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Deformity correction with external fixator in pseudoachondroplasia.

Patients with pseudoachondroplasia have complex, difficult to correct deformities including angular deformity, rotational deformity, and ligament laxity. We retrospectively reviewed seven patients (two children, five adults) with 26 segmental deformities (12 femora, 14 tibiae). We performed bilateral femoral and tibial osteotomies in six patients and bilateral tibial osteotomies in one patient. Distraction osteogenesis was used in 20 segments and acute deformity correction was done in six segments. External fixation was applied to all segments. Of 26 segments, there were five good, 12 fair, and nine poor radiographic results with nine major and 12 minor complications. Recurrent deformity in children and refracture in adults were related to poor results. Of 14 limbs, there were four good, five fair, and five poor clinical results with five major and 14 minor complications. Knee stiffness was the most common complication related to poor results in our series, and occurred particularly in patients with simultaneous correction of the ipsilateral tibial and femoral deformities. Therefore, two-stage surgery including bilateral tibial osteotomies first and then bilateral femoral osteotomies is recommended instead of simultaneous correction of the ipsilateral tibial and femoral deformities to avoid knee stiffness.

Adolescent↗

Upper airway resistance syndrome--one decade later.

PURPOSE OF REVIEW: The term upper airway resistance syndrome (UARS) was coined to describe a group of patients who did not meet the criteria for diagnosis of obstructive apnea-hypopnea syndrome and thus were left untreated. Today, most of the patients with UARS remain undiagnosed and are left untreated. RECENT FINDINGS: Today, the clinical picture of UARS is better defined. We have learned that patients usually seek treatment with a somatic functional syndrome rather than sleep-disordered breathing or even a disorder of excessive daytime sleepiness. Therefore, most of these patients are seen by psychiatrists. In addition, recent technologic advances have allowed a better recognition of the problem. We have learned that obstructive apnea-hypopnea syndrome is associated with a local neurologic impairment that is responsible for the occurrence of the hypopnea and apneas. In contrast, patients with UARS have an intact local neurologic system and have the ability to respond to minor changes in upper airway dimension and resistance to airflow. New treatment options including internal jaw distraction osteogenesis are used and are promising for treatment of patients with UARS. SUMMARY: The clinical presentation of patients with UARS is similar to the presentation of subjects with functional somatic syndrome. To diagnose UARS, nocturnal polysomnography should include additional measurement channels.

Airway Resistance↗

Microvascular free flap reconstruction in pediatric lower extremity trauma: a 10-year review.

BACKGROUND: Limb-threatening wounds of the adult distal lower extremity pose a challenge to the microvascular surgeon and are the subject of a sizable body of literature. The microsurgical management of these injuries in the pediatric population has some unique features but has not previously been the subject of a separate study. METHODS: A review was performed of all pediatric patients (<18 years of age) who received lower extremity free flaps for trauma at the University of Kentucky Medical Center between 1992 and 2002. Charts were reviewed and patients were contacted via telephone and given a standardized questionnaire. Logistic regression was used to identify factors predictive of worse outcome or complications. RESULTS: Twenty-six patients (28 flaps) met the selection criteria. The most common mechanism of injury was all-terrain vehicle accidents (n = 6), followed by motorcycle accidents, lawnmower injuries, and gunshot wounds. The latissimus dorsi muscle flap was chosen in 15 cases, with four other muscle flaps chosen for the remaining 13 cases. There were 12 Gustillo 3B or 3C fractures of the tibia. External fixation was used in 10 cases and internal fixation was used in two. Four patients had a bone gap greater than 4 cm. Three were successfully treated with distraction osteogenesis. The postoperative complication rate was 62 percent, with infection and partial skin graft loss being the most common complications. Three flaps were lost. Growth disturbances were seen in two patients. Patients receiving free flaps within 7 days after injury were seen to have a statistically significant lower rate of complications than those undergoing the procedure beyond the first week. Follow-up ranged from 2 to 11 years, with a mean follow-up of 4.5 years. All limbs were successfully salvaged, and all patients were ambulatory at the time of the survey. CONCLUSIONS: There is a significant role for the microvascular surgeon in the management of limb-threatening injuries in children. If the unique features of this population are taken into consideration, a high rate of limb salvage and good function can be expected.

Accidents, Traffic↗

Le Fort III advancement osteotomy in the growing child affected by Crouzon's and Apert's syndromes: presurgical and postsurgical growth.

This is a longitudinal cephalometric study of patients with craniofacial synostosis (Crouzon's and Apert's) syndromes who underwent Le Fort III advancement during early childhood. The objectives of the study were to quantify presurgical growth, surgical results, and surgical stability as well as long-term postsurgical growth. Of the 31 patients operated on during their growth period (less than 10 years of age), 17 had sufficient records to be included in the study: at infancy, before the Le Fort III osteotomy (average age=7.3 years, range: 4.8-10 years), and immediately after surgery. Ten of the patients were followed radiographically long term for an average of 6.1+/-2.7 years (range: 4.5-10.8 years). The presurgical abnormal midfacial vertical growth pattern was accurately described with a progressive class III discrepancy and an increasing exophthalmos. Remarkable postoperative stability of the maxillary segment was observed. After surgery, growth of the midface was measured, together with minimal, if any, anterior growth, similar to the presurgical growth pattern and rate. No actual detrimental or beneficial effect of surgery on subsequent growth was seen. This study quantitatively confirms that the standard Le Fort III osteotomy is a stable and effective but generally not definitive procedure in childhood. This study might also serve as a control sample to compare with groups of patients undergoing distraction osteogenesis to verify the actual advantages and shortcomings of this alternative technique.

Acrocephalosyndactylia↗

Congenital constriction band syndrome: a seventy-year experience.

Seventy-one cases of congenital constriction band syndrome (CCBS) were reviewed. The year of birth, sex, ethnic background, gestational history, family history, and concurrent diagnoses were obtained. The anatomic patterns of involvement, degree of impairment, and number and type of surgical interventions were recorded. The incidence of CCBS appears to be rising. The average patient had three involved limbs, with a predilection for distal, central digits of the upper extremity. Abnormal gestational histories were found in 60% of the cases; 50% had concurrent diagnoses, and nearly one-third had club-feet. The average patient had a 20% whole body impairment and required three reconstructive procedures. Distraction osteogenesis and free osteocutaneous transfer were useful. We feel that the term "early amnion rupture sequence" more accurately reflects the true pathogenesis of CCBS.

Abnormalities, Multiple↗

Management of inter-dental/inter-implant papilla.

OBJECTIVES: The aims of this paper are to review and compare existing techniques for creation of interdental/interimplant papillae, to address factors that may influence its appearance and to present an approach that authors developed that could help clinicians to manage and recreate the interproximal papillae. METHODS: Papers related to interdental and interimplant papillae published over the last 30 years were selected and analyzed. RESULTS: Thorough treatment planning is essential for maintenance of the height of the interproximal papillae following tooth removal. The key for achieving an esthetically pleasing outcome is the clinicians' ability of properly managing/creating interdental/interimplant papillae. Bone support is the foundation for any soft tissue existence, techniques such as socket augmentation, orthodontic extrusion, guided bone regeneration, onlay graft and distraction osteogenesis are often used for this purpose. Soft tissue grafts as well as esthetic mimic restorations can also be used to enhance the esthetic outcomes. CONCLUSIONS: An esthetic triangle is developed to address the foundations that are essential for maintaining/creating papilla. These include adequate bone volume, proper soft tissue thickness as well as esthetic appearing restorations.

Alveolar Bone Loss↗

Systemic application of growth hormone for enhancement of secondary and intramembranous fracture healing.

Hormones are known to influence bone metabolism and cellular mechanisms of fracture healing. Recent technologies in molecular biology offer recombinant production of hormones, which makes them applicable for pharmacological use. To investigate the effect of systemic growth hormone (GH) application experiments were performed in micropig animal models. Systemic daily subcutaneous injection of species-specific recombinant GH was investigated in Yucatan micropigs to evaluate the effect on secondary fracture healing in a standardized gap model (1 cm) and on intramembranous bone formation in distraction osteogenesis (DO). Quantitative computed tomography (qCT), biomechanical testing, measurement of systemic insulin-like growth factor 1 (IGF-1) levels as well as histomorphometric analyses were performed to investigate differences in regenerate formation. Systemic GH administration significantly increased the torsional stability of the regenerate in comparison to the contralateral side in both experiments. qCT showed accelerated fracture bridging in the GH-treated animals in bone defect healing, while in DO histomorphometry elicited larger callus areas in the case of GH application. Systemic IGF-1 levels were significantly increased in both GH-treated groups. These experiments show that the systemic administration of recombinant GH accelerates fracture healing in standardized animal models. Clinical studies have now been initiated in order to prove the safety and the effectiveness of this therapeutical option.

Animals↗

Treatment of infected pilon nonunions with small pin fixators.

Six consecutive patients with infected intra-articular fractures of the distal tibia were studied. They were treated with resection of all infected or necrotic bone, systemic antibiotics, and instrumentation with a small pin fixator (Ilizarov external fixator). The fixator was used to perform an ankle arthrodesis and to fill the defect created by bone resection with distraction osteogenesis. In all cases, the infections were eradicated, and a solid arthrodesis was attained. The patients required from zero to two revision procedures (average 1.3), and their time in the fixator varied from 3 to 13 months (average 8 months). All of the patients experienced at least minor complications during treatment (superficial pin tract infections). At final follow-up, no patient demonstrated shortening of more than 1.5 cm. One patient has an internal rotation deformity of 15 degrees; a second has a varus deformity of 10 degrees and occasionally uses lateral support (a cane) secondary to unsteadiness on uneven ground; and one patient uses aspirin occasionally for subtalar pain. All are pleased with their results and would undergo the same procedure again without reservation.

Adult↗

Transplantation of new autologous biomaterials into jaw cleft.

This study compared bone-marrow grafting using chondroid or fibula bone grafts transplanted into simulated alveolar bone defects in mice. The osteogenic procedure was also investigated. As an experimental model of the maxillary alveolar bone cleft, suitable for testing bone-inductive materials, a surgical trephine with a low-speed dental engine was used to form critical-sized defects in the pre-maxillary bones of male mice. Distraction osteogenesis was performed using an external fixation device. The osteotomy site was surrounded by an external callus, consisting of hyaline cartilage, that contained a large quantity of chondroid bone. Transplanted bone within chondroid bone was characterized by bone formation and remodelling 30 days post-transplantation, and bone adhesion following chondroid bone grafting was better than adhesion following fibula grafting. The present findings are the first to demonstrate the potential of chondroid bone transplantation as a new therapeutic system of bone grafting, suitable for bone substitution in craniofacial bone defects.

Animals↗

A role for ultrasound in limb lengthening.

The distraction gap at eight sites in six patients undergoing limb lengthening was monitored by both standard radiographs and ultrasound. Using the measurements obtained we were able to assess the rate and quality of callus formation at the distraction site, something that radiographs alone do not enable one to do. We suggest that ultrasound has a valuable role to play in guaging the optimum rate of lengthening for a particular patient and commend it as a technique to complement radiographs in the follow-up of the increasing numbers of patients undergoing distraction osteogenesis.

Adolescent↗

The Ilizarov method in nonunion, malunion and infection of fractures.

We have reviewed a series of 56 consecutive patients treated by the Ilizarov circular fixator for various combinations of nonunion, malunion and infection of fractures. We used segmental excision, distraction osteogenesis and gradual correction of the deformity as appropriate. Treatment was effective in eliminating 40 out of 46 nonunions and all 22 infections. There were two cases of refracture some months after removal of the frame, both of which healed securely in a second frame. Correction of malunion was good in the coronal plane but there was a tendency to anterior angulation, often occurring in the regenerate bone rather than at the original fracture site, after removal of the frame. This was associated with very slow maturation of regenerate bone in some patients, occurring largely, but not exclusively, in those who smoked heavily. Patients expressed high levels of satisfaction with the outcome, despite relatively modest improvements in pain and function, presumably because their longstanding and intractable nonunion had been treated. None the less, the degree of satisfaction correlated strongly with the degree of improvement in pain and function. We emphasise the importance of a multidisciplinary team in the assessment and support of patients undergoing long and demanding treatment. The Ilizarov method is valuable, but research is needed to overcome the problems of delayed maturation of the regenerate and slow or insecure healing of the docking site.

Adolescent↗

Advances in orthodontics.

There has been tremendous progress in orthodontics since Edward Angle first popularised the fixed orthodontic appliance at the turn of the century. Recent years have seen an increased demand for orthodontic treatment from both adolescents and adults and, in addition, patient and clinician expectations of treatment outcomes continue to rise. A desire for more aesthetic materials has resulted in both smaller and 'tooth-coloured' appliances. Improvements in technology, often outside orthodontics, have also led to the development of new materials. The best example of this was the development of nickel titanium alloy by the NASA space programme, which was subsequently adapted for use in nickel titanium archwires. Other technological advances adopted for use in orthodontics include magnets, computerised imaging systems and distraction osteogenesis. This review paper looks at some of the innovations in the fields of materials as well as in techniques and appliance systems.

Adolescent↗

Evaluation of temporomandibular joint in patients with hemifacial microsomia.

OBJECTIVE: The purpose of this study was to elucidate positional relationships between temporomandibular joint (TMJ) components, including the articular discs, using magnetic resonance imaging (MRI) in patients with hemifacial microsomia (HFM). SUBJECTS AND METHODS: Twenty TMJs in 10 patients with HFM were examined at closed- and open-mouth positions using an MRI scanner. The condyle-fossa and disc-condyle relationships, disc configuration at the closed-mouth position, and the reduction of the disc at the open-mouth position were evaluated. RESULTS: On the unaffected side, the condyle-fossa and disc-condyle relationships appeared fairly normal at the closed mouth position. The disc-condyle relationship at the open-mouth position was also normal. The TMJ disc showed normal biconcave configurations at both closed- and open-mouth positions. On the affected side, there was considerable variation in the state of the TMJ. At the closed-mouth position, 5 of the 10 patients revealed fairly normal disc-condyle relationships, one patient showed anterior displacement of the disc, and four patients had no disc. Two patients appeared biconcave, three patients appeared biplanar, and one patient was hemiconvex. At the open-mouth position, the condyle and disc moved in harmony in five patients with normal disc-condyle relationships, but the disc was reduced in a patients with anterior disc displacement. The degree of the TMJ disc dysplasia did not necessarily correspond with the degree of mandibular dysplasia. CONCLUSIONS: The present study contributes to an improved understanding of TMJ pathology in patients with HFM. The results suggest that, in HFM patients, the examination of the TMJ using MRI is helpful for determining treatment procedures in mandibular distraction osteogenesis.

Adolescent↗

Prediction of deterioration of velopharyngeal function associated with maxillary advancement using electromyography of levator veli palatini muscle.

OBJECTIVE: The purpose of the present study was to examine the possibility that postoperative velopharyngeal function following maxillary advancement could be predicted using preoperative electromyography of the levator veli palatini. DESIGN: Levator muscle electromyography was recorded preoperatively during speech and blowing. Levator activity was expressed as a percentage relative to the maximum value observed throughout the experiment. Postoperative velopharyngeal function was evaluated by means of perceptual judgment and nasoendoscopy. PARTICIPANTS: The subjects were four patients with repaired cleft palates who underwent maxillary advancement, two by osteotomy and two by distraction osteogenesis. None of the subjects presented with preoperative hypernasality, and nasoendoscopy demonstrated complete velopharyngeal closure in all subjects prior to maxillary advancement. RESULTS: Preoperative levator activity for speech of two subjects was similar to that for normal speakers (< 60% of total range), and postoperative nasality and nasoendoscopic findings revealed no detectible changes. For the other two subjects, levator activity for speech exceeded 60% of the total range, similar to that of speakers with velopharyngeal incompetence. These subjects showed increased hypernasality and deteriorated velopharyngeal closure following maxillary advancement. CONCLUSION: The deterioration of velopharyngeal function associated with maxillary advancement was demonstrated for subjects whose levator activity was at higher levels during speech in comparison with maximal activity observed during blowing, regardless of the amount of maxillary advancement. Preoperative levator muscle electromyography could be a predictor in identifying patients at higher risk of postsurgical deterioration of velopharyngeal function.

Cleft Palate↗

Low-intensity pulsed ultrasound accelerates maturation of callus in patients treated with opening-wedge high tibial osteotomy by hemicallotasis.

BACKGROUND: Opening-wedge high tibial osteotomy by hemicallotasis for osteoarthritis in the medial compartment of the knee requires external fixation for a long time, until callus maturation is complete. The aim of this study was to determine if low-intensity pulsed ultrasound would accelerate callus maturation when applied after distraction to limbs treated with opening-wedge high tibial osteotomy by hemicallotasis. METHODS: Twenty-one patients with symmetric grades of osteoarthritis and similar degrees of varus deformity in the two knees underwent bilateral one-stage opening-wedge high tibial osteotomy by hemicallotasis. After completion of distraction, the bone mineral density of the distraction callus was measured. Then, one randomly selected limb was subjected to ultrasound treatment for twenty minutes daily until removal of the external fixator. The contralateral limb was left untreated to serve as the control. After four weeks of treatment, bone mineral density was measured again. RESULTS: During the four-week treatment period, the mean increase in callus bone mineral density was significantly greater in the ultrasound-treated tibiae (0.20 +/- 0.12 g/cm(2)) than in the control tibiae (0.13 +/- 0.10 g/cm(2)) (p = 0.02, unpaired t test). In eighteen patients the increase in the bone mineral density was greater in the ultrasound-treated limb than in the control limb, whereas in three patients the increase was greater in the control limb. CONCLUSIONS: We found that low-intensity pulsed ultrasound applied during the consolidation phase of distraction osteogenesis accelerates callus maturation after opening-wedge high tibial osteotomy by hemicallotasis in elderly patients.

Absorptiometry, Photon↗

Alternative gene therapy strategies for the repair of craniofacial bone defects.

Craniofacial anomalies, bone defects and cartilage lesions pose a difficult and challenging problem for both the doctor and for patients and their families. Conventional therapies focus on orthopaedic surgery, grafting with autogenous bone, allogenic bone grafts, or distraction osteogenesis. However, the efficacy of these techniques is limited by high cost, donor morbidity, scarcity of tissue resources, and alterations in volume [Marx & Morales, 1988]. On the basis of recent insights into the development, growth, and adaptation of bone, together with the significant advances in recombinant DNA technology, gene therapy is increasingly becoming recognised as an alternative technique for augmenting and promoting bone regeneration in vivo. It can be applied in craniofacial skeletal tissues by transferring genes encoding for specific growth factors such as BMPs in osteoblasts, chondrocytes or progenitor cells for the purpose of enhancing protein production [Scaduto & Lieberman, 1999]. It can be performed by either direct administration of gene delivery vectors, or by transplantation of genetically modified cells. This review will focus on recent advances in molecular mechanisms of bone formation, and development in various viral and non-viral vectors for direct in vivo therapeutic gene transfer and genetically engineered cells ex vivo therapy.

Adenoviridae↗

[Bone augmentation at present and in the near future].

Autologous bone graft is a gold standard for bone augmentation and is routinely applied. As methods for bone augmentation in the dental field, GBR, distraction osteogenesis, bone substitutes of calcium-phosphate materials and PRP have already been applied clinically. Furthermore, clinical results of the applications of recombinant proteins of BMP and various growth factors, and the applications of the cells from bone marrow and periostium have been reported. A new method for bone augmentation is thus likely to appear. Society accepts medical treatments that are "effective", "safe", "simple" and "economical". Taking the initials of these words, this paper proposes the concept of "2E2S". Based on recent studies of bone augmentation from the viewpoint of "2E2S", the author will predict bone augmentation in the near future.

Bone Regeneration↗

The structure and development of osteogenetic repair tissue according to Ilizarov Technique in man. Characterization of extracellular matrix.

Distraction osteogenesis by the method of Ilizarov has permitted the study of bone formation. This is an analysis of 64 human biopsies in patients who were undergoing tibial lengthening by the method of Ilizarov. After analysis by conventional and polarized light microscopy, four stages of bone formation were identified. Bone formation is of the direct type with no cartilaginous phase.

Biopsy↗