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SAM 1: Bicycle injury with scrotal pain. What is the most 2014 SPR Postgraduate important diagnosis from the urologist’s perspective? Course A. Testicular Torsion B. Testicular Hematoma

Module 4: Genitourinary C. Testicular Infarction

Moderators: Lane F. Donnelly, MD and Pinar Karakas, MD D. Testicular Rupture E. Washington DC

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14 year old boy with bicycle–related straddle injury presents 14 year old boy with bicycle–related straddle injury presents with left with left scrotal pain, erythema and edema. Based on the scrotal pain, erythema and edema. Based on the ultrasound images, ultrasound images, what is the most important diagnosis from what is the most important diagnosis from the urologist’s perspective? the urologist’s perspective? • Option C is not correct. Testicular infarction is identified by lack of blood flow within the on color Doppler imaging and may coexist with testicular rupture. No color Doppler images were included in this • Answer is D. question, therefore it is not possible to evaluate for this. • Longitudinal and transverse images of the left show an enlarged heterogeneous left testicle with • Option D is correct. In testicular rupture, there is hemorrhage and extrusion of testicular indistinct lower testicular margin indicating disruption of the echogenic linear tunica albuginea contents into the scrotal sac. Discontinuity of the echogenic tunica albuginea is indicative of surrounding the testicle, confirming testicular rupture. Adjacent heterogeneously echogenic material testicular rupture and necessitates emergent surgery in most urologist’s training and experience likely represents extruded testicular material as well as associated extratesticular hematoma/hematocele (although a conservative approach has been advocated in some instances recently). and more peripheral less complex . • Option E is not correct. Hematocele, a complex collection containing blood that separates the visceral • Option A is not correct. Testicular torsion should always be kept in mind when interpreting a post- and parietal layers of the , is acutely echogenic and becomes more complex and more traumatic as torsion may coexist. Decreased or absent intra-testicular Doppler flow, hypoechoic with age. Although present here, this is not the salient finding that will alter urologic abnormal transverse lie of the testicle or absent cremasteric reflex should raise suspicion for testicular management. torsion. No Doppler flow images are included with this question; therefore evaluation of testicular References perfusion and torsion cannot be made. • Pogorelic Z, Juric I, Biocic M et al. Management of testicular rupture after blunt trauma in children. Pediatric Surgery International 2011; 27: 885-889. • Cubillos J, Reda EF, Gitlin J et al. A conservative approach to testicular rupture in adolescent boys. The Journal of Urology 2010; 184, 1733-1738. • Option B is not correct . Although the testicle is heterogeneous, no focal intratesticular hematoma is • Adams RJ, Attia M, Cronan K. Report of 4 cases of testicular rupture in adolescent boys secondary to sports-related trauma. Pediatric Emergency Care visible on the provided images. Testicular hematoma is usually focal, but may be multiple, and may be 2008; 24 (12), 847-848. • Deurdulian C. Mittelstaedt CA, Chong WK, Fielding JR. US of acute scrotal trauma: optimal technique, imaging findings and management. Radiographics hyperechoic (in acute bleeding) or hypoechoic (as the hemorrhage ages) and lacks vascularity. Although 2007; 27: 357-369. testicular hematoma may coexist with testicular rupture, if present alone with maintained adjacent • Guichard G, Ammari JE, Del Coro C et al. Accuracy of Ultrasonography in diagnosis of testicular rupture after blunt scrotal trauma. Journal of Urology 2008; testicular perfusion, is treated non-operatively. 71 (1), 52-56. 2014 SPR PG Course 2014 SPR PG Course

1 14 year old boy with bicycle–related straddle injury SAM 2: Scrotal injury two weeks ago; persistent pain and presents with left scrotal pain, erythema and edema. swelling but no fever. What is the leading diagnosis and Based on the ultrasound images, what is the most appropriate management? important diagnosis from the urologist’s perspective? A. Testicular hematoma, • Answer is A. Ultrasound follow-up • Longitudinal and transverse ultrasound images of the right testicle (and left testicle included on transverse gray scale image) without and with color Doppler show focal heterogeneous echogenicity B. Testicular hematoma, (with several septated cystic components in addition to solid component) in the upper right testicle observation only anteriorly with decreased flow, relative to the remainder of the right testicle, and left testicle. There is a background of testicular microlithiasis bilaterally and anterior right scrotal edema. C. Testicular abscess, • Option A is correct. Testicular hematoma is usually focal, but may be multiple, and may be antibiotic therapy hyperechoic (acutely) or hypoechoic (as the hemorrhage ages) and lacks vascularity. Given the history of trauma, the most likely diagnosis is testicular hematoma, however the differential D. Testicular abscess, diagnosis includes neoplasm and less likely focal infarction or focal orchitis/abscess. Therefore ultrasound follow up is indicated and will show evolution and decreased size of hematoma over time (whereas a neoplasm will not change or slightly increase in size). The lack of internal E. Testicular neoplasm, vascularity is helpful as most intra-testicular tumors greater than 1cm in size will have internal Ultrasound follow-up vascularity with color Doppler imaging. F. Testicular neoplasm, • Option B is not correct. Testicular hematoma is the correct answer; however observation only instead of follow up testicular ultrasound is incorrect. Follow up ultrasound is indicated for reasons stated in option orchiectomy A response above. 2014 SPR PG Course 2014 SPR PG Course

14 year old boy with bicycle–related straddle injury presents with left scrotal pain, erythema and edema. Based on the ultrasound images, SAM 3: 2 year old with left scrotal pain. Left what is the most important diagnosis from the urologist’s perspective? • Option C is not correct. Testicular abscess could have this imaging appearance; however, the history stated that this blood flow is: patient does not have fever. This highlights the importance of correlating the imaging findings with clinical history, as often there may be no provided history of trauma or minor trauma may have been forgotten. If there was clinical concern for testicular infection (fever, white blood cell elevation, elevated inflammatory markers), antibiotics would be the correct initial treatment. • Option D is not correct. As stated in option C response above, testicular abscess could have this imaging appearance but does not correlate with the provided history. However, orchiectomy would not be the initial management for focal testicular infection. A. Normal • Option E is not correct. Intratesticular hematoma may be mistaken for a primary testicular malignancy by ultrasound if a detailed clinical history is not correlated. There is a differential diagnosis for an intratesticular lesion, as stated in B. Decreased option A response above. Follow up ultrasound is indicated, with orchiectomy performed if the lesion does not evolve and retract over time, as would be expected with an intratesticular hematoma. C. Increased • Option F is not correct. Intratesticular hematoma may be mistaken for a primary testicular malignancy by ultrasound if a detailed clinical history is not correlated. There is a differential diagnosis for an intratesticular lesion, as stated in D. Indeterminate option A response above. Follow up ultrasound is indicated, with orchiectomy performed if the lesion does not evolve and retract over time, as would be expected with an intratesticular hematoma. References • Purushothaman H, Sellars MRK, Clarke JL, Sidhu PS. Intratesticular haematoma: differentiation from tumour on clinical history and ultrasound appearances in two cases. The British Journal of 2007; 8, e184-e187. • Bhatt S, Jafri ZH, Wasserman N, Dogra VS. Imaging of non-neoplastic intratesticular masses. Diagnostic and Interventional Radiology, Turkish Society of Radiology 2011; 17; 52-63. • Deurdulian C. Mittelstaedt CA, Chong WK, Fielding JR. US of acute scrotal trauma: optimal technique, imaging findings and management. Radiographics 2007; 27: 357-369. • Sung EK, Setty BN, Castro-Aragon I. Sonography of the pediatric scrotum: Emphasis on the Ts—Torsion, Trauma and Tumors. AJR 2012; 198: 996-1003.

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2 Two year old with left scrotal pain. Blood flow on the left is: SAM 4: The most likely • Answer: B diagnosis for this child • Rationale: Images demonstrate blood is: flow to both testes with power and color Doppler. Spectral Doppler demonstrates an asymmetric appearance with a low resistance pattern on the symptomatic A. Normal left side. A small left hydrocele is also partially visualized. Option A is not B. Torsed appendix testis correct because the flow is asymmetric. Option C is not correct because the flow C. Testicular torsion is diminished on the symptomatic side. Option D is not correct because there is D. Testicular fracture decreased flow on the side of clinical concern.

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The most likely diagnosis for this child is: SAM 5: Teenager with 8 hours • Correct answer C. References right scrotal pain. Which is • Rationale: Images demonstrate blood • Boettcher M, Bergholz R, Krebs TF, et al. Differentiation of flow to both testes with power and color and appendix testis torsion by clinical and ultrasound signs in children. Urology 2013; 82:899-904. true? Doppler. Spectral Doppler demonstrates • Cassar S, Bhatt S, Paltiel HJ, Dogra VS. Role of spectral an asymmetric appearance with a low Doppler sonography in the evaluation of partial testicular torsion. resistance pattern on the symptomatic J Ultrasound Med 2008; 27:1629-1638. A. The diagnosis of left testicular torsion is • Dogra VS, Rubens DJ, Gottlieb RH, Bhatt S. Torsion and left side. A small left hydrocele is also beyond: new twists in spectral Doppler evaluation of the scrotum. clear. partially visualized. The patient was J Ultrasound Med 2004; 23:1077-1085. • Karmazyn B, Steinberg R, Livne P, et al. Duplex sonographic B. Left testis viability is likely. taken to surgery and there was testicular Image 1A torsion with a 360 degree twist in the findings in children with torsion of the testicular appendages: overlap with epididymitis and epididymoorchitis. Journal of C. Indications for manual detorsion are well- spermatic cord. Pediatric Surgery 2006; 41:500-504. supported by clinical context and • Option A is not correct because there is • Munden MM, Williams JL, Zhang W, Crowe JE, Munden RF, Cisek LJ. Intermittent testicular torsion in the pediatric patient: sonographic features. decreased flow on the symptomatic side. sonographic indicators of a difficult diagnosis. AJR Am J Option B is not correct because the Roentgenol 2013; 201:912-918. D. Detorsion should be monitored by CDU. testicular appendage is not demonstrated • Paltiel HJ, Rupich RC, Babcock DS. Maturational changes in in the given images and because the flow arterial impedance of the normal testis in boys: Doppler sonographic study. American Journal of Roentgenology 1994; E. All of the above. is diminished on the symptomatic side. 163:1189-1193. Option D is not correct because the are both intact. 2014 SPR PG Course 2014 SPR PG Course

3 Pre-detorsion Teenager with 8 hours scrotal pain Which is SAM 6: Subsequent true? management must be:

References • Answer is E. The sonographic diagnosis of • Kalfa N, Veyrac C, Lopez M, Lopez C, Maurel A, Kaselas C, Sibai S, A. Conservative with close torsion is based upon the triad of: Arena F, Vaos G, Bréaud J, Merrot T, Kalfa D, Khochman I, Mironescu A, Minaev S, Avérous M, Galifer RB. Multicenter assessment of clinical monitoring – testicular swelling ultrasound of the spermatic cord in children with acute scrotum. J Urol. – knot sign (visibility of the coiled cord) 2007;177(1):297-301. B. Conservative with close US • Middleton WD, Middleton MA, Dierks M, Keetch D, Dierks S. – absence of testicular flow at CDU Sonographic prediction of viability in testicular torsion: preliminary surveillance • Testicular viability can be inferred from the observations. J Ultrasound Med. 1997;16(1):23-7. • Kaye JD, Shapiro EY, Levitt SB, Friedman SC, Gitlin J, Freyle J, preservation of the normal echostructure. Palmer LS. Parenchymal echo texture predicts testicular salvage after C. Discharge from the • Preoperative manual detorsion can restore torsion: potential impact on the need for emergent exploration. J Urol. emergency room testicular perfusion in cases when time delay 2008;180:1733-6. • Chmelnik M, Schenk JP, Hinz U, Holland-Cunz S, Günther P. Testicular (duration of torsion, access to operating room) torsion: sonomorphological appearance as a predictor for testicular D. Elective surgery is an additional threat to subsequent testicular viability and outcome in neonates and children. Pediatr Surg Int. viability. In the presented case, the abnormal 2010;26(3):281-6. E. Rapid surgical exploration • Garel L, Dubois J, Azzie G, Filiatrault D, Grignon A, Yazbeck S. contralateral testis was a further clinical Preoperative manual detorsion of the spermatic cord with Doppler and bilateral orchidopexy incentive for immediate action. ultrasound monitoring in patients with intravaginal acute testicular torsion. Pediatr Radiol. 2000;30(1):41-4. Post-detorsion with 2014 SPR PG Course 2014 SPR PG ImmediateCourse pain relief

Subsequent management must be: SAM 7: 14-year-old genetic and phenotypic boy. Most References: • Answer is E. • Sessions AE, Rabinowitz R, Hulbert WC, Goldstein MM, Mevorach • Manual detorsion is not a substitute for RA. Testicular torsion: direction, degree, duration and disinformation. J likely diagnosis? exploration and fixation, and immediate bilateral Urol. 2003;169(2):663-5. • Haynes BE, Haynes VE. Manipulative detorsion: beware the twist that orchidopexy remains imperative. Indeed, does not turn. J Urol. 1987;137(1):118-9. residual torsion can still be present at surgery • Cattolica EV. Preoperative manual detorsion of the torsed spermatic A. Seminoma despite restoration of flow at CDU. Moreover, cord. J Urol. 1985;133(5):803-5. • Kiesling VJ Jr, Schroeder DE, Pauljev P, Hull J. Spermatic cord block the underlying anatomic predisposition (bell- and manual reduction: primary treatment for spermatic cord torsion. J B. Testicular tumor of adrenogenital clapper deformity) is a definite risk factor for Urol. 1984;132(5):921-3. syndrome torsion recurrence, ipsilateral or contralateral. • Betts JM, Norris M, Cromie WJ, Duckett JW. Testicular detorsion using Doppler ultrasound monitoring. J Pediatr Surg. 1983;18(5):607-10. C. Nonseminomatous germ cell tumor D. Lymphoma E. Orchitis

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4 14-year-old boy. Which is the most likely diagnosis? 14-year-old boy. Which is the most likely diagnosis?

• US demonstrates multiple bilateral hypoechoic nodules clustered around the mediastinum testis • Option D is not correct. Although lymphoma is often bilateral, the testes are diffusely infiltrated which are traversed by vessels on the color Doppler image. These are the characteristic findings and enlarged with disorganized increased vascularity, while these testes show bilateral small of testicular tumor of adrenogenital syndrome which is typically bilateral. focal masses clustered around the mediastinum testis.

• Option A is not correct. Seminomas are rare in the pediatric population, are typically unilateral, • Option E is not correct. Orchitis is typically unilateral, with ill-defined hypoechogenicity and and exert mass effect. Bilateral seminomas may occur in adult patients with abdominal or pelvic increased flow. The epididymis is also enlarged and hypoechoic with increased flow. gonads in some patients with DSD. These testes are in the scrotum. • Option B is correct. Testicular tumors of adrenogenital syndrome typically are bilateral, cluster • 1. Stikkelbroeck NM, Suliman HM, Otten BJ, Hermus AR, Blickman JG, Jager GJ. Testicular around the mediastinum testis, hypoechoic when small, and do not exert mass effect on vessels adrenal rest tumours in postpubertal males with congenital adrenal hyperplasia: sonographic and which traverse through the masses. When large, the testicular tumors may appear hyperechoic MR features. Eur Radiol 2003;13(7):1597–1603. with posterior acoustic shadowing. • 2. Moshiri M, Chapman T, Fechner PY, et al. Evaluation and management of disorders of sex • Option C is not correct. Nonseminomatous germ cell tumors are typically heterogeneous in development: multidisciplinary approach to a complex diagnosis. echotexture, almost always unilateral and exert mass effect, although bilateral germ cell tumors • 3. Shah RU, Lawrence C, Fickenscher KA, et al. Imaging of pediatric pelvic neoplasms. Radiol may occur in abdominal or pelvic gonads in some patients with DSD. These testes are in the Clin North Am. 2011 Jul;49(4):729-48. scrotum. • 4. Carkaci S, Ozkan E, Lane D, Yang WT. Scrotal sonography revisited. J Clin Ultrasound. 2010 Jan;38(1):21-37.

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SAM 8: 4 year old female with 72 hours right A four-year-old female with right lower quadrant pain for 72 lower quadrant pain. What is true? hours. Which of the following is true?

• Answer: C • Rationale: The most common imaging finding suggestive of an adnexal torsion is asymmetrical enlargement of the ovary (>5cm or 5-6 times larger than the contralateral ovary) with multiple peripheral cysts with or without an associated mass. A twisted vascular pedicle is the most specific and diagnostic finding of an ovarian torsion. The twisted vascular A. There is a right adnexal cystic mass. pedicle appears as a “whirlpool” sign of concentric B. There is a right adnexal enhancing solid mass. vessels. Both of these findings are seen in this case (option C is the correct answer). C. There is right adnexal torsion. • There is no solid or cystic mass in the ovary and the D. There may be a right ovarian torsion but this needs confirmation with ultrasound. enhancing structure in the right ovary is the twisted E. There is right ovarian edema due to an extra-ovarian cause such as vascular pedicle (A and B are not correct). appendicitis.

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5 A four-year-old female with right lower quadrant pain for 72 SAM 9: 16-year-old female with 18 hours right lower quadrant hours. Which of the following is true? pain. What is true? • The diagnostic performance of CT is similar to US in identifying ovarian torsion and identification of the torsed pedicle is diagnostic. If a CT exam demonstrates findings of ovarian torsion another imaging exam (i.e. US) may delay therapy and is unlikely to improve preoperative diagnostic yield (option D is not correct). • Although an inflamed appendix or Meckel’s diverticulitis can be closely related to an ovary and cause reactive inflammation and edema there are no findings to suggest this (option E is not correct). Findings are conclusive of ovarian torsion. References A. Periadnexal fat stranding is suggestive of an underlying infectious etiology. • Swenson DW, Lourenco AP, Beaudoin FL, Grand DJ, Killelea AG, McGregor AJ. Ovarian torsion: Case-control study comparing the sensitivity and specificity of ultrasonography and B. A “beak sign” as seen in this case can confirm tubal torsion. computed for diagnosis in the emergency department. Eur J Radiol. 2014 Jan 8. pii: S0720-048X(14)00002-3. doi: 10.1016/j.ejrad.2014.01.001. [Epub ahead of print] C. There is an isolated tubal torsion. • Rha S, Byun J, Jung S, et al. CT and MR imaging features of adnexal torsion. D. Since there is no intrinsic mass lesion in the right ovary, ovarian torsion is unlikely. Radiographics. 2002;22:283-294. • Duigenan S, Oliva E, Lee SI. Ovarian Torsion: Diagnostic Features on CT and MRI With E. Rightward uterine deviation suggests a short utero-ovarian ligament increasing risk of Pathologic Correlation. AJR Am J Roentgenol. 2012 Feb;198(2):W122-31. • Wilkinson C, Sanderson A. Adnexal torsion -- a multimodality imaging review. Clin Radiol. torsion. 2012 May;67(5):476-83.

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A 16-year-old female with right lower quadrant pain for 18 hours. Which of the following is true? Touch the finding in • Answer: E • Periadnexal fat stranding is a nonspecific finding and can be seen in infectious, inflammatory or other this VCUG that processes such as ovarian or tubal torsion (option A is an incorrect statement). • Torsion of the adnexa may involve the ovary (~31%), the fallopian tube (~10%), or both (~59%). The clinical indicates the presence presentation is usually indistinguishable and accurate imaging diagnosis is very difficult especially in isolated tubal torsion. In this case there is enlargement of the ovary, twisted ovarian pedicle and cystic enlargement of the tube with “beak sign” suggesting simultaneous torsion of the tube and the ovary (Option B is a correct of Mullerian duct statement and option C is an incorrect statement). Most common cause of isolated tubal torsion is a tubal or paratubal cyst. structures • A normal ovary can torse due to disproportionately elongated utero-ovarian ligament; jarring movements of a relatively large ovary in a small infant; and associated Müllerian anomalies (Option D is an incorrect statement). • The uterine deviation towards the site of the torsion occurs due to shortening of the utero-ovarian ligament and tube. Hence, the deviation is the result of the torsion and not the cause (Option E is an incorrect statement). References • Duigenan S, Oliva E, Lee SI. Ovarian torsion: diagnostic features on CT and MRI with pathologic correlation. AJR Am J Roentgenol. 2012 Feb;198(2):W122-31. Gross M, Blumstein SL, Chow LC (2005) Isolated fallopian tube torsion: a rare twist on a common theme. AJR 185:1590– 1592 Orazi C, Inserra A, Lucchetti MC et al (2006) Isolated tubal torsion: a rare cause of pelvic pain at menarche: sonographic and MR findings. Pediatr Radiol 36:1316–1318 Wilkinson C, Sanderson A. Adnexal torsion -- a multimodality imaging review. Clin Radiol. 2012 May;67(5):476-83.

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6 Touch the finding in this VCUG that indicates the Touch the finding in presence of Mullerian duct structures

this VCUG that • The image shows abnormal filling of a tubular structure posterior to the urethra as well as the urethra and bladder. The superior cervical indicates the presence impression on this structure, representing the vagina in a patient with UG of Mullerian duct sinus, indicates the presence of mullerian structures in this patient.

structures • Moshiri M, Chapman T, Fechner PY, et al. Evaluation and management of disorders of sex development: multidisciplinary approach to a complex diagnosis.

• Chavhan GB, Parra DA, Oudjhane K, et al. Imaging of ambiguous genitalia: classification and diagnostic approach. Radiographics. 2008 Nov-Dec;28(7):1891-904.

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You are shown a sagittal ultrasound image of the pelvis in a Sagittal pelvic US in a newborn with ambiguous genitalia. newborn with ambiguous genitalia. Please indicate the uterine Touch the uterine cervix. cervix.

Answer:

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7 9-year-old female with RLQ pain. Please indicate the portion of 9-year-old female with RLQ pain. Please indicate the portion the lesion that is diagnostic. of the lesion that is diagnostic.

• Diagnosis: mature ovarian • Explanation: are neoplasms defined by the presence of tissue from all three primitive cell lines—endoderm, mesoderm, and ectoderm. While many neoplasms originating in the ovary may have cystic or calcified components, teratomas are the only one that contains fat.

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A 17 year old girl with hirsutism. Please point A 17 year old girl with hirsutism. Please point out the abnormality. out the abnormality.

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8 SAM 10: Undescended testis. The most likely A 17 year old girl with hirsutism has a pelvic MR diagnosis based on the appearance of the vas and performed. Please point out the abnormality. internal spermatic vessels is

• DIAGNOSIS: Enlarged left ovary with References peripheral cysts and central stromal • Barber TM, Alvey C, Greenslade T et-al. prominence is consistent with polycystic A. Normal vas and vessels entering the Patterns of ovarian morphology in polycystic ovarian morphology canal – inguinal testis. ovary syndrome: a study utilizing magnetic Int ring • EXPLANATION of the correct answer: MRI resonance imaging. Eur Radiol. 2010;20 (5): B. Atretic vas and vessels – absent testis. demonstrates an enlarged ovary with more 1207-13. doi:10.1007/s00330-009-1643-8 – than 12 follicles similar in size measuring 2-9 C. Normal vas and atretic vessels – high • Brown M, Par A, Shayya R et al Ovarian mm. The distribution is peripheral with a imaging by MR in adolescent girls with PVOS intraabdominal testis. “string of pearls” appearance. The central Vas and age matched controls J Magn Reson stroma is prominent. In this patient with D. Atretic vas and normal vessels – low Imagin Epub Jan 4 2013 hirsutism and polycystic ovarian morphology intraabdominal testis. (PCOM) , PCOS should be considered in the Int spermatic vessels differential.

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Undescended testis. The most likely diagnosis based on the appearance of the vas and internal spermatic vessels is SAM: Laparoscopic findings for intraabdominal testis that necessitate a two stage Fowler-Stephens • Answer is B. • The laparoscopic image shows atretic vas and vessels. After making this observation, the include diagnosis is absent testis. • Option B is correct. The diagnosis is absent testis. No further exploration is necessary. This is A. Being able to maneuver the testis to the contralateral internal inguinal different from the situation where cord structures enter the internal inguinal ring which mandates an exploration. An atrophic testis within the canal should be resected because of its risk for ring. testicular cancer. A normal testis may occasionally be identified in the canal in obese patients or B. Seeing an atrophic testis with a diminutive vascular cord. when the internal inguinal canal is open and the increased intraabdominal pressure from CO2 insufflation pushes a low abdominal testis into the inguinal canal. C. Identifying a testis with a vascular cord that will not reach the • Option A is not correct. The vas and vessels are not normal in appearance. scrotum. • Option C is not correct. The vas is not normal in appearance. D. Finding normal cord structures entering the internal inguinal ring. • Option D is not correct. The vessels are not normal in appearance. References • Gundeti MS, Wilcox DT. Impalpable testis. In: Godbole PP, ed. Pediatric Endourology Techniques. London: Springer- Verlag, 2007; 49-53. • Esposito C, Caldamone AA, Settimi A, El-Ghoneimi A. Management of boys with nonpalpable undescended testis. Nature Clinical Practice Urology, 2008; 5:252-260.

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9 Laparoscopic findings for intraabdominal testis that In this newborn with ambiguous genitalia and nonpalpable necessitate a two stage Fowler-Stephens orchiopexy testes, touch the US feature confirms the gender? include • Answer is C. • Option C is correct. A testis with a vascular cord that will not reach the scrotum requires a two stage orchiopexy. The risk of testicular loss is high if the blood vessel supplying the vas is stretched too much. Therefore a conventional or single stage procedure is not performed. • Option A is incorrect. If the testis is can be displaced to the contralateral inguinal ring, the vascular cord is long enough for a conventional orchiopexy. • Option B is incorrect. An atrophic testis is usually resected. • Option D is incorrect. Normal cord structures entering the internal inguinal ring indicate that a testis or remnant is in the inguinal canal. A standard orchiopexy can be performed. A remnant can be resected. Left inguinal region long References Bladder long • Hutson JM, Clarke MCC. Current management of the undescended testicle. Seminars in Pediatric Surgery, 2007;16; 64-70. • Laparoscopic Methods for Implalpable Testis. In Hinman F, Atlas of Pediatric Urologic Surgery, W B Saunders, 1994, 513-519.

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In this newborn with ambiguous genitalia and nonpalpable SAM: 14 year old with 6 week follow-up US for a large testes, what US feature confirms the gender? hemorrhagic adnexal cyst. Current scan is in the first half of the menstrual cycle. How should this lesion be managed?

A. Surgical consult due to size. B. MRI with and without Bladder long Left inguinal region long contrast for mural nodule. C. Continued US follow-up in 6 weeks to assess for • DIAGNOSIS or FINDING TO BE MADE: A testis is present in the left inguinal region. No uterus is seen posterior to the bladder resolution. • EXPLANATION of the correct answer: This patient has a disorder of sexual differentiation with microphallus and no palpable testes in the scrotum. On sonography, a testis is seen in the left D. No follow-up is necessary inguinal region and there is no uterus. Therefore, the patient is male. as finding is not abnormal.

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10 14-year-old with 6 week followup ultrasound for large hemorrhagic cyst. Current scan is in the first half of the SAM: 14-year-old girl with RLQ pain shows a complex lesion (arrow) menstrual cycle. How should this lesion be managed? without internal vascular flow. How should this lesion be managed? • Answer is D. A. Surgical consult for possible • The image demonstrates a dominant ovarian follicle containing a well-defined curved line at the periphery representing the cumulus oophorus of a mature ovum. torsion. • Option A is not correct. The finding is physiologic and does not require surgery. A simple ovarian cyst, B. MRI with and without contrast otherwise asymptomatic, must persist for several menstrual cycles and be larger than 7 cm to be consisted surgical. for malignancy. • Option B is not correct. The peripheral cyst within the follicle is not a solid lesion and, therefore, not a mural nodule. C. Follow-Up US in the • Option C is not correct. A normal finding does not require follow-up. approximately 6 weeks for • Option D is correct. Dominant follicles can measure up to 3 cm in diameter. The peripheral size and resolution. cyst within the follicle represents the mature ovum. This configuration is seen just prior to ovulation. D. No follow-up is necessary. References: • Ackerman S, Irshad A, Lewis M, Anis M. Ovarian Cystic Lesions: A current Approach to Diagnosis and Management. In: Lalwani N and Dubinsky TJ, eds. Female Pelvic Imaging. Radiol Clin N Am 51(2013);1067-1085. • Laing FC and Allison SJ. US of the Ovary and Adnexa: To Worry or Not to Worry? RadioGraphics 2012; 32:1621-1639. • Brown DL, Dudiak KM, and Laing FC. Adnexal Masses: US Characterization and Reporting. Radiology 2010; 254:342-354.

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14-year-old girl with RLQ pain shows a complex lesion (arrow) SAM: 16 year old with amenorrhea. What question should the without internal vascular flow. How should this lesion be managed? radiologist ask to confirm the diagnosis of PCOS? • Answer A is correct. • The image shows a complex lacy lesion within a solid mass without internal vascular flow. The A. What are the ovarian volumes? right ovary was not otherwise seen. At surgery, the lesion was a torsed right ovary with internal hemorrhage. B. How long has the patient had • Option A is correct. An enlarged solid lesion without vascular flow causing pain in a young amenorrhea? female should raise suspicion for torsion when the ovary is not otherwise seen. Surgical consult is warranted. C. Does the patient have acne or • Option B is not correct. While an ovarian lesion of any type may be present, serving as a lead point obesity? for torsion, the patient’s symptoms coupled with the ultrasound finding make surgery rather than further imaging the correct next step in management. D. Is the patient sexually active so • Option C is not correct. The lack of vascular flow in a solid lesion that could be the ovary puts that a transvaginal US can be torsion in the differential considerations. Torsion is an emergency, and imaging follow up in 6 weeks is not appropriate management at presentation. performed? • Option D is not correct. The finding is abnormal so further management is required. References: E. All of the above. • Ziereisen F, Guissard G, et.al. Sonographic imaging of the paediatric female pelvis. Eur Radiol 2005; 15:1298-1309. • Brown DL, Dudiak KM, and Laing FC. Adnexal Masses: US Characterization and Reporting. Radiology 2010; 254:342-354. • Schultz KAP, Ness KK, et. al. Adnexal masses in infancy and childhood. Clin Obstet Gynecol 2006; 49(3): 464-479.

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11 16 year old with amenorrhea. What question should the 6 year old with amenorrhea. What question should the radiologist ask to confirm the diagnosis of PCOS? radiologist ask to confirm the diagnosis of PCOS?

• Answer is E. • Option D is not correct. While TVUS is useful in further assessment of follicular distribution • Option A only is not correct. While ovarian volume of >10.8 ml is one criteria in the diagnosis and size, Polycystic Ovarian Morphology (PCOM) with cysts and echogenic stroma has a high of PCOS in adolescents, many normal adolescents have large ovarian volumes. The presence prevalence in normal adolescents. Thus TVUS, while helpful in documenting cyst number, size of enlarged ovaries is not sufficient to confirm the diagnosis of PCOS. In addition, adolescents and distribution, is not specific for the diagnosis of PCOS and may be contraindicated in young with PCOS may have ovarian volumes < 10 ml. nonsexually active girls. • Option B only is not correct. Oligo / anovulation may be physiologic. Thus, anovulation must • Option E is correct. be present for two years after menarche or primary amenorrhea present at age 15 to • While the transabdominal ultrasound demonstrates bilaterally enlarged ovaries, all the above suggestive PCOS, with additional criteria required to confirm the diagnosis questions are important in helping further confirm the diagnosis of PCOS. • Option C only is not correct. Clinical features including obesity , insulin resistance, and References • Lakhani K, Seifalian AM, Atiomo WU et-al. Polycystic ovaries. Br J Radiol. 2002;75 (889): 9-16. hyperandrogenism are associated with PCOS. However, hyperandrogenism can be present in • Atiomo WU, Pearson S, Shaw S et al. Ultrasound criteria in the diagnosis of polycystic ovary syndrome (PCOS). Ultrasound Med Biol. 2000;26 normal adolescents (75%) with acne. Thus, acne can only be considered abnormal if it (6): 977-80. develops early, is inflammatory, persistent, severe and/or unresponsive to therapy. • Carmina E, Oberfield S, Lobo R. The diagnosis of PCOS in adolescents. Am J Obstet Gynecol 2010:203 :201 e 201-205 Biochemical hyperandrogenemia may be difficult to document. With obesity present in over • Horn M, Geraci S. P olycystic ovary syndrome in Adolescents. Southern Medical Journal 2013:106:10 570-576 • Franks S. Polycystic ovary syndrome in Adolescents. International journal of Obesity 2008:32:1035-1104 17% of normal adolescents this marker is weak for PCOS in the adolescent population as well. • Balen A, Laven J Tan S et al. US assessment of the polycystic ovary: international consensus definitions. Hum Reprod Update 2003 9:505-514 • Jonard S, Robert Y, Cortet-Rudelli C et-al. Ultrasound examination of polycystic ovaries: is it worth counting the follicles? Hum. Reprod. 2003;18 (3): 598-603.

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16 year old obese girl, anovulatory for 3 months. The ovarian volumes were over 16 year old obese girl, anovulatory for 3 months. Ovarian volumes were 22 mL bilaterally. Does the patient have over 22 mL bilaterally. Does the patient have PCOS? PCOS? A. No, as she has been amenorrheic for only 3 • Answer is E • Option A is not correct. Oligo / anovulatin may be physiologic. Anovulation should be present months for greater than 2 years since the start of menses or primary amenorrhea at age 15 to consider B. Yes, because ovarian volumes are > 10.8 mL the diagnosis of PCOS. In this patient we do not know how long her menses have been irregular. • Option B is not correct. Ovarian volumes can be > 10.8 ml in healthy young women or in C.Yes, because the ovaries have > 12 small adolescents with other ovarian pathology and thus cannot be the sole criteria in the diagnosis of peripheral follicles PCOS. Adolescents with PCOS may have ovarian volumes < 10 mL. D.Yes, because the ovarian volumes are > 10.8 • Option C is not correct. A Polycystic Ovarian Morphology (PCOM) prevalence of up to 30-40% has been describe in healthy girls and young women. Thus the presence of multiple peripheral cm, multiple small peripheral follicles are cysts cannot be the sole criteria in the diagnosis of PCOM. present and the patient is obese • Option D is not correct. With obesity present in over 17% of normal adolescents this marker is E. Maybe. Additional clinical features including relatively weak for diagnosing PCOS in the adolescent population. documentation of hyperandrogenism are required before making the final diagnosis.

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12 16 year old obese girl anovulatory for 3 months. The ovarian volumes 10 day old girl. Finding were over 22 mL bilaterally. Does the patient have PCOS?

• Option E is correct. PCOS is a prevalent disorder that can go unrecognized in the adolescent is bilateral. Diagnosis? because symptoms may be attributed to benign adolescent conditions or isolated obesity. However, over diagnosis can also be a problem as PCOM can be present in a normal healthy girl. • A. Inguinal adenopathy Understanding the issues in accurate diagnosis allows for improvement in early diagnosis and effective interventions, reducing long term morbidity and mortality. • B. Herniated bowel – While cut off points for identifying a pathologic degree of PCOM remain controversial, advanced imaging by US and/or MR should report ovarian volume, follicle size and number and can help confirm the diagnosis. Left Long • C. Disorder of sexual References • Azziz R, Carmina E, Dewailly D et al The Androgen Excess and PCOS Society criteria for the PCOS: The complete task force report. Fert Steril development 2009;91:456-488 • Lakhani K, Seifalian AM, Atiomo WU et-al. Polycystic ovaries. Br J Radiol. 2002;75 (889): 9-16. • Atiomo WU, Pearson S, Shaw S et al. Ultrasound criteria in the diagnosis of polycystic ovary syndrome (PCOS). Ultrasound Med Biol. 2000;26 (6): • D. Herniated “bladder ears” 977-80. • Carmina E, Oberfield S, Lobo R. The diagnosis of PCOS in adolescents. Am J Obstet Gynecol 2010:203 :201 e 201-205 • Horn M, Geraci S. P olycystic ovary syndrome in Adolescents. Southern Medical Journal 2013:106:10 570-576 • E. Incarcerated inguinal hernia • Franks S. Polycystic ovary syndrome in Adolescents. International journal of Obesity 2008:32:1035-1104 • Balen A, Laven J Tan S et al. US assessment of the polycystic ovary: international consensus definitions. Hum Reprod Update 2003 9:505-514 • Jonard S, Robert Y, Cortet-rudelli C et-al. Ultrasound examination of polycystic ovaries: is it worth counting the follicles? Hum. Reprod. 2003;18 (3): 598-603.

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10 day old girl. Finding is bilateral. 10 day old girl. Finding is bilateral. Diagnosis? Diagnosis? • Option A is not correct. The ultrasound shows the structure extending into the inguinal canal from the abdomen. • Moshiri M, Chapman T, Fechner PY, et al. Evaluation • Option B is not correct. Inguinal hernias commonly contain bowel but and management of disorders of sex development: this structure does not have the appearance of bowel which typically has a multilayered appearance to the wall. multidisciplinary approach to a complex diagnosis. • Option C is correct. The herniated structure is ovoid with anechoic follicles representing an ovary. Bilateral inguinal ovaries suggest disorder of sexual development. • Chavhan GB, Parra DA, Oudjhane K, et al. Imaging of • Option D is not correct. The structure is not contiguous with the bladder ambiguous genitalia: classification and diagnostic and is not filled with anechoic fluid. approach. Radiographics. 2008 Nov-Dec;28(7):1891- • Option E is not correct. The herniated structure does not resemble 904. bowel.

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13 Pre-menarchal female with intermittent RLQ pain. A premenarchal 14-year-old female presenting with intermittent Which is false? right lower quadrant pain. • A. There is uterine agenesis • B. There is ovarian torsion • Diagnosis: Uterine agenesis and References incomplete right ovarian torsion with • Sullivan EM, Golden NH, Adams Hillard • C. Sacrum is normal vascular twisting “whirlpool” sign on MRI. PJ. Ovarian torsion in a patient with • D. Ovarian follicles are müllerian agenesis: increased risk? J • Explanation: Ovarian torsion is more Pediatr Adolesc Gynecol. 2012 unremarkable common in cases of uterine agenesis due Dec;25(6):e125-8. • Kives SL, Bond SJ, Lara-Torre E. Müllerian to poor fixation of the ovary. The ovaries agenesis and ovarian torsion. A case are not tethered by the broad ligament, report and review of literature. J Pediatr mesosalpinx, or the utero-ovarian Surg. 2005 Aug;40(8):1326-8. Review. ligament and only attached laterally to the pelvic sidewall by the infundibulopelvic ligament, which carries the ovarian vasculature and nerves.

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SAM: Ultrasound in 12 year old girl with deepening voice and some facial Ultrasound in a 12 year old girl with deepening voice and some facial hair, hair, status post thyroidectomy 1 year earlier, shows a midline mass. The status post thyroidectomy 1 year earlier, shows a midline mass. The right right ovary was not visualized. The left ovary is normal. Elevated alpha- ovary was not visualized. The left ovary is normal. Elevated alpha- fetoprotein. The lesion is most likely of which adnexal tumor class? fetoprotein. The lesion is most likely of which adnexal tumor class?

A. Germ cell tumor • Answer is C. • The image shows a complex pelvic mass with cystic and solid components and with prominent vascular flow in the solid elements. The lesion was suspected to emanate from the right ovary B. Epithelial tumor as the ovary was not otherwise visualized. • Option A is not correct. Malignant germ cell tumors may elaborate alpha-fetoprotein. Most C. Sex cord stromal tumor commonly this marker is present with yolk sac tumors, immature teratomas, and mixed varieties. D. Hemangioma However, virilization is not a presentation feature. • Option B is not correct. Epithelial tumors usually have a more dominant cystic component, do E. Metastatic disease not elaborate alpha-fetoprotein, and do not cause virilization. • Option C is correct. This lesion is hormonally active based on the patient’s deepening voice and facial hair, making a sex cord stromal tumor the most likely diagnosis. Sex cord stromal tumors can elaborate various tumor markers although malignant germ cell tumors do so more frequently.

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14 US image with power Doppler of the pelvis in a 12 year old girl with deepening voice and some facial hair, status post thyroidectomy 1 year earlier, shows a midline mass. The right ovary was not visualized. The left ovary is normal. Tumor marker labs were positive for elevated alpha- fetoprotein. The lesion is most likely of which adnexal tumor class?

• Option D is not correct. Ovarian hemangiomas do not cause virilization. • Option E is not correct. Metastatic disease to the ovaries is rare in children. Most common neoplasms to spread to the ovaries are leukemia, lymphoma, neuroblastoma, and GI tumors. These lesions are usually solid, may be bilateral, and occur in the setting of widespread abdominal involvement. They are not characterized by virilization of the patient. References: • Anthony EY, Caserta MP, et.al. Adnexal Masses in Female Pediatric Patients. AJR 2012; 198(5):426–431. • Loh AH, Ong CL, et. al. Pediatric risk of malignancy index for preoperative evaluation of childhood ovarian tumors. Pediatr Surg Int 2012; 28(3):259-266. • Schultz KAP, Ness KK, et. al. Adnexal masses in infancy and childhood. Clin Obstet Gynecol 2006; 49(3): 464-479.

2014 SPR PG Course

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