Craniofacial and extracraniofacial anomalies in craniofacial microsomia: a multicenter study of 755 patients'

PURPOSE: Craniofacial microsomia (CFM) is a congenital malformation of structures derived from the first and second pharyngeal arches leading to underdevelopment of the face. However, besides the craniofacial underdevelopment, extracraniofacial anomalies including cardiac, renal and skeletal malformation have been described. The aim of this study is to analyse a large population of patients with regard to demographics, typical phenotypes including craniofacial and extracraniofacial anomalies, and the correlations between the different variables of this condition. MATERIAL AND METHODS: A retrospective study was conducted in patients diagnosed with CFM with available clinical and/or radiographic images. All charts were reviewed for information on demographic, radiographic and diagnostic criteria. The presence of cleft lip/palate and extracraniofacial anomalies were noted. Pearson correlation tests and principal component analysis was performed on the phenotypic variables. RESULTS: A total of 755 patients were included. The male-to-female ratio and right-to-left ratio were both 1.2:1. A correlation was found among Pruzansky-Kaban, orbit and soft tissue. Similar correlations were found between ear and nerve. There was no strong correlation between phenotype and extracraniofacial anomalies. Nevertheless, extracraniofacial anomalies were more frequently seen than in the 'normal' population. Patients with bilateral involvement had a more severe phenotype and a higher incidence of extracraniofacial and cleft lip/palate. CONCLUSION: Outcomes were similar to those of other smaller cohorts. Structures derived from the first pharyngeal arch and the second pharyngeal arch were correlated with degree of severity. Extracraniofacial anomalies were positively correlated with CFM. The findings show that bilaterally affected patients are more severely affected and should be approached more comprehensively.

Electronic Follow-Up of Developing World Cleft Patients: A Digital Dream?

OBJECTIVE: To identify potential access to telemedicine follow-up of children with clefts operated on a humanitarian mission. METHODS: A cross-sectional study of parents of children presenting to a humanitarian cleft lip and palate mission in a Provincial Hospital in the Philippines. A purpose designed questionnaire was used to assess access to electronic and digital resources that could be used to aid follow-up. Forty-five (N = 45) parents of children having primary cleft lip and or palate surgery participated. There were no interventions. Access to the Internet was through Parent Perceived Affordability of Internet Access and Parent Owned Devices. RESULTS: Thirty-one (N = 31) respondents were female. There was 93% mobile phone ownership. The mean distance traveled to the clinic was 187 km. Majority (56%) were fluent in English. Thirty-one percent accessed the Internet daily. Sixteen percent reported use of e-mail. Fifty-one percent accessed the Internet on a mobile device, and short message service use was the most affordable means of communication. CONCLUSIONS: Due to perceived unaffordability and low levels of access to devices with cameras and the Internet, as well as issues with privacy, we cannot recommend relying on electronic follow-up of patients in the developing world.

High Density Crossbar Arrays with Sub- 15 nm Single Cells via Liftoff Process Only.

Emerging nano-scale technologies are pushing the fabrication boundaries at their limits, for leveraging an even higher density of nano-devices towards reaching 4F(2)/cell footprint in 3D arrays. Here, we study the liftoff process limits to achieve extreme dense nanowires while ensuring preservation of thin film quality. The proposed method is optimized for attaining a multiple layer fabrication to reliably achieve 3D nano-device stacks of 32 × 32 nanowire arrays across 6-inch wafer, using electron beam lithography at 100 kV and polymethyl methacrylate (PMMA) resist at different thicknesses. The resist thickness and its geometric profile after development were identified to be the major limiting factors, and suggestions for addressing these issues are provided. Multiple layers were successfully achieved to fabricate arrays of 1 Ki cells that have sub- 15 nm nanowires distant by 28 nm across 6-inch wafer.

Splint-assisted disc plication surgery.

Chronic disc displacement may lead to long-term pain. Temporomandibular joint surgery is reserved for those patients whose symptoms remain severe despite conservative treatment. We looked at the of effect of modified meniscopexy on patients with chronic disc displacement without reduction who did not respond to non-surgical pain management treatment. In this retrospective study a total of 59 joints was treated and all patients except one underwent splint assisted bilateral meniscopexy: this patient had splint assisted unilateral meniscopexy. At the time of presentation and following treatment all patients underwent clinical examination and were required to complete a pain and functional questionnaire. All patients reported improvement following treatment.

Esthetic and functional rehabilitation in patients with cleft lip and palate.

Oral rehabilitation of missing teeth in cleft patients has acceptable success rates. A two-stage approach is indicated; however, timing of implant placement in the grafted maxilla varies within existing protocols. This case highlights successful implant osseointegration and esthetic oral rehabilitation following placement of two implants at 5 months after maxillary grafting (alveolar bone grafting) with a corticocancellous block obtained from the iliac crest. A 31-year-old male patient had already undergone repair of his bilateral cleft lip and soft palate according to established guidelines for cleft patients. Initial closure of his alveolar clefts and further correction of the maxillary hypoplasia with a bi-maxillary osteotomy were completed in 2002. However, bone resorption due to infection in 2003 necessitated removal of all maxillary incisors. The patient was not satisfied with the removable partial denture provided. In 2007, he did undergo anterior maxillary augmentation under general anesthesia, and 5 months later two implants were placed. A 3-unit bridge did replace functional and esthetic demands. Postoperative recovery was uneventful, and overall bone loss, and oral health remain within standards 28 months following implant placement. Optimal outcome is achievable when replacing missing teeth in cleft patients when timing does not exceed approximately a 6-month interval from bone grafting to implant placement. This article demonstrates that overall esthetic and functional rehabilitation is feasible in cleft lip and palate patients. In this patient, overall oral treatment was achieved with an implant prosthesis.

Segmental maxillary distraction with a novel device for closure of a wide alveolar cleft.

Treatment of a wide alveolar cleft with initial application of segmental distraction osteogenesis is reported, in order to minimise cleft size prior to secondary alveolar bone grafting. The lesser maxillary segment was mobilised with osteotomy at Le Fort I level and, a novel distractor, facilitated horizontal movement of the dental/alveolar segment along the curvature of the maxillary dental arch. Following a latency period of 4 days distraction was applied for 7 days at a rate of 0.5 mm twice daily. Radiographic, ultrasonographic and clinical assessment revealed new bone and soft tissue formation 8 weeks after completion of the distraction phase. Overall the maxillary segment did move minimising the width of the cleft, which allowed successful closure with a secondary alveolar bone graft.

The surgical management of Treacher Collins syndrome.

Treacher Collins syndrome (TCS), mandibulofacial dysostosis, or Franceschetti-Zwahlen-Klein syndrome, is a rare genetic disorder characterised by dysgenesis of the hard and soft tissues of the first and second branchial arches. Early operations focus on maintaining the airway, protecting the eyes, and supporting auditory neurological development. Later operations include staged reconstruction of the mouth, face, and external ear. Bimaxillary surgery can improve the maxillomandibular facial projection, but correction of malar, orbital rim, and temporal defects may be more difficult. We present a clinical review of the syndrome with a chronological approach to the operations.

The role of free flap reconstruction in paediatric caustic burns.

Ingestion of caustic soda can cause severe scarring of the oral cavity and the surrounding soft tissues. Free flap reconstruction for burns in the oral cavity has been described as a viable option in adults, but to the best of our knowledge has not been reported in children. We describe cases of successful microvascular reconstruction for burns caused by caustic soda in the oral cavity in children.

Orbital fractures in children.

In children, differences in the properties and proportions of bone in the craniofacial skeleton and the lack of development of the paranasal sinuses result in orbital fractures that present differently from those in adults. Facial growth may be disturbed by such injuries and also by surgical intervention, which should therefore be as conservative as possible. However, urgent operation is needed to prevent irreversible changes when fractures of the orbital floor involve entrapped muscle. We present an approach to such injuries.

The effect of the new "24 hour alcohol licensing law" on the incidence of facial trauma in London.

On 24 November 2005 the new 2003 Licensing Act was implemented. It permits licensed premises to close at different times under English and Welsh law, rather than at 2300h as under the previous law. The aim of this study was to assess whether head and neck trauma secondary to alcohol-associated assaults had increased, decreased, or stayed the same since the introduction of the act. Data were collected from the Accident and Emergency Department, University College Hospital, attendance databases for two six-month periods: 24 November 2004 to 30 April 2005, and 24 November 2005 to 30 April 2006. There were 1102 attendances for head and neck trauma secondary to alcohol-associated assaults during the six months before the introduction of the 2003 Licensing Act and 730 such attendances during the similar period after the introduction of the law, with fewer cases in each corresponding month during the later period. There were more cases at weekends than on weekdays during both periods. There were fewer cases but more at weekends in 2005-6 than in 2004-5 (423, 58% compared with 584, 53%, respectively). Neither rainfall nor temperature had any influence on the results. The 2003 licensing Act seems to have reduced the number of attendances at the A&E department for head and neck trauma secondary to alcohol associated assaults.

Bruxism secondary to brain injury treated with botulinum toxin-A: a case report.

We report a successful treatment of bruxism in a patient with anoxic brain injury using botulinum toxin-A (BTX-A). On examination the mouth opening was 0 mm, no feeding was possible through the mouth. Botulinum toxin was injected into the masseter and temporalis; great improvement in trismus and bruxism was noted after 3 weeks. One further treatment improved the mouth opening on the following week and the patient was discharged from our care to be reviewed when required.

The oral implications of caustic soda ingestion in children.

The morbidity related to caustic soda (sodium hydroxide) ingestion is well described in the literature. The majority of publications have concentrated on the effects to the trachea and gastrointestinal tract, with little reference to the oral and peri-oral areas. Accidental ingestion of sodium hydroxide-containing substances is fortunately rare; however the consequences can be devastating. Three cases of children who drank caustic substances are described. Treatment included fitting splints, injecting steroids, local surgical procedures, and the use of dynamic appliances to maintain mouth opening. Despite these interventions, all patients developed severe scarring, resulting in stenosis of the oral musculature and extra-articular ankylosis. There is perhaps a role for further investigation of early use of antiproliferative agents to prevent scarring, more aggressive surgery, and long-term physiotherapy appliance use. These patients require lifelong follow-up.