MARATTO

article · Dr Sulaiman Al Habib Medical Journal

A Rare Syndromic Coexistence: Bilateral Duplicated Collecting Systems with Bilateral Ureterovesical Junction Obstruction in a Child with Heterotaxy Syndrome and Polysplenia: A Case Report

In plain language

This report details a rare clinical case involving a two-year-old girl who presented with recurrent urinary tract infections, lower abdominal pain, and incontinence. Ultrasound and contrast-enhanced computed tomography urography revealed bilateral duplicated collecting systems alongside bilateral ureterovesical junction obstruction. Imaging also identified features of heterotaxy syndrome with polysplenia, which included a right-sided stomach, a hypoplastic pancreas, an elongated left liver lobe, and azygous continuation of the inferior vena cava with absent intrahepatic and infrahepatic segments. The patient exhibited normal cardiac and sigmoid colon positioning with no cardiac abnormalities. Following these findings, the patient was referred to paediatric urology and nephrology. The findings demonstrate the utility of cross-sectional imaging in detecting complex congenital anomalies and support a multidisciplinary approach to clinical care for patients with complex, co-occurring developmental conditions.

Key takeaways

  • A two-year-old patient presented with recurrent urinary tract infections, incontinence, and lower abdominal pain.
  • Cross-sectional imaging revealed bilateral duplicated collecting systems with ureterovesical junction strictures and moderate hydroureteronephrosis.
  • Imaging simultaneously confirmed heterotaxy syndrome with polysplenia, featuring a right-sided stomach, hypoplastic pancreas, and venous anomalies without cardiac involvement.
  • Comprehensive cross-sectional imaging and multidisciplinary referral are crucial for diagnosing and managing complex congenital anomalies.

Why it matters

Congenital anomalies involving multiple organ systems can produce complex clinical symptoms that are difficult to diagnose. By detailing the simultaneous occurrence of urinary tract duplications and heterotaxy syndrome, this case shows how advanced imaging techniques help clinicians identify multiple internal anatomical variations accurately, guiding appropriate specialist care and preventing potential complications.

Commercialisation angle

The abstract does not indicate an application pathway or potential commercial product, as it is a single clinical case report documenting diagnostic findings and specialist referrals.

AI-generated from the published abstract. Always read the original work before citing.

Abstract

Abstract The simultaneous occurrence of bilateral duplicated collecting systems and heterotaxy syndrome is extremely rare, with no previously reported cases from our setting to our knowledge. In this report, we present the case of a 2-year-old girl who experienced recurrent urinary tract infections, lower abdominal pain, and incontinence. An ultrasound revealed bilateral hydroureteronephrosis, and a contrast-enhanced computed tomography intravenous urography showed enlarged kidneys on both sides with duplicated collecting systems, moderate hydroureteronephrosis of the upper moiety, and dilated ureters due to stricture at the uretovasical junction. Additionally, the imaging revealed features consistent with heterotaxy syndrome (situs ambiguous) with polysplenia, including a right-sided stomach, hypoplastic pancreas, an elongated left liver lobe that extends laterally (beaver tail liver), a normal morphological variant of the liver, and azygous continuation of the inferior vena cava with absence of intrahepatic and infrahepatic segments. The heart and sigmoid colon were in their usual positions, with no associated cardiac abnormalities identified. The patient was referred to pediatric urology and nephrology for further evaluation. This case highlights the diagnostic value of cross-sectional imaging in identifying complex congenital anomalies and emphasizes the importance of a multidisciplinary approach in managing such rare presentations.

Research topics

  • Congenital Anomalies and Fetal Surgery
  • Pediatric Hepatobiliary Diseases and Treatments
  • Intestinal Malrotation and Obstruction Disorders

Read the original research

This page summarises published work. The authoritative version sits with the publisher.

DOI: 10.4103/dshmj.dshmj_51_25

Is something wrong with this record? Report it or request removal.

Discussion

Discuss this research

Have you built on this work, tried to replicate it, or seen it applied in practice? Share what you know. Verified researchers and MARATTO™ domain experts can open a discussion, and any member can reply. Contributions are reviewed before they appear.

No discussion yet. Open the first thread.