Cranioplasty in Frontometaphyseal Dysplasia

Frontometaphyseal dysplasia is a rare genetic syndrome first described by Gorlin and Cohen in 1969. This disease affects the skeletal system and connective tissue, and could be characterized by hyperostosis of the skull, prominence of supraorbital ridges, additional skeletal and extraskeletal abnormalities. It is believed that the condition is an X-linked dominant trait with severe manifextations in males and extreme variability in females. We described a case of 15-year-old boy manifested a pronounced supraorbital ridge associated with exorbitism. He also had bilateral progressive hearing deficit, thoracic spine scoliosis, chest wall deformity, bilateral maxillary sinusitis and both 5th finger arachnodactyly. The patient underwent a fronto-temporo-orbital cranioplasty through a coronal incision. The frontal bone including supraorbital region, orbital lateral rim and temporal bone were extensively contoured with burr. And then, burring of the medial aspect of lateral orbital wall was made to increase both orbital volume for correction of exorbitism. Postoperative results show well corrected prominent supraorbital ridge, hyperostosis of frontotemporal bone and exorbitism. The patient was satisfied with the improved appearance. No recurrence occurred during the 6 months of follow-up period. We report this case as it shows esthetically good result without any complication.

Noble Tip for Reduction of V-limb of C-V Flap for Nipple Reconstruction

A nipple can be reconstructed either by nipple sharing or local flap. But donor site scar is inevitable when nipple is reconstructed with local flap method. The scar which remains inside the nipple areolar complex can be hidden by medical tattooing, but elongated scar, which cross over the areolar border leads to poor esthetic result. So as to solve this problem, we used purse string suture method. We reconstructed nipple with C-V flap for 9 patients who previously reconstructed breast with extended latissimus dorsi flap. Purse string suture with 3-0 nylon was used for donor site closure. We minimize 10.7% of the scar length with this method. Enhancement of nipple projection due to the flattening of V limb was attained in addition. We expect the reduction of V-limb of C-V flap and amount of absorption of the reconstructed nipple with purse string suture method.