Unilateral renal agenesis and uterine anomalies: how and when can we make this diagnosis?

Joana Gaspar, Natália Ferreira, Luísa Lobo, Carla Simão


A 12-year-old female with a prenatal diagnosis of left kidney agenesis was hospitalized with a history of four months of recurrent abdominal pain and dysmenorrhea after menarche. Imaging investigation revealed a gynecological malformation associated with Müllerian developmental defects – duplicated uterus with an obstructed left hemi-uterus filled with hematic content (haematometra), one cervix and a normal vagina. We pretend to alert pediatric physicians about the association of renal agenesis and gynecological malformations, most frequently uterine, but also vaginal. Early detection of these malformations by imaging will anticipate therapeutic measures to relieve symptoms, mostly recurrent abdominal or pelvic pain, and to avoid complications such as endometriosis, pelvic adherences and infections.


Pediatrics; renal agenesis; uterine anomalies

Full Text:



Tozcan T. Prenatal diagnosis of renal agenesis. UpToDate. acessed in April 2015.

Gassner I, Geley TE. Ultrasound of female genital anomalies. Eur Radiol. 2004; 14:L107-22.

Iverson RE, DeCherney AH, Laufer MR. Clinical manifestations and diagnosis of congenital anomalies of the uterus. UpToDate. acessed in April 2015.

The American Fertility Society Classification of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, Müllerian anomalies and intrauterine adhesions. Fertil Steril 1988; 49:944-5.

Behr SC, Courtier JL, Qayyum A. Imaging of Müllerian duct anomalies. Radio Graphics. 2012; 32: E233-50.

Junqueira BLP, Allen LM, Spitzer RF, Lucco KL, Babyn PS, Doria AS. Müllerian duct anomalies and mimics in children and adolescents: correlative intraoperative assessment with clinical imaging. Radio Graphics. 2009; 29:1085–103.

Barakat AJ. Association of unilateral renal agenesis and genital anomalies. Case Rep Clin Pract Rev. 2002; 3: 57-60.

Kiechl-Kohlendorfer U, Geley T, Maurer K, Gassner I. Uterus didelphys with unilateral vaginal atresia: multicystic dysplastic kidney is the precursor of “renal agenesis” and the key to early diagnosis of this genital anomaly. Pediatr Radiol. 2011; 41:1112–6.

Riccabona M, Lobo ML, Willi U, Avni F, Damasio B, Ording-Mueller, et al. ESPR uroradiology taskforce and ESUR paediatric work group -Imaging recommendations in paediatricuroradiology, part VI: childhood renal biopsy and imaging of neonatal and infant genital tract. Minutes from the task force session at the annual ESPR Meeting 2012 in Athens on childhood renal biopsy and imaging neonatal genitalia. Pediatr Radiol. 2014; 44:496-502.

Hollander MH, Verdonk PV, Trap K. Unilateral Renal Agenesis and Associated Müllerian Anomalies: A Case Report and Recommendations for Pre-adolescent Screening. J Pediatr Adolesc Gynecol. 2008, 21:151-3.

Fedele L, Motta F, Frontino G, Restelli E, Bianchi S. Double uterus with obstructed hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases. Human Reproduction. 2013; 28; 6:1580-3.


  • There are currently no refbacks.

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

Journal indexed in