Craniofacial Patients Research Articles

Brain electrophysiology reveals intact processing of speech sounds in deformational plagiocephaly.

The prevalence of deformational plagiocephaly has risen dramatically in recent years, now affecting 15 percent or more of infants. Prior research using developmental scales suggests that these children may be at elevated risk for developmental delays. However, the low positive predictive value of such instruments in identifying long-term impairment, coupled with their poor reliability in infants, warrants the development of methods to more precisely measure brain function in craniofacial patients. Event-related potentials offer a direct measure of cortical activity that is highly applicable to young populations and has been implemented in other disorders to predict long-term cognitive functioning. The current study used event-related potentials to contrast neural correlates of auditory perception in infants with deformational plagiocephaly and typically developing children. Event-related potentials were recorded while 16 infants with deformational plagiocephaly and 18 nonaffected controls passively listened to speech sounds. Given prior research suggesting their association with subsequent functioning, analyses focused on the P150 and N450 event-related potential components. Deformational plagiocephaly patients and normal controls showed comparable cortical responses to speech sounds at both auditory event-related potential components. Children with deformational plagiocephaly demonstrate neural responses to language that are consistent with normative expectations and comparable to those of typical children. These results indicate that head shape deformity secondary to supine sleep is not associated with impairments in auditory processing. The applicability of the current methods in early infancy suggests that electrophysiologic brain recordings represent a promising method of monitoring brain development in children with cranial disorders. Risk, II.

Study on Utilizing Rapid Prototyping Technology to Build Oral and Maxillofacial Models

Traditional dental impression approach is not applicable to most of the oral and craniofacial trauma patients. Without a physical model, it is not easy to evaluate a patients fracture and occlusion, limiting the treatment process. Especially, the accuracy of the maxillofacial model for occlusion evaluation needs to meet strict clinical demands. Therefore, in this research, we attempted to use computed tomography (CT), without damaging the oral and craniofacial tissues of patients, together with image processing and Rapid Prototyping (RP) technique to obtain physical oral and maxillofacial models with high accuracy. Initially, a set of procedures of generating maxillofacial model was developed. CT images were segmented and converted to a CAD file by a commercial medical image processing software. RP technique was used to fabricate maxillofacial model. After comparison, the deviations were greater than clinical demands of less than 1 mm. After analyzing the sources of errors, issues of CT slice thickness, images threshold selection and editing, and RP fabrication were investigated to improve the accuracy. As a result, updated standard procedures were suggested to obtain RP maxillofacial models with higher accuracy. The improved average deviation can be reduced to 0.22 mm. The biological RP models with high accuracy generated in this research can be used to improve success rate and safety in a surgery, to reduce complications after the surgery, and to decrease the time and cost of treatment.

The Impact of a Parent Liaison at a Major Craniofacial Center

The psychosocial impact of craniofacial disfigurement affects both the developing child and his/her family. The Facial Reconstruction Center at the Children's Hospital of Philadelphia has employed a Parent Liaison (PL) to provide psychosocial support to families and has been an invaluable resource in this regard. We hypothesize that a PL impacts the overall outcome of the surgery by building trust between the parents and medical institution, and increasing satisfaction. An anonymous satisfaction survey was sent to families of craniofacial patients treated between January 1976 and June 2012. All patients who had undergone at least 1 craniofacial procedure had addresses on file and were included in this study. Statistical analyses were performed using the Mann-Whitney U test.During the study, 441 surveys were mailed to families meeting the inclusion criteria. A total of 151 families returned completed surveys (34.2%), and 121 surveys were included for analysis (27.4%). In rating overall satisfaction, families who met with the PL had statistically higher scores than those who had not (P = 0.0011). Parents who met with the PL preoperatively reported greater satisfaction in time spent answering questions (P = 0.0029) and the perception that questions were adequately answered (P = 0.0039). No statistical difference was observed in postoperative preparedness between families that did and did not meet the PL. The results demonstrate that the PL is beneficial in the education, experience, and satisfaction of families treated at a large Craniofacial Center. The PL complements the surgeon's treatment of the physical by adding psychosocial support.

Transdermal Scopolamine and Perioperative Anisocoria in Craniofacial Surgery

Postoperative nausea and vomiting (PONV) is a common complaint after plastic and reconstructive surgery. Transdermal scopolamine is a commonly used agent for prevention of PONV. Anisocoria from transdermal scopolamine use is an adverse effect that has not been reported in the plastic surgery literature. We present a series of 3 craniofacial patients in which ipsilateral mydriasis occurred and spontaneously resolved after removal of the scopolamine patch. Given the various causes and potentially grave implications of unilateral mydriasis, we discourage the use of transdermal scopolamine in craniofacial surgery, and especially in orbital surgery. However, if transdermal scopolamine is decided to be used for PONV prophylaxis, we recommend educating the patient, the operating room staff, and the surgical team regarding this potential adverse effect and to avoid finger-to-eye contamination after patch manipulation.

Anesthetic Risks Associated With Antley-Bixler Syndrome

Antley-Bixler syndrome is an autosomal recessive disorder characterized by multiple bone and cartilaginous abnormalities. The main features of this syndrome include brachycephaly, midface hypoplasia, dysplasia of ears and nose, radiohumeral synostosis, choanal stenosis, or atresia. Distinctive features are based on craniofacial deformity and humeroradial synostosis. In this report, we describe the anesthesia management of a 20-year-old Antley-Bixler syndrome patient who underwent maxillary advancement via Le Fort I osteotomy. During surgical management of craniofacial syndrome patients, particularly Antley-Bixler syndrome, the whole surgical team should be aware of possible deformities involving the airway, which may be underestimated or nondetected prior to surgery. These deformities including choanal atresia/stenosis may lead to failure of nasotracheal intubation and mask ventilation, therefore jeopardizing the surgical procedure and/or patient safety. Accurate preoperative preparation and being aware of the components of this syndrome is vital to eliminate respiratory complications and enable uneventful anesthetic and surgical management.

Resonance and Speech Articulation After Midface Advancement in Craniofacial Dysostosis

This study aimed to analyze changes in resonance and speech articulation after midface advancement in syndromic craniofacial patients and to assess the influence of craniofacial diagnosis and the presence or absence of a cleft palate. This study is a retrospective analysis of resonance and speech articulation in patients after midface advancement. This project was carried out in a multidisciplinary pediatric craniofacial service. Eighteen patients underwent midface advancement between 2002 and 2009. Three were excluded because of inadequate records or presence of tracheostomy. Midface advancement was done by Le Fort III, facial bipartition, or monobloc, either conventional surgery or distraction osteogenesis. Outcomes include perceptual assessment of articulation and resonance using GOS.SP.ASS.98 revised and recommendation for speech surgery. Hypernasality scores decreased in 7 patients (46.7%), and 5 patients were recommended for speech surgery. Hyponasality scores improved in 10 patients (66.7%), were unchanged in 3 patients (20%), and decreased in 2 patients (13%). Articulation changed (improved) in 1 patient (6.7%) only. Hypernasality scores decreased in 33.3% of Crouzon and 71.4% of Apert patients. Five patients had a cleft palate, 4 had Apert syndrome, and hypernasality scores decreased in 3 patients. Of 3 patients with Apert syndrome but no cleft palate, 2 (66.7%) also had a decrease in hypernasality scores. Our findings suggest a high incidence of deterioration in velopharyngeal function after midface advancement, particularly in Apert syndrome, regardless of the presence of a cleft, and an improvement in hyponasality, but minimal change in articulation. Larger prospective multicenter studies are required to investigate these findings further.

Development of 3D statistical mandible models for cephalometric measurements

PurposeThe aim of this study was to provide sex-matched three-dimensional (3D) statistical shape models of the mandible, which would provide cephalometric parameters for 3D treatment planning and cephalometric measurements in orthognathic surgery.Materials and MethodsThe subjects used to create the 3D shape models of the mandible included 23 males and 23 females. The mandibles were segmented semi-automatically from 3D facial CT images. Each individual mandible shape was reconstructed as a 3D surface model, which was parameterized to establish correspondence between different individual surfaces. The principal component analysis (PCA) applied to all mandible shapes produced a mean model and characteristic models of variation. The cephalometric parameters were measured directly from the mean models to evaluate the 3D shape models. The means of the measured parameters were compared with those from other conventional studies. The male and female 3D statistical mean models were developed from 23 individual mandibles, respectively.ResultsThe male and female characteristic shapes of variation produced by PCA showed a large variability included in the individual mandibles. The cephalometric measurements from the developed models were very close to those from some conventional studies.ConclusionWe described the construction of 3D mandibular shape models and presented the application of the 3D mandibular template in cephalometric measurements. Optimal reference models determined from variations produced by PCA could be used for craniofacial patients with various types of skeletal shape.

Pattern of craniofacial anomalies seen in a tertiary care hospital in Saudi Arabia.

BACKGROUND AND OBJECTIVE:To date, there are no published studies from Saudi Arabia on the incidence or etiology of craniofacial anomalies. This study aimed to report the patterns of craniofacial anomalies in Saudi Arabia.DESIGN AND SETTING:Hospital-based, descriptive study conducted during 2002 to 2009 in the Cleft Lip/Palate and Craniofacial Anomalies Registry at King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.PATIENTS AND METHODS:Data was collected on craniofacial patients in the registry.RESULTS:Of the 447 craniofacial patients (male, 242; female, 205), 109 (24.4%) had only cranial anomalies, 261 (58.4%) had only facial anomalies and 77 (17.2%) had both of these conditions. Craniosynostosis was seen in 33.3% of the total patients (81 males and 68 females). Of the 65 craniosynostosis syndromic patients, 25 (38.5%) had Apert syndrome and 18 (27.7%) had Crouzon syndrome. Among facial anomalies, 47 (19.4%) had dysmorphic features, followed by 35 (14.5%) with micrognathia. Among facial syndromes, 72 (59.0%) were observed to have Pierre-Robin sequence, 17 (13.9%) had Goldenhar syndrome and another 17 (13.9%) had Van der Woude syndrome. Cleft palate was more common in 171 (56.8%) patients as an associated deformity, followed by cleft lip with cleft palate in 99 (32.9%) and cleft lip in 23 (7.6%) patients. Of the 224 patients having other congenital anomalies, the cardiovascular system was most commonly affected, with 46 (20.5%) children diagnosed with congenital heart disease. A family history of anomalies was observed more in children born to parents of a consanguineous marriage than in those whose parents were unrelated (P=.01).CONCLUSIONS:Additional efforts should be made towards creating awareness among the general population about these deformities in relation to consanguinity.

Analysis of Comprehensibility of Patient Information Regarding Complex Craniofacial Conditions

Health care consumers are increasingly turning to the Internet for information regarding medical and surgical procedures. When an elective procedure is under consideration, the Internet is often the first resource used by a patient. Caregivers of craniofacial patients are typically overwhelmed during the surgical planning process. A firm understanding of craniofacial condition and the associated procedures is crucial to obtain satisfactory outcomes. Furthermore, health care providers are increasingly referring their patients to on-line sources of patient education material. Currently, the National Institutes of Health suggests the information be at the fourth- to sixth-grade reading level to maximize comprehension. Much of the information available regarding health care targeted at patients is written at a 10th-grade reading level or higher. The purpose of this study was to evaluate readily available on-line patient education information for readability; being aware of this information will aid craniofacial surgeons in appropriately educating their patients. Texts were extracted from commonly used craniofacial educational Web sites regarding reconstructive procedures. Three objective and accepted methods (SMOG, Flesch-Kincaid, and Dale-Chall) were used to assess readability of each condition and its corresponding procedure's text. The results from all 3 of the methods used were higher than the recommended seventh-grade reading level. The mean reading level for eMedicine was 13.8, 15.2, and 15 for the Flesch-Kincaid, SMOG, and Dale-Chall methods, respectively. Likewise, the mean reading levels for FACES were 7.5, 10.7, and 8.3; and for World Craniofacial Foundation, the levels were 11.9, 13.8, and 13. Patient education and understanding is a critical factor in planning for surgery; this is especially true of reconstructive craniofacial procedures. Craniofacial surgery is a diverse field, and its surgeons have correspondingly diverse practices. It is up to each individual surgeon to determine what is appropriate for his or her patients. Our results show that on-line educational material is at a level that is substantially higher than the national reading average. The ultimate impact of this fact will vary from practice to practice, but all surgeons should be aware of the possible conflicts between information distributed and the patient's ability to comprehend that information. This may assist surgeons in preoperative evaluations by discussing conditions with more level appropriate means.

Reduction in donor exposure with use of reconstituted blood in craniofacial patients – an alternative explanation

Reduction in donor exposure with use of reconstituted blood in craniofacial patients – an alternative explanation

V-Y Advancement Flaps for Scalp Closure After Acute Cranial Volume Expansion

Sometimes the surgeon who contemplates a coronal incision for craniofacial surgery, such as fronto-orbital advancement, faces a situation in which closure by primary suture becomes impossible because of acute cranial volume expansion. To avoid such a situation, the authors invented V-Y advancement flap method. This flap was found to be highly effective in 6 craniofacial patients who were treated between March 2007 and March 2008. The method consists of an incision line that starts from the highest spot on the ear and proceeds in the direction of the hairline at 45 degrees, and then runs up to the vertex in a sawtooth manner. This line is used when acute cranial volume expansion is expected after surgery. Another incision line is symmetrically drawn on the opposite side of the head. These 2 lines are conjoined at the vertex. The lengths of the 2 sides of a single sawtooth are both about 2 to 3 cm. When the scalp of an expanded cranium is closed, some V-shaped scalp margins under normal tension can simply be sutured using a traditional V-V advancement flap. However, the others under increased tension need to be in a Y-shaped pattern. During the follow-up period (6 months on average), application of this V-Y advancement flap method led to good functional and aesthetic outcomes in all 6 cases. The authors attribute these results to scalp closure using a mixture of V-V and V-Y flaps.

The Risk of Growing Skull Fractures in Craniofacial Patients

Background/Aims: Growing skull fractures (GSFs) are unusual sequelae of head injury in young children which have also been reported to occur after craniofacial procedures complicated by inadvertent durotomy. We reviewed the craniofacial experience in a single institution, detailing 180 cases of craniofacial surgery and suspected dural tears and their relationship to the subsequent development of GSFs. This experience was then compared to that of the pertinent published literature. Methods: A retrospective review of the senior authors’ craniofacial surgical experience from 2000 to 2007 was performed. This was compared to an English-language literature review of GSFs after craniofacial surgery. Results: In our institution, 180 cases of craniofacial surgery (83 open, 97 endoscopic) were performed, with an average follow-up of 23.5 months. Twenty-five patients (15 open, 10 endoscopic surgeries) had operative dural compromise. One of these patients developed a persistent pseudomeningocele requiring reoperation. Twelve cases of GSF after craniofacial surgery were identified in a review of the English-language literature, of which 7 (58%) had coronal suture fusion. Conclusions: While durotomy may occur during craniofacial surgery, the subsequent development of a GSF appears to be an unlikely event with an aggressive intraoperative approach of identification and repair. Coronal craniosynostosis may confer an increased risk for this complication.

Coronal Incision With Preserved Temporoparietal Fascia

The conventional or zigzag bicoronal incision has been widely used in craniofacial surgery. Unfortunately, after the incision, the temporoparietal (TP) fascia has to be abandoned because of its vascular or structural damages. The author describes a technique to preserve the TP fascia during the incision. The coronal incision stopped at the subcutaneous plane. The dissection continued subcutaneously under the posterior edge of the incision as in the elevation of TP fascia. After cutting through the TP fascia, the dissection continued anteriorly in the subgaleal plane as in the coronal dissection. A total number of 15 craniofacial patients underwent bicoronal incision with this technique. The time spent for preservation of the TP fascia was 10 to 15 minutes for both sides. The bicoronal incision with preserved TP fascia as described provides an alternative to the patient who may need TP fascia for future uses.

Treatment strategies for total skeletal correction in craniofacial patients from birth to adulthood

Treatment strategies for total skeletal correction in craniofacial patients from birth to adulthood

Clinical Applications of Orthodontic Microimplant Anchorage in Craniofacial Patients

Microimplant anchors, also known as temporary anchorage devices, mini- and micro-screws, have been used to enhance orthodontic anchorage for difficult tooth movements. Here, the authors describe how microimplants can be used to help treat craniofacial patients by supporting distraction osteogenesis procedures, maxillary protraction procedures, cleft segment expansion and stabilization, and tooth movement into narrow alveolar cleft sites. While most craniofacial patients are treated without microimplants, it would be worthwhile to identify which cases could benefit from microimplant anchorage. As an adjunct to orthodontic treatment, the microimplant offers a potential method for solving troublesome orthodontic and surgical problems such as guiding distraction procedures with orthodontics when primary teeth are exfoliating, addressing residual maxillary cants after vertical distraction osteogenesis of a ramus, stabilizing an edentulous premaxilla, and moving teeth into atrophic alveolar ridges. These cases are presented to open a dialogue on their possible uses in craniofacial patients.

Oral Health-Related Quality of Life of Children with Craniofacial Conditions

Evaluation of the Dutch Child Oral Health Impact Profile (COHIP), assessing the level of concordance between parents and children. The internal consistency and the predictive validity of the COHIP for self-reported general health were examined. Sample size was 35 pairs of parents and children age 11 to 14 with craniofacial conditions. Cronbach alphas were calculated and the level of concordance between parents and children was studied using t tests and intraclass correlations. Predictive validity was assessed using Pearson correlations and linear regression analyses. The COHIP and its subscales, except for one, had satisfactory to high Cronbach alphas (.59 to .94). Parents and children did not differ significantly. Correlations between parents and children were high (.62 to .91). Only "Oral symptoms" proved to be a significant predictor of general health, but only in the parent sample. In spite of the high level of concordance found, proxy reports have to be considered complementary to the reports of the children themselves. The Dutch version of the COHIP performs adequately, but could use some further psychometric evaluation and revision. It does not seem advisable to use the subscales separately as predictors in the same regression model, since they are strongly intercorrelated. For use in craniofacial patients, further validation is needed on a larger sample and some items need to be revised or removed. Finally, given the small number of cases, conclusions must be drawn with caution.

Endoscopic-assisted osteotomies for the treatment of craniosynostosis

The development of multidisciplinar units for Craniofacial Surgery has led to better postoperative results and a considerable decrease in morbidity in the treatment of complex craniofacial patients. Standard correction of craniosynostosis involves calvarial remodeling, often considerable blood losses that need to be replaced and lengthy hospital stay. The use of minimally invasive techniques for the correction of some of these malformations are widespread and allows the surgeon to minimize the incidence of complications by means of a decreased surgical time, blood salvage, and shortening of postoperative hospitalization in comparison to conventional craniofacial techniques. Simple and milder craniosynostosis are best approached by endoscopy-assisted osteotomies and render the best results. Extended procedures other than simple suturectomies have been described for more severe patients. Different osteotomies resembling standard fronto-orbital have been developed for the correction, and the use of postoperative cranial orthesis may improve the final cosmetic appearance. Thus, endoscopic-assisted procedures differ from the simple strategy of single suture resection that rendered insufficient results in the past, and different approaches can be tailored to solve these cases in patients in the setting of a case-to-case bases.

Craniofacial surgery for orbital malformations

Paediatric craniofacial surgery is a highly specialised field requiring a multidisciplinary team input. Orbital and ocular involvement is relatively common in craniofacial patients. This is more in syndromic patients. In nonsyndromic patients, orbital involvement is common in frontal plagiocephaly and trigonocephaly. The management of these conditions requires close working between the craniofacial surgeon and ophthalmologist. An outline of the management of these deformities is discussed.

Tolerance of deviations in eye and mouth position

To develop and refine a technique for measuring the tolerance for deviations in facial appearance. A psychophysical method was administered using photocopies of altered facial photographs to measure tolerances for deviations in eye position and mouth angulation and judges' reaction time. Stimulus photos were displayed as Kodachrome slides to 76 individuals grouped by their familiarity with craniofacial anomalies, i.e. dental professionals, orthodontic and craniofacial patients, also a group of normal patients. (1) Tolerance for deviation of facial appearance varies inversely with the magnitude of the physical deviations from normal; (2) tolerance varies directly as a function of assumed familiarity with deviation; (3) response and reaction time varied inversely with the tolerance for facial deviation. Significant differences in tolerance and reaction time were found among the groups and depended on whether an isolated feature was judged or if the feature was judged in the context of the whole face.

Resorbable PLLA-PGA plate and screw fixation in pediatric craniofacial surgery: clinical experience in 1883 patients.

The need to provide rigid bony fixation in the surgical treatment of craniofacial deformities has inspired an on-going evolution of surgical innovations and implants. Because of the young age of many treated craniosynostosis patients and the unique pattern of cranial vault growth, the extensive implantation of metal devices is potentially problematic. The use of resorbable plate and screw devices offers all of the benefits of rigid fixation without many of their potential risks. Since the introduction of resorbable plate and screw devices in 1996, tens of thousands of craniofacial patients have received implants, but long-term results from a large series have yet to be reported. A combined prospective and retrospective analysis was done on 1883 craniosynostosis patients under 2 years of age treated by 12 surgeons from seven different geographic locations over a 5-year period who used the same type of resorbable bone fixation devices (poly-L-lacticpolyglycolic copolymer). Specifically, the incidence of postoperative infection, fixation device failure, occurrence of delayed foreign-body reactions, and the need for reoperation resulting from device-related problems were determined. Technical difficulties and trends in device use were also noted. From this series, significant infectious complications occurred in 0.2 percent, device instability primarily resulting from postoperative trauma occurred in 0.3 percent, and self-limiting local foreign-body reactions occurred in 0.7 percent of the treated patients. The overall reoperation rate attributable to identifiable device-related problems was 0.3 percent. Improved bony stability was gained by using the longest plate geometries/configurations possible and bone grafting any significant gaps across plated areas that were structurally important. The specific types of plates and screws used evolved over the study period from simple plates, meshes, and threaded screws to application-specific plates and threadless push screws whose use varied among the involved surgeons. This report documents the safety and long-term value of the use of resorbable (LactoSorb) plate and screw fixation in pediatric craniofacial surgery in the infant and young child. Device-related complications requiring reoperation occurred in less than 0.5 percent of the implanted patients, which is less frequent than is reported for metallic bone fixation. Resorbable bone fixation for the rapidly growing cranial vault has fewer potential complications than the traditional use of metal plates, screws, and wires.