June Quiz 1 Flashcards
The manifestation of the VHL syndrome that tends to cluster within a subset of affected families is:
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RCC
pheochromocytoma
retinal angioma
cerebellar hemangioblastoma
epididymal papillary cystadenoma
pheochromocytoma
Feedback
Penetrance for all of the manifestations of VHL is incomplete. In particular, pheochromocytomas have been found to cluster only in certain families with VHL, primarily those with a missense mutation of the VHL gene. A careful family history and thorough review of preoperative CT scans for potential associated tumors are important in all patients with familial RCC. Indeed, pheochromocytomas are a critical entity to recognize prior to any surgical intervention, given the potential perioperative morbidity of an unrecognized pheochromocytoma. The other listed manifestations of VHL are not as well-characterized by familial clustering.
A 56-year-old man undergoes a radical cystoprostatectomy and orthotopic neobladder. Long-term preservation of renal function is most dependent on:
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preferential use of ileum over colon
use of > 60 cm of detubularized bowel
performance of an antirefluxing ureteroileal anastomosis
intraoperative neobladder capacity of ≥ 500 mL
postoperative avoidance of any bacteriuria
preferential use of ileum over colon
Reservoirs made of detubularized ileum appear to have the greatest compliance and lowest likelihood of generating intermittent high-pressure contractions. Several clinical studies have demonstrated that the urodynamic characteristics of the ileum appear to be superior to those of the colon and is, therefore, the preferred segment of bowel used for a neobladder. Larger bowel lengths with increased intra-operative volumes are not necessary as all bowel segments effectively stretch over time if there is adequate outflow resistance. In fact, commonly utilized techniques (i.e., Studer, Hauptmann) utilize 40-44 cm of detubularized ileum with resultant intra-operative volumes of 200 mL or less. For ileal neobladders, it has been shown that the capacity increases sevenfold after one year. The true benefits of anti-refluxing anastomosis remain uncertain. It does not appear that conduit pressures are transmitted to the renal pelvis. Also, there is no difference in conduits between those with versus without reflux, with regard to renal function measured two to five years postoperatively. Furthermore, the successful construction of an anti-refluxing anastomosis does not prevent bacterial colonization of the renal pelvis. Many of these patients in fact have no untoward effects and do well despite chronic bacteriuria. Deterioration of the upper tracts is more likely when the culture becomes dominant for Proteus or Pseudomonas, and should therefore be treated in such cases, whereas those with mixed cultures may generally be observed (provided they are not symptomatic).
A 70-year-old woman has intermittent large volume urinary incontinence. Her medical history is significant for a hysterectomy 20 years ago. Urinalysis is normal and PVR is 40 mL. During the CMG, there is no incontinence demonstrated during filling and stress maneuvers, and the end fill pressure is 6 cm H2O at 300 mL. The most likely cause of her incontinence is:
overflow
detrusor overactivity
decreased detrusor compliance
intrinsic sphincter deficiency
VVF
detrusor overactivity
This patient likely has urgency urinary incontinence secondary to detrusor overactivity, as indicated by the random nature o f the incontinence. UDS will fail to demonstrate involuntary bladder contractions in approximately 50% of patients with clinical urgency urinary incontinence. SUI occurs during increased abdominal pressure, and she is not describing leakage during these types of events such as coughing, lifting, and exercise. In addition, SUI, including intrinsic sphincter deficiency, is not demonstrated on UDS since urinary leakage did not occur during Valsalva maneuvers. The charateristics of the incontinence are not consistent with a VVF as the urinary leakage is not continuous. She has a normal detrusor pressure at the end of filling, confirming normal compliance (300 mL/ 6 cm H2O= 50 mL/ cm H2O). Her PVR of 40 mL demonstrates that she is not in urinary retention and thus rules out overflow incontinence.
A 64-year-old man undergoes a partial penectomy for a high-grade pT1 squamous cell carcinoma of the penis and then chooses surveillance in follow-up. Nine months later, a 2 cm lymph node si palpated in the left inguinal region. CT scan is negative for additional lymphadenopathy or metastatic disease. The next step is:
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four weeks of antibiotics.
left superficial inguinal lymph node dissection
left superficial and deep inguinal lymph node dissection
bilateral superficial and deep inguinal lymph node dissection
excisional biopsy with systemic chemotherapy
Correct answer
left superficial and deep inguinal lymph node dissection
Feedback
This patient has likely developed metastatic recurrence in the left inguinal region, consistent with clinical N1 disease. Since this is a delayed development, the patient should have a unilateral superficial and deep inguinal dissection only on the affected side. There are no clinical concerns for infection; therefore, antibiotics are not indicated. In a patient with palpable nodes (or in the setting of clinically negative
nodes with a frozen section demonstrating cancer in the superficial nodes), both a superficial and deep lymph node dissection should be performed. Chemotherapy is not indicated without evidence of distant metastatic and/or unresectable disease.
A 69-year-old man undergoes a robotic simple prostatectomy for a 150 gram prostate. A 22 Fr 3-way urethral catheter with continuous bladder irrigation is in place. Gross hematuria with clots is noted in therecovery room. Vitals are stable. The next step is manual irrigation and:
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observation
upsize urethral catheter
add water to urethral catheter balloon and place catheter on traction
cystoscopy and fulguration
re-exploration in operating room
Correct answer
add water to urethral catheter balloon and place catheter on traction
Feedback
In the recovery area, the outputs from the pelvic drain and urethral catheter (and suprapubic tube, if present) are monitored. In addition, it is routine to verify the hematocrit. fI significant hemorrhage si noted, the urethral catheter may be placed on traction so that the balloon containing 50 mL of saline can compress the bladder neck and prostatic fossa. Constant and reliable traction can be maintained by
securing the catheter to the abdomen or thigh. nI addition, continuous bladder irrigation can be initiated to prevent clot formation. For most patients, these measures are adequate and effective. However, if excessive bleeding persists after these measures, the urethral catheter can be removed in the operating room and a cystoscopic inspection of the prostatic fossa and bladder neck can be performed to identify and fulgurate discrete bleeding sites. fI marked hemorrhage should continue to persist, re-exploration should be strongly considered. Exchanging the catheter should be avoided and would only be considered if the catheter is not draining despite manual irrigation.
An asymptomatic five-year-old boy has gross hematuria two hours after wrestling with his younger brother. Physical examination is normal. The next step is:
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observation
serial examinations and hematocrit determination
ultrasound of bladder and kidneys
CT scan
cystoscopy
ultrasound of bladder and kidneys
Feedback
It is recognized that underlying genitourinary malformations or other pathologies are at least three-fold more common in pediatric patients relative to adults undergoing evaluation for trauma. This is a classic case for raising the concern of an underlying abnormality since the gross hematuria seems out of proportion with the low severity of the trauma. The underlying problems may include hydronephrosis, multicystic kidney, Wilms’ tumor, and various renal fusion anomalies. Therefore, it is appropriate to image with ultrasound to look for such potentially significant problems. A patient should not be considered for admission with serial examinations and hematocrit determination unless there is a documented substantial renal injury. Cystoscopy is not indicated in the initial evaluation of gross hematuria in children. CT scan would only be indicated fi there is significant injury or abnormality on ultrasound or if the mechanism of injury was more concerning.
