Sage Journals: Discover world-class research

Abstract

Objective

Spondyloepiphyseal dysplasia congenita (SEDC) is a rare, inheritable condition that can include dwarfism, cleft palate, and C_1–2 instability. When repairing a cleft palate in a patient with SEDC, there is a significant risk of cord compression at the C_1–2 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.

Keywords

cervical cord compression cleft palate repair Ferno vacuum splint SEDC spondyloepiphyseal dysplasia congenita

Get full access to this article

View all access options for this article.

References

Fraser

G.R.

Friedmann

A.I.

Maroteaux

Glen-Bott

A.M.

Mittwoch

Dysplasia spondyloepiphysaria congenita and related generalized skeletal dysplasias among children with severe visual handicaps.

Arch Dis Child. 1969; 44: 490–498.

Horton

W.A.

Hall

J.G.

Scott

C.I.

Pyevitz

R.E.

Rimoin

D.L.

Growth curves for height for diastrophic dysplasias, spondyloepiphyseal dysplasia congenita, and pseudoachondroplasia.

Am J Dis Child. 1982; 136: 316–319.

Lee

Vissing

Ramirez

Rogers

Rimoin

Identification of the molecular defect in a family with spondyloepiphyseal dysplasia.

Science. 1989; 244: 978–980.

Mogera

Muralidhar

Spondyloepiphyseal dysplasia congenita syndrome: anesthetic implications.

Anesth Analg. 1996; 83: 433–442.

Nakamura

Miyoshi

Haga

Kurokawa

Risk factors of myelopathy at the atlantoaxial level in spondyloepiphyseal dysplasia congenita.

Arch Orthop Trauma Surg. 1998; 117: 468–470.

Park

Aiba

Haga

Kitano

Cleft palate in spondyloepiphyseal dysplasia congenita: case reports.

Scand J Plast Reconstr Hand Surg. 2001; 35: 419–423.

Redl

Massive pyramidal tract signs after endotracheal intubation: a case report of spondyloepiphyseal dysplasia congenita.

Anesthesiology. 1998; 89: 1262–1264.

Rodney

G.E.

Callander

C.C.

Harmer

Spondyloepiphyseal dysplasia congenita—caesarean section under epidural anaesthesia.

Anaesthesia. 1991; 46: 648–650.

Spranger

Wiedemann

H.R.

Dysplasia spondyloepiphysaria congenita.

Helv Pediatr Acta. 1966; 21: 598–611.

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