The WILD syndrome is characterised as a group of symptoms such as warts, immunodeficiency, lymphoedema and anogenital dysplasia as a common occurrence. Kai Xue, 13, is one case out of 21 recorded cases of WILD syndrome worldwide. It is a rare and complex condition.
She has been moving from hospital to hospital, almost her entire life of existence, going undiagnosed and untreated. She was also suffering from chylous ascites, which is lymphatic fluid collecting in abdominal spaces. Staffordshire Children’s Hospital at the Royal Stoke made an impactful difference. She is now cured once and for all of a disease that could not be distinguished from by many doctors.
There is a criteria for WILD syndrome which is as follows:
▶Essential Criteria-
Presence of Early life-onset multi segmental PL (pleural lavage) at <2 years of age (100%)
⇨Major Criteria to look out for which occur in at least 70% of cases-
Cutaneous lymphovascular malformation on the chest/neck (95%) (may not be present in the first few years of life!)
Genital Swelling (86%)
Recurrent warts (81%)
CD4 lymphopaenia (89%) (less number of WBCs)
Systemic involvement (70%) (this involves the body rather than just an organ)
Dr Mona Mossad is an expert in lymphatic interventional methods and is also a Consultant Radiologist. Along with Dr Yvonne Slater who is a Consultant Paediatric Gastroenterologist try to uncover the root cause and solution. They attempt a procedure that involves the dilatation of the thoracic duct to improve lymphatic drainage-this is commonly used on adults and not on children. Smaller needles were equipped as the patient is quite young. She is also the 1st to undergo this form of treatment.
The doctors were presented with two surgical problems in this case; one was if the fluid was drained out she would have lack of essential nutrients and electrolytes. Another one is that, this tends to be a multi-factorial cause, in addition to the lymphatic fluid giving pressure on her internal vital organs, so an invasive surgery might be needed and that the patient they were operating on was a child an not an adult! Hospitals in China and UK were unable to breakdown the causes for this. When thoracic dilatation did not work, the surgeons decided to take a look at her liver. They found out that she had a leak in the left lobe of her liver.
She remained for five-weeks, 28 litres of fluid was removed and her liver was repaired with the help of a surgical glue. This would avoid any future leaks. The leaking had spread the lymphatic fluid in her abdomen, and due to less abdominal space it caused abdominal distension and pressure on other organs.
The intervention turns out to be successful. Kai is able to go home and is currently discharged. It was a super-specialised case that took up a huge team of staff of UHNM and the Staffordshire Children’s Hospital, it was two procedures in hand.
Kai’s mother is incredibly indebted to the staff for having performed these miracles of healing for her daughter.
Nivea Vaz
Manipal College of Medical Sciences, Pokhara
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