Cystic Uterus Masculinus

1. Cystic Uterus Masculinus The Duderus. Jaime Rechy, DVM

2. Steps to Becoming a Real Boy • Testosterone • Leydig cells • Maintenance of Wolffian ducts • Ductusdeferans & epididymis • 5α-reductase converts testosterone to dihydrotestosterone • Formation of the prostate and external genitalia Vegter, et. al., "Persistent Mullerian Duct Syndrome in a Miniature Schnauzer Dog with Signs of Feminization and a Sertoli Cell Tumour.“ Disney.wikia.com Poth, T., W. Breuer, B. Walter, W. Hecht, and W. Hermanns. "Disorders of Sex Development in the Dog—Adoption of a New Nomenclature and Reclassification of Reported Cases." Animal Reproduction Science 121.3-4 (2010): 197-207. Print.

3. Steps to Becoming a Real Boy • Anti-Müllerian hormone (AMH) • Sertoli cells • Regression of Müllerian structures (uterine horns, uterus, cervix, portion of vagina) • Critical ambisexual stage of reproductive tract differentiation Meyers-Wallen, "Mullerian Inhibiting Substance Is Present in Embryonic Testes of Dogs with Persistent Mullerian Duct Syndrome” Gray, et. al., Gray's Anatomy. Edinburgh: C. Livingstone, 1989. Poth, T., et. al., "Disorders of Sex Development in the Dog—Adoption of a New Nomenclature and Reclassification of Reported Cases." Animal Reproduction Science 121.3-4 (2010): 197-207. Print.

4. The Uterus Masculinus • Persistent Müllerian Duct Syndrome (PMDS) • Form of inherited male pseudohermaphroditism • Masculinized XY male • Uterus and uterine horns • PMDS in humans • Autosomal-recessive trait • Miniature Schnauzer Vegter, et. al., "Persistent Mullerian Duct Syndrome in a Miniature Schnauzer Dog with Signs of Feminization and a Sertoli Cell Tumour.“ Atilola, et. al., “Cystic Uterus Masculinus in the Dog. Six Case History Reports."

5. PMDS: Of Mice and Men • Mutation in the AMH producing gene • Insensitivity of target organs • Mutation in AMH receptor gene • Canines *Absence of AMH action results in differentiation of Müllerian ducts www.gleuclassbooks.com Vegter, et. al., "Persistent Mullerian Duct Syndrome in a Miniature Schnauzer Dog with Signs of Feminization and a Sertoli Cell Tumour.“

6. Rusty Rowdy • CC: “Recurrent cystitis” • rDVM Hx: “For about 2 months has been urinating in his sleep and urinates on owners when picked up”

7. Rusty Rowdy • PE: bright, alert, and rowdy • Urogenital • Micropenis and testicular hypoplasia • Small UB, urethra and prostate WNL on rectal • Neurologically normal; no spinal/LS pain • Dribbled “urine” when picked up • Reducible umbilical hernia • Remainder of PE WNL

8. Diagnostic Imaging

12. Radiographic Conclusions • “Possible causes for the ovoid soft tissue mass adjacent to the urinary bladder include cystic uterus masculinus, paraprostatic cyst, or ureterocele.”

15. SAG

16. TRANS

19. Sonographic Conclusions • “Bi-cornuate fluid-filled thick-walled structure caudal and dorsal to the neck of the urinary bladder and connecting to the prostate is most consistent with cystic uterus masculinus which is a rare congenital defect associated with fused Müllerian ducts.”

22. Pre-contrast Post-contrast

23. CT Conclusions • “Irregular, fluid-filled structure is consistent with cystic uterus masculinus. No evidence of ectopic ureter.”

24. 6 dogs (Doberman, Golden, GSD, GSD X, Collie x 2); 4-11 years old • CS: Dysuria and constipation most common • Serosanguineous fluid inside • Bi-horned, cystic viscus; columnar epithelium Atilola, Matthew A. O., and Paul W. Pennock. "CYSTIC UTERUS MASCULINUS IN THE DOG. Six Case History Reports." Veterinary Radiology Ultrasound 27.1 (1986): 8-14. Web.

26. Sig: 10 month old neutered male cat • CS: Intermittent fever, lethargy, stranguria • PE: Purulent discharge below rectum • Sx: Bicornuate cystic structure terminated caudally into urethra • Klebsiella pyometra Schulman, Joanne. "Pyometra Involving Uterus Masculinus in a Cat." JAVMA 16.1 (2006): 50-53. Web.

27. Schulman, Joanne. "Pyometra Involving Uterus Masculinus in a Cat." JAVMA 16.1 (2006): 50-53. Web.

28. Sig: 6 year old neutered male cat • CC: Urethral obstruction • PE: Unable to acquire urine after catheterization • Dx: Catheter accidentally passed into UM Sfiligoi, Gabriella, Kenneth J. Drobatz, and H. Mark Saunders. "Uterus Masculinus: An Unusual Complication in a Case of Feline Urethral Obstruction." Journal of Veterinary Emergency and Critical Care 0.0 (2005): n. pag. Web.

30. Treatment for UM UB = uterine body B = urinary bladder • In humans, surgical excision is the definitive treatment of symptomatic Müllerian duct remnants • Surgery has been successful in veterinary patients Krstic, Zoran D. "Surgical Treatment of the Mullerian Duct Remnants." Journal of Pediatric Surgery 36 (2001): 870-76. Web. Atilola, Matthew A. O., and Paul W. Pennock. "CYSTIC UTERUS MASCULINUS IN THE DOG. Six Case History Reports." Veterinary Radiology Ultrasound 27.1 (1986): 8-14. Web.

31. Summary • Uterus masculinusis seen in the abnormal male as fused remnants of the Müllerian ducts • Form of male pseudohermaphroditism • “Zebra” for ddx in diagnostic imaging • Sig: male, dog/cat, +/- cryptorchid • Hx: UTI, dysuria, constipation, pyometra, prostatitis

32. References • Vegter, Ar, Hs Kooistra, Fj Van Sluijs, Lwl Van Bruggen, J. Ijzer, C. Zijlstra, and Ac Okkens. "Persistent Mullerian Duct Syndrome in a Miniature Schnauzer Dog with Signs of Feminization and a Sertoli Cell Tumour." Reproduction in Domestic Animals (2008): n. pag. Web. • Meyers-Wallen, V. N. "Mullerian Inhibiting Substance Is Present in Embryonic Testes of Dogs with Persistent Mullerian Duct Syndrome." Biology of Reproduction 48.6 (1993): 1410-418. Web. • Atilola, Matthew A. O., and Paul W. Pennock. "CYSTIC UTERUS MASCULINUS IN THE DOG. Six Case History Reports." Veterinary Radiology Ultrasound 27.1 (1986): 8-14. Web. • Adin CA, Proc. 8th Annual Dog Owners & Breeders Symposium. 2004. • Schulman, Joanne. "Pyometra Involving Uterus Masculinus in a Cat." JAVMA 16.1 (2006): 50-53. Web. • Sfiligoi, Gabriella, Kenneth J. Drobatz, and H. Mark Saunders. "Uterus Masculinus: An Unusual Complication in a Case of Feline Urethral Obstruction." Journal of Veterinary Emergency and Critical Care 0.0 (2005): n. pag. Web. • Meyers-Wallen, V. N. "The Critical Period for Mullerian Duct Regression in the Dog Embryo." Biology of Reproduction 45.4 (1991): 626-33. Web. • Krstic, Zoran D. "Surgical Treatment of the Mullerian Duct Remnants." Journal of Pediatric Surgery 36 (2001): 870-76. Web. • Poth, T., W. Breuer, B. Walter, W. Hecht, and W. Hermanns. "Disorders of Sex Development in the Dog—Adoption of a New Nomenclature and Reclassification of Reported Cases." Animal Reproduction Science 121.3-4 (2010): 197-207. Print.

33. 9/12/2013, 12:06 PM, CT ABDOMEN Medical Record: 243715 Patient Name: Rusty Rowdy Client Name: Palmer CT FINDINGS A transverse multidetector helical data set was acquired from T10-T11 to the proximal tibiae and reconstructed at 2 mm and 4 mm slice thickness, using a soft tissue algorithm. Additional datasets were acquired immediately, 3 minutes post-administration, and 5 minutes post-administration of contrast medium and similarly reconstructed. The urinary bladder is minimally distended and slightly displaced toward the left. Immediately dorsal to the urinary bladder, in the region of the trigone, there is an irregularly marginated, fluid-filled structure with peripheral contrast enhancement. The fluid filled lumen of this structure is also irregularly marginated and does not appear to communicate with the urinary bladder. This structure continues caudally to the level of the prostate. The insertion of the ureters is normal. Two tubular structures extend from the region of the trigone through the inguinal rings to connect to ovoid structures lying on either side of the prepuce in the inguinal area. The medial iliac and jejunal lymph nodes are at the upper limits of normal size. The stomach is distended with fluid and a moderate amount of kibble-like ingesta. The remaining abdominal structures are normal. CONCLUSIONS An irregular, fluid-filled structure dorsal to the trigone the prostate is consistent with a cystic uterus vasculitis. Bilateral cryptorchidism. Gastric distension warrants caution during anesthetic recovery. ELF

34. 9/11/2013, 11:27 AM, ABDOMEN Medical Record: 243715 Patient Name: Rusty Rowdy Client Name: Palmer RADIOGRAPHIC FINDINGS Right lateral and ventrodorsal views of the abdomen are available. The liver and spleen are normal. The kidneys are normal in size and smoothly marginated. In the caudal abdomen, there are 2 ovoid soft tissue opacities. One of these, likely the more cranial, is the urinary bladder. The other is of uncertain origin. There is a moderate volume of gas in the stomach. The stomach and intestinal tract are otherwise normal. There is mildly decreased serosal detail in the mid abdomen, likely due to decreased intra-abdominal fat. On the ventral abdomen within the subcutaneous tissues, there is a broad-based fat and soft tissue opacity nodule. CONCLUSIONS Possible causes for the ovoid soft tissue mass adjacent to the urinary bladder include cystic uterus masculinus, paraprostatic cyst, or ureterocele. Abdominal ultrasound is recommended for further assessment. Probable umbilical hernia. This structure may also be examined using ultrasound. ELF

35. Medical Record: 243715 Exam Date: 09/11/13 Animal’s Name: Rusty Owner’s Name: Palmer ----- Sonographic Findings: ----- ----- ABDOMEN ----- Peritoneal cavity: Normal cavity. Abdominal wall: An umbilical hernia is observed with only peritoneal fat observed in the hernial sac which can be reduced. A small defect in the wall measuring approximately 4.3 mm is observable at the umbilicus. Liver: Normal Gall bladder: Normal Spleen: Normal Kidneys: Left: Decreased corticomedullary distinction is observed with slight pyelectasis measuring approximately 1.5 mm. No evidence of renal mineralization or calculi are observed. Right: Decreased corticomedullary distinction is observed with slight pyelectasis measuring approximately 1.5 mm. No evidence of renal mineralization or calculi are observed. Urinary bladder: The bladder is moderately distended with fluid containing suspended echogenic debris that is freely movable. The urinary bladder wall is normal. A thickwalledbicornuate fluid-filled structure is observed dorsal to the neck of the bladder continuing to and connecting with the prostate. The structure measures approximately 3.9 cm in length and 3.4 cm width with the 2 fluid-filled side-by-side structures in the cranial aspect measuring approximately 1.3 cm and 1.4 cm in diameter. Adrenal glands: Left: Normal measuring 3.6 mm in transverse diameter. Right: Normal measuring 4.1 mm in transverse diameter. Lymph nodes: Left medial iliac: Mildly enlarged measuring 0.61 cm in diameter. Right medial iliac: Normal Mesenteric: Normal

36. GI: Stomach: Normal Duodenum: Normal Jejunum: Normal Colon: Normal Pancreas: Normal Genital: Ovaries: Uterus: Prostate: Testicle: The right testicle measures approximately 5.65 mm in diameter. The left testicle is slightly smaller measuring 5.1 mm and diameter. The parenchyma of the testicles is normal. ----- Sonographic Conclusions: ----- Bicornuate fluid-filled thickwalled structure dorsal to the neck of the urinary bladder and connecting to the prostate is most consistent with uterus masculinus which is a rare congenital defect associated with fused müllerian ducts. Bilateral renal changes and echogenic urine compatible with chronic renal disease and possible pyelonephritis/cystitis. These findings should be correlated with serum chemistries and urinalysis. Mild enlargement of the left medial iliac lymph node likely reactive lymphadenopathy associated with either urinary tract infection or associated with the uterus masculinus Reducible umbilical hernia containing only peritoneal fat. Multiple attempts at sonographically guided fine needle aspiration of the fluid of the suspected uterus masculinus were unsuccessful in obtaining fluid. George A. Henry, DVM, Diplomate ACVR