Pfeiffer syndrome twins: despite improved correction in one twin, growth disturbance results in similar need for subsequent monobloc advancement.

Background: Twins with Pfeiffer syndrome (or acrocephalosyndactyly) had a similar phenotypic appearance with findings of classic or type 1 Pfeiffer syndrome, including bicoronal and sagittal craniosynostoses, midface hypoplasia, and broad thumbs/toes. We document their treatment with 2 monobloc advancements and discuss growth disturbances in craniofacial dysostosis.

Methods: At 6 months, both twins underwent release of multisuture (bilateral coronal and sagittal) fusions for improvement of scaphocephalic shape and multisuture release; however, one twin had a more aggressive procedure with advancement of the frontal-orbital region.

Sagittal synostotic twins: reverse pi procedure for scaphocephaly correction gives superior result compared to endoscopic repair followed by helmet therapy.

A unique situation of twins with similar sagittal synostosis pathology who underwent different surgical corrective procedures allowed us an opportunity to compare an endoscopic technique to the more traditional technique of a modified cranial vault remodeling (CVR). At 4 months of age, 1 twin underwent an endoscopic-assisted extended strip craniectomy with postoperative helmet therapy for 12 months, and the other underwent a reverse pi CVR procedure. Cephalic index, the Whitaker Aesthetic score, and developmental tests were used for comparison during a 6-year follow-up.

Unilateral vision impairment from a carotid-cavernous fistula after a monobloc osteotomy in a patient with Apert syndrome.

A patient with Apert syndrome who underwent a monobloc osteotomy with distraction advancement sustained partial unilateral vision loss as a complication from a direct carotid-cavernous fistula. Successful embolization was used to treat the fistula. Precaution should be taken by craniofacial surgeons in performing similar procedures in patients with Apert syndrome because of their unique skull abnormalities.