FABRY DISEASE/SYNDROME
DESCRIPTION Fabry disease is a rare X-linked disorder caused by deficient activity of the lysosomal enzyme α-galactosidase A. Progressive accumulation of the substrate Globotriasylceramide (Gb3) leads to progressive organ failure and premature death. Findings consist of multiple cutaneous lesions (angiokeratoma corporis diffusum), corneal opacification, and progressive renal insufficiency. Symptoms of severe burning pain in the extremities usually begin in the 1st decade, and can cause febrile episodes. Cardiovascular effects include coronary artery disease and congestive heart failure. Renal failure leads to uremia and hypertension in the 3rd–5th decades.
TREATMENT
• Enzyme replacement therapy with agalsidase-β
• Renal replacement therapy
REFERENCE
Mehta A, Ricci R, Widmer U, et al. Fabry disease defined: Baseline clinical manifestations of 366 patients in the Fabry Outcome Survey. Eur J Clin Invest. 2004;34(3):236–242.
FAMILIAL TESTOTOXICOSIS
DESCRIPTION Cause of isosexual precocity inherited as an autosomal dominant pattern. Markedly elevated levels of testosterone with normal LH secretion are noted, but sleep-associated LH pulses are absent. Patients typically present with family history and testicular enlargement around ages 3–4. Diagnosis is a lack of testosterone response to hCG administration, despite a measurable increase in LH. Hyperplasia of Leydig cells is noted on biopsy. Ketoconazole, antiandrogens, aromatase inhibitors, and/or medroxyprogesterone acetate have been used in different combinations with success.
REFERENCE
Lenz AM, Shulman D, Eugster EA, et al. Bicalutamide and third-generation aromatase inhibitors in testotoxicosis. Pediatrics. 2010;126(3):728–733.
FANCONI SYNDROME
DESCRIPTION An acquired or inherited disorder characterized by abnormalities of renal proximal tubular function, including glucosuria, phosphaturia, aminoaciduria, and bicarbonate wasting. The aminoaciduria is generalized, and defects in uric acid, water, potassium, and sodium absorption can also occur. The basic abnormality is unknown. Acquired disease is caused by 6-mercaptopurine or outdated tetracycline, renal transplantation, multiple myeloma, amyloidosis, intoxication with heavy met als or other chemical agents, and vitamin D deficiency. Inherited form (usually seen with other disorders) presents in infancy with proximal tubular acidosis, hypophosphatemic rickets, hypokalemia, polyuria, and polydipsia. In the nephropathic form, failure to thrive and growth retardation are common, with progressive renal failure. Diagnosed by demonstrating the abnormalities of renal function.
TREATMENT
• Sodium bicarbonate for acidosis
• Renal transplantation has been successful
REFERENCES
Bickel H, Manz F. Hereditary tubular disorders of the Fanconi type and the idiopathic Fanconi syndrome. Prog Clin Biol Res. 1989;305:111–335.
Palmer LS, Trachtman H. Renal functional development and diseases in children. In: Wein AJ, Kavoussi LR, Novick AC, et al., eds. Campbell-Walsh Urology. 10th ed. Philadelphia, PA: Saunders; 2011:3002–3027.
FATTY CASTS
DESCRIPTION Fatty casts contain fat globules embedded within tubular epithelial casts. Polarized light microscopy may reveal a “Maltese cross” appearance if cholesterol is present. These are most commonly associated with nephrotic syndrome, but occasionally seen also after long-bone fractures, and classically seen in fat embolism syndrome.
REFERENCE
Schrier RW. Clinical evaluation. In: Diseases of the Kidney and Urinary Tract. Philadelphia, PA: Lippincott Williams & Wilkins; 2007:294.
FECAL INCONTINENCE, UROLOGIC CONSIDERATIONS
DESCRIPTION Numerous studies have identified a relationship between urinary symptoms and fecal incontinence. In the Nurses’ Health Study of over 64,000 women ages 62–87 yr, the prevalence of dual urinary and fecal incontinence was 7% (fecal incontinence alone was 4% and urinary incontinence alone was 38%). Risk factors for dual incontinence in women included: age >80 yr; depression; neurologic disease; functional limitations, multiparity; and childbirth of a heavy newborn (>9.5 lb). As compared with white race, black race was associated with a decreased risk of dual incontinence. Men and women with overactive bladder (OAB) are significantly more likely to report having chronic constipation or fecal incontinence compared with those without OAB. Causes of fecal incontinence include vaginal delivery with anal sphincter damage, surgical trauma, diabetes mellitus, decreased rectal compliance (ulcerative proctitis and radiation proctitis), impaired rectal sensation (diabetes, MS, demential, meningo-myelocele, SCI), fecal impaction, medications, food intolerance, and idiopathic. Stool impaction with the rectum in close proximity to the bladder can elicit local, spontaneous neurogenic activity, prompting urinary frequency, and/or incontinence.
In children with fecal incontinence urinary tract issues are often present. Up to 29% children have daytime wetting and 34% have nighttime wetting. Urinary tract infections have been associated with fecal incontinence in 33% of girls and 3% of boys. (See also Section I: “Dysfunctional elimination Syndrome.”)
REFERENCES
Ferry GD. Definition, clinical manifestations, and evaluation of functional fecal incontinence in infants and children. www.UpToDate.com; Wolters Kluwer, Accessed April 1, 2014.
Robson K, Lembo AJ. Fecal incontinence in adults. www.UpToDate.com; Wolters Kluwer, Accessed April 1, 2014.
FECALURIA
DESCRIPTION The presence of fecal matter passed per urethra, suggestive of a fistulous communication between the urinary and intestinal tract. Etiologies include a pathologic process such as Crohn’s disease, diverticulitis, and cancer, or iatrogenic causes such as perineal surgery, radiation, or trauma. Initial evaluation should include cross-sectional imaging in the form of CT (modality of choice) or MRI to help delineate the location of the fistulous tract.
REFERENCE
Rovner ES. Urinary tract fistulae. In: Wein AJ, Kavoussi LR, Novick AC, et al. Campbell-Walsh Urology. 10th ed. Philadelphia, PA: Saunders; 2012:2223–2261.
FEMALE HYPOACTIVE SEXUAL DESIRE DISORDER
DESCRIPTION Female sexual dysfunction has been defined in the DSM-IV manual as persistently or recurrently deficient (or absent) sexual fantasies and desire for sexual activity that causes marked distress or interpersonal difficulty. Prevalence ranges from 39–43% in recent studies. The operational definition of desire/arousal is currently being reviewed. The DSM-V is scheduled for release in May 2013.
CAUSES
• Hormonal: Hypothalamic/pituitary dysfunction, menopause, chronic oral contraceptive pills
• Musculogenic: Hyper- or hypotonicity of pelvic floor
• Neurogenic: Spinal cord injury (SCI), other nervous system disorders (DM, CVA)
• Psychogenic: Relationship problems, poor body image/self-esteem, mood disorders
• Vasculogenic: Poor blood flow, pelvic atherosclerosis, trauma
• Iatrogenic: Medication use (antidepressants, esp. SSRIs)
TREATMENT
• Education: Desire-arousal-orgasm axis, emotional intimacy, anatomic explanation
• Lifestyle modification: Stress management, adequate rest, regular exercise
• Pharmacology: Topical/vaginal estrogens improve vaginal lubrication and atrophy, but have shown no effect on sexual desire. Testosterone (300 μg/d transdermally) has shown benefit in postmenopausal women, but remains unapproved by the FDA. Phosphodiesterase inhibitors have not shown improvement for women with diminished desire.
REFERENCE
Frank JE, Mistretta P, Will J. Diagnosis and treatment of female sexual dysfunction. Am Fam Physician. 2008;77(5):635–642.
FEMALE SEX FUNCTION INDEX (FSFI)
DESCRIPTION The FSFI is a validated questionnaire to assess female sexual function. It was developed for the specific purpose of assessing domains of sexual functioning (eg, desire, sexual arousal, lubrication, orgasm, satisfaction, pain) in clinical trials. It was not designed for use as a diagnostic instrument and should not be used as a substitute for a complete sex history in clinical evaluation. The FSFI was validated in 2 groups of women, including subjects with sexual arousal disorder (determined by history) and age-matched controls. The instrument reliably differentiated these 2 groups in all domains of sexual functioning.
REFERENCE
Rosen R, Brown C, Heiman J, et al. The Female Sexual Function Index (FSFI): A multidimensional self-report instrument for the assessment of female sexual function. J Sex Marital Ther. 2000;26(2);191–208.
FEMINIZING GENITOPLASTY
DESCRIPTION Surgical treatment of ambiguous genitalia may be indicated in the genetic female with virilization of external genitalia for psychosocial development. The most common cause of virilization in the female newborn is CAH. Feminizing genitoplasty may be performed early in infancy to facilitate gender-appropriate upbringing, or delayed until adolescence when the patient can participate in consent. Extensive counseling of parents of any infant with a disorder of sexual development is critical before considering genitoplasty. Goals of surgery are to create external genitalia with an esthetic female appearance, and permit sexual function and, if possible, fertility. (See also Section I: “Disorders of Sexual Development [DSD]”; Section II: “Congenital Adrenal Hyperplasia.”)
REFERENCE
Schober JM. Feminization (surgical aspects). In: Stringer MD, Oldham KT, Mouriquand PDE, eds. Pediatric Surgery and Urology: Long-term Outcomes. New York, NY: Cambridge; 2006:595–611.
FERTILE EUNUCH DISEASE/SYNDROME
DESCRIPTION Syndrome characterized by prepubertal androgen deficiency or eunuchoidism caused by LH deficiency but pubertal or almost adult-sized testes in which advanced-stage spermatogenesis is present due to relatively preserved FSH secretion. However, spermatogenesis usually is not completely normal in these men, and they are not fertile. Because there is only relative gonadotropin deficiency and some spermatogenesis is present, treatment with LH-like activity (hCG) stimulates Leydig cell testosterone production and ameliorates androgen deficiency, stimulating spermatogenesis sufficient for induction of fertility.
REFERENCE
Matsumoto AM, et al. Testicular disorders. In: Melmed S, lonsky KS, Larsen PR, et al. Williams Textbook of Endocrinology. 12th ed. Philadelphia, PA: Elsevier; 2011.
FIBROEPITHELIAL POLYP, GENITOURINARY
DESCRIPTION The most common benign ureteral tumor, arising from the upper 3rd of the ureter, these polyps resemble a smooth nodule or may be pedunculated. Histologically, a central fibrous core surrounded by normal or hyperplastic urothelium is seen. Patients present with flank pain and hematuria, usually as a young adult. Radiographically, smooth filling defects are seen. Hydroureteronephrosis can be seen, as well as ureteral intussusception. They can recur locally.
TREATMENT
Ureteroscopic resection, open ureterotomy with polypectomy, or partial ureterectomy if the diagnosis cannot be confirmed preoperatively.
REFERENCE
Sun Y, Xu C, Wen X, et al. Is endoscopic management suitable for long ureteral fibroepithelial polyps? J Endourol. 2008;22(7):1459–1462.
FIBROEPITHELIAL POLYP, PENIS
DESCRIPTION Fibroepithelial polyp of the penis is a rare benign finding with only a few reported cases in the literature. Most of these patients have a long-term history of usage of condom catheter. The possible pathogenesis is a chronic irritation. Histologically, it contains loosely arranged spindle to stellate cells in a collagenous and edematous stroma with variable-sized thin-walled vascular channels, with perivascular hyalinization. Fibroepithelial polyp of prepuce differ from conventional cutaneous fibroepithelial polyp (also known as a skin tag, acrochordon) by being larger, having notable stromal edema and vascular dilatation and by having greater stromal cellularity. Skin tags are usually <5 mm in size and they have a predilection for the axilla, neck, and eyelid.
REFERENCE
Banerji JS, Shah S, Kekre NS. Fibroepithelial polyp of the prepuce: A rare complication of long-term condom catheter usage. Indian J Urol. 2008;24(2):263–264.
FIBROUS HAMARTOMA OF INFANCY
DESCRIPTION Uncommon subcutaneous and lower dermis proliferative lesion, with the most common anatomic distributions being the axillary region, upper arm, upper trunk, inguinal region, and external genital area. Vast majority of cases occur within the 1st year of life and present as a painless nodule with rapid growth. There is a predilection for boys with a male/female ratio of 2.4. Local excision is curative with very low recurrence rates even in the setting of incomplete excision. Should be in the differential diagnosis of genital masses. Histologically, it demonstrates mature adipose tissue, scattered mesenchymal cells, and bundles of fibrous tissue.
REFERENCE
Dickey GE, Sotelo-Avila C. Fibrous hamartoma of infancy: Current review. Pediatr Dev Pathol. 1999;2(3):236–243.
FIBROUS PSEUDOTUMOR OF TESTICULAR TUNIC
DESCRIPTION Reactive, benign process of the tunica vaginalis in which multiple firm nodules occur in the tunica or within it. Presents as testicular mass, sometimes associated with trauma or hydrocele. It may be difficult to distinguish from malignant processes. Histologically, the lesion can demonstrate granulation tissue, fibroblastic proliferation, and nodules of hyalinized tissue.
SYNONYMS
• Fibrous pseudotumors
• Multiple fibromas of the tunica vaginalis testes
• Reactive periorchitis
TREATMENT
Orchiectomy usually necessary to confirm diagnosis but local excision can be considered.
REFERENCE
Parker PM. Benign fibrous pseudotumor of tunica vaginalis testis. Urology. 2006;68(2):427:e17–e19.
FIDUCIAL MARKERS
DESCRIPTION External beam radiotherapy is a valuable tool in the treatment of localized prostate cancer; however, this form of treatment is limited by the difficulty in accurately localizing the prostate gland. The implantation of intraprostatic gold fiducial markers under transrectal ultrasound guidance is a safe outpatient procedure that aids in identifying anatomic structures of interest during radiation treatment (most importantly the prostate-rectal interface). Markers are usually cylindrical allowing ease of placement with a hollow bore needle. Surface features prevent migration. The preprocedure regimen is similar to transrectal ultrasound-guided prostate biopsy. Prophylactic antibiotics are administered, anticoagulant medications withheld 7 days before procedure, and cleansing enemas given to empty the rectal vault. Patients are routinely placed in the left lateral decubitus position. A transrectal ultrasound probe is utilized to calculate prostate volume in the standard fashion. Local anesthesia should be placed bilaterally at the level of the neurovascular bundles. An 18-gauge implant needle is utilized to place markers into the right base, left base, and apex of the prostate. Standard prostate biopsy discharge instructions are given. Patients then follow up with a radiation oncologist for pretreatment planning imaging and subsequent radiotherapy (Image 
).
REFERENCE
Linden RA, Weiner PR, Gomella LG, et al. Technique of outpatient placement of intraprostatic fiducial markers before external beam radiotherapy. Urology. 2009;73(4):881–886.
FILARIASIS, UROLOGIC CONSIDERATIONS
DESCRIPTION Filariasis is transmitted by mosquitoes, most commonly Wuchereria bancrofti, endemic to areas of the Caribbean, Venezuela, Colombia, the Guianas, Brazil, Central America, sub-Saharan Africa, North Africa, Turkey, and Asia. Filariasis (Bancroftian, Malayan, and Timorian) is often asymptomatic. The parasite causes symptoms due to inflammation and dysfunction of the lymphatics, where the adult worms develop (fever, headache, myalgia, and lymphadenitis). In lymphatic disease, manifestations usually occur 3 mo–1 yr after acquisition. Occasionally, moderate lymphadenopathy, particularly involving the inguinal lymph nodes, occurs. Inflammation of the lymphatics of the extremities and genitalia leads to retrograde adenolymphangitis. Epididymitis, orchitis, and funiculitis can also occur, along with fever, chills, and other nonspecific systemic symptoms. Lymphatic dysfunction, with resulting chronically progressive edema of the limbs and genitalia, is relatively infrequent in children. Elephantiasis can result from fibrosis caused by chronic dysfunction of the lymphatic channels. Chyluria can occur as a manifestation of bancroftian filariasis. Lymphatic filariasis must be diagnosed clinically because serologic assays are not available, and in elephantiasis the microfilariae may no longer be present. Eosinophilia of 25% frequently occurs in early disease. (See also Section I: “Edema, External Genitalia [Peno-Scrotal Edema].”)
TREATMENT
• Diethylcarbamazine citrate is the drug of choice. The late obstructive phase of the disease is not affected by chemotherapy.
• Ivermectin, an investigational drug in the United States, is effective against the microfilariae of W. bancrofti, but is unlikely to become the drug of choice for lymphatic filariasis.
• Complex, decongestive physiotherapy may be effective in treating elephantiasis.
• Plastic surgical repair of the genitalia gives variable results.
• Chyluria originating in the bladder responds to fulguration; chyluria originating in the kidney is much more difficult to correct.
REFERENCE
Kehinde EO, Anim JT, Hira PR. Parasites of urological importance. Urol Int. 2008;81(1):1–13.
FINE-NEEDLE ASPIRATION (FNA) OF PROSTATE
DESCRIPTION In the detection of prostatic carcinoma, FNA cytology of the prostate has largely been replaced by core needle biopsy of the prostate, as cytology does not allow Gleason grading. However, the detection rates of prostatic carcinoma by either core needle biopsy or FNA appear to be comparable. Largely replaced by core biopsy techniques. (See also Section II: “Cytology, Prostate.”)
REFERENCE
Hautmann SH, Conrad S, Henke RP, et al. Detection rate of histologically insignificant prostate cancer with systematic sextant biopsies and fine needle aspiration cytology. J Urol. 2000;163:1734–1738.
FISH: URINARY FLUORESCENT IN SITU HYBRIDIZATION (UROVYSION TEST)
DESCRIPTION Cytogenetic studies describe frequent alterations in chromosomes 1, 3, 4, 7, 8, 9, 11, 17, etc., in urothelial cancers. FISH allows the study of genetic abnormalities within formalin-fixed cancer cells. UroVysion test is a multitargeted multicolor FISH assay that stains exfoliated cells from urine specimens with probes for chromosome 3, 7, 17, and 9p21, and allows observation of the cells under a fluorescence microscope. Reported sensitivity of UroVysion test is higher for higher-grade tumors (83–97%) and CIS (almost 100%), than with low-grade low-stage tumors (36–57%). Specificity is high (89–96%). A false-positive UroVysion test may predict for future recurrence or simply reflect urothelium that is at risk of malignant transformation. A study to detect bladder cancer in a high-risk population showed FISH to be comparable to urine cytology with a higher false-positive rate (Image ).
REFERENCE
Banek S, Schwentner C, Täger D, et al.; UroScreen Study Group. Prospective evaluation of fluorescence-in situ-hybridization to detect bladder cancer: Results from the Uroscreen-Study. Urol Oncol. 2013;31(8):1656–1662.
FISTULA, ENTEROVESICAL
DESCRIPTION An abnormal fistulous communication between the bowel (such as colovesical fistula) and urinary bladder due to various inflammatory and neoplastic causes. Usually presents with fecaluria, pneumaturia, and/or recurrent UTI. Gouverneur syndrome is the “classic” presentation of vesicoenteric fistula: Suprapubic pain, urinary frequency, dysuria, and tenesmus. Causes include inflammatory processes (diverticulitis, Crohn disease), neoplasia (bladder or colonic malignancy), and radiation. Diagnosis may require the use of ≥1 diagnostic modality, such as endoscopy (colonoscopy, cystoscopy), contrast imaging (cystography, contrast enema), and CT. Oral-activated charcoal and urinary straining on a stone filter may be useful, as well as the Bourne test. (See Section II: “Bourne Test” and (Image ).)
TREATMENT
• Surgical resection of fistulous tract with or without fecal and urinary diversion
• Fecal and urinary diversion
• Conservative management
REFERENCES
Kavanagh D, Neary P, Dodd JD, et al. Diagnosis and treatment of enterovesical fistulae. Colorectal Dis. 2005;7(3):286–291.
Randall D, Tittle V, Wright G, et al. Crohn’s disease and enterovesical fistulae: Common things are common. Br J Hosp Med. 2010;71(9):530–531.
FISTULA, RECTOURETHRAL
DESCRIPTION An abnormal communication between the urethra and rectum, almost exclusively in males due to anatomic reasons. May present with passage of urine in the stools. Causes include iatrogenic (transurethral instrumentation and surgery, RP, cryosurgery or radiotherapy of prostate), trauma, inflammatory bowel disease, pelvic infection, or congenital malformations. Diagnosis may require the use of ≥1 diagnostic modality, such as endoscopy (proctosigmoidoscopy, cystoscopy), contrast imaging (urethrocystography, contrast enema), CT or MRI with endorectal coil.
TREATMENT
• Surgical excision of fistulous tract, urethral repair, tissue interposition, and rectal closure with possible use of advancement flaps or vascularized flaps
• Posterior trans-ano-sphincteric, transperineal, transanal, transabdominal approaches described
• Possible vascularized flaps include dartos flap, scrotal myocutaneous flap, island groin flap, gracilis flap, and omental flap
• Proximal bowel diversion usually recommended
• Fecal and urinary diversion
• Conservative management
REFERENCE
Gupta G, Kumar S, Kekre NS, et al. Surgical management of rectourethral fistula. Urology. 2008;71(2):267–271.
FISTULA, URETEROARTERIAL
DESCRIPTION May present with microscopic hematuria, intermittent gross hematuria, or torrential hemorrhage in extremis. Risk factors include prior pelvic surgery, chronic indwelling ureteric stents, pelvic irradiation, and arterial disease. Rarely, this is the etiology for hematuria, but should be considered for persistent gross hematuria or torrential bleeding in a patient with associated risk factors. General guideline to reduce the risk of fistula development is the use of the smallest caliber, softest flexible ureteric stent for the shortest possible period. In a stable patient, CT, retrograde uretero-pyelography, and angiography may be nonspecific but aid in planning reconstructive options. Removal of stents and ureteral manipulation should be performed with caution and in a facility where immediate angiographic or surgical intervention is available.
TREATMENT
• If stable, early reconstruction of vascular and urinary structures:
– Vascular occlusion with angiographic stent or embolization; OR
– Vascular ligation, with or without bypass procedure
– Ureteric reconstruction by uretero-ureterostomy, cutaneous ureterostomy, transverse ureteroureterostomy, or ureteric ligation with nephrostomy
– Endovascular stenting is increasingly used in lieu of open techniques due to the high operative risk and comorbidities in patients with ureteroarterial fistulas
• In the actively bleeding patient, immediate surgical intervention or angiographic occlusion
REFERENCE
Fox JA, Krambeck A, McPhail EF, et al. Ureteroarterial fistula treatment with open surgery versus endovascular management: Long-term outcomes. J Urol. 2011;185(3):945–950.
FISTULA, VESICOCUTANEOUS
DESCRIPTION An abnormal communication between the urinary bladder and skin of the anterior abdominal wall or groin. The fistulous tract that exists after placement of suprapubic catheter is the most commonly seen. Causes include urinary diversion (suprapubic catheterization); infected urachal remnant; radiation; dehiscence of urinary bladder repair, usually in association with complex pelvic and bowel surgery; and others (bladder calculus, inguinoscrotal hernia). Diagnosis is made by cystoscopy and CT contrast imaging.
TREATMENT
• Surgical excision of fistulous tract and repair
• Conservative management
REFERENCE
Kobori Y, Shigehara K, Amano T, et al. Vesicocutaneous fistula caused by giant bladder calculus. Urol Res. 2007;35(3):161–163.
FISTULA, VESICOUTERINE
DESCRIPTION Rare, usually caused by simultaneous injury to uterus and bladder. Urinary incontinence is present if the cervical os is incompetent. The lesion occasionally presents with Youseff syndrome,which describes menouria (urine in menses), cyclic hematuria with apparent amenorrhea, infertility, and urinary incontinence in patients with prior low-segment cesarean section, which is the most common cause. Other causes include uterine rupture during obstructed labor tearing the posterior bladder wall, placenta percreta, and others (IUD, brachytherapy, traumatic bladder catheterization). Differential diagnosis includes vesicovaginal fistula (VVF), ureterovaginal fistula (UVF), and endometriosis of bladder. Diagnosis is made by cystography, cystography, and contrast CT or MRI of the pelvis, which helps exclude concomitant ureteric injury and (Image ).
TREATMENT
• Prolonged bladder drainage with or without fulguration of fistula tract, and await spontaneous resolution. Option of hormonal induction of menopause to help induce involution of uterus
• Surgical management:
– Hysterectomy and bladder repair, if fertility not desired
– Uterine-sparing surgery if fertility preferred
REFERENCE
Rao MP, Dwivedi US, Datta B, et al. Post caesarean vesicouterine fistulae-Youssef syndrome: Our experience and review of published work. ANZ J Surg. 2006;76(4):243–245.
FISTULA, VESICOVAGINAL AND URETEROVAGINAL
DESCRIPTION Vesicovaginal fistula (VVF) is an abnormal communication between the urinary bladder and vagina that may be associated with urethrovaginal fistula (UVF) in 12%. Patients present with continuous urinary incontinence, with prior history of recent pelvic or gynecologic surgery or other causes.
CAUSES
• Iatrogenic following obstetric and gynecologic surgery
• Pelvic malignancy
• Pelvic radiation
• Inflammatory: Pelvic and abdominal infections
• Penetrating trauma
• Foreign body
DIAGNOSIS
• Pelvic exam
• Cystoscopy with cystography and/or retrograde pyelography
• Contrast imaging (eg, CT urography with delayed imaging, CT cystogram)
TREATMENT
UVF should be managed by reimplantation of ureter. Before undergoing a ureteral reimplantation, a patient can be temporarily managed with ipsilateral percutaneous nephrostomy drainage. Small VVFs may close spontaneously with prolonged bladder drainage. If a VVF requires primary repair, a multilayer closure can be achieved through a transvaginal or transabdominal approach. If diagnosis has been delayed by several weeks, then it is prudent to delay repair for approximately 3 mo. Principles of VVF repair include:
• Demarcation of fistula
• Approaches: Transvaginal, transabdominal
• Excise diseased tissue if present
• Multilayered closure with interrupted absorbable sutures and nonoverlapping suture lines
• Consider vascularized tissue interposition (eg, Martius flap, pedicled muscle flap, peritoneal flap, omental flap), especially when quality of tissue healing is expected to be compromised (eg, previous failed repair, postirradiation)
• Adequate postoperative bladder drainage (Image )
REFERENCE
Wong MJ, Wong K, Rezvan A, et al. Urogenital fistula. Female Pelvic Med Reconstr Surg. 2012;18(2):71–78.
FITZ–HUGH–CURTIS SYNDROME
DESCRIPTION Perihepatic inflammation and right upper quadrant pain found in a small segment of patients with pelvic inflammatory disease (PID). PID is a polymicrobial, ascending, postcoital infection of the upper genital tract usually associated with gonorrhea, Chlamydia, Haemophilus, or Streptococcus. Fitz–Hugh–Curtis is believed to occur from transperitoneal or vascular dissemination of PID organisms, often suggesting a profound pathologic inoculation. The diagnosis is confirmed by laparoscopic visualization of filmy perihepatic adhesions.
REFERENCE
Torrealday S, Torrealday S. Benign gynecologic conditions. Surg Clin North Am. 2008;88:245–264.
FLANK HERNIA FOLLOWING NEPHRECTOMY
DESCRIPTION True flank hernias are rare, and careful palpation may reveal the fascial edges. Obesity, immunocompromised states, and poor nutrition status are risk factors. Flank “bulge” is not a true hernia and is believed to be due to laxity of the transversus and oblique abdominal wall muscles, caused by injury to the intercostal nerves, in particular the 11th intercostal, and accentuated in part by unopposed contraction of contralateral musculature. About 15% of patients develop flank bulge after a retroperitoneal flank incision. Care should be taken to avoid injury to the intercostal nerves during incision and closure.
TREATMENT
• Flank hernia: Generally should be repaired with or without mesh, based on surgeon preference, patient comorbidities, and clinical factors specific to each case. If the patient is asymptomatic or debilitated, a corset can be offered
• Flank bulge: Repair seldom needed except for cosmesis (Image )
REFERENCE
Baumann DP, Butler CE. Lateral abdominal wall reconstruction. Semin Plast Surg. 2012;26(1):40–48.
FLUORESCENT (BLUE LIGHT) CYSTOSCOPY
DESCRIPTION Drugs for fluorescence diagnosis, such as 5-ALA and hexaminolevulinate (Hexvix [EU]; Cysview [US]), are placed intravesically where they preferentially stain malignant or premalignant tissue and emit a red fluorescence when excited by visible blue light. Requires specific endoscopic equipment fitted with the blue light, camera, and lens with filters. In a meta-analysis, fluorescent cystoscopy (92.4%) sensitivity was superior to white-light cystoscopy (60.5%). Reports have shown fluorescent cystoscopy to be limited by specificity, which is equivalent to or poorer than white-light cystoscopy. Fluorescent cystoscopy can enhance the diagnosis of patients with positive cytology and no visible lesion on white-light cystoscopy, and for surveillance of high-risk bladder cancers and/or CIS (Image ).
REFERENCE
Isfoss BL. The sensitivity of fluorescent-light cystoscopy for the detection of CIS of the bladder: A meta-analysis with comments on gold standard. BJU Int. 2011;108(11):1703–1707.
FOLEY Y-V PYELOPLASTY
DESCRIPTION The triangular portion of the Y is incised in the dependent portion of the pelvis, with the apex pointing to the stricture, and a single 2- to 3-cm longitudinal incision is continued from the apex anteriorly down across the stricture to complete the Y configuration. The apex of the triangle flap is then brought down to the lower apex of the ureterotomy and a 5-0 chromic stay suture is placed. Interrupted 5-0 chromic sutures are used to complete the anastomosis. Used for UPJ repair.
REFERENCE
Tsivian A, Tsivian M, Sidi AA. The Y-V pyeloplasty revisited. Urology. 2010;75(1):200–202.
FORDYCE SPOTS (ECTOPIC SEBACEOUS GLANDS), PENIS
DESCRIPTION Fordyce spots are ectopic sebaceous glands on the lips and buccal and genital mucosa (glans penis and labia minor). Lesions are multicentric and whitish to yellowish in color with slightly elevated papules and plaques with sizes ranging from 1–3 mm. Most patients are asymptomatic but some consider receiving treatment for cosmetic reasons since the lesions do not resolve spontaneously. CO2 laser can be used for ablation.
REFERENCE
Lee JH, Lee JH, Kwon NH, et al. Clinicopathologic manifestations of patients with Fordyce’s Spots. Ann Dermatol. 2012;24(1):103–106.
FOREIGN BODY, BLADDER AND URETHRA
DESCRIPTION Almost every conceivable foreign body has been inserted into the urinary bladder and urethra, usually for erotic exploration and curiosity, or because of psychiatric disorder or mental retardation. Amazonian parasitic catfish (Candiru) and leeches have also been reported to enter the urethra while bathing in a river. Symptoms include urethral pain, dysuria, urinary retention, hematuria, frequency, painful voiding, weak stream, and sepsis. (See also Section II: “Bladder Filling Defect.”)
TREATMENT
• Urethral foreign body: Endoscopic retrieval may be easier by 1st pushing back into bladder; alternatively, urethrotomy, especially when periurethral abscess is present
• Bladder foreign body: Endoscopic retrieval; open vesicostomy (Image )
REFERENCE
Van Ophoven A, DeKernion JB. Clinical management of foreign bodies of the genitourinary tract. J Urol. 2000;164(2):274–287.
FORMALIN INSTILLATION, INDICATIONS AND TECHNIQUE
DESCRIPTION Formalin (37% formaldehyde) instillation is an option in the management of hemorrhagic cystitis which is refractory to more conservative measures. A solution of 50 mL of 1% formalin is typically utilized. Instillation must be done in the operating room and usually with general anesthesia, as the procedure may cause pain. Prior to instillation, a cystogram must be performed to rule out vesicoureteral reflux. If reflux is present, then formalin may cause damage to the ureters and intrarenal collecting system. Use of Fogarty catheters to occlude the ureters in the case of reflux has been reported. Bladder fibrosis with reduced capacity and increased urinary frequency is a common outcome after formalin instillation. (See also Section II: Cystitis, Hemorrhagic.”)
REFERENCE
Joseph CM, Bowley DM. Formalin treatment of refractory hemorrhagic cystitis. J Pediatr Urol. 2005;1(5):365–367.
FOSSA NAVICULARIS DIVERTICULUM
DESCRIPTION 1st described by Guérin (1864), this diverticulum is partially separated from the urethra by a septum. On voiding cystorethrogram (VCUG), it can often be seen as a small spherical collection of contrast at the tip of the penis. It is thought to result embryologically from an incomplete breakdown of the wall between the ectoderm and the urethra being formed by the urethral folds. It is a common anatomic finding with rare symptoms, including dysuria, gross hematuria, spotting of blood, or hematospermia.
SYNONYMS
• Valve of Guérin
• Dorsal urethral diverticulum
• Lacuna magna
TREATMENT
If symptomatic, the wall can be divided with tenotomy scissors or under direct vision with a resectoscope.
REFERENCE
Seskin FE, Glassberg KI. Lacuna magna in 6 boys with postvoid bleeding and dysuria: Alternative approach to treatment. J Urol. 1994;152(3):980–982.
FOWLER SYNDROME (PRIMARY DISORDER OF URETHRAL SPHINCTER RELAXATION)
DESCRIPTION This syndrome describes young women (usually under the age of 30) with idiopathic urinary retention and no evidence of neurologic disease. The classic patient will present with the inability to void for an entire day and will have a large volume of urine in her bladder. For diagnosis, a retained urine volume of 1 L or more and lack of sensation or urgency is necessary. EMG activity of the urethral sphincter indicates abnormally increased sphincter activity making appropriate relaxation difficult. There is an association with polycystic ovarian syndrome.
TREATMENT
Use of botulinum toxin, pharmacotherapy, and hormonal modulation have not proven to be successful. Sacral neuromodulation has proven to be successful. 1 study reported efficacy approaching 70%.
REFERENCES
De Ridder D, Ost D, Bruyninckx F. The presence of Fowler’s syndrome predicts successful long-term outcome of sacral nerve stimulation in women with urinary retention. Eur Urol. 2007;51(1):229–233.
Fowler CJ, Christmas TJ, Chapple CR, et al: Abnormal electromyographic activity of the urethral sphincter, voiding dysfunction, and polycystic ovaries: A new syndrome? BMJ. 1988;297:1436–1438.
FOWLER–STEPHENS ORCHIOPEXY
DESCRIPTION This procedure is used in the treatment of high intra-abdominal testes. It entails ligating the spermatic vessels and hinges on the premise that the testicle will survive from the vasal and cremasteric collaterals. The operation was originally described as a 2-stage procedure in which the vessels are divided, and then 6 mo later the testicle is brought down to the scrotum, after collaterals have become well developed. However, the procedure is now performed also a 1-stage procedure. Both techniques have a fairly high success rate, but 2-stage is reported to have a slightly better success rate (85% for 2-stage vs. 80% for 1-stage). It is now commonly performed using laparoscopy.
REFERENCES
Elyas R, Guerra LA, Pike J, et al. Is staging beneficial for Fowler-Stephens orchiopexy? A systematic review. J Urol. 2010: 183(5):2012–2018.
Esposito C, Vallone G, Savanelli A, et al. Long-term outcome of laparoscopic Fowler-Stephens orchiopexy in boys with intra-abdominal testis. J Urol. 2009;181(4):1851–1856.
FRACTURE RISK ASSOCIATED WITH PROSTATE CANCER AND ANDROGEN DEPRIVATION THERAPY
DESCRIPTION Androgen deprivation therapy (ADT) for treatment of prostate cancer leads to a hypogonadal state. This state predisposes men to osteopenia or osteoporosis with subsequent increased risk of fracture. There is a direct correlation between length of time on ADT and risk of fracture. 1 study reported that after 5 yr on ADT, the risk of fracture rises nearly 7% compared to controls.
TREATMENT
Men being treated with ADT should be considered for a baseline DEXA scan to assess bone mineral density (BMD). Increased weight-bearing exercise and cessation of smoking should be encouraged. Also, calcium and vitamin D supplementation can help improve BMD. Daily supplementation of calcium (1,200 mg/d with most taken through dietary means) and vitamin D (1,000–1,200 IU daily) is recommended. Systemic therapy (bisphosphonate or denosumab) therapy has also been shown to play a role in the prevention of osteoporosis and reduce fracture risk. Densoumab is approved for men on ADT or with osteoporosis 60 mg SC Q 6 mo (Prolia). Bisphosphonates such as zoledronic acid (Reclast) can be given IV 5 mg/yr, alendronate (Fosamax, Fosamax Plus D) and risedronate (Actonel) are given orally weekly. Alternative dosing forms are given with the presence of bone metastasis to decrease skelet al related events (SRE). Densosumab (Xgeva) is given 4 mg SC monthly and zoledronic acid (Zometa) is administered IV 4 mg monthly.
REFERENCES
Shahinian VB, Kuo YF, Freeman JL, et al. Risk of fracture after androgen deprivation for prostate cancer. N Engl J Med. 2005;352:154–164.
Suzman DL, et al. Bone-targeting agents in prostate cancer. Cancer Metastasis Rev. 2014 Jan 8 [Epub ahead of print]
Zhumkhawala AA, Gleason JM, Cheetham TC, et al. Osteoporosis management program decreases incidence of hip fracture in patients with prostate cancer receiving androgen deprivation therapy. Urology.2013;81(5):1010–1015.
FRAGILE X SYNDROME
DESCRIPTION The most common cause of inherited mental retardation. The affected gene encodes a protein known as FMR1, which is required for normal cognitive development. Facial dysmorphism and bilateral macro-orchidism (MO) are also seen. Measurement of testis size in mentally retarded males has been suggested as a simple screening test for this condition.
REFERENCE
Healy A, Rush R, Ocain T. Fragile X syndrome: an update on developing treatment modalities. ACS Chem Neurosci. 2011;2(8):402–410.
FRALEY SYNDROME
DESCRIPTION A condition in which vascular obstruction of the superior infundibulum might lead to hydrocalyx, bleeding, and intermittent flank pain or infection. Vessels causing obstruction may be arteries, veins, or both. Impaired drainage on delayed films or isotope renography must be confirmed before surgery. On ultrasound, diuretics will accentuate the caliectasis.
TREATMENT
Surgery can provide relief in severely symptomatic patients. Various techniques including infundibulo-infundibulostomy, infundibulopyelostomy, infundibulorrhaphy, and vasopexy have been reported to be successful in treating symptoms.
REFERENCES
D’Amico A, Lusuardi L, Ficarra V, et al. Experience in the surgical treatment of Fraley’s syndrome. Eur Urol. 2000;38(4):410–414.
Fraley EE. Vascular obstruction of superior infundibulum causing nephralgia: A new syndrome. N Engl J Med. 1966;275:1403–1409.
FRENCH CATHETER SCALE
DESCRIPTION Used to measure the outer diameter of catheters, cystoscopes, and other endoscopes. The diameter in millimeters of the instrument is determined by dividing the French size by 3 (eg, an 18 Fr catheter has a diameter of 6 mm). The system was introduced by a 19th century French medical instrument manufacturer. (See Section VII: “Catheter Guide.”)
REFERENCE
Bedside procedures. In: Gomella LG, Haist SA, eds. Clinicians Pocket Reference. 11th ed. New York, NY: McGraw-Hill; 2007.
FREQUENCY, URINARY
DESCRIPTION Urinary frequency is defined as the patient’s perception that he/she voids too often by day. Although often associated with an overactive bladder and/or bladder outlet obstruction, urinary frequency is 1 of many complaints included in the nonspecific, nondiagnostic symptom complex known as lower urinary tract symptoms or LUTS. Frequency is further categorized as 1 of the storage symptoms (experienced during the bladder filling phase or storage phase of micturition), as opposed to a voiding or postmicturition symptom.
REFERENCE
Haylen BT, de Ridder D, Freeman RM, et al. An International Urogynecological Association (IUGA)/International Continence Society (ICS) joint report of the terminology for female pelvic floor dysfunction. Neurourol Urodyn.2010;29(1):4–20.
FREQUENCY–DYSURIA SYNDROME
DESCRIPTION Occurring in children and women, this is also referred to as the “urethral syndrome.” Patients present with complaints of frequency and dysuria, but evaluation finds no infectious, anatomic, functional, or physiologic abnormalities. Because this term is so nonspecific, it is not a currently accepted meaningful term for diagnosis or treatment planning. In childhood, hypercalciuria was theorized and in adults, fastidial organisms were once thought to be the cause. (See also Section II: “Urethral Syndrome.”)
REFERENCE
Brock JW III. The frequency and frequency dysuria syndromes of childhood: Hypercalciuria as a possible etiology. Urology. 1994;44(3):411–412.
FUHRMAN NUCLEAR GRADING CLASSIFICATION, RENAL CELL CARCINOMA (RCC)
DESCRIPTION A classification used to grade renal cell carcinoma, based on the concept that nuclear features correlate with survival, this scale consists of 4 grades based on size, contour, and conspicuousness of nucleoli. Large series have confirmed the correlation with survival. Grade 1 is round, uniform nuclei with minute or absent nucleoli. Grade 2 is slightly irregular nuclei about 15 μm, with nucleoli visible at 400×. Grade 3 is more irregular nuclei, 20 μm, with nucleoli visible at ×. Grade 4 is similar to grade 3, with bizarre features noted. There is a noted correlation between tumor size and Fuhrman grade (Image ).
REFERENCE
Zhang, C, Li X, Hao H, et al. The correlation between size of renal cell carcinoma and its histopathological characteristics: a single center study of 1867 renal cell carcinoma cases. BJU Int. 2012;110(11 Pt b):E481–E485.
FUNGURIA
DESCRIPTION Funguria (sometimes called candiduria due to the frequent finding of Candida species) refers to fungus in the urine (fungal UTI of the bladder or kidney). It is a common nosocomial infection. Organisms are typically C. albicans and C. glabrata. Other organisms can involve the kidney through disseminated infection (eg, Aspergillus sp., Fusarium, others). Associated predisposing factors include catheters, antibiotics, diabetes mellitus, hospitalization, and immunocompromised states. Urinary colonization is usually asymptomatic, whereas invasive fungal infection of bladder may have irritative voiding symptoms. Fungal infection of the kidney is often hematogenous in origin from other sources or the GI tract; fungal renal or perirenal abscesses present similar to pyelonephritis. Infection should be suspected when urine microscopy shows budding fungal hyphae. Positive fungal urine culture demands investigation. (See Section I: “Fungal Infections, Genitourinary”; Section II: “Bezoars, Genitourinary.”)
TREATMENT
• Remove predisposing factors
• Asymptomatic candiduria rarely requires antifungal therapy (unless in the setting of neutropenia, neonates, or urinary tract instrumentation):
– Fluconazole, 200–400 mg (3–6 mg/kg) daily for several days if urinary tract instrumentation is planned
• Symptomatic cystitis: Fluconazole 200 mg (3 mg/kg) daily for 2 wk or amphotericin B 0.3–0.6 mg/kg for 1–7 days; or flucytosine 25 mg/kg QID for 7–10 days. Amphotericin B bladder irrigation is recommended only for fluconazole-resistant organisms (eg, C. krusei and C. glabrata).
• Symptomatic infections should be treated
• Bezoars should be removed
REFERENCE
Pappas PG, Kauffman CA, Andes D, et al. Clinical practice guidelines for the management of candidiasis: 2009 update by the Infectious Diseases Society of America. Clin Infect Dis. 2009;48:503–535.
FUNICULITIS
DESCRIPTION Inflammation of the spermatic cord; the entire spermatic cord is subject to inflammatory diseases, usually as a result of trauma or pyogenic bacteria, and this condition is occasionally seen with scrotal inflammation or epididymitis. Parasitic infections (filariasis, schistosomiasis) can also induce inflammatory changes in the cord (see also Section I: “Spermatic Cord Mass and Tumors.”)
REFERENCE
Sabiston D. Textbook of Surgery. 19th ed. Philadelphia, PA: Saunders; 2012.