Temporomandibular Joint Reconstruction in the Growing Child.

Indications and considerations for reconstruction of the temporomandibular joint (TMJ) differ between growing and skeletally mature patients. Osteoarthritis, which is the most common cause of TMJ destruction in adults, is comparatively rare in children. The most common indications in young patients are congenital deformities, pathology, ankylosis and progressive resorptive processes. Options for reconstruction include distraction osteogenesis, autologous reconstruction (ie, costochondral graft, free fibula flap), and total alloplastic joint replacement. The choice of the ideal reconstruction is based on multiple factors, which include extent and laterality of the deformity, patient age, jaw growth pattern, and potential for progressive destruction.

Congenital Abnormalities of the Temporomandibular Joint.

Congenital deformities of the temporomandibular joint (TMJ) complex can present as a heterogeneous continuum of growth disturbances of the mandibular condyle, articular eminence, and temporal bone. This article describes several syndromes with congenital condylar deformity, including mandibulofacial dysostosis (Treacher Collins syndrome), hemifacial microsomia, oculoauriculovertebral syndrome, oculomandibulodyscephaly (Hallermann-Streiff syndrome), and Nager syndrome. Variations in the extent of TMJ deficiency seen in each individual case influence the timing and techniques of TMJ reconstruction.

Overexpression of Indian hedgehog partially rescues short stature homeobox 2-overexpression-associated congenital dysplasia of the temporomandibular joint in mice.

The role of short stature homeobox 2 (shox2) in the development and homeostasis of the temporomandibular joint (TMJ) has been well documented. Shox2 is known to be expressed in the progenitor cells and perichondrium of the developing condyle. A previous study by our group reported that overexpression of shox2 leads to congenital dysplasia of the TMJ via downregulation of the Indian hedgehog (Ihh) signaling pathway, which is essential for embryonic disc primordium formation and mandibular condylar growth. To determine whether overexpression of Ihh may rescue the overexpression of shox2 leading to congenital dysplasia of the TMJ, a mouse model in which Ihh and shox2 were overexpressed (Wnt1-Cre; pMes-stop shox2; pMes-stop Ihh mice) was utilized to assess the consequences of this overexpression on TMJ development during post-natal life. The results showed that the developmental process and expression levels of runt-related transcription factor 2 and sex determining region Y-box 9 in the TMJ of the Wnt1-Cre; pMes-stop shox2; pMes-stop Ihh mice were similar to those in wild­type mice. Overexpression of Ihh rescued shox2 overexpression-associated reduction of extracellular matrix components. However, overexpression of Ihh did not inhibit the shox2 overexpression-associated increase of matrix metalloproteinases (MMPs) MMP9, MMP13 and apoptosis in the TMJ. These combinatory cellular and molecular defects appeared to account for the observed congenital dysplasia of TMJ, suggesting that overexpression of Ihh partially rescued shox2 overexpression­associated congenital dysplasia of the TMJ in mice.

Intrauterine temporomandibular joint dislocation: prenatal sonographic evaluation.

Congenital temporomandibular joint (TMJ) diseases are very rare disorders and are usually diagnosed in childhood. Developmental disorders of the TMJ such as hypoplasia, hyperplasia, and aplasia of the TMJ compartments are characterized by TMJ dysfunction. In childhood, these patients experience recurrent dislocation, pain, and malocclusion. We present the case of a 25-week fetus with unilateral TMJ dislocation with fluid retention in the joint diagnosed by ultrasonography. To the best of our knowledge, this is the first case of TMJ dislocation diagnosed by ultrasonographic evaluation during the prenatal period.

Congenital infiltrating lipomatosis of the face with associated involvement of the TMJ structures. Case report and review of the literature.

We report a case of congenital infiltrating lipomatosis of the face (CILF) with right TMJ ankylosis causing asymmetry and reduced mouth opening. The management involved soft tissue debulking combined with a right TMJ arthroplasty and is explained in detail. A review of the relevant literature revealed the rarity of this condition. The diagnosis of CILF remains challenging. The currently accepted treatment strategy is less aggressive as facial asymmetry tends to recur. Infiltration of the facial structures from adipose tissue requires several surgical procedures in most cases. Bony hypertrophy on the affected side has been a common finding in reported cases. There have been no previous reports of TMJ ankylosis associated with this condition. CILF is a benign condition with a good long term prognosis. After 2 years of follow up our patient, has maintained full function despite facial asymmetry.

Current concepts in pediatric temporomandibular joint disorders: Part 1. Etiology, epidemiology, and classification.

BACKGROUND: Pediatric temporomandibular joint dysfunction, resulting from either soft-tissue or skeletal disorders, may be congenital or acquired. Congenital temporomandibular joint disorders are uncommon. The authors review their experience with pediatric temporomandibular joint disorders and propose a new classification system. METHODS: Clinical records, cephalograms, computed tomographic scans, magnetic resonance images, and pathologic specimens of all pediatric patients (younger than 18 years) with trismus or restricted mandibular excursion from 1976 to 2008 were reviewed. Cases were stratified according to soft-tissue or skeletal pathologic findings; skeletal abnormalities were further characterized as intracapsular or extracapsular. RESULTS: Thirty-eight patients, ranging in age from 1 day to 18 years at diagnosis, were identified with temporomandibular joint disorders. Ten cases (26.3 percent) were attributable to soft-tissue abnormality. The remaining 28 cases (73.7 percent) were attributable to skeletal abnormality, consisting of 14 congenital and 14 acquired cases (50 percent each). Acquired skeletal deformities included 12 intracapsular ankyloses (85.7 percent) and two extracapsular ankylosis (14.3 percent) (extraarticular bone blocks). Congenital skeletal deformities accounted for five intracapsular ankyloses (35.7 percent) and nine extracapsular ankyloses (64.3 percent). CONCLUSIONS: On initial survey, the data are consistent with published reports that attribute temporomandibular joint dysfunction to acquired abnormality (i.e., trauma and infection). However, the authors observed a significantly higher percentage (50 percent) of congenital temporomandibular joint skeletal disorders than previously reported. Most congenital cases involved extracapsular abnormality (i.e., coronoid hypertrophy); only a minority of cases had glenoid-condylar fibro-osseous fusion (i.e., intracapsular ankyloses). Because the diagnosis and classification of temporomandibular joint disorders determine treatment options, the authors provide a new classification that characterizes the extent of capsular involvement.

Congenital syngnathia: case report and review of literature.

Congenital bony fusion of the maxilla and mandible, especially as an isolated occurrence, is a very rare condition. The very few cases reported in the literature are mostly inadequate in description and confusing in nomenclature. An isolated case of syngnathia in a 60-year-old female patient with unilateral bony fusion (synostosis) of the maxilla and mandible associated with fibrous adhesions (synechiae) of the opposite site is reported. The existent literature is also reviewed.

Management of congenital bilateral temporomandibular joint ankylosis with secondary mandibular hypoplasia.

The aim of this article is to present the course of the condition in a case of congenital temporomandibular joint ankylosis that caused facial disfigurement, significant reduction of mouth opening, difficulty in feeding and breathing, and general interference with physical development.

Congenital alveolar fusion.

Congenital fusion of the jaws is rare. It may be unilateral or bilateral and may involve only the soft tissues or both the hard and soft tissues. This anomaly may be seen separately or in association with other syndromes. Congenital alveolar fusion restricts mouth opening, causing problems with feeding, swallowing, and respiration. Case 1 had membranous bands between the alveoli that required tracheostomy for stabilization, followed by osteotomy for release. Postoperatively, both patients had adequate mouth opening.

Craniofacial characteristics evidenced in Bartsocas-Papas syndrome from birth to five years. Case report.

Bartsocas-Papas Syndrome (BPS) is a rare congenital disorder, first described in 1972. It is usually characterized by neonatal or intrauterine death, and, as such, only 26 cases have been reported. Physical manifestations such as antecubital and popliteal pterygia (webbing), syndactyly of fingers, toes and talipes are frequently reported. However, oral, facial, cranial and dental anomalies are often overlooked. This case focuses on the latter anomalies and the effect they have on the facial growth of a 5-year-old patient.

Temporomandibular joint bony ankylosis: comparison of treatment with transport distraction osteogenesis or the matthews device arthroplasty.

Temporomandibular joint (TMJ) bony ankylosis with micrognathia is a rare congenital condition that is difficult to treat and may result in recurrence. In a series of affected patients, we compared two new methods of treatment: transport distraction osteogenesis and Matthews Device arthroplasty. All patients had computed tomography scan documented bilateral TMJ bony ankylosis. Group I (transport distraction osteogenesis) underwent distraction advancement of the mandible (for micrognathia) followed by resection of the condyles, recontouring of the glenoid fossas with interposition temporoparietal-fascial flaps, and transport distraction osteogenesis of mandibular rami segments. Group II (Matthews Device arthroplasty) underwent all of the above procedures except for transport distraction osteogenesis. Instead, the Matthews Devices were anchored to the temporal bone and mandibular rami. Hinged arms allowed for motion at the reconstructed TMJ. In both groups, patients underwent extensive postoperative therapy. Preoperative, postoperative, and follow-up lateral cephalograms were obtained, and incisor opening distances were recorded. All patients but one had severe micrognathia (n = 9). For group I (transport distraction osteogenesis), mean age was 6.8 years. and mean advancement was 28.5 mm. For group II (Matthews Device arthroplasty) mean age was 8.2 years, and mean advancement was 23.5 mm. In group I (transport distraction osteogenesis), mean incisor opening was 1 mm preoperatively and 27.5 mm postoperatively; however, it relapsed to 14.3 mm by 12.5 months follow-up (48% relapse). Mean incisor opening in group II (Matthews Device arthroplasty) was 3.9 mm preoperatively and 33.4 mm postoperatively and remained at 30.6 mm after 11.1 months follow-up (8% relapse). One patient in group I (transport distraction osteogenesis) underwent surgical revision because of relapse. Our data showed that for congenital TMJ bony ankylosis both transport distraction osteogenesis and Matthews Device arthroplasty techniques were successful initially; however, the Matthews Device arthroplasty avoided long-term relapse.

Severe proliferative congenital temporomandibular joint ankylosis: a proposed treatment protocol utilizing distraction osteogenesis.

The classical treatment for temporomandibular joint (TMJ) ankylosis in children: 1) joint release; 2) arthroplasty; 3) reconstruction; and 4) postoperative physical therapy (PT), is often unsuccessful. Postoperative physical therapy is difficult in the young patient due to poor cooperation. Moreover, there is a subgroup of patients who have a refractory congenital proliferative bony process that is the cause of their disease. In these patients, a role for distraction osteogenesis (DO) has been defined. We present a series of young patients with congenital proliferative TMJ ankylosis. Some have failed classic treatment. In such cases, DO is used to expand the mandibular size and soft tissue matrix. This creates a static open bite, facilitates mid-facial growth, and avoids compromise of the airway, speech, nutrition, and oral hygiene. To maintain these objectives, mandibular DO may be repeated as the child matures. Once skeletal maturity is reached, DO is used to normalize occlusion and further expand the soft tissue envelope prior to definitive reconstruction and aggressive post-op PT. In seven patients, this protocol has been used. Five patients are currently in the active phase of growth and undergoing interim treatment with mandibular DO. Two patients have reached skeletal maturity and have completed the protocol of DO with definitive arthroplasty and reconstruction. DO is a valuable aid in the treatment of the problematic child with congenital proliferative TMJ ankylosis. Interim DO, prior to definitive arthroplasty and reconstruction, can provide a static open bite that prevents progressive deformity and its associated functional disturbances.

Congenital temporomandibular joint ankylosis: clinical characterization and natural history of four unrelated affected individuals.

The objective of this study was to describe four unrelated patients affected by congenital temporomandibular joint ankylosis. Clinical delineation, natural history, and possible etiologies are discussed. Clinical aspects that are important for follow-up are outlined.

Moulding of the generate to control open bite during mandibular distraction osteogenesis.

Distraction osteogenesis of the craniofacial skeleton has become a widely accepted, safe, and effective means of craniofacial reconstructive surgery. Despite excellent results in general, there are still some uncertainties related to the procedure, such as development of an anterior open bite (AOB) during mandibular distraction. The aim of this study was to examine whether 'moulding of the generate', i.e. use of intermaxillary elastics during the active distraction phase is possible to close the mandibular plane angle and open bite. Three subjects, 13- and 15-year-old males and a 7-year-old female, underwent mandibular linear and angular bilateral distraction osteogenesis with moulding of the generate. Lateral cephalograms were obtained before the introduction of elastics and following distraction, once the activation was stopped and the patients were ready for the consolidation phase. Conventional cephalometric measurements were used to assess possible changes in the mandibular plane angle and incisor position. Three different anchorage systems (dental, orthopaedic, and skeletal) were used for placement of the intermaxillary elastics. Cephalometric examination showed that the mandibular plane angle was decreased during active distraction osteogenesis with the introduction of elastics and angulation of the distraction device. Depending on the type of elastic anchorage system, smaller or greater amounts of extrusion of the incisors were noted. Moulding of the generate during active distraction can be performed to reduce the mandibular plane angle and open bite. To prevent unwanted dentoalveolar changes from occurring during elastic traction, skeletal rather than dental fixation of the elastics is recommended. Intrusive mechanics may be incorporated into the orthodontic appliances to balance extrusive force by the moulding elastics.

Displacement of the mandible in a child with congenital unilateral temporomandibular joint ankylosis treated with two-stage condylar replacement: a long-term study with the aid of roentgen stereometric analysis.

OBJECTIVE: To monitor mandibular displacement in three dimensions with a high degree of accuracy before, during, and after treatment. DESIGN: Prospective roentgen stereometric analysis from age 3 years 7 months to age 13 years 8 months. SETTING: Center for Craniofacial Anomalies and Department of Maxillofacial Surgery, Malmö University Hospital, Malmö, Sweden. PATIENT: Girl with unilateral congenital temporomandibular joint (TMJ) ankylosis and marked facial asymmetry. INTERVENTIONS: Epipharyngeal inspection at age 2 years 6 months and insertion of implants under general anesthesia. Resection of the affected right condyle at age 5 years 4 months, replacement of the ramus-condyle complex with a custom made titanium prosthesis at age 5 years 5 months, and replacement of the artificial condyle by a costochondral graft at age 9 years 7 months. Roentgen examinations were performed at intervals from age 3 years 7 months to age 13 years 8 months. Body length was measured annually. MAIN OUTCOME MEASURES: Stereo roentgenograms were digitized by the Department of Orthopedic Surgery, Malmö University Hospital. RESULTS: Unexpected lowering of the mandible occurred before treatment. Each procedure had a distinct effect on the direction of mandibular displacement. There was little or no pathological involvement of the functional matrix. Growth of the costochondral graft exceeded growth of the unaffected TMJ. Anterior displacement as well as rotation and tilting of the mandible to the unaffected side accelerated after the age of body peak height velocity. CONCLUSIONS: Accurate monitoring of mandibular displacement may contribute to a rationale for clinicians' decision on treatment protocol and reveal compensatory differential growth.

Congenital temporomandibular joint ankylosis--a case report.

A case of congenital temporomandibular joint (TMJ) ankylosis which caused facial disfigurement, significant reduction in mouth opening, difficulties in feeding and breathing, and general interference with physical and mental development is presented. The wide range of manifestations exceed the typical picture of TMJ ankylosis and resemble a syndrome rather than an isolated defect of the TMJ.

Incidence of maxillofacial involvement in arthrogryposis multiplex congenita.

PURPOSE: This study determined the incidence of maxillofacial involvement in patients diagnosed with arthrogryposis multiplex congenita (AMC). PATIENTS AND METHODS: Twenty-three patients were evaluated by the pediatric physical medicine and rehabilitation, orthopedic surgery, and pediatric oral and maxillofacial surgery departments. Any patient in whom the diagnosis of AMC was in doubt was excluded from the study. All patients with limited mandibular function underwent computed tomography (CT) examination of their temporomandibular joints (TMJ). The results of physical therapy were followed. RESULTS: Five of the 23 patients diagnosed with AMC were found to have maxillofacial involvement, eg, presence of cleft palate, Robin-like sequence, high-arched palate, open-bite deformity, facial muscle weakness, esophageal dysfunction, and limited mandibular opening. No TMJ abnormalities were found by CT scan. Physical therapy was used for treatment of the limited opening, but relapse occurred quicky after therapy was discontinued. CONCLUSION: The incidence of maxillofacial findings is similar to that of most other reports. Treatment involves surgical correction of abnormal anatomy when possible (ie, cleft repair), symptomatic management (ie, esophageal dysfunction), and physical therapy.