Igor Sorokin, MD

A 36-year-old female presented 1 month ago with right sided flank pain and two proximal ureteral stones were seen on KUB (figure 1a). She had not seen the stones pass since that KUB and pain did not improve. Therefore, she underwent right ureteroscopy with laser lithotripsy and stent placement (figure 1b). Intra-operatively, only one of the stones in the proximal ureter was seen. The patient was discharged home the same day but returned to the emergency room 2 days later with intractable right flank pain. Based on the imaging in figure 1, what are you suspicious of?

Continue to the next page for the answer.

Duplication of the collecting system is the most common anomaly of the urinary tract. On closer inspection of the image in figure 1a, the stones are not aligned with the course of one ureter. In figure 2b, the arrow points to the second stone seen just medial to the ureter. Often in an effort to reduce radiation by using the settings of “pulse” and “low dose” mode, the very radiopaque appearing stones on KUB are only seen faintly on intra-operative imaging. The patient went back to the operating room where she was found to have a completely duplicated collecting system. The second stone was found in the proximal ureter of the upper pole moiety and was fragmented and removed with stent placement (figure 2b).

Ureteral duplication and its complications. 

 Assistant Professor at the University of Massachusetts, Worcester, MA, is section editor for Urology Times Clinical Quiz.

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Igor Sorokin, MD, presents the case of a 36-year-old female in whom two proximal stones are seen on KUB, but intraoperatively, only one stone is seen.

Quiz: The case of the missing stone

A 40-year-old female presents with a history of right flank pain and first urinary tract infection (UTI). After treatment of the UTI, she noted her right flank pain continued. Furthermore, she remembered that her flank pain was on/off for several years. The pain is not related to menses or intercourse. There is no gross or microscopic hematuria. She was athletic and also noted that abdominal crunches exacerbated the pain.

A computed tomography scan was performed (figure 1a), which showed a slightly dilated right ureteropelvic junction (UPJ) and a dilated upper pole with kinking of the proximal ureter at the level of a crossing gonadal vein. The kinking of the ureter can also be seen in the scout film taken during the 10-minute delayed contrast phase while patient was prone (figure 1b). What is the best diagnosis that can explain the patient’s symptoms?

Nephroptosis has been defined as a renal descent of two vertebral bodies (or >5 cm) during positional change from supine to upright. High clinical suspicion for this rare phenomenon should be ascribed to young female patients with low body mass index and chronic right flank pain. Theories as to the origin of pain have been related to intermittent ureteric obstruction or forceful traction on the vascular pedicle leading to renal ischemia from lack of supportive perirenal fat. In this case, the renal descent caused intermittent obstruction at the level of the proximal ureter.

An intravenous pyelogram (IVP) performed in the supine (figure 2a) and upright (figure 2b) position is shown. There was a descent of 5.5 cm noted. The left kidney was mobile as well, but she was asymptomatic on that side, and descent was only one vertebral body. By performing a right nephropexy, the ureter can drain in both upright and supine positions without being kinked or obstructed by the gonadal vein.

The patient went on to have a robotic right nephropexy using sutures for a 3 point-fixation to the posterior abdominal wall, which resolved her symptoms. Repeat IVP 3 months later showed resolution of right nephroptosis without kinking of the ureter when upright.

Pelvic congestion syndrome would typically result in engorged or congested veins around the ovaries with pelvic pain. The caliber of the gonadal vein in this particular case appears normal. The problem is at the level of the proximal ureter when the kidney descends and not at the level of the UPJ and therefore UPJ obstruction alone cannot explain all her symptoms. Loin pain hematuria syndrome is a diagnosis of exclusion and requires hematuria to be present, which this patient did not have.

Nephroptosis: A “disparaged” condition revisited. 

Nephroptosis and nephropexy – hung up on the past? 

 Assistant Professor at the University of Massachusetts, Worcester, MA, is section editor for Urology Times Clinical Quiz.

This quiz focuses on a 40-year-old female who presents with a history of right flank pain and first urinary tract infection (UTI). After treatment of the UTI, she noted her right flank pain continued.

Quiz: Chronic right flank pain in a slender female

A 35-year-old male from Sudan was hospitalized with a right obstructing 1-cm ureteral stone seen on KUB. The KUB also revealed curvilinear calcification in the bladder wall. The patient was taken to the operating room for ureteroscopy and laser lithotripsy.

During cystoscopy, a rim of calcification was seen along the right ureteric ridge (figure 1a). A biopsy of this area was taken with the pathology shown in figure 1b.

B. Squamous cell carcinoma of the bladder

The biopsy shows inflamed bladder mucosa with calcified structures consistent with calcified schistosomal eggs. Bladder schistosomiasis is caused by the 

 species. The larvae (cercariae) are released from freshwater snails (intermediate host) into the water. They then penetrate through the human skin and travel to the lungs and liver.

Once the larvae mature into adult worms, they travel into pelvic veins. The eggs are deposited along the bladder wall vessels and are excreted in urine. The eggs incite a chronic inflammatory response with granuloma formation. Rarely, metaplasia of the bladder epithelium occurs, which can lead to squamous cell carcinoma. A rim of calcification around the bladder is commonly seen on X-ray and signals fibrosis of the bladder wall, which is seen later in the course of an infection. Praziquantel is an anti-helminthic drug that destroy the adult worms and can cure schistosomiasis in 80%-90% of patients. The chronic fibrotic changes to the bladder wall cannot be cured. While tuberculosis may also cause severe bladder fibrosis and is highly prevalent in Sudan, the biopsy in this case points to schistosomiasis.

Genitourinary Schistosomiasis: Life Cycle and Radiology-Pathologic Findings. 

 Assistant Professor at the University of Massachusetts, Worcester, MA, is section editor for 

Poll

A 35-year-old male from Sudan was hospitalized with a right obstructing 1-cm ureteral stone seen on KUB. The KUB also revealed curvilinear calcification in the bladder wall. The patient was taken to the operating room for ureteroscopy and laser lithotripsy. During cystoscopy, a rim of calcification was seen along the right ureteric ridge. What is the diagnosis?

Quiz: Sandy patch on cystoscopy

A 24-year-old male is seen in the emergency room for complaints of bilateral testicular pain for the past several weeks. He admitted that he recently shaved his scrotum with a razor a few weeks ago. The patient immigrated to the U.S. from Pakistan 3 months ago and has a history of contact with his uncle, who has tuberculosis. Other notable past medical history is uveitis causing partial blindness and a history of recurrent epididymo-orchitis for several months.

On physical exam, the patient has multiple, superficial, tender scrotal ulcers that appear chronic as well as an oral ulcer. No inguinal lymphadenopathy is present. He underwent a scrotal biopsy (figure) that revealed inflammation and disruption of elastic fibers in the venules and arteries at the base of the ulcer.

et’s disease is a rare disorder that causes blood vessel inflammation throughout the body. The disease can lead to numerous symptoms that may seem unrelated at first. The etiology of Beh

et’s is uncertain but occurs mostly in the HLA-B51 phenotype. Beh

et’s disease is a triad of recurrent oral ulcers, recurrent genital ulcers, and uveitis. The diagnosis is essentially clinical, but confirmatory biopsy of the ulcers would reveal inflammation of the blood vessels and disruption of elastic fibers in the vessels. Oftentimes, patients will present with recurrent epididymo-orchitis that is inflammatory in nature and non-infectious.

It is treated with anti-inflammatory medications and typically resolves without sequelae. Genitourinary TB may also rarely present with non-healing scrotal ulcers, epididymo-orchitis, and uveitis but the vasculitis present in this case is more characteristic of Beh

et’s disease. The genital ulcers that present with both syphilis and granuloma inguinale are painless and would not account for all his other findings.

Urologic screening for men with BehÃ§et’s syndrome. 

https://rarediseases.info.nih.gov/diseases/848/behcet-disease

A 24-year-old male is seen in the emergency room for complaints of bilateral testicular pain for the past several weeks.

Author | Igor Sorokin, MD

Articles

Quiz: The case of the missing stone

Quiz: Chronic right flank pain in a slender female

Quiz: Sandy patch on cystoscopy

Quiz: Young male with scrotal ulcers