Prenatally diagnosed hydronephrosis (fluid-filled kidneys) suggest post-natal follow-up examination.

The strongest neo-natal presentation is urinary tract infection. A hydronephrotic kidney may present as a palpable abdominal mass in the newborn, and may suggest an ectopic ureter or ureterocele.

In older children, ureteral duplication may present as:

- Urinary tract infection - most commonly due to vesicoureteral reflux (flow of urine from the bladder into the ureter, rather than vice versa).

- Urinary incontinence in females occurs in cases of ectopic ureter entering the vagina, urethra or vestibule.

Duplicated ureter or Duplex Collecting System is a congenital condition in which the ureteric bud, the embryological origin of the ureter, splits (or arises twice), resulting in two ureters draining a single kidney. It is the most common renal abnormality, occurring in approximately 1% of the population. The additional ureter may result in a ureterocele, or an ectopic ureter.

The signs and symptoms of hydronephrosis depend upon whether the obstruction is acute or chronic, partial or complete, unilateral or bilateral. Hydronephrosis that occurs acutely with sudden onset (as caused by a kidney stone) can cause intense pain in the flank area (between the hips and ribs). Historically, this type of pain has been described as "Dietl's crisis". Conversely, hydronephrosis that develops gradually will generally cause either attacks of a dull discomfort or no pain. Nausea and vomiting may also occur. An obstruction that occurs at the urethra or bladder outlet can cause pain and pressure resulting from distension of the bladder. Blocking the flow of urine will commonly result in urinary tract infections which can lead to the development of additional stones, fever, and blood or pus in the urine. If complete obstruction occurs, kidney failure may follow.

Blood tests may show impaired kidney function (elevated urea or creatinine) or electrolyte imbalances such as hyponatremia or hyperchloremic metabolic acidosis. Urinalysis may indicate an elevated pH due to the secondary destruction of nephrons within the affected kidney. Physical examination may detect a palpable abdominal or flank mass caused by the enlarged kidney.

Ectopic ureter (or ureteral ectopia) is a medical condition where the ureter, rather than terminating at the urinary bladder, terminates at a different site. In males this site is usually the urethra, in females this is usually the urethra or vagina. It can be associated with renal dysplasia, frequent urinary tract infections, and urinary incontinence (usually continuous drip incontinence). Ectopic ureters are found in 1 of every 2000–4000 patients, and can be difficult to diagnose, but are most often seen on CT scans.

Ectopic ureter is commonly a result of a duplicated renal collecting system, a duplex kidney with 2 ureters. In this case, usually one ureter drains correctly to the bladder, with the duplicated ureter presenting as ectopic.

The signs and symptoms of ureterocele in the latter two forms can easily be confused with other medical conditions. Symptoms can include:

- Frequent urinary tract infections

- Pyelonephritis

- Obstructive voiding symptoms

- Urinary retention

- Failure to thrive

- Hematuria

- Cyclic abdominal pain

- Urolithiasis

- Cobra head sign is seen in radiography

- In females: salpingitis, hydrosalpinx with sepsis or torsion. T.O. mass.

Symptoms, less likely in chronic obstruction, are pain radiating to the T11 to T12 dermatomes, anuria, nocturia, or polyuria.

Hydronephrosis—literally "water inside the kidney"—refers to distension and dilation of the renal pelvis and calyces, usually caused by urinary retention due to obstruction of the free flow of urine from the kidney. Untreated, it leads to progressive atrophy of the kidney. One or both kidneys may be affected. In cases of hydroureteronephrosis, there is distention of both the ureter and the renal pelvis and calices.

Obstructive uropathy is a structural or functional hindrance of normal urine flow, sometimes leading to renal dysfunction (obstructive nephropathy).

It is a very broad term, and does not imply a location or cause.

A ureterocele is a congenital abnormality found in the ureter. In this condition the distal ureter balloons at its opening into the bladder, forming a sac-like pouch. It is most often associated with a duplicated collection system, where two ureters drain their respective kidney instead of one. Simple ureterocele, where the condition involves only a single ureter, represents only twenty percent of cases. Ureterocele affects one in 4,000 individuals, at least four-fifths of whom are female. Patients are frequently Caucasian.

Since the advent of the ultrasound, most ureteroceles are diagnosed prenatally. The pediatric and adult conditions are often found incidentally, i.e. through diagnostic imaging performed for unrelated reasons.

Bladder stones are small mineral deposits that can form in the bladder. In most cases bladder stones develop when the urine becomes very concentrated or when one is dehydrated. This allows for minerals, such as calcium or magnesium salts, to crystallize and form stones. Bladder stones vary in number, size and consistency. In some cases bladder stones do not cause any symptoms and are discovered as an incidental finding on a plain radiograph. However, when symptoms do occur, these may include severe lower abdominal and back pain, difficult urination, frequent urination at night, fever, painful urination and blood in the urine. The majority of individuals who are symptomatic will complain of pain which comes in waves. The pain may also be associated with nausea, vomiting and chills.

Bladder stones vary in their size, shape and texture- some are small, hard and smooth whereas others are huge, spiked and very soft. One can have one or multiple stones. Bladder stones are somewhat more common in men who have prostate enlargement. The large prostate presses on the urethra and makes it difficult to pass urine. Over time, stagnant urine collects in the bladder and minerals like calcium start to precipitate. Other individuals who develop bladder stones include those who have had spinal cord injury, paralysis, or some type of nerve damage. When nerves to the back are damaged, the bladder cannot empty, resulting in stagnant urine.

Most children with vesicoureteral reflux are asymptomatic. Vesicoureteral reflux may be diagnosed as a result of further evaluation of dilation of the kidney or ureters draining urine from the kidney while in utero as well as when a sibling has VUR (though routine testing in either circumstance is controversial). Reflux also increases risk of acute bladder and kidney infections, so testing for reflux may be performed after a child has one or more infections.

In infants, the signs and symptoms of a urinary tract infection may include only fever and lethargy, with poor appetite and sometimes foul-smelling urine, while older children typically present with discomfort or pain with urination and frequent urination.

Vesicoureteral reflux (VUR), also known as vesicoureteric reflux, is a condition in which urine flows retrograde, or backward, from the bladder into the ureters/kidneys. Urine normally travels in one direction (forward, or anterograde) from the kidneys to the bladder via the ureters, with a 1-way valve at the vesicoureteral (ureteral-bladder) junction preventing backflow. The valve is formed by oblique tunneling of the distal ureter through the wall of the bladder, creating a short length of ureter (1–2 cm) that can be compressed as the bladder fills. Reflux occurs if the ureter enters the bladder without sufficient tunneling, i.e., too "end-on".

The hallmark of a stone that obstructs the ureter or renal pelvis is excruciating, intermittent pain that radiates from the flank to the groin or to the inner thigh. This pain, known as renal colic, is often described as one of the strongest pain sensations known. Renal colic caused by kidney stones is commonly accompanied by urinary urgency, restlessness, hematuria, sweating, nausea, and vomiting. It typically comes in waves lasting 20 to 60 minutes caused by peristaltic contractions of the ureter as it attempts to expel the stone.

The embryological link between the urinary tract, the genital system, and the gastrointestinal tract is the basis of the radiation of pain to the gonads, as well as the nausea and vomiting that are also common in urolithiasis. Postrenal azotemia and hydronephrosis can be observed following the obstruction of urine flow through one or both ureters.

Pain in the lower left quadrant can sometimes be confused with diverticulitis because the sigmoid colon overlaps the ureter and the exact location of the pain may be difficult to isolate due to the close proximity of these two structures.

Jackstone calculi are rare bladder stones that have an appearance resembling toy jacks. They are almost always composed of calcium oxalate dihydrate and consist of a dense central core and radiating . They are typically light brown with dark patches and are usually formed in the urinary bladder and rarely in the upper urinary tract. Their appearance on plain radiographs and computed tomography in human patients is usually easily recognizable. Jackstones often must be removed via cystolithotomy.

Renal colic typically begins in the flank and often radiates to the hypochondrium (the part of the anterior abdominal wall below the costal margins) or the groin. It is typically colicky (comes in waves) due to ureteric peristalsis, but may be constant. It is often described as one of the strongest pain sensations known.

Although this condition can be very painful, kidney stones usually cause no permanent physical damage. The experience is said to be traumatizing due to pain, and the experience of passing blood, blood clots, and pieces of the stone. Depending on the sufferer's situation, nothing more than drinking significant amounts of water may be called for; in other instances, surgery may be needed. Preventive treatment can be instituted to minimize the likelihood of recurrence.

Renal colic is a type of abdominal pain commonly caused by kidney stones.

Kidney stone disease, also known as urolithiasis, is when a solid piece of material (kidney stone) occurs in the urinary tract. Kidney stones typically form in the kidney and leave the body in the urine stream. A small stone may pass without causing symptoms. If a stone grows to more than it can cause blockage of the ureter resulting in severe pain in the lower back or abdomen. A stone may also result in blood in the urine, vomiting, or painful urination. About half of people will have another stone within ten years.

Most stones form due to a combination of genetics and environmental factors. Risk factors include high urine calcium levels, obesity, certain foods, some medications, calcium supplements, hyperparathyroidism, gout and not drinking enough fluids. Stones form in the kidney when minerals in urine are at high concentration. The diagnosis is usually based on symptoms, urine testing, and medical imaging. Blood tests may also be useful. Stones are typically classified by their location: nephrolithiasis (in the kidney), ureterolithiasis (in the ureter), cystolithiasis (in the bladder), or by what they are made of (calcium oxalate, uric acid, struvite, cystine).

In those who have had stones, prevention is by drinking fluids such that more than two liters of urine are produced per day. If this is not effective enough, thiazide diuretic, citrate, or allopurinol may be taken. It is recommended that soft drinks containing phosphoric acid (typically colas) be avoided. When a stone causes no symptoms, no treatment is needed. Otherwise pain control is usually the first measure, using medications such as nonsteroidal anti-inflammatory drugs or opioids. Larger stones may be helped to pass with the medication tamsulosin or may require procedures such as extracorporeal shock wave lithotripsy, ureteroscopy, or percutaneous nephrolithotomy.

Between 1% and 15% of people globally are affected by kidney stones at some point in their life. In 2015, 22.1 million cases occurred, resulting in about 16,100 deaths. They have become more common in the Western world since the 1970s. Generally, more men are affected than women. Kidney stones have affected humans throughout history with descriptions of surgery to remove them dating from as early as 600 BC.

Megaureter is a medical anomaly whereby the ureter is abnormally . Congenital megaureter is an uncommon condition which is more common in males, may be bilateral, and is often associated with other congenital anomalies. The cause is thought to be aperistalsis of the distal ureter, leading to dilatation.

The cutoff value for megaureter is when it is wider than 6 or 7 mm.

A functional obstruction at the lower end of the ureter leads to progressive dilatation and a tendency to infection. The ureteric orifice appears normal and a ureteric catheter passes easily.

Definitive surgical treatment involves refashioning the lower end of the affected ureter so that a tunnelled reimplantation into the bladder can be done to prevent reflux.

The symptoms of reflux nephropathy are comparable to nephrotic syndrome and infection of the urinary tract, though some individuals may not exhibit any evidence (symptom) of reflux nephropathy.

A Gartner's duct cyst (sometimes incorrectly referred to as "vaginal inclusion cyst") is a benign vaginal cystic lesion that arises from the Gartner's duct, which is a vestigial remnant of the mesonephric duct (wolffian duct) in females. They are typically small asymptomatic cysts that occur along the lateral walls of the vagina, following the course of the duct. They can present in adolescence with painful menstruation (Dysmenorrhea) or difficulty inserting a tampon. They can also enlarge to substantial proportions and be mistaken for urethral diverticulum or other structures.

There is a small association between Gartner's duct cysts and metanephric urinary anomalies, such as ectopic ureter & ipsilateral renal hypoplasia. Because of this, imaging is recommended before excision.

In patients with this condition, the central portion of the kidney may be found just inferior to the inferior mesenteric artery because the normal embryologic ascent of the kidneys is arrested by its presence in people with central fusion of the kidneys. Horseshoe kidney is often asymptomatic, though persons affected by this condition may experience nausea, abdominal discomfort, kidney stones and urinary tract infections at greater frequency than those without renal fusion. There is currently no treatment for renal fusion other than symptomatic treatment.

Imaging Findings -

The 2 kidneys on opposite sides of the body with the lower poles fused in midline. Midline or symmetrical fusion (90% of cases).

May be missed on US, therefore pay careful attention to identification of lower poles of kidneys.

Renal long axis medially orientated,

Lower poles with curved configuration, elongation and poorly defined

Isthmus crosses midline anterior to spine and great vessels.

US for diagnosis in utero

IVP followed by CT or scintigraphy for pre-operative assessment

Variant arterial supply -

Bilateral renal arteries,

Inferior mesenteric Artery,

Arteries arising from aorta or common iliac, internal iliac, external iliac or inferior mesenteric arteries.

The lower poles of these kidneys fuse in the midline anterior to the aorta and spine. The isthmus is usually located at L4/5 level between the aorta and IMA.

Nuclear medicine (DMSA) scan confirms horseshoe kidney with fusion of both renal lower poles.

The underlying calyces lose their normal concave shape and show clubbing.

Horseshoe kidney, also known as "ren arcuatus" (in Latin), renal fusion or super kidney, is a congenital disorder affecting about 1 in 600 people, more common in men.

In this disorder, the patient's kidneys fuse together to form a horseshoe-shape during development in the womb. The fused part is the isthmus of the horseshoe kidney.

Fusion abnormalities of the kidney can be categorized into two groups: horseshoe kidney and crossed fused ectopia. The 'horseshoe kidney' is the most common renal fusion anomaly.

Cystinuria is a cause of persistent kidney stones. It is a disease involving the defective transepithelial transport of cystine and dibasic amino acids in the kidney and intestine, and is one of many causes of kidney stones. If not treated properly, the disease could cause serious damage to the kidneys and surrounding organs, and in some rare cases death. The stones may be identified by a positive nitroprusside cyanide test. The crystals are usually hexagonal, translucent, white. Upon removal, the stones may be pink or yellow in color, but later they turn to greenish due to exposure to air. Cystinuria is usually asymptomatic when no stone is formed. However, once a stone is formed, or if stone production is severe or frequent, symptoms may be present:

- Nausea/vomiting

- Dull ache or "colicky" pain

- Chronic pain

- Hematuria

- Obstructive syndromes like hydronephrosis

- Infective syndromes like pyelonephritis

Cystinurics can also experience chronic pain in one, or both, kidneys due to the scars that the jagged edges of the stones can leave or damage from multiple stone removal surgeries. This can leave a cystinuric in constant pain which often requires medical intervention, such as long-term use of analgesics or surgical procedures, including T11, T12 or T13 nerve blocks (although, these procedures are often not successful, they can provide some relief). Aside from the chronic pain, a cystinuric will often have severe breakthrough pain from passing stones. People with cystinuria pass stones monthly, weekly, or daily, and need ongoing care. Cystinurics have an increased risk for chronic kidney disease and since kidney damage or poor function is often present in cystinurics, the use of nonsteroidal anti-inflammatory drugs (NSAIDs) or over the counter (OTC) medications should be used with caution.

Cystine stones are often not visible on most x-rays, CT's, and ultrasounds. This does not mean the cystinuric doesn't have a stone. It takes a trained eye and experience to spot a cystine stone. It is not unusual for a cystinuric to pass a stone, or stones, after being released from the hospital with a CT or x-ray result of no stones in the kidneys.

Urine odor in cystinuria has a smell of rotten eggs due to the increase in cystine.

Signs and symptoms of acute pyelonephritis generally develop rapidly over a few hours or a day. It can cause high fever, pain on passing urine, and abdominal pain that radiates along the flank towards the back. There is often associated vomiting.

Chronic pyelonephritis causes persistent flank or abdominal pain, signs of infection (fever, unintentional weight loss, malaise, decreased appetite), lower urinary tract symptoms and blood in the urine. Chronic pyelonephritis can in addition cause fever of unknown origin. Furthermore, inflammation-related proteins can accumulate in organs and cause the condition AA amyloidosis.

Physical examination may reveal fever and tenderness at the costovertebral angle on the affected side.

Pyelonephritis that has progressed to urosepsis may be accompanied by signs of septic shock, including rapid breathing, decreased blood pressure, shivering, and occasionally delirium.