Case Presentation Stephanie Peng July 10, 2010

CC/HPI “I’ve got this little girl in the ED with LLQ pain for 24h” –Intermittent, then progressive –Localized –Sharp –Nausea and vomiting –No BM for 5 days –Flatus this morning History of constipation

PMH/PSH Full term, NSVD CCAM (congenital cystic adenomatoid malformation) of right lower lobe, s/p RLL resection age 6mo No sick contacts Recent trip to Disneyland Prenatal ultrasound revealed: –Solitary kidney (parents unsure which side)

DDx Constipation UTI Gastroenteritis Incarcerated hernia Ovarian torsion Nephrolithiasis

PEX Afebrile, VS normal, skin pink and warm Laying down, miserable but calm and cooperative Abd: soft, flat. Palpable mass in LLQ. No hernias …20 minutes later: Acutely worsened LLQ pain, significant guarding

Management: Detorsion vs resection Historically torsed ovaries were resected –Risk of malignancy as lead point –Risk of VTE –Black-blue ovaries thought non-viable

The Case for Conservation Multiple case series suggest normal ovarian function following simple detorsion –3 case series, 200+ patients over 20 years –Age range 2.5 yr – 35 yrs –Function determined by appearance of follicles on ultrasound –Follow-up range 3mo – 3 yrs

How dead is the ovary? In resected human specimens, > 50% viable tissue on histologic examination Rat studies looking at ischemia time: ovaries torsed or clamped for 4-36 hours –Half resected, and half were detorsed and allowed to reperfuse for 1 week (then sacrificed) –Endpoints: expression of cellular necrosis markers, and histologic findings –All ovaries grossly black-blue at sacrifice –Resection group: > 70% viable tissue at 24h clamp time. After 36h, all tissues found to be necrotic –Detorsion group had similar results Taskin et al. The effects of twisted ischaemic adnexa managed by detorsion on avian viability and hisotology: an ischaemia- reperfusion rodent model. Hum Reprod 1998; 13:

Complications Contralateral torsion 5% –Asynchronous bilateral torsion up to 11% in otherwise normal ovaries Recurrent torsion 2-5%

Oophoropexy or not? Kokoska, et al. Am J Surg –Retrospective review of 51 children with OT at St. Louis University –All underwent salpingoophrectomy –Due to unknown risk to fertility, recommended against oophoropexy, unless a “normal appearing” ovary Abes et al. Eur J Pediatr Surg –7 children, mean age 7.7 years –All but one patient had salpingoophorectomy –All patients had pexy of remaining ovaries –At 11 mo follow-up, all patients had normal menses

??

Renal Agenesis and Mullerian Duct Abnormalities Combination described since 1922 Most common triad – uterine didelphys – unilateral imperforate hemivagina – ipsilateral renal agenesis Typical presentation is peri-menarche –Dysmenorrhea –Abdominal pain

Vaginoscopy Findings Vagina patent, no septum Left cervix absent Right cervix patent Normal external genitalia Suspect unicornuate uterus with obstructed L horn

Epilogue… Abdominal ultrasound confirms absence of L kidney Uterus is visualized but small for age Plan for repeat pelvic U/S in 6-8 weeks in Ped Gyn Clinic Symptoms develop at menarche (if at all)

References 1.Moore, K. The Developing Human: Clinically Oriented Embryology. 7 th Ed. Philadelphia, Cass, D. Ovarian torsion. Seminars in Pediatric Surgery (2005) 14, Kokoska, E. Acute ovarian torsion in children. The American Journal of Surgery (2000), 180, Beaunoyer, M. Asynchronous bilateral ovarian torsion. Journal of Pediatric Surgery (2004) 39, Reichman DE, Laufer MR. Congenital uterine anomalies affecting reproduction. Best Prac Res Clin Obstet Gynecol 2009 (epub). 6.Breech et al. Adnexal torsion in pediatric and adolescent girls Curr Opin Obstet Gynecol 17: