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

Clinical Pathways Home Emergency ICU Inpatient Outpatient Specialty Care Primary Care

Goals and Metrics

Patient Education

Provider Resources

Related Pathways
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
Epic Chat PHL Genetics BWS

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

BWS Clinical Features (use .bwschart to score)
Cardinal Features 2 pts for each feature	Suggestive Features 1 pt for each feature
Macroglossia Omphalocele Lateralized overgrowth/hemihypertrophy Wilms tumor or nephroblastomatosis Hyperinsulinism Adrenal cortex cytomegaly Placental mesenchymal dysplasia Pancreatic adenomatosis	Macrosomia (birth wt > 2 SD above mean) Facial nevus simplex Polyhydramnios/placentomegaly Ear creases/pits Transient Hypoglycemia, lasting < 1 wk Typical BWS tumors: Neuroblastoma Rhabdomyosarcoma Unilateral Wilms tumor Hepatoblastoma Adrenocortical carcinoma Pheochromocytoma Nephromegaly/hepatomegaly Umbilical hernia and/or diastasis recti

Consult Genetics
If clinical score ≥ 4 or if only feature is lateralized overgrowth,
send Epic Secure Chat to PHL Genetics BWS

Obtain AFP and Abdominal Ultrasound

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

Evaluation and Treatment
Subspecialty Consults

Prioritize Endocrine, Plastic Surgery, and Pulmonology Consults
Genetic Testing

Discharge and Case Management
Complete safety fast and sleep study prior to discharge

Posted: August 2020
Last Revised: March 2024

Authors:
- J. Kalish, MD;
- J. Lioy, MD;
- M. Hill, MD;
- K. Smith, CRNP;
- E. Hathaway, LCGC;
- S. Nesbitt, RN;
- M. Byrne, CRNP;
- L. Voss, LCGC

Editors: Clinical Pathways Team

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.