[MIM 234 100]

(bird-like face dyscephaly, Francois dyscephaly, Hallermann-Streiff syndrome, dyscephalic syndrome, oculo-mandibulo-facial syndrome, oculo-mandibulo-facial syndrome of Hallermann-Streiff-François, Hallermann syndrome, oculo-mandibulo dyscephaly with hypotrichosis,  Ullrich and Fremerey-Dohnas syndrome, mandibulo-facial dysmorphism type Francois)

Sporadic or autosomal recessive transmission. Malformative syndrome secondary to abnormal embryological development of the first and sometimes the second branchial arch combining a cranial deformation (brachycephaly) with  prominent frontal bossing, a beaked nose, micrognathia (mandibular hypoplasia) and microstomia. Dwarfism. Obstructive apnea. Microphthalmia and congenital cataract. Cutaneous atrophy with hypotrichosis. Immunodeficiency with frequent respiratory infections. Normal intelligence.

Anesthetic implications: 

difficult intubation. 

References : 

-        Cheong KF, Tham CSL. 
Anaesthetic management of a child with Hallermann-Streiff-François syndrome.
Paediatr Anaesth 2003; 13: 551-2.

-        Krishna HM, Bhagat S, Vinodhadevi V. 
Difficult intubation in an infant with Hallerman-Streiff syndrome-easy with Airtraq laryngoscope. 
Pediatr Anesth 2012; 22: 497-8.

Updated: April 2019