The Cleft Palate-Craniofacial Journal 50(2) pp.

242–248 March 2013

Ó Copyright 2013 American Cleft Palate-Craniofacial Association

CASE REPORT

Conservative Management of Severe Open Bite and Feeding Difficulties in

Patient With Noonan Syndrome
Cristiane Sá Roriz Fonteles, D.D.S., M.S., Ph.D., Ana Catarina de Miranda Mota, D.D.S., M.S., Ramille Araújo Lima,
D.D.S., M.S., Priscilla Cavalcante Borges, S.L.P., Adriana da Silveira, D.D.S., M.S., Ph.D.

Noonan syndrome is a multiple malformation disorder with an autosomal dominant inheritance

pattern. Congenital heart defects, short stature, thoracic deformities, short neck with webbing,
hypertelorism, malocclusions, and feeding difficulties are some of the commonly observed
clinical features. We report on a case of a patient with Noonan syndrome, severe open bite,
associated feeding difficulties, and strong gag reflex, discussing conservative management with
myofunctional therapy. Myofunctional therapy has demonstrated a successful outcome,
reestablishing masticatory function and routine dental home care after an 18-month follow-up.

KEY WORDS: Noonan syndrome, open bite, feeding difficulties

Noonan syndrome is a multiple malformation disorder 2002; Bertola et al., 2003; Ogawa et al., 2004); short neck
with an apparent autosomal dominant inheritance pattern with webbing, redundancy of skin, and low posterior
(Tartaglia and Gelb, 2005) and an estimated incidence of hairline (Addante and Breen, 1996; Bertola et al., 1999;
1:1000 to 1:2500 live births (Tartaglia et al., 2001). The first Tartaglia and Gelb, 2005); bleeding diathesis (Bertola et al.,
published illustration of this condition was done by 2003); cryptorchidism (Pierpont, 1996; Bertola et al., 1999;
Kobylinsky in 1883. Nevertheless, it was only described Saenger, 2002; Bertola et al., 2003); and ophthalmologic
as a syndrome by Jacqueline Noonan in 1968. Noonan and orthoptic findings, including hypertelorism with
affirmed the existence of a new syndrome with associated downward sloping palpebral apertures, epicanthal folds,
congenital cardiac disease. Mutations in the PTPN11 gene, ptosis, strabismus, and amblyopia (Lee et al., 1992;
encoding the nonreceptor protein tyrosine phosphatase Tartaglia and Gelb, 2005).
SHP2, which maps to the long arm of chromosome 12, were Craniofacial characteristics have been described as dental
identified in more than 50% of studied patients (Bertola et malocclusions (Tartaglia and Gelb, 2005); philtrum with
al., 1999; Bertola et al., 2003; Tartaglia et al., 2001). It has deep grooves and low-set and posteriorly rotated ears
also been affirmed that more than half the cases represent (Pierpont, 1996); high arched palate (Pierpont, 1996;
new mutations (Pierpont, 1996). Ogawa et al., 2004, Tartaglia and Gelb, 2005); microgna-
The most commonly observed clinical features associated thia (Addante and Breen, 1996; Ogawa et al., 2004);
with Noonan syndrome are congenital cardiac defects retrognathia (Pierpont, 1996); and dental abnormalities,
(Pierpont, 1996; Bertola et al., 1999; Saenger, 2002); bifid uvula, and a rarely fissured palate (Addante and
thoracic deformities with superior pectus carinatum and Breen, 1996). These patients usually present with moderate
inferior pectus excavatum (Addante and Breen, 1996); developmental delay during infancy, affecting motor,
short stature (Pierpont, 1996; Bertola et al., 1999; Saenger, learning, and language skills (Pierpont, 1996; Pierpont et
al., 2009).
In 2010, Romano et al. published a state-of-the-art
Dr. Fonteles is Associate Professor, Division of Pediatric review article that summarized a meeting of health care
Dentistry, Department of Clinical Dentistry, Federal University providers coordinated by the Noonan Syndrome Support
of Ceará, Brazil. Dr. de Miranda Mota is Postgraduate Student and
Dr. Lima is Ph.D candidate, Postgraduate Program in Dentistry, Group; this meeting gathered experts in various aspects of
Federal University of Ceará, Brazil. Ms. Borges is SLP at the the disorder with the aim of developing guidelines for its
Pediatric Special Care Clinic, Department of Clinical Dentistry, diagnosis and management. The authors reported that 55%
Federal University of Ceará, Brazil. Dr. da Silveira is Chief of
Orthodontics, Dell Children’s Craniofacial Center and Adjunct to 100% of patients with Noonan syndrome presented with
Assistant Professor, Department of Biomedical Engineering, high arched palate, 50% to 67% manifested dental
University of Texas at Austin. malocclusion, 72% expressed articulation difficulties, and
Submitted September 2011; Accepted April 2012.
Address correspondence to: Dr. Cristiane Sá Roriz Fonteles, 33% to 43% presented with micrognathia. Development of
Unidade de Pesquisas Clı́nicas/Universidade Federal do Ceará, mandibular cysts was another observed feature, character-
Laboratório de Farmacologia Metabólica e Fisiologia Celular, ized by multinucleated giant cells within a fibrous stroma,
Avenida José Bastos, 3390, sala 106, Caixa Postal 3229, CEP
60.436-160, Fortaleza-Ce, Brazil. E-mail [email protected]. indistinguishable from cherubism. However, these two
DOI: 10.1597/11-214 conditions are genetically distinct; SH3BP2 gene mutations

242
 Fonteles et al., CONSERVATIVE MANAGEMENT OF OPEN BITE IN NOONAN SYNDROME 243

FIGURE 2 Panoramic radiograph showing erupting second permanent

molars, unerupted third molars, generalized horizontal bone loss,
localized vertical bone defects, symmetric condyles, and an irregular
FIGURE 1 Lateral occlusal view before treatment, demonstrating lack mandibular border.
of dental contact, visible gross plaque, and calculus accumulation.

On extraoral examination, the patient was found to have

are found in people with cherubism, whereas PTPN11126
short stature, pectus carinatum, pectus excavatum, clino-
and SOS1 mutations are found in patients with Noonan
brachydactyly, short neck, a low posterior hairline, low-set
syndrome who have giant cell lesions. ears, and a flattened zygomatic bone. Intraoral exam was
Severe feeding difficulties are a common pattern in performed with great difficulty because of the patient’s
Noonan syndrome. However, it often goes unrecognized strong gag reflex and constant vomiting. The following
until failure to thrive and/or malnourishment becomes an were observed during examination: visible gross plaque and
obvious issue for these patients. Though the prevalence and calculus accumulation in the sub- and supragingival areas,
underlying cause are usually poorly understood, delayed including the occlusal thirds of all permanent molars;
gastrointestinal motor development has been suggested as gingival bleeding; periodontal pockets (average measure-
the plausible explanation for such condition (Shah et al., ment of 4 mm); and no malpositioned teeth, aside from a
1999). Great management difficulties may be encountered rotated mandibular central incisor (Fig. 1). The patient was
by pediatric dentists, because the association between caries free. Also noted were the presence of hypotonic lips,
widespread intestinal dysmotility and gastrointestinal lip incompetence, and tongue thrusting at rest and during
reflux, coupled with developmental delay of the central deglutition, generating lack of maxillary-mandibular inter-
nervous system, may lead to strong gag reflexes in these dental contact. Thus, clinical evaluation of overbite and
patients. Furthermore, craniofacial discrepancies may pose overjet was not possible. No underlying chronic respiratory
an added challenge, enhancing feeding difficulties because condition was identified to explain the observed open-
of the patient’s inability to chew on solid foods. The mouth posture, suggesting that the patient’s mouth was
purpose of this article is to present the case of a patient with kept open as a habit or because of low muscle tone. In spite
Noonan syndrome, associated severe open bite, and a of a nasolabial angle within normal range (100.98), facial
strong gag reflex and to discuss a conservative approach analysis showed dolicocephalic pattern and convex profile
with myofunctional therapy. because of mandibular rotation. Also noted were an
increased lower facial height with asymmetry and mandib-
CASE REPORT ular deviation to the right on opening. Interpupil and
epicanthal distances were altered. Analysis of the models
A boy aged 13 years and 5 months presented to the showed an intermolar mandibular arch length 7 mm greater
Pediatric Special Care Clinic at the Federal University of than the intermolar distance in the maxillary arch, while the
Ceará Dental School for his first dental evaluation. He was anterior length of the mandibular arch was reduced by 3
referred from Clinical Genetics. The patient’s mother mm, with no maxillary or mandibular crowding. A
reported a chief complaint of the great difficulty in flattened palate was also noted (25% Korkhaus Index).
performing routine oral hygiene on her son, who wasn’t In a panoramic view, radiographic examination showed
able to chew solid foods. The son was diagnosed with the presence of all 32 permanent teeth, with erupting second
Noonan syndrome soon after birth but moderate develop- permanent molars and unerupted third molars, generalized
mental delay and very limited learning and language skills horizontal bone loss, localized vertical bone defects,
had been identified at the time of presentation. The patient symmetric condyles, and an irregular mandibular border
had reduced hearing ability in the right ear. However, other (Fig. 2). Cephalometric analysis demonstrated vertical
common congenital defects, such as cardiac, lymphatic, and growth and a skeletal open bite (Fig. 3). Osseous bases
bleeding abnormalities, were absent. were well interrelated but retruded relative to the cranial
244 Cleft Palate-Craniofacial Journal, March 2013, Vol. 50 No. 2

chin region, and chewing muscles was performed with

isotonic, isometric, and isokinetic procedures for mobility,
tonus, and strength, respectively.
Initially, a relaxing massage was performed on the
patient’s face to release the muscles for the planned exercise
sequence. Subsequently, mobility exercises of the tongue
were performed with rotation, vibration, and crackles. The
patient was instructed to thrust the tongue against the
cheeks and hard palate for strength and tonus. To correct
tongue posture, the patient was coached to place the tongue
on the incisal papilla. All of these exercises were alternated
and sequentially followed with a specific number of
repetitions. To minimize lip incompetence and exercise
breathing, the patient was asked to blow into balloons.
Muscle strengthening of the orbicularis oris superior,
orbicularis oris inferior, buccinator, masseter, and glossal
muscle groups was obtained by using a wooden tongue
blade and applying contra-resistance on the muscle or on
the focused group of muscles, for instance, lips, cheeks, and
tongue.
Breathing exercises were performed by placing the
wooden spatula on the mouth to promote nasal breathing.
FIGURE 3 Cephalometric radiograph showing vertical growth, Finally, a rubber band was placed between the maxillary
skeletal open bite, retruded osseous bases relative to the cranial base, and mandibular posterior teeth, and the patient was asked
anterior cranial base and mandibular lengths within normal range,
inclined mandibular plane, increased lower facial height, proclined
to occlude and attempt chewing, resembling natural
maxillary, and mandibular incisors. mastication cycles (Fig. 5). Two different rubber band sizes
were used, and exercises were initiated from the larger to the
base. Anterior cranial base and mandibular lengths were smaller size. Once chewing ability improved, swallowing
found to be within normal range. However, an inclined exercises were established and slowly progressed from
mandibular plane, rotated clockwise, resulted in mandib- liquids to soft and solid foods. Once this exercise sequence
ular retrusion, generating open bite, a lowered positioned was concluded, speech therapy was instituted to improve
chin, and an increased lower facial height. In spite of the language and breathing. The patient’s mother was guided
and asked to perform home supervision of exercises in
predominance of vertical growth, proclined maxillary and
order to collaborate with treatment. She was asked to
mandibular incisors were noted. The first permanent
gradually introduce solids at daily meals. Thus, in addition
molars were well positioned in the anterior posterior
to a 45-minute weekly therapy session, 10-minute home
direction, and excessive eruption of mandibular incisors
daily exercises were prescribed as part of the child’s routine.
was present. However, despite the lack of generalized
Initially, preventive measures and periodontal treatment
interdental contact, supraeruption of other teeth was not
were instituted with great difficulty, using behavior
observed (Table 1). Soft-tissue abnormality was noted,
guidance techniques in an attempt to desensitize patient
specifically, the lips surpassed the facial esthetic plane and and to achieve a better control of vomiting and gag reflex.
there was a prominent lower lip. At age 15, a hand and wrist When the patient was 15 years old, myofunctional therapy
evaluation using the Eklöf Rinjertz index identified a bone was initiated. After 4 months of therapeutic sessions and a
age of 9 years and 5 months, indicating a 6-year growth 16-month follow-up, a significant increase in tongue, lip,
delay (Fig. 4). and facial muscle tonus was achieved, allowing patient to
The speech-language pathologists functional evaluation chew solid foods and facilitating dental treatment, which
of the patient’s respiration, chewing, and swallowing consisted of re-examination, six sessions of scaling, root
showed an atypical deglutition, superior respiration, planing, dental prophylaxis, and oral hygiene instructions.
hypotonic facial muscles, lip incompetence, and lack of (Fig. 6A and 6B). Cephalometric analysis demonstrated a
dental occlusion. Myofunctional therapy was instituted to decrease in lower facial height and an anticlockwise
improve function of the stomatognathic system; muscle mandibular rotation, thus reducing the mandibular plane
posture; and mobility of the lips, tongue, cheeks, and soft angle (Figs. 7 and 8). A decrease in facial convexity was also
palate. Another aim was to prepare facial musculature for noted (Table 1). In addition, a reduction in the previously
future orthognathic surgery and orthodontic treatment. To observed prominent lower lip led to improvement of the
achieve these goals, awareness and training of the functions facial esthetic plane. The mother reported great improve-
were conducted. Therefore, exercises for the lips, tongue, ment in the patient’s feeding ability and gag reflex. The
 Fonteles et al., CONSERVATIVE MANAGEMENT OF OPEN BITE IN NOONAN SYNDROME 245

TABLE 1 Ricketts-Simplified Cephalometric Analysis of Patient With Noonan Syndrome, Before and After Myofunctional Therapy

Cephalometric Measurements Before 13 y 5 mo After 16 y 8 mo Norm SD

Dental
Interincisal angle8 115.10 104.19** 130 610
Maxillary-mandibular relation
Convexity (mm) 5.25 5 2 62
Inferior facial height8 58.28** 52.63 47 64
Teeth/osseous bases
Maxillary molar position (mm) 15.23 19.56 Age þ 3 63
Mandibular incisor protrusion (mm) 6.11** 6.88** 1 62
Inclination of mandibular incisor 24.78 32.48** 22 64
Esthetics
Labial protrusion (mm) 8.38** 4.05** 2 62
Craniofacial relation
Facial depth (8) 83.48 86.96 87 63
Facial axis (8) 87.01 92.23 90 63
Facial cone (8) 66.02 66.69 68 63.5
Mandibular plain angle (8) 30.50 26.34 26 64
Maxillary depth (8) 88.92 92.36 90 63
Internal structures
Cranial deflection (8) 21.35 22.42 27 63
Anterior cranial length (mm) 54.78 56.61 55 62.5
Mandibular arch (8) 31.74 23.83 26 64
Mandibular body length (mm) 65.00 68.00 65 62.7
* Patient’s age in years þ 3 mm. ** Values over 2 standard deviations (SD) above or below the norm.

patient has been on monthly follow-up visits for mainte-

nance of periodontal health and daily home oral care.

DISCUSSION

In 2007, Shaw et al. reported a long-term follow-up of

112 patients with Noonan syndrome. The authors found
that dental caries was a common cause of dental morbidity,
with 15% of patients requiring extractions due to caries,
whereas periodontal disease with significant bone loss and
dental mobility was previously reported by Torres-Carmo-
na et al. (1991). We have found no carious lesions
associated with this patient, whereas periodontal disease
with generalized bone loss was a significant finding. Most
descriptions of dental abnormalities related to Noonan
syndrome refer to dental malocclusions, high arched palate,

FIGURE 4 Hand and wrist radiograph showing growth delay at 15 FIGURE 5 Rubber band between the maxillary and mandibular
years of age. posterior teeth while patient exercised masticatory movements.
246 Cleft Palate-Craniofacial Journal, March 2013, Vol. 50 No. 2

FIGURE 6 A: Right and B: Left occlusal view after myofunctional

therapy (16-month follow-up), showing open bite improvement.

micrognathia, and anterior open bite as common charac-

teristics (Torres-Carmona et al., 1991; Bertola et al., 1999;
Tartaglia and Gelb, 2005; van der Burgt, 2007). In contrast FIGURE 8 Superimposition of cephalometric tracings, before (black)
and after (gray) myofunctional therapy, showing decreased lower facial
to previous case descriptions (Pierpont, 1996; Ogawa et al., height, anticlockwise mandibular rotation, and reduced mandibular plane
2004; Tartaglia and Gelb, 2005), a flattened palate was angle.
observed. We have reported on a patient with Noonan
syndrome in whom cephalometric analysis demonstrated contributed to the severe skeletal open bite of our patient.
vertical growth, mandibular retrusion, increased lower No other clinical reports have mentioned such a finding.
facial third, proclined incisors, and skeletal open bite. These Masticatory function is of the utmost importance for an
findings concur with previous reports (Okada et al., 2003). acceptable dentofacial development; for instance, its
Apparently, hypo-developed muscles, a current finding, has absence has been proposed to lead to dental crowding;
disrupted dental eruption, which would generate an altered
anterior-inferior facial height; and abnormal vertical
growth (Profitt and Fields, 2000). Feeding difficulties in
Noonan syndrome usually manifest during infancy, but
there is remarkable improvement after the age of 3 or 4
years (Shah et al., 1999). It has been suggested that these
abnormalities are probably an additional feature of
developmental delay. Thus, when present, they could be
considered an early marker of delayed language develop-
ment and long-term educational achievement (Shaw et al.,
2007). In this patient, reported feeding difficulties have
persisted into adolescence, but there have been no
descriptions of symptoms other than extreme sensitivity
in the oral cavity with strong gag reflex and inability to
chew solid foods, which frequently leads to immediate
vomiting. To our knowledge, there are no reports in the
literature of feeding problems associated with Noonan
FIGURE 7 Cephalometric radiograph showing a decrease in lower
syndrome in adolescent patients. We believe that severe
facial height, an anti-clockwise mandibular rotation, and reduced open bite, when associated with feeding difficulties, could
mandibular plane angle. possibly exacerbate such a condition. Hence, masticatory
 Fonteles et al., CONSERVATIVE MANAGEMENT OF OPEN BITE IN NOONAN SYNDROME 247

and speech problems have been attributed to this skeletal Hypersensitive gag reflex is a somatic natural response in
and/or dental discrepancy (Ngan, 1997). which a body reaction leads to muscle contraction at the
Changes in functional patterns may cause deviations in base of the tongue and the pharyngeal wall in an attempt to
craniofacial development, resulting in facial skeletal remove instruments or agents from the oral cavity (Bassi et
discrepancies and dental malocclusions (Proffit and Fields, al., 2004). Routine dental care in patients with strong gag
2000). Craniofacial muscles perform a series of functions reflex is usually a challenge for both the patient and the
involving head movement, posture, chewing, swallowing, dental professional, generating increased anxiety that must
speech, and facial expression. For instance, a reduction in be overcome. Interestingly, in the case described here,
muscle tone, a feature of many syndromes, may allow myofunctional therapy also led to a significant reduction in
excessive displacement of the jaws, rendering a significant gag reflex, allowing routine oral hygiene procedures to be
vertical growth, excessive eruption of the posterior easily performed. Apparently, improvement of the stoma-
dentition, and severe open bite (Kiliaridis et al., 1989). tognathic system function and muscle posture helped the
Therefore, alterations in these functions have the potential patient deprogram a previously acquired behavior pattern,
to establish changes in the facial skeleton and the which is the main goal in such cases (Wilks and Marks,
development of occlusion. Open bite has been defined as 1983; Ramsay et al., 1987). A pharmacologic approach
an open vertical dimension between the incisal edges of the using nitrous oxide (Kaufman et al., 1988) or intravenous
upper and lower dentition (Subtelney and Sakuda, 1964). sedation with propofol (Yoshida et al., 2007) has been
Although vertical malocclusion is an abnormality of a described as successfully depressing strong gag reflex in
multifactorial nature, variations in growth intensity, anxious young adult patients. However, in the current case
function of the soft tissues and jaw musculature, and report, gagging was not solely associated with dental
individual dentoalveolar development seem to influence the treatment, but rather a limiting factor for routine tooth
evolution of open bite problems (Ngan and Fields, 1997). brushing and feeding, so we believed a slower, more
Orofacial myofunctional disorders are specific conditions conservative approach, with behavior guidance as the main
or behaviors that can negatively affect oral postures and focus, seemed more appropriate. This treatment strategy
functions, changing the vertical rest posture dimension of showed marked improvement in quality of life and
the interdental arch (Mason, 2005). Myofunctional therapy concomitantly facilitated dental treatment. Orthodontic
is a treatment procedure designed to establish new
treatment was not considered during this stage for the
neuromuscular patterns, promoting correction of function-
reasons stated previously. Clearly, the patient would benefit
al and resting postures; improving chewing, swallowing,
from full orthodontic appliances and orthognathic surgery
and feeding patterns; and eliminating deleterious behaviors
for complete correction of skeletal and dental malocclu-
(Benkert, 1997). We have chosen a conservative approach
sions, including the remaining skeletal open bite. The extent
with myofunctional therapy as a means for increasing
of treatment will depend on the family’s decision and the
muscle tonus in order to regain occlusion, with improve-
patient’s ability to cooperate and maintain good oral
ment in masticatory ability as the final outcome. The
hygiene. Muscle hypotonia is a factor that increases relapse
effectiveness of treatment was measured through cephalo-
in anterior open bite cases, and caution should be taken if
metric analysis, as has been previously described (Haruki et
orthognathic surgery is not considered. Also, the patient
al., 1999).
was identified as having skeletal growth delay, and any
In the present case report, myofunctional therapy led to a
change in the mandibular rotation pattern, reducing treatment, as well as timing, should take this factor into
mandibular plane angle and lower facial height and account.
favoring reduction of the clinically observed open bite.
These favorable skeletal changes allowed establishment of CONCLUSION
masticatory function and resulted in great improvement in
feeding disorder associated with the syndrome. Further- Myofunctional therapy has demonstrated acceptable
more, therapeutic measures favored lip competence, efficacy in the management of a patient with Noonan
allowing closure. In agreement with the observed outcome, syndrome with feeding difficulties, hypersensitive gag reflex,
it has been suggested that orofacial myofunctional therapy and severe open bite associated with muscle hypotony,
can lead to improvement of open bite and overjet in the successfully rehabilitating masticatory function after a 16-
absence of prior or concomitant orthodontic treatment month follow-up.
(Benkert, 1997). There is an understanding that orthodontic
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