150

J. max.-fac. Surg. 14 (1986)

J. max.-fac. Surg. 14 (1986) 150-152

Georg Thieme Verlag Stuttgart - New York

Summary

Report o f a Case N o t e d at Birth*

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 feeding was established later by bilateral coronoidectomy
to release the ankylosis. The pathogenesis of congenital
ankylosis is also discussed.

Alagumba L. Nwoku, T. Ayodele Kekere-Ekun

Key-Words

Congenital Ankylosis of the

Mandible

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

Congenital - TMJ-ankylosis - Obstetric ankylosis Coronoidectorny

Submitted 14.12. 1982; accepted 28.8. 1985

Physical examination showed an otherwise normal infant,

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 temporomandibular joint are very rare. Many reported cases of temporomandibular 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 Maxillofacial 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.

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. However, lateral views revealed that the coronoid process was
elongated, deformed and fused to the region of the maxillary tuberosity and temporal bone on both sides (Fig. 2). It
was not possible to obtain a good radiograph of the temporomandibular 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 performed. 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 following bilateral intraoral coronoidectomy. The interalveoJar distance
measured 30 mm.

Fig. 4 Postoperative condition 1 year later spontaneous mouthopening 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.

the interalveolar ridge distance established during hospitalisation, the temporomandibular joint is still functional on
each side. When last seen in 1982, five years after operation, 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.

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. interalveolar 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

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 validity 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 ankylosis. 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 subsequent 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 coronoidectomy 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 disability" 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 campodactyly, multiple ankylosis, facial anomalies and pulmonary 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 palpebral 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 experimentally 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 between 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
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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 infection. Br. J. Oral Surg. 7 (1969) 40
Guilhem, P., E. Cadenat: Etiology of so-called congenital temporomandibular joint ankylosis Abstract in Oral Surg. 8 (1955)
449
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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 maxilia. Oral Surg. 20 (1965) 74
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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