Lumbo-Costo-Vertebral Syndrome with Congenital Lumbar Hernia: Case Report
Beza Eshetu1, Teferi Mekonnen1, Melkamu Berhane1
1Department of Pediatrics and Child Health Jimma University, Jimma, Ethiopia.
Insights
Lumbo-costo-vertebral syndrome is a rare congenital condition affecting the spine, ribs, and abdominal wall. This case highlights associated organ malformations, emphasizing the need for tailored surgical repair.
Area of Science:
- Pediatric Surgery
- Medical Genetics
- Developmental Biology
Background:
- Lumbo-costo-vertebral syndrome is a rare congenital disorder characterized by abnormalities of the vertebral bodies, ribs, and abdominal wall.
- It represents a spectrum of developmental defects with potential for significant morbidity.
Observation:
- A 6-month-old female infant presented with a congenital, reducible right lumbar swelling.
- Diagnostic imaging revealed a lumbar defect containing bowel loops, scoliosis, hemivertebrae, absent right lower ribs, and a cross-fused kidney.
Findings:
- The case illustrates a complex presentation of Lumbo-costo-vertebral syndrome with associated gastrointestinal and renal malformations.
- Imaging confirmed a lumbar hernia sac containing abdominal organs and aberrant renal anatomy.
Implications:
- This case underscores the importance of thorough diagnostic evaluation for associated anomalies in Lumbo-costo-vertebral syndrome.
- Surgical management requires careful consideration of defect size and associated organ malformations for optimal outcomes.
Background:
Lumbo-costo-vertebral syndrome is a set of rare abnormalities involving vertebral bodies, ribs, and abdominal wall.
Case:
We present a case of Lumbo-costo-vertebral syndrome in a 6-month old female infant who had a progressive swelling over the right lumbar area since birth. Clinical examination revealed a reducible swelling on the right flank with positive cough impulse. Ultrasonography showed a defect containing bowel loops in the right lumbar region. Chest X-ray revealed scoliosis and hemivertebrae with absent lower ribs on the right side. Computer tomography scan showed hernia sac containing the bowel and the right lobe of the liver with cross fused kidney.
Conclusion:
Lumbo-costo-vertebraly syndrome is a rare condtion which could be associated with different organ malformations. Simple closure or meshplasty could be done depending on the size of the defect.
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