Twenty-year experience with early surgery for XXXX : II. The @entity2122 and pansynostosis--results and unsolved problems.

@entity1 @entity2122 @entity410 @entity1276 @entity428 @entity8213 @entity509 @entity52829 @entity91 @entity10267 @entity1224
As the second of a two-part series, 76 @entity1 with pansynostosis and @entity2122 were retrospectively analyzed. Diagnoses included pansynostosis (7), @entity52829 (8), and Apert (24), @entity10267 . All @entity1 underwent primary fronto-orbital advancement-calvarial vault remodeling procedures at less than 18 months of age (mean 6.1 months). Twenty-eight @entity1 (36.8 percent) required a secondary cranial vault operation (mean age 28.4 months). Additionally, a major tertiary procedure was necessary in 5 @entity1 to deal with persistent unacceptable craniofacial form. To address the associated finding of midface @entity509 , 64.8 percent (n = 35) of @entity1 underwent Le Fort III midface advancement or had that procedure recommended for them. The remainder were awaiting appropriate age for this reconstruction. The more extensive pathologic involvement of the pansynostosis and @entity2122 group is illustrated. As compared with the isolated craniofacial @entity8213 group previously reported, the incidence of major secondary procedures (36.8 versus 13.5 percent), perioperative complications (11.3 versus 5.0 percent), follow-up complications (44.7 versus 7.7 percent), @entity1276 (42.1 versus 3.9 percent), shunt placement (22.4 versus 1.0 percent), and @entity410 (11.8 versus 2.9 percent) was significantly increased. Complex problems including those of @entity428 , @entity1224 , and recurrent turricephaly or cranial vault maldevelopment are repeatedly encountered. In addition, that early fronto-orbital advancement-cranial vault remodeling failed to promote midface development and @entity509 of this region is almost a consistent finding in the craniofacial syndromic group. The average length of postoperative follow-up was 6 years. According to the classification of Whitaker et al., which assesses surgical results, 73.7 percent of @entity1 were considered to have at least satisfactory craniofacial form (category I-II) at latest evaluation. An algorithmic approach to the treatment of all @entity1 with @entity91 is presented utilizing early surgical intervention as the key element.
