Transforming treatment of spina bifida with pioneering open fetal surgery
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FEATURE ARTICLE
Transforming treatment
of spina bifida with
pioneering open fetal
surgery
According to Neli Garbuzanova and colleagues,
collaborative working underpins the delivery of highly
specialised open surgery for spina bifida
O pen spina bifida is a lifelong
condition that occurs in
about six in every 10,000
births.1 With clinical evidence
pointing to improved outcomes
About the authors
Neli Garbuzanova (pictured)
Senior Procurement Manager, NHS Arden & GEM Commissioning Support Unit
* neli.garbuzanova@nhs.net
for babies who received pioneering @NeliGarbuzanova
fetal surgery, NHS England Highly @ardengem
Specialised Services sought to
establish a service that would enable Bernie Stocks
eligible women in the UK to benefit Commissioning Manager, Highly Specialised Services, NHS England and NHS
from this treatment. Improvement
Dr Ayesha Ali
Following a collaborative
Medical Advisor, Highly Specialised Services, NHS England and NHS Improvement
provider‑selection project delivered
in partnership with NHS Arden & Fiona Marley
GEM Commissioning Support Unit Head of Highly Specialised Commissioning, NHS England and NHS Improvement
(CSU), a new service is now in place,
Kate Steele
with two specialist centres providing
this complex procedure.2,3 As well as Chief Executive, Shine
serving the population of England,
the service is accessible to women
from Northern Ireland, Scotland, There are different forms of the
and Wales following agreement with About the condition condition, but the most serious
their health commissioners. Between are forms of open spina bifida—
November 2019 and December Public Health England describes spina myelomeningocele and myeloschisis—
2020, 20 women from across the UK bifida as ‘a neural tube defect … where in which the spinal cord and nerve
underwent open fetal surgery for part of the baby’s neural tube does not tissue bulge through a hole or area
congenital open spina bifida (figures develop or close properly. This leads to of thin membrane, referred to as
derived from NHS England internal the baby’s spinal cord (the big bundle the ‘defect’ or ‘lesion’, on the baby’s
activity monitoring), which has a of nerves running from the brain down back.1 Open spina bifida is typically
transformative impact on the lives the baby’s back) or vertebrae (the accompanied by changes in the brain,
of babies and their families bones of the spine) not developing as which include hydrocephalus—excessive
(see Box 1). 4 they should.’1 accumulation of fluid in the brain—and
Specialised Medicine | February 2021 | Volume 5 | Issue 1 Promoting excellence in specialised healthcare 11
FEATURE ARTICLE
Box 1: Case study4
fetal surgery, the likelihood of evolving
hydrocephalus is significantly reduced,
Carrie, parent of Max and surgery recipient the leg weakness experienced is less
‘I had fetal surgery … at UCLH to repair the defect on my unborn [baby’s] spine. Max had severe than would have been predicted,
… myelomeningocele [Figure 1] and we were told he had around a 20% chance of walking and the abnormality at the back of the
with aids, but would most likely have no movement or feeling from the waist down and be brain (Chiari II malformation) is often
in a wheelchair full time. He would need a shunt and have bowel and bladder issues. My reversed or significantly improved.’
local Fetal Medicine Unit … were extremely knowledgeable and offered a pioneering surgery
at UCLH as one of the options for me and my baby.
‘Max has absolutely thrived since surgery. His chiari II malformation reversed, the
Developing a new service
hydrocephalus levelled out and the fluid remained stable throughout my pregnancy. Max
was born at 36 weeks [Figure 2] … He came into the world kicking and screaming and
Previously, open fetal surgery was not
hasn’t stopped moving since. He has full feeling and movement all the way to his toes, the commissioned in the UK, although up
fluid on his brain has remained stable … and he has full control of his bladder and bowels to 10 women from England each year
… He is now a 10-month-old cheeky chappie, starting to take his first aided steps, and were travelling to mainland Europe,
crawling around the house faster than I can catch him [Figure 3].’ mostly Belgium, for this treatment via
reciprocal healthcare arrangements with
UCLH=University College London Hospitals NHS Foundation Trust
other European Union (EU) member
states. Following a review by the NHS
a herniation of the hindbrain through A randomised controlled trial—the England Rare Diseases Advisory Group
the foramen magnum known as Chiari II Management of Myelomeningocele in December 2016, NHS England began
malformation.5,6 Study (MOMS)—was stopped early work to commission a service in line with
because of superior outcomes in the the UK Strategy for Rare Diseases, which
The resulting disabilities vary according fetal surgery group, which included would develop this speciality within
to the position of the lesion and extent less need for a shunt to drain excess England.11,12 With EU exit on the horizon,
of damage to the spinal cord, but can fluid from the brain, improvement in it was critical to establish an NHS‑funded
include curvature of the spine, weakness movement and motor function/walking service with clear contractual
or paralysis of the lower limbs, as well at 30 months of age, and improvements arrangements in place with potential
as bladder and bowel dysfunction.6–8 in several secondary outcomes. However, providers in the UK or overseas.
Complications caused by hydrocephalus the MOMS Trial identified additional risks
and its treatment can also lead to for the mother and fetus and for future The project required a multidisciplinary
learning and behavioural issues.6,7 pregnancies, which have informed the approach from the outset. This began
suitability criteria for the surgery.10 with the development of a detailed
The exact cause of spina bifida is service specification drawing on
unknown, but several factors are specialist input from a wide range of
associated with the condition, including stakeholders, including service users,
insufficient folate in the diet and … open fetal surgery clinicians, and providers, and through
hereditary factors.6 public consultation. This work was also
for congenital open supported by health commissioners
spina bifida … has and clinical teams from Northern
Treatment options and Ireland. Shine is one of the specialist
challenges a transformative charities for spina bifida, and its team
Traditionally, postnatal surgery has
impact on the lives were instrumental in developing the
specification and updating their existing
been carried out very soon after of babies and their patient communication materials
birth to close the lesion and reduce for the new service. Since the start
the likelihood of further damage or families … of the service, commissioners have
infection. However, prenatal treatment been working in partnership with both
of spina bifida, where surgery is carried Shine and Spina Bifida Hydrocephalus
out to close the lesion before birth, The complexity of this surgery aside, Scotland, which together cover the
has also become available around the the potential improvements in quality whole UK population.
world in recent years.9 This is extremely of life both for the baby and its
complex surgery, which involves parents are significant. Mr Dominic The involvement of these charities has
opening the uterus, closing the spina Thompson, Consultant Paediatric showcased the importance of the patient
bifida lesion, and then repairing the Neurosurgeon, Great Ormond Street voice and the difference that it can
uterus (see Box 2).6 Hospital (GOSH), explains: ‘Following make when developing and delivering
12 Specialised Medicine | February 2021 | Volume 5 | Issue 1 Promoting excellence in specialised healthcare
FEATURE ARTICLE
a high-quality, person‑centred service.
Figure 1: Second-trimester ultrasound scan of Max showing
Patients have provided observations and
myelomeningocele
feedback and been able to ask questions
to continually inform and shape this
service, which will potentially give babies
and their parents a much better quality
of life and hope for the future.
Designing procurement strategies
to overcome challenges
NHS Arden & GEM CSU worked in
partnership with commissioners
from NHS England Highly Specialised
Services to jointly design the
procurement and provider-selection
strategy for the new service. The
hurdles to overcome in securing
viable options for commissioning and
delivering the new service included:
• the specialist nature of the treatment Reproduced with permission.
• the small number of patients that
would be eligible for treatment
(approximately 10 per year) Figure 2: The site of myelomeningocele repair on Max’s back after birth
• the small gestational age window for
surgery
• the need to maintain expertise.
A collaborative approach was
essential, and included setting
up a multidisciplinary project
group comprising clinical advisors,
commissioners, procurement and
communications leads, and experts
in finance, contracting, quality,
information governance, paediatric
neurosurgery, and fetal medicine. An
international expert in spina bifida
surgery (from outside Europe, to
Reproduced with permission.
avoid a conflict of interest) provided
invaluable input, enabling the
specification to incorporate global best the best possible opportunity to make those meeting the service specification
practice. Two patient representatives an informed submission. in full; and those involving collaboration
actively participated in all stages of the with other centres and specialists to
process, keeping patients at the heart of Following analysis of clinical, financial, acquire the level of expertise needed.
service provision. and market considerations, a lotting Collaboration between both FSCs and
model was put forward suggesting two referring regional fetal medicine units
Dedicated market-engagement fetal surgery centres (FSCs). Finding the (RFMUs) was essential for developing
sessions for interested providers were right balance between providing care and maintaining expertise; a requirement
set up to share provider selection close to patients and maintaining service built into the provider‑selection
intentions and open dialogue. Case quality was a challenge. Few UK centres questionnaires and evaluation criteria.
studies, tips on developing successful had the necessary experience in this This process prioritised fully compliant
proposals, and a dedicated session to complex surgery, which involves a high and experienced centres, and led to the
discuss common bid mistakes were level of risk for both mother and fetus, selection of two FSCs: one in the UK,
facilitated to give potential providers so two types of solution were invited: and one in mainland Europe.
Specialised Medicine | February 2021 | Volume 5 | Issue 1 Promoting excellence in specialised healthcare 13
FEATURE ARTICLE
The two FSCs (UCLH and UHL),
Figure 3: Max at 10 months of age
in conjunction with paediatric
neurosurgery and general surgery
teams at GOSH, operate a joint
surgical decision-making team and
provide cross cover, which effectively
means one service across two sites.
Patient assessments and surgeries are
distributed between the two sites via
geographic mapping based on traditional
fetal medicine referral routes from the
RFMUs.2,13,14 Enquiries and referrals are
welcomed by the service from all RFMUs
throughout the UK, which provide
specialist maternity care and refer to the
service when there is a concern for the
health of the unborn child.
Comprehensive briefing documents
describing the service and eligibility
criteria have been provided to all
RFMUs. Referring clinicians and their
teams have also been invited to attend
surgeries to increase their knowledge
of the condition and build first-hand
local experience and expertise. Reports
to disseminate information to RFMUs
and patient groups have been issued
following two periodic clinical review
meetings with the FSCs and NHS
England commissioners.
As part of the service—and in order to
deliver on the Government’s UK Strategy
for Rare Diseases aim of co‑ordination
of care12 and for all patients to have an
Reproduced with permission.
‘alert card’15—all those involved in the
patient’s maternity care, including the GP
and local midwifery unit, are fully briefed
Mobilisation The new service about the surgery and requirements for
care of the mother between surgery and
Joint service initiation meetings For the next 4 years (2020–2024), delivery of the baby in case the mother
were held with the wider clinical eligible women carrying a fetus with goes into spontaneous labour ahead of
staff and management teams on open spina bifida will be offered the planned caesarean section.
consecutive days in preparation for high‑quality surgery from two specialist
the service starting. Weekly calls have FSCs: University College London Shine and Spina Bifida Hydrocephalus
been held by commissioners with Hospitals NHS Foundation Trust (UCLH), Scotland offer additional emotional
clinical leads since November 2018 to with specialist input from GOSH, in support and advice to the mother
troubleshoot any teething issues and London; and University Hospitals Leuven before, during, and after surgery to
consider any organisational, referral, (UHL), in Leuven, Belgium.3 Leuven is a complete the service’s holistic approach.
or communications issues. Formal very experienced specialist centre for
clinical review meetings have been this and other types of fetal surgery
held every 6 months, and the service and, for many women, offers a shorter Outcomes
has developed learning updates for travel time than to the UK FSC. Some 10
referring clinicians to educate teams surgeries are expected to take place each According to Dr Michael Belfort,
and improve understanding. year in Leuven for women from England. Specialist in Maternal–Fetal Medicine
14 Specialised Medicine | February 2021 | Volume 5 | Issue 1 Promoting excellence in specialised healthcareFEATURE ARTICLE
and Fetal Intervention at Texas
Box 2: Delivering highly specialised treatment
Children’s Hospital in the US and a
key stakeholder in the procurement Professor Anna David, Clinical Lead, UCLH and UCL
process, ‘The impact of this fetal
‘Our work begins from the moment a fetus is diagnosed with spina bifida. We work with
surgery on the life of the patient cannot families to explain what this means and the options available to them. Thanks to this
be overstated. The reduction in the rate project, those options now include fetal surgery, provided there are no other complications
of hydrocephalus, and the increase in with the fetus, the mother is well, and type of spina bifida defect in the fetus meets the
the rate of independent ambulation are eligibility criteria. The surgery does carry risks for both the mother and fetus, which are
simply life changing … in some cases, the carefully explained before consent is sought to proceed.’
difference between a life in a wheelchair
and a life of independent movement.’4 Professor Jan Deprest, Fetal Surgeon, UHL and UCLH
‘When surgery is carried out, it is a team effort—we are effectively performing two different
Between November 2019 and December surgeries on two patients at the same time. During the procedure, there are numerous
2020, 20 surgeries were delivered via specialists present, including two anaesthetists (one for the mother, one for the fetus) with
the new service to women across the supporting staff, two obstetricians, two neuro-paediatric surgeons, two people scanning and
UK, all of whom went on to deliver their monitoring the fetus, a paediatric neonatologist, and the scrub team.’
babies safely via planned caesarean UCLH=University College London Hospitals NHS Foundation Trust; UCL=University College
section (figures derived from NHS London; UHL=University Hospitals Leuven
England internal activity monitoring).
After surgery, women continue to publications/spina-bifida-information-for- 11. NHS England. Urgent clinical commissioning
parents/spina-bifida-information-for-parents policy statement: prenatal surgery for open
receive care from the RFMU and their (accessed 18 January 2021) spina bifida. London: NHS England, 2018.
local maternity service until birth, which 2. NHS England. Open fetal surgery to treat Available at: www.england.nhs.uk/publication/
is via planned caesarean section at fetuses with open spina bifida. London: NHS urgent-clinical-commissioning-policy-statement-
England, 2018. Available at: www.england.nhs.uk/ prenatal-surgery-for-open-spina-bifida/
around 37 weeks of gestation as per the
commissioning/publication/open-fetal-surgery- 12. DH. UK Strategy for Rare Diseases. London: DH,
MOMS Trial.2,7,10 After delivery, babies are to-treat-fetuses-with-open-spina-bifida/ 2013. Available at: assets.publishing.service.
followed up at their regional paediatric 3. Shine. Prenatal surgery. www.shinecharity.org. gov.uk/government/uploads/system/uploads/
neurosurgical spina bifida unit, which uk/spina-bifida/prenatal-surgery (accessed attachment_data/file/260562/UK_Strategy_for_
18 January 2021). Rare_Diseases.pdf
comprises experts on their coordinated
4. Health Care Supply Association. Transforming 13. NHS England. E09. Specialised women’s services.
care, including bladder assessment, www.england.nhs.uk/commissioning/spec-
the lives of babies with spina bifida.
physiotherapy, and neurodevelopment. nhsprocurement.org.uk/transforming-the-lives- services/npc-crg/group-e/e09/ (accessed 18
of-babies/ (accessed 18 January 2021). January 2021).
The regional unit liaises closely with the 5. Shine. Chiari II malformation (also known as 14. NHS England. Women and children. www.
Arnold-Chiari Malformation, ACM, and hindbrain england.nhs.uk/commissioning/spec-services/
FSC to ensure that long-term outcomes npc-crg/group-e/ (accessed 18 January 2021).
herniation). www.shinecharity.org.uk/related-
are recorded—in this way, service quality conditons/chiari-ii (accessed 18 January 2021). 15. NHS England Specialised Services.
can be continually reviewed. Weekly 6. University College London Hospitals NHS Implementation plan for the UK Strategy for
meetings are also held between the FSCs Foundation Trust. Fetal spina bifida and surgical Rare Diseases. London: NHS England Specialised
closure during pregnancy—information for Services, 2018. Available at: www.england.nhs.
and NHS England to review outcomes uk/wp-content/uploads/2018/01/implementation-
pregnant women & families. London: University
and ensure that the necessary help is in College London Hospitals NHS Foundation Trust, plan-uk-strategy-for-rare-diseases.pdf
place to support this new service. 2018. Available at: www.ucl.ac.uk/womens-health/
sites/womens-health/files/fetal_spina_bifida_
and_surgical_closure_during_pregnancy.pdf
This important project has made
7. University College London Hospitals The authors wish to acknowledge the
available an evidence-based, NHS Foundation Trust. Fetal surgery following contributors:
ground‑breaking intervention for for spina bifida. www.youtube.com/
women, improving the outcomes for watch?v=0r76PARXN10&feature=youtu.be Professor Anna David
(accessed 18 January 2021). Clinical Lead, University College London
their babies. The success of the project
8. Brock J, Thomas J, Baskin L et al; for the Eunice Hospitals NHS Foundation Trust and
is down to the collaborative efforts Kennedy Shriver NICHD MOMS Trial Group. University College London
of everyone involved in the design, Effect of prenatal repair of myelomeningocele
commissioning, mobilisation, and on urological outcomes at school age. J Urol Professor Jan Deprest
2019; 202 (4): 812–818.
delivery of the new service. SM Fetal Surgeon, University Hospitals
9. Sacco A, Simpson L, Deprest J, David A. A study Leuven and University College London
to assess global availability of fetal surgery for
myelomeningocele. Prenat Diag 2018; 38 (13):
Hospitals NHS Foundation Trust
References 1020–1027.
Mr Dominic Thompson
10. Adzick N, Thom E, Spong C et al. A randomized Consultant Paediatric Neurosurgeon,
trial of prenatal versus postnatal repair of
1. Public Health England. Spina bifida—information myelomeningocele. N Engl J Med 2011; 364:
Great Ormond Street Hospital
for parents. www.gov.uk/government/ 993–1004.
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