Anaesthetic implications of the child with Robinow syndrome.
Robinow syndrome, originally described in 1969, consists of mesomelic brachymelia, short stature, genital hypoplasia and characteristic facies. Associated organ system involvement may include developmental delay, congenital heart disease, obstructive disorders of the urinary tract and renal cysts. As there is a potential for airway problems related to the facial features, such as midface hypoplasia and micrognathia, perioperative management of these patients is important. The authors present an 8-year-old boy, previously diagnosed with Robinow syndrome, who was admitted for an emergency surgical procedure. The perioperative implications of this disorder are reviewed.
Sleesman
Jay B
JB
Departments of Anesthesiology and Child Health, University of Missouri, Columbia, MO 65212, USA.
Tobias
Joseph D
JD
D000015:Abnormalities, Multiple / Q000235:genetics*; D000758:Anesthesia / Q000379:methods; D018685:Anesthetics, Inhalation / Q000627:therapeutic use; D018686:Anesthetics, Intravenous / Q000627:therapeutic use; D001062:Appendectomy; D001064:Appendicitis / Q000601:surgery*; D002648:Child; D005145:Face / Q000002:abnormalities*; D005283:Fentanyl / Q000627:therapeutic use; D005837:Genitalia, Male / Q000002:abnormalities; D006130:Growth Disorders / Q000235:genetics*; D006801:Humans; D007530:Isoflurane / Q000627:therapeutic use; D010535:Laparoscopy; D017880:Limb Deformities, Congenital / Q000235:genetics; D008297:Male; D013577:Syndrome; D013874:Thiopental / Q000627:therapeutic use
D018685:Anesthetics, Inhalation; D018686:Anesthetics, Intravenous; D007530:Isoflurane; D013874:Thiopental; D005283:Fentanyl