Algorithm to the treatment of Crouzon syndrome.

Clinical reports regarding the entire surgical sequence in Crouzon syndrome from the neonatal period to the adult age are rare. The purpose of this study is to trace an operative algorithm with a long term follow up in a homogenous group of patients affected by Crouzon syndrome. A retrospective review was conducted for all patients affected by Crouzon syndrome who completed the entire surgical sequence. 7 Crouzon patients (4 females, 3 males) completed the entire surgical sequence at different ages: fronto-orbital advancement (0.9 years), LF III distraction osteogenesis (11.5 years) and orthognathic surgery (18 years). The mean age at the last follow up was 19.3 years; normalization of the face was obtained in all cases with improvement of the respiratory problems. After orthognathic surgery, all patients had stable occlusion. A one-year postoperative CBCT scan revealed almost complete ossification of all osteotomy sites. Frontoorbital advancement and modified Le Fort III distraction osteogenesis are reliable surgical procedures. SARME and conventional orthognathic surgery with reductive genioplasty and fat grafting are performed at the end of the surgical sequence to enhance facial aesthetics.

Three-Dimensional Analysis of Long-Term Stability After Bilateral Sagittal Split Ramus Osteotomy Fixed With a Single Miniplate With 4 Monocortical Screws and 1 Bicortical Screw: A Retrospective 2-Center Study.

PURPOSE: This study evaluated the long-term stability of bilateral sagittal split ramus osteotomy fixed with a single miniplate with 4 monocortical screws and 1 bicortical screw (hybrid technique [HT]) using 3-dimensional (3D) analysis and an objective measuring tool, cone-beam computed tomography (CBCT). MATERIALS AND METHODS: Sixty-four patients who underwent bimaxillary surgery with mandibular advancement fixed with the HT were selected from 2 different institutions and enrolled in this retrospective study. All patients underwent CBCT preoperatively, 1 month after surgery, and 12 months after surgery. To estimate the long-term stability of the HT, volumetric comparisons were performed using the following measurements: distance between the gonion and the B point in the sagittal plane; distance between the right and left gonion transversally; and the angle of the line connecting the mandibular notch and the gonion and the line connecting the gonion and the B point vertically. RESULTS: Statistical analysis showed no relevant relapse (<1 mm or <1°) when using the HT. However, a positive correlation between the amount of advancement and the amount of postoperative relapse was observed. CONCLUSION: The HT produces stable postoperative 3D results after 12 months.

Full Facial Feminization Surgery: Patient Satisfaction Assessment Based on 180 Procedures Involving 33 Consecutive Patients.

BACKGROUND: Gender dysphoria refers to the discomfort and distress that arise from a discrepancy between a person's gender identity and sex assigned at birth. The treatment plan for gender dysphoria varies and can include psychotherapy, hormone treatment, and gender reassignment surgery, which is, in part, an irreversible change of sexual identity. Procedures for transformation to the female sex include facial feminization surgery, vaginoplasty, clitoroplasty, and breast augmentation. Facial feminization surgery can include forehead remodeling, rhinoplasty, mentoplasty, thyroid chondroplasty, and voice alteration procedures. Described here is an assessment of patient satisfaction after facial feminization surgery, including outcome measurements after forehead slippage and chin remodeling. METHODS: Thirty-three patients between 19 and 40 years of age were referred for facial feminization surgery between January of 2003 and December of 2013, for a total of 180 procedures. Surgical outcome was analyzed both subjectively through questionnaires administered to patients and objectively by serial photographs. RESULTS: Most facial feminization surgery procedures can be safely completed in 6 months, barring complications. All patients showed excellent cosmetic results and were satisfied with their procedures. Both frontal and profile views achieved a loss of masculine features. CONCLUSIONS: Patient satisfaction after facial feminization surgery is high. The reduction of gender dysphoria has psychological and social benefits and significantly affects patient outcome. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Giant trigeminal schwannoma with parapharyngeal extension: report of a case.

The authors present their experience in the treatment of a giant trigeminal schwannoma with wide extension in the parapharyngeal space using a combination of the orbito-zygomatic and the transcervical-transmandibular approaches. The clinical and radiological findings, advantages of surgical approach and clinical outcome will be discussed.

Malignant peripheral nerve sheath tumor of the vagus nerve in a teenager with the neurofibromatosis 1 gene mutation: a case report.

Malignant peripheral nerve sheath tumors account for approximately 5% to 10% of all soft tissue sarcomas in which 25% to 50% are diagnosed in patients with neurofibromatosis 1 (NF1). Tumors are often located in the proximal portion of the upper and lower extremities and trunk, whereas cervical vagus nerve localizations are extremely rare, and the English literature is limited to isolated case reports. Malignant peripheral nerve sheath tumors usually affect adults. However, earlier presentation is described in patients with the NF1 mutation. The authors describe a very rare case of malignant peripheral nerve sheath tumor of the vagus nerve in a teenage patient with NF1 focusing on surgical management of this uncommon pathology and its histopathologic features to underline the importance of differential diagnosis and early treatment of this rare and aggressive tumor.

Huge orbital teratoma with intracranial extension: a case report.

The authors present an unusual case of huge orbital teratoma extended to the cranial fossa in a newborn baby. The clinical features, radiologic findings, and surgical approach are described. Discussion will focus on the surgical technique and on the sparing of the affected eye, comparing the authors approach with other few cases described in literature. Finally, detailed histopathologic finding is provided.

A novel technique for cheek mucosa defect reconstruction using a pedicled buccal fat pad and buccinator myomucosal island flap.

Reconstruction of cheek mucosa defects following tumor resections can be approached with several techniques, depending on size of the defect. Fasciocutaneous and perforators free flaps are widely employed today for such reconstructions. However, small defects or general health of the patient may limit their indications. Furthermore, approaching moderate size defects, some techniques, like temporalis muscle or fascia pedicled flaps, lead to contracture with limitation of mouth opening or trisma, and others, like intraoral local flaps, do not provide enough tissue for the reconstructions. In this work the authors propose, for reconstructing these kind of defects, the use of a buccinator myomucosal island flap and a buccal fat pad pedicled flap association. A case is reported and the surgical technique is explained. This new reconstructive technique can easily be used for reconstructing moderate-sized cheek defects, achieving optimal results: the internal mucosal lining is restored in few weeks without any retraction, contracture, of scars on the face limiting the aesthetic outcome and mouth opening.