Characterization of the Beckwith‐Wiedemann spectrum: Diagnosis and management

KA Duffy, CM Cielo, JL Cohen… - American Journal of …, 2019 - Wiley Online Library
Beckwith‐Wiedemann syndrome (BWS) is the most common epigenetic overgrowth and
cancer predisposition disorder. Due to both varying molecular defects involving …

The utility of early tongue reduction surgery for macroglossia in Beckwith-Wiedemann syndrome

JL Cohen, CM Cielo, J Kupa, KA Duffy… - Plastic and …, 2020 - journals.lww.com
Background: Macroglossia, a cardinal feature of the (epi) genetic disorder Beckwith-
Wiedemann syndrome, is associated with obstructive sleep apnea, speech and/or feeding …

Positive airway pressure for the treatment of OSA in infants

CM Cielo, P Hernandez, AM Ciampaglia… - Chest, 2021 - Elsevier
Background Positive airway pressure (PAP) is a standard therapy for the treatment of OSA in
children, but objective data on the effectiveness of PAP in infants are sparse. The aim of this …

Updates on management of pediatric obstructive sleep apnea

JL Yu, O Afolabi-Brown - Pediatric Investigation, 2019 - mednexus.org
Obstructive sleep apnea (OSA) affects about 1%-5% of the pediatric population. The
consequences of untreated OSA in children include neurocognitive deficits, behavioral …

Respiratory indices during sleep in healthy infants: a prospective longitudinal study and meta-analysis

D Stefanovski, IE Tapia, J Lioy, S Sengupta… - Sleep medicine, 2022 - Elsevier
Study objectives Healthy infants may have a greater apnea hypopnea index (AHI) than older
children during the newborn period, but the trajectory of these sleep-related events beyond …

Associations between the timing of tongue reduction surgery,(Epi) genotype, and dentoskeletal development in patients with Beckwith-Wiedemann syndrome

CS Wagner, ME Pontell, CE Barrero… - Journal of Cranio …, 2023 - Elsevier
Tongue reduction surgery is often pursued to manage the adverse effects of macroglossia in
patients with Beckwith-Wiedemann syndrome (BWS). This study characterized dental …

Anterior “W” tongue reduction for macroglossia in Beckwith-Wiedemann syndrome

BY Ainuz, EL Geisler, RR Hallac… - The Cleft Palate …, 2022 - journals.sagepub.com
Introduction Macroglossia occurs in 80% to 99% of patients with Beckwith-Wiedemann
syndrome (BWS) and a variety of surgical techniques for tongue reduction are offered by …

The prevalence of difficult airway in children with Beckwith-Wiedemann syndrome: A retrospective cohort study

L Sequera-Ramos, KA Duffy, JE Fiadjoe… - Anesthesia & …, 2021 - journals.lww.com
BACKGROUND: Beckwith-Wiedemann syndrome (BWS) is the most common congenital
overgrowth disorder with an incidence of approximately 1 in 10,000 live births. The condition …

A Novel Macroglossia Severity Index for Beckwith-Wiedemann Syndrome

DJ Romeo, M Banala, M Wu, JJ Ng… - Annals of …, 2024 - journals.lww.com
Methods: Patients with molecularly confirmed BWS seen from 2004-2023 were included to
develop this system. Relationships among BIG scores, tongue reduction surgery, BWS …

Evaluation of keyhole-pattern reduction glossoplasty for macroglossia in beckwith-wiedemann syndrome: A multidimensional analysis of postoperative course and …

E Şimşekcan, G Sert, M Calis, F Özgür - Journal of Cranio-Maxillofacial …, 2024 - Elsevier
The aim of this study was to evaluate the postoperative course and long-term functional and
aesthetic outcomes in patients with Beckwith-Wiedemann syndrome (BWS) following …