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The Utility of Early Tongue Reduction Surgery for Macroglossia in Beckwith-Wiedemann Syndrome.
Plast Reconstr Surg. 2020 04; 145(4):803e-813e.PR

Abstract

BACKGROUND

Macroglossia, a cardinal feature of the (epi)genetic disorder Beckwith-Wiedemann syndrome, is associated with obstructive sleep apnea, speech and/or feeding difficulties, and dental or jaw malalignment. These sequelae may be treated and/or prevented with tongue reduction surgery; the authors sought to determine whether certain Beckwith-Wiedemann syndrome patients may benefit from early surgical intervention before age 12 months.

METHODS

The authors conducted a retrospective review of patients with Beckwith-Wiedemann syndrome who underwent tongue reduction from 2014 to 2019. The authors assessed primary outcomes of change in obstructive sleep apnea by polysomnography, respiratory support required, and feeding route before and after tongue reduction, and reviewed postoperative complications and the need for repeated tongue reduction.

RESULTS

Of the 36 patients included, the median age at tongue reduction was 9.5 months (interquartile range, 3.8 to 22.8 months). For those with severe obstructive sleep apnea, there was a significant reduction in the obstructive apnea hypopnea index from 30.9 ± 21.8 per hour to 10.0 ± 18.3 per hour (p =0.019) and improvement in nadir oxyhemoglobin saturation from 72 ± 10 percent to 83 ± 6 percent (p =0.008). Although there was no significant change in overall supplemental feeding tube or respiratory support, there were specific patients who experienced clinically meaningful improvement. Of note, these positive outcomes applied equally to those who underwent surgery at a younger age (<12 months). To date, only one patient required a repeated tongue reduction.

CONCLUSION

Based on improved polysomnographic findings and rarity of surgical complications or repeated surgery, the authors' data support the safety and efficacy of this early intervention when clinical indications are present and an experienced multidisciplinary team is available for consultation.

CLINICAL QUESTION/LEVEL OF EVIDENCE

Therapeutic, IV.

Authors+Show Affiliations

From the Divisions of Human Genetics, Pulmonary Medicine, and Plastic and Reconstructive Surgery, Children's Hospital of Philadelphia; and the Departments of Pediatrics and Surgery, Perelman School of Medicine at the University of Pennsylvania.From the Divisions of Human Genetics, Pulmonary Medicine, and Plastic and Reconstructive Surgery, Children's Hospital of Philadelphia; and the Departments of Pediatrics and Surgery, Perelman School of Medicine at the University of Pennsylvania.From the Divisions of Human Genetics, Pulmonary Medicine, and Plastic and Reconstructive Surgery, Children's Hospital of Philadelphia; and the Departments of Pediatrics and Surgery, Perelman School of Medicine at the University of Pennsylvania.From the Divisions of Human Genetics, Pulmonary Medicine, and Plastic and Reconstructive Surgery, Children's Hospital of Philadelphia; and the Departments of Pediatrics and Surgery, Perelman School of Medicine at the University of Pennsylvania.From the Divisions of Human Genetics, Pulmonary Medicine, and Plastic and Reconstructive Surgery, Children's Hospital of Philadelphia; and the Departments of Pediatrics and Surgery, Perelman School of Medicine at the University of Pennsylvania.From the Divisions of Human Genetics, Pulmonary Medicine, and Plastic and Reconstructive Surgery, Children's Hospital of Philadelphia; and the Departments of Pediatrics and Surgery, Perelman School of Medicine at the University of Pennsylvania.From the Divisions of Human Genetics, Pulmonary Medicine, and Plastic and Reconstructive Surgery, Children's Hospital of Philadelphia; and the Departments of Pediatrics and Surgery, Perelman School of Medicine at the University of Pennsylvania.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32221229

Citation

Cohen, Jennifer L., et al. "The Utility of Early Tongue Reduction Surgery for Macroglossia in Beckwith-Wiedemann Syndrome." Plastic and Reconstructive Surgery, vol. 145, no. 4, 2020, pp. 803e-813e.
Cohen JL, Cielo CM, Kupa J, et al. The Utility of Early Tongue Reduction Surgery for Macroglossia in Beckwith-Wiedemann Syndrome. Plast Reconstr Surg. 2020;145(4):803e-813e.
Cohen, J. L., Cielo, C. M., Kupa, J., Duffy, K. A., Hathaway, E. R., Kalish, J. M., & Taylor, J. A. (2020). The Utility of Early Tongue Reduction Surgery for Macroglossia in Beckwith-Wiedemann Syndrome. Plastic and Reconstructive Surgery, 145(4), 803e-813e. https://doi.org/10.1097/PRS.0000000000006673
Cohen JL, et al. The Utility of Early Tongue Reduction Surgery for Macroglossia in Beckwith-Wiedemann Syndrome. Plast Reconstr Surg. 2020;145(4):803e-813e. PubMed PMID: 32221229.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The Utility of Early Tongue Reduction Surgery for Macroglossia in Beckwith-Wiedemann Syndrome. AU - Cohen,Jennifer L, AU - Cielo,Christopher M, AU - Kupa,Jonida, AU - Duffy,Kelly A, AU - Hathaway,Evan R, AU - Kalish,Jennifer M, AU - Taylor,Jesse A, PY - 2020/3/30/entrez PY - 2020/3/30/pubmed PY - 2020/7/9/medline SP - 803e EP - 813e JF - Plastic and reconstructive surgery JO - Plast Reconstr Surg VL - 145 IS - 4 N2 - BACKGROUND: Macroglossia, a cardinal feature of the (epi)genetic disorder Beckwith-Wiedemann syndrome, is associated with obstructive sleep apnea, speech and/or feeding difficulties, and dental or jaw malalignment. These sequelae may be treated and/or prevented with tongue reduction surgery; the authors sought to determine whether certain Beckwith-Wiedemann syndrome patients may benefit from early surgical intervention before age 12 months. METHODS: The authors conducted a retrospective review of patients with Beckwith-Wiedemann syndrome who underwent tongue reduction from 2014 to 2019. The authors assessed primary outcomes of change in obstructive sleep apnea by polysomnography, respiratory support required, and feeding route before and after tongue reduction, and reviewed postoperative complications and the need for repeated tongue reduction. RESULTS: Of the 36 patients included, the median age at tongue reduction was 9.5 months (interquartile range, 3.8 to 22.8 months). For those with severe obstructive sleep apnea, there was a significant reduction in the obstructive apnea hypopnea index from 30.9 ± 21.8 per hour to 10.0 ± 18.3 per hour (p =0.019) and improvement in nadir oxyhemoglobin saturation from 72 ± 10 percent to 83 ± 6 percent (p =0.008). Although there was no significant change in overall supplemental feeding tube or respiratory support, there were specific patients who experienced clinically meaningful improvement. Of note, these positive outcomes applied equally to those who underwent surgery at a younger age (<12 months). To date, only one patient required a repeated tongue reduction. CONCLUSION: Based on improved polysomnographic findings and rarity of surgical complications or repeated surgery, the authors' data support the safety and efficacy of this early intervention when clinical indications are present and an experienced multidisciplinary team is available for consultation. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV. SN - 1529-4242 UR - https://www.unboundmedicine.com/medline/citation/32221229/The_Utility_of_Early_Tongue_Reduction_Surgery_for_Macroglossia_in_Beckwith_Wiedemann_Syndrome_ L2 - https://Insights.ovid.com/pubmed?pmid=32221229 DB - PRIME DP - Unbound Medicine ER -