Nonmalignant renal disease in pediatric patients with Beckwith-Wiedemann syndrome

Peter L. Choyke, Marilyn J. Siegel, Orhan Oz, Cirilio Sotelo-Avila, Michael R. Debaun

Research output: Contribution to journalArticle

46 Citations (Scopus)

Abstract

OBJECTIVE. The objective of this retrospective review was to determine the incidence and spectrum of nonmalignant renal disease in patients with Beckwith-Wiedemann syndrome. MATERIALS AND METHODS. Patient records were obtained from the Beckwith-Wiedemann Registry of the National Cancer Institute. Imaging findings and medical records of 152 neonates, infants, children, and adults with Beckwith-Wiedemann syndrome (age range, 1 day to 30 years old; median age, 1 year 3 months old) were retrospectively reviewed by three radiologists. Available pathologic material also was reviewed. RESULTS. Thirty-eight (25%) of 152 patients with Beckwith-Wiedemann syndrome had 45 nonmalignant renal abnormalities, including medullary renal cysts (n = 19, 13%), caliceal diverticula (n = 2, 1%), hydronephrosis (n = 18, 12%), and nephrolithiasis (n = 6, 4%). Thirty-three (87%) of the 38 patients with nonmalignant renal disease were asymptomatic. Clinical manifestations of the remaining five patients included urinary tract infections (n = 4) and flank pain due to obstructive stone disease (n = 1). Nonmalignant renal disease was mistaken for Wilms' tumor in two patients, resulting in unnecessary nephrectomies. Seven children (18%) had Wilms' tumor and nonmalignant renal disease. CONCLUSION. Nonmalignant renal abnormalities occur in approximately 25% of patients with Beckwith-Wiedemann syndrome but are generally asymptomatic. Nonmalignant renal abnormalities should be considered in the differential diagnosis of a mass revealed during screening sonography of a patient with Beckwith-Wiedemann syndrome to avoid unnecessary surgery.

Original languageEnglish (US)
Pages (from-to)733-737
Number of pages5
JournalAmerican Journal of Roentgenology
Volume171
Issue number3
StatePublished - Sep 1998

Fingerprint

Beckwith-Wiedemann Syndrome
Pediatrics
Kidney
Wilms Tumor
Flank Pain
Unnecessary Procedures
Asymptomatic Diseases
Nephrolithiasis
National Cancer Institute (U.S.)
Hydronephrosis
Diverticulum
Nephrectomy
Urinary Tract Infections
Medical Records
Registries
Cysts
Ultrasonography
Differential Diagnosis
Newborn Infant

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology

Cite this

Choyke, P. L., Siegel, M. J., Oz, O., Sotelo-Avila, C., & Debaun, M. R. (1998). Nonmalignant renal disease in pediatric patients with Beckwith-Wiedemann syndrome. American Journal of Roentgenology, 171(3), 733-737.

Nonmalignant renal disease in pediatric patients with Beckwith-Wiedemann syndrome. / Choyke, Peter L.; Siegel, Marilyn J.; Oz, Orhan; Sotelo-Avila, Cirilio; Debaun, Michael R.

In: American Journal of Roentgenology, Vol. 171, No. 3, 09.1998, p. 733-737.

Research output: Contribution to journalArticle

Choyke, PL, Siegel, MJ, Oz, O, Sotelo-Avila, C & Debaun, MR 1998, 'Nonmalignant renal disease in pediatric patients with Beckwith-Wiedemann syndrome', American Journal of Roentgenology, vol. 171, no. 3, pp. 733-737.
Choyke, Peter L. ; Siegel, Marilyn J. ; Oz, Orhan ; Sotelo-Avila, Cirilio ; Debaun, Michael R. / Nonmalignant renal disease in pediatric patients with Beckwith-Wiedemann syndrome. In: American Journal of Roentgenology. 1998 ; Vol. 171, No. 3. pp. 733-737.
@article{da931716526f442891e5b8e5274d1eae,
title = "Nonmalignant renal disease in pediatric patients with Beckwith-Wiedemann syndrome",
abstract = "OBJECTIVE. The objective of this retrospective review was to determine the incidence and spectrum of nonmalignant renal disease in patients with Beckwith-Wiedemann syndrome. MATERIALS AND METHODS. Patient records were obtained from the Beckwith-Wiedemann Registry of the National Cancer Institute. Imaging findings and medical records of 152 neonates, infants, children, and adults with Beckwith-Wiedemann syndrome (age range, 1 day to 30 years old; median age, 1 year 3 months old) were retrospectively reviewed by three radiologists. Available pathologic material also was reviewed. RESULTS. Thirty-eight (25{\%}) of 152 patients with Beckwith-Wiedemann syndrome had 45 nonmalignant renal abnormalities, including medullary renal cysts (n = 19, 13{\%}), caliceal diverticula (n = 2, 1{\%}), hydronephrosis (n = 18, 12{\%}), and nephrolithiasis (n = 6, 4{\%}). Thirty-three (87{\%}) of the 38 patients with nonmalignant renal disease were asymptomatic. Clinical manifestations of the remaining five patients included urinary tract infections (n = 4) and flank pain due to obstructive stone disease (n = 1). Nonmalignant renal disease was mistaken for Wilms' tumor in two patients, resulting in unnecessary nephrectomies. Seven children (18{\%}) had Wilms' tumor and nonmalignant renal disease. CONCLUSION. Nonmalignant renal abnormalities occur in approximately 25{\%} of patients with Beckwith-Wiedemann syndrome but are generally asymptomatic. Nonmalignant renal abnormalities should be considered in the differential diagnosis of a mass revealed during screening sonography of a patient with Beckwith-Wiedemann syndrome to avoid unnecessary surgery.",
author = "Choyke, {Peter L.} and Siegel, {Marilyn J.} and Orhan Oz and Cirilio Sotelo-Avila and Debaun, {Michael R.}",
year = "1998",
month = "9",
language = "English (US)",
volume = "171",
pages = "733--737",
journal = "American Journal of Roentgenology",
issn = "0361-803X",
publisher = "American Roentgen Ray Society",
number = "3",

}

TY - JOUR

T1 - Nonmalignant renal disease in pediatric patients with Beckwith-Wiedemann syndrome

AU - Choyke, Peter L.

AU - Siegel, Marilyn J.

AU - Oz, Orhan

AU - Sotelo-Avila, Cirilio

AU - Debaun, Michael R.

PY - 1998/9

Y1 - 1998/9

N2 - OBJECTIVE. The objective of this retrospective review was to determine the incidence and spectrum of nonmalignant renal disease in patients with Beckwith-Wiedemann syndrome. MATERIALS AND METHODS. Patient records were obtained from the Beckwith-Wiedemann Registry of the National Cancer Institute. Imaging findings and medical records of 152 neonates, infants, children, and adults with Beckwith-Wiedemann syndrome (age range, 1 day to 30 years old; median age, 1 year 3 months old) were retrospectively reviewed by three radiologists. Available pathologic material also was reviewed. RESULTS. Thirty-eight (25%) of 152 patients with Beckwith-Wiedemann syndrome had 45 nonmalignant renal abnormalities, including medullary renal cysts (n = 19, 13%), caliceal diverticula (n = 2, 1%), hydronephrosis (n = 18, 12%), and nephrolithiasis (n = 6, 4%). Thirty-three (87%) of the 38 patients with nonmalignant renal disease were asymptomatic. Clinical manifestations of the remaining five patients included urinary tract infections (n = 4) and flank pain due to obstructive stone disease (n = 1). Nonmalignant renal disease was mistaken for Wilms' tumor in two patients, resulting in unnecessary nephrectomies. Seven children (18%) had Wilms' tumor and nonmalignant renal disease. CONCLUSION. Nonmalignant renal abnormalities occur in approximately 25% of patients with Beckwith-Wiedemann syndrome but are generally asymptomatic. Nonmalignant renal abnormalities should be considered in the differential diagnosis of a mass revealed during screening sonography of a patient with Beckwith-Wiedemann syndrome to avoid unnecessary surgery.

AB - OBJECTIVE. The objective of this retrospective review was to determine the incidence and spectrum of nonmalignant renal disease in patients with Beckwith-Wiedemann syndrome. MATERIALS AND METHODS. Patient records were obtained from the Beckwith-Wiedemann Registry of the National Cancer Institute. Imaging findings and medical records of 152 neonates, infants, children, and adults with Beckwith-Wiedemann syndrome (age range, 1 day to 30 years old; median age, 1 year 3 months old) were retrospectively reviewed by three radiologists. Available pathologic material also was reviewed. RESULTS. Thirty-eight (25%) of 152 patients with Beckwith-Wiedemann syndrome had 45 nonmalignant renal abnormalities, including medullary renal cysts (n = 19, 13%), caliceal diverticula (n = 2, 1%), hydronephrosis (n = 18, 12%), and nephrolithiasis (n = 6, 4%). Thirty-three (87%) of the 38 patients with nonmalignant renal disease were asymptomatic. Clinical manifestations of the remaining five patients included urinary tract infections (n = 4) and flank pain due to obstructive stone disease (n = 1). Nonmalignant renal disease was mistaken for Wilms' tumor in two patients, resulting in unnecessary nephrectomies. Seven children (18%) had Wilms' tumor and nonmalignant renal disease. CONCLUSION. Nonmalignant renal abnormalities occur in approximately 25% of patients with Beckwith-Wiedemann syndrome but are generally asymptomatic. Nonmalignant renal abnormalities should be considered in the differential diagnosis of a mass revealed during screening sonography of a patient with Beckwith-Wiedemann syndrome to avoid unnecessary surgery.

UR - http://www.scopus.com/inward/record.url?scp=0031753750&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0031753750&partnerID=8YFLogxK

M3 - Article

C2 - 9725306

AN - SCOPUS:0031753750

VL - 171

SP - 733

EP - 737

JO - American Journal of Roentgenology

JF - American Journal of Roentgenology

SN - 0361-803X

IS - 3

ER -