Sarah Sindell MD January 14, 2009

 EI 6 yo F with hour history of acute onset left lower quadrant pain with multiple episodes of nonbilious emesis, afebrile at home.  WBC 17K at PCP 12 hours before presentation  Normal birth and development  No significant PMH, no previous episodes of pain  Other other positive on ROS: significant constipation over previous 5 days no BM

ED Workup  Afrebrile, VSS  Sleeping throughout exam  Awakens to report minimal tenderness in LLQ on palpation  WBC 15  1+ Leukocyte esterase and 2+ ketones on UA  AXR- nonobstructive bowel gas pattern with stool in left colon

Ultrasound  Left ovary measuring 4.8 x 2.6 x 2.0 cm for an estimated volume of 13 ml. Multiple sub-centimeter peripheral hypoechoic cysts are present. No internal arterial or venous vascular flow is demonstrated with color Doppler. A small amount of adjacent free fluid is present.  The right ovary unremarkable in appearance measures 2.5 x 1.7 x 1.3 cm for an estimated volume of 2.8 ml. Internal vascular flow is demonstrated with Doppler.

 Twisting of ligamentous supports to ovary  Causes vasculature to undergo excessive rotation producing mechanical impairment to flow Up to 90˚ of rotation occurs normally  Leads to venous congestion, edema of tissue and ultimately compromise of arterial flow, ischemia and necrosis of affected tissue

Causative Pathology  Causative pathology identified in 50-80% of patients  Cause varies based on age group Neonates/infants- cysts Premenarchal girls- cysts and neoplams, elongated utero- ovarian ligament Premenopausal women- cysts and neoplasms, ovarian hyperstimulation syndrome, pregnancy Postmenopausal women- cysts and neoplasms Cass D. Ovarian Torsion. Seminars in Pediatric Surgery : 86-92

Ovarian Cysts  Most common etiology across all age groups  90% benign  Functional cysts develop as a result of perturbed hormonal stimulation. Two age peaks  Fetal period to first year of life  Around the time of menarche- associated with gonadotropin release  20% of females have multicystic or enlarged ovaries at the time of menstruation  Predispose ovary to swing on vascular pedicle more readily due to mechanical imbalance as growth occurs

Other common causes  Congenitally long pelvic ligaments  Abrupt changes in intrabdominal pressure such as with vigorous activity  Adnexal venous congestion associated with severe constipation  Neoplastic processes Teratomas Dysgerminoma Juvenile granulosa cell tumors Serous borderline tumors

 Usually significant delay between onset of symptoms and presentation due to the nonspecific nature of signs/symptoms  Abdominal Pain-100%  Abdominal Tenderness- 90%  Nausea/Vomitting 50-70%  Fever %  Peritoneal Signs %  WBC >12K- 30%  With a careful history most girls/parents will report a history of similar pain previously that resolved spontaneously

 Relies on maintaining high index of suspicion  Clinical exam cannot make diagnosis  Urinalysis, pregnancy test, CBC  Abdominal plain films can all be helpful in suggesting alternative diagnosis but never diagnostic of torsion  Diagnosis usually suggested by abdominopelvic ultrasound findings  Definitive diagnosis rests on intraoperative identification of torsed ovary

 Ultrasound highly operator and situation dependent Must have full bladder to identify ovaries Overlying bowel gas can limit visualization of the ovaries  Most common findings in confirmed cases of torsion Enlarged ovary Echogenic mass Nonvisualization of ovary  Presence of vascular flow does not rule out torsion

 Historically all cases of torsion were treated with oophorectomy +/- salpingectomy for three reasons Concern for malignancy associated with the torsed ovary Risk of thromboembolism from detorsing ovarian veins Uncertainty about the future viability of a torsed ovary  Over the past ten years data to support detorsion and conservation of the ovary, regardless of intraoperative appearance

Concern for malignancy in torsed ovaries.  114 patient series from Texas 60% with underlying pathology identified 4 cases of malignancy (3.5%)  444 other reported cases of pediatric ovarian torsion in 13 papers (in past 15 years) 31% with underlying pathology identified 6 reported cases of malignancy (1.4%)  Combined total of 558 cases 10 identified malignancies Malignancy rate of 1.8% Oltman S et al. Pediatric ovarian malignancy presenting as ovarian torsion: incidence and relevance. Journal of Pediatric Surgery :

Risk of thromboembolism secondary to detorsion  Case study of 981 cases of adnexal (ovary or fallopian tube) torsion 309 underwent detorsion 672 underwent resection  2 cases of pulmonary embolism found Both in group that underwent adnexal resection  Total rate of thromboembolism associated with operative treatment of adnexal torsion 0.2%  No cases of thromboembolism associated with detorsion McGovern PG et al. Adnexal Torsion and Pulmonary Embolism: Case Report and Review of the Literature. Obstetrical and Gynecologic Surgery :

 14 patient case series from Turkey 1 of conservatively managed torsion(including simple detorsion, cyst unroofing or oophoropexy + detorsion) 12 (85%) patients had normal arterial signal in affected ovary at 3 months  40 patient case series from France 2 with 26 patients treated conservatively with 14 month followup 17 (71%)ovaries follicular on ultrasound followup 2 subsequent oophorectomies for neoplasm at a later date  Similar results have been found in adult patients with larger case series 1. Celik A et al. Long-term results of conservative management of adnexal torsion in children. Journal of Pediatric Surgery : Galinier P et al. Ovarian torsion. Management and ovarian prognosis: A Report of 45 Cases. Journal of Pediatric Surgery :

 Goal is to prevent future episodes of torsion  Multiple proposed techniques Plication of utero-ovarian ligament with a single permanent suture Fixation of the ovary to the pelvic sidewall Fixation of the ovary to the uterine serosa  No studies exist that look at recurrence rates after detorsion vs. detorsion + oophoropexy Despite lack of evidence, gynecology literature suggests performing pexy bilaterally 1  The impact of oophoropexy in children on subsequent fertility has not been well studied One existing study of 10 patients suggested normal ovarian functionality after oophorectomy for torsion and contralateral oophoropexy Crouch NS et al. Ovarian torsion: To pex or not to pex? Case report and review of the literature. Journal of Pediatric and Adolescent Gynecology : Abes M, Sarihan HL Oophoropexy in children with ovarian torsion. Eur J Pediatric Surg 2004: 14: 168

 The risk of irreversible ischemia and necrosis of a nonviable ovary is weighed against the risk of reperfusion injury to the ovary There is a risk of peritonitis and systemic infection if an ischemic ovary is retained and this has been reported in one pregnant adult patient.  Biochemical evidence of reperfusion injury to ovaries has been shown in many animal model studies and various agents to prevent reperfusion injury have been proposed The clinical significance of reperfusion injury is more difficult to study in humans and animal models have yielded conflicting results.  The only clinically significant increase in morbidity the literature has demonstrated are higher rates of self-limited fever post- operatively in patients treated with ovarian conservation.

 If ovary is conserved, followup with ultrasound to document regression to normal size and return of arterial flow is suggested 6 weeks post-operatively If ovary fails to return to normal size re-exploration is indicated to perform biopsy or oophorectomy  If arterial flow fails to return, ultrasound can be repeated every 3 months, revascularization and subsequent fertility has occurred as late as 3 years postoperatively.  Ultrasound may also diagnose early polycystic ovarian syndrome- a proposed etiology of unexplained ovarian torsion Adolescent gynecology literature suggests hormonal screening for PCOS as well in cases of unexplained torsion. (free and total testosterone levels) Shah A, Likes C and Price T. Early Polycystic Ovary Syndrome as a Possible Etiology of Unexplained Premenarchal Ovarian Torsion. Journal of Pediatric and Adolescent Gynecology :

Galinier P et al. Ovarian torsion. Management and ovarian prognosis: A Report of 45 Cases. Journal of Pediatric Surgery :

 Diagnostic laparoscopy confirmed torsion of adnexa and enlarged, violaceous, hemorrhagic left ovary. Fallopian tube normal in appearance. 10cc collection of hemorrhagic fluid in left pelvis  Left oopherectomy performed using Ligasure  Final pathology- diffusely hemorrhagic ovary, few residual viable oocytes identified in the blood. Small cystic structures seen macroscopically correspond to small follicular cysts. The largest cyst 0.3 cm in maximal diameter. No atypia or neoplastic process is identified.  Phone call followup 2 weeks post-op= doing fine, back to baseline

 Data suggests that despite the long time to presentation, and necrotic appearance intraoperatively- detorsion +/- oophoropexy would have been a safe choice.  Since ovarian torsion is relatively rare it is difficult to change clinical practice  Most pediatric surgeons still perform oophorectomy while more gynecologists perform detorsion +/- pexy. 1 Surgeons not frequently operating on abnormal appearing ovaries have a lower threshold for deciding that ovary will not be viable.  There is no reduction in fertility rates/hormone levels in women with single ovaries 2 unless our patient needs the contralateral ovary removed for some reason, there will likely be no adverse functional sequelae of her oophorectomy. Bristow RE et. al. The impact of surgeon specialty on ovarian conserving surgery in young females with an adnexal mass. Journal of Adolescent Health : Lass A. The fertility potential of women with a single ovary. European Society of Human Reproduction and Embryology :

 Breech LL and Hillard PJ. Adnexal torsion in pediatric and adolescent girls. Current opinion in Obstetrics and Gynecology :  Anders JF and Powell EC. Urgency of Evaluation and Outcome of Acute Ovarian Torsion in Pediatric Patients. Archives of Pediatric and Adolescent Medicine :  Cass D. Ovarian Torsion. Seminars in Pediatric Surgery :  Celik A et al. Long-term results of conservative management of adnexal torsion in children. Journal of Pediatric Surgery :  Rousseau V et al. Emergency Management and Conservative Surgery of Ovarian Torsion in Children: A Report of 40 Cases. Journal of Pediatric and Adolescent Gynecology :  Galinier P et al. Ovarian torsion. Management and ovarian prognosis: A Report of 45 Cases. Journal of Pediatric Surgery :  Shah A, Likes C and Price T. Early Polycystic Ovary Syndrome as a Possible Etiology of Unexplained Premenarchal Ovarian Torsion. Journal of Pediatric and Adolescent Gynecology :  Oltman S et al. Pediatric ovarian malignancy presenting as ovarian torsion: incidence and relevance. Journal of Pediatric Surgery :  McGovern PG et al. Adnexal Torsion and Pulmonary Embolism: Case Report and Review of the Literature. Obstetrical and Gynecologic Surgery :  Abes M, Sarihan HL Oophoropexy in children with ovarian torsion. European Journal of Pediatric Surgery 2004: 14: 168