Mandibular fracture and myotonic dystrophy

Mandibular fracture and myotonic dystrophy

Section of the Federal dental services Mandibular fracture and myotonic dystrophy Report of a case Ii. W. Hungerford, Captain, USAF (DO),* and 1’...

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Section

of the

Federal dental services Mandibular fracture and myotonic dystrophy Report

of a case

Ii. W. Hungerford, Captain, USAF (DO),* and 1’. L. illunsat, Lieutenant Commander (MC) USN&‘** uNm2~

STATES

NAVAL

HOSPITAL,

GREAT

LAKES,

ILL.

M

yotonic muscular dystrophy (dystrophia myotonica, myotonia atrophica, Steinert ‘s disease) is a heredofamilial degenerative disorder characterized by (1) progressive weakness and atrophy of distal extremity musculature, (2) a predilection for facial and neck muscle degeneration, (3) myotonia and abnormally slow relaxation of a contracted muscle, and (4) evidence of dystrophic features elsewhere, such as early balding, ocular lenticular opacities, and testicular atrophy. The disorder can be inherited with either a dominant or a recessive pattern. The onset is usually in the second or third decade, and the disability is slowly progressive in nature, although occasionally spontaneous arrest occurs. The predilection for facial and neck muscle involvement is a constant and somewhat striking feature of this disease. Temporalis and masseter atrophy, as well as degeneration of other facial muscles, leads to an appearance of hollowed temporal fossae, sunken cheeks, ptosis of the eyelids, and flattening of facial expression (the so-called “myopathic fs,cies”) . The resulting prominence of facial bony structures in the later stages has given rise to the term “hatchet face,” while sternoeleidomastoid atrophy with forward sloping of the neck produces a “swan neck” appearance. Oropharyngeal muscles are not infrequently affected and can, late in the course of the disease, give rise to dysa,rthria, dysphagia, and masticatory difficulty.’ *Formerly Resident in Oral Surgery, TTnited States Naval Hospital, Great Lakes, Ill. Present address: Dental Service, 3535th USAF Hospital, Mather Air Force Base, Calif. **Formerly of the Neurology Service, United States Naval Hospital, Great Lakes, Tll. Present address: Division of Neurology, University of California at Los Angeles Medical Center, Los Angeles, Calif.

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We have recently seen and treated a young man with this disorder who sustained a traumatic fracture of the nlantlihle. The possibility that his underlying muscle disease played a coutrihuting role in the mandibular fracture has led us to report the case and disruss this possible relationship. CASE REPORT On June 12, 1962, the patient, a 25-year-old Caucasian male National Guardsman, was admitted to the United States Naval Hospital at Great Lakes, Illinois, because of a facml trauma problem. The chief complaint on admission was pain and deformity of the lower jaw. The patient allegedly had been assaulted and injured by fist blows after being LCrun off the emergency treatment, road” by persons unknown who mere driving another car. Rc received aimed at preventing shock, at a nearby civilian hospital and was then t,ransferrrd to the Naval Hospital at Great Lakes for definitive treatment. Physical evaluation on admission revealed that the patient’s trauma pro&m was limited to the louver jaw. We was a well-developed, thin, Caucasian man in moderate distress, with the main presenting symptoms of pain, moderate trismus, and deformity of the lower jaw. There was a clinically demonstrable compound fracture of the left mandibular body, involving t,he alveolus of the left second molar, with swelling and ecchgmosis of the soft tissues adjacent to the fracture. The dentition was in a good sta.te of repair. At that time was cleared for oral surgical prono other remarkable findings were made, an<1 the patient crdures. The results of laboratory stud& rout.iwly made on admission were reported to 1~ within normal ranges, and the chest x-ray \vas reported as negative. Skull series radiographs demonstrated a fracture through the body of the mandible on the left side, which involved the alveolus of the left, second molar. The mesial root of that, tooth was fractured. There were no other fractures of the mandible or other facial bonrs. This fracture was treated conservatively by meaus of a closed reduction, wit,11 a maxillary arch bar and continuous loop wiring bilaterally on the mandible and \vith intermaxillary The lower left, second molar was retaiued elastic traction for reduction and immobilization. of the proximal fragment. That during the period of immobilizatiou to cnwre the stability tooth was removed 6 weeks later without c~omplication. The patient \vas placed on vigorous support,ive care which included antibiotics i 0 combat infwtion.

Fig. atrophy

1. Full-face photograph of the temporalis and

demonstrating prominermc masseter muscles.

of

the

zygomat,ic

arch

due

to

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18

Number 1

Fig. vault,

and

b. Lateral-skull radiograph moderate hyperostosis

demonstrating frontalis.

a small

sella,

a generally

thickmrd

cranial

During the period of convalescence, it was noted that the patient had considerable difficulty performing fine-skilled acts with his hands. Neurologic consultation was obtained. Further questioning revealed that during the past 8 years the patient had been aware of difficulty in relaxing his grip after forceful contraction. The problem had been slowly progressive since its onset, especially over the 4 months prior to admission. If movements were carried out more slowly, no difficulty was present. Recently he had noted some mild diffuse weakness which he attributed to “being out of shape.” He was unaware of any difficulty with his lower extremities or facial musculature. More specifically, there was no dysarthria or dysphagia; nor was he aware of any change in his facial appearance. Family history revealed that the patient’s father had a unilateral cataract removed at the age of 59 and that a paternal uncle had a progressive cataract of unknown duration. There was no family history of early balding, muscle weakness, or oropharyngeal difficulty. The patient’s past history was unremarkable. Examination showed the vital signs to be normal, except for a sinus bradycardia. Examination of the heart and lungs revealed no abnormalities. Ko testicular changes, lenticular opacities or balding mere noted. Neurologic examination yielded several positive findings. There was moderate weakness and atrophy of the temporalis and masseter muscles, as well as weakness of the periorbital musculature, giving the face a flattened and angular appearance (Fig. 1). Pharyngeal motor function was intact, with no dysphagia or dysarthria. The tongue showed a strong myotonic response to percussion. The reflexes were hypoactive but equal bilaterally. Atrophy and weakness of the interossei and hypothenar muscles were present in both hands. Myotonia could be easily elicited with a rapid, forceful hand grasp or by percussion of almost any muscle group. Laboratory evaluation revealed no evidence of thyroid or adrenal dysfunction. The electromyogram disclosed electrical abnormalities diagnostic of myotonic dystrophy. Skull films revealed a somewhat small sella, a generally thickened cranial vault, and moderate hyperostosis frontalis (Fig. 2).

DISCUSSION A revieTv of the literature between muscular dystrophy

shows no papers which discuss a relationship and mandibular fracture. Adams and associatrs,”

O.S., ox. 8s0.1’. .lu14’, 1964

in their text on muscle diseases, that, “recurrclll tlisl0e;ltion of thci jaw is common,” but they give no illustrat,ions 01’ referenccbs. Alt 1loUgh the> i~SsWi~tiO~1 between myotonic muscular dystrophy and mandibular fracture has not been discussed, several papers report abnornlal radiologic (~hangc~s in t,hc skull which could result in a propensity for bony fracture. Thus, reports arc’ available in the literature which show an increased incidence ot’ c&argc~l frontal sinuses, thickened cranial vault, hyperstosis frontalis, small sella turcica, and elongation of the mandible with a prognathic jaw.+” None 01’ these findings, e&her alone or in combination, are pathologic per se. They frequently o~ur in normal persons and are usually considered t,o bc “physiologic” or “within the range of normal.” Nevertheless, it is interesting-and possibly pcrti~~~~nt-thi~t these “physiologic skull changes should occur with a high Prcquency in patient,s with myotonic muscular dystrophy. A logical supposition would bc that in patients with this muscle disorder these changes do, indeed, have pathologic significance and may result in a propensity for easy fracture. It is also worthy of note that other authors”. 7 have described “dystrophic” bone changes in patients with progressive muscular dystrophy which they believe are not xecondary to the muscle degeneration per se but, rather, l,rprcscntativr ot’ yet another dystrophic feature of the disease process itself. Although adrenal and thyroid dysfunction in patients with myotonic muscular dystrophy has been reported in the past, recent evidence t,ends to discount. these earlier reports.X Otherwise, we are unaware of any documented metabolic abnormalities in patients with myotonic muscular dystrophy that, mag hc operative in producing a tendency toward easy bone fracture. In addition to the possible bony abnormalities, the relationship of facial and oropharyngeal muscle wasting to mandibular fracture must be considered. Muscle loss, per se, may not be primarily responsible for an increased propensity for jaw fracture. However, bone change secondary to decreased muscle function, according to Wolff’s law, could he an important factor. Besides myotonic muscular dystrophy, there are ot,her muscle disorders in which facial and oropharyngeal musculature is afferted. In t,hr facie scapula humeral form of progressive muscular dystrophy there is marked involvrment of the facial, neck, and shoulder girdle muscles. Patients with myasthenia gravis frequently have similar involvement. Thus, a propensitp for easy fracture might, also be present in these conditions. Stil,?

SUMMARY A ease of mandibular fracture with a concurrent muscle disease has been reported, and the possible interrelationship of the fracture and the possible predisposing bony weakness secondary to muscle loss has been discussed. REFERENCES

1. Rowland, L. P.: Muscular Dystrophies, Polymyositis, and Other Myopathies, 5. Chron. Dis. 8: 510, 1958. 2. Adams, R. D., Denny-Brown, D., and Pearson, C. M.: Diseases of Muscle; a Study in Pathology, ed. 2, New York, 1962, Paul B. Hoeber, Inc., p. 335.

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fracture

and myotonic

dystrophy

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3. Caughey, J. E.: Bone Changes in the Skull in Dystrophia Myotonica, J. Bone PC Joint Surg. 34-B: 343, 1952. 4. Slatt, B.: Myotonia Dystrophia: ,4 Review of 17 Cases, Canad. M. A. J. 85: 250, 1961. 5. DiChiro, G., and Caughey, J. E.: Skull Changes in 18 Cases of Dystrophia Myotonica, Acta radio]. 54: 22, 1966. 6. Maybarduk, P. K., and Levine, M.: Osseous Atrophy Associated With Progressive Muscular Dystrophy, Am. J. Dis. Child 61: 565, 1941. 7. Ashley, D. W., Williams, G. E. O., and Smith, 0. E.: Bone Dystrophy in Association With Muscular Dystrophy (Myopathy), Brit. M. J. 1: 1486, 1951. 8. Drucker, W. D., Rowland, L. P., Sterling, K., and Christy, N. P.: On the Functions of Endocrine Glands in Myotonie Dystrophy, Am. J. Med. 31: 941, 1961.