Monobloc distraction in an infant, using the rigid external distractor: problems and solutions--a case report.

INTRODUCTION

Patients with craniofacial dysostosis frequently develop functional problems including raised intracranial pressure, ocular dysfunction, obstructive sleep apnoeas and failure to thrive. These functional problems can be treated by a number of different techniques. The monobloc frontofacial advancement has the ability to correct all of these functional problems in one procedure, but can be associated with high morbidity particularly in the young infant.

AIM: A case report of a 4-month-old infant with Pfeiffers syndrome and severe functional problems treated by monobloc osteotomy and distraction using the rigid external distractor is reported. The management, problems and complications encountered with this patient are discussed together with the role of monobloc distraction in the infant. Adaptation of the rigid external distraction (RED) technique using two titanium mesh sheets to prevent penetration of the skull by the cranial pins is described.

CONCLUSIONS

Monobloc frontofacial advancement in the very young can be successfully achieved using the RED frame, thus treating exophthalmus, raised intracranial pressure and upper airway problems in one operation. However, it may be associated with significant complications and should only be used for those extreme cases where the severity of the functional problems prevent treatment being delayed.