On Discovering It Was Twins…

As the ultrasound tech quickly scanned through I caught glimpse of two small bubbles and asked her to stop. “Is that two?” I asked, as she measured, snapped, and moved on. “The doctor will be in to talk to you in a second.”

We’d been trying for months and I could hardly grasp the two pink lines, much less the thought of this baby being babies.

… “No seriously, IS THAT TWO?”

She stopped scanning and looked at me with a ‘not-saying-yes-not-saying-no’ look and said “Everything looks fine. I’m going to get Dr. Yeomans now, just sit tight.”

I knew Dr. Yeomans, he was an incredible MFM physician who also happened to be my mentor. As a third year med student, I also knew a (tiny) bit about ultrasound. I looked at my husband – “I think there might be two. I think I saw two in there. YOU DID THIS! Did you see two? YOU PRAYED FOR THIS! Do you think it’s TWO? What if it’s three?! YOU THOUGHT TWINS WOULD BE COOL AND NOW IT’S HAPPENING – WHAT ARE WE GOING TO DO?! Oh my gosh what are we going to do? I’m going to have to drop out of medical school!”

If you’ve been following for more than half a minute you know it was, indeed, twins…and I did not, in fact, drop out of anything. I would leave that ultrasound appointment and give a collaborative presentation to 150 people for my Family Medicine rotation. Shaking, nauseated, and in a full-on cold sweat, I would get up in front of 150 people who thought I was a terrible public speaker and discuss hypertension, while silently (but not covertly) panicking about the state of affairs in my uterus.

Those two blips on the screen are now smart, sassy, beautiful 5yo girls. Someday I’ll tell them of the months it took me to go from terrified and overwhelmed to excited about the fact that there were two of them (and how I simultaneously could not imagine it any other way after that first moment). The incredible chaos they bring to our lives is exactly what we need every single day.

Finding out about their little brother was similarly shocking, but in a totally different way. Getting pregnant with the twins had been this whole ordeal involving infertility, doctors, ultrasounds, prayers, medicine, and third year rotations with ovulation prediction kits in hospital bathrooms. With Milo, however, it happened so fast that I was completely unprepared and, nearly embarrassingly, blindsided.

That positive test, however, was followed by a threatened miscarriage in the midst of a busy and mentally-challenging night float rotation. That was followed by un ultrasound with no heart beat and so little hope (from me) that he would actually stick that I just had my residency BFF scan me alone, because I so desperately did not want my husband to witness what I knew would break his heart.

As rain pattered on the clinic window, my co-resident and best friend turned the screen to me and laughed – “look, it’s fine. See right there – a little grain of rice with a heartbeat.” My tears swiftly joined the downpour outside, “I would’ve told a patient to stop stressing and wait for the second scan…I am never, ever saying that to someone again…”

In the next few days I’ll share the rest of this story and answer one of the most frequatly asked questions on MamaDoctorJoneswhat was it like having kids in medical school and residency?

Twin-Twin Transfusion Syndrome

I want to start sharing a bit more educationally-focused information here. However, in line with my long history of utilizing social media to connect with patients and understand the human side of the story, I hope to employ my non-medical, digital-friends to inspire these topics.

Case courtesy of Dr Alexandra Stanislavsky, Radiopaedia.org. From the case rID: 51114
Fetal MRI of Stage III Twin-to-Twin Transfusion – Case courtesy of Dr Alexandra Stanislavsky, Radiopaedia.org. Case: rID: 51114

Hannah from Daytrips & Diapers has graciously given me permission to discuss/link her in this post. She is pregnant with monochorionic – diamniotic twins and was diagnosed with twin-twin transfusion syndrome (TTTS) at 16 weeks. She recently traveled to the nearest fetal surgery center, >8 hours from her home, and underwent a procedure aimed at decreasing placental connections and improving blood flow (and hopefully, outcomes) for both babies.

She then experienced a known complication of the surgery, however at the time of my writing is still joyfully pregnant with both of her twins. Hop over to her blog or Instagram and follow her story for the human side of medicine. Read on below if you’re interested in the medical side – I’ll discuss diagnosis, pathophysiology, and treatment of TTTS below.

 

What is Twin-Twin-Transfusion Syndrome?

  • A complication isolated to monochorionic (one placenta) twin pregnancies where vascular connections on the shared placenta develop pressure/flow gradients. This allows one twin (“donor”) to become anemic and the other (“recipient”) to become plethoric and begins a cascade of physiologic changes which can become catastrophic.

How Often Does This Occur?

  • The incidence in mo/di pregnancies is about 10-15% and bout 6% in mo/mo twins develop TTTS. Dichorionic twins cannot develop twin-twin transfusion syndrome, as they have two placentas.

How Is Twin Twin Transfusion Syndrome Diagnosed?

  • The diagnosis is made clinically by ultrasound. It is classically identified by “Poly-Oli Sequence”
    • !! This will be how it’s presented on USMLE-type exams. !!
      • Refers to polyhydramnios of recipient twin and oligohydramnios of donor twin.
    • Occasionally, there will be such severe oligo in the donor twin that it develops “Stuck Twin,” which is essentially the ultrasound appearance of a donor twin being stuck to the placenta. Anatomically, this results from such severe oligohydramnios that the amniotic sac is lying directly against the fetus.

What’s The Pathophysiology?

Basic overview of donor and recipient pathophysiology in TTTS.
Basic TTTS Pathophysiology
  • DONOR: Relative Hypovolemia –> Kidney Injury –> Hypertension
  • RECIPIENT: Relative Hypervolemia –> Vasodilation/Diuresis –> Polyhydramnios –> Hypertension
  • BOTH: Hypertension –> Cardiac Dysfunction –> Hydrops Fetalis
  • Click Flow Chart to enlarge for more detail

How Bad Is It?

  • It depends on the stage.
  • Quintero Staging
    • I: Poly-Oli Sequence (Visible Donor Bladder + Normal Doppler Flow)
    • II: Poly-Oli Sequence + Collapsed Donor Bladder (Normal Doppler Flow)
    • III: Poly-Oli Sequence + Non-Visualized Bladder + Abnormal Doppler Flow
    • IV: Hydrops Fetalis in one or both twins
    • V: Fetal Death in One or Both Twins

How Is It Treated?

  • The treatment course depends on gestational age at diagnosis and severity/stage, however there are basically 3 options:
    • Expectant Management (i.e. No intervention, just watch)
    • Fetoscopic Laser Ablation
    • Amnioreduction (removing the “Poly” of the poly-oli sequence)
Fetoscopic Laser Ablation - Edited by me, click for original source from Japan Fetal Therapy Group
Fetoscopic Laser Ablation – Edited by me, click image for detail. For original source – Japan Fetal Therapy Group

What is Fetoscopic Laser Ablation?

  • This is a surgical procedure which is performed by fetal surgeons. It involved laser cauterization of superficial anastomotic placental connections through minimally invasive fetoscopes.
  • Maternal preparation includes complete ob ultrasound (including placental location, distance between umbilical cord insertions, and fetal locations/presentation/sizes) and medications (antibiotic + tocolytic +/- steroid for fetal lung maturity depending on gestational age). The procedure is enerally performed with local or regional anesthesia + sedation

Fetoscopic Laser Ablation Procedure Steps

  • Skin incision with scalpel (site determined by placental and fetal locations)
  • Entry Options
    • 18g hollow-needle introduced through maternal abdominal wall and placenta
      • J-Wire guide placed –> Needle removed –> Catheter placed over guidewire with US guidance
      • Metal trocar placed through cannula to allow for uterine entry
    • 2-3mm Fetoscope (with or without angled scope, depending on entry-technique and surgeon preference) inserted
  • Identify all fetal extremities to avoid injury
  • Identify vascular equator
    • Typically in recipient amniotic sac (parallel to “stuck twin” if present)
  • Anastomoses are mapped out and typed
    • Types include arterial-venous (AV), venous-arterial (VA), arterial-arterial (AA), and venous-venous (VV)
  • Visible connections are coagulated using laser ablation
    • Sequential selective ablation (AV –> VA –> AA –> VV) reduces intrauterine fetal death rates
  • Vascular equator re-identified and coagulated

What are the Complications and Outcome?

  • Complications of the Procedure
    • Preterm Labor
    • Preterm Delivery (Avg. gestational age is 31-33w)
    • Preterm Premature Rupture of Membranes (PPROM)
    • Intertwin Membrane Rupture
      • Essentially bringing all the risks of monoamniotic twins into play (like cord-entanglement)
    • Pseudo-Amniotic Band Syndrome
    • Fetal Demise
      • Perinatal Survival Rates: 65%
        • Still with long-term neurodevelopmental, cardiac, & renal morbidities associated with survival.
      • OVERALL AFTER LASER
        • 50% both survive
        • 33% one survivor/one fetal demise
        • 33% both with fetal demise

Wow, Those Odds Look Bad…

  • They are daunting, for sure. However, TTTS severe enough to warrant laser-intervention which is left completely un-treated will result in fetal death of one twin in nearly 100% cases and both twins in >90%. These outcomes, when appropriate candidates are selected, are far superior to no treatment.

 

That’s a brief overview of TTTS and fetoscopic laser ablation. I’ve done a few LIVE Q&As on Twins, I’ll try to round those up to get on YouTube for a more thorough discussion of the pathophysiology and also the different types of twins.

It’s so important when learning about things like this that we don’t forget there’s a human (or, in this case 3) on the other side of our text books. Hop over to the blog and/or IG linked in the intro paragraph for a view into how this looks from a patient perspective.