucosa, genital, and rectal areas, + evaluate lymph nodes 

    • Isolation from others

    • Consultation with infectious disease 

  • Diagnosis 

    • Two-step process requiring initial identification of an orthopoxvirus 

    • If orthopoxvirus is confirmed, specimens are sent for monkeypox virus-specific testing 

    • Multiple samples should be collected, ideally from different lesions (2-3 from different areas of the body with diff appearance) for PCR testing 

    • Please follow your own hospital’s guidelines on how to obtain these samples!

    • As there aren’t really any other orthopox viruses in the US, we shouldn’t wait for the confirmatory testing before initiating infection-control procedures and preventative strategies + treatment 

  • Healthcare provider precautions 

    • Standard precautions and wear PPE: gown, gloves, eye protection, and N95 mask 

    • Any procedure where there is aerosolization (ie. intubation/extubation), should be done in airborne infection-isolation room 

Treatment 

  • Disease is usually self-limited, but disease can progress to severe, so certain populations at risk of severe disease 

    • This includes pregnant patients, people who are breastfeeding, and those with oral, ocular, genital, or anal lesions 

  • No specific treatment for monkeypox virus infection

    • However, there are 2 antivirals +immune globulin available 

    • Tecovirimat (Tpoxx) - antiviral (limited to health department/CDC Expanded access protocol) 

      • Approved by FDA for treatment of smallpox virus infection and may prove beneficial for monkeypox 

      • Available in oral and IV formulations 

      • Works by blocking cellular transmission of the virus 

      • Both forms have been used to treat patients during the current outbreak in the US

      • No human data is available during pregnancy, but no fetal toxic effects were observed in mice studies using oral medication 

      • Not known if present in breastmilk 

    •  Cidofovir - antiviral (Off-label use, available for use in outbreak setting) 

      • Approved by the FDA for treatment of CMV retinitis in patients with AIDS 

      • Can be used for orthopoxviruses in an outbreak setting 

      • In animal studies, cidofovir has been associated with embryotoxicity and teratogenicity, but no adequate or well-controlled studies in humans 

    • Brincidofovir - antiviral (availability limited to Strategic National Stockpile distribution) 

      • Approved by FDA to treat smallpox 

      • Unfortunately, in animal studies, there have been embryo-fetal toxicity demonstrated + structural malformations

      • Therefore, alternative therapy is recommended in pregnancy  

    • IVIG - also available in outbreak setting 

      • Also no human data or animal data in pregnancy 

  • Prevention 

    • Primary prevention is from isolating from individuals with infection

      • Avoid close contact and sexual activity with people with infection 

    •  Postexposure prophylaxis 

      • CDC has tools to assess the risk of monkeypox virus infection and recommends post-exposure vaccination for specific risk exposures or risk factors 

        • Criteria

          • Within 4 days of known exposure to reduce likelihood of infection or between 4-14 days to reduce severity symptoms 

          • Known contacts of monkeypox cases ID’ed by public health via case investigation 

          • Presumed contacts who meet criteria: 

            • Know that sexual partner in the past 14 days was diagnosed with monkeypox or 

            • Had multiple sexual partners in past 14 days in a jurisdiction with known monkeypox 

      • If given within 4 days of exposure, vaccine is likely to prevent monkey pox virus infection 

      • Of note, there are two types of vaccines 

        • JYNNEOS = live-non-replicated viral vaccine - There are no studies in pregnant patients

          • Pregnancy, however, is not a contraindication to post exposure prophylaxis with vaccination if the individual is otherwise eligible 

        • ACAM2000 - repliating viral vaccine licensed for prevention of smallpox 

          • Contraindicated in pregnant or breastfeeding people due to risk of pregnancy loss, congenital defects, and vaccinia virus infection 

    • Preexposure prophylaxis 

      • Attenuated live-virus vaccine and replication-competent vaccine are available 

      • Routine immunization of all healthcare workers is not currently recommended 

      • Only recommended for those whose jobs may expose them to monkeypox (ie. lab personnel and healthcare workers who administer a replication-competent vaccinia virus vaccine or anticipate caring for many patients with monkeypox) 

Principles of Electrosurgery, feat. Dr. Gary Frishman

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Here’s the RoshReview Question of the Week:

A 45-year-old woman in the postoperative recovery unit develops dyspnea. Her serum sodium is 130 mEq/L. Which of the following was the most likely distending medium used during her hysteroscopic monopolar fibroid resection?

Check your answer and get a sweet deal on RoshReview at the links above!

Today we welcome Dr. Gary Frishman to the podcast. He is a clinical professor of obstetrics and gynecology at the Warren Alpert Medical School of Brown University, and has served in varying leadership capacities at Brown as a residency program director and in other organizations, such as for MIGS Fellowship at ABOG, and within ASRM, SRS, and AAGL. While we were residents at Brown, Dr. Frishman used to share his wisdom with us on electrosurgery and we have wanted to put this podcast together for a long time! It’s more than just cut and coag!

What is electrosurgery?

  • Delivery of radio frequency waves that heat up tissue, which then causes tissue desiccation.

    • Cautery: not a specific term for this!

    • Electrosurgery represents alternating current where the body is part of the conductive circuit.

      • Modulating the delivery of this electricity is how we can get differing tissue effects.

Monopolar and Bipolar

  • Bipolar: movement of electricity from one point to another direct point.

    • i.e., bipolar forceps, LigaSure device (has two ends)

    • More precise delivery of energy, less smoke.

  • Monopolar: movement of electricity from one point (in dispersive fashion) to a return point.

    • i.e., the “grounding pad” when you are using a Bovie / electrosurgical instrument.

      • Energy still goes through body, to a second electrode and back to the machine!

Modifying the Delivery of Energy

  • Waveforms:

    • Think about energy delivered as a garden hose with a spray nozzle at the end.

      • The amount of energy delivered is your watts setting on the machine (i.e., 35W).

        • This you can adjust linearly — you can go up to 40W, or down to 30W, but it’s a linear adjustment — less efficient.

      • If you adjust your spigot at the end of the nozzle, that is another way to adjust your energy delivery. Like if you get a dispersed spray (sprays all over the wall) versus a fine spray (that gives you a directed, straight line of water).

        • Cut: the directed, fine, straight line of water spray

        • Coag: the dispersed spray that goes everywhere

          • The energy is still our original 35W, it’s just differently applied!

      • Importantly, the cut and coag settings also are different in the time energy is applied.

        • Cut: energy is constantly applied — so, for example, 100W of energy delivered over 100 seconds.

          • Clinically, this delivers good cutting, but poor hemostasis.

        • Coag: energy is applied in bursts, and it’s only on 6% of the time — so 100W of energy delivered over 6 seconds.

          • It’s an uncontrolled, massive burst!

          • Clinically, this delivers a lot of lateral spread, and you get good hemostasis, but not great cutting.

        • Blend curve: tries to get you both: excellent cutting, excellent hemostasis, little thermal damage.

  • Spot size:

    • You can also adjust the spot size of energy delivery by changing the tip of delivery instrument (i.e., Bovie)

      • By flattening the tip, you make the spot size larger — much larger surface area to deliver energy across.

      • By using the fine point of the tip, your spot size is much smaller — delivers energy to more concentrated area (i.e., needle tip electrodes).

Complications of Electrosurgery

  • Direct coupling

    • Accidentally touch something metal with your electrosurgery device

      • i.e., touching a retractor or a laparoscope, which is in turn touching something else

      • Good news — just like with the “spot size,” if you touch a retractor, it’s such a large surface area it’s unlikely to cause damage. However, with smaller instruments, this can modify injury and cause injury.

        • Plastic trocars help prevent this in laparoscopic surgery.

  • Insulation failure

    • Tiny cracks in an instrument that can cause insulation to fail — as you might get inadvertent direct coupling from the device if electricity is leaking out through the insulation.

      • Fortunately this is very uncommon.

      • If there are gross breaks — don’t use the instrument.

      • Limit the use of coag (i.e., use cut exclusively) to prevent this kind of injury.

  • Capacitive coupling

    • Two conductors separated by an insulator

      • Energy is stored in the separated conductor, and can then deliver energy.

        • Less likely to occur with cut than coag

        • Jewelery and electrosurgery - a possible (though rare) complication of wearing jewelry in surgery.

Role of Tissue Resistance

  • Electricity heats up water in the cell

    • Cut: heats up water in the cell very rapidly, and it explodes.

    • Coag: heats up water in cell more slowly, and energy dissipates laterally.

  • Electricity follows path of least resistance

    • As tissue dessicates (water removed), it becomes harder for electricity to pass through.

    • Think about a car on cruise control, set to a speed of 30W:

      • If you’re going uphill, you’re going slower unless car adjusts — higher tissue resistance.

      • If you’re going downhill, you’re going faster unless car adjusts — lower tissue resistance.

  • Newer generators measure tissue resistance and can adjust your energy appropriately!

    • Newest bipolar instruments also take surgeon out of equation entirely — and automatically adjust energy to resistance and shut off automatically when resistance is so high energy can’t pass.'

  • Importantly, if you’re using monopolar — electricity will go around high-resistance areas! This is how you can get capacitive coupling to jewelry and other areas.

    • A “grounding pad” is usually very large to help accommodate for this and prevent this injury.

      • It’s also in two halves, and both must be connected to make the machine work.

      • Bipolar instruments reduce this risk significantly.

Fulguration

  • Taking the tip of the device and placing it close to, but not on the tissue — may have heard of this as “arcing” the device.

    • Use coag on this because you want a huge burst of energy to leap across the space.

    • This energy “follows the blood” back to the original bleeder.

Cutting on Skin??

  • You can! But you need to know how to deliver energy — need a small spot size and to use cut.

von Willebrand's Disease, feat. Dr. David Abel

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Today we welcome Dr. David Abel, Assistant Professor in obstetrics and gynecology at the Oregon Health and Sciences University. Dr. Abel’s expertise and interest is in the most commonly encountered bleeding disorder for OB/GYNs: von Willebrand disease.

You can join the Foundation for Women and Girls' with Blood Disorders as a student, resident, or fellow for FREE and get access to their educational content by visiting this link.

Some light reading to accompany today’s podcast:

What is von Willebrand’s disease?

  • First described in 1926 by Erik von Willebrand, a Finnish pediatrician.

  • Most common inherited bleeding disorder, accounting for about 80 to 85% of all bleeding disorders.

  • Prevalence about 1 in 10,000, though possibly as high as 1 to 2% of the general population.

    • With respect to women with heavy menstrual bleeding, the prevalence appears be greater, ranging from 5 to 24%. 

  • Von Willebrand Factor is a large glycoprotein encoded by a gene on chromosome 12 that is synthesized in endothelial cells and megakaryocytes, the hematopoietic cells that produces platelets.

    • It is assembled from identical subunits into strings of subunits that vary in size and are referred to as multimers.

    • Two main functions:

      • Needed for normal adhesion of platelets to sites of injured endothelium

      • Serves as a carrier for Factor VIII, which also protects vWF from proteolysis

        • When vascular injury occurs, this multimeric protein uncoils which results in platelet adhesion, activation and aggregation.

  • Three main types of disease:

    • Type I: A partial quantitative deficiency of both von Willebrand factor and Factor VIII. This reduction is usually mild to moderate. 

      • Most common, accounting for 70-80% of cases of vWD

    • Type II: Quantitative reduction in von Willebrand Factor, with subtypes noted as A, B, M and N.

      • Accounts for 10 to 30 percent of cases of vWD

      • Patients with Type 2B vWD may have a moderate to severe bleeding phenotype that may present with thrombocytopenia during pregnancy.

        • Though uncommon, when you go through your differential for thrombocytopenia during pregnancy, you can keep type 2B von Willebrand disease on your radar!

    • Type 3 Most severe, characterized by the virtual absence of von Willebrand factor. 

      • Least common type, accounting for only 1-5% of cases.

  • VWD is usually inherited in an autosomal dominant fashion

    • Baby generally has a 50% chance of inheriting this condition — obstetric/neonatal implications.

    • Rarely vWDz is inherited as an autosomal recessive condition, namely in type 3,  and some cases of type 2. 

 How do we establish a diagnosis of von Willebrand’s disease?

  • Most common bleeding symptoms: bruising, epistaxis, bleeding after injury, surgery or tooth extraction, postpartum bleeding, and menorrhagia (most commonly reported symptom).

    • In addition to mucocutaneous and soft tissue bleeding, joint and muscle bleeding can also occur.

    • Severity of bleeding is usually related to the degree of von Willebrand factor and Factor VIII deficiency. 

  • Initial work up typically consists of nonspecific tests such as a CBC, PT, PTT and fibrinogen which are helpful in excluding a clotting factor deficiency.

    • PTT may be normal in patients with von Willebrand’s.

    • If thrombocytopenia is detected, type 2B vWDz is in the differential.

  • Next step are tests that are specific for von Willebrand Disease, three:

    • von Willebrand factor antigen (vWF:Ag) measures the quantity of von Willebrand Factor protein in the plasma.

    • Factor VIII assay measures Factor VIII activity which is essentially a surrogate marker for the activity of von Willebrand factor.

    • von Willebrand factor activity a functional assay that measures the interaction between VWF and platelets.

      • Historically this was measured by the von Willebrand Factor ristocetin cofactor activity assay (VWF:RCo).

      • Consultation with hematology will be helpful with diagnostic testing and interpretation of results. 

Why does von Willebrand’s disease matter for us in OB/GYN?

  • Strong association between von Willebrand Disease and heavy menstrual bleeding.

    • Among women with heavy menstrual bleeding, von Willebrand Dz is found to be the etiology frequently, with a reported prevalence ranging from 5 to 20%. 

  • Many treatment options are available for women with von Willebrand disease and heavy menstrual bleeding, including hormonal and nonhormonal therapies.

    • Association of VWD with other gynecologic problems, including ovarian cysts, endometriosis, and leiomyomas is unclear. 

  • Prior to procedures as egg retrieval if IVF is planned, or invasive procedure during pregnancy such as CVS and amniocentesis, DDAVP or VWF concentrates can be administered immediately prior to these procedures (more on treatment later).

Antepartum and Intrapartum Management of vWD

  • Both von Willebrand Factor and Factor VIII levels increase during pregnancy, an increase that usually starts in the second trimester and peaks in the third trimester.

    • The increase in VWF and FVIII levels usually reduces the likelihood that our patients warrant treatment during the antepartum, intrapartum or even postpartum period.

    • If a patient was previously undiagnosed, the increase in levels during pregnancy may obscure the diagnosis.

      • In general, women with baseline levels of VWF and FVIII of >30 U/dL, suggesting type 1 VWD, usually have a high likelihood to achieve normal levels by the end of pregnancy.

    • In the case of the severe and uncommon type III vWDz, both VWF and FVIII levels do not increase during pregnancy.

  • Levels of vWF and FVIII fall rapidly after delivery.

    • Levels start to approach patient’s baseline by one week postpartum and fully reach baseline by three weeks postpartum. 

  • Potential complications of vwD during pregnancy include antepartum bleeding, postpartum hemorrhage and perineal hematoma which are all increased by 2–10-fold.

  • Importantly the risk of delayed postpartum hemorrhage (bleeding occurring after 24 hours post-delivery) is also increased.

    • 16-29% of women with VWD will have postpartum hemorrhage within the first 24 hours of delivery,

    • 20-29% of women will experience delayed postpartum bleeding.

    • Bleeding is frequently reported to occur more than 2 to 3 weeks postpartum.

  • Factor VIII and von Willebrand Factor ristocetin cofactor activity assay are checked early in pregnancy and again in the third trimester.

    • Consult hematology to decide if any additional testing is needed

  • Women with type I vwDz with Factor VIII and ristocetin cofactor activity levels less than 50 IU/dL, and no history of severe bleeding, do not require special treatment at the time of delivery.

    • They can often be cared for by the general obstetrician in a community setting if the provider is comfortable and hematology available if needed. 

  • If levels are less than 50 IU/dl, the patient is at risk of hemorrhagic complications including delayed postpartum hemorrhage. These patients require treatment usually close to delivery or after cord clamping.

    • The classic teaching is to administer DDAVP.

      • DDAVP causes release of VWF that has been stored in secretory granules within the endothelium, resulting in FVIII and VWF levels three to five times above basal levels within 30‐60 minutes.

      • Effective in patients with type 1 VWD with baseline VWF and FVIII levels higher than 10 IU/dL.

      • The recommended dose of DDAVP is 0.3 μg/kg IV given over 30 minutes or 300 μg given intranasally. 

        • Onset of action of DDAVP is approximately 15-30 minutes.

        • Usually given at the time of cord clamping, but because the peak effect is 1.5 to two hours after administration, it may be more beneficial if administered during the second stage of labor or immediately before cesarean delivery. 

      • Risks: water retention that can lead to hyponatremia and seizures.

        • Need fluid restriction to less than 1L in the 24 hours following DDAVP administration.  

        • However, fluid restriction after delivery can be very difficult, and is the reason why many experts do not use DDAVP as a first line treatment.

    • Alternative: plasma derived VWF concentrates.

      • Options:

        • Plasma derived VWF concentration that contains VWF alone without Factor VIII.

        • Recombinant VWF concentrate that contains only VWF without Factor VIII.

        • Plasma derived concentrate that contains both VWF and Factor VIII.

          • The decision regarding which one of these to use will depend upon levels of both VWF and Factor VIII, what is available in your hospital and input from hematology.

Regional anesthesia

  • No consensus on levels that are safe for regional anesthesia, but if levels are greater than 50 IU/dL and assuming a normal platelet count, regional anesthesia is usually considered reasonable.

  • Recent ASH guidelines recommend VWF activity levels should be maintained at 50 IU/dL while the epidural is in place and for at least 6 hours after removal. 

Mode of Delivery

  • Fetal status unknown in most cases —> given AD inheritance and 50% risk, best to avoid procedures such as a fetal scalp electrode.

  • Operative delivery is discouraged due to the potential risk of intracranial hemorrhage - cesarean delivery preferred to operative vaginal delivery. 

  • The pediatrician should be made aware of the mother’s status and male circumcision should be postponed until the baby’s VWDz status can be determined.  

    • Sending cord blood at the time of delivery for a VonWill panel is recommended to determine the status of the newborn.

Postpartum Care

  • High risk of both primary and delayed postpartum hemorrhage. Perineal hematomas can be seen.

  • VWF and Factor VIII levels can significantly decrease postpartum, with a return to baseline within 7-21 days postpartum.

    • These levels are typically checked in the immediate postpartum period and then periodically.

    • Hematology consultation will be valuable to help work out these details.

    • NSAIDS should be avoided in the postpartum period. 

  • ASH guidelines recommend the use of oral tranexamic acid in the postpartum period.

    • Administration of one mg intravenously can be administered prophylactically immediately after delivery and continued PO for 10-14 days postpartum.