New Technologies in Rhinoplasty: A Comprehensive Workflow for Computer-assisted Planning and Execution.

Rhinoplasty in facial cleft patients is among the most challenging types of reconstructive facial surgery due to its variability. Advances in 3-dimensional imaging enable improved preoperative assessment in rhinoplasty. In complex cases with bony support irregularities and asymmetry, it is rational to initiate planning with reconstruction of the aberrant substructure (ie, "bottom-up" planning) rather than starting the surgical design with soft-tissue morphing. We present a new comprehensive workflow in which novel advanced technologies are implemented to perform "bottom-up" computer-assisted planning and execution in complex rhinoplasty cases. This workflow enables meticulous planning, use of grafting templates, and 3-dimensional-guided osteotomies with integration of piezotome and intraoperative navigation. Previous reports separately discuss some of these innovations. However, greater benefit lies in the combination of these techniques, with emphasis on preoperative computer analysis, virtual planning, and transfer to the operation theater. Surgeons are seeking new ways to enhance minimally invasive approaches and to obtain predictable and favorable clinical results. The presently introduced workflow allows clinicians to plan complex cases in a simple, effective, and safe manner, with the combination of different techniques to produce consistent results.

The Smith-Lemli-Opitz syndrome and dentofacial anomalies diagnostic: Case reports and literature review.

OBJECTIVE: Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive disorder. It is due to a deficiency of 7-dehydrocholesterol reductase (DHCR7) that catalyses the reduction of 7-dehydrocholesterol (7-DHC) to cholesterol. The aim of this review is to gather all information, concerning diagnostic characteristics of this syndrome, with an emphasis on intraoral symptom presentation. MATERIALS AND METHODS: We conducted a review of the literature, including articles between 1964 and 2017. Data was collected regarding the clinical diagnosis, pathophysiology and treatment of SLOS patients. Moreover, two clinical cases are described, illustrating the oral and facial anomalies of SLOS patients, at the regional university hospital of Lille, France. DISCUSSION: Low cholesterol levels provoke a broad spectrum of clinical presentations, from mild to lethal forms. They can cause mental retardation, growth deficiency and congenital malformations. The SLOS features are often present at birth. Moreover, all the patients have facial anomalies. The dento-maxillofacial symptoms consist of crowded teeth, widely spaced incisors, oligodontia, polydontia, premature tooth eruption, enamel hypoplasia, a bifid uvula, broad alveolar ridges, bifid tongue, and Pierre-Robin syndrome symptoms (glossoptosis, retrognathia and cleft palate). These symptoms are warning signs and should increase the awareness of clinicians. CONCLUSIONS: All healthcare professionals can contribute to the SLOS patient diagnostics. The dento-maxillofacial anomalies, illustrated by two case reports, could help to detect undiagnosed patients. An early detection might improve the outcome of these patients, as cholesterol supplementation can improve symptoms. This study can benefit orthodontists by enabling them to recognize the clinical signs of SLOS in order to refer these young patients to a specialist if the diagnosis has not been established.

Fetal dental panorama on three-dimensional ultrasound imaging of cleft lip and palate and other facial anomalies.

OBJECTIVES: Craniofacial deformities have a high psychosocial impact. The aim of this paper is to improve obstetric ultrasonography and prenatal detection of facial anomalies by providing a new fetal dental panorama. MATERIAL AND METHODS: The present study describes a new modality to visualize the fetal tooth germs and an easy step-by-step diagnostic approach. Image acquisition was performed between 23 and 32 weeks of gestation using a Voluson E10 GE ultrasound machine with an RM6C transducer (GE Medical Systems, Zipf, Austria). Reconstruction was performed using Omniview from the axial image. Volume contrast imaging (VCI) was used with a thickness of 20 mm, and a render mode that combined "Rx mode" and "surface texture." RESULTS: The resulting imaging allows a more precise visualization of the fetal dental arch and can be obtained between 14 and 28 weeks of gestation. The presence of dental anomalies can be a clue for the diagnosis of various congenital defects, in particular conditions with a shortage of other physical abnormalities, such as ectodermal dysplasia and Binder syndrome. CONCLUSIONS: The creation of a precise fetal dental panorama allows an improved detection of facial deformities. CLINICAL RELEVANCE: With the current paper, we want to increase prenatal diagnostics facial anomalies, and help to establish a tailored multidisciplinary treatment plan. This paper should be of interest to readers who are currently treating patients with craniofacial malformations and readers who are performing diagnostic prenatal sonography.

Workflow for Pediatric Midface and Orbital Reconstruction With a Patient-Specific Autogenous Bone Graft.

Extensive midface and orbital reconstruction is challenging, especially in a pediatric population. A 13-year-old patient was diagnosed with fibrous dysplasia of the midface, which required complete resection of the zygomatic bone and orbital floor. The authors present a complete midface and orbital reconstruction, using a patient-specific autologous bone graft, in a growing pediatric patient. Postoperative analysis showed a symmetric orbital floor and good clinical outcome. After 12 months of follow up, no bone resorption was observed. Patient-specific autologous bone grafts have a clear role in the treatment algorithm of patients with large skull bone defects, with emphasis on the pediatric patient. The advantages can be even greater in a pediatric population, because they can benefit more from the minimal invasive approach. Furthermore, this technique allows single-stage complex reconstructive surgeries with a decreased operating time.

Postoperative Respiratory Complications After Cleft Palate Closure in Patients With Pierre Robin Sequence: Operative Considerations.

BACKGROUND: In cleft palate surgery, there is currently no consensus on the management of patients with Pierre Robin Sequence (PRS). The authors aimed to evaluate the treatment strategy of cleft palate in our centers, with emphasis on patients with PRS, as the authors noted some patients with severe respiratory distress. Moreover, the authors aimed to investigate the prevalence of postoperative respiratory complications, using a modified-Furlow palatoplasty in combination with intravelar veloplasty in both patients with PRS and patients with non-PRS. METHODS: The authors retrospectively identified all consecutive patients, both PRS and non-PRS, who underwent palate repair between January 1, 2012 and December 15, 2014 at 2 cooperating cleft centers (Bruges, Belgium; Budapest, Hungary). The treatment modality was uniform and performed by the same 2 surgeons. RESULTS: In 92 consecutive patients, 4 patients experienced respiratory distress after palate repair. The female-to-male ratio was 1:1. The mean age at surgery in these 4 patients was 15 months (range 13-19 months). Fifteen percent (2/13) of patients with PRS experienced respiratory distress in comparison to 3% (2/79) of non-PRS (χ = 4.43; P = 0.035). CONCLUSIONS: This is the first report of postoperative respiratory difficulties, while using a modified-Furlow palatoplasty in combination with intravelar veloplasty. In the present author's experience, the authors suggest to perform a 2-stage closure of the cleft palate in patients with PRS and to do so at a later age, when the palatal tissues and airway structures are more mature. Moreover, patients with PRS should be monitored closely, as they can present with different degrees of respiratory distress after palatoplasty.

Erectile implants in female-to-male transsexuals: our experience in 129 patients.

BACKGROUND: The combination of a neourethra and erection prosthesis in a single neophallus in the female-to-male transsexual remains a challenge. No good data are available on this subject. OBJECTIVE: To report the outcome in 129 female-to-male transsexuals with a neophallus after the implantation of an erectile prosthesis. DESIGN, SETTING, AND PARTICIPANTS: From March 1996 until October 2007, 129 female-to-male transsexuals with a neophallus underwent the implantation of an erectile prosthesis. The mean follow-up was 30.2 mo (range: 0-132 mo). INTERVENTION: A Dynaflex prosthesis was implanted initially in 9 patients, a three-piece hydraulic device (AMS CX or AMS CXM) in 50 patients, and a CX Inhibizone, Ambicor, and Coloplast/Mentor prosthesis in 17, 47, and 6 patients, respectively. MEASUREMENTS: Data on outcome in these patients were retrospectively evaluated. RESULTS AND LIMITATIONS: Of 129 patients, 76 patients (58.9%) still have their original implant in place. Fifty-three patients (41.1%) needed to undergo either removal or revision of the prosthesis due to infection, erosion, dysfunction, or leak. Forty-one patients underwent a replacement of the prosthesis, nine needed a second revision, five needed a third revision, and one patient needed a fourth revision of prosthesis. Malposition of prosthesis was corrected by surgical repositioning so that removal or revision could be avoided. Of 185 prostheses used in 129 patients, 108 (58.4%) still remain in place, with a total infection rate of 11.9%, a total protrusion rate of 8.1%, a total prosthesis leak rate of 9.2%, a total dysfunction rate of 13%, and a total malposition rate of 14.6%. The period of follow-up in the more recent types of prostheses (Ambicor, Coloplast/Mentor) is much shorter; therefore, comparison with earlier types is difficult to make. CONCLUSIONS: Despite high complication rates, implantation of a hydraulic erectile prosthesis remains the best option for achieving the possibility of sexual intercourse in female-to-male transsexuals.