Matos Rojas Irma,  Katekaru Tokeshi Doris

Final Pr. ID: Poster #: CR-06 (R)

OEIS complex is the most severe end of the expectrum of the exstrophy - epispadias complex . It is characterized by omphalocele, extrophy, imperforate anus and spinal defects and is often associated with other malformations on chest, abdomen genitourinary, skeletal and neurologic. The incidence of OEIS complex is very rare, estimated to occur in 0, 5 – 1 per 200 000 live births.
We present a case of OEIS complex associated with horseshoe kidney. The baby was delivered by vaginal delivery (GA 39 weeks). Present normal male kariotype.
On physical examination there were omphalocele with herniation of a segment of the large bowel, cloacal exstrophy with two hemi bladders, imperforate anus and spina bifida. No external genitalia were identified on physical examination but bilateral cryptorchidism was observed in pelvic MRI
Renal ultrasound show hoershoe kidney with left pelvic ectasia that was confirmed on abdominal MRI and urotomography. Spinal ultrasound and MRI show lipomyelomenigocele and low spinal cord insertion. Also the baby has sacral segmentation defects and congenital hip subluxation. Read More

Authors:  Matos Rojas Irma , Katekaru Tokeshi Doris

Keywords:  OEIS complex, horseshoe kidney, extrophy - epispadias complex, magnetic resonance imaging, ultrasound

Sala Simone,  Cirillo Marco,  Raimondi Edoardo,  Bassi Matteo

Final Pr. ID: Poster #: CR-016

The incidence of upper genitourinary anomalies in children has been reported up to 4-6% and the association of cryptorchidism, hypospadias, double collecting system or fused kidney with Wilms’ tumor is well known. Horseshoe kidney is a rare anomaly of fusion occurring between 4^th and 6^th weeks of gestation. The association of Wilms’ tumor with horseshoe kidney is uncommon, with limited cases described. The incidence varies from 0.4% to 0.9% of all Wilms’ tumor. Rare variants of Wilms’ tumor like extrarenal teratoid tumor have also been described in children with horseshoe kidney. We describe two cases of Wilms’ tumor arising from horseshoe kidney, detected on US and later confirmed on CE-CT with low-dose imaging protocol. Both cases underwent surgical resection of the abnormal kidney and the diagnosis of Wilms’tumor was confirmed on microscopic examination of the excised tumor. In presence of kidney abonormalities imaging surveillance and follow-up evaluation is mandatory in order to grant an early detection of neoplastic evolution.
In our experience, multislice CT allows complete preoperative evaluation of this rare condition mainly using multiplanar reconstructions and may facilitate surgery. Read More

Authors:  Sala Simone , Cirillo Marco , Raimondi Edoardo , Bassi Matteo

Keywords:  wilms, horseshoe kidney