Anaesthetic management of a case of Haberland's syndrome (encephalocraniocutaneous lipomatosis)

Abstract

While the Haberland syndrome was first reported in 1970, the anaesthetic management of this rare cohort of patients has not been described. With only 54 such cases reported, describing primarily the unilateral cutaneous, ocular, and neurologic malformations associated with this syndrome without focussing on the anaesthetic management. We describe the case of a 7 year old case of Harberland syndrome with special focus on the difficulties faced by us in mask ventilation, as well as intubation and the need for elective ventilation and planned extubation. We also discuss the precautions to be taken while undertaking such a case like preparations for emergency tracheostomy and possibility of re-intubation after extubation. A meticulous preoperative workup along with neurological and airway examination along with preparation for elective ventilation and tracheostomy is a prerequisite for the successful management of this case.

Keywords

• Difficult airway
• Haberlands syndrome
• Mallampatti grade
• nasal intubation
• odontogenic cyst