Beckwith-Wiedemann Syndrome
Pronunciation: BECK-with VEE-deh-mahn
A congenital overgrowth disorder characterized by macrosomia, macroglossia, and omphalocele, requiring multidisciplinary surgical management.
Full Definition
Beckwith-Wiedemann Syndrome is a complex congenital overgrowth disorder that presents with multiple features requiring surgical intervention. The classic triad includes macrosomia (large body size), macroglossia (enlarged tongue), and omphalocele (abdominal wall defect). Additional features may include hemihypertrophy, ear creases, and predisposition to embryonal tumors. Pediatric surgeons manage the omphalocele repair and may collaborate on tongue reduction procedures. The syndrome requires lifelong surveillance for tumor development, particularly Wilms tumor and hepatoblastoma.
Usage
Usage note: Always hyphenate the syndrome name; abbreviate as BWS in subsequent references.
In Context
- "The neonate presented with classic Beckwith-Wiedemann Syndrome features requiring immediate omphalocele repair." — Surgical case report
- "Beckwith-Wiedemann Syndrome patients require regular abdominal ultrasounds for tumor surveillance." — Clinical protocol