1 5 0 J. max.-fac. Surg. 14 (1986)

J. max.-fac. Surg. 14 (1986) 150-152 © Georg Thieme Verlag Stuttgart - New York

Congenital Ankylosis of the Mandible Report o f a Case Noted at Birth*

Alagumba L. Nwoku, T. Ayodele Kekere-Ekun

Department of Oral and Maxillofacial Surgery (Head: Prof. A. L. Nwoku, M.D., D.M.D., F.W.A.C., F.M.C.D.S.) College of Medicine, University of Lagos, Nigeria

Submitted 14.12. 1982; accepted 28.8. 1985

Summary

A rare case of true congenital ankylosis of the mandible to the maxilla, noticed at birth without any associated deformity, is presented. The child was initially kept alive by nasogastric tube feeding and proper oral feed- ing was established later by bilateral coronoidectomy to release the ankylosis. The pathogenesis of congenital ankylosis is also discussed.

Key-Words

Congenital - TMJ-ankylosis - Obstetric ankylosis - Coronoidectorny

Introduction

The aetiology of temporomandibular joint ankylosis is varied. It includes inflammatory diseases, neoplasia, trauma, infection, radiation and systemic disease (Thoma 1945, Schulte 1967, El Mofty 1972). However, the cause of loss of mandibular movement in some cases has so far not yet been identified. Authentic cases of congenital ankylosis of the temporoman- dibular joint are very rare. Many reported cases of tem- poromandibular joint ankylosis occurring in early infancy were erroneously described as congenital (Topazian 1964, Guilhem and Cadenat 1955). Topazian (1964), in a review of the literature, found 5 cases of congenital ankylosis of the temporomandibular joint out of 185 cases of ankylosis reviewed. Mathis (1962) and Salleh (1965) reported two cases of true congenital fusion of the mandible and the maxilla. A review of the literature appears to reveal only seven known cases of true congenital fusion of the mandible to the maxilla. This case report of bony union of the mandible to maxilla, noticed at birth, represents an additional case of true congenital fusion of the jaws to the extremely scanty literature on this subject.

Report of Case

On November 28th, 1977 a twelve-day-old baby girl (C.N. Luth, No. 1977) was admitted to the paediatric ward of the Lagos University Teaching Hospital, Lagos, because of complete immobility of the mandible and inability of the mother to feed her. She was then referred to the Maxillofa- cial Surgeon in the Hospital.

History: The baby, the last of six siblings, was delivered in a private hospital after a full-term normal pregnancy. Her weight at birth was 2.8 kg. and none of the other siblings in the family had any deformity. She cried feebly, but could not open her mouth.

* Paper read at the 4th Congress EAMFS in Venice, Italy, September 1978. Dedicated to my teacher Professor H. Obwegeser, M.D., D.M.D., F.D.S.R.C.S., on the anniversary of his 65th birthday.

Physical examination showed an otherwise normal infant, active, not jaundiced or pale. The chest was clinically clear with normal I and II heart sounds and no pathological murmurs. The central nervous system examination revealed no abnormality. Immobility of the mandible was complete so that neither lateral nor protrusive excursion was possible (Fig. 1). The middle and upper third of the facial skeleton were normal and no auricular deformity was present. The mandible was slightly retruded with concomitant loss of prominence of the chin. The lower alveolar ridge was behind the upper ridge from which it was separated by a narrow slit, not more than 2 ram. wide.

Investigations Laboratory investigations and blood chemistry were within the normal limits. Radiographic examination of the chest and extremities was noncontributory. Antero-posterior views of the mandible showed a normal mandible. How- ever, lateral views revealed that the coronoid process was elongated, deformed and fused to the region of the maxil- lary tuberosity and temporal bone on both sides (Fig. 2). It was not possible to obtain a good radiograph of the tem- poromandibular joints.

Treatment: On admission, a nasogastric tube was inserted for feeding, combined with supportive therapy of multivite, 20 drops tds, ferrous sulphate 5 ml. tds and folic acid. By the end of two weeks the general condition of the baby was found to be suitable for us to contemplate an operation. On January 6th, 1978, the baby was taken to the operating theatre and blind naso-endotracheal intubation was per- formed. An intraoral incision was made on each side in the buccal sulcus extending from the ramus upward towards the coronoid process. Exposure of the ramus and coronoid process confirmed the clinical and radiological diagnosis of bony fusion between the coronoid process and the maxillary tuberosity. The temporalis muscle was inserted laterally. Coronoidectomy was performed on both sides and the temporalis muscle divided, immediate opening of the mouth was achieved, the interalveolar ridge space being 30 ram. (Fig. 3). The tongue, palate and oral cavity could then be examined, and were found to be normal. Partial closure of the surgical wound was performed using 4.0 chromic catgut sutures. The post-operative regime prescribed consisted of 125 nag. Ampicillin syrup 8 hourly for 7 days, valium 2.5 rag. b.i.d.

Congenital Ankylosis of the Mandible J. max.-fac. Surg. 14 (1986) 151

Fig. 1 Preoperative photograph of the 12-day-old infant. Attempt to force the mouth open shows that there is complete ankylosis.

Fig. 2 Lateral view radiograph of the jaws showing elongated and deformed coronoid process which appears fused to the maxillary tuberosity. The condylar head cannot be clearly assessed in this radiograph,

Fig. 3 Photograph showing immediate post-operative result follow- ing bilateral intraoral coronoidectomy. The interalveoJar distance measured 30 mm.

Fig. 4 Postoperative condition 1 year later spontaneous mouth- opening is 25 mm, Note normal mandibular growth.

for 2 days, Paracetamol elixir 5 ml. when necessary, and toilet of the oral cavity with normal saline 5 times daily.

Result: Mouth-opening exercises were commenced on the second post-operative day using finger pressure three times daily. In order to encourage passive exercise of the joint the mother was advised to start breast-feeding the baby at the same time. Assessment of mouth opening was made by making her cry. She was discharged home on March 20th 1978 with a constant mouth opening of 25 mm. inter- alveolar ridge distance (Fig. 4). She has been under our supervision on an outpatient basis since, and although there has been a decrease in the interincisal distance, compared to

the interalveolar ridge distance established during hospital- isation, the temporomandibular joint is still functional on each side. When last seen in 1982, five years after opera- tion, the deciduous teeth had all erupted and spontaneous mouth-opening was 22 mm. (I1 D). Even though we strongly advised 6-monthly follow-up, the patient did not come back for further review.

Discussion

Burket (1936) pointed out that some of the so-called cases of congenital ankylosis of the temporomandibular joint were a sequel to injuries caused during delivery. The valid- ity of this traumatic aetiology was disputed by Guilhem and

152 1. max.-fac. Surg. 14 (1986) A.L. Nwoku, T. A. Kekere-Ekun: Congenital Ankylosis of the Mandible

Cadenat (1955). They observed that traumatic forceps delivery did not lead to ankylosis and concluded that staphylococcal infection during delivery was probably responsible for the so-called congenital or obstetric anky- losis. This suggestion would appear not to corroborate the observation made by Rushton (1944) that the anatomy of the condylar head implied that it was fragile and susceptible to crush injuries in early childhood. The finding by Beavis (1928) that children between the age of one and ten years could be struck on the chin with significant force, and yet only a few might develop ankylosis, does not appear to support Rushton's (1944) observation. This dichotomy of opinion may in fact only be apparent rather than real, because it is possible to injure the TMJ without the subse- quent complication of ankylosis. The loss in mouth opening in our patient over a 5 years period is apparent rather than real, because this would be a logical expectation since eruption of the maxillary and mandibular dentition must occupy part of the interalveolar ridge space initially established when bilateral coronoidec- tomy was performed. It is also significant that even though the mouth opening of 25 mm. attained at the time the baby was discharged from hospital was not maximal, the impetus for mandibular growth was adequate, because there is no facial asymmetry or disfigurement five years after the operation. Still, since a five years old child is expected to have an interincisal distance of about 40 ram., it must be assumed the scars prevented mouth opening becoming completely normal. The authors therefore submit that provided the mandible is not immobilised, trauma or injury to the joint alone may not necessarily lead to temporomandibular joint ankylosis and that, ankylosis can only occur if there is infection in addition and/or inherited sussceptibility to develop such pathological change. This hypothesis appears to reconcile the contradiction in the observations of Beavis (1928) and Guilhem and Cadenat (1955) on the one hand, and that of Rushton (1944) on the other. Therefore the suggestion by Kazanjian, as reported by Topazian (1964) that "without infection the majority of injured joints will heal without causing permanent disabil- ity" can be regarded as being valid, at least to some extent. Ankylosis of the TMJ may occur in association with other congenital malformations such as the syndrome of cam- podactyly, multiple ankylosis, facial anomalies and pulmo- nary hypoplasia described by Pena and Shokeir (1974) and Punnett et al. (1974) and Franceschetti's Syndrome, also known as Treacher-Collins Syndrome or mandibulo-facial dysostosis (Franceschetti and Klein 1949), discussed by Gray (1969). In this syndrome, the antimongoloid palpeb- ral fissures are a prominent sign, together with auricular deformity, deficient malar bones and micrognathia. But in this infant all these signs were absent; the facial appearance as well as the external ears were normal. The malar bones were not deficient. There is therefore no finding to suggest even an atypical case of Franceschetti's Syndrome or any other syndrome. At operation the temporomandibular joints were found to be normal, but the coronoid process was fused to the maxillary tuberosity and temporal bone. Separation of the coronoid process just below the level of the zygomatic arch, and division of the temporalis muscle permitted opening of

the mouth. One possible explanation therefore could be that of an anomalous fusion of the embryonic mesenchyme in the early development of the temporomandibular joints. But what here was the impetus for such anomalous fusion at the embryonic stage? Hohl et al. (1981) showed experi- mentally that chemicals injected into the condylar capsule, and induced bacterial infection of the temporomandibular joints may cause ankylosis. In this case there was no history of maternal illness or treatment during pregnancy. Moreover, there was no other anomaly discovered at birth. Although the baby was referred to us about twelve days after she was born, there was no doubt about the presence of ankylosis at birth because no amount of damage due to trauma at birth could produce the severe bony union bet- ween the mandible and maxilla noted on radiographs and at operation, in such a short time, and yet leave no tell-tale evidence on the overlying facial skin. We must therefore conclude, that this is probably a freak of nature in the development of the temporomandibular joint region.

References

Beavis, J. 0.: Intra-articular bony ankylosis of the temporomandibu- lar articulation J.A.D.A. 15 (1928) 874

Burket, L. W.: Congenital bony temporomandibular ankylosis and facial hemiatrophy: review of literature and report of case J.A.M.A. 106 (1936) 1748

E1Mofty, S.: Ankylosis of the temporomandibular joint. Oral Surg. 33 (1972) 650

Franceschetti, A., D. Klein: The mandibulo-facial dysostosis: A new hereditary syndrome. Acta Ophth. 27 (1949) 143

Gray, R. L. M.: Corono-condylar ankylosis in childhood due to infec- tion. Br. J. Oral Surg. 7 (1969) 40

Guilhem, P., E. Cadenat: Etiology of so-called congenital tem- poromandibular joint ankylosis Abstract in Oral Surg. 8 (1955) 449

Hohl, T. H., P. A. Shapiro, B. C. Moffett, A. Ross: Experimentally induced ankylosis and facial asymmetry in the Macaque. J. max.- fac. Surg. 9 (1981) 199

Mathis, H.: giber einen Fall yon Ern~ihrungsschwierigkeit bei con- nataler Syngnathie. Dt. zahn~irztl. Z. 17 (1962) 1167

Pena, S. D. J., M. H. K. Shokeir: Syndrome of campodactyly, multiple ankylosia, facial anomalies and pulmonary hypoplasia: a lethal condition. J. Paediatr. 85 (1974) 373

Punnet, H. H., M. L. Kistenmacher, M. Valdes-Dapena, R. T. Ellison: Syndrome of ankylosis, facial anomalies and pulmonary hyper- plasia. J. Paediatr. 85 (1974) 375

Rushton, M. A.: Growth at the mandibular condyle in relation to some deformities. Brit. Dent. J. 76 (1944) 57

Salleh, N. B. M.: Congenital partial fusion of the mandible and max- ilia. Oral Surg. 20 (1965) 74

Schulte, W. C.: Ankylosis of the temporomandibular joint. Report of three cases. Oral Surg. 24 (1967) 270

Thoma, K. H.: Functional disturbances following fractures of the mandibular condyle and their treatment. Amer. J. Orth. Oral Surg. 31 (1945) 575

Topazian, R. G.: Etiology of ankylosis to temporomandibular joint: analysis of 44 cases. J. Oral Surg. 22 (1964) 227

A. L. Nwoku, M.D., D.M.D. College of Medicine of the University of Lagos Department of Oral and Maxillofacial Surgery Private Mail Bag 12003 Lagos/Nigeria