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Managing Symptoms: Standard of Care for BWS

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Managing Beckwith-Wiedemann syndrome (BWS) focuses on supportive care for newborn hypoglycemia, feeding assistance for an enlarged tongue (macroglossia), and surgical repair for abdominal wall defects. Most physical symptoms improve with standard medical care or as the child grows.

Key Takeaways

  • • Neonatal hypoglycemia is common in newborns with BWS but usually resolves within a few days with extra feeding or IV fluids.
  • • An enlarged tongue (macroglossia) often requires no medical intervention as the child's jaw grows to accommodate it over the first few years of life.
  • • Tongue reduction surgery is only necessary if macroglossia causes severe sleep apnea, feeding failure, or extreme jaw malformation.
  • • Abdominal wall defects like omphalocele are typically corrected through primary or staged surgery shortly after birth.

Managing the physical symptoms of Beckwith-Wiedemann Syndrome (BWS) focuses on stabilizing blood sugar, supporting feeding and breathing, and repairing abdominal wall defects. While every child’s journey is different, knowing what to expect can help you navigate the daily care of your child [1].

Managing Neonatal Hypoglycemia

Hearing that your newborn has neonatal hypoglycemia (low blood sugar) can be terrifying, but it is important to know that for the vast majority of babies with BWS, this is temporary [2]. About half of babies with BWS produce too much insulin right after birth, but this often resolves within a few days with extra support [2].

  • Standard Care: Most babies only require frequent feedings or high-glucose intravenous (IV) fluids in the hospital until their bodies adjust [3][4]. Once stabilized and sent home, you usually do not need to perform daily finger pricks unless instructed by your endocrinologist.
  • Persistent Cases: For a very small number of infants (often those with the pUPD11 subtype), the low blood sugar persists [5]. Doctors may prescribe diazoxide, an oral medication, to reduce insulin production [6].
  • Severe Cases: Extremely rarely, if medications fail, a partial pancreatectomy (surgery on the pancreas) may be required, but this is an absolute last resort [4][7].

Addressing Macroglossia (Enlarged Tongue)

Macroglossia is a hallmark of BWS [8]. For many children, the tongue simply appears large but does not cause major health issues. As your child’s jaw grows over the first few years of life, the tongue often fits better, requiring no medical intervention [9].

At-Home Management

If your baby’s tongue makes feeding difficult, you can work with a speech-language pathologist or feeding specialist. They may recommend special longer nipples or specific bottle types that make it easier for your baby to latch and swallow without choking [10][11].

When is Surgery Considered?

Tongue reduction surgery (partial glossectomy) is not a standard preventative measure for every child [9]. It is only considered if the large tongue causes severe, non-resolving functional issues, such as:

  • Obstructive Sleep Apnea (OSA): Severe snoring, gasping, or pauses in breathing during sleep [12][13].
  • Severe Feeding/Speech Failure: Inability to eat safely or articulate words [10][14].
  • Severe Dental/Jaw Malformation: Extreme forward growth of the lower jaw [15].

If surgery is deemed medically necessary, it is often performed between 12 and 24 months of age to prevent permanent changes to the jaw [15][16]. When required, it is generally a safe procedure with high parental satisfaction and excellent functional outcomes [10][17].

Repairing Abdominal Wall Defects

Many children with BWS are born with an omphalocele—an opening in the abdominal wall where some organs develop in a sac outside the body [18].

  • Surgical Repair: This is corrected through surgery, which can happen shortly after birth (primary closure) or in stages if the opening is large (staged reduction) [19][20].
  • Monitoring: Smaller defects, like an umbilical hernia, may simply be monitored to see if they close on their own as the child grows [21].

Frequently Asked Questions

Will my baby with BWS always have low blood sugar?
For most babies with Beckwith-Wiedemann syndrome, low blood sugar is temporary and resolves within a few days with extra feeding or IV fluids. Only a small number of infants need medication like diazoxide to manage persistent low blood sugar.
Does every child with macroglossia need tongue reduction surgery?
No, tongue reduction surgery is not a standard preventative measure. It is only considered if the enlarged tongue causes severe functional issues like obstructive sleep apnea, speech or feeding failure, or extreme jaw malformation.
How can I help my baby feed if they have an enlarged tongue?
Working with a feeding specialist or speech-language pathologist can be very helpful. They may recommend special longer nipples or specific bottle types that make it easier for your baby to latch and swallow safely without choking.
What is an omphalocele and how is it treated?
An omphalocele is an opening in the abdominal wall where some organs develop in a sac outside the body. It is typically corrected through surgery, which can happen shortly after birth or in stages if the opening is particularly large.

Questions for Your Doctor

  • • Is my child's hypoglycemia completely resolved, or do we need to continue any monitoring at home?
  • • Are there special bottles or feeding techniques that could help my baby manage their macroglossia?
  • • Does my child's macroglossia meet the criteria for severe functional impairment (like severe sleep apnea) that would warrant a surgical consultation?
  • • Does my child's omphalocele require immediate surgical closure or a staged approach?
  • • Who should I contact if I notice my baby becoming excessively jittery or lethargic?

Questions for You

  • • Have I noticed any pauses in my child's breathing or heavy snoring during sleep that I should report to the doctor?
  • • How is my child's feeding progressing at home? Am I struggling with lip seal or excessive drooling?
  • • Am I keeping a clear log of my child's symptoms to share with their specialists?

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References

  1. 1

    [Beckwith-Wiedemann Syndrome (BWS) Current Status of Diagnosis and Clinical Management: Summary of the First International Consensus Statement].

    Elbracht M, Prawitt D, Nemetschek R, et al.

    Klinische Padiatrie 2018; (230(3)):151-159 doi:10.1055/a-0591-9479.

    PMID: 29660755
  2. 2

    Bridging the Gaps: Multidisciplinary and Dental Strategies for Beckwith-Wiedemann Syndrome Management.

    Sharma K, Jamdade A, Yadav SP, Aggarwal A

    International journal of clinical pediatric dentistry 2024; (17(6)):702-705 doi:10.5005/jp-journals-10005-2873.

    PMID: 39391147
  3. 3

    Rare association of Beckwith-Wiedemann syndrome with Hirschsprung's disease in an infant with hypoglycemia.

    Shah N, Khadilkar A, Khadilkar V, Lad S

    BMJ case reports 2020; (13(4)) doi:10.1136/bcr-2020-235121.

    PMID: 32341092
  4. 4

    Genetic characteristics of patients with congenital hyperinsulinism.

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    Current opinion in pediatrics 2018; (30(4)):568-575 doi:10.1097/MOP.0000000000000645.

    PMID: 29750770
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    Determinants of Hyperinsulinism Severity in Children with Beckwith-Wiedemann Syndrome.

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    The Journal of clinical endocrinology and metabolism 2026; doi:10.1210/clinem/dgag053.

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    Necrotizing enterocolitis following diazoxide therapy for persistent neonatal hypoglycemia.

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    Journal of pediatric surgery case reports 2020; (52()) doi:10.1016/j.epsc.2019.101356.

    PMID: 32161713
  7. 7

    Congenital hyperinsulinism in children with paternal 11p uniparental isodisomy and Beckwith-Wiedemann syndrome.

    Kalish JM, Boodhansingh KE, Bhatti TR, et al.

    Journal of medical genetics 2016; (53(1)):53-61 doi:10.1136/jmedgenet-2015-103394.

    PMID: 26545876
  8. 8

    (Epi)genotype-phenotype correlations of Beckwith-Wiedemann syndrome in China.

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    Italian journal of pediatrics 2025; (51(1)):276 doi:10.1186/s13052-025-02122-4.

    PMID: 41024278
  9. 9

    Conservative Management of Macroglossia in Beckwith-Wiedemann Syndrome.

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    Pediatrics 2025; (155(4)) doi:10.1542/peds.2024-068618.

    PMID: 40068827
  10. 10

    Tongue Reduction Surgery and Feeding Difficulties in Infants With Beckwith Wiedemann Syndrome: A Case Series.

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    The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association 2019; (56(5)):679-689 doi:10.1177/1055665618794070.

    PMID: 30111162
  11. 11

    Partial Glossectomy Combined With Radiofrequency Ablation for Macroglossia in Beckwith-Wiedemann Syndrome.

    Lan D, Gao X, Zhang S, et al.

    The Journal of craniofacial surgery 2023; (34(2)):650-655 doi:10.1097/SCS.0000000000009018.

    PMID: 36168118
  12. 12

    The Peripheral Reduction With Keyhole Tongue Reduction Technique for Macroglossia in Beckwith-Wiedemann Syndrome.

    Romeo DJ, Massenburg BB, Wilson AT, et al.

    The Journal of craniofacial surgery 2024; doi:10.1097/SCS.0000000000010621.

    PMID: 39248722
  13. 13

    Improved Quality of Life for Children With Beckwith-Wiedemann Syndrome Following Tongue Reduction Surgery.

    Harada T, Yamanishi T, Kurimoto T, Nishio J

    The Journal of craniofacial surgery 2019; (30(1)):163-166 doi:10.1097/SCS.0000000000004823.

    PMID: 30376501
  14. 14

    Beckwith-Wiedemann Syndrome: Open bite evolution after tongue reduction.

    Alonso-Rodriguez E, Gómez E, Martín M, et al.

    Medicina oral, patologia oral y cirugia bucal 2018; (23(2)):e225-e229 doi:10.4317/medoral.21319.

    PMID: 29476667
  15. 15

    Outcomes of Tongue Reduction Surgery in Beckwith-Wiedemann Syndrome: A Systematic Review.

    Abraha B, MacIntyre O, Brennan H, et al.

    The Journal of craniofacial surgery 2025; (36(2)):542-546 doi:10.1097/SCS.0000000000011045.

    PMID: 39774467
  16. 16

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

    Wagner CS, Pontell ME, Barrero CE, et al.

    Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery 2023; (51(9)):568-573 doi:10.1016/j.jcms.2023.07.006.

    PMID: 37599200
  17. 17

    Reduction Glossectomy for Macroglossia in Beckwith-Wiedemann Syndrome: Is Post-Op Intubation Necessary?

    Geisler EL, Jeffers J, Salhi S, Perlyn CA

    The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association 2022; (59(1)):126-131 doi:10.1177/1055665621991739.

    PMID: 33550827
  18. 18

    Prenatal imaging throughout gestation in Beckwith-Wiedemann syndrome.

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    PMID: 30784096
  19. 19

    Expert consensus document: Clinical and molecular diagnosis, screening and management of Beckwith-Wiedemann syndrome: an international consensus statement.

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    PMID: 29377879
  20. 20

    Omphalocele-What should we tell the prospective parents?

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  21. 21

    Clinical and Molecular Evaluation of Beckwith-Wiedemann Syndrome with the BWSICS Score.

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    PMID: 41230207

This page explains symptom management for Beckwith-Wiedemann syndrome for educational purposes only. Always consult your child's pediatric specialist for personalized medical advice and treatment plans.

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