After six years of failed IVF, Nicole was onto her last embryo.
There were many happy tears when she was told the transfer was successful and she was finally having a baby boy - Emmett.
But those tears soon turned to that of devastation and worry during Nicole’s twelve-week scan when doctors detected some abnormalities.
“I was referred to Queensland Ultrasound for Women, and they found that Emmett had an exomphalos or omphalocele.”
Exomphalos (or omphalocele) is a congenital abdominal wall defect where abdominal organs herniate into the umbilical cord, covered by a protective sac. It occurs when the intestines fail to return to the abdominal cavity between the 6th–10th week of gestation.
“Generally, they organs should go back inwards, but his didn’t.”
At 20 weeks, Nicole was asked if she was going to continue with the pregnancy. She said yes and was referred to Gold Coast University Hospital’s Medical Director of Paediatric Surgery, Dr Jennifer Ah Toy.
“I met Dr Ah Toy who developed a plan for when I gave birth,” Nicole said.
“I was having scans every four weeks, and Emmett was measuring around the 98th percentile.”
Nicole was put on bed rest at 34 weeks due to a risk of pre-eclampsia, and Dr Ah Toy was preparing for Emmett’s birth.
“Roughly one in every 2,500 births have exomphalos,” Dr Ah Toy said.
“Normally the baby has the membrane sac over the top of the organs which protect them. We’ve had a few patients where we’ve left the sac intact, allowed skin to grow over the top, and repaired the muscle when the baby is about two years old, so that was our original plan.”
There were more than 25 clinicians in the theatre room the day Nicole was scheduled for a caesarean.
“Majority were there ready for Emmett, it was all hands-on deck,” Nicole said.
However, all plans went out the window when Emmett was born.
“The words I heard were, ‘the membrane’s ruptured,’” Nicole said.
Dr Ah Toy never had a membrane rupture before in her career, and immediately needed to come up with Plan B.
“For a sac to rupture is incredibly uncommon and Emmett was at great risk of infection,” Dr Ah Toy said.
Emmett immediately had emergency surgery where the surgeons put a “silo” on top of his organs.
“It was like a plastic bag to help cover his organs, and let gravity do it’s work,” Dr Ah Toy said.
“We let the swelling come down, let everything sink back inside the tummy. The issue was it’s not just that the belly’s open—it’s that the belly is open and very small, because the cavity hasn’t had the organs inside it the whole time, so we did the start of the staged closure for him.”
Over the next 10 days, Dr Ah Toy contacted clinicians across the country to develop a surgical plan.
“We sought advice from so many people in different areas – adult general surgeons, radiologists, rehab physicians. In the end, we thought, ‘let’s try something a little bit different.’”
Soon, a plan was in place: remove the silo, attach mesh to each side of the muscle wall, and every second day take Emmett back to surgery to remove a piece of mesh and pull it tighter.
“Emmett started with a 10-centimetre opening, which was essentially the same width as his abdomen,” Dr Ah Toy said.
“We estimated we’d get half a centimetre to a centimetre more closure each time and it would take around eight operations to be fully complete.
In the end, it only took five operations to get complete muscle closure and Dr Ah Toy even gifted Emmett a belly button.
“We got the opening down to six centimetres in the first round,” Dr Ah Toy said.
Nicole’s happy tears soon came flooding back.
“I got my first cuddle with him when he was exactly three weeks old, which made me cry even more.”
Emmett has thrived since the surgeries and is now enjoying home life with Nicole.
“I just can’t thank everyone enough,” Nicole said.
“They’re so incredible and talented. The support from the beginning has been amazing. Dr Ah Toy instantly made me feel calmer from the moment I met her, and the NICU doctors spoke to me beforehand, offered a walkthrough so it wouldn’t be overwhelming. I’ve had a lot of people helping me here.”
“Emmett has no more planned operations,” Dr Ah Toy said.
“We might tidy the scar in the future because the baby skin wasn’t great quality, but we’re hoping this is it for him.”
Dr Ah Toy will be presenting Emmett’s case at a conference in May, as the first surgery of its kind in Australia.
