Orthognathic surgery in Melnick-Needles syndrome: a review of the literature and report of two siblings.

Melnick-Needles syndrome (MNS) is a rare congenital X-linked dominant skeletal dysplasia, characterized by exophthalmos, a prominent forehead, and mandibular hypoplasia and retrognathism. Dental features may include anodontia, hypodontia, or oligodontia. Increased collagen content, unpredictable collagen synthesis, and abnormal bony architecture have raised concerns regarding bone splitting intraoperatively and bone healing postoperatively. This report describes the cases of two sisters with MNS, who successfully underwent orthognathic surgery consisting of bilateral mandibular ramus osteotomies combined with advancement genioplasty and iliac crest bone grafting, to correct the classical MNS facial deformity of mandibular retrognathia.

Odontogenic myxoma of the maxilla: surgical management and case report.

BACKGROUND: Odontogenic myxoma is a benign odontogenic tumour of the jaw [1]. This tumour often presents as an asymptomatic expansile lesion without sensory nerve changes [2]. It is thought to arise from mesenchymal origin with cells of microscopic similarity to dental pulp and follicle [3]. Radiographically it presents most often as a multiloculated radiolucency [2]. It is a locally aggressive lesion which may require extensive treatment to prevent recurrence. METHOD: The authors present the case of a 13-year-old boy with an extensive lesion in the maxilla. CONCLUSION: We discuss various treatment approaches for management of this tumour.

Melanotic neuroectodermal tumour of infancy: surgical and chemotherapeutic management.

Melanotic neuroectodermal tumour of infancy (MNTI) is a rare pigmented neoplasm of neural crest origin. It usually presents in the first year of life in the maxilla as a fast growing lesion. We describe the case of a 3-month-old boy who presented with an enlarging swelling of left maxillary alveolus. He was treated with combined surgical and chemotherapy modalities. MNTI is complicated by high recurrence rate, local invasion and malignancy has been reported. This report describes the diagnosis, treatment and follow-up of recurrent MNTI.

Buccal mucosa urethroplasty: a 10-year retrospective review of maxillofacial and urological outcomes.

BACKGROUND: Reconstruction of a urethral stricture poses a difficult surgical problem. Anastomotic repair remains the gold standard. Strictures longer than 2 cm may require substitution urethroplasty. This is a retrospective review of all patients who underwent urethral reconstruction with an autologous free buccal mucosa graft at a Regional hospital between 1998 and 2009. METHODS: Variables recorded included; demographics: patient gender/age; follow-up period. Urology: pre-operative diagnosis/aetiology; presenting complaint; previous urological surgery, pre-operative retrograde urethrogram, stricture length, graft size, operative time/blood loss, morbidity, complications. Maxillofacial: pre-/post-operative inter-incisal opening, patency of Stenson's parotid duct, ipsilateral parotid swelling, sensory nerve deficit. RESULTS: A total of eight male patients were included. Mean age was 33 years. Two patients had one-stage dorsal onlay urethroplasty, and the remaining six had a two-stage BMG urethroplasty. All patients underwent a urethrogram 20 days post-operatively, which demonstrated no leak, and a good caliber grafted urethra in all cases. A flexible cystoscopy scope was accommodated in all patients 8 weeks post-operatively. Mean follow-up was 42 months. At long-term follow-up, there was no evidence of stricture formation, and all patients were voiding well. There were no long-term intra-oral complications. CONCLUSION: This study suggests that anterior urethral strictures up to 6 cm in length may be predictably and safely managed with buccal mucosal urethroplasty. The buccal mucosa is easy to harvest, and can be used successfully in one- and two-stage grafting procedures. The rate of complications, from both a urological and maxillofacial perspective, in the group of patients studied was low.

Aggressive giant cell granuloma of the jaws treated with interferon alpha: a report of two cases.

BACKGROUND: Giant cell granulomas (GCGs) are benign tumours of the jaws of unknown aetiology. Aggressive lesions are difficult to manage and demonstrate a tendency to recur after surgical curettage. In the early 1980s, interferon alpha-2a was found to inhibit angiogenesis through a series of laboratory experiments and was subsequently used to treat a child with pulmonary haemangiomatosis. It has been hypothesised that GCGs are proliferative vascular lesions and would, therefor, be expected to respond to antiangiogenic therapy. The purpose of this study is to report a treatment protocol consisting of enucleation, followed by subcutaneous interferon alpha. METHODS: Patients with a biopsy-confirmed giant cell lesion satisfying criteria for "aggressive" giant cell tumours were included. All lesions were enucleated, and the patients commenced interferon alpha-2a (3,000,000 units/m(2)) 48-72 h post-operatively. RESULTS: Two patients satisfied the criteria for aggressive giant cell lesions. All tumours were enucleated. There were no post-operative complications, and all patients tolerated the interferon therapy well. To date, there has been no evidence of tumour recurrence. The follow-up periods were 144 and 81 months, respectively. CONCLUSION: Antiangiogenic therapy, in combination with curettage, has proven to be a useful strategy for the management of these tumours. The use of interferon alpha-2a, following enucleation of these lesions, resulted in complete remission of all lesions, and decreased operative morbidity compared with conventional treatment.

Submental intubation: a retrospective review of 45 cases.

BACKGROUND: Airway management in patients undergoing maxillofacial surgery requires special consideration. A number of options including oro- or naso-tracheal intubation and tracheostomy are available. Submental intubation is now a recognised method of airway control during maxillofacial surgery. It provides a secure airway and does not interfere with maxillomandibular fixation or access to naso-orbito-ethmoid fractures. It avoids potential complications associated with nasotracheal intubation and tracheostomy in patients with multiple facial fractures, and obviates the need to alternate between oral and nasal intubation intra-operatively. METHODS: This is a ten year retrospective review of patients who underwent submental intubation in a Regional Oral and Maxillofacial Surgery Department. The following variables were recorded: patient gender and age, preoperative diagnosis, duration of intubation, and complications associated with the intubation technique. RESULTS: Submental intubation was performed 45 times on 45 patients. There were no complications relating to the submental intubation procedure. CONCLUSION: Submental intubation should be considered as an option for airway management in selected cases of craniomaxillofacial surgery. It is a quick and safe technique with minimal morbidity and a low complication rate. It allows access to the maxillofacial skeleton intra-operatively and does not preclude the use of intermaxillary fixation.

A role for C-terminal cross-linking telopeptide (CTX) level to predict the development of bisphosphonate-related osteonecrosis of the jaws (BRONJ) following oral surgery?

BACKGROUND: Bisphosphonates are a class of chemical compounds used in the treatment of a variety of bone-related conditions. Bisphosphonate-related osteonecrosis of the jaws (BRONJ) is a well-recognised complication. C-terminal cross-linking telopeptide (CTX) estimation has been suggested as an indicator for the risk of BRONJ. It was reported that values <100 pg/ml represent a high risk of developing BRONJ following surgery and those between 100 and 150 pg/ml, a moderate risk. The aim of this study was to determine the effectiveness of the CTX test in predicting the development of BRONJ. METHODS: This is an 18-month-prospective study of patients taking bisphosphonates, referred to a regional Maxillofacial Surgery Unit for dento-alveolar surgery. The following variables were recorded: age, gender, reason for referral, bisphosphonate type, indication for and duration of bisphosphonate treatment, medical co-morbidities, CTX value, development of BRONJ, and follow-up period. RESULTS: 23 patients underwent a fasting CTX test. The mean age was 59 years (range, 44-78 years). Nineteen were taking alendronic acid, two risedronate sodium and two zoledronic acid. The mean duration of bisphosphonate treatment was 30 months (range, 8-72 months). The mean CTX value was 180 pg/ml (range, 50-370 pg/ml), with 11 patients having a value at or less than 150 pg/ml. The mean follow-up period was 5 months (range, 3-11 months). None of the patients, who underwent removal of one or more teeth, subsequently developed BRONJ. CONCLUSION: The CTX test was not predictive for the development of BRONJ following oral surgery.

Extended genioplasty: long-term cephalometric, morphometric and sensory results.

The incision, dissection, osteotomy design and fixation are important technical considerations when performing a genioplasty. The purpose of this study was to describe an extended genioplasty technique and to evaluate stability of position, form, surface area of the chin and incidence of postoperative sensory deficit. Records of 15 consecutive adult patients who underwent the extended genioplasty procedure were reviewed. The technique included incision in the labial vestibule from 2nd premolar to 2nd premolar, dissection, mobilization and retraction of the mental nerves, osteotomy parallel to the occlusal plane extending proximally to the antegonial notch and rigid fixation. Lateral cephalograms pre- and postoperatively and at the latest follow-up (> 6 months) were analyzed by linear and computer morphometric measurements to evaluate changes in position, shape and surface area of the chin. Neurosensory data from examination or questionnaire were recorded. Immediately postoperatively (T1), mean advancement in the sagittal plane was +8.7 mm and increase in surface area was +1.1 cm2. At the end of follow-up (T2), there were no significant changes (T2-T1) in chin position or surface area. Inferior border form was rated as smooth in all cases. Neurosensory evaluation revealed that 12/12 patients evaluated had functional sensory return at T2.

Progression of facial asymmetry in hemifacial microsomia.

Hemifacial microsomia is a common craniofacial anomaly, variably affecting structures derived from the first and second pharyngeal arches. Correction of the skeletal deformity in children has been advocated to improve growth potential and reduce secondary deformity. However, contrary reports have suggested that facial asymmetry in hemifacial microsomia does not increase with growth; therefore, skeletal correction can be postponed, even until adolescence. The purpose of this study was to test the hypothesis that facial asymmetry in hemifacial microsomia is progressive. This is a retrospective evaluation of 67 patients with untreated hemifacial microsomia. The patients were categorized as: group I (mandible type I, IIa), n = 38, and group II (mandible type IIb, III), n = 29. Pretreatment posterior-anterior cephalometric radiographs were used to analyze asymmetry by measuring the angle between the true horizontal and the following planes: piriform rim, maxillary occlusal plane, and intergonial angle. Angular measurements were averaged for patients in the deciduous (<6 years), mixed (> or =6<13 years), and permanent dentition (> or =13 years). In group I, angle piriform rim, maxillary occlusal plane, and intergonial angle increased from 7.0, 4.3, and 4.4 to 8.4, 6.6, and 6.1 degrees, respectively [mean age, 4.1 (deciduous) to 8.6 (mixed) to 21.0 (permanent) years]. In group II, angle piriform rim, maxillary occlusal plane, and intergonial angle increased from 9.5, 6.2, and 5.3 to 11.7, 7.6, and 8.0 degrees, respectively [mean age, 3.4 (deciduous) to 8.0 (mixed) years]. These data demonstrate that hemifacial microsomia is progressive and underscores the importance of early surgical correction of mandibular asymmetry in this disorder.

Simultaneous maxillary and mandibular distraction osteogenesis with a semiburied device.

Distraction osteogenesis is a technique utilizing natural healing mechanisms to generate new bone; it is commonly used to lengthen the hypoplastic mandible. Distraction of the maxilla and mandible as a unit is an obvious extension of the technique. We describe the application of a semiburied distractor to simultaneously lengthen the mandible and maxilla and level a canted occlusal plane in three cases. The indications for bimaxillary distraction are reviewed, including its advantages, disadvantages and limitations.

Adenomatoid odontogenic tumour: an unusual cause of gingival swelling in a 3-year-old patient.

The adenomatoid odontogenic tumour (AOT) is an uncommon, benign lesion of the jaws. It has been subdivided into two broad categories: central AOT and peripheral AOT. A case of peripheral AOT in a 3-year-old female with a history of congenital adrenal hyperplasia is reported. The lesion presented as a gingival mass associated with a fully erupted deciduous canine tooth. This is the eleventh reported case of peripheral AOT and the first in a 3-year-old patient.

Reconstruction of a complex midfacial defect with the folded fibular free flap and osseointegrated implants.

Refinements in microsurgical techniques, plate fixation, and osseointegration have changed the conceptual approach to midface reconstruction. Free tissue transfer has emerged as the ideal method of reconstructing complex midfacial defects. Single-stage bony restoration of the palate and orbital rim using the folded fibular osteocutaneous free flap is described. The fibular free flap is our first choice for reconstructing complex midfacial defects. The thin, soft, pliable tissue is ideal for intraoral and palatal reconstructions. The bone can be tailored precisely to fit any desired shape, and forms a sturdy support for both orbital and dental prostheses. With a single flap, rapid and reliable restoration of midfacial appearance, orbital support, and palatal function can be achieved.

A protocol for the management of failed alloplastic temporomandibular joint disc implants.

PURPOSE: This is a retrospective evaluation of a protocol for management of failed alloplastic temporomandibular joint (TMJ) disc implants. PATIENTS AND METHODS: The criteria for implant failure were defined as any one or combination of the following symptoms and signs: TMJ pain, jaw hypomobility, occlusal changes, and radiographic evidence of bone pathology related to the implant. The protocol consisted of removal of the implant, aggressive joint debridement, recontouring of the articulating surfaces, and placement of a pedicled temporalis muscle/fascia flap (TF) for joint lining. Arch bars and maxillomandibular guiding elastics were used if extensive condylar recontouring was necessary. No attempt was made to reconstruct the condyle or correct occlusal abnormalities at the time of implant removal. All 27 patients (42 joints) treated by this protocol during the study period were included for evaluation. There were 24 Proplast/Teflon (PTI) (Vitek, Inc, Houston, TX), 11 Silastic (SI) (Dow Corning, Midland, MI), and 7 Christensen Fossa implants (CFI) (TMJ Implants, Golden, CO) implants removed. RESULTS: The mean follow-up period was 38.3 months (range, 3 to 65 months). Pain was well controlled in 24 of 27 patients (88.9%). Preoperative and postoperative mean maximal incisal opening (MIO) was 32.1 mm and 39.8 mm, respectively. Two patients (7.4%) required a second TMJ operation for persistent pain and limitation of opening. To date, 7 patients (25.9%) have required a secondary procedure (unilateral vertical ramus osteotomy, n = 1; Le Fort I osteotomy, n = 6) to correct occlusal prematurity on the operated side or bilateral open bite. The remaining patients have required no additional surgical treatment. CONCLUSIONS: The results of this study indicate that a proposed protocol is an effective means of controlling pain and improving jaw motion in patients with failed alloplastic TMJ disc implants.

Rigid fixation of mandibular fractures: does operator experience reduce complications?

Rigid internal fixation (RIF) is a frequently used method of reduction and immobilization of mandibular fractures. It has been postulated that the unexpectedly high complication rates reported in early studies of RIF were related to operator inexperience. The objective of this study was to determine if increased operator experience with RIF would result in a lower complication rate. Two 24-month patient care periods, separated by an intervening 12 months to avoid overlap, were evaluated retrospectively. Group 1 (n = 69 patients) represented the early experience and group 2 (n = 76 patients) the more recent data. The results of the study demonstrate a downward trend in the complication rate as surgeon experience increased. This improvement, however, was not statistically significant, and other factors may play a more important role in reducing complications.