Long-Term Follow-Up of Adamantinoma of the Tibia Complicated by Metastases and a Second Unrelated Primary Cancer: A Case Report and Literature Review.

Adamantinoma is a rare, low-grade malignant tumor of the bone which grows slowly and typically occurs in the diaphysis of long bones, particularly in the tibia. Adamantinomas have the potential for local recurrence and may metastasize to the lungs, lymph nodes, or bone. We report a case of a 14-year-old female with a tibial adamantinoma who underwent wide resection with limb salvage and has subsequently been followed up for 18 years. The patient went on to have both a local soft tissue recurrence 5 years after the resection and metastases to both an inguinal lymph node and the right lower lobe of the lung 8 years after that recurrence, all of which have been treated successfully with marginal resections. Unique to this case, the patient was also incidentally found to have chromophobe-type renal cell carcinoma when undergoing a partial nephrectomy to resect a presumed metastasis of her adamantinoma. Genetic testing has not revealed any known genetic predisposition to cancer.

Mandibular Body Resection and Setback for Severe Malocclusion in Lymphatic Malformations.

Extensive lymphangiomas of the facial skeleton result in deforming forces leading to ongoing masticatory, speech, oral hygiene, and airway problems. This paper presents a small series of patients with severe mandibular overgrowth secondary to lymphatic malformations. Following debulking of the malformations and tongue reductions, the authors describe the results of their treatment with bilateral mandibular body resections and setback. The authors' results suggest that severe functional impairment from deforming malformations can be addressed in childhood with orthognathic surgery. Improved occlusion, oral closure, and airway opening can be achieved using this procedure.

Repair of a Critical Porcine Tibial Defect by Means of Allograft Revitalization.

BACKGROUND: The authors previously described the generation of vascularized bone in a pig model, using a hemimandibular allograft scaffold, adipose-derived stem cells, recombinant human bone morphogenetic protein-2, and periosteum. This study tests the hypothesis that this "allograft revitalization" technique is as effective as vascularized autograft for repairing critical bony defects. METHODS: Three groups of pigs had 3-cm defects created in their bilateral tibial diaphyses for repair using rigid fixation and one of three modalities. Negative control tibias were repaired with allograft tibia alone. To simulate repair using vascularized autograft, the osteotomized bone in positive control animals was left in situ, with the posterior periosteum intact. Experimental animals' defects were repaired with allograft tibia packed with autologous adipose-derived stem cells and recombinant human bone morphogenetic protein-2, with native periosteum intact. After 8 weeks, unilateral midgraft osteotomies were performed to assess graft healing potential. Serial radiographs and terminal micro-computed tomography and histology enabled evaluation of healing. RESULTS: At week 7 after ostectomy, no negative control tibias had healed (zero of six) whereas most positive control (five of six) and all experimental tibias (six of six) had healed. Unilateral midgraft osteotomies were performed at 8 weeks to assess graft ability to heal. As expected, no negative control tibias (three of three) had radiographic union 7 weeks later. However, all positive control (two of two; p = 0.05) and experimental (three of three; p = 0.01) tibias had healed their repeated osteotomies by this time. CONCLUSION: Similar to vascularized autograft, revitalized allograft successfully repaired a critical tibial defect, including after refracture, suggesting that this technique may be an alternative to osseous free flaps.

Establishing a Reproducible Hypertrophic Scar following Thermal Injury: A Porcine Model.

BACKGROUND: Our complete understanding of hypertrophic scarring is still deficient, as portrayed by the poor clinical outcomes when treating them. To address the need for alternative treatment strategies, we assess the swine animal burn model as an initial approach for immature scar evaluation and therapeutic application. METHODS: Thermal contact burns were created on the dorsum of 3 domestic swine with the use of a branding iron at 170°F for 20 seconds. Deep partial-thickness burns were cared for with absorptive dressings over 10 weeks and wounds evaluated with laser and negative pressure transduction, histology, photographic analysis, and RNA isolation. RESULTS: Overall average stiffness (mm Hg/mm) increased and elasticity (mm) decreased in the scars from the initial burn injury to 8 weeks when compared with normal skin (P < 0.01). Scars were thicker, more erythematous, and uniform in the caudal dorsum. The percent change of erythema in wounds increased from weeks 6 to 10. Histology demonstrated loss of dermal papillae, increased myofibroblast presence, vertically oriented vessels, epidermal and dermal hypercellularity, and parallel-layered collagen deposition. Immature scars remained elevated at 10 weeks, and minimal RNA was able to be isolated from the tissue. CONCLUSIONS: Deep partial-thickness thermal injury to the back of domestic swine produces an immature hypertrophic scar by 10 weeks following burn with thickness appearing to coincide with the location along the dorsal axis. With minimal pig to pig variation, we describe our technique to provide a testable immature scar model.

Evolution in minimal-incision palatoplasty: surgical technique and outcomes in 67 consecutive cases.

BACKGROUND: Conventional palatoplasty relies on extensive mucosal incisions and isolation of flaps on the palatine vessels to facilitate midline closure and velar reconstruction. This introduces substantial scarring, which has adverse effects on vascularity and growth. The authors have developed a minimally invasive palatoplasty technique that may have advantages over traditional techniques. The authors present their operative experience and outcomes when using the minimally invasive method paired with a novel cranial base maneuver for nasal mucosa closure. METHODS: Based on cadaveric dissections, the authors developed a modified minimal-incision approach that permits anatomical reconstruction from a midline approach. From 2003 to 2010, a retrospective review was performed on 67 consecutive minimal-incision palatoplasties. Cases requiring relaxing incision and/or conversion to other palatoplasty techniques were compared with minimal incision alone. RESULTS: Minimally invasive palatoplasty alone was able to be performed in 78 percent (n=52) of all cases. Fistula rates were 7.6 percent (n=4) in the minimally invasive palatoplasty group and 20 percent (n=3) in the relaxing/conversion group (p=0.04). Of patients requiring relaxing incisions/conversion, a higher percentage were syndromic (73 percent; p=0.01). Eighty-nine percent of all Veau class I defects were able to be successfully closed with the minimally invasive palatoplasty approach (p=0.01). CONCLUSIONS: Minimal-incision palatoplasty paired with a cranial base maneuver for nasal mucosa elevation results in adequate soft-tissue mobility and length to arrive at a tension-free closure. Fistula and velopharyngeal insufficiency rates are comparable to that of other techniques, and theoretical advantages of this technique will be borne out by longer term follow-up. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Bone tissue engineering by way of allograft revitalization: mechanistic and mechanical investigations using a porcine model.

PURPOSE: "Allograft revitalization" is a process in which cadaveric bone is used to generate well-vascularized living bone. We had previously found that porcine allograft hemimandibles filled with autologous adipose-derived stem cells (ASCs) and recombinant human bone morphogenetic protein-2-soaked absorbable collagen sponge (rhBMP-2/ACS) were completely replaced by vascularized bone, provided the construct had been incubated within a periosteal envelope. The present study sought to deepen our understanding of allograft revitalization by investigating the individual contributions of ASCs and rhBMP-2 in the process and the mechanical properties of the revitalized allograft. MATERIALS AND METHODS: Porcine allograft hemimandible constructs were implanted bilaterally into rib periosteal envelopes in 8 pigs. To examine the contributions of ASCs and rhBMP-2, the following groups were assessed: group 1, periosteum alone; group 2, periosteum+ASCs; group 3, periosteum+rhBMP-2/ACS; and group 4, periosteum+ASCs+rhBMP-2/ACS. After 8 weeks, the allograft constructs were harvested for micro-computed tomography (CT) and histologic analyses and 3-point bending to assess the strength. RESULTS: On harvesting, the constructs receiving rhBMP-2/ACS had significantly greater bone shown by micro-CT than those receiving periosteum only (51,463 vs. 34,310 mm3; P = .031). The constructs receiving ASCs had increased bone compared to group 1 (periosteum only), although not significantly (P = .087). The combination of rhBMP-2/ACS with ASCs produced bone (50,399 mm3) equivalent to that of the constructs containing rhBMP-2/ACS only. The 3-point bending tests showed no differences between the 4 groups and a nonimplanted allograft or native mandible (P = .586), suggesting the absence of decreased strength of the allograft bone when revitalized. CONCLUSIONS: These data have shown that rhBMP-2/ACS significantly stimulates new bone formation by way of allograft revitalization and that the revitalized allograft has equivalent mechanical strength to native bone.

Outcomes of mandibular distraction osteogenesis in the treatment of severe micrognathia.

IMPORTANCE: Patients with severe micrognathia are predisposed to airway obstruction. Mandibular distraction osteogenesis (MDO) is an alternative to tracheotomy that lengthens the mandible in order to improve the retrolingual airway. This study presents outcomes from one of the largest cohorts reported. OBJECTIVE: To assess the rate and predictors of surgical success and complications among (1) patients who underwent MDO prior to other airway procedures (MDO first), and (2) patients who required an initial tracheotomy and were subsequently treated with MDO (tracheotomy first). DESIGN, SETTING, AND PARTICIPANTS: Retrospective cohort study at a tertiary care pediatric medical center of patients diagnosed as having micrognathia resulting in symptomatic airway obstruction (Pierre Robin sequence) and who underwent MDO from September 1995 to December 2009. INTERVENTIONS: Electronic medical records were reviewed. Multivariable regression analysis was used to assess for predictors of outcome. MAIN OUTCOMES AND MEASURES: Rates of surgical success (defined as either tracheotomy avoidance or decannulation) and complications. Potential predictors included demographics, syndrome presence, follow-up time, and surgical history. RESULTS: A total of 123 patients (61 in MDO-first subgroup, 62 in tracheotomy-first subgroup) underwent MDO during the study period. Median age at time of distraction was 21 months (range, 7 days-24 years). Surgical success and complication rates were 83.6% and 14.8% in the MDO-first subgroup and 67.7% and 38.7% in the tracheotomy-first subgroup. Tracheotomy-first patients were more likely to have a syndromic diagnosis (66.0% vs 43.0%; P = .009) and were older at the time of MDO (median age, 30 months vs 5.1 months; P < .001). Poorer odds of success were associated with the need for 2 or more other airway procedures (odds ratio [OR], 0.14 [95% CI, 0.02-0.82]) in the MDO-first subgroup and craniofacial microsomia or Goldenhar syndrome (OR, 0.07 [95% CI, 0.009-0.52]) in the tracheotomy-first subgroup. CONCLUSIONS AND RELEVANCE: Mandibular distraction osteogenesis has a high rate of success in avoiding tracheotomy. Patients who required a tracheotomy before MDO had a lower success rate in achieving decannulation and a higher rate of complications. However, these patients also had a higher rate of syndromic diagnoses and associated comorbidities. Patients with Goldenhar syndrome have a decreased likelihood of surgical success.

Tissue expander complications in the pediatric burn patient: a 10-year follow-up.

BACKGROUND: This study is a 10-year follow-up to our previous publication reviewing the complication rates of tissue expansion in the pediatric burn population. The purpose of this study was to determine if our institutional experience with tissue expanders had remained stable during the subsequent 10 years. METHODS: There were 240 patients who were identified at a major pediatric burn center who underwent reconstruction with a tissue expander (256 tissue expanders) from 1996 to 2006. Data were obtained retrospectively by reviewing patient medical records. Complications were categorized into absolute and relative complications. RESULTS: Absolute complications occurred in 36 (14.1%) of 256 expanders placed and relative complications occurred in 26 (10.2%) of 256 expanders placed. There was no statistical difference between this study and our previous study for overall complication rates and complications for all sites. The highest complication rate occurred when the scalp was a surgical site. Betadine skin preparation was associated with a 10% reduction in infection-related complications compared to other skin preparations. The operating surgeon or age of patient was not found to be associated with increased complication rates. CONCLUSIONS: After changing our tissue expander protocol, the complication rates at our institution have remained stable during the 10-year follow-up period. Tissue expansion in the pediatric burn population continues to be a safe and effective reconstructive option with acceptable complication rates.

Dissection in the subgaleal and subperiosteal plane: implications on scalp wound healing.

Craniofacial reconstruction often involves the use of dissection in the subperiosteal or subgaleal plane to access the cranial vault and facial skeleton. Clinically, physical changes to the periosteal layer and underlying cortex were observed in the re-operative field. This article compares aspects of wound healing, structural integrity of the progenitor periosteal layer, and underlying bony changes when elevating a subgaleal or subperiosteal flap in a Lewis rat calvarial model. Cranial dissection in the subperiosteal or subgaleal plane was performed on 14 Lewis rats. En bloc resection of the calvarium and overlying soft tissue was harvested at days 0, 1, 3, 7, 14, 28, and 56. Samples underwent SEM imaging and were analysed histologically after trichrome and haematoxylin and eosin staining. One sample of native periosteum underwent cellular expansion to determine periosteal cell regenerative capability. Up to 56 days, subperiosteal dissection results in diffuse hypercellularity within the cambial layer (p < 0.001). There are irregular cortical changes at the periosteal interface and increased disorganised bone remodelling at the temporal ridges. Subgaleal dissection did not reveal any underlying bony changes, and cell counts were not significantly different from controls (p < 0.001). Subperiosteal dissection causes structural and cellular changes to the periosteum and underlying bone composition with a possible influence on its regenerative capability.

Current practice in assessing and reporting speech outcomes of cleft palate and velopharyngeal surgery: a survey of cleft palate/craniofacial professionals.

OBJECTIVE: To determine methods by which professionals serving cleft palate/craniofacial teams are evaluating velopharyngeal function and to ascertain what they consider as a successful speech outcome of surgery. DESIGN: A 12-question survey was developed for professionals involved in management of velopharyngeal dysfunction. PARTICIPANTS: The survey was distributed through E-mail lists for the American Cleft Palate-Craniofacial Association and Division 5 of the American Speech-Language-Hearing Association. Only speech-language pathologists and surgeons were asked to complete the survey. A total of 126 questionnaires were completed online. RESULTS: Standard speech evaluations include perceptual evaluation (99.2%), intraoral examination (96.8%), nasopharyngoscopy (59.3%), nasometry (28.9%), videofluoroscopy (19.2%), and aerodynamic measures (4.3%). Significant variation existed in the types and levels of perceptual rating scales. Pharyngeal flap (52.9%) is the most commonly performed procedure for velopharyngeal insufficiency, followed by sphincter pharyngoplasty (27.5%). Criteria for surgical success included normal speech (50.8%), acceptable speech (27.9%), and "improved" speech (8%). However, most respondents felt that success should be defined as normal speech (71.2%). Most respondents believed that surgical success should be determined by the team speech-language pathologist (81.5%); although, some felt success should be determined by the patient/family (17.7%). CONCLUSION: This survey shows considerable variability in the methods for evaluating and reporting speech outcomes following surgery. There is inconsistency in what is considered a successful surgical outcome, making comparison studies impossible. Most respondents thought that success should be defined as normal speech, but this is not happening in current practice.

Pediatric thermal injury: acute care and reconstruction update.

BACKGROUND: The acute and reconstructive care of each pediatric burn patient presents unique challenges to the plastic surgeon and the burn care team. METHODS: : The purpose of this review article is to highlight the interdependence between the acute and reconstructive needs of pediatric burn patients as it pertains to each anatomical site. Relevant principles of acute pediatric burn care and burn reconstruction are outlined, based on the authors' experience and review of the literature. RESULTS: The need for late reconstruction in pediatric burn survivors is significantly influenced by the acute surgical and rehabilitative treatments. With their vulnerability to airway swelling, hypothermia, pulmonary edema, and ischemia-reperfusion injury, pediatric patients with large burns require precise, life-saving treatment in the acute phase. Decision-making in pediatric burn reconstruction must take into account the patient's future growth, maturity, and often lack of suitable donor sites. CONCLUSION: Appropriately selected reconstructive techniques are essential to optimize function, appearance, and quality of life in pediatric burn survivors.

Prevention and management of outpatient pediatric burns.

Burns are common injuries in the pediatric population, with an estimated 250,000 pediatric burn patients seeking medical care annually. A relative few require inpatient management. This article discusses suggestions for burn prevention, as well as acute burn care and long-term management of small burns.

Management of pediatric burns.

It is estimated that in the United States there are 2.0 million burn injuries every year. There are 30,000 inpatient admissions and between 1,000 and 5,000 deaths per year related to burns in children. Along with the elderly, children suffer the highest rates of morbidity and mortality from thermal injuries. Achieving good clinical outcomes requires early, accurate diagnosis and aggressive treatment. Once acute burn injuries have evolved into fixed deformities they can be next to impossible to treat, leading to life-long limitations in form and function. Rehabilitation is a critical factor in achieving an acceptable functional and cosmetic outcome for both adult and pediatric burn patients. Without a rigorous and well-orchestrated rehabilitation program, the treatment of a burn is not complete. The management of burn injuries also frequently involves reconstructive surgical intervention. Acute or sub-acute reconstruction is aimed at restoring lost anatomic structures such as the eyelids and nose. In general, scars lead to surgical evaluation because of either functional loss or cosmesis. This paper discusses our experience and procedures for managing burns in the paediatric population. As a general rule, if wounds have failed to heal within 5 to 7 days, the patient should be referred to a surgeon familiar with paediatric burn injuries.

Neonatal kyphectomy in the patient with myelomeningocele.

STUDY DESIGN: A retrospective cohort study was used to investigate a group of neonates with myelomeningocele who had a kyphectomy performed in conjunction with dural sac closure during the first few days of life. OBJECTIVES: To assess the effectiveness of operative intervention in the neonatal period to correct the kyphotic deformity in the patient with myelomeningocele and to monitor its long-term results. SUMMARY OF BACKGROUND DATA: Orthopedic management originally focused on the immediate treatment of the kyphotic deformity in the infant with myelomeningocele. However, there has been a movement toward postponing surgical treatment of the kyphos until a later age. This study included the longest follow-up of the largest group of neonates that a single surgeon has managed surgically since the treatment of this condition was originally described. METHODS: The radiographic and clinical results for all neonates treated with a kyphectomy at the time of myelomeningocele closure between 1980 and 2000 were analyzed. RESULTS: Neonatal kyphectomy was performed on nine males and two females. The average preoperative kyphotic angle measured 67 degrees. The average initial correction was 77 degrees, and the average loss of correction at follow-up assessment was 55 degrees. There were no serious complications, and wound closure was successful in all patients. One patient required a repeat kyphectomy and posterior spinal fusion at the age of 9 years and 2 months. The average follow-up period was 7 years and 4 months (range 44-174 months). CONCLUSIONS: Kyphectomy performed at the time of dural sac closure in the neonate is a safe procedure with excellent initial correction. Eventual recurrence is expected despite the procedure. However, it occurs in the form of a longer, more rounded deformity that is less technically demanding.

Skin resurfacing for the burned patient.

It has been estimated that 2 million people per year have burns requiring medical attention in the United States. The available and expert clinicians in dedicated burn centers around the country have cared successfully for these patients and given them a second chance at a functional life. It still behooves current-day plastic surgeons to be knowledgeable and adept in their care, not only because they may be called upon at times to manage some of the smaller acute burns, but also because many of the general principles of burn reconstruction and wound management are relevant to other areas of general plastic surgery. Acute burns should be dealt with like any other major trauma with the ABCs of aggressive resuscitation and airway management. Like any other wound, debridement and nutrition are important (i.e., early escharectomy of the burn wound and enteral nutrition during the hypermetabolic state). Early coverage of the open wound is essential to limit bacterial colonization and prevent infection and to reduce fluid and electrolyte and heat loss. If autografts are not available immediately, temporary coverage with one of the above-mentioned barrier materials should be used. Still, autografts, when available, should be the burn surgeon's first choice. Donor sites may be reharvested to provide more autograft than was anticipated with large-percentage TBSA burns. Physicians should keep in mind the advantages (and disadvantages) of using the scalp and back. As far as research and technological advances in the area of plastic surgery, burn surgery may be the most progressive, with the evolution of biologic tissue-engineered skin substitutes and the research of growth factors in healing. Further improvements in tissue engineering and technology should result in even more effective skin substitutes and hence better functional and aesthetic outcomes with economic efficiency in large burns.