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Published on: November 1, 2018
OEIS complex with glomerulocystic kidney disease: a case report
Ran Hong1, Sung-Chul Lim, Jung-Whan Jang
1College of Medicine, Chosun University, Gwangju, Korea.
Summary
This case report details a rare OEIS complex (omphalocele, exstrophy of bladder, imperforated anus, spinal defect) in a newborn, complicated by colonic agenesis and glomerulocystic kidney disease.
Area of Science:
- Pediatric Surgery
- Medical Genetics
- Developmental Biology
Background:
- The OEIS complex is a rare congenital anomaly characterized by a specific set of defects.
- Glomerulocystic kidney disease (GCKD) is a distinct form of cystic kidney disease.
Observation:
- A male infant presented with omphalocele, exstrophy of bladder, imperforated anus, and spinal defect (OEIS complex).
- The infant also exhibited colonic agenesis and glomerulocystic kidney disease (GCKD), confirmed by histopathology.
- Prenatal ultrasound and postnatal imaging revealed spina bifida, hydroureter, polycystic kidney, and abdominal wall defects.
Findings:
- Surgical and histopathological findings confirmed the diagnosis of OEIS complex with associated colonic agenesis and GCKD.
- Surgical interventions included nephrectomy, colostomy, and abdominal wall repair.
- The patient had an uneventful postoperative course during 4 months of follow-up.
Implications:
- This case highlights the importance of comprehensive evaluation in OEIS complex, including renal and gastrointestinal anomalies.
- Understanding the co-occurrence of OEIS complex, colonic agenesis, and GCKD can aid in diagnosis and management.
- Further research into the genetic and developmental pathways underlying these combined conditions is warranted.
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