Associated Urological Anomalies in Children With Unilateral Renal Agenesis

Cascio, Salvatore; Paran, Sri; Puri, Prem

doi:10.1097/00005392-199909000-00036

Cited by 34 publications

(50 citation statements)

References 14 publications

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“…The left kidney is more commonly absent in URA as compared to the right side, as was the finding in our case [3]. URA may be an isolated malformation or may be associated with anomalies of the cardiac, genital, skeletal, gastrointestinal, and respiratory systems [4]. In our case, there were no other anomalies in other systems other than the abdominal left testis which had developed a seminoma.…”

Section: Discussionsupporting

confidence: 74%

Seminoma Arising from Intra-abdominal Cryptorchidism with Ipsilateral Renal Agenesis: A Rare Association

Sk¹,

Ryntathiang²,

Ray³

et al. 2012

Indian J Surg

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Unilateral renal agenesis is usually asymptomatic and may be found incidentally during examination for other causes. The incidence of testicular tumors arising from cryptorchidism is well established, but if it is coexistent with ipsilateral renal agenesis, the diagnosis and management becomes a challenge. Only three cases of such association have been reported in literature so far. This association has to be kept in mind when dealing with a case of testicular tumor arising from abdominal cryptorchidism, so that the function of the other kidney can be assessed before surgery, and protection given to it in the event of using radiotherapy in an adjuvant setting.Keywords Seminoma . Intra-abdominal cryptorchidism . Unilateral renal agenesis Case ReportA 43-year-old man presented with the history of a progressively increasing lump in the left side of the abdomen for 4 months. On examination a 20×15 cm large, hard, intraabdominal lump was found involving the left hypochondrium, lumbar, and iliac fossa. The left testis was absent and the scrotum was poorly developed on the left side.Ultrasonography of the whole abdomen revealed a large, heterogeneous mass measuring 16×12 cm in the region of the left kidney. The right kidney and the ureter were normal. Contrast-enhanced computerized tomography (CECT) of the whole abdomen revealed a large variegated mass in the left lumbar region. No normal renal parenchyma and ureter could be identified. The right kidney and the ureter were normal. CECT findings were suggestive of a renal neoplasm, possibly renal cell carcinoma (Fig. 1a).Keeping in mind the possibility of the neoplasm arising from an abdominal cryptorchidism, tumor markers were done. Serum LDH (939 units/l) and β-HCG (10.43 mIU/ml) were raised, while serum AFP (3.25 ng/ml) was within normal limits. Thus, the diagnosis was further confounded, as the normal renal parenchyma and ureter were not visualized at all in either the normal or any ectopic position. A decision was taken to do a radionuclide renogram using Tc-99 m-DTPA. This confirmed the absence of the left kidney and ureter (Fig. 1b). Cystoscopy findings also failed to reveal the ureteric orifice on the left side further confirming absence of the left ureter.An exploratory laparotomy was performed. A huge mass was found arising from the retroperitoneum and adherent to surrounding structures (sigmoid colon and mesocolon). Instead of a definite vascular pedicle, leashes of vessels were seen all around the mass. The left kidney and ureter were not visualized separately. En bloc resection of the mass was done. No lymph nodes were seen.The resected specimen was 15.5 cm at its longest axis (Fig. 2a), with smooth surface and areas of necrosis (Fig. 2b). Histopathological examination gave the impression of a malignant tumor (seminoma) (Fig. 2c). The immunohistochemistry report revealed tumor cells expressing ckit (Fig. 2d) and PLAP. It was negative for CD 20 and CD 30. The final impression was that of testicular seminoma (pT2 pN0M0S2), stage IS.Postoperatively, t...

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Section: Discussionsupporting

confidence: 74%

Seminoma Arising from Intra-abdominal Cryptorchidism with Ipsilateral Renal Agenesis: A Rare Association

Sk¹,

Ryntathiang²,

Ray³

et al. 2012

Indian J Surg

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“…Bilateral agenesis is incompatible with life and is associated with pulmonary hypoplasia and limb defects. Unilateral renal agenesis is not uncommon, seen in 1/1300 pregnancies [11] . It is often asymptomatic and compensatory hypertrophy in other kidney may cause glomerulosclerosis in adults.…”

Section: Anomalies In Numbermentioning

confidence: 99%

Multi-modality imaging review of congenital abnormalities of kidney and upper urinary tract

Ramanathan¹,

Kumar²,

Khanna³

et al. 2016

WJR

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Congenital abnormalities of the kidney and urinary tract (CAKUT) include a wide range of abnormalities ranging from asymptomatic ectopic kidneys to life threatening renal agenesis (bilateral). Many of them are detected in the antenatal or immediate postnatal with a significant proportion identified in the adult population with varying degree of severity. CAKUT can be classified on embryological basis in to abnormalities in the renal parenchymal development, aberrant embryonic migration and abnormalities of the collecting system. Renal parenchymal abnormalities include multi cystic dysplastic kidneys, renal hypoplasia, number (agenesis or supernumerary), shape and cystic renal diseases. Aberrant embryonic migration encompasses abnormal location and fusion anomalies. Collecting system abnormalities include duplex kidneys and Pelvi ureteric junction obstruction. Ultrasonography (US) is typically the first imaging performed as it is easily available, noninvasive and radiation free used both antenatally and postnatally. Computed tomography (CT) and magnetic resonance imaging (MRI) are useful to confirm the ultrasound detected abnormality, detection of complex malformations, demonstration of collecting system and vascular anatomy and more importantly for early detection of complications like renal calculi, infection and malignancies. As CAKUT are one of the leading causes of end stage renal disease, it is important for the radiologists to be familiar with the varying imaging appearances of CAKUT on US, CT and MRI, thereby helping in prompt diagnosis and optimal management.

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“…Thus assessment of the relative importance of a reduced nephron endowment per se in the onset of adult disease is extremely difficult. In human patients with unilateral renal agenesis, in whom nephron endowment is reduced from birth, the incidence of renal insufficiency and proteinuria is common (2,61), and, in many cases, infants with one kidney also have contralateral renal abnormalities (18,35,62), the most common of which may further contribute to renal problems. Children with unilateral renal agenesis often develop elevated arterial pressure (73,95), although it is difficult to know whether this is directly due to a reduced nephron endowment.…”

Section: Assessing the Importance Of A Reduced Nephron Endowmentmentioning

confidence: 99%

Developmental programming of a reduced nephron endowment: more than just a baby's birth weight

Moritz¹,

Singh²,

Probyn³

et al. 2009

American Journal of Physiology-Renal Physiology

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Associated Urological Anomalies in Children With Unilateral Renal Agenesis

Cited by 34 publications

References 14 publications

Seminoma Arising from Intra-abdominal Cryptorchidism with Ipsilateral Renal Agenesis: A Rare Association

Seminoma Arising from Intra-abdominal Cryptorchidism with Ipsilateral Renal Agenesis: A Rare Association

Multi-modality imaging review of congenital abnormalities of kidney and upper urinary tract

Developmental programming of a reduced nephron endowment: more than just a baby's birth weight

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