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Joseph Junewick, MD FACR
over 7 years ago
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Twin-Twin Renal Dysgenesis

Case Detail

Anatomy: Genitourinary
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Vascular
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: US
Activities:
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History

Premature infant with oliguria.


Case Images


Diagnosis

Twin-Twin Renal Dysgenesis

Clinical Notes

Recipient of twin-twin transfusion.

Findings

US – Markedly disordered renal echogenicity; note the increased cortical echogenicity and ring-like echogenicity in the medullary pyramids. Mild hydronephrosis is also present.

Discussion

Twin-twin transfusion syndrome occurs in approximately 15% of monochorionic diamniotic gestations and is characterized by placental shunting of blood from a small growth-restricted donor to a large plethoric recipient. This mismatched circulation has a significant impact on the developing visceral organs of both the donor and the recipient.

With volume overload, congestive heart failure may ensue following birth when the recipient is forced to maintain an independent circulation. In a similar fashion, the kidneys undergo changes in response to the shift in blood flow with enlargement and increased urine output from the recipient kidney. In contrast, the donor kidney is smaller than expected, even when adjusted for growth restriction, and has a variably reduced urine output. With the loss of urine output and the resultant oligohydramnios, a cascade of events occurs in the donor leading to Potter sequence.

Donors tended to demonstrate a paucity of proximal tubules with crowding of glomeruli characteristic of renal tubular dysgenesis. The degree of dysgenesis is associated with more severe growth restriction. Donors in twin-twin transfusion are at risk for the development of renal tubular dysplasia. Ischemia has been suggested as the underlying cause of renal tubular dysplasia although hypoperfusion leading to decreased glomerular filtration may also be responsible.

Reference

Oberg KC, Pestaner JP, Bielamowicz L, Hawkins EP. Renal tubular dysgenesis in twin-twin transfusion syndrome. Pediat Devel Pathol (1999); 2:25-32.



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