[MIM 103 300]

(hypoglossia-hypodactyly syndrome, Jussieu syndrome)

Not to be confused with: Hanhart or Richner-Hanhart syndrome by tyrosine aminotransferase deficiency: see Richner-Hanhart syndrome

Oromandibular syndrome with limb shortening. Association of limb anomalies with hypoglossia or aglossia and micrognathia. According to the importance of the limbs anomalies, there are 5 grades ranging from 0 (absence of pathology) to 5 (full or partial hemimelia).

Anesthetic implications: 

difficult intubation. Difficulties of peripheral venous access.

References : 

-        Karakaya D, Baris S, Belet N, Güneren E, Ustün E. 
Anaesthetic management in a child with hanhart's syndrome. 
Paediatr Anaesth 2003; 13: 263-6.

Updated: October 2018