Neural tube defects in the neonatal period

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Neural tube defects in the neonatal period

• Dr Mohamed Anas Barakat
• Consultant Neonatologist

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In the USA , New England has seen the
incidence of spina bifida drop from
2.31/1000births during 1930s to 0.77/1000 births
during the 1960s .
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Incidence
Authors Data published Defects Termination of pregnancy Incidence/Birth
Waleed Murshid (KSA) 1 2000 Spina bifida - 1.09/1000
Amira Masri (Jordan) 2 2006 NTD - 1.1/1000
( Syria ) 3 - - - -
A.K.Njamnshi (Cameroon) 4 2008 NTD - 1.99/1000
Louis duplessis (Australia )5 2008 NTD 79 1.3/1000 Including termination of pregnancy ( 0. 26)
CDC (USA)6 2007 Spina bifida - 0.17/1000 ( 2005)
Data collection Dr Anas Barakat
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Admission To NICU
Age At Admission Number of cases Percentage
First 48 hours 21 12.4
48hs- first week 14 8.2
After 135 79.4
Dr Hamza Ali, Jihad Kazah Damascus children
hospital 2008 Spina bifida ( 170 cases reported
2005-2007) 3 Geographic distribution of cases
Damascus city and region ( 52.3)
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Neural tube defects position
Spina bifida cystica Encephalocele Anencephaly
A.K.Njamnshi Cameroon 2008 (98cases) 4 71 21.1 5.4
Hamza Ali Children hospital Damascus 2008 ( 170 cases) 3 155 cases 15 cases -
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Consanguinity
consanguinity Percentage
Waleed Murshid (KSA) 1 89
Hamza Ali Children hospital Damascus 2008 ( 170 cases) 3 67 cases /170 39.4
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Recurrence
No of cases Percentage
recurrence risk USA Belfast London 2 - 4 1 - 3 10.4 4.12
Cowchock (USA) 1980 7 - 4.3
Hamza Ali Children hospital Damascus 2008 ( 170 cases) 3 8/170 4.7
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Folic acid prevention
Periconceptional folic Acid prevention During pregnancy Folic Acid prevention
A.K.Njamnshi Cameroon 2008 (98cases) 4 0 -
Hamza Ali Children hospital Damascus 2008 ( 170 cases) 3 2/170 (1.1) 78/170 ( 45.8)
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CDC trends in Spina -bifida in the united
states, 1991-2005
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CDC trends in Anencephalus in the United
States , 1991-2005
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Screening

• AFP (In the amniotic fluid or maternal
  bloodstream )
• Detection of a neural tube defect with fetal
  Ultrasonography

• risk factors include
• previous pregnancies have been affected with NTDs
  
• some anticonvulsants such as carbamazepine and
  sodium valproate
• pregnancies complicated by diabetes at conception

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 Prevalence of babies born alive with a neural
tube defect by birth year, 19902006
What has happened with neural tube defects and
womens understanding of folate in Victoria since
1998? Louise du Plessis, Rod W Hunt, Ashley S
Fletcher, Merilyn M Riley and Jane L Halliday MJA
2008 189 (10) 570-574
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Detection during pregnancy

• Diagnosis often made at 16-18 week
• An axial cross section through the fetal
  skull may demonstrate a lemon head shape
• Visualization of the posterior fossa may
  demonstrate a banana sign
• USS should evaluate for other structural
  abnormalities of limbs, brain, kidneys, and other
  organs.

Detection During Pregnancy Early detection Before 7 months
Hamza Ali Children hospital Damascus 2008 ( 170 cases) 3 37/170 (21.7) 14/170
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Clinical signs
Total Paralysis Of lower limb Percentage
Amira Masri (Jordan) 2 77/ 91 (84 )
Hamza Ali Children hospital Damascus 2008 ( 170 cases) 3 128/170 (75.3 )
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Complications
Leakage of CSF Proved Meningitis CNS Abscess
Hamza Ali Children hospital Damascus 2008 ( 170 cases) 3 95/170 57/170 2/170
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Management
Surgical treatment No
Hamza Ali Children hospital Damascus 2008 ( 170 cases) 3 125/170 Reparation Shunt 45/170 34 death (20) 11 discharged upon the request of their parents
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Follow-up

• Multidisciplinary approach
• Pediatric neurologist, Neurosurgeon, Urologist
  , Orthopedic and Physiotherapist .
• Hydrocephalus is a common occurrence after
  surgery but may not develop for some weeks.
  However most become evident in the first 10 days
  post closure.
• 1 -The baby should be watched with serial head
  circumference measures and ultrasound imaging.
• 2 -A shunt should be inserted if there is clear
  evidence of progressive ventriculomegaly or
  increasing head circumference.
• 3 -Shunt infections and revisions are common
  throughout childhood.
• Normal intellect should be assumed in the absence
  of significant hydrocephalus or dysmorphism.
• Children with MM have a lifetime increased risk
  of latex sensitivity and therefore need to be
  cared for in a latex-free environment.

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Recommendation

• Refer patients as early as possible to
  special centers which cover different areas in a
  given region .
• Periconceptional folate supplementation has been
  shown to reduce the incidence of NTDs, with data
  suggesting that a daily intake of 0.4mg of folic
  acid reduces the risk of 1st neural tube defects
  by up to 70 and Women at high risk require 5mg
  daily.
• Screening and Detection
• Alpha feto protein (AFP) from amniotic fluid is
  very reliable except when the defect is closed.
• Maternal serum AFP is less reliable, detecting
  between 50-90 of open anomalies, but is falsely
  positive in 5.
• Ultrasonography Diagnosis often made at 16-18
  week
• Very reliable when done by experienced
  ultrasonographer
• 4. Termination of pregnancy still a hard fact to
  deal with

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References

• 1- waleed.R.Murchid , Spina bifida in Saudi
  Arabia is consanguinity among the parents a risk
  factor . Pediatric neurosurgery2000 3210-12.
• 2-Amira Masri, Neural tube defects in Jordan, A
  hospital based study , Journal of pediatric
  neurology, 2006, 4 245-249 .
• 3- Hamza Ali , Jihad kazah Children hospital,
  Damascus 2008, A hospital based study ( 170
  cases) , data not published .
• 4- A . Njamnshi , Neural tube defects are rare
  among black Americans but not in Sub- saharan
  black Africans the case of Yaoundé - Cameroon .
  Journal of the neurological sciences ,
  Volume270,issue1 , 13-17 .
• 5- Louise Du Plessis , Rod W Hunt , Ashley. S .
  Fletcher , Marilyn .M . Riley .
• What has happened with neural tube defects
  and women's understanding of folate in Victoria
  since 1998 ? The medical Journal of Australia .
  MJA 2008 , 198,10570-574 .
• 6- CDC features , Centers for disease control
  and prevention Trends in Spina bifida , United
  States, 1991-2005 .

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References

• 7- Q . I . Alatise, A . A . Adeoler , Pediatric
  neurosurgery ,2006, 42 277-283 .
• 8- Richard . G . Ellenbogen BA. MD. Professor and
  chairman , Theodore Roberts Endowed chair in
  pediatric neurosurgery, department of
  neurological surgery , The university of
  Washington, updated Jan 30,2009.
• Neural tube defects in the neonatal period
  E-medicine .
• 9- Kat . Kolaski ,MD , Assistant professor ,
  departments of orthopedics and pediatrics, Wake
  forest, university school of medicine . Updated
  Apr20,2006
• Myelomeningocele . E-medicine .