Neonatology Book

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Myelomeningocele

Aka: Myelomeningocele
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  1. Epidemiology
    1. Occurs in 1 per 1000 live births
  2. Risk Factors
    1. Maternal Folate Deficiency
    2. Prior history of child with Myelomeningocele (4% risk)
    3. Valproic Acid (1-2% risk if taken during pregnancy)
  3. Pathophysiology
    1. Severe form of Spinal Dysraphism
    2. Affects lumbosacral region in 75% of cases
    3. Associated with dysfunction of multiple organ systems
  4. Signs
    1. Flaccid paralysis of lower extremities
    2. Deep Tendon Reflexes absent
  5. Associated Conditions
    1. Hydrocephalus and Chiari II Malformation (80% of cases)
    2. Neurogenic Bladder
    3. Urinary Incontinence
    4. Stool Incontinence
  6. Management
    1. Supportive care may be appropriate in severe cases
    2. Surgery in first few days of life
      1. Surgical repair of Myelomeningocele
      2. CSF Shunt for Hydrocephalus
    3. Frequent Bladder Catheterization for neurogenic Bladder
      1. Reduces risk of Pyelonephritis and Hydronephrosis
  7. Prognosis
    1. Mortality with aggressive treatment: 10-15% by age 4
    2. Normal intelligence in 70% of survivors
  8. Prevention
    1. Folic Acid supplementation started before conception
  9. References
    1. Behrman (2000) Nelson Pediatrics, Saunders, p. 1804
    2. Goetz (1999) Neurology, Saunders, p. 515-16

Meningomyelocele (C0025312)

Definition (MSH) Congenital, or rarely acquired, herniation of meningeal and spinal cord tissue through a bony defect in the vertebral column. The majority of these defects occur in the lumbosacral region. Clinical features include PARAPLEGIA, loss of sensation in the lower body, and incontinence. This condition may be associated with the ARNOLD-CHIARI MALFORMATION and HYDROCEPHALUS. (From Joynt, Clinical Neurology, 1992, Ch55, pp35-6)
Definition (CSP) hernial protrusion of the spinal cord and its meninges through a defect in the vertebral canal.
Concepts Congenital Abnormality (T019)
MSH D008591
ICD10 Q05
SnomedCT 203995002, 203984008, 203999008, 203988006, 203994003, 414667000, 7096005
English Meningomyelocele, Meningomyeloceles, Myelomeningocele, Myelomeningoceles, MENINGOMYELOCELE, Myelocystocele NOS, Meningomyelocele NOS, Meningomyelocele of unspecified site, Meningomyelocele-unspecif.site, Myelocystocele of unspecified site, Myelocystocele-unspecif.site, meningomyelocele (diagnosis), meningomyelocele, Meningomyelocele of unspecified site (disorder), Myelocystocele of unspecified site (disorder), Meningomyelocele NOS (disorder), Myelocystocele NOS (disorder), Meningomyelocele [Disease/Finding], myelomeningoceles, meningomyeloceles, Myelocystocele, Myelocystocele (disorder), myelomeningocele, myelocystocele, Meningomyelocele (disorder)
French MENINGOMYELOCELE, Méningomyélocèle, Myéloméningocèle
Portuguese MENINGOMIELOCELE, Meningomielocelo, Meningomielocele, Mielomeningocele
German MENINGOMYELOZELE, Meningomyelozele, Myelomeningozele
Spanish MIELOMENINGOCELE, meningomielocele, mielocistocele (trastorno), mielocistocele, SAI (trastorno), mielocistocele, SAI, mielocistocele, de sitio no especificado (trastorno), mielocistocele, de sitio no especificado, mielocistocele, mielomeningocele, SAI (trastorno), mielomeningocele, SAI, mielomeningocele, de sitio no especificado (trastorno), mielomeningocele, de sitio no especificado, mielomeningocele (trastorno), mielomeningocele, Meningomielocele, Mielomeningocele
Swedish Ryggmärgsbråck
Dutch myelomeningokèle, meningomyelokèle, Myelocele, meningo-, Meningomyelokèle, Myelomeningokèle
Italian Mielomeningocele, Meningomielocele
Japanese ｾｷｽﾞｲｽﾞｲﾏｸﾘｭｳ, ｽﾞｲﾏｸｾｷｽﾞｲﾘｭｳ, 脊髄髄膜瘤, 脊髄瘤, 脊髄髄膜瘤ヘルニア, 髄膜脊髄瘤, 髄膜脊髄ヘルニア
Czech meningomyelokela, Meningomyelokéla, Myelomeningokéla
Finnish Meningomyeloseele
Russian MIELOTSELE, MIELOMENINGOTSELE, МИЕЛОМЕНИНГОЦЕЛЕ, МИЕЛОЦЕЛЕ
Croatian MENINGOMIJELOKELA, MIJELOMENINGOKELA
Polish Przepuklina oponowo-rdzeniowa
Hungarian ((gerincvelő- és gerincvelőburok sérv)), Meningomyelokele
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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