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Agnathia-otocephaly complex (AOC) is a rare malformation complex of the first pharyngeal arch that is characterized by agnathia/dysgnathia, microstomia, aglossia/hypoglossia and variable displacement of the ears. Only 11 post-infancy patients with severe AOC have been described in the literature, and the incidence of this malformation complex is estimated to be 1 per 70,000 births. In this brief clinical study, the authors describe the case of an 18-year-old female diagnosed with AOC who underwent a 3-step mandibular distraction protocol with an external distraction device. The surgical protocol the authors used was unique in that we first placed a tissue expander in the submental area to enlarge the skin envelope in an effort to mitigate skeletal relapse from soft tissue forces. Furthermore, the way in which the authors slowed the activation of the distraction device to allow for soft tissue healing behind the pins was a novel component of the patient's treatment. The 3-step mandibular distraction protocol the authors present in this study increased the length of the mandible by 20 mm, and nearly doubled the size of the patient's mandible from an initial volume of 3.62 cm to a post-operative volume of 6.89 cm. Future surgeries will aim to improve the function of our patient's expanded mandible. Most important of all, the surgical treatment authors are presenting led to a significant improvement in our patient's physical appearance and 3d quality of life.

Background Distraction osteogenesis (DO) is a biologic process of new bone formation between the surfaces of bone segments that are gradually separated by incremental traction. It consists of 4 primary phases, namely, corticotomy and device placement, a latency period, active distraction, and consolidation. The objectives of the current study were to review DO as it applies to maxillomandibular defects and to share our clinical experience in the cases we have done. Methods A clinical narrative review of the literature was performed to evaluate the use and efficacy of maxillomandibular osteogenesis in maxillomandibular defects. A systematic search of the literature was performed using PubMed, with special interest in the history of DO and its application in dentistry and maxillofacial surgery. medical subject headings terms included surgical procedures, osteogenesis, distraction, and orthodontics. Two cases of maxillomandibular DO managed at the Aminu Kano Teaching Hospital (AKTH), Nigeria, were reported and discussed. Results Articles involving maxillary and midface distractions, bilateral distraction for airway obstruction, and distraction for hemifacial microstomia were all reviewed. In the first case reported, a unidirectional distractor was used to achieve simultaneous mandibular lengthening and maxillary occlusal correction. Gains of 10 mm in mandibular ramal height and 23 mm in corpus length were achieved in the second reported case, using a bidirectional distractor. The literature search revealed no previous Nigerian reports of maxillomandibular DO. Conclusions The DO is a viable and available treatment option for reconstructing maxillomandibular discrepancies and accompanying soft and hard tissue deficiencies.

Freeman-Sheldon syndrome is a congenital disorder that has been suggested to be associated with malignant hyperthermia. Clinical features of the Freeman-Sheldon syndrome include flexion contractures and characteristic facial features, including microstomia and a whistling shape to the lips. We report a case of malignant hyperthermia in a 3-year-old girl with microstomia but no other features of Freeman-Sheldon syndrome. The purpose of this report was to review the diagnosis and treatment of malignant hyperthermia as craniofacial surgeons have an increased exposure to this rare and potentially fatal condition.

Microstomia reconstruction due to the presence a blunted oral commissure is a challenging task because it requires the restoration of intricately balanced distinct layers of tissues: the oral mucosa, the orbicular muscle, the vermilion border, and the perioral skin. The reliability of commissural reconstruction depends on 2 factors: the first one is breaking the contraction vectors causing blunting of the commissure and the second one is restoring the integrity of the oral sphincter. We have used local skin, vermilion border-muscle, and mucosa flaps designed in an asterisk pattern to break the contraction vectors and have paid certain attention to the restoration of the sphincter function of the circular muscle fibers. Our results have shown that, with the use of our asterisk design, a new commissure aesthetically comparable to the natural one can be created with the reestablishment of reliable oral competence.

Despite major advances in the area of burn management, burn injury continues to be a leading cause of pediatric mortality and morbidity. Facial burns in particular are devastating to the affected child and result in numerous physical and psychosocial sequelae. Although many of the principles of adult burn management can be applied to a pediatric patient with facial burns, the surgeon must be cognizant of several important differences. Facial burns and subsequent scar formation can drastically affect the growth potential of a child's face. Structures such as the nose and teeth may become deformed due to abnormal external forces caused by contractures. Serious complications such as occlusion amblyopia and microstomia must be anticipated and urgently addressed to avert permanent consequences, whereas other reconstructive procedures can be delayed until scar maturation occurs. Furthermore, because young children are actively developing the concept of self, severe facial burns can alter a child's sense of identity and place the child at high risk for future emotional and psychologic disturbances. Surgical reconstruction of burn wounds should proceed only after thorough planning and may involve a variety of skin graft, flap, and tissue expansion techniques. The most favorable outcome is achieved when facial resurfacing is performed with respect to the aesthetic units of the face. Children with facial burns remain a considerable challenge to their caregivers, and these patients require long-term care by a multidisciplinary team of physicians and therapists to optimize functional, cosmetic, and psychosocial outcomes.

Despite significant burn treatment advances, modern multidisciplinary care, and improved survival after burns, facial burn scars remain clinically challenging. Achieving a successful reconstruction requires a comprehensive approach, entailing many advanced techniques with an emphasis on preserving function and balancing intricate aesthetic requirements. Pediatric facial burns present the same reconstructive challenges seen in adults, with additional developmental and psychologic concerns. In this paper, we describe the basic principals of facial burn care in the pediatric burn population, with a specific focus on lower-eyelid burn ectropion and oral commissure burn scar contracture leading to microstomia. Several cases are demonstrated.

The author has used various designs of single-stage resorbable distractors to lengthen the mandible in 100 patients aged 7 days to 16 years (mean, 4.24 y; 49 girls, 51 boys) with predictable results and minimal morbidity since 2002. The range of distraction was 15 to 30 mm (mean, 25.4 mm). Indications for surgery included mandibular hypoplasia associated with Pierre Robin sequence, craniofacial microsomia, Treacher Collins, and Nagers syndrome. The history of the development of resorbable polymers for use in craniofacial surgery and the evolution of distraction osteogenesis are reviewed. The melding of these 2 innovative technologies has led to the development of a new class of single-stage resorbable devices. These devices are quite different from the titanium distraction devices that have been used in clinical practice for more than 15 years. Through continued clinical application, the surgical methodology for resorbable distraction has been refined and simplified, although still-evolving continued experience with resorbable distraction has greatly decreased operative time and improved results.

Distraction osteogenesis has been advocated for treatment of the mandibular deformity in patients with hemifacial microsomia (HFM). During the active phase, the force of distraction pushes the distal segment of the mandible down, creating a distraction gap. Because of the abnormal temporomandibular joint anatomy in HFM patients, the proximal segment may not seat in the glenoid fossa and thus may be displaced with distraction. The purpose of this study was to determine the vector(s) of proximal segment movement during mandibular distraction using a semiburied device. Two investigators traced the immediate pre-and postdistraction panoramic radiographs of 12 HFM patients (mean age at operation = 8.4 years, mean distraction = 28 mm) who had mandibular distraction with a semiburied device. Radiographic analysis, based on a vertical maxillary reference line, measured change in condylar position with angular and linear measurements. Inter-rater reliability for the tracing and analysis was shown with a correlation coefficient between 0.89 and 0.99 for all measures. Based on the angular and linear measurements, 10 of the 12 patients had superior movement of the proximal segment with distraction. Sagittal movement of the proximal segment could not be judged adequately. This study was based on measurements made on panoramic radiographs. Direct measurements could not be made; thus, it was not possible to estimate proximal segment movement in millimeters or as a percentage of total movement. Further studies to document proximal segment movement using computed tomography scans may provide more quantitative data.

Craniofacial microsomia (CFM) involves asymmetric hypoplasia and dysmorphogenesis of the facial skeleton. Certain aspects of CFM may be treated by surgical osteotomy and distraction osteogenesis (DO). Mandibular osteotomy places the inferior alveolar nerve at risk. The aim of this study was to investigate radiological landmark relationships to the anatomy of the inferior alveolar nerve in CFM. Application of this understanding will aid intraoperative protection of the inferior alveolar nerve. Six subjects with similar presentations of hemifacial microsomia were selected. Three-dimensional reconstruction CT images was used to locate bony structures that held important relationships with the inferior alveolar nerve. Measurements (of the normal and microsomic sides) were made between fixed landmarks: mandibular notch, mandibular foramen, condyle, back of second and third molar tooth, and mental foramen. The unaffected sides acted as controls. The distance between the normal and the microsomic sides from condyle to mandibular foramen was significantly different. The sizes of condyles differed significantly between the normal and microsomic sides. Most of the remainder of the vertical distance of the ramus and most of the horizontal distance of the body were similar. In conclusion, the inferior alveolar nerve should be found in very similar locations on both normal and microsomic sides in low-grade hemifacial microsomic patients.

Treatment of microstomia, whether congenital or acquired, has long challenged the ingenuity of surgeons. In all instances, the challenge remains the ability to preserve function and provide and maintain reasonable aesthetics. The following case report presents 2 different cases of surgical correction of microstomia, resulting from flap reconstructions after perioral tumor surgery. We developed a technique that uses pericommissural mucomuscular advancement flap, "a simple fishtail flap," and used this to correct deformed oral commissures in an attempt to elongate the oral aperture in a functional and aesthetically acceptable manner without recurrence. Results have shown that, with the use of our fishtail design, we can restore the unique architecture of the oral commissure with the postoperative intercommissural distance improved by more than 10 mm and the interincisal width enlarged by approximately 5 mm. Our solution was functional and aesthetically acceptable, as well as simpler and cheaper, in comparison with the traditional surgical methods that are usually expensive, complex, and less practical. We believe that, in cases in which either the contracture is mild or a commissurotomy can be expected to increase the width and the general size of the oral aperture, this relatively conservative technique, if judiciously used, can be performed with ease and safety with good results.

Facial-nerve paralysis is seldom seen and may occur because of a broad spectrum of causes. The most commonly seen cause of facial paralysis is the Bell palsy; iatrogenic causes and tumors are relatively rare. Facial asymmetry, drooling, garbled speech, and difficulty in feeding: all adversely affect the psychosocial conditions of the patients. Fascial and tendon sling procedures may be performed for the static treatment of the unilateral permanent facial paralysis. These techniques are used both for the correction of the asymmetry of the face, especially by providing static support for the corner of the mouth, and to prevent drooling. Microstomia after a sling procedure is not a previously observed complication in the literature. A patient is presented with the surgical management of the complication of microstomia that had risen because of a static treatment of his unilateral facial paralysis via a tendon that passes circularly through his orbicularis oris muscle. Oral rehabilitation thereafter was maintained with the support of dental implants and fixed prosthodontics. The most efficient treatment protocol was decided with an interdisciplinary consultation of the oral and maxillofacial surgeon, the plastic surgeon, and the prosthodontist.

Microstomia is rarely seen in pediatric patients, but usually results from burns, trauma, or caustic ingestions. There have been multiple studies reporting various techniques for oral commissure reconstruction, but few reports in infants. The authors present another modification of previous techniques of microstomia repair performed in a 10-week-old infant using multiple z-plasties and bilateral mucosal rhomboid flaps.

Distraction osteogenesis has been shown to be an effective method of lengthening and augmenting endochondral bone. It has also been applied effectively in the reconstruction of the membranous bones of the craniofacial skeleton. With the accumulation of clinical experience in mandibular distraction, the differences between endochondral and membranous bone distraction have become apparent, especially in the limitations of uniplanar distraction for the three-dimensional reconstruction of the deficient mandible. Distraction of the mandible in a single plane cannot satisfy fully the functional and structural requirements of the patient with malocclusion as well as deficiency of the skeletal and soft tissue. This study reports the development and clinical use of a multiplanar mandibular distraction device with the ability to achieve linear distraction (Z-plane or sagittal), angular distraction (Y-plane or vertical), and transverse distraction (X-plane or coronal). The device contains two independent gear arrangements attached to two arms that extend from the central unit. Therefore, the trajectory of the regenerated bone may be changed during the distraction process. The device also allows manipulation of the various planes of movement independent of each other. Furthermore, the rotational points for the multiplanar distraction devices are located at a single point; therefore only a single osteotomy and two pin sites are required. The multiplanar distraction device allows the surgeon to customize and contour the dimensions of the distraction process by controlling the trajectory of the translation of the regenerated bone.

Eighteen adult patients with hemifacial microsomia were treated with a combination of skeletal and augmentation surgery. Three typical cases are presented. In principle, skeletal and augmentation surgery have recently been performed in combination in a single stage. Groin flaps and scapular or scapular ostocutaneous flaps have mainly been employed for augmentation surgery.