Diagnostic Ultrasound - Abdomen and Pelvis
Renal Ectopia TERMINOLOGY Abbreviations • Renal ectopia (RE), crossed fused ectopia (CFE) Definitions • Aberrant location of kidney • Separated into multiple categories, of which simple RE and CFE represent most common forms ○ Crossed nonfused RE, solitary crossed RE, and bilateral crossed RE represent rare variants IMAGING General Features • Best diagnostic clue ○ Absence of kidney in expected renal fossa ○ In both CFE and RE, affected kidney noted in abnormal location • Location ○ Normal renal position: Between transverse processes of T12-L3 ○ RE can range in location from pelvic (most common) to thoracic (rare) ○ Simple RE: Kidney located ipsilateral to its ureteral insertion ○ CFE: Kidney located contralateral to its ureteral insertion • Size ○ Ectopic kidneys vary in size Ultrasonographic Findings • Grayscale ultrasound ○ Simple RE – Aberrant location of kidney, from thorax to pelvis, with kidneys on contralateral sides – Ectopic kidney small and malrotated, often with dysmorphic features – Morphology described as "pancake," "disc," or "lump" – Collecting system located near surface of kidney (anterior orientation) □ Normal sinus echo complex absent or eccentrically positioned – Intrathoracic RE □ Mediastinal location □ Note intact diaphragm below kidney ○ CFE – Both kidneys located on same side of spine – Empty contralateral renal fossa – 90% of crossed ectopia involves fusion to normally located kidney □ Crossed nonfused RE rare – Left kidney more often ectopic than right – Ectopic kidney is malrotated □ Usually fusion of upper pole of ectopic kidney to lower pole of normally positioned kidney □ S-shaped mass with 2 renal sinuses □ While normal ipsilateral ureter enters ipsilateral trigone, ureter of ectopic kidney crosses midline and enters at contralateral trigone • Color Doppler ○ RE:Arterial supply from regional arteries – For example, pelvic kidney supplied by common or internal iliac arteries – Often with multiple arterial supply ○ CFE – Separate vascular supply to each kidney, with aberrant supply to ectopic kidney □ Aberrant arteries may cross ureter and cause obstruction – Ureteric jets from ureterovesical junctions located in their normal position – Color Doppler may aid in prenatal diagnosis Nuclear Medicine Findings • Can confirm location and assess function of ectopic kidney CT Findings • CECT ○ Arterial phase for optimal delineation of vascular supply in both CFE and RE ○ Excretory phase for ureteral course and number MR Findings • MR arteriography/urography ○ Can appreciate course of ureters without use of ionizing radiation or intravenous contrast ○ Dilated ureters easier to follow ○ Contrast-enhanced sequences can delineate vascular anatomy and allow for assessment of crossing vessels DIFFERENTIAL DIAGNOSIS Renal Transplant (Iatrogenic Ectopia) • Most common location for transplant is right lower quadrant • Atrophic echogenic kidneys appreciated in bilateral renal fossae • Renal vessels anastomosed to ipsilateral external iliac artery and vein Horseshoe Kidney • Fusion of lower poles of kidneys in low mid-abdomen • Isthmus connecting kidneys in midline: Fibrous or renal tissue Ptotic Kidney • Mobile, low-lying kidney • May mimic pelvic kidney • Low position due to poor fascial reinforcement Abdominal, Pelvic, or Thoracic Mass • Do not have characteristic renal morphology or function Acquired Renal Displacement • Secondary to mass effect from hepatomegaly, splenomegaly, or abdominal mass lesion • Autotransplantation for vascular diseases such as renal artery stenosis • Diaphragmatic hernia can result in acquired intrathoracic kidney PATHOLOGY General Features • Etiology Diagnoses: Urinary Tract 429
Renal Ectopia Diagnoses: Urinary Tract ○ Arrested migration during embryologic development ○ RE inherited as autosomal recessive trait; reported in monozygotic twins ○ CFE related to abnormal development of ureteric bud and metanephric blastema during 4th-8th weeks of development • Associated abnormalities ○ Other genitourinary abnormalities in ~ 1/2 of cases – RE □ Vesicoureteral reflux (most common associated abnormality) □ Contralateral renal abnormalities in up to 50% of RE such as renal agenesis □ Absent or hypoplastic vagina – CFE □ Megaureter, cryptorchidism, urethral valves, multicystic dysplasia ○ Occur in conjunction with anomalies in other organs in ~ 1/3 of cases – Skeletal (up to 50%) □ Rib and vertebral anomalies; scoliosis may impact renal ascent □ Absence of radius – Cardiovascular (40%) – Gastrointestinal (33%) □ Anorectal malformations such as imperforate anus – Ears, lips, palate (33%) □ Low-set or absent ears □ External canal atresia □ Cleft palate DIAGNOSTIC CHECKLIST Image Interpretation Pearls • Do not confuse RE with mass in pelvis or thorax • Do not confuse CFE with renal mass SELECTED REFERENCES 1. Solanki S et al: Crossed fused renal ectopia: challenges in diagnosis and management. J Indian Assoc Pediatr Surg. 18(1):7-10, 2013 2. Chang PL et al: Prenatal diagnosis of cross-fused renal ectopia: does color Doppler and 3-dimensional sonography help? J Ultrasound Med. 30(4):578- 80, 2011 3. Siegel MJ, ed. Pediatric Sonography. 4th edition. Baltimore: Lippincott Williams & Wilkins, 2011 4. Singer A et al: Spectrum of congenital renal anomalies presenting in adulthood. Clin Imaging. 32(3):183-91, 2008 5. Guarino N et al: The incidence of associated urological abnormalities in children with renal ectopia. J Urol. 172(4 Pt 2):1757-9; discussion 1759, 2004 6. Goodman JD et al: Crossed fused renal ectopia: sonographic diagnosis. Urol Radiol. 8(1):13-6, 1986 7. McCarthy S et al: Ultrasonography in crossed renal ectopia. J Ultrasound Med. 3(3):107-12, 1984 430 CLINICAL ISSUES Presentation • Most common signs/symptoms ○ Commonly asymptomatic, incidental finding ○ May present with signs and symptoms of obstruction, urolithiasis, reflux, and infection Demographics • Epidemiology ○ RE: Pelvic ectopia, most common – Noted in 1:500 to 1:1,200 ○ CFE represents 2nd most common fusion abnormality after horseshoe kidney – Male predominance, with incidence reported as M:F = 3:2 – Incidence reported ranging from 1:1,000 to 1:7,500 on autopsy studies ○ Crossed nonfused RE: Very rare, reported as 1:75,000 Natural History & Prognosis • Complications ○ ~ 50% have complications related to vesicoureteral reflux, hydronephrosis, stones, and infection – Repeated infections can lead to scarring ○ May have increased susceptibility to trauma ○ Increased incidence of multicystic dysplasia Treatment • Treat complications • No need to separate fused components
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Renal Ectopia<br />
Diagnoses: Urinary Tract<br />
○ Arrested migration during embryologic development<br />
○ RE inherited as autosomal recessive trait; reported in<br />
monozygotic twins<br />
○ CFE related to abnormal development of ureteric bud<br />
<strong>and</strong> metanephric blastema during 4th-8th weeks of<br />
development<br />
• Associated abnormalities<br />
○ Other genitourinary abnormalities in ~ 1/2 of cases<br />
– RE<br />
□ Vesicoureteral reflux (most common associated<br />
abnormality)<br />
□ Contralateral renal abnormalities in up to 50% of<br />
RE such as renal agenesis<br />
□ Absent or hypoplastic vagina<br />
– CFE<br />
□ Megaureter, cryptorchidism, urethral valves,<br />
multicystic dysplasia<br />
○ Occur in conjunction with anomalies in other organs in ~<br />
1/3 of cases<br />
– Skeletal (up to 50%)<br />
□ Rib <strong>and</strong> vertebral anomalies; scoliosis may impact<br />
renal ascent<br />
□ Absence of radius<br />
– Cardiovascular (40%)<br />
– Gastrointestinal (33%)<br />
□ Anorectal malformations such as imperforate anus<br />
– Ears, lips, palate (33%)<br />
□ Low-set or absent ears<br />
□ External canal atresia<br />
□ Cleft palate<br />
DIAGNOSTIC CHECKLIST<br />
Image Interpretation Pearls<br />
• Do not confuse RE with mass in pelvis or thorax<br />
• Do not confuse CFE with renal mass<br />
SELECTED REFERENCES<br />
1. Solanki S et al: Crossed fused renal ectopia: challenges in diagnosis <strong>and</strong><br />
management. J Indian Assoc Pediatr Surg. 18(1):7-10, 2013<br />
2. Chang PL et al: Prenatal diagnosis of cross-fused renal ectopia: does color<br />
Doppler <strong>and</strong> 3-dimensional sonography help? J <strong>Ultrasound</strong> Med. 30(4):578-<br />
80, 2011<br />
3. Siegel MJ, ed. Pediatric Sonography. 4th edition. Baltimore: Lippincott<br />
Williams & Wilkins, 2011<br />
4. Singer A et al: Spectrum of congenital renal anomalies presenting in<br />
adulthood. Clin Imaging. 32(3):183-91, 2008<br />
5. Guarino N et al: The incidence of associated urological abnormalities in<br />
children with renal ectopia. J Urol. 172(4 Pt 2):1757-9; discussion 1759, 2004<br />
6. Goodman JD et al: Crossed fused renal ectopia: sonographic diagnosis. Urol<br />
Radiol. 8(1):13-6, 1986<br />
7. McCarthy S et al: Ultrasonography in crossed renal ectopia. J <strong>Ultrasound</strong><br />
Med. 3(3):107-12, 1984<br />
430<br />
CLINICAL ISSUES<br />
Presentation<br />
• Most common signs/symptoms<br />
○ Commonly asymptomatic, incidental finding<br />
○ May present with signs <strong>and</strong> symptoms of obstruction,<br />
urolithiasis, reflux, <strong>and</strong> infection<br />
Demographics<br />
• Epidemiology<br />
○ RE: Pelvic ectopia, most common<br />
– Noted in 1:500 to 1:1,200<br />
○ CFE represents 2nd most common fusion abnormality<br />
after horseshoe kidney<br />
– Male predominance, with incidence reported as M:F =<br />
3:2<br />
– Incidence reported ranging from 1:1,000 to 1:7,500<br />
on autopsy studies<br />
○ Crossed nonfused RE: Very rare, reported as 1:75,000<br />
Natural History & Prognosis<br />
• Complications<br />
○ ~ 50% have complications related to vesicoureteral<br />
reflux, hydronephrosis, stones, <strong>and</strong> infection<br />
– Repeated infections can lead to scarring<br />
○ May have increased susceptibility to trauma<br />
○ Increased incidence of multicystic dysplasia<br />
Treatment<br />
• Treat complications<br />
• No need to separate fused components