Levels of plasma matrix metalloproteinases (MMP-2 and MMP-9) in response to INTEGRA® dermal regeneration template implantation.

BACKGROUND: Cutaneous wound healing results in scar formation. Matrix metalloproteinases (MMP) transform extracellular matrix proteins and modulate inflammation and cell signaling, thus determining scar outcome. To provide rapid wound closure and reduced scarring, dermal scaffolds were introduced. Little is known about the influence of these materials on MMPs levels. MATERIAL AND METHODS: In this in vivo study the levels of MMP-2, MMP-9, and mediators of inflammation and fibrosis (IL-4 and TGF-beta1) in patients treated with Integra® dermal regeneration template (IDRT) were investigated. In the group of 11 pediatric patients treated with IDRT, levels of selected molecules were analyzed before surgery and at day 1, 7, and 25 after scaffold implantation. RESULTS: The mean IDRT take rate was 89.5 ± 4.7% with 4 patients (36%) who developed local infection. Patients were divided into 2 groups according to presence of infection (1 group with complications and 1 group without complications). In the group with complications, the IDRT take rate was significantly reduced compared to the group without complications (71.5 ± 5.4 vs. 100 ± 0.1; p<0.005). Plasma levels of MMP-2 were significantly (p<0.05) elevated in both groups on day 7 after the scaffold placement compared to baseline. Positive correlations between IL-4 and MMP-2 (p=0.01) in the group with complications and TGF-beta1 and MMP-9 (p=0.012) in both groups were observed. CONCLUSIONS: These findings suggest that Integra® scaffold degradation is mainly caused by MMP-2, whereas inflammation associated with local infection increases levels of this molecule and it is not associated with elevation of MMP-9. This shows that dermal regeneration with Integra® uses molecular mechanisms other than scar formation during dermal wound healing.

Multidisciplinary approach to the treatment of a patient with chondrodystrophic myotonia (Schwartz-Jampel vel Aberfeld syndrome): case report and literature review.

Chondrodystrophic myotonia, Schwartz-Jampel syndrome, is a rare congenital disorder, which results from disturbance in a perlecan protein synthesis. Most affected are the muscles, acting in generalized myotonia, leading to joint contractures, weird-looking mask-like face appearance, and causing vision disturbances. Also, impaired bones and cartilages result in skeletal anomalies and dental disorders. Allergic reactions to numerous drugs occur in affected individuals. Surgical treatment is risky, due to a low tolerance of anesthetics and high risk of malignant hyperthermia. In this article, a case of 17-year-old girl with Schwartz-Jampel syndrome is presented. The patient with typical syndrome debilitations underwent long diagnostic scheme and multiple treatments in order to restore acceptable appearance and function. All procedures were performed with cautious anesthesiologists' supervision. Modern diagnostic tools can improve the diagnosis rate while multidisciplinary evaluation and advanced treatment techniques can reveal symptoms, minimizing the hazard of anesthesia and improving the quality of life.

Changes in the plasma cytokine and growth factor profile are associated with impaired healing in pediatric patients treated with INTEGRA® for reconstructive procedures.

BACKGROUND: Large full thickness skin defects caused by trauma or surgery require skin grafting, often in conjunction with dermal scaffolds such as INTEGRA(®). Due to the size and severity of these procedures, complications such as infection may occur. This can lead to poor healing outcomes. OBJECTIVE: To identify early biomarkers of complications during INTEGRA(®) healing. METHODS: Levels of EGF, TGF-ß1, FGF-2, VEGF, IFN-α, GM-CSF, IL-4 and IL-8 were measured pre-surgery and at days 1, 7 and 25 post-surgery in peripheral blood of 15 pediatric patients treated with INTEGRA(®) for reconstructive procedures. The levels of these molecules were analysed with respect to the occurrence of complications. RESULTS: Complications (local infection) occurred in a group of 4 patients. This resulted in a reduced INTEGRA(®) take rate comparing to the group without complications (71.5±5.4% vs. 98.1±0.7%). In cases with complications there were significantly higher plasma concentrations of IL-4 and FGF-2 on day 7 (p=0.037 and p=0.008 respectively). Other markers were not significantly different between groups or at very low level at all time-points. WCC and CRP remained within normal ranges at all time-points. CONCLUSIONS: This data suggests that elevated levels of IL-4 and FGF-2 at early time-points after surgery may predict the development of complications in patients with INTEGRA(®). This may enable early interventions to prevent complications in procedures involving the use of INTEGRA(®).

Expansion of the posterior cranial vault using distraction osteogenesis.

BACKGROUND: Expansion of the posterior cranial vault more profoundly enlarges intracranial volume compared with the anterior region. Conventional vault remodeling techniques are limited by scalp forces and may relapse with supine positioning. The purpose of this study was to demonstrate the efficacy of posterior vault distraction and evaluate perioperative variables compared with conventional methods in syndromic children. METHODS: This was a retrospective analysis of consecutive children who underwent posterior vault expansion using distraction osteogenesis. Information was compiled regarding demographics, perioperative details, distraction protocol, and complications. RESULTS: Eight children were identified, two boys and six girls. Diagnoses of Apert, Crouzon, Saethre-Chotzen, and Pfeiffer syndromes were present. Chiari malformation was present in two children. The posterior distraction procedure was undertaken at a mean of 21 months (range, 5 to 36 months). Mean operative time was 3.8 hours (range, 2.6 to 5 hours), blood loss averaged 487 ml (range, 300 to 2000 ml), and hospital stay was 3.25 days (range, 2 to 4 days). A latency period of 72 hours and rate of 2/3 mm/day was used in three patients, and 1 mm/day was used in five children. The mean advancement was 23 mm (range, 19 to 32 mm) and consolidation was 77 days (range, 42 to 100 days). One child experienced fracture of distraction arms during the activation period. Mean follow-up was 278 days (range, 90 to 548 days). CONCLUSIONS: These preliminary findings indicate that posterior vault distraction is a viable technique with a favorable perioperative profile compared with conventional treatment. Posterior distraction can be the initial strategy with which to address intracranial pressure, allowing delay of definitive frontoorbital advancement until later in childhood.

Tongue dysmorphology in craniofacial microsomia.

BACKGROUND: Craniofacial microsomia is one of the most common and well-characterized craniofacial anomalies. Tongue dysmorphism, however, has been neither thoroughly investigated nor reported in the context of this disease. This review focuses on the true prevalence of tongue dysmorphology in craniofacial microsomia and its relation to the deformities seen in this condition. METHODS: A 20-year retrospective study was performed to determine the number of patients who had a documented tongue anomaly and any relation to the development of abnormal speech. In recognition of the limitations of this approach, a 1-year prospective study was also performed to see the true prevalence of tongue dysmorphology in these patients. RESULTS: Eight of 167 patients (4.8 percent) in the retrospective study were found to have tongue dysmorphologies, as opposed to 24 of 55 (43.6 percent) in the prospective study. The majority of tongue anomalies were mild. Of the eight retrospective patients, seven currently have intelligible speech with a combination of intensive speech therapy and/or surgical correction. The eighth patient is without intelligible speech. Tongue dysmorphology was positively correlated with the degree of hard- and soft-tissue deformity. CONCLUSIONS: Tongue dysmorphologies in craniofacial microsomia, although usually mild, are frequently overlooked. The correlation of the tongue, soft tissue, and mandible anomalies may point to a common error early in gestation or an interdependence of adjacent growth centers.