Beckwith Wiedemann Syndrome C0004903
A genetic overgrowth disorder affecting multiple body systems. It causes accelerated growth in infancy, enlarged tongue, and abdominal wall defects. Children require regular tumor screening and supportive care.
Key facts at a glance
- ICD-10
- Q89.9
- ICD-11
- LA00.0
- Body system
- congenital
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Omphalocele
Hypoglycemia
Generalized symptoms
(congenital)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Macrosomia
(growth)Nephromegaly
(renal)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Tumor Surveillance Program
Regular abdominal ultrasound screening and serum alpha-fetoprotein monitoring to detect Wilms tumor and hepatoblastoma early. Typically performed every 3 months until age 8 years.
Macroglossia Surgical Correction
Surgical reduction of tongue size to improve breathing, feeding, speech, and dental alignment. May involve anterior wedge resection or posterior reduction techniques.
Additional treatment options
Hypoglycemia Management
Dietary modifications and frequent feeding schedules to prevent neonatal hypoglycemia. May include complex carbohydrate-rich foods and coordinated feeding times.
dietaryAbdominal Wall Defect Repair
Surgical reconstruction for omphalocele or diastasis recti when medically indicated. Timing depends on defect size and associated complications.
procedureDevelopmental and Orthodontic Monitoring
Regular dental and orthodontic evaluations to address malocclusion, jaw development, and speech concerns related to macroglossia and accelerated growth patterns.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Beckwith Wiedemann Syndrome C0004903?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Shuman C, Kalish JM, Weksberg R. Clinical management of Beckwith-Wiedemann syndrome. Am J Med Genet C Semin Med Genet. 2020.
Choufani S, Shuman C, Weksberg R. Beckwith-Wiedemann syndrome. Am J Med Genet C Semin Med Genet. 2010.
Mussa A, Ferrero GB, Caiazzo T. Multicenter study of hemihyperplasia and tumor risk. J Pediatr. 2016.
Brioude F, Kalish JM, Mussa A. Clinical and molecular diagnosis and tumor surveillance in BWS. Nat Rev Endocrinol. 2018.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy