Hemifacial atrophy and temporomandibular joint pain- dysfunction syndrome

Case Report

A. A. Talacko and P, C. Reade Department of Dental Medicine and Surgery, University of Melbourne, Australia

A. A. Talacko and P. C. Reade. Hemifacial atrophy and temporomandibular joint pain-dy@mction syndrome. Int. J. Oral Maxillofae. Surg. 1988," 17." 224-226.

Abstract. A case is described of a pa t i en t with progressive H A who presented because of left T M J pain, j aw locking and ipsi lateral mas t ica tory muscle spasm. Several reports exist in the l i tera ture of mas t ica tory muscle spasm associated wi th HA. A diagnosis of T M J P D S was made and an occlusal spl int was p rov ided which produced resolut ion of the T M J pain and j aw locking and significantly reduced the mas t ica tory muscle spasms.

Key words: hemifacial atrophy; mastictory muscle spasm; temporomandibular joint pain- dysfunction syndrome.

Accepted for publication 16 February 1988

Hemifacial a t r ophy (HA) generally commences in the first 3 decades of life and is a condi t ion in which one side of the face is m u c h smaller or wasted when compared with the n o r m a l side. Numer - ous theories of pa thogenes is have been proposed wi th mos t emphasis placed on a l tera t ions in the per iphera l t rophic sympathet ic nerve system 1°, bu t the aeti- ology of the condi t ion remains obscure. The mani fes ta t ions include progressive a t rophy of the soft tissues of the face, changes in the eyes, skin and hair, mi- graine headaches , ipsi lateral t r igeminal neuralgia, ipsi lateral mas t i ca to ry muscle spasms and cont ra la te ra l Jack- sonian epilepsy. In a b o u t 7 % of pa- tients, there may be associated a t r ophy of ha l f o f the body. Since it is a self- l imiting condi t ion, t rea tment , if any, is main ly symptomat ic . Plastic surgery is f requent ly helpful in alleviating the cos- metic e m b a r r a s s m e n t I 3, ~3.

Case Report

This report concerns a female patient who first presented at the Oral Medicine Clinic of The Royal Dental Hospital of Melbourne at the age of 24 years complaining of a painful left temporomandibular joint (TMJ) click which was frequently associated with jaw locking. She also suffered from left masseter- ic (and occasionally teft temporalis) muscle spasms and a "pins and needles" sensation over the left side of her face. During the spasms, the patient was unable to open her mouth; spasms were initiated by cold, eating, stress or arose spontaneously, and were oc- casionally relieved by massage of the affected muscle. The spasms lasted from a few seconds

to 1 min and occurred 15-20 times each day; they persisted during the night and disrupted sleep. The patient also reported episodes of involuntary jaw movements when she would unintentionally bite her cheeks and tongue; these episodes would be aggravated during times of fatigue and illness. Previous treat- ment for her condition had included a course of oral diazepam tablets (5 mg, nocte) in order to obtain muscle relaxation and also equlibration of the occlusion. Neither of these treatments had been successful.

On examination, left facial asymmetry was noted and this was interpreted as being due to a congeni[al hypoplasia of the bony skel- eton of the left side of the face, with sub- sequent development of a malocclusion in the left buccal segment. An orthopantomogram of the facial skeleton revealed an increased interarticular space of the right TMJ and mild flattening of the right condylar head (Fig. 1): these changes were assumed to be secondary to the facial asymmetry. The muscles of mastication on the left hand side of the face and the left TMJ were tender to palpation. A diagnosis of temporomandibu- lar joint pain-dysfunction syndrome (TMJPDS) was made.

An occtusal splint was constructed for this patient and after 6 months, the symptoms had improved considerably, although the masticatory muscle spasms had not com- pletely resolved. A cobalt-chrome overlay denture was subsequently inserted and the occlusion equilibrated to achieve a stable oc- clusion at an increased vertical dimension, in an attempt to prevent the musculature of the left side of the face from achieving the degree of shortening which was necessary to trigger spasms.

The patient was lost to recall and repre- sented 5 years later when she was first seen by the authors. She reported the loss of the splint and inability to wear her denture after

some conservative dental procedures, with the subsequent exacerbation of the painful left masseteric muscle spasms and return of moderate, persistent left-sided facial and TMJ pain. In the intervening period, she had sought a neurological opinion regarding the muscle spasms and had been prescribed both carbamazepine (an anti-convulsant and anal- gesic) and baclofen (a relaxant of skeletal muscle spasm caused by lesions of the CNS at the spinal levelg). Neither medication, how- ever, had afforded her significant relief.

On examination, atrophy was noted of the left side of the face (Fig. 2). The patient had a circumscribed area of alopecia in the left paramedian region of the anterior scalp (Fig. 3). Hair loss was also evident at the medial portion of the left eyebrow; there was no facial hair in the region of the atrophy, nor did the patient have any eyelashes on the lower lid of the left eye. There was enophthal- mos of the left eye due to loss of periorbital fat and a small region of skin pigmentation was evident in the left infraorbital region. Otherwise the skin was parchment-like and conformed closely to the underlying struc- tures; subcutaneous vessels were visible on the forehead. Intra-orally the left posterior teeth were in a cross-bite relationship. There was no hemiatrophy of the tongue and the teeth exhibited normal crown and root mor- phology. The left TMJ, left masseter muscle, and left zygoma region were all tender to palpation.

At this stage, it was felt that the patient's appearance was consistent with HA, the ear- liest manifestations of which she had first become aware at the age of 17 years. This had gradually progressed until at the age of 25 years, the disorder had become static. She gave a history of migraine which had ceased at the age of 19 years. There was no history of eye disorders, epilepsy, trigeminal neural- gia or atrophy extending beyond the face.

Facial pain and hemifacial atrophy 225

Fig. 1. An orthopantomogram of the patient's facial skeleton showed an increased interarticu- lar space of the right temporomandibular joint and mild flattening of the right condylar head.

A diagnosis was made of TMJPDS in a patient with HA. A removable occlusal splint was constructed and the patient wore this nightly for 6 months. Once again, the facial and TMJ pain completely resolved but only moderate, although significant relief was ob- tained from the left masseteric muscle spasms.

Discussion

TMJPDS is a painful condition of one or both temporomandibular joints and associated muscles which is considered to be precipitated by a sprain and main- tained by a variety of factors I1 in exist-

Fig. 2. Anterior view showing atrophy of the left side of the face, hair loss at the medial portion of the left eyebrow, lack of eyei]ashes on the lower lid of the left eye, enophthalmos of the left eye and the relative prominance of the left masseter muscle.

ence before the traumatic event, includ- ing occlusal discrepancies which act sin- gly or together to prevent resolution of the joint injury and hence cause the painful condition to be maintained. In a small group of people, gross bony or occlusal discrepancies may be con- sidered as the primary aetiological fac- tors. Masticatory muscle spasm is one of the many features of this disorder, which can be regarded as a muscle splin- ting in an attempt to protect the injured joint. Included in the standard treat- ment protocol is the construction of an occlusal splint, which is worn over the posterior upper or lower teeth.

Masticatory muscle spasm is one of the more unusual manifestations of pro- gressive HA. This association has been

~reported previously in the literature by SACHS 12, KRUEGER 6, KNIGHT ~ MOORE 5 and KAUFMAN 4. The nature of the spasms has been similar in all reported cases, being of short duration, lasting from a few seconds to 1 min. They were painful, and in most cases, were the rea- son for those patients seeking treatment. Mouth opening and speech were im- possible during the spasms and spasms were precipitated by cold 12, exacerbated by chewing or anxiety and persisted during sleep 4. The muscle consistently affected was the masseter, but both in our case and in that reported by SA- CHS 12, contraction of the temporalis muscle was also noted.

It was particularly interesting in the case that we have reported here that a TMJPDS was the reason for presen- tation and that this pain-dysfunction syndrome may have been a contributing factor in the production of the masticat-

Fig. 3. Vertical view showing a circumscribed area of alopecia in the left paramedian region of the anterior scalp.

ory muscle spasms. Only one other re- port of a patient with HA, presenting with clicking and pain of the TMJ, has been found in the literature 7, although no mention of an associated masticat- ory muscle spasm was made.

The relative contribution that each of the 2 conditions (TMJPDS and HA) made to the initiation of muscle spasms is difficult to determine. KAUFMAN 4, in describing his case, did not report signs or symptoms of a TMJPDS but stated that spasms in his patient were relieved by wide opening of the mouth. There- fore, splint therapy may have a role to play in treatment of patients with HA and associated masticatory muscle spasm without other signs of a TMJPDS, by increasing the vertical di- mension to obtain a reduction in masse- teric activity 8 and thus diminishing the potential for triggering muscle spasm.

In conclusion, patients with progress- ive HA presenting with masticatory muscle spasms should be thoroughly in- vestigated for signs and/or symptoms of a TMJPDS and the standard therapy should be instituted where appropriate. Even where a full TMJPDS is not ap- parent, however, occlusal splint therapy may have a role to play in alleviating the distressing masticatory muscle spasms. This, in combination with anti- spasmodic/anticonvulsant medication may afford these patients significant re- lief. If this regime proves successful, the need for a permanent bite-raising ap- pliance should be considered.

226 Talacko and Reade

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Address: Professor P. C. Reade Department of Dental Medicine and Surgery Faculty of Dental Science University of Melbourne 711 Elizabeth Street Melbourne, Victoria 3000 Australia