Search

A question that remains unanswered is at what level of surgical correction does the public perceive a head shape to be "normal" or "acceptable?" For most cases of non-syndromic asymptomatic craniosynostosis, the parents desire for surgical correction is to improve the cosmetic appearance of head shape. At the time of this writing, the intraoperative surgeons' perspective of what constitutes an acceptable head shape is the target for surgical correction. In introducing an improved objectively cosmetic goal, an appropriate outcome measure would be to assess what the general public considers a normal or acceptable head shape in children with craniosynostosis. Method Twenty-two unique images were presented via an online crowdsourcing survey of a severe case of non-syndromic sagittal craniosynosis gradually corrected to an age and gender matched normalized head shape. Participants were recruited via the Sick Kids Twitter account. Participants were invited to rate the head shapes as "normal" or "abnormal." Results The 538 participants completed the online survey. Participants were able to reliably and consistently identify normal and abnormal head shapes with a Kappa Score >0.775. Furthermore, participants indicated that a correction of 70% is required in order for the cranial deformity to be regarded as "normal." This threshold closely reflects a normal Cranial Index, which is a widely used morphometric outcome in craniosynostosis. Conclusion Crowdsourcing provides an ideal method for capturing the general population's perspective on what constitutes a normal and acceptable head shape in children with sagittal craniosynostosis. Laypersons are able to reliably and consistently distinguish cranial deformities from a "normal" head shape. The public indicates a threshold correction of 70% in sagittal craniosynosis to regard it as a "normal" head shape.

Background Minor cranial sutural synostosis is currently regarded as a rare diagnosis. As clinical awareness grows, a greater number of cases are being documented. This study aims to describe the variants of unicoronal synostosis with regard to major and minor sutural involvement and secondary effects on cranial and orbital morphology. The information is aimed to improve clinical diagnosis and management. Methods A retrospective study was conducted collecting preoperative computed tomographic scans of patients diagnosed with unicoronal synostosis and listed for surgical interventions, identified from a craniofacial database. Within these patients, different synostotic variants were identified based on which suture was affected. Scans of normal pediatric skulls (trauma) were used for a control group. Computed tomographic scans were analyzed for sutural involvement, cranial base deflection, and ipsilateral and contralateral orbital height and width. One-way analysis of variance was used to detect differences between synostotic variants and controls. Results A total of 57 preoperative computed tomographic scans of patients with unicoronal synostosis were reviewed, in addition to 18 computed tomographic scans of normal skulls (control group). Four variants of unicoronal synostosis were identified: frontoparietal, frontosphenoidal, frontoparietal and frontosphenoidal, and frontosphenoidal and frontoparietal. The last two variants differ in their temporal involvement in the direction of sutural synostosis and ultimately cranial and orbital morphology. Three variants have been previously identified, but the fourth is presented for the first time. Conclusions An understanding of the variants of unicoronal synostosis and their temporal relationships is integral for accurate clinical diagnosis and surgical correction. Recommendations for treatment are based on discrete changes in orbital morphology.

The authors performed bilateral malar reconstruction using polyether ether ketone implants in 3 patients with Treacher-Collins syndrome with absent, as opposed to hypoplastic, zygomata. These patient-specific implants were fabricated using computed-aided design software reformatted from three-dimensional bony preoperative computed tomography images. The first time the authors performed this procedure the implant compressed the globe resulting in temporary anisocoria that was quickly recognized intraoperatively. The implant was immediately removed and the patient made a full-recovery with no ocular disturbance. The computer-aided design and manufacturing process was adjusted to include periorbital soft-tissue boundaries to aid in contouring the new implants. The same patient, and 2 further patients, subsequently underwent malar reconstruction using this soft tissue periorbital boundary fabrication process with an additional 2 mm relief removed from the implant's orbital surface. These subsequent procedures were performed without complication and with pleasing aesthetic results. The authors describe their experience and the salutary lessons learnt.

Predicting outcomes based on a variety of fixation techniques remains problematic in the treatment of mandible fractures. There is inherent difficulty in comparing the hundreds of published articles on the subject because of the large number of variables, including injury patterns, assessment techniques, treatment approach, device selection and application, and definition of outcome.The authors review the behavior of the human mandible. Behavior of the intact mandible, multiple fracture scenarios, and small and large (single and multiple) plating applications are reviewed.Several misconceptions in the literature are clarified. Factors that will resolve the dichotomy between clinical results and current biomechanical theories are presented such that a more logical biomechanical model may be used to approach fixation of the mandibular fracture being treated.Current mandibular biomechanics theory must be expanded to reflect the complex nature of the system and to more accurately describe conditions that exist in the physical world. Otherwise, further analysis in advancements in outcome and treatment will be relegated to chance.

Background: Mandible advancement osteotomy in juvenile rheumatoid arthritis patients for class II anterior open-bite malocclusion is controversial; however, there is no literature discussing outcomes of surgery in this group of patients. An evaluation of such outcomes was undertaken at The Hospital for Sick Children. Methods: A retrospective chart review revealed that eight patients with juvenile arthritis underwent orthognathic surgery during a period of 5 years. All of the patients had a bilateral sagittal split osteotomy of the mandible, six had a simultaneous Le Fort I osteotomy, and four had an additional advancement genioplasty. The age of the patients at the time of surgery ranged between 17 and 22 years (mean, 18 years), with three male patients and five female patients. The patients were under follow-up for a minimum of 8 months after surgery, and measurements were performed studying preoperative and postoperative lateral cephalometric radiographs. Results: All patients have an improved occlusion and improved facial aesthetics. One patient continued to suffer from temporomandibular joint pain. The mean mandible advancement was 9.6 mm (range, 3.9 to 18.3 mm). The mean mandible relapse after surgery was 2.1 mm (range, 1 to 3.1 mm). No exacerbation of the juvenile rheumatoid arthritis was encountered as a result of the surgical procedure. Conclusions: Orthognathic surgery in juvenile rheumatoid arthritis patients improves the patient’s occlusion, facial aesthetics, and therefore quality of life. The relapse rate postoperatively did not significantly affect the clinical outcome, and the authors recommend orthognathic surgery for this patient population. Future presurgical plans in this patient population will include a small posterior open bite and positioning the incisors edge to edge to allow for some postoperative relapse.

Background: Mandible reconstruction in the growing facial skeleton is challenging, especially with reconstructions necessitating free vascularized bone grafts. The need for further combined orthodontic-orthognathic intervention at skeletal maturity must be anticipated. The growth potential of these grafts and potential new problems associated with performing a sagittal split osteotomy at skeletal maturity are poorly understood. Methods: A retrospective chart review revealed 11 patients who underwent reconstruction with a free fibula at The Hospital for Sick Children from 1990 to 2000. Radiographic and photographic studies were assessed for long-term growth of the reconstructed mandible, with follow-up ranging from 2 to 12 years. Results: Of the 11 patients who underwent reconstruction with a free fibula, two had surgery at skeletal maturity, two moved out of the country, one died as a result of her malignancy, and one was lost to follow-up. The remaining five patients are being followed by the craniofacial and orthodontic services and have not reached skeletal maturity. Of the two patients who have had orthognathic surgery, the one patient whose reconstruction involved the temporomandibular joint exhibited no growth on the reconstructed side and required a 57-mm advancement on the reconstructed side. The other patient, who had preservation of the temporomandibular joint at the time of reconstruction, required only a 5-mm advancement on the reconstructed side. Both patients had significant surgical complications as a result of the initial reconstructive techniques. Technical modifications necessary at the initial reconstruction became apparent when the authors performed a sagittal split osteotomy on the reconstructed side. Conclusions: Certain technical modifications to the original free fibula reconstructed pediatric mandible should be considered in anticipation of the need for a subsequent bilateral sagittal split at skeletal maturity.

The purpose of this study was to appraise the value of preoperative speech assessments, nasopharyngoscopy, and surgical models as predictors of velopharyngeal deterioration after a Le Fort I maxillary advancement in cleft patients. This retrospective study involved a series of 26 cleft patients (16 unilateral complete and nine bilateral complete cleft lips and palates, and one isolated complete cleft palate) who had Le Fort I maxillary advancements between March 1, 1993, and February 7, 1996. The 13 male patients and 13 female patients ranged in age from 15.3 to 46 years (mean age, 19.5 years). Four of these patients had previously undergone pharyngeal flap surgery. Eleven patients had palatal fistulas and one had a bifid uvula that was repaired at the time of orthognathic surgery. Patients with perceived hypernasal speech preoperatively all had hypernasality after advancement (nine of nine). Velopharyngeal insufficiency was observed in two of the 16 whose resonance preoperatively was within normal limits. Speech assessment, therefore, predicted accurately the postoperative status in 23 of 26 patients. Twelve patients had preoperative nasopharyngoscopy that indicated a high risk for velopharyngeal insufficiency (borderline or inadequate closure). Nine of these patients had postoperative velopharyngeal insufficiency. Two of the 14 patients not judged at risk by nasopharyngoscopy developed velopharyngeal insufficiency. Therefore, 21 of the 26 patients were accurately predicted by nasopharyngoscopy. Scoping detected borderline velopharyngeal insufficiency in one patient who was not detected by speech alone. The combined predictive value of speech and scope identified all but one patient who would develop postoperative velopharyngeal insufficiency. The degree of anteroposterior movement determined from surgical models was not predictive of the outcome. Patients with hypernasal speech preoperatively continue to have hypernasal speech after Le Fort I advancement. Preoperative perceptual speech assessment by specially trained speech-language pathologists is an excellent test for predicting postoperative velopharyngeal insufficiency status. Nasopharyngoscopy is an invasive and resource-dependent test that should be assessed with respect to cost effectiveness. In this series, only one patient's risk was more accurately predicted using nasopharyngoscopy than by speech assessment alone.

The lack of tip projection on the affected side of a unilateral cleft lip nasal deformity can be difficult to correct due to lack of adequate structural support. A new technique for the correction of the unilateral cleft lip nasal deformity is described. The key components of the technique involve the use of a dorsally angulated unilateral spreader graft on the cleft side and the use of an L-shaped septal graft to provide support to the cleft nasal tip.

Hemifacial microsomia is a hypoplastic disorder of the first and second branchial arches that significantly impacts on the development of the jaws, leading to malocclusion and facial asymmetry. There is little in the literature regarding the application of orthodontic/orthognathic approaches to the correction of these deformities and the stability of the surgical results. To address this, a retrospective chart review of 10 patients with complete orthodontic records and greater than 1 year of follow-up was performed. Posteroanterior cephalograms were assessed by modified Grummons analysis to determine mandibular offset (deviation of the chin point from the skeletal midline) and occlusal cant. These measurements were performed at 3 time points (T1: preoperative, T2: immediate postoperative, T3: follow-up) to elucidate the surgical movement (T2-T1), the postoperative relapse (T3-T2), and the net gain movement (T3-T1). Maxillary movements were quantified, and the occlusal cant was expressed as a ratio between vertical heights of the maxilla at the first molar on each side. One sample t test demonstrated statistically significant surgical movement and net gain. Relapse was statistically insignificant. Repeated-measures analysis of variance demonstrated similar results for chin point position relative to the putative midline. Our results suggest that a combined orthodontic/orthognathic approach at skeletal maturity delivers improved occlusal outcomes in the long term as assessed by chin point deviation and occlusal cant, but secondary surgery rates are higher than those for orthognathic surgery in other patient groups. We advocate limiting surgery to skeletal maturity whenever possible to achieve stable long-term results while limiting morbidity and number of procedures.

Morphological characteristics of the "cleft face" were analyzed by calculating proportion indices in young adults who had undergone surgery in childhood for cleft lip and/or palate: 592 in 37 subjects with unilateral clefts and 432 in 27 with bilateral clefts. Three areas of the face with 16 indices based on 12 projective linear measurements were analyzed in each subject: 5 indices in the general face, 6 in the upper face, and 5 in the lower. Although a balanced relationship was found in two thirds of the indices assessed, severe disproportions, which greatly influence appearance, were noted in about one quarter. In the general face, the most frequent abnormal anthropometric findings contributing to severe disproportions were small upper-face height and a severely high or moderately narrow mandible. In the soft tissues, disproportions included wide nose, small nasal tip protrusion, and short or long columella. Deviations from normality were rarely noted in the nose and upper-lip height, mouth width, total facial height, and width of the upper face. In the upper face, severe disproportions occurred much more frequently in bilateral cleft subjects (67.6%) than in unilateral clefts (30.3%). In the lower face, however, severe disproportions were twice as frequent among unilateral clefts (39.4%) than bilateral (14.7%). The results, although interesting, require complementary preoperative data for reliable analysis of the adult "cleft face."

We have suggested that rigid fixation of membranous bone grafts in the presence of infection may improve graft-recipient bone union by facilitating graft revascularization. To test this hypothesis, we grafted autogenous membranous bone grafts to the mandibles of 94 New Zealand White rabbits. Lag screw fixation was applied in half the animals. The wounds were inoculated with a range of Staphylococcus aureus doses. Infected and noninfected rabbits were injected weekly over a 5-week course with fluorescein bone markers and with a marker of vascular endothelium (procion red) just prior to sacrifice. Revascularization and new bone deposition in the grafts were then quantified histologically for the 75 rabbits available for data collection. Infection decreased the amount of graft revascularized and the amount of new bone deposited for both rigidly fixated and nonfixated grafts. Grafts fixated with a lag screw showed a greater amount of revascularization and new bone deposition in the presence and absence of infection when compared with nonfixated grafts, supporting the hypothesis that rigid fixation of membranous bone grafts in the presence of infection may promote graft survival and union by improving revascularization and osteogenesis within the graft.

Guided tissue regeneration has been shown to permit osteoconduction in otherwise nonhealing cranial defects. The relative importance of preventing the prolapse of soft tissue versus the infiltration of individual connective tissue cells has not been determined. A fibrillar form of polylactic acid (PLA) was tested in 13-mm-diameter defects in the parietal bones of 12 sheep. The polymer was hypothesized to prevent the prolapse of dura and periosteum but allow entrance of individual cells. Control defects in the same sheep were either filled with autogenous bone shavings or left unfilled. The animals were killed at times ranging from 6 to 25 weeks and the defects examined grossly, radiologically, and histologically. The autogenous bone-filled defects were spanned by trabeculated bone by 6 weeks. The unfilled defects demonstrated prolapse of soft tissues into the defect; however, progressive centripetal bone growth was evident. The fibrillar PLA-filled defects were occupied by a full-thickness mixture of fibrous tissue interspersed with PLA. After 19 weeks, small "fingers" of bone were seen to minimally infiltrate the fibrous tissue. Although separation of the dura and periosteum was maintained by the fibrillar PLA, invasion of fibrous tissue restricted osteoconduction.

A stereotactic system has been designed to address the problem of achieving symmetry in complex and extensive craniofacial defects. Preliminary testing suggests that such a system, which allows for the intraoperative application of preoperative CT planning, will be useful in guiding the reconstruction of congenital or acquired bony time, is being used to investigate the correlation of intraoperative globe position following enophthalmos correction with long-term outcome, particularly as it relates to the size and location of the orbital defect, and the timing of the procedure.

A significant release of catecholamines within the heart has been observed during myocardial ischemia. Because this can be markedly inhibited by amine-uptake-blocking agents, it has been suggested that its mechanism is a carrier-mediated efflux from neurons, which is not operative under normal conditions. The present work examined this release process in chromaffin cells isolated from the bovine adrenal medulla, a model system for studying the sympathetic nervous system. Chromaffin cells in primary culture retained normal secretory responses for up to 7 days. Conditions designed to mimic ischemia, that is, anoxia or metabolic inhibition, resulted in a significant release of catecholamines. This release was shown to be independent of extracellular calcium but, in contrast to the release observed in ischemic hearts, was not inhibited by amine-uptake blockers. Electrophoresis with immunoblotting demonstrated that significant levels of the chromaffin granule protein, chromogranin A, were released during metabolic inhibition, indicative of an exocytotic mechanism. However, there was no release of the cytosolic protein, lactate dehydrogenase, indicating that there was no concomitant breakdown of the cell membrane. These results provide evidence for an exocytotic release of catecholamines mediated by the direct action of conditions of metabolic inhibition.

Following craniofacial procedures that involve stripping of the periosteum and soft tissue over the zygomatic maxillary complex, descent of soft tissue with a decrease in anterior projection over the malar area and increase in fullness in the nasolabial fold have been seen to be a problem by these authors. Simple repositioning of the soft tissues to their normal anatomic position may be used to alleviate this problem.

The pronounced variability of the cranial base angle and the degree of maxillary hypoplasia in Crouzon patients is well established in the literature. However, the correlation between these two variables has not been studied previously in Crouzon patients who have not undergone surgery. We studied the nasion-sella-basion and sella-nasion-subspinale (SNA) angles in 16 patients with Crouzon's syndrome, but who had not undergone surgery, and found that such a correlation does not exist. Patients with basilar kyphosis and lordosis manifested variable degrees of maxillary hypoplasia. Furthermore, normal skull base angle in Crouzon patients was not associated with normal SNA angle.

Thirty-five AIDS patients (mean CD4 count 44 x 10(6)/L) with chronic cryptosporidiosis were treated with letrazuril at an initial oral daily dose of 50 mg in an open-label Phase I prospective trial. Treatment was continued for > or = 10 days and for as long as there was a response. The majority of subjects (91%), had previously failed paromomycin treatment. At baseline, 74% of patients had moderate (five to nine bowel movements per day) to severe (> 10 bowel movements per day) diarrhea. Twenty-three subjects (66%) had a clinical response within a mean of 1.7 weeks of treatment initiation. Twenty-two patients had a partial response (> 50% reduction in bowel movements per day for > or = 1 week), one patient had a complete response (two or fewer bowel movements per day). Of the responders, 15 (65%) had a clinical relapse with worsening diarrhea at an average of 1.2 months following initiation of letrazuril. The other eight (35%) had had symptom control for an average of 2.9 months from initiation of letrazuril to the latest follow-up. Microbiologic eradication was demonstrated in 10 (40%) of 25 patients with follow-up stool examinations. Seven patients (20%) experienced a rash, all within 1 week of starting the drug, and resolved in all patients when the drug was discontinued. In conclusion, severely immunocompromised AIDS patients with refractory cryptosporidiosis may show a modest, short-lived response to letrazuril. Microbiologic response is variable and relapse high. Rash is a major limiting side effect of the drug.

The choice of an internal fixation system for maxillofacial surgery is made difficult because of lack of information with respect to functional load. This study attempted to clarify some of the controversy with respect to maxillofacial use of these implants. Maximal compressive force to torque values were measured in standardized bone thicknesses of 1, 2, 3, and 4 mm. The screws tested were pretapped AO 1.5-, 2.0-, 2.7-, and 3.5-mm rescue screws and self-tapping Luhr, Champy, and AO 1.5- and 2.0-mm screws. Ten measurements were made for each screw type/bone thickness combination using a piezoelectric washer and torque screwdriver. It was apparent that for 1- and 2-mm bone thicknesses the use of self-tapping screws resulted in the highest compression values. In 3- and 4-mm bone thicknesses, pretapped screws offered the highest compression values. As expected, self-tapping screws had the highest torque values on insertion owing to torque loss in cutting the screw threads. The 2.7-mm screw offered no advantage over the 2.0-mm screws in 1- and 2-mm bone thicknesses but resulted in higher compression values in 3- and 4-mm bone thicknesses.

Tissue mast cells have been a source of great curiosity since their conclusive description by Ehrlich over 80 years ago. They have been implicated in a vast majority of connective tissue reactions both of physiologic and pathologic nature. In some species at least, mast cells have been implicated in the production, storage and release of potent cardiovascular substances including heparin, serotonin and histamine. The present report is directed particularly toward man. Evidence is presented suggesting that human tissue cells previously lumped into one large group on the basis of granular metachromasia and termed tissue mast cells, represent at least two morphologically distinct cellular types. Difference in function of these cells may be great. There is evidence that one type may be a local tissue source for epinephrine, norepinephrine, or both. It is suggested that mast cells as a whole may represent a spectrum of cell types having numerous differences in ultrastructure and function. It is believed that their continued study in various physiologic and pathologic states should prove fruitful.

Four patients with idiopathic enlargement of the right atrium are described. All patients were evaluated carefully by clinical means and two were subjected to extensive investigation including cardiac catheterization, angiocardiography, and intracavitary electrocardiography. The diagnosis of idiopathic enlargement of the right atrium is based primarily on radiographic examination with exclusion of other causes of right atrial enlargement by appropriate clinical and laboratory means. In the patients studied no cause for the right atrial abnormality could be established. Nonetheless, it is concluded that this disease is a distinct clinical entity that deserves further recognition and investigation.

These studies demonstrated the existence of chromaffin-reacting granules in the human skin. The chromaffin reaction was provided by bichromate fixation. Subsequent special staining by the modified Sevki procedure revealed these granules to be localized usually to characteristic long, eel-like cells with distinctive tinctorial features. Although the true significance of these cells is not certain, they are believed to represent the local source of a vasopressor material for the peripheral tissues. Much indirect physiologic evidence supports this belief.

Difficulties arise in the prediction of maintenance of graft volume over time when bone grafts are used for facial contour reconstruction. We hypothesize that graft fixation will decrease movement and lead to decreased resorption. Fixed and nonfixed endochondral (rib) and membranous (skull) onlay bone grafts measuring 30 X 10 X 4 mm were grafted to the mandible bilaterally in 10 adult sheep. Fixation was achieved using the lag-screw technique. Volume measurements using caliper technique were made 20 weeks postoperatively. The volume of graft present at 20 weeks was significantly greater for the fixed bone grafts (p less than 0.001): fixed membranous, 85.9 percent; fixed endochondral, 76.2 percent; nonfixed membranous, 55 percent; and nonfixed endochondral, 16.6 percent. The results are explained using biomechanical theories related to the effects of strain. At present, it is suggested by this study that when onlay bone grafts are stabilized, improved results with respect to graft resorption can be expected.