Orthognathic surgery in a patient with Noonan's syndrome

Sugar, Adrian; Ezsias, A.; Bloom, A. L.; Morcos, Wafik E.

doi:10.1016/0278-2391(94)90454-5

Cited by 26 publications

(13 citation statements)

References 10 publications

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“…There (15,16). Sugar et al (17) described a patient with Noonan syndrome who had marked mandibular prognathism with an anterior open bite. Although the present patient showed a severe deep bite with a hyperdivergent pattern, this could have been attributable to congenitally missing teeth.…”

Section: Discussionmentioning

confidence: 99%

Noonan syndrome: a case report

Emral

Akçam

2009

J Oral Sci

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Cranio-dento-facial findings in a case of Noonan syndrome in a 13-year, 3-month-old male are reported. Oral examination of the patient revealed a narrow, high arched palate, malocclusion and congenitally missing teeth. Even though cephalometric measurements showed an increased vertical facial pattern (GoGn/SN = 38.46 degrees ), a severe deep bite (9 mm) was evident. Noonan syndrome is characterized by ocular, facial, cardiac, and dental anomalies, although it is often unrecognized by clinicians. Although differential diagnosis of this syndrome is difficult, the cranio-dento-facial structures show specific findings that can be diagnosed by a dental specialist. The present report describes a new case of this syndrome from a dental viewpoint.

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Section: Discussionmentioning

confidence: 99%

Noonan syndrome: a case report

Emral

Akçam

2009

J Oral Sci

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“…Of 45 studies identified, 1 , 4 – 6 , 8 – 48 31 4 – 6 , 8 – 35 had relevant data from 428 patients with NS ( Figure 1 ). Of the 31 studies included, 13 were single case studies, five included <10 patients, and 13 included >10 patients.…”

Section: Methods and Resultsmentioning

confidence: 99%

Evaluation of bleeding disorders in patients with Noonan syndrome: a systematic review

Nugent¹,

Romanò²,

Sabharwal³

et al. 2018

JBM

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BackgroundNoonan syndrome (NS) is an autosomal dominant genetic condition that has a number of clinical features, including bleeding diathesis and a number of hematological abnormalities including clotting factor deficiencies, von Willebrand disease and abnormal platelet count/function.MethodsWe evaluated the frequency/types of bleeding disorders, and associated hematological laboratory findings, in patients with NS, using published data from 1965 to 2014.ResultsOf 45 studies identified, 31 included data for 428 patients with NS. Of these patients, 43% had reported bleeding, 26% had no reported bleeding and no bleed data was reported for 31%. Most patients (90%) had bleeding-related laboratory test abnormalities, but only 194 (45%) had a confirmed diagnosis of a specific bleeding disorder. Abnormal laboratory tests included: prolonged prothrombin time, activated partial thromboplastin time, and other platelet-related disorders. Of the 194 patients with a confirmed diagnosis of a specific bleeding disorder, 153 (79%) had single clotting factor deficiencies, von Willebrand disease or platelet-related disorders, and 41 (21%) had multiple deficiencies including platelet-related disorders.ConclusionAs patients with NS can experience multiple bleeding disorders, including abnormal platelet function, clinical evaluations should be performed at diagnosis, after diagnosis, before any surgery is undertaken, and if patients become symptomatic.

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“…The patient presents with many common NS features such as shortness of stature, down-slanting palpebral fissures, low set posteriorly rotated ears, full lips, a short neck, and his face shape is like an inverted triangle, wide at the forehead and tapered to a pointed chin (Figure 1). Furthermore he exhibits a high arched palate, [14][15][16][17] an anterior open bite tendency (edge to edge bite), [17][18][19] prognathic mandible [17][18][19][20] and dental malocclusion common in NS patients. [14][15][16][17][18][19] However, he also exhibits uncommon (or less reported) features such as severe localized gingivitis, supernumerary teeth, large inferior-alveolar canals and mental foramens, and he is negative for cardiac issues and bleeding disorders which are common for NS patients (Table II).…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore he exhibits a high arched palate, [14][15][16][17] an anterior open bite tendency (edge to edge bite), [17][18][19] prognathic mandible [17][18][19][20] and dental malocclusion common in NS patients. [14][15][16][17][18][19] However, he also exhibits uncommon (or less reported) features such as severe localized gingivitis, supernumerary teeth, large inferior-alveolar canals and mental foramens, and he is negative for cardiac issues and bleeding disorders which are common for NS patients (Table II). 2 In contrast to our patient with localized severe gingivitis around the canines and first premolars, most dental case reports of NS patients were negative for severe gingivitis 17,18,20,21 Only two articles reported periodontal issues: Ortega et al 22 noted generalized gingival inflammation in two adolescent cases (although the intraoral pictures did not show much gingival inflammation); Sugar et al 19 indicated periodontal problems that precluded their 22 Patient data is represented by black dots; he started GH treatment before the first dot at age 12 years and 10 months.…”

Section: Discussionmentioning

confidence: 99%

Atypical Orofacial Conditions in Noonan Syndrome: A Case Report

Toureno

Park

2011

Journal of Clinical Pediatric Dentistry

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Noonan syndrome (NS) is a relatively common condition characterized by chest deformation, congenital heart disease, short stature and distinctive facial features. Due to its genetic heterogeneity NS patients exhibit a range of clinical signs. Severe gingivitis and supernumerary teeth are rarely seen in connection with NS. In addition, there has not been a report on NS patients with atypical bilateral enlargement of the mental foramens and inferior-alveolar canals. This case report describes a NS patient who has undergone growth hormone (GH) therapy and is presenting with classical and rare NS phenotypes.

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Orthognathic surgery in a patient with Noonan's syndrome

Cited by 26 publications

References 10 publications

Noonan syndrome: a case report

Noonan syndrome: a case report

Evaluation of bleeding disorders in patients with Noonan syndrome: a systematic review

Atypical Orofacial Conditions in Noonan Syndrome: A Case Report

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