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Published on: October 12, 2017
Upper urinary tract abnormalities: a case of bilateral kidneys within a left-sided omphalocele
Raffaella DeRosa1, John E Musser2, Veronica J Rooks3
1Division of Urology, Department of Surgery, Tripler Army Medical Center, Honolulu, HI.
Insights
This study reports the first case of bilateral kidney herniation into the omphalocele sac in a child with a severe form of birth defects. Understanding these rare anatomic variations is crucial for surgical planning and improved outcomes.
Area of Science:
- Pediatric Surgery
- Medical Imaging
- Developmental Biology
Background:
- Omphalocele, exstrophy, imperforate anus, and spinal defects complex represents a severe spectrum of congenital anomalies.
- Improved survival rates necessitate a thorough understanding of potential anatomical variations for optimal surgical management.
- While upper urinary tract anomalies are uncommon, kidney malposition has been previously documented.
Observation:
- This report details the first documented instance of bilateral kidney herniation into the omphalocele sac.
- The patient presented with the complex of omphalocele, exstrophy, imperforate anus, and spinal defects.
- Prenatal diagnosis of this complex can be challenging.
Findings:
- The kidneys were observed to have herniated bilaterally into the omphalocele sac.
- This represents a previously unreported anatomical manifestation within this complex.
- The upper urinary tract, apart from the herniation, was otherwise unremarkable.
Implications:
- This finding expands the known spectrum of renal anomalies associated with this complex.
- Accurate prenatal imaging and surgical planning are critical for managing such rare presentations.
- Further research into the embryological basis of these herniations may offer insights into prevention or treatment.
Abstract:
Children with omphalocele, exstrophy, imperforate anus, and spinal defects complex present with the most severe form of birth defects in the exstrophy-epispadias spectrum. Prenatal diagnosis is difficult, but improved survival over the past several decades makes understanding the potential anatomic manifestations imperative for expeditious and appropriate surgical care. The upper urinary tract is often normal in children with omphalocele, exstrophy, imperforate anus, and spinal defects complex, but malposition of one of the kidneys has previously been reported. We present the first case of bilateral kidney herniation into the omphalocele sac.
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