Neonatology Book

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Myelomeningocele

Aka: Myelomeningocele, Severe Spina Bifida
  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 (80% of cases)
    2. Arnold Chiari II Malformation
    3. Neurogenic Bladder
    4. Urinary Incontinence
    5. 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. Complications
    1. See Ventriculoperitoneal Shunt Malfunction
    2. See Arnold-Chiari II Malformation
    3. Urinary Tract Infections
      1. Related to neurogenic Bladder
      2. Urine may be difficult to interpret (due to chronically dirty urine, especially if prior intestinal graft)
      3. Symptoms may be difficult to assess (due to cognition, differential diagnosis including shunt malfunction)
      4. Treat symptomatic Urinary Tract Infections
    4. Skin Infections
      1. Sensory deficits below Myelomeningocele allow for skin breakdown and infection
      2. Undress and fully examine all Spina bifida patients for fever source
  9. Prevention
    1. Folic Acid supplementation started before conception
  10. References
    1. Majoewsky (2012) EM:Rap-C3 2(9): 5
    2. Behrman (2000) Nelson Pediatrics, Saunders, p. 1804
    3. Goetz (1999) Neurology, Saunders, p. 515-16

Meningomyelocele (C0025312)

Definition (MSHCZE) Vrozená vada s výhřezem páteřní míchy a mozkových plen při spina bifida (rachischisis), při které dochází k herniaci míšní tkáně spolu s nahromaděním tekutiny v arachnoidei přes zadní stranu defektního vertebrálního oblouku, vyklenující se na povrch zad. Celý útvar je kryt kůží, má tendenci k nekróze. Incidence této vady je přibližně 1:1000 narozených dětí. Klinický obraz je závislý na lokalizaci m., 75% případů je v lumbosakrální oblasti. Při m. je často hydrocefalus. Léčba vyžaduje spolupráci multidisciplinárního týmu. Úmrtnost na m. u dětí do 4 let je přibližně 10–15%. Inteligence dětí je normální, epilepsie se vyskytuje se stejnou prevalencí jako v ostatní populaci. M. s trvalými neurologickými obtížemi představuje pro děti chronický handicap, vyžadující trvalou péči odborníků. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) A congenital abnormality in which the spinal cord and meninges protrude through a defect in the spinal column. The protrusion is above the skin surface.
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 7096005, 203988006, 203984008, 203999008, 203995002, 203994003, 414667000
English 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 [Disease/Finding], myelomeningoceles, meningomyeloceles, myelomeningocele, Meningomyelocele, Meningomyelocele of unspecified site (disorder), Myelocystocele NOS (disorder), Myelocystocele of unspecified site (disorder), Meningomyelocele NOS (disorder), Myelocystocele (diagnosis), congenital spinal anomaly spina bifida myelocystocele, Myelocystocele, Myelocystocele (disorder), myelocystocele, Meningomyelocele (disorder)
French MENINGOMYELOCELE, Méningomyélocèle, Myéloméningocèle
Portuguese MENINGOMIELOCELE, Meningomielocelo, Meningomielocele, Mielomeningocele
German MENINGOMYELOZELE, Meningomyelozele, Myelomeningozele
Spanish MIELOMENINGOCELE, mielocistocele, SAI (trastorno), mielomeningocele, SAI, mielocistocele, SAI, mielomeningocele, de sitio no especificado, mielomeningocele, SAI (trastorno), mielocistocele, de sitio no especificado (trastorno), mielocistocele, de sitio no especificado, mielomeningocele, de sitio no especificado (trastorno), mielocistocele (trastorno), mielocistocele, meningomielocele, mielomeningocele (trastorno), mielomeningocele, Meningomielocele, Mielomeningocele
Swedish Ryggmärgsbråck
Dutch myelomeningokèle, meningomyelokèle, Myelocele, meningo-, Meningomyelokèle, Myelomeningokèle
Italian Mielomeningocele, Meningomielocele
Japanese セキズイズイマクリュウ, ズイマクセキズイリュウ, 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
Finnish Meningomyeloseele
Russian MIELOTSELE, MIELOMENINGOTSELE, МИЕЛОМЕНИНГОЦЕЛЕ, МИЕЛОЦЕЛЕ
Czech Meningomyelokéla, Myelomeningokéla, meningomyelokéla, meningomyelokela, meningomyelocele
Croatian MENINGOMIJELOKELA, MIJELOMENINGOKELA
Polish Przepuklina oponowo-rdzeniowa
Hungarian ((gerincvelő- és gerincvelőburok sérv)), Meningomyelokele
Norwegian Myelomeningocele, Brokk fra ryggmargskanalen, Meningomyelocele, Ryggmargsbrokk
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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