Adolescent Gynecology: Evaluation and Management of Adnexal Mass, PCOS, and Endometriosis. Shanna M. Combs, MD

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Adolescent Gynecology: Evaluation and Management of Adnexal Mass, PCOS, and Endometriosis Shanna M. Combs, MD

Adolescent Visit and Exam

Adolescent Reproductive Health Visit Initial visit should take place between 13 and 15 years old Excellent opportunity to build relationship and trust with young patients Model office visit 1. Initial consult with patient and parent 2. Confidential visit with patient 3. Concluding visit with patient and parent Examination: depends on the patient, her concerns, and previous encounters with other clinicians General exam, visual breast exam, and an external pelvic exam may be indicated Internal pelvic exam is generally unnecessary

Adolescent Reproductive Health Visit If sexually active Annual screening for gonorrhea and chlamydia Can be done from urine sample or vaginal swab (can be collected by patient or provider) Screen for HIV at least once Discussion regarding contraception and prevention of STIs Review other risky behaviors Tobacco, alcohol, and substance abuse Dating violence Abuse Psychiatric: eating disorders, depression, anxiety

Adnexal Mass

Background Ovarian tumors are uncommon lesions in children and adolescents Approximate incidence of 2.6 cases annually/100,000 Malignant ovarian tumors are approximately 1% of childhood cancers Benign and functional cysts are the most common ovarian lesions in pediatric and adolescent population Follicular, corpus luteum, and theca lutein cysts Majority of ovarian tumors are of non-epithelial origin

Presentation Most common presenting symptom is pain in 60-80% of children and adolescents Pain may be intermittent, constant, or acute Acute pain with nausea, vomiting, or fever requires immediate attention and likely surgical intervention Other symptoms: Abdominal distension Menstrual irregularity Precocious puberty Virilization Urinary frequency Constipation Asymptomatic with incidental finding on ultrasound or other imaging

Differential Diagnosis Ovarian: Tubal Torsion Functional cysts PCOS Endometrioma Benign or Malignant neoplasm Paraovarian/paratubal cysts Hydrosalpinx

Differential Diagnosis Infectious PID Tubo-ovarian abscess Obstructive Imperforate hymen Hematocolpos Gastrointestinal Appendicitis

Evaluation History (including sexual history) Physical Exam (+/- pelvic exam or rectal exam) CBC Pregnancy test Ultrasound MRI Tumor Markers (CA-125, AFP, HCG, CEA, LDH, Inhibin, Estradiol, Testosterone)

Functional Cysts Follicular, corpus luteal, and theca lutein cysts are usually benign and usually resolve spontaneously 2-3 months of observation is appropriate Predictive factors for resolution Right-sided cysts Cysts less than 7cm OCPs can suppress future large cyst development but do not cause cyst regression Persistence warrants re-evaluation of diagnoses

Neoplasms Germ cell tumors are the most common type in adolescents Mature and immature cystic teratomas, Dysgerminomas, etc. Other neoplasms include serous and mucinous cystadenomas, sex-cord stromal tumors, and other malignancies that metastasize to the ovary

Mature Cystic Teratomas (Dermoids) Grow slowly at a rate of 1.8mm per year Occur bilaterally in 10-15% of cases Recurrence rate of 3-4% Asymptomatic dermoids less than 5cm can be managed expectantly Laparoscopic cystectomy for larger dermoids or symptomatic patients

Malignant Neoplasm Incidence in the adolescent population (4.2-7%) is low compared to children (10-14%) Most common malignancy in adolescence is the germ cell tumor Management is controversial Consideration for fertility preservation vs full staging

Tubo-Ovarian Abscess (TOA) In sexually active adolescents an ovarian mass may be TOA Signs and Symptoms: Pelvic pain, pelvic mass, adnexal tenderness, leukocytosis, or fever Treatment: Inpatient admission with IV Cefotetan or Cefoxitin plus Doxycycline or Gentamicin and Clindamycin; may also add Flagyl to either regimen Transition to oral Clindamycin or Doxycycline +/- flagyl for a total of 14 days May require IR drainage or surgery if does not improve with medical management

Torsion Torsion is rare with an incidence of 4.9/100,000 in females age 1-20yo Usually associated with adnexal pathology or a long utero-ovarian ligament Diagnosis is commonly delayed or misdiagnosed Involves the right adnexa more frequently Symptoms: Unilateral pain, nausea, vomiting, and/or low-grade fever Treatment: More emphasis on untwisting procedures with cystectomy if cyst is present Necrotic-appearing ovaries have demonstrated follicular activity weeks after conservative management Bivalve procedure of ovary may relieve vascular congestion May consider oophoropexy after untwisting

Conclusions While rare, adnexal masses due occur in adolescent patients Trends in surgical management support ovarian-sparing surgery whenever possible

Endometriosis

Introduction Estimated 25-38% of adolescents with chronic pelvic pain have endometriosis Endometriosis has been identified in girls even prior to onset of menses Some genetic predisposition towards developing endometriosis 6.9% of first-degree female relatives of patients with endometriosis are affected, as compared to 1% of controls

Diagnosis Symptoms Acyclic and cyclic pain 62.5% Acyclic pain 28.1% Cyclic pain 9.4% Gastrointestinal pain 34.3% Urinary symptoms 12.5% Irregular menses 9.4% Vaginal discharge 6.3% Endometriomas are rare prior to the age range of mid-twenties

Diagnosis Evaluation: Complete history, consider a pain or symptom diary Family history Physical exam: goal is to determine etiology and rule out an ovarian tumor or reproductive anomaly Can consider rectal-abdominal exam in non-sexually active females Q-tip can be inserted in vagina to evaluate for obstruction Ultrasound: to evaluate for possible pelvic mass or structural anomaly or other cause of pain Laboratory Studies: pregnancy test, CBC, ESR, urinalysis, urine culture, STD testing if appropriate

Empiric Treatment NSAIDs: begin before the onset of symptoms or menses OCPs: may improve dysmenorrhea and decreasing menstrual flow If pain continues despite OCPs and NSAID use for 2-3 months and pain is adversely affecting quality of life, further assessment is needed

Surgical Diagnosis and Treatment Operative laparoscopy Must be familiar with appearance of endometriosis in an adolescent Red flame lesions and clear lesions are more common Peritoneal Alan-Masters windows are also common If no evidence of endometriosis is identified, consider a cul-de-sac biopsy to rule out microscopic disease Destroy or resect any visible disease Consider waiting at least 2 years before repeating surgery for recurrent pain

Ongoing Treatment Endometriosis is a chronic disease that has shown to be progressive Goal of medical therapy is to treat pain and suppress progression Continuous oral contraceptives Hormonally decreases stimulus to endometriotic tissue Can also use contraceptive patch or vaginal ring Progestin-only therapy NSAIDs Can use oral or intramuscular progestins Helpful adjuvant agent for the treatment of pelvic pain associated with endometriosis

Ongoing Treatment GnRH Agonists Only if the patient is over 16yo (when most bone is formed) and has persistent pain despite other medical therapy Depot leuprolide acetate 11.25mg q 3 months Use the 3 month formulation to avoid flare effect Use add-back therapy to minimize side effects Norethindrone Acetate 5mg/day Conjugated estrogen (0.625mg) plus norethindrone acetate (5mg) daily Calcium and Vitamin D supplementation Consider bone scan if using for longer than 9 months

Conclusions Endometriosis occurs in adolescents If endometriosis is identified in adolescents with chronic pain, may be able to keep disease from progressing Decrease lifelong pain Preserve future fertility

Polycystic Ovarian Syndrome (PCOS)

Introduction Once considered an adult illness, PCOS is now recognized as having origins prenatally and manifests itself through early childhood, puberty, and adolescence How to define and diagnose PCOS in adolescence remains controversial Estimated that 11-26% of adolescents may be affected Associated with obesity, but lean PCOS also exists Lean and Obese PCOS begin to show signs of insulin resistance in childhood

Diagnosis Criteria Hyperandrogenism Anovulation Polycystic Ovaries Lack of Other Diagnoses NIH Criteria Required Required Not required + Rotterdam Criteria (2 of first 3 required) +/- +/- +/- + AE-PCOS Society Criteria (1 st and either 2 nd or 3 rd findings required) Required +/- +/- +

Diagnosis Use of previously described criteria in adolescent patients is difficult as there is no adolescent specific definition of PCOS Oligo-ovulation with subsequent menstrual irregularities is common in the first 18 months after menarche Puberty is a state of physiologic insulin resistance Insulin decreases hepatic SHBG production leading to increased free testosterone in the serum Hyperandorgenism diagnosed by acne may actually reflect increased free androgen production in puberty and not pathology Ovarian volume continues to increase 2-3 years after menarche Ovarian volume measurements are more accurate when done transvaginally, but most ultrasounds are done transabdominally in adolescents

Diagnosis Consider waiting at least 2 years after menarche prior to making diagnosis Consider girls with hyperandrogenism and oligomenorrhea as having probable but unconfirmed PCOS Disadvantage of using this criteria is a delay in evaluation for insulin resistance associated with PCOS Must exclude other possible diagnoses Laboratory evaluations is mainly for excluding other diagnoses Include lab evaluation for metabolic syndrome risk factors Oral glucose tolerance test, fasting insulin level, fasting lipid panel

Management Must address each of the following concerns: Menstrual dysfunction, risk of endometrial cancer, and future fertility Hyperandrogenism, including acne, hirsutism, and alopecia Metabolic syndrome risks Psychological concerns Risks of other comorbidities

Management-Menstrual Dysfunction Dysmenorrhea and oligomenorrhea are common findings in adolescent PCOS Combined OCPs Low to moderate estrogen dose (20-35mcg) and less adrogenic progesterone (Norgestimate, Norethindrone, and Ethynoidiol) May also help with hyperandrogenism symptoms May also use vaginal ring If history of clotting disorders may use progesterone-only therapies Monthly medroxyprogesterone, norethindrone, or prometrium for 5-10 days Levonorgestrel IUS

Management-Hyperandrogenism OCPs with low androgenic potential Will lead to a gradual decrease in new hair growth and acne Hair that is already present must be removed by conventional methods Spironolactone can also be used Should be used in conjunction with contraception in sexually active adolescents due to teratogenicity

Management-Metabolic Syndrome Lifestyle interventions 150 minutes per week of aerobic activity Dietary modifications Weight loss of 5-10% associated with significant improvements Metformin is used to help with insulin resistance, decrease risk of metabolic syndrome, and development of further abnormalities in glucose metabolism Check annual creatinine and B12 levels Check LFTs before initiation then every 4-6 months

Management-Psychological Concerns Physical stigma of acne and hirsutism can be associated with social distress and school phobia or avoidance Obesity and acanthosis nigricans also have similar issues, and some girls are reluctant to exercise for fear of taunting Recognition of psychological concerns is important as they may complicate adolescents adherence to treatments Goals of therapy should be discussed at initial visit and re-discussed at subsequent visits to help maintain compliance

Management-Other Comorbidities Obstructive sleep apnea (OSA)-seen in a significant numbers of adolescents with PCOS Nonalcoholic fatty liver disease (NAFLD)-common in PCOS and a marker of insulin resistance Renal insufficiency-can occur with obesity and is a significant problem for adolescents with PCOS due to the medications used for PCOS (i.e. metformin, spironolactone)

Management Management of PCOS in adolescents must address the multiple healthcare issues, both short-term and long-term Incorporate the adolescent s desires and goals into the management plan Work with a multidisciplinary team will help in achieving better outcomes

References The Initial Reproductive Health Visit, ACOG Committee Opinion 598, May 2014 Zhang, et al. Ovarian masses in children and adolescents-an analysis of 521 clinical cases. Journal Pediatric and Adolescent Gynecology 2014; 27 : e73-e77 Rogers, et al. Preoperative risk stratification of adnexal masses: Can we predict the optimal surgical management? Journal of Pediatric and Adolescent Gynecology 2014; 27: 125-128 Al Jama, et al. Ovarian tumors in children and adolescents-a clinical study of 52 patients in a university hospital. Journal of Pediatric and Adolescent Gynecology 2011; 24: 25-28 Brown, et al. Ovarian masses in children: A review of 91 cases of malignant and benign masses. Journal of Pediatric Surgery 1993; 28: 930-932 Kirkham, et al. Ovarian cysts in adolescents: Medical and surgical management. Adolescent Medicine 2012; 23: 178-191 Kansra, et al. PCOS: Perspectives from a pediatric endocrinologist and a pediatric gynecologist. Current Problems in Pediatric and Adolescent Health Care 2013; 43: 104-113 Auble, et al. Differenced in the management of adolescents with polycystic ovary syndrome across pediatric specialties. Journal of Pediatric and Adolescent Gynecology 2013; 26: 234-238 Hardy, et al. Diagnosis of adolescent polycystic ovary syndrome. Steroids 2013; 78: 751-754 Connor. Adolescent polycystic ovary syndrome. Adolescent Medicine 2012; 23: 164-177 Ozyer, et al. Endometriomas in adolescents and young women. Journal of Pediatric and Adolescent Gynecology 2013; 26: 176-179 Laufer. Helping adult gynecologists diagnose and treat adolescent endometriosis: Reflections on my 20 years of personal experience. Journal of Pediatric and Adolescent Gynecology 2011; 24: 513-517 Laufer, et al. Adolescent endometriosis: diagnosis and treatment approaches. Journal of Pediatric and Adolescent Gynecology 2003; 16: S3-11 Doyle, et al. The effect of combined surgical-medical intervention on the progression of endometriosis in a adolescent and young adult population. Journal of Pediatric and Adolescent Gynecology 2009; 22: 257-263

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