Saethre-Chotzen Syndrome

Incidence: 1:25,000–50,000 live births.

Difficult airway.

Seizures.

Oculocardiac reflex.

The severity of cardiac lesion may affect the hemodynamic stability of the pt during anesthesia.

A type of acro-cephalo-syndactyly syndrome, characterized by premature fusion of the coronal sutures, facial dysmorphism, syndactyly, skeletal deformity, and congenital heart malformations.

Named after two physicians who independently reported it in the early 1930s—Haakon Saethre, a Norwegian psychiatrist, and F. Chotzen, a German psychiatrist.

It may lead to brachycephaly and plagiocephaly, late closure of fontanels, and raised ICP, eventually provoking seizures.

Midfacial hypoplasia leads to small maxilla and relative mandibular prognathia, as well as high arched palate.

These pts can have a beaked nose; deviated nasal septum; narrow palate; cleft palate; super numerary teeth; small, low-set, unusually shaped ears; and enamel hypoplasia.

Facial appearance tends to improve with age throughout childhood.

It may involve multiple organs. The predominating involved systems are the cardiac system, the skeletal system, as well as the sensory and motor systems.

Less common signs and symptoms include congenital heart defects (ASD, VSD, pulm stenosis, PDA, TOF), renal anomalies, cryptorchidism, and anorectal malformations.

Ruling out increased ICP is important, either by using CT scan or fundus examination.

High arched palates make placement of the tube difficult, because of limited lateral space availability.

Facial features can lead to difficulty in bag and mask ventilation, intubation, and LMA insertion.

Vertebral fusion is progressive and hence may present at a more advanced stage, leading to cervical instability.