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Revista Española de Cirugía Oral y Maxilofacial

On-line version ISSN 2173-9161Print version ISSN 1130-0558

Rev Esp Cirug Oral y Maxilofac vol.30 n.2 Madrid Mar./Apr. 2008




What should the diagnosis and treatment be?

¿Cuál sería su diagnóstico y tratamiento?



A 15-year-old girl presented in the first year of life an index episode of high fever (39-40ºC) followed by transitory generalized rash after the fever disappeared. Two years later, she exhibited inflammation of various joints and intestinal villous atrophy resulting in malabsorption.

She has been receiving corticoid treatment for her articular inflammation, which accounts for a cushingoid phenotype. Since the age of 9 years her joint disease has not allowed her to walk and she is confined to a wheelchair for daily activities. She has undergone several operations, such as adductor tenotomy to prevent more articular deformity of her limbs.

With the evolution of her disease, her mouth opening has decreased progressively to the point where she can barely open her mouth.

At present, the patient is afebrile and has secondary osteoporosis. She weighs 25 kg, which is related to her muscular atrophy and malabsorption. She receives medical treatment with folic acid, methotrexate, Ca2+, Inacid® (indomethacin) and Kineret® (anti-interleukin 1).

She has developed no functional impairment and her cardiologic, pulmonary, neurologic and renal studies are normal.

CAT with axial and coronal slices and 3-D reconstruction revealed condensation in both temporomandibular joints and at the C2-C4 level (Fig. 1).



Bilateral temporomandibular joint ankylosis in patients with juvenile idiopathic arthritis

Anquilosis bilateral de ATM en paciente con artritis idiopática juvenil



I. Navarro1, J.L. Cebrián2, M. Chamorro3, J.M. López-Arcas1, R. Sánchez1, M. Burgueño4

1 Médico Residente.
2 Médico Adjunto.
3 Jefe de Sección.
4 Jefe de Servicio.
Servicio de Cirugía Oral y Maxilofacial. Hospital La Paz, Madrid, España




Juvenile idiopathic arthritis (JIA) is the most frequent and most disabling connective tissue inflammatory disease in childhood. Cornil described it in 1864 and Still differentiated its systemic form from adult rheumatoid arthritis in 1897.1

Consensus on the criteria that define the disease was reached in Durban in 1997. These criteria are: arthritis of unknown cause that lasts more than six weeks and occurs before the age of 16 years.

JIA is a pathology with a worldwide distribution that affects all races. In Sweden, the incidence is 11/100,000 inhabitants and the prevalence is 86.3/100,000 inhabitants. It affects 60,000 to 250,000 children annually in the United States.

Its etiology is unknown, but it is believed that a specific genetic substrate exists and that different immunologic and infective diseases can trigger it.

JIA is classified into 8 different subgroups based on the number of joints affected, associated pathology, rheumatoid factor status, and systemic impact. The percentage of patients in each subgroup varies in different studies but all of them report that one subgroup can change into another.2

The systemic form, or Still’s disease, accounts for about 10% of all JIA. It affects girls and boys in the same proportion and two-thirds are diagnosed before the age of 5 years (it is rare after the age of 10 years). It is associated with the HLA-DR4, DR5, and DR8 genotypes. Infective bacterial (intestinal bacteria) or viral agents (rubella, parvovirus, EBV and others) can act on the genetic substrate. The incidence in immunodepressed patients (IgA deficit, hypogammaglobulinemia, C2 deficit, etc) is also greater. Around half of the patients present a polycyclic form that remits within 2 to 6 years of diagnosis. The other half develop chronic disease and in 33% of cases it is destructive, producing local and general growth disorders. Onset before the age of 5 years, early radiologic alterations, pericarditis, and thrombocytosis are considered poor prognostic factors. The mortality of JIA is 4% due to intercurrent infections or amyloidosis, although the latter is rare.3

Clinically, it debuts as a febrile condition that precedes the articular disease by months. It is generally studied as a fever of unknown origin. After the febrile episode, a transitory morbiliform skin rash appears on the trunk, upper limbs, and face in 90% of patients. Generalized lymph node enlargement with hepatosplenomegaly occurs in 50% of cases. Polyserositis is not uncommon, manifesting as pericarditis, pleuritis, or peritonitis.

The articular condition is characterized by polyarthritis (it can affect any joint) and it shows a special predilection for the TMJ and cervical spine that originates otalgia, condylar pain, progressive limitation of mouth opening, and wry neck. The limbs become asymmetric and there is noticeable muscular atrophy. The delayed gain in weight and stature is attributed to a centralized reduction in GH secretion. Low weight is due to anorexia secondary to intestinal atrophy.4

The analytical parameters are usually the following: increased PCR and ESR, normocytic or macrocytic anemia, leukocytosis, thrombocytosis, and hypergammaglobulinemia. RF and ANA are usually negative.

Imaging studies disclose: impingement with conventional radiology, enthesitis with magnetic resonance imaging, or ankylosis with CAT. (Fig.2)

Treatment is based on the use of second-line medications (anti-interleukin 1), surgery (tenotomies), postural measures, and bone marrow transplantation.5

The word "ankylosis" is a Greek term that means stiff joint. It can be defined as the impossibility to open the mouth as consequence of the development of a fibrous or bony union between the mandibular condyle and glenoid fossa.6

There are many classifications of temporomandibular ankylosis: true (due to direct TMJ-related pathology) or false(due to indirectly TMJ-related diseases), fibrous or bony, intraarticular or extra-articular, partial or complete, unilateral or bilateral.7

TMJ ankylosis has multiple causes. The star mechanisms are direct or indirect trauma to the TMJ region, such as intracapsular condylar fracture. Iatrogenesis due to surgery on the joint and ear infections in childhood (more open Glaserian fissure) is still an important mechanism in triggering the arthritic condition that precedes ankylosis. Finally, another, less frequent, series of causes, including parotitis virus infection, scarlet fever, bifid mandibular condyle, burns, and certain rheumatologic diseases, oblige us to make an exhaustive differential diagnosis in cases of uncertain origin.6

If ankylosis is congenital (due to birth trauma or forceps use) or appears in early childhood, it manifests as an inability to open the mouth associated with abnormal mandibular growth and muscular dysfunction. It manifests clinically as facial asymmetry with either deviation to the affected side if it is unilateral or micrognathia and class II dentofacial deformity if it is bilateral. Other symptoms arising from restricted mouth opening and deficient mandibular growth are: tooth decay, malocclusion, difficulties of speech, phonation, and mastication, and apnea type respiratory disorders. Condylar pain, stiffness, muscular pain, and TMJ crepitation are also appreciated.

The diagnosis should be clinical, backed by the results of imaging studies like orthopantomography, 3D CT, and MRI. Imaging studies show reduction of the articular space, a bony and/or fibrous neoformation, and articular destructuring.8

Laskin defines the therapeutic principles of ankylosis as: surgery as soon as possible after the diagnosis is established, conservation of the height of the mandibular ramus, prevention of recurrence by interposing material, and postoperative mandibular exercises.9 The aim is to remove the ankylotic block to correct the articular morphology and function and to correct any possible dentofacial deformity and impaired growth.

The surgical treatment of TMJ ankylosis is a controversial topic in the literature. Options include simple arthroplasty, arthroplasty with interposition material (grafts of auricular dermis and fat, costochondral cartilage, and temporal muscle), prosthetic joint replacement, and even mandibular bone distraction. Corrective surgery of the dentofacial deformity in a growing patient is delicate, but the association of a costochondral graft with sagittal osteotomy, as proposed by Stringer and Boyne,10 seems to be a good therapeutic solution. Another good option could be to associate arthroplasty with a temporal muscle flap in the first stage, followed by bone distraction four months later.

In the case of our patient (Fig. 3), interposition arthroplasty with a temporal muscle flap by means of an extended preauricular approach to the temporal region was chosen. Both ankylotic blocks were freed and excised bilaterally and the coronoid apophyses were associated (Fig. 4). The achievement of an oral opening of more than 30 mm was confirmed during surgery and the patient began oral opening exercises aided by a Terabite® device in the immediate postoperative period (Fig. 5).

The rate of recurrence of ankylosis due to rheumatologic disease is greater than with other pathologies. For that reason, if the patient continues to have a good oral opening, corrective surgery for the dentofacial deformity or an intraoral distraction cannot be ruled out in the future.



Ignacio Navarro Cuéllar
c/ del Abedul 5-7, 2º dcha
28036 Madrid, España




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10. Stringer DE, Gilbert DH, Boyne PJ. A Method of treating the patient with postpubescent juvenile rheumatoid arthritis.        [ Links ]

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