1)What kind of conjoined twins are they? JS: They are Omphalopagus twins, meaning “Belly,” or “abdomen”. They were fused at the lower chest through the abdomen.
2)What percentage of conjoined twins are born like this? JS: This is the most common kind of conjoined twin, representing around one in 200,000 live births. Additionally, the fact that they were triplets makes it very unusual. Omphalopagus represent an estimated 30 percent of all conjoined twins born annually.
3)What organs and bones did they share? JS: They had a fused liver – each one had a full liver but they were fused together across their anterior surfaces. Because they only shared the liver and the Xiphoid, without pelvic structures or intestine, this surgery took less time than previous separations we have performed at Children's Hospital. This was obviously a complex undertaking but we were able to compile appropriate resources in Los Angeles to do the surgery in Haiti.
4) Where was the first incision made? JS: The first cut was made along the abdominal wall and skin that connected the two sisters.
5)Discuss when Michelle’s heart rate rose, alarming the team.JS: Due to the fusion of Michelle’s liver with Marian’s, a cross circulation occurred where fluids, blood and medication that were being given to Michelle were crossing over to Marian and having less effect on Michelle, causing her heart rate to rise. This occurred early in the surgery. Surgeon Henri Ford, the team leader, and surgeon Aaron Jensen and I divided the livers within 20 minutes of starting. By expeditiously and safely dividing it early in the procedure – we stabilized Michelle. The fluids, blood and medication we gave her stayed with her and blood pressure responded accordingly to the anesthesiologists’ intervention.
6)Can you provide a number of procedures for the surgery? JS: We did 11 procedures: the separation of the Xiphoid bone; the transection of the shared abdominal wall; the splitting of the liver; the removal of a left ovarian cyst in Michelle; the reconstruction of their abdominal walls, including inserting patch material. The patch material was was required up high in the abdominal wall where the Xiphoids were joined and there was insufficient muscle tissue to bring together. We also put in central lines, arterial lines for monitoring – a central jugular venous line or catheter in the neck of each baby into the heart to infuse fluids and drugs and monitor the hydration status to assist the anesthesiologists in managing fluids and other medications. Lastly, we reattached the front part of the diaphragms where the Xiphoid connected.
7)Walk readers through the separation process. JS: We spent 20 minutes assessing the belly (which resulted in our identifying the ovarian cyst in one of the girls)and verifying the liver anatomy. The liver separation took another 20 minutes, and then 30 minutes to divide the Xiphoid and complete the remainder of the separation.
8) Anything unexpected occur? JS: The cross circulation – the anesthesiologists were aware it could happen and so were completely on top of it. We didn’t expect the ovarian cyst since we didn’t have the same level of imaging in Haiti that we have at CHLA. Beyond that our entire timeline and plan were right on.
9) How are the girls? What do they face? JS: I don’t expect any more surgery – I think they are done. I have been skyping every day – they look great and were ready to go home 12 days after surgery. They are feeding with formula and breast milk. Both were off oxygen within 48 hours. We are working with physical therapy on their necks because each girl has always preferentially turned one way – Michelle turned her head left and Marian – right – so they faced outward as best they could and we need to work on range of motion on their necks.
10) What is the Prognosis? JS: They may be a tiny bit delayed in learning to walk – maybe by a month or two…and each will have a midline vertical scar but that will become non-noticeable as they heal. We all expect them to live completely normal lives.
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