BACKGROUND: Frontofacial monobloc advancement by distraction osteogenesis is now accepted as an effective treatment for children with syndromic and other complex forms of craniosynostosis because it combines cosmetic advantages with the ability to treat such complications as raised intracranial pressure, advancing exorbitism, and upper airway obstruction in one procedure. In the severely affected child, these complications may be present at an early age, but because of the scale of monobloc surgery, surgeons have been reluctant to perform it on very young children. METHODS: The authors report a series of 12 children younger than 30 months with various craniosynostosis-associated syndromes complicated by multiple functional problems--including raised intracranial pressure, exposure of the eyes, airway obstruction, and feeding problems--who underwent frontofacial monobloc surgery by distraction osteogenesis. RESULTS: The mean age at operation was 18 months. Mean advancement achieved was 16.6 mm for the upper face and 17 mm for the midface. Ocular protection and reduction of intracranial pressure (when raised) were achieved in all children. Airway improvement was achieved in all but one child. Although not the primary indication for surgery, there was also a marked improvement in every patient's appearance. Complications included two cerebrospinal fluid leaks, pin-site infections, frame slippage requiring replacement, and enophthalmos in one patient. CONCLUSION: The frontofacial monobloc advance incorporating distraction osteogenesis can be undertaken with acceptable morbidity even in the very young child and good functional outcomes achieved with one operation where previously more were required. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.
BACKGROUND: Frontofacial monobloc advancement by distraction osteogenesis is now accepted as an effective treatment for children with syndromic and other complex forms of craniosynostosis because it combines cosmetic advantages with the ability to treat such complications as raised intracranial pressure, advancing exorbitism, and upper airway obstruction in one procedure. In the severely affected child, these complications may be present at an early age, but because of the scale of monobloc surgery, surgeons have been reluctant to perform it on very young children. METHODS: The authors report a series of 12 children younger than 30 months with various craniosynostosis-associated syndromes complicated by multiple functional problems--including raised intracranial pressure, exposure of the eyes, airway obstruction, and feeding problems--who underwent frontofacial monobloc surgery by distraction osteogenesis. RESULTS: The mean age at operation was 18 months. Mean advancement achieved was 16.6 mm for the upper face and 17 mm for the midface. Ocular protection and reduction of intracranial pressure (when raised) were achieved in all children. Airway improvement was achieved in all but one child. Although not the primary indication for surgery, there was also a marked improvement in every patient's appearance. Complications included two cerebrospinal fluid leaks, pin-site infections, frame slippage requiring replacement, and enophthalmos in one patient. CONCLUSION: The frontofacial monobloc advance incorporating distraction osteogenesis can be undertaken with acceptable morbidity even in the very young child and good functional outcomes achieved with one operation where previously more were required. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.