Uterine didelphys with concomitant renal anomalies in both m
Published in the Journal of Pediatric Surgery Case Reports, the authors present a case of didelphys uterus with a left solitary kidney in a mother whose female fetus also demonstrated uterine didelphys with right multicystic dysplastic kidney.

This report highlights the significant contribution and complementary role of fetal MRI in identifying fetal anomalies.

A 31-year-old, G2P0010 female presented at 25 weeks gestation to the Colorado Fetal Care Center (CFCC) for consultation regarding a fetal genitourinary anomaly detected on routine prenatal ultrasound (US). The 20-week US could not identify the right kidney and raised concern for unilateral renal agenesis on the right. On follow-up, the ultrasound demonstrated a low-lying, enlarged, and echogenic right kidney. The left kidney was enlarged but with normal echo texture. This case was compounded by the fact that oligohydramnios, bilateral clubfeet, and bilateral multicystic dysplastic kidneys resulting in Potter sequence complicated the mother's first pregnancy.

A later microarray revealed trisomy 16. Moreover, on prenatal US the mother was found to have uterine didelphys, which led to a renal US demonstrating an absent right kidney with compensatory hypertrophy of the left kidney. Given the prior fetal history and current renal concerns, further evaluation of the current pregnancy was sought at the CFCC.

Upon referral, repeat US confirmed the previous findings, including a low-lying, echogenic right kidney. Fetal magnetic MRI was obtained and confirmed maternal didelphys uterus and an absent right kidney. The fetal right kidney was ectopic; specifically it was low-lying with transverse orientation with mild hydronephrosis and diffuse microcysts, consistent with multicystic dysplastic kidney. The left kidney and pelvis were duplicated, but no dilated or abnormal ureter was seen. Additionally, the fetal MRI demonstrated fetal uterine didelphys.

Genetic consultation revealed that the maternal family history was otherwise negative for known congenital anomalies of the kidney and urinary tract (CAKUT). The amniotic fluid index was normal and fetal lung development appeared normal. The mother was counseled on the pregnancy risks associated with uterine didelphys, including preterm birth and the potential for fetal mal-presentation.

With regards to the neonatal course, she was counseled for anticipated normal outcome of the fetus in the setting of duplex renal pelvis but not ureteral duplication. However it was discussed that complete duplication of the left ureter remained a potential possibility for which postnatal renal US, voiding cystourethrogram (VCUG), and renal function panel could further evaluate.

Major takeaways:-
- The diagnosis of uterine didelphys in a 25-week fetus has not been previously described, but it is now possible with the level of detail provided by fetal MRI.

- The maternal–fetal matching phenotype may suggest a possible inheritance pattern or genetic cause, rather than teratogenic or maternal influences.

- Finally, it is important to note the role of fetal MRI in this case. While fetal MRI is not indicated in the routine assessment of the fetal urinary and reproductive tract, when abnormalities are identified by ultrasound, fetal MRI is a welcome adjunctive study to further accurately delineate the genitourinary anatomy.

- In this case, the details of fetal microcystic dysplasia to the parenchyma of the entire right kidney, duplication of the left renal pelvis and didelphys uterus were added to anomalies found on ultrasound.

Read more here: https://pxmd.co/zHJWk