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Renal cysts were found in 2% of pediatric autopsies. These often occurred with other congenital malformations, particularly esophageal atresia, suggesting a link between renal cystic disease and these conditions.
Area of Science:
- Pediatric Pathology
- Nephrology
- Congenital Malformations
Background:
- Renal cysts are a recognized pathology in pediatric populations.
- The association between renal cysts and other congenital anomalies requires further elucidation.
Purpose of the Study:
- To determine the incidence and types of renal cysts in infants and children.
- To investigate the co-occurrence of renal cysts with extrarenal malformations, focusing on gastrointestinal anomalies.
Main Methods:
- Retrospective analysis of 6,521 consecutive pediatric autopsies.
- Classification of identified renal cystic diseases.
- Documentation of associated extrarenal malformations.
Main Results:
- Renal cysts were identified in 136 cases (2%), with renal cystic dysplasia being the most common type.
- Extrarenal malformations were present in 102 cases (75%) with renal cysts.
- Esophageal atresia was significantly associated, found in 21% of cases with renal cysts.
Conclusions:
- Renal cystic diseases are not rare in pediatric autopsies and frequently accompany other congenital anomalies.
- The strong association with esophageal atresia highlights the importance of considering renal cystic disease in infants with gastrointestinal malformations.
Abstract:
In a series of 6,521 consecutive autopsies of infants and children, renal cysts were found in 136 cases (2%). Of these, 71 were females and 65 males; 103 patients had died in their first month of life. The different types of cystic disease represented were as follows: renal cystic dysplasia 65, cortical cysts in syndromes of multiple malformations 42, polycystic disease 16, simple cortical cysts 7, renal cysts in hereditary syndromes 3, and renal medullary cystic disorders 3. Extrarenal malformations were encountered in 102 of the 136 cases with renal cysts. Gastrointestinal malformations and in particular esophageal atresia very often appeared to be associated with renal disease. Esophageal atresia was found in 29 (21%) cases, 20 in cases with renal cystic dysplasia and 9 in cases with cortical cysts in syndromes of multiple malformations. The possibility of renal cystic disease should therefore be kept in mind in infants with congenital malformation(s), especially those with esophageal atresia.