Beckwith-Wiedemann Syndrome Clinical Pathway — N/IICU | Children's Hospital of Philadelphia

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Persistent Hypoglycemia

General Questions
bwsclinic@chop.edu
267-425-2467

Research Questions
BWS@chop.edu

Neonates/Infants with Suspected Beckwith-Wiedemann Syndrome (BWS)

Care Prior to Transport or Admission to CHOP

Initial referral through CHOP Transport Call:
Notify BWS Team

Making the Diagnosis
History and Physical Examination
Assess for BWS Features
Genetic Testing

BWS Clinical Features
Cardinal Features	Suggestive Features
Omphalocele Macroglossia Lateralized overgrowth Hemihypertrophy Hemihyperplasia Hyperinsulinism Lasting > 1 week, requiring escalated treatment Multifocal and/or bilateral Wilms tumor or Nephroblastomatosis	Umbilical hernia and/or diastasis recti Birthweight > 2 SDS above the mean Polyhydramnios and/or placentomegaly Transient hypoglycemia, lasting < 1 week Typical BWS tumors, consult Genetics Neuroblastoma Rhabdomyosarcoma Unilateral Wilms tumor Hepatoblastoma Adrenocortical carcinoma Pheochromocytoma Facial nevus simplex Ear creases and/or pits Nephromegaly and/or hepatomegaly

Consult Genetics
- If ANY Cardinal or Multiple Suggestive Features
- Concern about possible BWS

Consult Case Management/Social Work
- Identify and Resolve Potential Barriers to Discharge

Evaluation and Treatment
Subspecialty Consults
Genetic Testing

Discharge and Case Management

Posted: August 2020
Revised: February 2023
Authors: J. Kalish, MD PhD; J. Lioy, MD; M. Hill, MD; K. Smith, CRNP; E. Hathaway, LCGC; S. Nesbitt, RN;
M. Byrne, CRNP; L. Voss, LCGC

Evidence

CHOP Programs

BWS Videos

Resources

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The clinical pathways are based upon publicly available medical evidence and/or a consensus of medical practitioners at The Children’s Hospital of Philadelphia (“CHOP”) and are current at the time of publication. These clinical pathways are intended to be a guide for practitioners and may need to be adapted for each specific patient based on the practitioner’s professional judgment, consideration of any unique circumstances, the needs of each patient and their family, and/or the availability of various resources at the health care institution where the patient is located.

Accordingly, these clinical pathways are not intended to constitute medical advice or treatment, or to create a doctor-patient relationship between/among The Children’s Hospital of Philadelphia (“CHOP”), its physicians and the individual patients in question. CHOP does not represent or warrant that the clinical pathways are in every respect accurate or complete, or that one or more of them apply to a particular patient or medical condition. CHOP is not responsible for any errors or omissions in the clinical pathways, or for any outcomes a patient might experience where a clinician consulted one or more such pathways in connection with providing care for that patient.