‘One-of-a-kind’ girl born with heart outside chest has pioneering surgery

Vanellope Hope Wilkins made medical history when she was born with her heart outside of her body in 2017.

Described by experts as “one of a kind”, Vanellope had three operations to place her heart back in her chest due to an extremely rare condition called ectopia cordis.

The hospital where she was born – Glenfield Hospital in Leicester – says it knows of no other case in the UK where the baby has survived.

Now seven years old, Vanellope has undergone groundbreaking surgery to reconstruct a protective cage around her heart – using her ribs.

The BBC was given exclusive access into theatre, when the operation took place at the East Midlands Congenital Heart Centre, based at the Leicester Royal Infirmary, last Wednesday.

Warning: This story contains an image of surgery

It’s early morning outside theatre, and a team of specialist medical experts gather for a briefing to discuss the unprecedented surgery about to take place.

Vanellope still has no breastbone, leaving her heart covered by just by a thin layer of skin.

Surgeons have come up with a plan to use her ribs to form a protective cage inside her chest.

Consultant paediatric surgeon Nitin Patwardhan was one of the 50-strong medical team at Vanellope’s birth on 22 November.

Now he is set to play a leading role again in surgery, which has never been performed like this before.

“I’d lie if I say I don’t get nervous,” he says. “But having been in this profession for so many years, you actually look forward to it because at the end of the day, you’re doing something that will change somebody’s life.”

He remembers how “everything was unknown” when Vanellope was born, because no-one in the country had ever dealt with a similar case.

A handful of children in the US have also survived this condition.

Ectopia cordis affects only a few cases per million births – and Vanellope was given a less than 10% chance of survival.

But she defied those odds and was allowed home after 14 months in hospital.

Since then, Vanellope has had to wear a brace around her chest to protect her heart.

She has complex medical needs, which require one-to-one care, 24 hours a day. She is autistic and non-verbal.

Mum Naomi Findlay, 39, who lives in Clifton, Nottinghamshire, described her daughter as “a happy little thing” who “brings a lot of joy and a lot of happiness”.

She added: “It makes me extremely proud to see actually how how far she’s come, what she’s overcome and what she is achieving. It’s a real journey of strength and bravery… she’s so brave.”

Having to kiss her daughter goodbye at the theatre door is difficult and the tears flow.

“A lot of anxiety, a lot of worry and so many emotions,” she says. “For me, the biggest fear is handing her over and not getting her back.”

The team waiting in theatre include specialists from the Congenital Heart Centre, Leicester Children’s Hospital and a visiting cardiothoracic surgeon from Great Ormond Street Hospital in London.

Consultant congenital cardiac surgeon, Ikenna Omeje, operated on Vanellope just 50 minutes after she was born.

He recalls there being a lot of “head scratching” among surgeons, because the condition was so rare.

“It was exciting for everyone because it was something we had never seen before. So you made the news, not just within the UK, but all over the world,” he says.

Explaining the current procedure, he points to a 3D scan of Vanellope’s chest, and describes how her heart has attached to the thin layer of skin protecting it.

Detaching it “is risky”, he confesses.

“You can damage the vessel trying to get into the chest cavity,” he said.

Now aged seven, surgeons decided Vanellope had reached the right age for reconstructive surgery to form a permanent structure around her heart.

Vanellope is first placed on a bypass machine, which temporarily takes over the functions of the heart and lungs.

This allows her heart to deflate, allowing the “very tricky” procedure to detach part of her heart – the right ventricular outflow tract – and the pulmonary artery from where it has stuck to her skin.

Next, the bilateral rib osteotomy takes place, which involves breaking Vanellope’s ribs on both sides to move them in order to form the protective cage around her heart.

Carrying out all of the procedures at once, on a patient with ectopia cordis, has never been done in this country before, the team says.

The extremely complex procedure lasts more than nine hours. And it’s a success. The surgeons are delighted.

Mr Omeje is beaming as he says it “went a lot better than we expected”.

He describes how he examined an X-ray of Vanellope’s chest after the operation, and called it “really beautiful”.

Mr Omeje called Vanellope “one of a kind” and explained how fulfilling it was to have everything go so well.

“The best satisfaction we derive from this is when you get a text message from the mum to say ‘thank you, you guys are amazing’,” he says.

“I think personally, I have just done my job, but it has made a difference to someone and that is very satisfying.”

Vanellope is taken to the children’s paediatric intensive care unit and will spend some time in hospital recovering.

After a few weeks, she will be able to take off her protective chest brace and won’t require any further surgery in the future.

Her mum called the NHS “amazing”, and says she can’t wait to take her home to her older brothers and little sister.

Looking to the future, she hopes this surgery will improve Vanellope’s “already really, really good quality of life”.

And Naomi says this time, she’s ready for the long journey ahead.

“When [Vanellope] was born I was fearful. It was very scary for me, but this time round I’m ready.

“We’ve got this,” she added.