Fetal surgery for spina bifida

A woman in the United Kingdom claims she has undergone what is known as “fetal repair” surgery after learning about her baby's diagnosis of spina bifida, a birth defect that affects the spine.

Bethan Simpson, of Maldon, Essex, was informed that her unborn daughter Eloise has spina bifida in December. At that time, Simpson said she was given three options: “continuing pregnancy, ending [the] pregnancy or a new option called fetal surgery - fixing her before she is born,” she wrote on Facebook.

The 26-year-old mom-to-be chose the third option — making her “one of the few” women in the U.K. to undergo the procedure to correct the defect, according to the BBC. Simpson claimed on Facebook she is the fourth woman in the country to undergo the surgery…

For Simpson, after she and Eloise were approved for the pioneering surgery — a process Simpson described as a “rollercoaster" — doctors spent roughly four hours correcting the baby’s defect. They opened Simpson’s womb to expose Eloise's bottom. From there, they "sewed up" the small gap in the baby’s lower spine and also repositioned her spinal cord, the BBC reported.

“We were a success. Her lesion was small and she smashed surgery like you wouldn't believe,” Simpson, who was 24 weeks along at the time of the surgery, later wrote on Facebook.

“I'm fragile and sore but as long as she is doing fine that all we care about,” she continued, adding “they took her out of my womb and popped her straight back in to stay there as long as she can.”

Dominic Thompson, a neurosurgeon who led the surgery, told the outlet the procedure is “not a cure,” but noted that previous trials have indicated “the outlook can be a lot better with surgery early on.”

In fact, according to the Children’s Hospital of Philadelphia, “fetal surgery for spina bifida greatly reduces the need to divert fluid from the brain, improves mobility and improves the chances that a child will be able to walk independently.”

For Simpson, she considers her daughter — who is due in April — to be “extra special.”

“I feel our baby kick me day in and day out. That's never changed. She's extra special. She's part of history and our daughter has shown just how much she deserves this life,” she wrote.

https://www.foxnews.com/health/womans-unborn-baby-undergoes-spinal-surgery-while-still-in-the-womb-she-deserves-this-life

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Prenatal repair of myelomeningocele (MMC), the most common and severe form of spina bifida, is a delicate surgical procedure where fetal surgeons open the uterus and close the opening in the baby's back while they are still in the womb. Because spinal cord damage is progressive during gestation, prenatal repair of myelomeningocele may prevent further damage.

Fetal spina bifida surgery is one of the most exciting developments in the history of treatment for birth defects. An extremely complex procedure available only to qualified candidates, fetal surgery for myelomeningocele requires significant commitment on the part of mothers who choose to go forward with it and extensive surgical experience to perform successfully.

Fetal surgery for spina bifida is not a cure, but studies show that prenatal repair can offer significantly better results than traditional postnatal repair. Fetal surgery for spina bifida greatly reduces the need to divert fluid from the brain, improves mobility and improves the chances that a child will be able to walk independently.

https://www.chop.edu/treatments/fetal-surgery-spina-bifida/about

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Farmer DL, Thom EA, Brock JW 3rd, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Gupta N, Adzick NS; Management of Myelomeningocele Study Investigators. The Management of Myelomeningocele Study: full cohort 30-month pediatric outcomes. Am J Obstet Gynecol. 2018 Feb;218(2):256.e1-256.e13.

Abstract

BACKGROUND:

Previous reports from the Management of Myelomeningocele Study demonstrated that prenatal repair of myelomeningocele reduces hindbrain herniation and the need for cerebrospinal fluid shunting, and improves motor function in children with myelomeningocele. The trial was stopped for efficacy after 183 patients were randomized, but 30-month outcomes were only available at the time of initial publication in 134 mother-child dyads. Data from the complete cohort for the 30-month outcomes are presented here. Maternal and 12-month neurodevelopmental outcomes for the full cohort were reported previously.

OBJECTIVE:

The purpose of this study is to report the 30-month outcomes for the full cohort of patients randomized to either prenatal or postnatal repair of myelomeningocele in the original Management of Myelomeningocele Study.

STUDY DESIGN:

Eligible women were randomly assigned to undergo standard postnatal repair or prenatal repair <26 weeks gestation. We evaluated a composite of mental development and motor function outcome at 30 months for all enrolled patients as well as independent ambulation and the Bayley Scales of Infant Development, Second Edition. We assessed whether there was a differential effect of prenatal surgery in subgroups defined by: fetal leg movements, ventricle size, presence of hindbrain herniation, gender, and location of the myelomeningocele lesion. Within the prenatal surgery group only, we evaluated these and other baseline parameters as predictors of 30-month motor and cognitive outcomes. We evaluated whether presence or absence of a shunt at 1 year was associated with 30-month motor outcomes.

RESULTS:

The data for the full cohort of 183 patients corroborate the original findings of Management of Myelomeningocele Study, confirming that prenatal repair improves the primary outcome composite score of mental development and motor function (199.4 ± 80.5 vs 166.7 ± 76.7, P = .004). Prenatal surgery also resulted in improvement in the secondary outcomes of independent ambulation (44.8% vs 23.9%, P = .004), WeeFIM self-care score (20.8 vs 19.0, P = .006), functional level at least 2 better than anatomic level (26.4% vs 11.4%, P = .02), and mean Bayley Scales of Infant Development, Second Edition, psychomotor development index (17.3% vs 15.1%, P = .03), but does not affect cognitive development at 30 months. On subgroup analysis, there was a nominally significant interaction between gender and surgery, with boys demonstrating better improvement in functional level and psychomotor development index. For patients receiving prenatal surgery, the presence of in utero ankle, knee, and hip movement, absence of a sac over the lesion and a myelomeningocele lesion of ≤L3 were significantly associated with independent ambulation. Postnatal motor function showed no correlation with either prenatal ventricular size or postnatal shunt placement.

CONCLUSION:

The full cohort data of 30-month cognitive development and motor function outcomes validate in utero surgical repair as an effective treatment for fetuses with myelomeningocele. Current data suggest that outcomes related to the need for shunting should be counseled separately from the outcomes related to distal neurologic functioning.