Journal of Pediatric Surgery Case Reports (Feb 2023)

A rare association between crossed fused renal ectopia, urethral stricture, bilateral cryptorchidism, and sub-coronal hypospadias in a non-syndromic 6-year-old child

  • Oadi N. Shrateh,
  • Afnan W.M. Jobran,
  • Saja Jaber,
  • Ahmad Kahla,
  • Mohammad Shker,
  • Wael Abu Arafeh

Journal volume & issue
Vol. 89
p. 102561

Abstract

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Introduction: From asymptomatic ectopic kidneys to potentially fatal renal agenesis, congenital abnormalities of the kidney and urinary tract (CAKUT) encompass a wide variety of anomalies (bilateral). With varied degrees of severity, many of them are found in the antenatal or immediately postnatal period, with a sizable fraction also found in the adult population. On the basis of embryology, CAKUT can be divided into three categories: defects in the formation of the renal parenchyma, aberrant embryonic migration, and abnormalities of the collecting system. Multiple cystic dysplastic kidneys, renal hypoplasia, number (agenesis or supernumerary), form, and cystic renal illnesses are examples of renal parenchymal abnormalities. Case presentation: A 6-year-old male patient was referred to the hospital complaining of painful and difficult urination. Past medical history was significant for recurrent urinary tract infections, with temporary improvement with antibiotic therapy. Physical assessment and imaging evaluation revealed a constellation of genitourinary anomalies, including sub-coronal hypospadias, penile urethral stricture, bilateral cryptorchidism, and a crossed fused ectopic left kidney. Discussion and conclusion: Congenital kidney and urinary tract anomalies are a group of several diseases that affect the kidney and/or urinary tract. Despite substantial differences in phenotypic and clinical consequences, CAKUT has a similar genetic foundation and shares molecular signals that influence kidney development. The prognosis and quality of life for affected families have improved thanks to advancements in prenatal diagnostics, imaging, genetic testing, laboratory surveillance, and medical management, as many of these congenital defects are inherited.

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