World-first treatment at Staffordshire Children’s Hospital at Royal Stoke for teenager with rare condition

The mother of a teenager suffering from a very rare and complex condition says she “still cannot believe” her daughter is finally cured following pioneering treatment at University Hospitals of North Midlands NHS Trust (UHNM).

As well as being one of just 21 globally recorded cases of a lymphatic condition called WILD syndrome, Kai Xue, of Bradford, also suffered from severe chylous ascites, when lymphatic fluid collects in spaces within the abdomen.

Kai was discharged from Staffordshire Children’s Hospital at Royal Stoke following a five week stay after surgeons fixed a leak in her liver and 28 litres of fluid was successfully drained from her abdomen.

Kai’s mum Ning Chen said “Kai was born with an abnormal lymphatic system, and her left arm was very swollen. Throughout her childhood we were under the care of a number of different hospitals to try to find out what the matter was, but nobody knew the cause. We tried everything, from restrictive diets to even flying to China for treatment. Having been transferred to so many hospitals, we’re very excited to finally be going home, I still cannot believe it.”

The 13-year-old was referred to Dr Mona Mossad, Consultant Interventional Radiologist at Royal Stoke and was admitted under the care of Dr Yvonne Slater, Consultant Paediatric Gastroenterologist at Staffordshire Children’s Hospital at Royal Stoke after those providing care to Kai in London identified Dr Mossad, a national expert in lymphatic intervention, as the only consultant in the UK who could perform such complex and rare interventions.

Dr Mossad said: “Kai was in a very difficult situation. After being seen by different centres in the UK and China, nobody could diagnose the cause of the leak that was leading to the build up of lymphatic fluid into her abdomen over the years. That leak caused two major problems. The first being the large amount of fluids which were leaking into her abdominal space causing severe abdominal distension and pressure on internal organs. The second was that if we drained these fluids to relieve the pressure, we would be removing all the nutritional fluid from her body- protein, fat, antibodies, electrolytes and white blood cells. These types of leaks are usually very difficult and multifactorial, there is not just one cause for it. So clinically and radiologically we tried to eliminate the causes starting with the least-invasive.

“We began with a procedure which has not been carried out in the UK on a child before and only handful of times on an adult - dilatation of the thoracic duct to improve lymphatic drainage. The procedure was challenging but went very successfully. Unfortunately though it did not help and Kai was still accumulating fluid in her abdomen.

“So the second stage was to see if the liver was the cause of the leak. This was a very challenging procedure, as we needed to visualise and block lymphatic vessels in the liver that measure less than one tenth of millimetre in adults. Because of Kai’s age and size, we had to especially order smaller needles that would work. We successfully managed to find a large leak that was going into her abdominal cavity from the left lobe in her liver and were able to repair the leak using a special surgical glue.”

Dr Mossad added: “I am extremely grateful to Dr Yvonne Slater who has been very supportive and highly knowledgeable in managing Kai clinically- draining 28 litres of highly-nutritional fluid safely, and optimising her nutritional state to enable us to perform the procedure safely.”

Dr Slater said: “We are all over the moon for Kai, who is the first child to undergo this treatment anywhere in the world. This case is unique for Staffordshire Children’s Hospital at Royal Stoke, as other children’s centres don’t have access to the skills of Interventional Radiologists like Dr Mossad, along with the specialist paediatric gastroenterology and intensive care available at UHNM.

“It was an extremely long, difficult, and specialised case, over the two procedures that involved the skills and dedication of many teams at UHNM including Radiology, Anaesthetics, Children’s Intensive Care, Gastroenterology and Dietetics. Despite this, Kai has remained remarkably robust, and after coming to us very weak, she has left a completely different girl, smiling for the first time in a long time. Once a patient has been with us for a period of time, you get to know them and the family, and I’m delighted that everybody has moved heaven and earth to do the right thing for them.”

Kai’s mum Ning added: “Kai is so special to me and I would like to say a very big thank you to everybody at both Staffordshire Children’s Hospital at Royal Stoke and UHNM for working hard to look after her. I’m so happy for the excellent care, and everybody was so nice and helpful and they tried their best to help us. The whole team is amazing.”