Anesthesia for infant Withwalker-Warburg Syndrome Walker-Warburg Sendromlu bebeǧe anestezi̇ uygulamasi

UZUN Ş., Erden I. A., Akbay Özkaya B., AYPAR Ü.

Anestezi Dergisi, cilt.17, sa.4, ss.216-218, 2009 (Scopus) identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 17 Sayı: 4
  • Basım Tarihi: 2009
  • Dergi Adı: Anestezi Dergisi
  • Derginin Tarandığı İndeksler: Scopus, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.216-218
  • Anahtar Kelimeler: Congenital muscular dystrophy, Difficult intubation, Hydrocephaly, Malignant hyperthermia, Walker-Warburg Syndrome
  • Lokman Hekim Üniversitesi Adresli: Evet

Özet

Walker-Warburg Syndrome is lethal and inherited as an autosomal recessive trait. Type 2 lissencephaly (Resulting in a lack of development of gyri and sulci), polymicrogyri (The most consistent features are a smooth appearance of the surface of the brain due to lack of normal folds), malformations of other brain structures (cerebellum and brain stem), posterior encephalocel and hydrocephaly can exist. In hypotonic infant with congenital muscle disorder, muscle atrophy, deep tendon reflex loss, mental retardation, central neuronal migration abnormalities and eye abnormalities, Walker Warburg Syndrome, Muscle-Eye-Brain Disease or Fukuyama Congenital Muscular Dystrophy must be considered in differential diagnosis (1,2). It can have megalocornea, bufthalmia, cataract, retinal hypopigmentation, optic nerve hypoplasia (3). In our case, the muscle biopsy confirmed, the muscular dystrophy and genetic department reported as Walker Warburg Syndrome. Anaesthesia induction and maintenance was performed by considering points to take care of in muscular dystrophy and by taking precautions for difficult intubation and malign hyperthermia. In this case, a ventriculoperitoneal shunt surgery to an infant with Walker Warburg Syndrome scheduled for ventriculoperitoneal shunt will be discussed.