Treatment of exophthalmos and strabismus surgery in thyroid-associated orbitopathy.

Endocrine orbitopathy (EO) can have important consequences, such as exophthalmos and restrictive strabismus. A retrospective study was performed of 35 patients with EO who underwent orbital decompression surgery and restrictive strabismus correction. Two surgical techniques for orbital decompression were analyzed: fat decompression by Olivari technique and three-wall bony expansion with fat decompression. Strabismus surgery was performed using adjustable or non-adjustable sutures under topical anaesthesia. Patients were divided into two groups according to the type of intra-orbital decompression performed, and the postoperative values resulting from the different fat decompression techniques were recorded. The preoperative and postoperative mean degrees of exophthalmos were 22.3 and 19.9mm, respectively, for the fat decompression group, and 24.3 and 19.8mm, respectively, for the bony expansion with transpalpebral fat decompression (combined form) group. The difference in residual prism dioptres between adjustable and non-adjustable suture techniques in patients who had previously undergone combined decompression was statistically significant. The management of patients with EO requires a multidisciplinary approach based on the collaboration of maxillofacial surgeons, ophthalmologists, and orthoptists. These results will allow the development of a more adequate strategy for the surgical treatment of restrictive strabismus in EO patients.

Peripheral ameloblastoma: case report.

Ameloblastoma is a benign tumour of the odontogenic tissues which may be aggressive in the involved area with a high rate of recurrence if not adequately removed, and represents 1% of all tumours of the mouth, generally appearing in the bones of the jaw. Although it is fairly rare, tumours with such histopathology have been described in the soft tissue and are named peripheral or extraosseus ameloblastoma. Potential cells of origin are ondontogenic residue of the dental lamina, pluripotent cells in the basal layer of the epithelial mucosa, and pluripotent cells of the minor salivary glands. Seven cases of extraosseus ameloblastoma in the extragengival area, unrelated to dental germs, have been described, six of which in the buccal mucosa and one in the mouth floor. We report a case of extraossous, extragengival ameloblastoma which originated in the subzygomatic area.

Study of the 12q13 region in nonsyndromic cleft lip with or without cleft palate.

The 12q13 region has been suggested as a candidate locus for orofacial cleft by different investigators. In the present study we tested the region for linkage with non syndromic cleft lip with or without cleft palate in a collection of 39 Italian multigenerational families, using microsatellite markers. No evidence of linkage was detected between the marker map and NSCLP under different mode of inheritance nor with a nonparametric method. Formal level of linkage exclusion, were obtained for each point of the map. Genetic heterogeneity and the different impact of the candidate locus among populations could explain conflicting results obtained in different studies.

Solitary fibrous tumor of the orbit: a case report.

Solitary fibrous tumor is a very rare neoplasm affecting the head and neck. We present a case located in the orbit in which clinical, pathological and surgical features are reviewed.

Rationale of treatment in Graves ophthalmopathy.

Graves ophthalmopathy is a chronic and multisystem disorder caused by an autoimmune process, characterized by the presence of antibodies that stimulate a general fibroblastic reaction (thyroid gland and lower extremities), and involves orbital fat tissue and muscles. The clinical findings and therapy for the treatment of the exophthalmos, such as changes in extrinsic eye motility, diplopia, optic nerve involvement, and lid retraction, were analyzed, and the various types of surgical treatment currently available for Graves ophthalmopathy were evaluated. The aim was to choose the best option to treat each case. The surgical techniques were transpalpebral decompression by removal of intraorbital fat, three-wall osseous expansion, and zygomatic osteotomy. Adjunctive procedures were lengthening of the levator muscle of the upper eyelid, lengthening of the retractor of the lower eyelid (if necessary), and surgery of the extrinsic muscles to correct diplopia. All these techniques were useful in treating the disease, which is characterized by chronic evolution and, at times, a "malignant" outcome. A total of 39 orbits were treated using different techniques of decompression and secondary adjunctive procedures. Results were analyzed after a minimum 6-month follow-up. It was evident that surgery greatly reduced the degree of exophthalmos and improved eye motility, diplopia, and visual acuity. Close cooperation among a team of specialists, including an endocrinologist, ophthalmologist, neuroradiologist, surgeon, anesthesiologist, and radiotherapist, is essential to manage and to quantify the postoperative results of this complex disorder. The authors' experience and application of different surgical strategies, as based on clinical data and histopathological classification, are presented.

Saethre-Chotzen syndrome: review of the literature and report of a case.

Saethre-Chotzen syndrome is an autosomal acrocephalosyndactyly syndrome whose gene has been assigned to chromosome 7p (TWIST). A case of a 13-year-old girl with Saethre-Chotzen syndrome (ACS III) is described. The features of the syndrome include: turriplagiocephaly with a cranial circumference of 52 cm, facial asymmetry, low hairline, proptosis, antimongoloid slanting of palpebral fissures, nasal deviation with high bridge, angled ears, scoliosis and torticollis, clinodactyly of the fourth and fifth toes, large halluxes, and neurosensorial hypoacusia. For correction of the deformity, a cranioorbital remodeling was performed. The craniofacial approach with remodeling of the frontal bar and reduction of the turricephaly resulted in a satisfactory morphological and functional outcome, with complete three-dimensional reshaping and remodeling of the frontonasoorbital area.

Dismantling and reassembling of the facial skeleton in tumor surgery of the craniomaxillofacial area. History, surgical anatomy, and notes of surgical technique: Part 1.

In recent years, access osteotomies have been suggested to reach areas of the craniofacial skeleton that hitherto would not have been easily resectable. Some techniques of disassembling of the facial skeleton have been described in the past. In some cases, however, when the bony fragments were not pedicled to the soft tissue, reabsorption was noted. For this reason, new dismantling techniques with adequate blood supply to the bony fragments have been developed. Apart from the maxillo cheek flap, other composite flaps have been described. These flaps may be combined if necessary in selected cases. An overview of the surgical anatomy and surgical strategies will be presented. These reported techniques may be considered a major step forward in the treatment of deeply localized tumors in the craniomaxillofacial area.

Neurofibromatosis of the orbit and skull base.

Von Recklinghausen's disease is characterized by multiple neurofibromas, pigmentations, and pachydermatoceles of the skin depending on the disorder of the neural crest derivative. We report on a 25-year-old patient suffering from neurofibromatosis localized in the cranio-orbital region, whose main problem was a pulsating right exophthalmos caused by herniation of the frontotemporal lobe through a defect of the greater wing of the sphenoid. The lesion was approached through a combined route (i.e., transfrontally and transfacially [upper eyelid incision]). Surgical strategy and associated problems are discussed.

Anatomic and magnetic resonance imaging bases for the naso-maxillo-cheek flap technique.

A transfacial approach to the deep cranio-maxillo-facial areas by the naso-maxillo-cheek flap technique (NMCF) is indicated for the treatment of some bulky tumors of the naso-pharynx. The procedure requires precise preoperative imaging. This study presents the morphologic bases of this surgical access and the reasonable limits of the excision preoperatively determined by magnetic resonance imaging (MRI). 18 facial and skull specimens were submitted to surgical facial dismantling by the NMCF technique according to Curioni's method. The clinical application in a 66-year-old patient suffering from a neuroblastoma of the olfactory nerve extended into the naso-pharynx is presented. Pre- and postoperative MRI correlations were made in transverse, sagittal and frontal acquisitions. Several structures were preserved in the procedure: facial reliefs, inferior orbital rim and orbital floor, posterior wall of the maxillary sinus covering the pterygopalatine fossa, lateral and medial pterygoid plates and pterygopalatine ganglion with its branches, lateral facial neurovascular pedicle, teeth and soft palate. Other structures were sacrificed: arteries and nerves located at the sites of skin and mucosal incision, and at the sites of osteotomies, ie the infraorbital nerve, the distal part of the greater palatine nerve, the nerves supplying the naso-pharynx, the nasal septum and the nasal conchae, nasolacrimal groove and lacrimal canal. The NMCF technique gives wide access to the deep nasal and nasopharyngeal areas. It is essential to preserve the lateral facial neurovascular pedicle to prevent necrosis of the midface structures. Preservation of the bony architecture surrounding the osteotomy sites is of great importance to allow precise final bone reassembly. Preoperative MRI appears of paramount importance to determine the borders of the lesion and the possibility of block resection.

Total facial rehabilitation: the evolving concept of reconstructive surgery.

Total facial rehabilitation requires an understanding of the contributing components of the so-called aging face syndrome. This syndrome may be due to atrophy of the jaws or may result from ablative surgery for cancer. The aging face requires correction of every anatomic component (i.e., facial skeleton, dentoalveolar processes, and soft tissue). The surgical armamentarium includes maxillofacial osteotomies, autogenous bone grafts, biomaterials, internal rigid fixation, and other ancillary procedures. The introduction of osseointegrated implants has allowed the surgeon to use a stable base on which a prosthesis can be built with functional and aesthetic long-lasting results. Last, but not least, repositioning of the facial mask is the "final touch" for these complex reconstructive procedures. The concept of total facial rehabilitation may be considered an evolution of reconstructive surgery. Surgical strategies, new technologies, as well as clinical cases will be presented.

Haemangioma of the zygoma: case report.

Haemangioma of bone in the zygoma is extremely rare. We report two cases presenting with swelling of the zygomatic region. The literature is reviewed and CT, angiographic and MRI findings are discussed.

Craniofacial fibrous dysplasia and Ollier's disease: combined transfrontal and transfacial resection using the nasal-cheek flap.

Fibrous dysplasia and Ollier's disease (skeletal chondromatosis) are bone lesions that result from disorders occurring during ossification. Here we report on a patient suffering from Ollier's disease in whom polyostotic fibrous dysplasia was detected. The main problem for this 20-year-old man was a nasoethmoidorbital cartilaginous mass causing nasal obstruction, nonreducible eye proptosis, and sleep apnea. The lesion was approached by means of a combined route, ie, transfrontally and transfacially (nasal-cheek flap). The possible pathogenetic links existing between the two lesions and the problems involved in surgical treatment are discussed.

The use of the temporalis muscle flap in facial and craniofacial reconstructive surgery. A review of 182 cases.

The authors report on their 16-year experience of reconstruction with the temporalis myofascial flap in 182 cases. All aspects of reconstructive cranio-maxillofacial surgery are covered: trauma, deformities, tumours, TMJ ankylosis, facial paralysis. The temporalis myofascial flap was used both as a single and as a composite flap with cranial bone, coronoid process or skin island. Major complications were not observed. On the basis of their experience, the authors confirm the reliability, versatility and reproducibility of the use of this flap. This is due both to its rich blood supply and to its proximity to the reconstruction site. It is suggested that the use of the temporalis muscle flap should be taken into consideration before deciding on more extensive reconstructive procedures.

Use of the microsystem in craniomaxillofacial surgery: preliminary report.

The last few years have been astonishing technological advances in craniomaxillofacial surgery, with particular regard to the introduction of internal rigid fixation. This technique has allowed craniomaxillofacial surgeons to achieve more precise preoperative planning and use of modern techniques of fixation during surgery. As a result, rigid internal fixation has become popular and currently represents a keystone in maxillofacial surgery for trauma, deformities, orthognathic surgery, as well as reconstruction procedures following tumor resection. We review a 2-year experience with the use of microsystem in 45 patients.

Histomorphological analysis of the effect of rigid fixation on growing sutures in the rabbit.

The effect of internal rigid fixation (IRF) on bone growth was studied in an experimental model set up in the rabbit. The frontonasal suture of the right side was surgically bridged by a microplate. As reference for bone growth, four screws were placed symmetrically in the four bony segments including the frontonasal suture on both sides. The suture development was followed dynamically for 40 days on the basis of the position of the screws established radiographically. The rate of bone formation along the sutural bones was evaluated by means of the tetracycline labelling technique. The structure of the newly formed bone and its degree of mineralization were respectively analysed under polarized light and with microradiography. It was demonstrated that IRF prevents growth of the sutural membrane but not of the osteogenic process; as a consequence the constrained sutures soon undergo synostosis. This fact must be taken into consideration when IRF is employed in children in order to avoid delayed removal of the plate irreversibly stopping the growth of the constrained suture.

Osteosarcoma of the facial bones.

Maxillo-facial osteosarcoma is a rare primary tumor in adults. Between 1980 and 1990, 11 patients were considered; 6 had primary tumors in mandible and 5 in the maxillo-paranasal region. All cases were treated with surgery as the primary modality. Resection was radical in 8 patients and palliative in the other 3. Adjuvant postoperative chemotherapy with adriamycin was administered for 6 months in the 8 patients treated with complete resection. After a median follow-up of 3 years, 7 patients are still alive and 4 died of progressive disease. In the group of patients treated with radical surgery and adjuvant chemotherapy only one died for distant metastases, and 7 are living free of disease. With complete surgical resection long term local tumor control was achieved in all patients. No patient treated with incomplete resection achieved local tumor control with subsequent radiotherapy. The possibility of performing a complete surgical resection of the primary appears to be an essential step to obtain long term local control and survival in maxillo-facial osteosarcoma. Our series is, however, too limited to evaluate the therapeutic benefit of adjuvant chemotherapy.

Haemangioma of the Zygoma. Report of two cases with a review of the literature.

Zygomatic haemangioma is a rare benign neoplasm occurring most frequently in adult females. The authors, after reviewing the available literature, report two cases which presented with a swelling of the zygoma. Resection of the neoplasm, immediate reconstruction with cranial bone grafts and internal rigid fixation was the treatment of choice. Results two years after surgery are presented.

Osteoma of the frontoethmoidal sinuses: craniofacial resection and reconstructive strategy.

Frontoethmoidal involvement by benign tumors may lead to aesthetic and functional sequelae. The key for removal of such lesions is a proper planned craniofacial approach based on the preoperative evaluation. If total extirpation requires resection of part of the forehead or orbit, immediate reconstruction is mandatory. In recent years, craniofacial techniques and strategies have become popular. Among these are the use of split cranial bone, rotation of skull bones, the use of galeal-pericranial flaps, and the introduction of internal rigid fixation. We present a case of frontoethmoidal osteoma treated with a combined craniofacial approach. For the reconstruction, modern principles of craniofacial surgery have been applied.

Frontoethmoidal meningoencephalocele. A one-stage correction, reconstruction, and plating by means of the micro system.

This case report on one stage surgical treatment of meningoencephalocele offers the opportunity for a revision of the most recent trends expressed by other authors on this topic. Surgical technique involved a system of rigid fixations by means of microplates which, according to our experience in other cases, is particularly advisable in pediatric age. We find that the simultaneous utilization of calvarial bone grafts facilitates a better reconstruction of the cranial base, and also offers greater stability in results.