Rare Diseases Symptoms Automatic Extraction

[Anesthetic considerations for a boy with non-bullous ichthyosiform erythroderma].

[lamellar ichthyosis]

We administered anesthesia for a 20-month-old boy with non-bullous ichthyosiform erythroderma who underwent orthopedic syndactyly repair on two occasions. Anesthetic considerations include the difficult fixation of the tracheal tube and iv cannulas and the risk of hypothermia. Intravenous access placement was also difficult due to the hyperkeratosis and the deformity of extremities. In the first surgery, pharyngeal temperature fell to 34.4 degrees C despite the use of the 37 degrees C circulating water mattress and room temperature set to 26 degrees C. It gradually returned to 36 degrees C during the surgery. We applied Steri-Strip compound benzoin tincture to the skin to increase adhesiveness before taping the tracheal tube. It resulted in a good fixation but difficult removal. In the second surgery, we fixed the tracheal tube with a tube holder which enabled the fixation without the use of adhesive tapes to the skin. To maintain body temperature we used forced air warming device Bair Huggar, which was very useful to avoid hypothermia but resulting in a rather high body temperature. In conclusion, a tube holder was an effective device to fix the tracheal tube in this patient. Forced air warming device was useful to prevent hypothermia.