A variety of treatment options exists for the management of giant congenital nevi. Confusion over appropriate management is compounded because not all giant congenital nevi are pigmented, and malignant potential varies between different types. The present study sought to define factors in the presentation of giant congenital nevi that could provide an algorithm for their management, with respect to both the extent of resection and subsequent reconstructive options. A retrospective review of all patients who presented with a congenital nevus of 20 cm2 or greater since 1980 was performed, distinguishing among nevi involving the head and neck, the torso, and the extremities. Sixty-one patients with giant congenital nevi were evaluated (newborn to age 16 years), of which 60 nevi in 55 patients have been operated on. Giant congenital nevi having malignant potential were pigmented nevi (53 patients) and nevus sebaceus (four patients). Those not having malignant potential were verrucous epidermal nevi (three patients) and a woolly hair nevus (one patient). Of the 60 giant congenital nevi operated on, expanded flaps were used in 25, expanded full-thickness skin grafts were used in 10, split-thickness or nonexpanded full-thickness skin grafts were used in 13, and serial excision was used in 30. After 1989, operations tended to use multimodality treatment plans, with an increased use of expanded full-thickness grafts and immediate serial tissue expansion. The use of serial excision, particularly in the extremities, also increased after 1989. Serial excision was the treatment of choice when it could be completed in two procedures or less, which occurred in more than 80 percent of cases using serial excision alone. Expanded flaps were the most common mode of reconstruction in the head and neck region and were used in 49 percent of these procedures. Serial excision was the most common form of treatment in the extremities, used in 50 percent of procedures. Tissue expansion in the extremities was infrequently used to provide an expanded flap (8 percent of procedures), whereas it was frequently used to provide expanded full-thickness skin grafts harvested from the torso (used in 31 percent of procedures). On the basis of these data, algorithms for the extent of resection and subsequent reconstructive options for giant congenital nevi were developed. Their management should be formulated relative to pigmentation, malignant potential, and anatomic location of the respective lesions. (Plast. Reconstr. Surg. 108: 622, 2001.)

Distraction osteogenesis has been used increasingly for midfacial advancement in patients with syndromic craniosynostosis and in severe developmental hypoplasia of the midface. In these patients, the degree of advancement required is often so great that restriction of the adjacent soft tissues may preclude stable advancement in one stage. Whereas distraction is an ideal solution by which to gradually lengthen both the bones and the soft tissues, potential problems remain in translating the distraction forces to the midface. In these patients, severe developmental hypoplasia may be associated with weak union between the zygoma and the maxilla, increasing the chance of zygomaticomaxillary dysjunction when using internal devices that translate distraction force to the maxilla through the zygoma. Eight cases are reported in which either internal or external distraction systems were used for midface advancement following Le Fort III (n = 7) or monobloc (n = 1) osteotomies. Cases of patients in whom hypoplasia at the zygomaticomaxillary junction altered or impaired plans for midface distraction were reported from three host institutions. Seven patients had midface hypoplasia associated with syndromic craniosynostosis, and one patient had severe developmental midface hypoplasia. The distraction protocol was modified to successfully complete midface advancement in light of weakness at the zygomaticomaxillaryjunction in seven patients. Modifications included change from an internal to an external distraction system in two patients, rigid fixation and bone graft stabilization of the midface in one patient, and plate stabilization of a fractured or unstable zygomaticomaxillary junction followed by resumption of internal distraction in four patients. Previous infection and bone loss involving both malar complexes precluded one patient from being a candidate for an internal distraction system. Using a problem-based approach, successful advancement of the midface ranging from 9 to 26 mm at the occlusal level as measured by preoperative and postoperative cephalograms was undergone by all patients. Advantages and disadvantages of the respective distraction systems are reviewed to better understand unique patient characteristics leading to the successful use of these devices for correction of severe midface hypoplasia. (Plast. Reconstr. Surg. 109: 1797, 2002.)

Transforming growth factor-betas (TGF-β) have been demonstrated to be upregulated during osteoblast function in vitro and during cranial suture fusion in vivo. The authors hypothesized that spontaneous reossification of calvarial defects was also associated with upregulation of TGF-β. The present study was designed to (1) evaluate the concept of a critical-size defect within the calvaria in an adult guinea pig model and (2) investigate the association between the reossification of calvarial defects and TGF-β upregulation. Paired circular parietal defects with diameters of 3 and 5 mm and single parietal defects with diameters of 8 or 12 mm were made in 45 six-month-old skeletally mature guinea pigs. Three animals per defect size were killed after survival periods of 3 days, 1 week, 4 weeks, 8 weeks, or 12 weeks. New bone ingrowth was evaluated by assessing for linear closure by a traditional linear method and by a modified cross-sectional area method using an image analysis system in which the thickness of new bone was taken into account. Immunohistochemistry was performed using rabbit polyclonal antibodies to localize TGF-β1, -β2, and -β3. All specimens were photographed, and the intensity of immunostaining was graded based on subjective photographic assessment by three independent reviewers. No defect demonstrated any measurable bone replacement after a survival period of 3 days. All 3- and 5-mm defects were completely reossified after 12 weeks based on the linear analysis of new bone, indicating these defects to be less than critical size. However, new bone formation in the 5-mm defects never exceeded a mean of 40 percent by cross-sectional area of new bone. Percent of new bone formation by cross-sectional area was significantly higher within 3-mm defects than in all larger defects 4 weeks after the craniotomy, reaching a mean of 89 percent new bone by 12 weeks. Persistent gaps were noted on linear analysis of the 8- and 12-mm wounds by 12 weeks, and mean percent new bone by cross-sectional area remained below 30 percent. Immunolocalization demonstrated osteogenic fronts at the advancing bone edge and the endocranial side, in which the osteoblasts stained strongly for all isoforms of TGF-β. The intensity of osteoblast expression waned considerably after the majority of the defect had reossified. These data indicate that histometric analysis based on cross-sectional area more accurately reflects the osteogenic potential of a cranial defect than does linear inspection of defect closure. Although the interpretation of immunolocalization studies is highly subjective, independent assessment by three reviewers indicates that isoforms of TGF-β were upregulated during a limited “window” of time corresponding to the period of active calvarial reossification, and expression of TGF-β corresponded to osteoblast activity within osteogenic fronts. (Plast. Reconstr. Surg. 106: 360, 2000.)

In summary, distraction osteogenesis is a safe and effective means of achieving bone lengthening. These techniques were originally applied to the long bones of the extremities; over the past 10 years they have been effectively applied to the bones of the craniofacial skeleton. The new bone regenerate that is observed after distraction osteogenesis is stable, and relapse rates after skeletal advancement are believed to be lower than with conventional osteotomy and bone graft techniques. There is considerable variability in distraction protocols employed in clinical practice, including differences in the types of devices used and in the rate, rhythm, latency, and period of consolidation for distraction osteogenesis. The greatest application for distraction osteogenesis in the craniofacial skeleton has been with mandible lengthening, for which there is presently a 10-year clinical experience. Midfacial advancement is a newer application of distraction osteogenesis, for which clinical experience has been accrued over the past 5 years. This latter experience indicates that distraction osteogenesis is a viable treatment option for lengthening of the hypoplastic mandible and midface. These techniques have advantages over conventional means of bone graft and rigid fixation because of the quality of the bone regenerate, the decrease in the long-term relapse rate of the advanced bone segments in both the mandible and the midface, and the simultaneous soft-tissue elongation that accompanies the distraction process. Distraction osteogenesis is particularly applicable to the correction of severe deformities of the mandible and midface in children with developmental hypoplasia and syndromic craniosynostosis. However, growth is an added variable in this patient population. The amount of overcorrection in lengthening of the hypoplastic bone required to compensate for continued growth discrepancy of the adjacent facial bones is difficult to predict. Therefore, the families of these patients should be informed that many children will require repeated operations at a later age as they reach skeletal maturity.

Background: Cleft lip represents one of the most common birth defects in the world. Although the timing of cleft lip repair is contingent on a number of factors, the “rule of 10s” remains a frequently quoted safety benchmark. Initially reported by Wilhelmsen and Musgrave in 1966 and modified by Millard in 1976, this rule referred to performing surgery once patients had reached cutoffs in weight, hemoglobin, and age/leukocyte count. Despite significant advances in both surgical and anesthetic technique, the oft-quoted “rule of 10s” has not been systematically investigated since its inception.Methods: Patients who underwent primary cleft lip repair were identified from the National Surgical Quality Improvement Program Pediatric database. Multivariate logistic regression models were used to determine the independent effect of each rule of 10 metric or violation of the rule of 10s as a whole on postoperative complications, and to determine independent risk factors for complications in cleft lip surgery.Results: One thousand three hundred thirteen patients met inclusion criteria, with a 3.6 percent complication rate. Of the included patients, 151 (11.5 percent) violated at least one facet of the rule of 10s. Other than patient weight, neither the rule of 10s nor any individual metric was significantly predictive of postoperative complications.Conclusions: Since its introduction nearly a half century ago, the risks associated with performing surgery in patients who violate the rule of 10s has undergone dramatic reductions. This analysis highlights the need to continually validate and evaluate dogma as the field continues to advance.CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Background: Cone beam computed tomography allows for a significantly lower radiation dose than conventional computed tomographic scans, with generation of accurate images of the maxillofacial skeleton. The authors investigated its accuracy in the volumetric analysis of alveolar cleft defects and simulated bone grafts. Methods: Five simulated alveolar clefts were created using a burr drill in three dry pediatric skulls and filled with simulated bone grafts. Pregrafting and postgrafting cone beam computed tomographic scanning of skulls was performed using specialized imaging software. The authors compared actual volumes of the simulated bone grafts obtained using a water displacement technique with scan-derived volumes of both the grafts and the defects. Results: The average of the five simulated bone grafts calculated by cone beam computed tomography scanning was 0.380 ml, which was lower than their mean volume of 0.392 ml calculated by water displacement. The percentage difference between measurements ranged from 2.9 to 8.6 percent (mean, 4.86 percent). The mean of the simulated defects of 0.399 ml derived from scanning was higher than the actual mean volume of 0.392 ml derived by water displacement. The mean difference in defect comparison was 2.52 percent. There was no statistically significant difference between real volume and scan-derived graft and defect volume. Conclusions: Cone beam computed tomography calculation of simulated alveolar cleft and bone graft volume is precise and accurate. The volume of bone graft needed to fill alveolar defects can be accurately predicted using volume measurements of the bony defect. These findings further validate its use in the perioperative assessment of alveolar grafting.