Cleft Palate Repair in Spondyloepiphyseal Dysplasia Congenita: Minimizing the Risk of Cervical Cord Compression

Abstract

Objective Spondyloepiphyseal dysplasia congenita (SEDC) is a rare, inheritable condition that can include dwarfism, cleft palate, and C1–2 instability. When repairing a cleft palate in a patient with SEDC, there is a significant risk of cord compression at the C1–2 level because of neck hyperextension required for the operation. This reports presents a patient with SEDC who underwent surgery for a cleft palate, using a Ferno vacuum splint to immobilize the spine. Intervention The patient underwent general anesthesia. Good access was gained to the palate, and it was repaired without any complications. Particular attention was paid to maintaining the neck in a neutral position. Conclusions The described technique provides the patient with the safest possible situation during anesthesia for cleft palate repair.