Faculty Bibliography

PURPOSE: Multi-vector distraction osteogenesis has been described for use in the reconstruction of complex mandible deformities. Using 3-dimensional computer simulation, the location of the osteotomy and arc of the device can be predetermined, allowing for precise planning and determination of end points. Here, we discuss our experience using this technique and propose methods to optimize outcomes and minimize discrepancies in the projected results. METHODS: Three patients who underwent curvilinear mandible distraction and preoperative computer-simulated planning are described. After simulation to determine level of osteotomy and arc of the curvilinear distractor device, design-specific guides were constructed. Intraoperatively, custom guides were used to precisely perform the osteotomy and for placement of each distractor. After the completion of distraction and device removal, the correlation of distraction end points, occlusion, and the volumes of the mandibles were analyzed. RESULTS: A vertical difference of 2.6 mm (shorter) from the proposed final position and an 8-mm sagittal difference (under correction) were seen in the first patient. In the second patient, the final position had a vertical difference of 5.7 mm (shorter) and a sagittal difference of 14 mm (undercorrected). In the third patient, after first simulating anterior rotation of the condyle before osteotomy and vector design, the final and simulated end points differed approximately 0.6 mm vertically (shorter) and 4.3 mm sagittally (undercorrected) CONCLUSIONS: Preoperative 3D simulation and planning is a useful adjunct in curvilinear mandible distraction; however, the forces of the muscles of mastication and the restrictive soft tissue envelope must be accounted for in the design to achieve precise end results.

PURPOSE: Posterior vault expansion using distraction osteogenesis has become a vital instrument in our institution, particularly as a first-line treatment in syndromic craniosynostosis. In this review, we highlight the several advantages, diverse utility, and technicalities of the operative procedure. METHODS: A review of the literature and explanation of the technical details of the procedures were described in this manuscript. RESULTS/CONCLUSION: Posterior cranial vault distraction offers several benefits over traditional expansion procedures.

BACKGROUND: Isolated nonsyndromic bicoronal synostosis is a relatively rare entity that produces a characteristic turribrachycephalic skull shape. The purpose of this study was to evaluate the long-term outcomes of the isolated nonsyndromic bicoronal synostosis patients treated at the authors' institution. METHODS: A retrospective chart review of all patients who underwent cranial vault remodeling for nonsyndromic bicoronal synostosis was performed at the Children's Hospital of Philadelphia from 1991 to 2011. Fifteen patients were identified for this study, and information regarding their demographic, perioperative, and postoperative details were analyzed. RESULTS: Four boys and 11 girls were identified for inclusion in this study. The average age at the time of the initial surgery was 9 months, with an average follow-up of 13.4 years. There were no reported complications. Six patients with at least a 10-year follow-up (six of 10 patients) underwent revision for contour improvement in the forehead and temporal regions, and two patients required a repeated fronto-orbital advancement. Four patients underwent subsequent strabismus surgery. No patients were documented to have midface hypoplasia requiring orthognathic surgery. One patient with an associated diagnosis of autism was noted to have significant developmental delay. CONCLUSIONS: Isolated nonsyndromic bicoronal synostosis confers a high rate of revisions for contour deformities but is associated with a low risk of complications. Overall, nonsyndromic patients require less secondary fronto-orbital advancement when compared with syndromic patients. In contrast to the syndromic population, there were no instances of midface hypoplasia necessitating surgery. There did not appear to be significant correlation between bicoronal synostosis and developmental abnormalities or delays.

Osteoradionecrosis (ORN) of the mandible is a potentially catastrophic complication of external beam radiation therapy for head and neck malignancies. A 55-year-old man treated with chemoradiation for base-of-tongue cancer presented with a necrotic left mandibular body and underwent left mandibulectomy with right free fibula osteocutaneous flap reconstruction. Two and a half years later, he presented with right mandibular body ORN and underwent a second mandibular resection and subsequent reconstruction with a left fibula osteocutaneous free flap fixed in the midline to the previously placed contralateral fibula. He recovered well from all procedures. To the best of our knowledge, we report the first case of a patient with metachronous ORN of the mandible requiring resection and subsequent reconstruction with sequential free fibula flaps. Even in the presence of prior microvascular reconstructions, patients can successfully undergo additional reconstructive procedures to restore their function, appearance, and quality of life.

BACKGROUND: The aesthetics of facial structure are used by humans to measure one's beauty, character, and overall "goodness." Individuals born with cleft lip and/or palate are often stigmatized and face much psychosocial adversity. Social attitudes and beliefs have a direct impact upon the psychological development of these individuals. Such social norms are in large part shaped by the physical representations of "good" and "attractive" in various art media including films, advertisements, and paintings. OBJECTIVE: Individuals born with a cleft have been portrayed in the artworks of different eras. The light in which they are portrayed stems from the prevalent beliefs of each period and sheds light on the social attitudes of each epoch toward clefts. Here we discuss the social and psychological ramifications of these works. We then review several artworks representing cleft lip and/or palate and propose an active role for the artist in shaping social attitudes regarding facial deformities. METHODS: Numerous articles and works of arts were examined and inspected for signs of facial deformity, with particular attention to cleft lip and/or palate. CONCLUSION: Social media have an important role in defining the norms of society. Much of the art of the past has depicted negatively individuals born with cleft lip and/or palate deformity, thus excluding them from the norm. In order to decrease the negative social stigmas of cleft lip and/or palate, it is now the responsibility of society to widen its range of norms to include individuals born with these deformities through "normal" representations in the various media.

INTRODUCTION: Lymphedema is a chronic disorder that occurs commonly after lymph node removal for cancer treatment and is characterized by swelling, fibrosis, inflammation, and adipose deposition. Although previous histological studies have investigated inflammatory changes that occur in lymphedema, the precise cellular make up of the inflammatory infiltrate remains unknown. It is also unclear if this inflammatory response plays a causal role in the pathology of lymphedema. The purpose of this study was therefore to characterize the inflammatory response to lymphatic stasis and determine if these responses are necessary for the pathological changes that occur in lymphedema. METHODS: We used mouse-tail lymphedema and axillary lymph node dissection (ANLD) models in order to study tissue inflammatory changes. Single cell suspensions were created and analyzed using multi-color flow cytometry to identify individual cell types. We utilized antibody depletion techniques to analyze the causal role of CD4+, CD8+, and CD25+ cells in the regulation of inflammation, fibrosis, adipose deposition, and lymphangiogenesis. RESULTS: Lymphedema in the mouse-tail resulted in a mixed inflammatory cell response with significant increases in T-helper, T-regulatory, neutrophils, macrophages, and dendritic cell populations. Interestingly, we found that ALND resulted in significant increases in T-helper cells suggesting that these adaptive immune responses precede changes in macrophage and dendritic cell infiltration. In support of this we found that depletion of CD4+, but not CD8 or CD25+ cells, significantly decreased tail lymphedema, inflammation, fibrosis, and adipose deposition. In addition, depletion of CD4+ cells significantly increased lymphangiogenesis both in our tail model and also in an inflammatory lymphangiogenesis model. CONCLUSIONS: Lymphedema and lymphatic stasis result in CD4+ cell inflammation and infiltration of mature T-helper cells. Loss of CD4+ but not CD8+ or CD25+ cell inflammation markedly decreases the pathological changes associated with lymphedema. In addition, CD4+ cells regulate lymphangiogenesis during wound repair and inflammatory lymphangiogenesis.

BACKGROUND: Although fat deposition is a defining clinical characteristic of lymphedema, the cellular mechanisms that regulate this response remain unknown. The goals of this two-part study were to determine the effect of lymphatic fluid stasis on adipogenesis and inflammation (part I) and how these changes regulate the temporal and spatial expression of fat differentiation genes (part II). METHODS: Adult female mice underwent tail lymphatic ablation and were euthanized 6 weeks after surgery (n = 20). Fat deposition, fibrosis, and inflammation were then analyzed in the regions of the tail exposed to lymphatic fluid stasis as compared with normal lymphatic flow. RESULTS: Lymphatic fluid stasis in the tail resulted in significant subcutaneous fat deposition, with a 2-fold increase in fat thickness (p < 0.01). In addition, lymphatic stasis was associated with subcutaneous fat fibrosis and collagen deposition. Adipogenesis in response to lymphatic fluid stasis was associated with a marked mononuclear cell inflammatory response (5-fold increase in CD45 cells; p < 0.001). In addition, the authors noted a significant increase in the number of monocytes/macrophages as identified by F4/80 immunohistochemistry (p < 0.001). CONCLUSIONS: The mouse-tail model has pathologic findings that are similar to clinical lymphedema, including fat deposition, fibrosis, and inflammation. Adipogenesis in response to lymphatic fluid stasis closely resembles this process in obesity. This model therefore provides an excellent means with which to study the molecular mechanisms that regulate the pathophysiology of lymphedema.