Врожденная деформация лица при синдроме Крузона с краниофациальным дистракционным остеогенезом

A 14 year old boy diagnosed with the a congenital facial deformity disorder- Crouzon's syndrome approached BDCH for correction of his deformity. The disorder causes early closure/fusion of the skull bones leading to uncoordinated and improper facial profile. For this boy, the amount of advancement required was large that was beyond the means of Lefort III advancement surgery. For this boy, a surgical treatment was to perform a Lefort III advancement of the midfacial region and then using Distraction osteogenesis, to advance the midface. For this treatment, a bicoronal incision was placed and the entire skin was reflected. Care was taken to preserve the periosteum of the cranial bone intact. The supra-orbital -trochlear region was identified and the cut was started from there on. Using gentle surgical manipulations, the entire content of the orbit was closely removed in such a way that the entire components of the eye was with the coronal flap. The bone cut was extended in to the frontozygomatic region creating a traditional Lefort III cut . Care was taken to place the bone cut along the frontonasal region so that the nasal architecture is not affected. Following this the zygoma is split carefully preserving the nasal bone regions. Then through the intra-oral approach, the pterygoid- maxillary dysjunction was carried out. The dysjunction was then completed from the extra-oral approach too. Using appropriate instruments, all the delicate structures were persevered. This type of approach led to the entire maxillary/ nasal component to be made mobile as a "monobloc". Hence the name - Monobloc advancement too. A Kawamotto's distractor was used to advance the dislodged maxillary/nasal complex. The arms of the distractor was carefully placed on the thin temporal bone (only 4mm long screw used) and on the zygoma also. This is an Internal distractor where only the active arm is only visible externally, that too along the scalp region. Such arrangement removes the requirement of cumbersome traditional rigid external framework based craniofacial distraction osteogenesis. In addition, along the docking site, recombinant Bone Morphogenetic Protein (rhBMP2) was placed to accelerate the bone formation. After careful checking of the distractors, all the layers were closed. A drain was placed and combination of subcutaneous stitches and staples were used to close