Case Presentation NEW ENGLAND SOCIETY OF INTERVENTIONAL RADIOLOGY (NESIR) Sept 12th, 2016 Department of Interventional Radiology Harshna Vadvala, MD Steven Dawson, MD Peter Mueller, MD

Case 38 y.o. female presented to MGH ED with 10/10 right sided abdominal pain since 3 AM that morning. Complaint of increased non-foul- smelling vaginal discharge. PMH – GYN: amenorrhea to oligomenorrhea. No h/o STI or PID. Sexually inactive for 1 year. Vitals: BP 105/66 mm Hg, HR 88 bpm, T 98.3 F. Physical exam: Abdomen- RLQ palpable mass, + tender and + guarding. No rebound. Labs: WBC 14.

Question: 1 38 yo F, G0, presents to the ED with RLQ pain. Per abdomen exam revealed palpable mass with tenderness and guarding. WBC 14. BP 105/66 mm Hg. T-98.3 F. What is the next step ? US CT abdomen-pelvis with contrast MRI Surgical exploration

Answer: 1 38 yo F, G0, presents to the ED with RLQ pain. Per abdomen exam revealed palpable mass with tenderness and guarding. WBC 14. BP 105/66 mm Hg. T-98.3 F. What is the next step ? US CT abdomen-pelvis with contrast MRI Surgical exploration

CT scan with contrast

CT scan with contrast

Question: 2 CT scan showed 8 cm right tubo-ovarian abscess (TOA) and a mullerian duct anomaly (MDA). Which type of MDA was detected ? Type II B- Unicornuate non communicating Type IV- Bicornuate Type V A- Septate complete Type III- Didelphus

Answer: 2 CT scan showed 8 cm right tubo-ovarian abscess (TOA) and a mullerian duct anomaly (MDA). Which type of MDA was detected ? Type II B- unicornuate non communicating Type IV- Bicornuate Type V A- Septate complete Type III- Didelphus

The American Fertility Society Classification of Mullerian Anomalies

Question: 3 Which of the following is not associated with Mullerian duct anomalies ? Renal agenesis Duplex kidney Congenital megaureter Crossed fuse renal ectopia

Answer: 3 Which of the following is not associated with Mullerian duct anomalies ? Renal agenesis Duplex kidney Congenital megaureter Crossed fuse renal ectopia

Question: 4 How will you manage the right TOA ? US guided aspiration US guided drain placement CT guided aspiration CT guided drain placement

Question: 4 How will you manage the right TOA ? US guided aspiration US guided drain placement CT guided aspiration CT guided drain placement

CT guided Right TOA drain placement 8 Fr Dawson-Mueller in right TOA using tandem trocar technique. Drained 20 cc greenish pus.

Management and Follow-up Interim management Cefoxitin, Doxycycline and Flagyl IV Follow up WBC downtrending: 10 (12)(15)(18) Micro: negative for aerobic, anerobic, fungal and mycobacterial organism Bloor culture: negative over 5 days

Question: 5 S/p IR drain in right TOA with reduced RLQ pain. Persistent endocervical abscess. WBC 15(14). BP 105/66 mm Hg. T- 101 (98.3) F. What is the next best step ? MRI Laproscopy Exam under anesthesia (EUA) & Per vaginal drainage Dilatation & curettage (D & C)

Answer: 5 S/p IR drain in right TOA with reduced RLQ pain. Persistent endocervical abscess. WBC 15(14). BP 105/66 mm Hg. T- 101 (98.3) F. What is the next best step ? MRI Laproscopy Exam under anesthesia (EUA) & Per vaginal drainage Dilatation & curettage (D & C)

Post contrast T2 sag Post contrast Post contrast

Restricted on DWI - abscesses ADC Right tubo-ovarian absces Endocervical abscess

Endometriosis T1 axial T2 axial: Shading sign

Herlyn-Werner-Wunderlich (HWW) syndrome Introduction: Mullerian duct anomalies (MDA) - prevalence of 2% to 3%, incidence of 1/200-600 among fertile women. Herlyn-Werner-Wunderlich (HWW) syndrome, is a rare anomaly characterized by a triad - didelphys uterus (Type III MDA) , obstructed hemivagina and ipsilateral renal agenesis AKA- Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Clinical presentation: Usually after menarche with pelvic pain due to the obstructed hemivagina. There may be a palpable pelvic mass.

Illustration Herlyn-Werner-Wunderlich syndrome (C) Drawing illustrates the triad. A 15-years-old girl presenting the triad of didelphys uterus (class III MDA), an obstructed right hemivagina (class I MDA), and ipsilateral renal agenesis. (A) Axial turbo spin-echo T2 weighted MR image (B) Axial turbo spin-echo T1 fat-saturated weighted MR image showing centrally a wide hematocolpos (asterisk), corresponding to the obstructed right hemivagina. On the left side the uterus normally communicating with non-obstructed hemivagina. Herlyn-werner-wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis. Del Vescovo R et al. BMC 2012.

EUA and per vaginal drainage Only 1 cervix was visualized along with a right side bulge US guided needle drainage - 22 gauge spinal aspiration through the vaginal epithelium at 7 o'clock from the visualized cervix toward the visualized pelvic collection. Total 8cc of purulent fluid drained.

Question: 6 Which of the following is not usually a laproscopic treatment for Herlyn-Werner-Wunderlich (HWW) syndrome ? Drainage of hematocolpos/hematometrocolpos Hemihysterectomy Septectomy Marsupialization of the blind hemivagina

Question: 6 Which of the following is not usually a laproscopic treatment for Herlyn-Werner-Wunderlich (HWW) syndrome ? Drainage of hematocolpos/hematometrocolpos Hemihysterectomy - preserve the obstructed uterus as there is equal chances of pregnancy Septectomy Marsupialization of the blind hemivagina

Role of IR in Mullerian duct anomalies In adjunct to hysteroscopy: otherwise limited role. In the case of a failed laparoscopic-assisted resection of a unicornuate non-communicating horn- US-guided tract dilatation. Asherman syndrome - where dense synechiae obstruct access to the tubal ostium, a wire can be inserted transvaginally into the fallopian tube to safely direct the hysteroscopic dissection. Cervical stenosis- an ultrasound-guided puncture into an occluded endocervical canal followed by balloon catheter angioplasty where standard attempts at cervical dilatation are unsuccessful. Müllerian duct anomalies: from diagnosis to intervention. Chandler TM et al. Br J Radiology. 2009 Dec.

Type II B Bicornuate non communicating horn A 22-year-old with intense menstrual pain. (a) Hysterosalpingogram reveals filling of the left horn (arrow) but not the obstructed right horn of unicornuate uterus. (b & c) Intraoperative ultrasound-guided needle is advanced into the cavity and dilatation of the tract is performed. a. b. c. Müllerian duct anomalies: from diagnosis to intervention. Chandler TM et al. Br J Radiology. 2009 Dec.

References Mullerian duct anomalies: imaging and clinical issues. Troiano RN et al. Radiology. 2004 Oct;233(1):19-34. Epub 2004 Aug 18. Herlyn-werner-wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis. Riccardo Del Vescovo,1 et al. BMC Med Imaging. 2012; 12: 4. 2012 Mar 9. doi: 10.1186/1471-2342-12-4 Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Orazi C1, et al. Pediatr Radiol. 2007 Jul;37(7):657-65. Epub 2007 May 15. Case 94: Uterus didelphys with obstructing hemivaginal septum and ipsilateral renal agenesis. Madureira AJ et al. Pediatr Radiol. 2007 Jul;37(7):657-65. Epub 2007 May 15. Uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis: MR findings in seven cases. Tanaka YO et al. Abdom Imaging. 1998 Jul-Aug;23(4):437-41. Successful pregnancy following surgery in the obstructed uterus in a uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: case report and literature review. Altchek A et al. J Pediatr Adolesc Gynecol. 2009 Oct Herlyn–Werner–Wunderlich syndrome presenting with infertility: Role of MRI in diagnosis. Zohra Ahmad et al. Indian J Radiol Imaging. 2013 Jul-Sep; 23(3): 243–246.

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