A baby’s bladder ballooned so big that it ‘crushed his own kidney’ due to a rare condition that triggered a build-up of urine in the organ.
Six-month-old Oliver Smith suffers from Eagle-Barrett syndrome, or Prune Belly, which prevented his urethra from forming properly in the womb.
It meant his body was unable to remove urine which lead to urine clogging up his bladder, causing it to expand.
Oliver, from Los Angeles, had to have surgery to drain the organ from inside his mother’s womb a month before he was born.
But it had already caused chronic kidney disease and the damage was irreparable by the time of his birth.
The baby was in complete kidney failure and had to be put on dialysis at just one-week-old.
He miraculously regained some function and began urinating normally, but his kidney disease diagnosis means he will need a transplant in the next few years.
His mother Violet and father Lionel are now saving up to pay for the operation, as well as any future surgeries their son may need.
Six-month-old Oliver Smith suffers from Eagle-Barrett syndrome, or Prune Belly, which caused his bladder to expand so big it crushed his own kidney
The condition prevented his urethra from forming properly in the womb – meaning his body was unable to remove urine
This lead to fluid clogging up his bladder, causing it to balloon. Prune Belly syndome also meant he was born without any stomach muscles
His mother Violet and father Lionel are now saving up to pay for the operation, as well as any future surgeries their son may need
Mrs Smith, 33, said: ‘Oliver is the sweetest baby you’ll ever meet. He’s always smiling and thinks his big sis is incredibly funny no matter what she’s doing.
‘He has spent a majority of his life in the hospital and has had five surgeries to date with many more to come. The nurses at his hospital joke that nobody bothered to tell him he’s sick.
‘We’re so proud of him and hope to encourage this sweet and carefree attitude throughout his life dispute the difficulties he will face.
‘He is going to face a lifetime of medical needs, but we’re prepared to face whatever challenges his condition presents.’
Eagle-Barrett syndrome affects around one in 40,000 people and causes problems in the urinary tract and complete absence of the stomach muscles.
Speaking about her son’s condition, Mrs Smith added: ‘Oliver’s urethra didn’t form properly, causing his urine to back up until his bladder was bigger than his whole body while in utero [the womb].
Mrs Smith explained that her son’s kidney is ‘very sensitive’ and can completely fail at any given moment
Doctors first became concerned for Oliver’s health at a 16-week scan to find out the sex of the baby. When the ultrasound came up on the screen, all it showed was a large black hole in her womb
‘The big black hole was Oliver’s bladder – it was so enlarged you couldn’t see anything else,’ according to his mother
‘His distended bladder was crushing him, causing kidney damage, prohibiting his lungs from growing properly, and making it so he was unable to build abdominal muscles.
‘He had to have a life-saving foetal surgery before he was born during which they placed two shunts to drain his bladder and restore his amniotic fluid.
‘The surgery was a success, but significant damage had already been done; Oliver was born with complete kidney failure and without abdominal muscles to keep his organs safe and help his body function like it should.
‘He had to go on dialysis at one week old and we were devastated because the mortality rate for infants on dialysis is very high.
‘After a few weeks of dialysis, Oliver’s dialysis catheter started leaking and the treatment was put on hold. Then something miraculous happened.’
Oliver was diagnosed with chronic kidney disease which means he will need a transplant by the age of four or five
He also suffers from decreased mobility and developmental setbacks because of his lack of abdominal muscles
WHAT IS PRUNE BELLY SYNDROME?
Prune Belly Syndrome (PBS) occurs when babies are born without abdominal muscles, causing the skin on their torsos to wrinkle and appear ‘prune like’.
PBS affects around one in 40,000 births, with 97 per cent of sufferers being male.
As well as lacking abdominal muscles, one of the main symptoms of PBS is undescended testicles.
Others symptoms, which vary in severity between patients, can include:
- Enlarged bladder and kidneys
- Heart defects
- Spine malformations
- Club foot
- Digestive issues
PBS’ cause is unclear but may be releated to a genetic mutation.
This may lead to a blockage in the flow of urine in the urinary tract, causing it to flow back to the bladder, enlarging the organ.
Treatment options vary depending on PBS’ severity, but generally require babies have surgery to repair their abdominal muscles, as well as relieving any genital or bladder problems.
Antibiotics may be required to prevent or treat urinary tract infections.
Mrs Smith explained that her son’s kidney is ‘very sensitive’ and can completely fail at any given moment.
She added: ‘He was able to come off of dialysis and his kidney function is managed with medications.
‘He will need a kidney transplant but will not meet the weight requirement until around three to five years old. I’m hoping I will be a match so I can be his donor, and Lionel hopes so too.
‘He also suffers from decreased mobility and developmental setbacks because of his lack of abdominal muscles and precarious medical condition.
‘He receives physical therapy twice per week and occupational therapy to work on his ability to eat by mouth once per week.’
Doctors first became concerned for Oliver’s health at a 16-week scan to find out the sex of the baby.
When the ultrasound came up on the screen, all it showed was a large black hole in her womb.
Mrs Smith added: ‘The doctor began the ultrasound and my stomach immediately sunk. There was no baby in sight, only a big black hole.
‘Something was very wrong; fear washed over us as the doctor tried to make sense of what we were looking at.
‘The big black hole was Oliver’s bladder – it was so enlarged you couldn’t see anything else.
‘We were referred to a foetal surgeon, who told us the baby needed an emergency life-saving surgery if he was going to survive.’
The surgery saw medics use a giant needle to drain fluid from Oliver through his mother’s belly.
His mother added: ‘It was the scariest thing I’ve ever done. I couldn’t be put under anaesthesia for the safety of the baby, so I tried to lay as still as possible even though I could feel them cutting through me,” she said.
‘In fact, the entire pregnancy was scary. I battled depression, walking around in a fog wondering if my baby boy was going to survive. I tried to put my energy towards my then-one-and-a-half-year-old daughter; I knew she needed me.’