Download Arthrogryposis: Diagnosis and Therapeutic Planning for Patients

Arthrogryposis: Diagnosis and Therapeutic
Planning for Patients Seeking Orthodontic
Treatment or Orthognathic Surgery
Patricia Valeria Milanezi Alves, DDS, MS,*1 Linping Zhao, PhD,2 Pravin K. Patel, MD,12
Ana Maria Bolognese, DDS, MS, PhD*
Rio de Janeiro, Brazil
Arthrogryposis is the name given to a group of
musculoskeletal disorders characterized by multiple joint contractures through the body that are
present at birth. There are many causes for congenital limitations of the range of motion of a joint.
However, the most common form of arthrogryposis,
present in 40% of patients, is a condition called
amyoplasia. In many patients, abnormal nerve,
muscle, and connective tissue development is
involved. Hands, wrists, elbows, shoulders, hips,
feet, knees, back, and jaws are affected. Because of
the complexity of tissue alterations and implications in normal facial growth, the authors of this
article address the aspects related to clinical
manifestations and therapeutic planning for
patients with this condition who seek orthodontic
treatment or orthognathic surgery.
Key Words: Arthrogryposis, orthodontic treatment,
orthognathic surgery
A
rthrogryposis is the name given to a group
of musculoskeletal disorders characterized by multiple joint contractures
through the body that are present at
birth. It is not a single disease but a spectrum of
congenital deformities that can be the end result of
more than 150 different medical syndromes, each
with its own cause and its own long-term outlook,
but all causing multiple joint contractures.1
From the *Federal University of Rio de Janeiro, Rio de Janeiro,
Brazil; 1The Craniofacial Center at University of Illinois at Chicago;
and 2Shriners Hospitals for Children, Northwestern University,
Chicago, Illinois.
Address correspondence and reprint requests to Patricia Valeria
Milanezi Alves, DDS, MS, Rua Tonelero, n.191/806. Copacabana,
Rio de Janeiro, RJ, Brazil, 22030-000; E-mail: [email protected];
[email protected]
Incidence of arthrogryposis is relatively rare. In
the United States, it occurs approximately one in
every 3,000 live births and affects both males and
females of all ethnic backgrounds.2
The literature presents as many as 10 to 20
different arthrogrypotic disorders. However, the
most common form of arthrogryposis multiplex
congenita (AMC) is amyoplasia, which accounts for
more than 40% of children with arthrogryposis.3,4
When a patient with this condition needs
orthodontic treatment or orthognathic surgery, the
practitioner involved should understand the disease
and the respective treatment because of the complexity of tissue alterations and implications in normal
facial growth. In consideration of such a necessity,
the authors of this article address the aspects related
to arthrogryposis as given in the current literature,
thus guiding both diagnosis of clinical manifestations and therapeutic planning for patients who seek
orthodontic treatment.
ETIOLOGY
T
he cause of arthrogryposis is varied and not
entirely understood.5 In most cases, AMC is
not a genetic condition. However, in approximately
30% of the cases, a genetic cause can be identified.
Environmental factors, such as drugs, alcohol, or
phenytoin (Dilantin), do play a part in many cases.6Y13
Typically, AMC is a result of problems with joint
growth and development, decreased fetal movement,
or problems with spinal development in the first 3
months of pregnancy. In normal embryonic development, the joints begin to develop by approximately 5
to 6 weeks of gestation. This motion of joints is
essential to their proper development and the
structures around them.14 The joint itself may be
normal; however, when a joint is not moved for a
period of time, extra connective tissue tends to
develop around it, fixing it in place. Lack of joint
movement also results in tendons connected to the
838
Copyright @ 2007 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
ARTHROGRYPOSIS / Alves et al
joint not stretching to their normal length. Short
tendons make normal joint movement difficult. The
same kind of problem can develop after birth in joints
that are immobilized for long periods of time.15
The causes for limitation of joint movement
before birth may be malformations or malfunctions
of the central nervous system, such as spina bifida
(meningomyelocele), brain malformations, or spinal
muscular atrophy.16,17 AMC can appear when
muscles do not develop properly or in cases of
agenesis. Suspected causes for muscular atrophy
include congenital muscular dystrophies, maternal
fever during pregnancy (above 39-C or 102.2-F for an
extended period), prenatal viruses such as German
measles (rubeola), which may damage cells that
transmit nerve impulses to the muscles, or by
infection of the fetus by a virus with neuromyal
tropism (e.g., coxsackie).4
When there is insufficient room in the uterus for
normal movement, AMC can also develop. The
mother may lack the normal amount of amniotic
fluid or have an abnormally shaped uterus.18 The
hypothesis that arthrogryposis is caused by immobilization of fetal limbs during the period of formation of joints received support from the findings of
studies of arthrogryposis in the offspring of women
who received tubocurarine in early pregnancy for
treatment of tetanus.14,15,19
CHARACTERISTICS
I
n some cases, only a few joints may be affected,
and the range of motion may be nearly normal. In
more severe cases, nearly every body joint may be
involved, including the jaws and back.20Y24
In regard to the oral cavity and face as it pertains
to this condition, a review of the literature describes
microgenia, facial asymmetry, limited mobility in
temporomandibular joints (TMJ), periodontal disease, cleft palate, high-arched palate, open-bite, facial
muscle weakness, and delayed teething.25Y27 Orthodontic treatment is difficult because of limited
mandible abduction and limited lateral movements.28 Patients with AMC should undergo intensive prophylactic procedures because their dental
treatment may be very complicated.29
Guimaraes and Marie30 studied the cause
of limited mouth movement in affected individuals.
The findings were small mandibles and contractures.
Magnetic resonance imaging (MRI) examinations
showed anterior disk displacement. Threedimensional computed tomography (3D-CT) scans
showed normal anatomic TMJ, with hyperplasia of
coronoid process protruding into the infratemporal
fossa or beneath the zygomatic arch, leading to a
mechanical limitation of mouth opening. TMJ was
normal, and the restriction of mouth movement was a
consequence of osseous dysplasia. Hodgson et al27
suggested in their study that clinical findings
observed during surgery to alleviate the limited
mandibular opening can differ from those classically
associated with other joints, suggesting that a different
pathophysiologic mechanism may be involved.
DIAGNOSIS
D
iagnosis is made by ruling out other causes.
Muscle biopsies, blood tests, and clinical findings help rule out other possible disorders and
provide evidence for arthrogryposis.31,32
AMC can sometimes be diagnosed during
pregnancy. Ultrasounds at approximately 20 weeks
gestation may show an abnormal position of joints or
lack of joint movement. Alternatively, the diagnosis
can be made on the basis of clinical symptoms and
findings. The specific subtype can often be pinpointed with radiographs. Sometimes, an electrical
nerve or muscle conduction study is necessary. CT
scan or MRI can identify any central nervous system
abnormalities or myopathic forms and may provide
important information.33
Szabo and Perjes34 studied differential diagnoses
between AMC and Larsen’s syndrome. The latter is
characterized by multiple congenital subluxations
and hyperteloric facies appearance. The radiographic
findings showed additional ossification centers. This
syndrome is inherited as sporadic, autosomal recessive, or dominant.35Y38
TREATMENT
T
reatment of arthrogryposis should not be undertaken by a single specialty but by a multidisciplinary team, including an orthodontist.39 The aim of
treatment is to improve function. Through physical
therapy and other available treatments, substantial
improvement is normally possible by stretching out
the contracted joints, developing the weak muscles,
and increasing the range of motion. Parents are encouraged to become active participants by continuing
their child’s therapy at home on a daily basis.40
Orthopedic surgery may also relieve or correct
joint problems. However, therapy and bracing is
always attempted before any consideration of surgical correction.41 Treatment of TMJ problems through
bilateral coronoidotomy surgery, right and left
meninscectomy, capsular release, and lateral pterygoid myotomies are elective choices.42 In cases of
839
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THE JOURNAL OF CRANIOFACIAL SURGERY / VOLUME 18, NUMBER 4
permanent constriction of the jaw, mechanicotherapy
is essential after orthognathic surgery and specialized electronic apparatus to maintain mobility
of the TMJ has been described by Ginstry et al.43 It is
necessary to develop muscle strength with early
stimulation of movement, and periods of immobilization should be minimized.44
Robinson45 described a high proportion of major
feeding difficulties in infants when individuals with
AMC were studied. These difficulties were related to
structural abnormalities of the jaw and tongue. In
this work, the author suggested that early identification of children with AMC is necessary and requires
continuing therapy from a number of specialists.
Despite the recommendation of physical therapy to
improve function, the literature does not relate
the importance of orthodontic evaluation and monitoring from early childhood for development of
problems.
The lifespan of an individual with arthrogryposis is usually long, but it may be altered by heart
defects or central nervous system problems. Fortunately, AMC is not a progressive disorder.46Y48
CLINICAL REPORT
Patient 1
K
S is a 13-year-old white female with the
diagnosis of arthrogryposis who was referred
by a private orthodontist for orthodontic treatment to
The Craniofacial Center at the University of Illinois at
Chicago. She presented with problems in the alignment of facial bones and teeth, enamel demineralization, clubbed feet, short stature, T3 to L5 spinal
fusion, compromised walk, and developmental
dysplasia of the right hip. Physical examination
revealed normal morphology of the forehead, orbits,
and external nose. Hypoplasia of the left face
July 2007
involving the midface and lower face was identified.
Dentally, the patient presented with full permanent
dentition, with the lower jaw deviated to the right
upon closure. There was limited opening of the lower
jaw, with a maximal incisal opening of approximately 35 mm. This condition plus the limitation of
hand movement made oral hygiene difficult.
A 3D-CT scan demonstrated hypoplasia of the
left ramus and deformity of the body of the
mandible, particularly on the left side. This results
in deformity of the floor of the mouth and deformity
of the mylohyoid muscles and anterior bellies of the
digastric muscles. The patient has multiple dentofacial problems, which require coordination between
different team members, including a plastic surgeon,
oral surgeon, orthodontist, general dentist, oralmotor speech pathologist, clinical psychologist, and
physical therapist. The treatment objectives are
continuing treatment for deformed legs, scoliosis,
and hip dysplasia; speech therapy; correction of
dental and skeletal malocclusions including mandibular prognathism, deviation, and asymmetry with
surgery; and correction of the occlusal plane cant
with maxillary surgery (Figs 1A, 2, and 3C).
Patient 2
TB is a 5-year-old African-American female with a
history of arthrogryposis and muscle rigidity who
was referred for orthodontic evaluation by the oral
surgeon. She walks with difficulty and presented with
hip dysplasia and clubfeet. Examination revealed an
orthognathic convex profile, with symmetrical frontal
facial appearance. Dentally, she has mixed dentition,
with mild generalized gingivitis, dental caries, and
tendency for an anterior crossbite. Maximum opening of month is decreased. The treatment objectives
are improvement of motion of joints with physical
therapy, with an emphasis on attempting to increase
Fig 1 (A) Patient has limitations to position of head in normal alignment. Face is asymmetrical with deviated horizontal
alignment of eyes and marked mandibular deviation to right side. (B) Right profile is orthognathic, straight to slightly
convex. Good lip balance. (C) Left profile picture. Appearance is different from right profile.
840
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ARTHROGRYPOSIS / Alves et al
Fig 2 (A) When in maximum intercuspation, there is lateral crossbite on right side with lower midline deviation that
appears essentially a result of asymmetry in mandible. (B) Right side reveals class I malocclusion, deep curve of Spee, and
enamel and periodontal problems. (C) Left side reveals class III malocclusion.
opening of the month, and monitoring of facial and
dental growth (Figs 4A and 5).
DISCUSSION
T
issue alterations secondary to arthrogryposis
may be adequate to change the dynamic growth,
resulting in a loss of facial growth equilibrium as
expressed at various adaptive growth sites. Disharmony between functional TMJ components can be
considered a local environmental disturbance with
the potential to affect condylar development.49Y52 In
other words, a functional environment and muscle
action are important in the development of condylar
cartilage and the mandible.53
Nebbe et al54 reported craniofacial deformities in
patients with internal derangement in the left or right
TMJ. In this study, a reduction in the ramus height
and a compensatory adaptation in the maxillary
dentoalveolar region were observed. The 3D-CT scan
in the first case reported in the present study
confirms this finding. The findings related to the
dimensions of the ramus and body of the mandible
suggest that loss of mobility on the left side was
more significant and probably interfered with the
normal development of all structures of the corresponding hemiface.
In accordance with Hodgson et al27 and
Guimaraes and Marie,30 3D-CT examinations of our
first patient showed that the mandibular condyles
were symmetric, and there were no translatory or rotational movements of the head of the mandible. This
suggests that a different pathophysiologic mechanism
in the decrease of the range of movement of the joint
may be involved. The authors find that early
identification of arthrogryposis is of extreme importance, and it is necessary to start functional therapy and
multidisciplinary care as soon as possible to prevent
further problems, such as those given by Robinson.45
CONCLUSIONS
B
ecause of the complexity of the facial and
associated problems, a multidisciplinary approach is recommended to provide optimal functional and aesthetic results in cases of AMC.
Fig 3 (A) Three-dimensional computed tomography (3D-CT) scan shows asymmetry of mandible. Also, it is possible to
observe abnormal development in maxilla and other structures in midface. (B) Right side of mandible. (C) 3D-CT scan
showing hypoplasia of left ramus and deformity of body of mandible.
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THE JOURNAL OF CRANIOFACIAL SURGERY / VOLUME 18, NUMBER 4
July 2007
Fig 4 (A) Frontal view showing symmetry. (B) Right profile is orthognathically convex.
Monitoring of facial growth and development should
be done so that problems can be identified early and
better results achieved. The cases reported here
demonstrate the variable dentofacial expressions of
patients with a history of AMC. The importance of
performing a thorough clinical investigation for
differential diagnosis is highlighted given the overlapping features involving tissue alterations and
interference of normal growth. Usual treatments
include stretching, strengthening, and independent
functioning of joints while attempting to integrate
the therapy as much as possible into the patients’
normal activities. The patients should be investigated not only for joint complications but also for
possible breathing, feeding, speech, occlusal, and
aesthetic problems related to their disorder. Appropriate counseling on issues such as risks, prognosis,
Fig 5 Intraoral picture showing decreased opening of
mouth secondary to limitation of temporomandibular joint
function.
and management of this condition should be
undertaken.
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