Related Experiment Video
Updated: Aug 6, 2026
Quantification of Levator Ani Hiatus Enlargement by Magnetic Resonance Imaging in Males and Females with Pelvic Organ Prolapse
Published on: April 17, 2019
Unilateral abdominal wall hypoplasia: radiographic findings in two infant girls
1Department of Radiology, Children's Hospital Medical Center, Cincinnati, OH 45229-3039, USA.
Insights
Pseudoprune belly syndrome (PPBS) affects girls with abdominal wall deficiency and genitourinary issues. Congenital CMV infection may contribute to PPBS and related complications.
Area of Science:
- Pediatric Medicine
- Medical Genetics
Background:
- Prune belly syndrome (PBS) is a rare congenital disorder characterized by abdominal wall deficiency, urinary tract anomalies, and cryptorchidism in males.
- While more common in males, PBS can occur in females, often presenting as pseudoprune belly syndrome (PPBS) with incomplete expression.
Observation:
- Two infant girls with PPBS were evaluated, presenting with unilateral abdominal wall hypoplasia.
- Associated anomalies included cardiac, genitourinary, gastrointestinal, pulmonary, and musculoskeletal involvement of varying severity.
Findings:
- One patient exhibited congenital cytomegalovirus (CMV) infection and focal jejunal hypoganglionosis.
- The co-occurrence suggests a potential link between CMV infection and the development of PPBS and aganglionosis.
Implications:
- This case highlights the variability in PPBS presentation and the potential role of infectious agents like CMV in its etiology.
- Further research is needed to elucidate the mechanisms by which CMV may induce these complex congenital anomalies.
Abstract:
Prune belly syndrome (PBS) is defined as an association of abdominal wall deficiency, genitourinary anomalies, and, in males, cryptorchism. Although PBS is more common in males, females can also have the condition. In both sexes, expression of the disease is often incomplete and prognosis depends upon the specific abnormalities present. PBS in girls or incomplete expression of PBS is called pseudoprune belly syndrome (PPBS). We recently evaluated two baby girls with PPBS. Both girls had unilateral abdominal wall hypoplasia associated with cardiac, genitourinary, gastrointestinal, pulmonary, and musculoskeletal involvement that ranged from normal to severe. One of the patients also demonstrated congenital cytomegalovirus (CMV) infection and focal jejunal hypoganglionosis. Since CMV has been associated with both aganglionosis and PBS, it is possible that CMV induced both conditions in this patient.
More Related Videos
Related Concept Videos
01:25Assessment of the Abdomen I: Inspection and Auscultation
The abdominal examination is a cornerstone of clinical medicine, serving as a critical tool in diagnosing various gastrointestinal (GI) diseases. It involves a systematic approach that includes inspection and auscultation, each with distinct yet complementary roles in assessing the abdomen. This article will delve into these two primary methods healthcare professionals use to examine the abdomen.
Inspection of the Abdomen
The first step in any abdominal examination is inspection.
01:20Imaging Studies for Cardiovascular System III: X-Ray
Definition and Purpose
An X-ray, or radiograph, is a non-invasive method that uses ionizing radiation to take images of internal structures. It is mainly used in cardiac imaging to examine the heart, lungs, and major blood vessels, aiming to identify abnormalities in the heart's size, shape, and position, such as heart failure, congenital defects, and vascular...
01:24Imaging Studies II: Ultrasonography