Beckwith-Wiedemann Syndrome: Open bite evolution after tongue reduction

Alonso-Rodríguez, Estefanía; Gómez, Elena; Martín, Mercedes; Muñoz, Jesús-Manuel; Hernández-Godoy, J.; Burgueño, Miguel

doi:10.4317/medoral.21319

Cited by 13 publications

(48 citation statements)

References 23 publications

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“…This leads us to hypothesize that the incidence of skeletal class III in patients with BWS is substantially lower than hitherto reported. Most of the data provided in the literature rely on single case reports or small case series involving a limited number of children [ 16 , 17 , 32 , 37 ]. Although macroglossia might stimulate mandibular growth, it is difficult to determine whether the prognathism of the mandibular basis is a result of macroglossia and dentoalveolar changes or if it is genetically driven [ 38 ].…”

Section: Discussionmentioning

confidence: 99%

“…Indications for tongue reduction surgery (TRS) have not been clearly defined. While some studies recommend early surgical treatment of macroglossia to prevent mandibular prognathism and open bite, others demonstrated that TRS does not control the pattern of mandibular growth and the development of class III skeletal malocclusion [ 16 , 17 , 18 , 19 ]. Indeed, the enlarged mandibular body observed in BWS might be caused by mandibular cartilaginous growth activated by IGF-2 expression, rather than being secondary to tongue overgrowth [ 20 ].…”

Section: Introductionmentioning

confidence: 99%

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Maxillo-Facial Morphology in Beckwith-Wiedemann Syndrome: A Preliminary Study on (epi)Genotype-Phenotype Association in Caucasians

Defabianis

Mussa

Ninivaggi

et al. 2022

IJERPH

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Beckwith–Wiedemann syndrome (BWS) is a congenital overgrowth disorder caused by various (epi)genetic alterations affecting the expression of genes on chromosome 11p15. Cardinal features include abdominal wall defects, macroglossia, and cancer predisposition. Several (epi)genotype–phenotype associations were described so far, but specific studies on the evolution over time of maxillo-facial phenotype in the molecular subtypes still are scanty. The aim of this cross-sectional study was to associate maxillo-facial morphology and growth pattern with genoype in 25 Caucasian children with BWS and macroglossia. Twelve patients experienced a loss of metilation at imprinting center 2 (IC2-LoM), five had mosaic paternal uniparental isodisomy of chromosome 11 (UPD(11)pat), and eight were negative. A more marked tongue enlargement was detected in patients with IC2-LoM and negative genotype, while UPD(11)pat children showed mild macroglossia (p = 0.048). A cluster analysis did not demonstrate any specific relationship between (epi)genotype and maxillo-facial phenotype, but separated BWS patients based on their cephalometric characteristics. Children with IC2-LoM or negative genotype displayed hyperdivergence values > 30°, clockwise growth tendency, and skeletal class II into the same cluster. They had a negative prognostic score. These preliminary data suggest the need for developing individualized protocols for early monitoring of the craniofacial growth in such patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Maxillo-Facial Morphology in Beckwith-Wiedemann Syndrome: A Preliminary Study on (epi)Genotype-Phenotype Association in Caucasians

Defabianis

Mussa

Ninivaggi

et al. 2022

IJERPH

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“…For most people with macroglossia, an oversized tongue can completely block the airways, leading to suffocation and sudden death ( Alonso-Rodriguez et al, 2018 ). Patients with macroglossia need closely monitored and lifesaving measures such as tracheotomy ( Junghaenel et al, 2012 ).…”

Section: Macroglossia Diseasementioning

confidence: 99%

The Oral Complications of COVID-19

Zhou

Dong

Guo

et al. 2022

Front. Mol. Biosci.

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Background: COVID-19 is a novel coronavirus infectious disease associated with the severe acute respiratory syndrome. More and more patients are being cured due to the development of clinical guidelines for COVID-19 pneumonia diagnosis, treatment, and vaccines. However, the long-term impact of COVID-19 on patients after recovery is unclear. Currently available reports have shown that patients recovered from COVID-19 continue to experience health problems in respiratory and other organ systems. Oral problem is one of the important complications which has serious impacts on the rehabilitation and future quality of life, such as ageusia and macroglossia, but the oral complication is often being neglected.Aim of Review: From the perspective of stomatology, we summarized and elaborated in detail the types, pathogenesis of oral complications from COVID-19 patients after rehabilitation, and the reported prevention or treatment recommendations which may improve the COVID-19 patients associated oral diseases.Key Scientific Concepts of Review: 1) To understand the common oral complications and the mechanisms of the development of oral complications after the COVID-19 recovery; 2) To summary the practical strategies to prevent the oral complications and construct the rehabilitation plans for patients with oral complications.

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“…Macroglossia in BWS becomes less noticeable with age and often requires no treatment; but it does cause problems for some children with BWS. In severe cases, macroglossia can cause respiratory, feeding, and speech difficulties may require surgery [20]. It is seen in about 90% of patients with BWS [7].…”

Section: ) Hepatoblastomamentioning

confidence: 99%

Beckwith-Wiedemann Syndrome: A Case Report at the Gynaeco-Obstetric and Pediatric Hospital in Yaounde, Cameroon

Tague¹,

Mah²,

Nguefack³

et al. 2020

AJP

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Beckwith-Wiedemann syndrome is a rare congenital syndrome, but one of the most common among overgrowth syndromes. It was described firstly by Beckwith in 1963. The incidence of BWS is about 1:13 700 births, with an equal sex distribution. It is characterized by macrosomia, macroglossia, omphalocele and anterior abdominal wall defects. It is a complex multigenic disorder caused by dysregulation of the gene printed on chromosome 11p. 15. A relationship has been established between assisted fertilization methods and its occurrence. This syndrome predisposes to childhood cancer. Its incidence is not known in developing countries such as Cameroon due to the rarity of reported cases, variability in the presentation of the syndrome, financial constraints and lack of access to genetic and molecular studies. In our opinion, we are reporting the first case of Beckwith-Wiedemann syndrome in our country. We present here the case of a newborn female baby delivered at the gyneco-obstetric and pediatric hospital in Yaoundé and admitted to our neonatology unit. The diagnosis was made on the basis of clinical signs suggestive of Beckwith-Wiedemann syndrome. She presented an omphalocele diagnosed on antenatal ultrasound, macrosomia, macroglossia and ear abnormalities. The case is presented to raise awareness and highlight the particularity of the management of this rare disease.

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Beckwith-Wiedemann Syndrome: Open bite evolution after tongue reduction

Cited by 13 publications

References 23 publications

Maxillo-Facial Morphology in Beckwith-Wiedemann Syndrome: A Preliminary Study on (epi)Genotype-Phenotype Association in Caucasians

Maxillo-Facial Morphology in Beckwith-Wiedemann Syndrome: A Preliminary Study on (epi)Genotype-Phenotype Association in Caucasians

The Oral Complications of COVID-19

Beckwith-Wiedemann Syndrome: A Case Report at the Gynaeco-Obstetric and Pediatric Hospital in Yaounde, Cameroon

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