Joni Reinkemeyer, a 22-year-old American girl, gave birth to her baby twice. At the end of the 19th week, she learns that her baby is suffering from spina bifida, a malformation of the fetus that develops during the first months of pregnancy. Specifically, it is a failure to close the back of one or more vertebrae, leaving bare and unprotected the contents of the spine (meninges, spinal cord, nerve roots…).
The consequences of this malformation are multiple: hydrocephalus (and associated cognitive or mental retardation), muscle paralysis, urinary and faecal incontinence, sexual dysfunction or malformation and limb deformity.
One solution to save the baby: a risky surgery. The doctors have therefore made a rare decision: extracting the baby from the mother’s womb by caesarean section, operating it and putting it back in the womb so that it continues to develop until the end of the pregnancy. A heavy intervention that mobilized a team of 32 people. Little Jackson was born 10 weeks later and is doing well.
In 2014, teams from Trousseau Hospital and Necker Hospital in France also operated on a fetus five months before birth. For this, the surgeons had to exteriorize the uterus. “The uterus has to be soft like a sock,” said Gynecologist and obstetrician Jean-Marie Jouannic, who led the maneuver, “The mother is given contraindications and anesthetic drugs that also work on the fetus”. In this way, the doctors had access to the back of the baby where the malformation was. “The surgeon covers the spinal cord by welding the envelope that normally covers it and then sutures the skin of the fetus,” he said.
Usually, the operation is performed a few days after the birth of the baby, but studies have shown that prenatal surgery reduces the risk of developing motor and intellectual disabilities, especially because the repair of this malformation stops the leak of cerebrospinal fluid. To be able to operate, Jean-Marie Jouannic had trained on fetuses of lambs for several years.
Practiced in a dozen hospitals around the world, this delicate operation seems to give encouraging results, despite the risks it entails. In the days following the operation, the mother can give birth at any moment, reducing to nothing the benefits of the intervention and putting the life of her baby in danger.
Some babies are affected by “spina bifida occulta” or ““closed spina bifida“: the vertebral arches are poorly welded but the marrow remains protected by the column and by the skin, unlike the “open” spina bifida (“spina bifida aperta”) which leave bare nerve material. Spina bifida can be detected in the sixth month of pregnancy. In 80% of cases, doctors offer an abortion.
If the parents refuse to undergo the surgery, these disorders must be managed from birth, otherwise they lead to a rapid deterioration of the urinary tract (bladder and kidney).
Repeated infections may result in pyelonephritis, itself causing renal function impairment. In the long term, due to a lack of well-conducted preventive treatment, the baby develops prematurely renal failure and can be waiting for transplantation as early as the age of 20. Calculi (urolithiasis) can also disturb daily life. On the side of the bladder, the mucosa, subjected to a permanent inflammatory state, also risks to carcinize (urothelial carcinoma, squamous cell carcinoma …).