CASE REPORT

Correction of a severe Class II malocclusion

in a patient with Noonan syndrome
Sergio A. Cardiel Rıos
Morelia, Michoacan, and Mexico City, Mexico, and Belgrade, Serbia

Noonan syndrome is a developmental disorder characterized by a dysmorphic facial structure, short stature, and
mild mental retardation, with associated cardiac defects and skeletal malformations. It may be sporadic or in-
herited as an autosomal dominant or recessive trait. The incidence of occurrence is 1 in 1000 to 2500 live births.
The responsible gene is located on the long arm of chromosome 12. Diagnosis of the syndrome is made by both
clinical inspection and karyotype. This is the case report of a 10-year-old Mexican boy who was referred for
correction of orofacial and occlusal defects. (Am J Orthod Dentofacial Orthop 2016;150:511-20)

N
oonan syndrome is characterized by a wide spec- referred to a geneticist, who performed a karyotype
trum of congenital heart and pulmonary defects, assessment to confirm the syndrome. The patient had
multiple skeletal defects (chest and spine), a systemic issues such as short stature, a slight mental
broad or webbed neck, cryptorchidism, and bleeding delay, heart trouble, otitis, language disturbances, and
anomalies. Although a differential diagnosis of this syn- asymmetry of the lower limbs. He had a significant
drome is difficult, the craniodentofacial structures show tongue thrust habit and a symmetrical face with severe
specific findings that can be diagnosed by a dental lip incompetence. When smiling, he showed full incisor
specialist. This case report describes the implications of display and excessive gingival tissues. He had a convex
this syndrome from an orthodontic viewpoint. profile with excessive vertical facial dimension and a
The facial construct includes a broad forehead, very recessive chin (Fig 1). The dental casts showed a
prominent eyes, hypertelorism, hooded eyelids, down- Class II Division 1 malocclusion. Overjet was 12 mm,
slanting palpebral fissures, low-set posteriorly rotated and overbite was 7 mm. The midlines were centered.
ears with a thick helix, and a bulbous tip of the nose The maxillary arch form was oval with a deep and
with a wide base and thick lips. Orthodontically, a severe constricted palatal vault. In the mandibular arch, there
maxillomandibular discrepancy is common. Addition- was moderate spacing in the anterior area. Some decid-
ally, there is normally a long face (hyperdivergence), mi- uous teeth remained in both arches, and all permanent
crognathia, excessive gingival display at smile, high teeth had shape and size deformities (Figs 1 and 2).
arched palate, an open bite or an increased overjet, Bol- The panoramic radiograph showed congenitally missing
ton discrepancies, oligodontia, and dental deformities.1 maxillary second premolars, short root lengths of all
permanent teeth, and resorption in both condyles. Range
DIAGNOSIS AND ETIOLOGY of motion and amount of opening were normal (Fig 3).
Skeletally, the patient had a severe maxillomandibu-
A Mexican boy with Noonan syndrome, aged 10 years lar discrepancy (ANB angle, 10 ). The maxilla was prog-
2 months, was seen for evaluation of his dentofacial nathic, and the mandible was mildly retrognathic. The
appearance. After a careful clinical evaluation, he was FMA and the facial index confirmed a hyperdivergent
Private practice, Morelia, Michoacan, Mexico; assistant professor, Department of growth pattern (Fig 4). Both the maxillary and mandib-
Orthodontics, Hospital for Children “Federico G omez”, Mexico City, Mexico; ular incisors were proclined. A steep occlusal plane and a
assistant professor, Department of Orthodontics, Stomatoloski Fakultet,
4-mm curve of Spee added to the complexity of the
University of Belgrade, Belgrade, Serbia.
The author has completed and submitted the ICMJE Form for Disclosure of problem. This severe malocclusion was the result of the
Potential Conflicts of Interest, and none were reported. genetic disorder and the tongue thrust.
Address correspondence to: Sergio A. Cardiel Rıos, Ortodoncia Clınica, Calle
A diagnosis was devised by using the differential
Sargento #40, Colonia Chapultepec Sur 58260, Morelia, Michoacan, Mexico;
e-mail, [email protected]. diagnostic analysis system2 and Merrifield's “dimensions
Submitted, May 2015; revised and accepted, September 2015. of the dentition” concept.3 The craniodentofacial total
0889-5406/$36.00
difficulty index for this patient was 231, a number that
Ó 2016 by the American Association of Orthodontists. All rights reserved.
http://dx.doi.org/10.1016/j.ajodo.2015.09.032 confirms a severe problem.2

511
512 Cardiel Rıos

Fig 1. Initial facial photographs and intraoral photographs.

TREATMENT OBJECTIVES This approach would eliminate some “bad” teeth

Treatment objectives for this patient included ob- (mandibular second premolars) and help with vertical
taining a balanced profile, improving function by arran- and sagittal discrepancies, and efficient overjet and
ging the teeth to achieve optimal efficiency and an Angle overbite reduction could be accomplished. However,
Class I occlusion with normal overbite and overjet, and the space would have to be carefully managed.
improving the health of the dentition, jaws, joints, 2. Extract the maxillary deciduous canines and first
and periodontal tissues. The teeth would be positioned molars, and the mandibular deciduous molars;
and arranged for maximum stability. enucleate the maxillary first premolars; and follow
with comprehensive orthodontic treatment to cor-
TREATMENT ALTERNATIVES rect the canine position.
Five treatement alternatives were considered. This plan offered efficient overjet and overbite reduc-
tion but would require moving all maxillary anterior
1. Extract the maxillary deciduous canines and first
teeth distally without anchorage loss.
molars, and the mandibular deciduous molars;
enucleate the maxillary first premolars and the 3. Combine orthodontic treatment with orthognathic
mandibular second premolars; and then use surgery (LeFort I impaction, mandibular advance-
comprehensive orthodontic treatment to correct ment and genioplasty). This option would produce
the Class II malocclusion. a better facial result, but the risks of surgery would

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Cardiel Rıos 513

Fig 2. Initial dental casts.

Fig 3. Initial temporomandibular joint tomographic, periapical, and panoramic radiographs.

need to be considered. Additionally, the condyles cooperation, and the results would be less predictable,
might be adversely impacted. given the patient's syndromic growth anomalies.
4. Mandibular “advancement” with a functional appli- 5. Extract the maxillary deciduous canines and first
ance, followed by comprehensive orthodontic molars, and the mandibular deciduous molars.
treatment. This plan would require more patient Await the eruption of the permanent dentition

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514 Cardiel Rıos

Fig 4. Initial cephalometric radiograph and tracing.

and perform comprehensive orthodontic treatment along with extraoral J-hook headgear. Elastic forces
to correct the Class II malocclusion. No permanent were used to correct the malocclusion.4,5 The steps of
teeth would need to be extracted, but patient coop- this treatment protocol are denture preparation,
eration would be the key to success. The maxillary denture correction, denture completion, and denture
deciduous second molar space would require pros- recovery (Fig 5).
thetic treatment in the future. Teeth were sequentially banded and bonded.
Maxillary and mandibular 0.016 3 0.022-in titanium
TREATMENT PLAN archwires were initially used. This denture preparation
Option 5 was selected, and treatment was accom- phase of treatment was completed with a maxillary
plished in 2 steps. 0.017 3 0.022-in stainless steel archwire and a
First, several deciduous teeth were extracted, mandibular 0.018 3 0.025-in stainless steel archwire.
including the maxillary canines and first molars and all During leveling, a pair of high-pull J-hook headgears
the mandibular deciduous molars. Tongue exercises with 8 oz of force were attached mesial to the maxillary
were prescribed to improve swallowing. canines and the mandibular canines. The headgears
The patient was sent to an otolaryngologist for eval- prevented incisor flaring and were worn 14 hours per
uation and care of the respiratory issues. day to control the anterior vertical dimension. Space
When the permanent dentition erupted, comprehen- management, correction of rotations, and axial
sive orthodontic treatment was started to correct the inclination correction were the goals of leveling. By
Class II malocclusion. the fourth month of treatment, all rotations were
corrected, and all teeth were aligned and leveled.
The denture preparation stage of treatment was
TREATMENT PROGRESS completed.
Treatment was accomplished with a 0.21 3 0.28-in The first step of denture correction was to fabricate a
nontipped, nontorqued edgewise appliance and the mandibular 0.019 3 0.025-in stainless steel working
Tweed-Merrifield directional force system, which uses archwire. This archwire had omega loop stops flush
directionally controlled precision archwire manipulation against the buccal tubes of the mandibular

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Cardiel Rıos 515

A mandibular 0.0215 3 0.028-in stabilizing archwire

with the same first-, second-, and third-order bends as
the previous archwire was fabricated. This archwire
was an exact duplicate of the previous “working”
archwire, only larger. A 0.020 3 0.025-in working
archwire was made for the maxillary arch. This archwire
had vertical helical bulbous loops in contact with the
second molar tubes. Twenty degrees of distal tip and
12 of lingual crown torque were incorporated into the
archwire distal to the helical bulbous loop.
Each helical bulbous loop was initially opened by
2 mm before the wire was inserted into the mouth.
Ligation of this archwire compressed the open helical
loops as it was tied into the teeth, thus placing a distal
force on the maxillary second molars. After the initial
insertion and after the second molars had moved distally
2 mm, the loops were subsequently opened in 1-mm
increments until the maxillary second molars had a Class
I relationship with the mandibular molars. A substantial
component of this mechanotherapy was the use of
directionally oriented high-pull J-hook headgear and
Class II elastics on an anchorage-prepared mandibular
arch while the bulbous loops are activated with
the headgear, Class II elastics, and anterior vertical
elastics.6,7
After distalization of the maxillary second molars to a
Class I relationship, the first molars were distalized by
trapping a coil spring mesial to them and continuing
the high-pull J-hook headgear with Class II elastics. Af-
ter the first molars were distalized, the deciduous
second molars, then the first premolars, and then the ca-
nines were distalized. Finally, the incisor segment (Fig 5,
B) was retracted with a conventional 0.20 3 0.25-in
closing-loop archwire. Treatment time up to this point
was 20 months. The denture correction stage of treat-
ment was completed.
Denture completion is the stage in which the maloc-
clusion correction is detailed. It is an important part of
Fig 5. A, Anchorage preparation; B, Class II mechanics; every orthodontic malocclusion correction. The arch-
and C, directional force system. wires used for this completion stage of treatment were
0.0215 3 0.0028-in stainless steel coordinated arch-
second molars. A second-order tip-back bend of 15 was wires with first-, second-, and third-order bends. Spurs
placed distal to the omega loop stop, and the mandib- were soldered for vertical cusp seating elastics (Fig 5,
ular second molar was tipped at a 15 distal inclination. C).6 In this patient, because of the resorption of the con-
To support the mandibular arch during tipping of the dyles, very light Class II and vertical elastics were used to
mandibular second molar, a high-pull J-hook headgear prevent condylar issues. Denture completion lasted
was attached to spurs soldered between the mandibular approximately 4 months.
lateral incisors and canines. After the second molar After appliance removal, a wraparound Hawley
reached its 15 distal inclination, the first molar was retainer was placed on the maxillary teeth. A Dohner
distally tipped to 8 to 10 . The position of the mandib- retainer from second molar to second molar was placed
ular second molar was maintained with a compensation on the mandibular teeth. The retention plan was for re-
bend mesial to the buccal tube. These clinical procedures tainers to be worn full time for a year and then only at
took approximately 12 months. (Fig 5, A). night for another 8 months. Retainer wear after this

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Fig 6. Posttreatment facial photographs and intraoral photographs.

8-month period of nighttime wear was to be on an as- The facial, skeletal, and dental changes are shown in
needed basis. Figures 4 and 9 through 12. Merrifield's Z angle, the
FMA, the occlusal plane, and the anterior facial
TREATMENT RESULTS height-posterior facial height ratio are the most signifi-
Patient cooperation was excellent. The photographs cant values that describe the patient's facial, skeletal,
show a symmetric, harmonious relationship of the facial and dental balance (Fig 9).8,9 The superimposition
soft tissues and a more pleasant profile (Fig 6).8,9 The (Fig 10) illustrates a favorable mandibular spatial change
smile line and the buccal corridors improved as well as in a downward and forward direction and proper control
did the nose-lip-chin relationship. Teeth and gingiva of the vertical dimension.
were healthy. Good architecture, proportions, and sym-
metry of the teeth and gingival complex were observed. DISCUSSION
A Class I occlusion with a normal anteroposterior Because of the popularity of orthognathic surgery, a
relationship was obtained (Figs 6 and 7). The maxillary patient with an excessive vertical facial dimension and
deciduous second molars were left for future considerable gingival display at smiling has often been
permanent prosthetic restoration; in the mandibular treated with surgery. However, if the patient has enough
dentition, normal alignment was achieved without growth potential, this type of severe malocclusion can be
altering the arch form and the intercanine width. The corrected with orthodontic treatment.
panoramic radiograph showed good health and root During the differential diagnosis, the orthodontist
parallelism of all permanent teeth. No further condylar must consider where the teeth should be positioned.
resorption was observed (Fig 8). For a patient with an excessive anterior vertical

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Cardiel Rıos 517

Fig 7. Posttreatment dental casts.

Fig 8. Posttreatment temporomandibular joint tomographic, periapical, and panoramic radiographs.

dimension who does not desire a surgical correction, the For this severe Class II high-angle patient, some clin-
mandibular incisors must be positioned over basal bone ical guidelines had to be considered to achieve optimal
to help resolve lip procumbency and to achieve optimal balance and harmony of the face with orthodontic treat-
facial and dental balance.10 ment and no surgery.11

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518 Cardiel Rıos

Fig 9. Posttreatment cephalometric radiograph and tracing.

Fig 10. Composite cephalometric tracings.

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Cardiel Rıos 519

Fig 11. Smile change: initial and posttreatment composite.

Guideline 3: There must be no expansion of the

dentition. When the treatment plan is devised, proper
archwire manipulation is necessary to achieve good
dimensions, proportions, and symmetry of both
dental arches. These guidelines are critical.
In this growing patient with severe vertical and sagittal
discrepancies, it was absolutely necessary to include
proper and efficient Class II mechanotherapy. During
the nonsurgical treatment of a patient with a high-
angle Class II malocclusion, it is important to prevent
extrusion of the maxillary and mandibular posterior teeth
with Class II forces. Equally important, the maxillary ante-
rior teeth should be intruded as they are retracted.
Without intrusion and control of the axial inclination of
the maxillary anterior teeth, the patient is likely to have
a longer face and more gingival display (Fig 10). A proper
force system is crucial.6,7

CONCLUSIONS
Fig 12. Facial change: initial, pretreatment, and post- The successful correction of this difficult Noonan
treatment composite. syndrome malocclusion depended on vertical dimension
control. To enhance a favorable change in the maxillo-
mandibular relationship, the orthodontist must use a di-
Guideline 1: The mandibular incisors had to be more
rectionally oriented force system to control the 3
than upright over basal bone. The higher the mandib-
horizontal planes: palatal, occlusal, and mandibular.12
ular plane angle, the more the incisors must be up-
right.
Guideline 2: Vertical dimension control was essential. REFERENCES
The clinician must carefully control the extrusion of 1. Allanson J. Noonan syndrome. J Med Genet 1987;24:9-13.
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system. Am J Orthod Dentofacial Orthop 1994;106:641-8.
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ment. However, no excessive extrusion in the poste- Orthod Dentofacial Orthop 1994;106:535-42.
rior areas can occur. If it does occur, the patient's 4. Merrifield LL. Edgewise sequential directional force technology. J
facial esthetics will be severely compromised. Charles Tweed Found 1986;14:22-37.

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