Which syndrome is characterized by prune belly features, including hypotonic abdominal wall muscles, a dilated fetal bladder, a small thorax, and imperforate anus?

• A. Eagle-Barrett syndrome
• B. Limb body wall complex
• C. Meckel-Gruber syndrome
• D. Pentalogy of Cantrell

Answer: (A)

The situation describes prune belly syndrome, also known as Eagle-Barrett syndrome. This condition is classically defined by a trio of features: markedly lax or absent abdominal wall muscles (hypotonic abdomen), urinary tract anomalies leading to a dilated bladder, and often pulmonary complications that can manifest as a small thorax. Imperforate anus can also be associated. The combination of a weak abdominal wall with urinary tract dilation and potential bowel or anal malformations fits prune belly syndrome very well, which is why it’s the best match.

The other syndromes have different signature findings. Limb body wall complex involves extensive anterior body wall defects with limb abnormalities. Meckel-Gruber syndrome presents with neural tube defects like occipital encephalocele, polydactyly, and cystic kidneys. Pentalogy of Cantrell includes defects such as a midline diaphragmatic, thoracic, and abdominal wall defects with intracardiac anomalies and pericardial defects. These do not align with the prune belly triad described.