Search

BACKGROUND The mandibular internal curvilinear distractor design produces a curvilinear vector to provide ideal three-dimensional curvilinear movements compared with the limited straight unidirectional predecessor devices. In this manner, it corrects craniofacial deformities as anatomically as possible, allowing simultaneous bidirectional (rotational and translational) mandibular movement and multidirectional distraction. The aim of this study was to quantify the sagittal and vertical mandibular changes achieved through curvilinear distraction and to assess the long-term effect of this generated bone. METHODS Forty patients (20 male and 20 female), with ages ranging from 5 to 55 years, who underwent mandibular distraction from December of 1999 to August of 2007 at Lucile Packard Children's Hospital in Stanford, California, were included. Preoperatively, postoperatively, and at follow-up (at least 2 years following distraction), panoramic and lateral cephalometric radiographs were traced by plotting different skeletal landmark points and were then analyzed. RESULTS All patients tolerated the curvilinear distraction process well through completion. The average of the mandibular body elongation recorded was 8 to 9 mm and 6 to 8 mm in the panoramic and cephalometric radiographs, respectively; whereas the vertical change of the mandibular ramus achieved was 10 to 12 mm and 10 to 11 mm, respectively. The curvilinear distraction effect on the mandible was found to be significantly stable when the long-term follow-up measurements were compared with the postoperative data revealed in the panoramic and lateral cephalometric radiographs for the two dimensions. CONCLUSION The internal curvilinear device is an effective tool that achieves a stable mandibular distraction, resulting in the correction of craniofacial deformities.

Advances in computing over the last 10 years have rapidly improved imaging and simulation in health care. Implementation of three-dimensional protocols and image fusion techniques are moving diagnosis, treatment planning, and teaching to a next-generation paradigm. In addition, decreasing cost and increasing availability make generalized use of these techniques possible.In this article, the authors present a Web-based, integrated simulation system for craniofacial surgical planning and treatment. Image fusion technology was utilized to create a realistic virtual image that can be manipulated in real time. The resultant data can then be shared over the Internet by distantly located practitioners.Initial use of this system proved to be beneficial from a planning standpoint and to be accurate as to the reliability of landmark identification. Additional case studies are needed to further document the results of actual surgical simulation.This technology presents significant advantages in surgical planning and education, both of which can improve patient safety and outcomes.

Background: The purpose of this study was to retrospectively evaluate the accuracy and reliability of a computer simulation system that predicts the results of an internal curvilinear mandibular distractor in the treatment of hemifacial microsomia and severe mandibular retrognathia. Methods: A two-dimensional computer analysis and simulation system was developed based on cephalometric radiographs and patient photographs. The software was used to generate and superimpose digital templates, corresponding to the actual distractor and logarithmic spiral, onto the lateral cephalometric tracings. The digital distractor's template was then adjusted to fit the radiographic projection of the actual distractor on the 1-week postoperative lateral cephalograph as precisely as possible. It was then activated along the spiral growth curve template until the ideal position of the mandible was reached. This computational surgical simulation tracing was then superimposed onto the actual postdistraction lateral cephalometric radiograph and tracing. Thus, the predicted and actual mandibular distraction movements were compared. Results: In all of the cases, the computational distraction (skeletal and soft tissues) tracing fit the actual postdistraction tracing with an accuracy to within 1 mm. Conclusions: The current study demonstrated that a specific curvilinear distraction can (1) closely reproduce the natural logarithmic spiral movement of the mandibular growth, (2) be closely approximated and thus predicted by a computer simulation system, and (3) be used for correction of mandibular deformities such as hemifacial microsomia and severe mandibular retrognathia.

Dentofacial deformities are traditionally treated by maxillary and mandibular osteotomies conducted separately or simultaneously. Recently, distraction osteogenesis has become an irreplaceable part of the surgical armamentarium, for its ability to induce new bone formation between the surfaces of bone segments that are gradually separated by incremental traction, along with a simultaneous expansion of the surrounding soft-tissue envelope. The aim of this article is to describe a combined surgical technique consisting of simultaneous maxillary Le Fort I advancement and mandibular surgical repositioning by means of bilateral sagittal split osteotomy with a curvilinear distractor based on a preliminary computerized presurgical prediction.

The purpose of this review was to evaluate the clinical outcomes regarding velopharyngeal insufficiency and fistulization in patients with cleft palate who underwent primary repair with the one-stage Delaire palatoplasty. All patients who had a primary Delaire-type palatoplasty performed by the senior surgeon over a 10-year period (1988 to 1998) were studied. During this period, each consecutive patient with an open palatal cleft underwent the same type of repair by the same surgeon. Speech quality and velopharyngeal competence as determined by a single speech pathologist were recorded. A total of 95 patients were included in this series. The average length of follow-up was 31 months (range, 1 to 118 months). Average age at time of surgery was 13.3 months (range, 6 to 180 months). Thirty-one patients (32.6 percent) had significant associated anomalies. The average length of hospital stay was 1.9 days (range, 1 to 8 days) with a trend in recent years toward discharge on postoperative day 1. There were no intraoperative complications, either surgical or anesthetic. Three patients (3.2 percent) developed palatal fistula; none of them required repair. Six patients (6.3 percent) had velopharyngeal incompetence. In patients with more than 1 year of follow-up, the incidence of velopharyngeal incompetence was 9.2 percent (6 of 65). The incidence of fistula after the Delaire palatoplasty was lower than usually reported. The incidence of velopharyngeal incompetence requiring pharyngoplasty was equal to or lower than that seen after other types of palatoplasty, suggesting superior soft-palate muscle function attributable to approximation of the musculus uvulae. The Delaire palatoplasty results in a functional palate with low risk for fistula formation and velopharyngeal incompetence.

Craniosynostosis following ventricular shunting procedures for hydrocephalus has become a recognized complication of shunting procedures. Secondary synostosis results from a decrease in intracranial volume leading to collapse of the cranial vault. Since this represents a distinct etiopathogenesis different from that typically involved, the surgical approach should be altered. Eight patients with secondary scaphocephaly underwent surgical reconstruction. The clinical data and radiographic studies were reviewed for these patients. The surgical approach consisted of sagittal or parasagittal strip craniectomies, lateral osteotomies with bone-flap expansion, occipital and frontal remodeling as needed, and the application of rigid fixation to maintain contour and prevent recurrent collapse of the cranial vault. Patient follow-up ranged from 3 to 37 months. Five of these patients were premature infants, an association not previously recognized in the literature. Satisfactory results were obtained in all patients. Keeping the craniectomy sites parent and achieving a more normal cranial contour through cranial remodeling have provided good results in this population.

Research has clarified the abnormal insertions and orientations of the involved musculature in cleft palate. However, little is known about the physiologic aspects of these muscles, specifically from a histochemical perspective. In this study, 30 muscle specimens were removed from the palatal cleft margin in patients undergoing primary palatoplasty. Nine patients had combined cleft lip and palate deformity, and seven had an isolated cleft palate. Biopsies were taken from the area of the musculus uvulus in all specimens and examined by histochemical techniques. The percentage of type 1 and type 2 fibers was different in isolated cleft palate as compared with cleft lip and palate specimens, being, respectively, 56.7 percent type 1 and 43.3 percent type 2 and 62.0 percent type 1 and 38.0 percent type 2 fibers. This is the opposite ratio from other facial muscles but is in line with the literature regarding normal palatal muscle. Isolated cleft palate muscle fibers also were minimally decreased in diameter as compared with normal facial muscle. Fiber diameters of the combined cleft lip and palate muscles were severely decreased in size compared with those muscles found in the cleft lip. Abnormal mitochondrial accumulations also were found in cleft lip and palate muscle specimens but not in isolated cleft palate muscle specimens. We consider the decrease in muscle diameter found in isolated cleft palate to be secondary to functional atrophy, while that in the cleft lip and palate specimens is secondary to a primary hypoplasia together with a functional atrophy.(ABSTRACT TRUNCATED AT 250 WORDS)

We present a series of 24 consecutive cleft lip and palate patients aged 16 to 46 years (mean age 27 years) who underwent Le Fort I maxillary advancement by the senior author over the past 8 years. Two groups, one of 12 patients with wire fixation and one of 12 patients with miniplate fixation, were evaluated. Each group had 10 unilateral and 2 bilateral clefts. All patients were grafted with autogenous bone (8 cranial, 14 iliac, and 2 mandibular). Horizontal advancement was 3 mm to 2 cm (with a mean of 7.8 mm). Vertical movement ranged from a shortening of 5 mm to a lengthening of 1.3 cm (mean 2.3 mm of lengthening). The amount and timing of relapse were compared in both the horizontal and vertical dimensions. The plated group was more stable in both the horizontal and vertical dimensions (p < 0.05). No significant skeletal relapses occurred after the first year. Statistically significant dental relapse occurred only in the wired group. Three patients developed transverse collapse of the small maxillary cleft segment, and four developed incisor angulation to compensate for maxillary skeletal relapse. The presence of a pharyngeal flap at the time of advancement appeared to increase relapse in both horizontal and vertical dimensions (p < 0.03), but there were too few patients (7 of 24) with pharyngeal flaps to prove this conclusively. We also concluded that pterygomandibular grafting is not necessary to achieve excellent results using miniplate fixation; autogenous grafting of the anterior maxillary osteotomy alone provides the necessary stability.

Muscle biopsy specimens taken from the upper lip and perialar area during the time of secondary lip revision and studied by histochemical techniques demonstrate persistent connective-tissue and muscle abnormalities even at a distance from the cleft margins. Some of these changes are consistent with surgically induced denervation-reinnervation of muscle groups in the surgical field. Increased amounts of connective tissue also were found, most likely secondary to the original deformity and the subsequent surgical procedures. Both these changes may be important factors in subsequent abnormal growth and development of the underlying midfacial structures. This study also demonstrated the resolution of previously noted mitochondrial abnormalities found in the primary cleft lip patient.

Growing skull fractures or posttraumatic leptomeningeal cysts are a known complication of skull fractures sustained in infancy and childhood. They usually present as progressively enlarging cranial masses at the site of the linear skull fractures. The pathogenesis is believed to require an underlying dural tear that allows herniation of the growing pulsatile brain and leads to resorption of the overlying bone. Similar enlarging cranial masses have been reported following craniofacial surgery. Our case occurred following reconstruction of a stenosed metopic suture. The treatment of this unusual complication of craniofacial surgery requires sound dural repair. An understanding of the similarity between this complication and the more common growing skull fractures of childhood is essential to a sound approach to diagnosis and treatment.

Numerous studies have found dura mater-calvarial mesenchyme interactions during calvarial bone induction; however, the exact molecular mechanisms governing these inductive events remain unknown. Recent studies have implicated basic fibroblast growth factor (FGF-2) and transforming growth factor-beta1 (TGF-beta1) in regulating bone formation. The purpose of this study was, therefore, to investigate the expression of FGF-2 and TGF-beta1 during calvarial bone formation in rats. Eight rats were killed on embryonic days 14, 18, and 20 and neonatal day 1 (n = 32). Four animals at each time point were analyzed by in situ hybridization, and the remainder were analyzed by immunohistochemistry. The results indicated that the dura mater underlying the developing calvarial bone strongly expressed FGF-2 and TGF-beta1 mRNA at all time points examined. In contrast, minimal growth factor expression was noted in the overlying calvarial mesenchyme until embryonic day 18, but it increased significantly with increasing age. Importantly, FGF-2 and TGF-beta1 mRNA expression in the dura mater underlying the developing calvarium preceded and was significantly greater than expression in the calvarium mesenchyme (p0.05). Interestingly, minimal expression of FGF-2 and TGF-beta1 mRNA was noted for all time points in the dura mater underlying the posterior frontal suture and within the posterior frontal suture connective tissue (p0.01 when compared with the dura mater underlying the developing calvarium). Immunohistochemical findings closely paralleled mRNA expression, with intense staining for FGF-2 and TGF-beta1 in the dura mater underlying the developing calvarial mesenchyme. Increasing FGF-2 and TGF-beta1 staining was noted within calvarial osteoblasts with increasing age, particularly in cells located near the endocranial surface (i.e., in contact with the developing dura mater). These findings, together with the known biologic functions of FGF-2 and TGF-beta1, implicate these growth factors in the regulation of calvarial bone growth by the developing dura mater. The possible mechanisms of this interaction are discussed.

The mechanisms involved in normal cranial suture development and fusion as well as in the pathophysiology of craniosyostosis are not well understood. The purpose of this study was to investigate the expression of several cytokines—transforming growth factor-beta-1 (TGF-β1), basic fibroblast growth factor (bFGF), and interleukin-6 (IL-6)—during cranial suture fusion. TGF-β exists in three mammalian isoforms that are abundant in bone and stimulate calvarial bone formation when delivered locally. Other bone growth factors including basic fibroblast growth factor and the interleukins regulate bone growth and are mitogenic for bone marrow cells and osteoblasts. The involvement of growth factors in the pathophysiology of craniosynostosis is supported by recent genetics data linking fibroblast growth factor receptor mutations to syndromal craniosynostoses. In this experimental study, in situ hybridization was used to localize and quantify the gene expression of TGF-β1, bFGF, and IL-6 during cranial suture fusion. In the Sprague-Dawley rat, the posterior frontal cranial suture normally undergoes fusion between 12 and 22 days of age, whereas all other cranial sutures remain patent. All in situ analyses of fusing posterior frontal sutures were compared with the patent, control, sagittal sutures. Posterior frontal and sagittal sutures, together with underlying dura, were harvested from rats at 8, 12, 16, and 35 days of postnatal life to analyze posterior frontal suture activity before, during, and after fusion. In situ hybridization was performed on frozen sections of these specimens using DNA probes specific for TGF-β1, bFGF, and IL-6 mRNA. A negative control probe to IL-6 in the sense orientation was also used to validate the procedure. Cells expressing cytokine-specific mRNA were quantified (in cells positive per 10-1 mm2) and analyzed using the unpaired Student's t test. Areas encompassing the fibrous suture and the surrounding bone plates were analyzed for cellular mRNA activity. IL-6 mRNA expression showed a minimal rise in the posterior frontal suture at days 12 and 16, with an average count of 10 and 6 cells per 10-1 mm2, respectively. The sagittal suture remained negative for IL-6 mRNA at all time points. TGF-β1 and bFGF analyses were most interesting, showing marked increases specifically in the posterior frontal suture during the time of active suture fusion. On postnatal day 8, a 1.5-fold increase in posterior frontal suture TGF-β1 mRNA was found compared with sagittal sutures (p = 0.1890, unpaired Student's t test). This difference was increased 26-fold on day 12 in posterior frontal suture TGF-β1 expression (p = 0.0005). By day 35, posterior frontal suture TGF-β1 mRNA had nearly returned to prefusion levels, whereas TGF-β1 mRNA levels in the sagittal suture remained low. A similar upregulation of bFGF mRNA, peaking at day 12, was observed in posterior frontal but not sagittal sutures (p = 0.0003). Furthermore, both TGF-β1 and bFGF mRNA samples with intact dura showed an intense dural mRNA expression in the time preceding and during active posterior frontal suture fusion but not in sagittal tissues. Our data demonstrate that TGF-β1 and bFGF mRNA are up-regulated in cranial suture fusion, possibly signaling in a paracrine fashion from dura to suture. TGF-β1 and bFGF gene expression were dramatically increased both in and surrounding the actively fusing suture and followed the direction of fusion from endocranial to epicranial. These experimental data on bone growth factors support the recent human genetics data linking growth factor/fibroblast growth factor receptor deletions to syndromal craniosynostoses. The ultimate aim of these studies is to understand the underlying mechanisms regulating suture growth, development, and fusion so surgeons may one day manipulate the biology of premature cranial suture fusion. (Plast. Reconstr. Surg. 101: 1431, 1998.)

Variable degrees of macroglossia have been noted in patients with Beckwith-Wiedemann syndrome. Past studies have shown that a major effect of the macroglossia is protrusion of dentoalveolar structures, resulting in an anterior open bite and a prognathic mandibular appearance secondary to an abnormally obtuse gonial angle and increased effective mandibular length. In our series of 11 Beckwith-Wiedemann syndrome patients we have observed that early correction of the macroglossia by means of partial glossectomy has resulted in decreased anterior open bite and mandibular prognathism as compared with patients managed conservatively.

In summary, Aicardi syndrome is defined by its tetrad of infantile spasms, agenesis of the corpus callosum, mental retardation, and chorioretinal lacunae. We report a case of Aicardi syndrome with associated cleft lip and palate. This is an infrequent finding that is present in approximately 3 percent of reported cases. Plastic surgeons should be aware of this association when treating patients with cleft lip and palate.

Although attention has been focused for decades on the correction of cleft lip deformities, our knowledge about the etiology of such deformities has remained presumptive. Sixty-six muscle biopsy specimens from cleft lip infants were obtained at the time of primary closure. Histochemical stains, histographic analysis, and electron microscopy were performed. A nonneurogenic muscle atrophy was seen that varied in severity, with muscle fibers near the cleft being the most atrophic and disorganized. Muscle fibers stained with the modified Gomori trichrome technique also demonstrated "ragged red" fibers typical of a mitochondrial myopathy. Electron microscopy confirmed large accumulations of mitochondria distorting the fibrils. These mitochondria also were increased in size and densely packed with cristae. This study thus demonstrates that the muscles in cleft lip deformities are not normal. Instead, they reflect either myopathy in the facial mesenchymal mitochondrion or at least a delay in maturation. We hypothesize that some of the morphologic deformities associated with cleft lip may cause a failure of mesenchymal reinforcement of the facial processes at a critical time in development.