Daily Trust

...One in 14,000 born with the rare genetic overgrowth disorder

- Source: BeckwithWi­edemann Syndrome Support Group and National Institutes of Healt

Beckwith-Wiedemann syndrome is a congenital overgrowth disorder, which can affect all parts of the body.

BWS is a rare genetic condition, affecting around one in every 13,700 births worldwide.

The figure is likely to be higher in reality, with mild cases rarely diagnosed.

Cases have been reported in most developed countries, and in the majority of cases it appears to be an isolated case with no known relatives suffering the disorder.

In some cases there is evidence the condition can be inherited.

Children with BWS are often born prematurel­y but are larger and heavier than expected, given their early arrival.

Typically a child born with BWS will have an enlarged tongue, which can causes breathing, feeding and speaking difficulti­es, as well as excessive dribbling. It can also cause respirator­y problems and result in a protruding lower jaw.

Babies can be born with reddended skin on the forehead and eyelids, which usually fades in the first few years.

Children born with BWS are at risk of developing various types of tumours.

Around 7.5 per cent will develop a Wilms Tumour, a tumour of the kidney.

Due to the aggressive nature of these tumours, a child with BWS will need to be screened every three months until the age of seven or eight, when the susceptibi­lty to the tumours diminishes.

Children with the condition are also likely to suffer enlarged abdominal organs, overgrowth of one half of the body, liver tumours and an enlarged heart or heart defects.

BWS is diagnosed geneticall­y in 80 per cent of cases, with the remainder being diagnosed after a clinical examinatio­n.

 ??  ?? Ocea Varney, now 18 months, was born with her tongue stuck poking out due to a rare genetic condition
Ocea Varney, now 18 months, was born with her tongue stuck poking out due to a rare genetic condition

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