Korean J Anesthesiol.  1981 Mar;14(1):101-105.

Anesthetic Experience in Three Patiente with Hurlers Syndrome

• Kim JC
• Jeon JK

Abstract

It is well known that many problems occur in patients with Hurler's syndrome both during anesthesia and after. Hurler's syndrome is a mucopolysaccharoidoses characterized by and abnormal metabolism of the mucopolysaccharide. The clinical manifestations include dwarfism, frontal bossing, hypertelorism, thick lips, large tongue, short neck and hepatosplenomegaly. Common complications from anesthesia are excessive secretions, difficult intubation, respiratory and heart failure. Most of the patients die before the age of 10 from pulmonary infection or cardiac failure. In this article, 3 patients anesthetized for surgical intervention are described. Case l: A 10-year old male with Hurler's syndrome was scheduled for umbilical herniorrhaphy. His clinical manifestations included all the typical appearance of the syndrome and large tonsils, excessive secretion, breathing difficulty and mental retardation, etc. Anesthesia was induced with 200mg of pentothal followed by 40 mg of anectin for intubation. The first attempt at endotracheal then a blind awake intubation was performed successfully. Anesthesia was maintained with N20-Halothane. Tracheostomy was performed post-operatively because the breathing became worse. He died of respiratory failure on the 114th post-operative day. Case ll: The patient was 7-year old brother of the above patients, scheduled ofr inguinal herniorrhaphy. Caudal anesthesia was performed with 30cc of 1 percent lidocaine is whole hospitalization was uneventful. Case lll : The above patient was readmitted for umbilical herniorrhaphy on the 9th day after his first operation. He was atropinized preoperatively and anesthesia was induced with 180mg of pentothal followed by Anectin injection for endotracheal intubation .The first attempt failed, then a blind oral intubation was carried out successfully and anesthesia was maintained with N20-Halothane. He recovered from anesthesia with excessive secretion and mild respiratory problems. The problems, complications and precautions for anesthesia are described in this article.

MeSH Terms

Anesthesia
Anesthesia, Caudal
Dwarfism
Heart Failure
Herniorrhaphy
Hospitalization
Humans
Hypertelorism
Intellectual Disability
Intubation
Intubation, Intratracheal
Lidocaine
Lip
Male
Metabolism
Mucopolysaccharidosis I
Neck
Palatine Tonsil
Respiration
Respiratory Insufficiency
Siblings
Thiopental
Tongue
Tracheostomy
Lidocaine
Thiopental