Girl born with heart outside her body survives surgery in UK first

Ectopia cordis is thought to affect five to eight children in every million and chances of survival are less than 10 per cent

*Warning* This article contains graphic images

A baby that needed surgery within an hour of being born to correct a rare condition that caused her heart to grow outside her body has survived a life-saving operation in what is thought to be a UK first.

Vanellope Hope Wilkins was diagnosed with the condition ectopia cordis after a nine week pregnancy scan showed her heart and part of her stomach had developed on the outside of her body.

Surgeons at Glenfield Hospital, a specialist children’s heart centre in Leicester, initially believed Vanellope would be impossible to save.

Surgeon’s recommended a termination to her parents as the chances of survival were so low, less than 10 per cent, but Vanellope’s mother, Naomi Findlay, said “this wasn’t an option” for her.

Frances Bu’Lock, the consultant paediatric cardiologist at Glenfield Hospital, said Vanellope’s chance of survival initially looked “remote”.

“I had seen one in foetal life around 20 years ago but that pregnancy was ended,” she said.

“I did a quick Google search, as everyone does, and then more of a literature search but that didn’t inform me an awful lot because there’s not much to go on and the cases are all very different.”

However after three surgeries, to move her heart into her chest and create an artificial rib cage and sternum, she is now believed to be the first successfully treated case of ectopia cordis in the UK.

Vanellope was due to be delivered on Christmas Eve but was born by caesarean on 22 November and life-saving surgery began within the hour.

Her father, Dean, said the couple were told the first 10 minutes after birth were crucial.

He said: “What they said is, when the baby is born she has got to be able to breathe in our oxygen.

“Twenty minutes went by and she was still shouting her head off – it made us so joyful and teary.”

The couple said the baby was named after a character in the Disney film, Wreck It Ralph.

Naomi said: “Vanellope in the film is so stubborn and she turns into a princess at the end so it was so fitting.”

Her doctors say that so far Vanellope’s health appears to be normal outside of her heart condition, but it is impossible to say for certain.

June Davison, senior cardiac nurse at the British Heart Foundation, said that the Vanellope’s future health would now depend on her ongoing treatment.

“The prognosis depends on the severity of the condition and if there are other abnormalities, but treatment needs to begin immediately after birth.

“We are very happy that this baby survived to undergo surgery and wish them all the best.”

Three surgeries, fifty staff, and an artificial rib cage

The medical effort to save Vanellope’s life

 

A team of around 50 staff got together from around 8am with specialised equipment before a theatre briefing at 8.30am.

At 9:50am Vanellope Hope was born and was immediately wrapped in a sterile plastic bag, and taken into an adjoining anaesthetic room.

The first operation involved special lines which were inserted into the blood vessels in her umbilical cord to give fluids and medications to support her heart, and to monitor her blood pressure accurately.

Consultant neonatologist Jonathan Cusack said: “At around 50 minutes of age, it was felt that Vanellope was stable enough to be transferred back to the main theatre where she had been born to the waiting anaesthetists, congenital heart disease and paediatric surgical teams who began the task of putting her entire heart back inside her chest.”

Vanellope was transferred to the Paediatric Intensive Care Unit where she will stay for several weeks, while she recovers.

After seven days, medics decided they could conduct the second operation, which was to open her chest a bit more so they could create more space for the heart to fit back in.

Over a period of around two weeks, her heart naturally made its way back into the chest due to gravity.

This allowed staff to carry out the final operation which involved taking skin from under her arms and moving it round to join in the middle of her body.

Surgeons had created a mesh which protected the heart as she did not have ribs or a sternum.

As her organs fight for space inside her chest, Vanellope is still attached to a ventilation machine.

East Midlands Congenital Heart Centre lead surgeon Branko Mimic said: “Cases such as Vanellope’s, where everything else appears essentially normal, are even rarer, and whilst therefore it would seem more hopeful she will do well, it is therefore almost impossible to be confident of this.”

Additional reporting by PA