Pediatric & Adolescent Gynecology – A How To Approach (Didactic)

PROGRAM CHAIR Joseph S. Sanfi lippo, MD

Heather Appelbaum, MD Robert K. Zurawin, MD

Sponsored by AAGL Advancing Minimally Invasive Gynecology Worldwide

Professional Education Information

Target Audience Educational activities are developed to meet the needs of surgical gynecologists in practice and in training, as well as, other allied healthcare professionals in the field of gynecology.

Accreditation AAGL is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.

The AAGL designates this live activity for a maximum of 3.75 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

DISCLOSURE OF RELEVANT FINANCIAL RELATIONSHIPS As a provider accredited by the Accreditation Council for Continuing Medical Education, AAGL must ensure balance, independence, and objectivity in all CME activities to promote improvements in health care and not proprietary interests of a commercial interest. The provider controls all decisions related to identification of CME needs, determination of educational objectives, selection and presentation of content, selection of all persons and organizations that will be in a position to control the content, selection of educational methods, and evaluation of the activity. Course chairs, planning committee members, presenters, authors, moderators, panel members, and others in a position to control the content of this activity are required to disclose relevant financial relationships with commercial interests related to the subject matter of this educational activity. Learners are able to assess the potential for commercial bias in information when complete disclosure, resolution of conflicts of interest, and acknowledgment of commercial support are provided prior to the activity. Informed learners are the final safeguards in assuring that a CME activity is independent from commercial support. We believe this mechanism contributes to the transparency and accountability of CME.

Table of Contents

Course Description ...... 1

Disclosure ...... 3

Developing a Pediatric and Adolescent Gynecology Minimally Invasive Practice – A How to Approach J.S. Sanfilippo ...... 5

Minimally Invasive Surgery in the Pediatric and Adolescent Patient: Vaginoscopy, Hysteroscopy, Laparoscopy and Robotics R.K. Zurawin ...... 17

Obstructive Müllerian Anomalies and Hematocolpos – What You Can Do H. Appelbaum ...... 38

Disorders of Sexual Development R.K. Zurawin ...... 46

Minimally Invasive Surgical Management of Adnexal Masses and Torsion H. Appelbaum ...... 60

Endometriosis in Adolescents – A Whole Different Ball Game J.S. Sanfilippo ...... 70

Minimally Invasive Surgical Management with Vaginal Agenesis H. Appelbaum ...... 80

Fertility Preservation – How and Why R.K. Zurawin ...... 88

Cultural and Linguistics Competency ...... 95

PG 110 Pediatric & Adolescent Gynecology – A How To Approach Developed in cooperation with the North American Society for Pediatric & Adolescent Gynecology

Joseph S. Sanfilippo, Chair Faculty: Heather Appelbaum, Robert K. Zurawin

Course Description

This course is designed to allow clinicians to establish an “adolescent-friendly environment” in their office setting. Strategies for practice development focused on minimally invasive surgical expertise will be provided. A “how to” Approach is the underlying theme for all lectures in the postgraduate course. Gynecologic surgeons are increasingly being called upon to manage Müllerian anomalies; pre-operative as well as intra-operative expertise will be emphasized. As surgeons we are asked with increasing frequency to assist is fertility preservation when a young patient is faced with a diagnosis of cancer or other chronic debilitating disease. Various surgical approaches that clinicians with advanced minimally invasive expertise should be able to acquire will be presented in a readily applicable manner. Current concepts with regard to management of adnexal masses, torsion, and endometriosis in the young adult will allow surgeons to garner the latest advances of gynecologic surgery in this age group.

Course Objectives

At the conclusion of this course, the participant will be able to: 1) Use the learning process to provide counseling and expertise to facilitate development of an adolescent and young adult gynecologic surgical practice focused on minimally invasive surgical techniques; 2) evaluate and manage Müllerian anomalies with surgical as well as non-surgical approaches will be stressed; and 3) discuss the challenges of managing disorders of sexual development, quantified and streamlined to facilitate counseling and surgical correction.

Course Outline

1:30 Welcome, Introductions and Course Overview J.S. Sanfilippo

1:35 Developing a Pediatric and Adolescent Gynecology Minimally Invasive Practice – A How to Approach J.S. Sanfilippo

2:00 Minimally Invasive Surgery in the Pediatric and Adolescent Patient: Vaginoscopy, Hysteroscopy, Laparoscopy and Robotics R.K. Zurawin

2:25 Obstructive Müllerian Anomalies and Hematocolpos – What You Can Do H. Appelbaum

2:50 Disorders of Sexual Development R.K. Zurawin

3:15 Questions & Answers All Faculty

3:25 Break

1

3:40 Minimally Invasive Surgical Management of Adnexal Masses and Torsion H. Appelbaum

4:05 Endometriosis in Adolescents – A Whole Different Ball Game J.S. Sanfilippo

4:30 Minimally Invasive Surgical Management with Vaginal Agenesis H. Appelbaum

4:55 Fertility Preservation – How and Why R.K. Zurawin

5:20 Questions & Answers All Faculty

5:30 Course Evaluation

2 PLANNER DISCLOSURE The following members of AAGL have been involved in the educational planning of this workshop and have no conflict of interest to disclose (in alphabetical order by last name). Art Arellano, Professional Education Manager, AAGL* Viviane F. Connor Consultant: Conceptus Incorporated Frank D. Loffer, Executive Vice President/Medical Director, AAGL* Linda Michels, Executive Director, AAGL* Jonathan Solnik Other: Lecturer - Olympus, Lecturer - Karl Storz Endoscopy-America

SCIENTIFIC PROGRAM COMMITTEE Arnold P. Advincula Consultant: CooperSurgical, Ethicon Women's Health & Urology, Intuitve Surgical Other: Royalties - CooperSurgical Linda Bradley Grants/Research Support: Elsevier Consultant: Bayer Healthcare Corp., Conceptus Incorporated, Ferring Pharmaceuticals Speaker's Bureau: Bayer Healthcare Corp., Conceptus Incorporated, Ferring Pharm Keith Isaacson Consultant: Karl Storz Endoscopy Rosanne M. Kho Other: Honorarium - Ethicon Endo-Surgery C.Y. Liu* Javier Magrina* Ceana H. Nezhat Consultant: Intuitve Surgical, Lumenis, Karl Storz Endoscopy-America Speaker's Bureau: Conceptus Incorporated, Ethicon Women's Health & Urology William H. Parker Grants/Research Support: Ethicon Women's Health & Urology Consultant: Ethicon Women's Health & Urology Craig J. Sobolewski Consultant: Covidien, CareFusion, TransEnterix Stock Shareholder: TransEnterix Speaker's Bureau: Covidien, Abbott Laboratories Other: Proctor - Intuitve Surgical

FACULTY DISCLOSURE The following have agreed to provide verbal disclosure of their relationships prior to their presentations. They have also agreed to support their presentations and clinical recommendations with the “best available evidence” from medical literature (in alphabetical order by last name). Joseph S. Sanfilippo* Heather Appelbaum* Robert K. Zurawin Consultant: Ethicon Endo-Surgery, Ethicon Women's Health & Urology, Conceptus Incorporated, CONMED Corporation, UpToDate Mark R. Hoffman*

3

Asterisk (*) denotes no financial relationships to disclose.

4 4141stst AAGL GLOBAL CONGRESS DisclosureDisclosure PEDIATRIC & ADOLESCENT GYNECOLOGY A How To Approach “Developing a Pediatric & Adolescent Gyn •• I have no financial relationships to Minimally Invasive PracticePractice--HowHow to Approach” disclose.disclose.

Joseph S. Sanfilippo, MD, MBA University of Pittsburgh MageeMagee--WomensWomens Hospital

ObjectivesObjectives

•• At the conclusion of this lecture participant will:will: •• ReviewReviewReview unique aspects of pediatric adolescent Gyn exam •• Discuss the evaluation & management of common PAG problems •• Establish a pediatric adolescent focused clinical setting

5 6 PEDIATRIC PATIENT •• Patient Involved in History •• FrogFrog--leggedlegged Position •• KneeKnee--chest Position •• “Show and Tell” •• Low power Magnification •• “Good job”job”“Good

ADOLESCENT EXAMEXAM--PARADIGMPARADIGM SHIFT •• TOOL KIT-KIT-ACOGACOG PELVIC EXAM •• First ExamExamFirst •• “Gynecologic Encounter” 1313--1515 Y/A – Collaborative with PrimaryPrimary Care Provider •• “Do You Use Tampons?” – Rapport with OB GYN –– No Pelvic Exam – Followed By Annual Visits •• Pap SmearSmear--2121 years of age – Exception: Sexual Abuse Immunocompromised – Not Sexually Active Stewart F et al JAMA 2001;286:671 ACS, NIH, ACOG 2002

7 SCREENING FOR SEXUALLY TRANSMITTED DISEASES WHAT DO I NEED TO KNOW •• Sexually Active Teens Should be Screened ABOUT EXAMINING A PEDIATRIC •• “Urine screening Should be Considered When Teens are Reluctant to Have a Pelvic Exam” ADOLESCENT PATIENT ? – Urine Ligase Chain Reaction-Reaction-LessLess Expense than Cervical Cultures •• “Vaginal Swab” Screening

Health Care for Adolescents ACOG 2002

8 TEEN HEALTH GAP RISK FACTORSFACTORS--TEENAGETEENAGE Time Magazine PREGNANCY •• Poor Educational Performance •• PovertyPoverty •• Adolescents Have Unique Medical Issues •• SingleSingle--ParentParent Family •• A Growing Specialty is Aimed at •• Family History Teen Pregnancy •• “Preggynancy Viewed as “+” Social Value Addressi ng Th em •• Risk Taking BehaviorBehavior--DrugsDrugs Alcohol Abuse Quote from a Teen ( 19 y/a) •• PROTECTIVE FACTORS – “Because many doctors shy from discussing •• Scholastic Achievement drugs & alcohol with teens, some kids •• Higher Socieoeconomic ClassClass struggle for years before finding help. (Teen •• Intact Family is a recovering addict and grateful for early •• Attendance at Religious Services intervention.) Elfenbein D, et al Pediatr Clin NA 2003;781

Issues & Answers NASPAG 2011

•• Teen Births: 400.000 Annually WHAT CAN WE LEARN FROM – 9 Times Greater Than Other Developed TEENS ??TEENS CountriesCountries – Greater Maternal MorbidityMorbidity--TeensTeens – “A Battle We Can Win” per Centers for Disease Control (CDC)

9 LABIAL AGGLUTINATION •• CommonCommon--AgeAge 22--33 years •• Chronic Vulvovaginitis •• Urine irritation •• Perineal Hygiene •• Estrogen Cream •• Zinc oxideoxideZinc •• Reoccurrence

10 LICHEN SCLEROSIS •• PruritisPruritis--BurningBurning •• 25%25%--AssociatedAssociated Autoimmune Disease – Thyroid Pernicious AnemiaAnemia HLA Class II Antigen •• Vesicles or Bullae •• 1%1%--2%2% Hydddrocortisone Cream •• 0.05% Clobetasol •• LongLong--termterm Recurrence •• 33--5%5% RiskRisk--SquamousSquamous cell Carcinoma – 75%75%--AdjacentAdjacent Lichen Sclerosis

Saravanamuthu J et al Gyn Oncol 2003;89:251

PSORIASISPSORIASIS

•• Incidence 11--3%3% Population •• TreatmentTreatment –– Domeboro’ ssSolution Solution – Low dose Glucocorticoids – Clobetasol 0.05%-0.05%-ShortShort course ––SystemicSystemic steroidssteroids--RefractoryRefractory Cases – Methotrexate

11 MOLLUSCUM CONTAGIOSUM

•• Etiology: MolluscumMolluscum--poxviruspoxvirus •• Inguinal Region & Gluteal Cleft •• Many Spontaneously Resolve 66--1212 mo. •• Observe U nl ess N ot R esol vi ng – Spread with Shaving ––TreinoinTreinoin – Cidofovir (Topical anti-anti-viral)viral) ––ImiquimodImiquimod – CurettageCurettage

HERPES SIMPLEX VIRUS •• In Pediatric Population 6 % Genital – Painful Vesicular Lesions-Lesions-UlcerateUlcerate--InguinalInguinal AdenopathyAdenopathy--SystemicSystemic Signs & Symptoms •• HSVHSV--1 and 2 Usually Gingivitis •• Always Look for Multiple Sites •• DDx: VaricellaVaricella--HerpesHerpes ZosterZoster--ImpetigoImpetigo •• Tx: AcyclovirAcyclovir--CompetitiveCompetitive Inhibitor of Viral DNA polymerasepolymerase--InhibitsInhibits DNA synthesis

Mulchahey K in Ped Adoles Gyn ed. J Sanfilippo Saunders 2001

12 13 PINWORMS •• Enterobius vermicularis •• Fecal bacteria Carry Pinworms-Pinworms-VulvitisVulvitis •• Flashlight Exam --Evening vs . “Scotch Tape Test”Test” •• Rx: Mebedazole 100mg repeat in 2 weeks •• Family Members Require Treatment – Except if PREGNANT

14 BREAST MASSMASS--ADOLESCENTADOLESCENT •• Ultrasound •• Little if any Role for Mammography •• Monitoring 11--33 cycles •• FNAFNA •• Malignancy 0.2% of CarcinomaCarcinoma--BreastBreast < 25 y/a – Incidence 0.1/1,000,000 per year •• BSE Instruction

Simmons P in Pediatric & Adolescent Gynecology ed. Sanfilippo 2001 Saunders

15 WHAT DO YOU NEED TO MAKE THE DIAGNOSIS ARE YOU UP TO DATE ON •• REVISED DIAGNOSTIC CRITERIACRITERIA--PCOSPCOS PCOS?PCOS? – 2003 CRITERIA •• OligoOligo--anovulationanovulation In the Adolescent? •• Clinical or Biochemical Signs of Hyperandrogenism •• Polycystic Ovaries And Exclusion of Other Etiologies (CAH, Cushing’s Syndrome)

Rotterdam ESHRE/ASRM PCOS Consensus Workshop Group Fertil Steril 2004’81:19

PCOSPCOS--ULTRASOUNDULTRASOUND

•• By Definition: – 12 or more Follicles @ 22--9mm9mm in diameter and/or Increased

16 DisclosureDisclosure

Minimally Invasive Surgery in Consultant: Ethicon EndoEndo--Surgery,Surgery, Ethicon Women's Health & Urology, Conceptus Incorporated, CONMED Children and Adolescents Corporation, UpToDate

Robert K. Zurawin, MD Associate Professor Director Minimally Invasive Gynecologic Surgery Baylor College of Medicine Houston, Texas

History of Pedi/Adolescent Adoption of Minimally Invasive Surgery Gynecologic Surgery

General Surgeons Gynecologists Pediatric Surgeons and Urologists Pediatric and Adolescent Gynecologists

Miller, CH Training in Minimally Invasive Surgery – You Say You Want a Revolution The Journal of Minimally Invasive Gynecology - March 2009 (Vol. 16, Issue 2, Pages 113-120

CREOG Objectives

Pediatric and Adolescent Gynecology ––““Understand the medical and surgical treatment of pediatric gynecologic disorders” –– ““DescribeDescribeDescribe appropriate medical and surgical treatments for patients with developmental anomalies”anomalies” ––““Treat adolescent gynecologic disorders medically or surgically”

Miller, CH Training in Minimally Invasive Surgery – You Say You Want a Revolution CREOG Educational Objectives Core Curriculum in Obstetrics and Gynecology, The Journal of Minimally Invasive Gynecology - March 2009 (Vol. 16, Issue 2, Pages 113-120 Ninth Edition, 2009

17 CREOG Objectives

Procedure Understand Understand and Point to Remember Perform Laparoscopic X Hysterectomy TLH or LSH In terms of surgery, especially endoscopic Laparoscopy, diagnostic X surgery:surgery: and/or operative Lysis of adhesions X Children are not “little adults” laparoscopic

CREOG Educational Objectives Core Curriculum in Obstetrics and Gynecology, Ninth Edition, 2009

Fundamentals

•• Optimal surgical outcome depends on the surgeon’s knowledge of ––AnatomyAnatomy •• Intimate, “autonomic” familiarity of pathologic conditions and relevant anatomic structures –– Technology •• TOTAL understanding of the surgical instruments – Electromechanical principles – Troubleshooting ANY malfunction – TechniqueTechnique •• Tissue handling •• Visual and proprioceptive coordination

Peritoneal Enty Extent of the Problem

The sine qua non of laparoscopy ~ 4 million laparoscopies per year in the If you can’t safely enter the peritoneum, U.S.U.S. you can’t do ANYTHING 0.5 - 3 percent of laparoscopic If you can safel y ent er th e perit oneum, you procedures have complications related to can do EVERYTHING peritoneal entry Number of complications = ~ 60,000 per yearyear

Chapron C, Querleu D, Bruhat MA, et al: Surgical complications of diagnostic and operative gynecological laparoscopy; a series of 29966 cases. Hum Reprod13(4):867–872, 1998

18 Challenges to Peritoneal Decision Tree AccessAccess Childhood Obesity Umbilicus or Alternative Site? Previous abdominal surgery Elevate abdominal wall? – Previous pediatric surgery – Hand elevation or towel clips? –– PiPrevious laparoscopy !! Veress needle or Direct Trocar Entry? Adhesions to the umbilical undersurface occur Bladed or Bladeless Trocar? in 21.2% of adult patients who have undergone a prior laparoscopy through an Optical trocar or nonnon--opticaloptical trocar? umbilical incision; 10.8% in children

Sepilian V Ku L, H, Liu C.Y., Phelps J “Prevalence of Infraumbilical Adhesions in Women With Previous Laparoscopy” JSLS (2007)11:41–44 Nwokoma NJ, Hassett S, Tsang TT. “Trocar Site Adhesions After Laparoscopic Surgery in Children”. Surg Laparosc Endosc Percutan Tech 2009;19:511–513

Consensus Guidelines UmbilicusUmbilicus

Middlesbrough Consensus - International Thinnest point on abdominal wall Collaborative Group met in 1999 Overlies vital bowel and vascular – Basic guidelines are still followed today11 structuresstructures Frequen t sit e of umbili ca l hern ias w ith Council of the Society of Obstetricians and hernia sacs and/or bowel contents Gynaecologists of Canada22 Adhesions from prior surgery

1 Gynaecological Endoscopy, Volume 8, Issue 6 (p 403-406) 2 Vilos GA et al, J Obstet Gynaecol Can 2007;29(5):433–447

Safe Veress Needle Entry Comparison of elevation of the abdominal wallwall – Hand elevation – Towel clips placed 2 cm on either side of umbilicusumbilicus – Towel clips placed at the edge of the umbilicusumbilicus Intraumbilical 2 cm lateral Hand elevation

Mean distance (cm) between parietal 6.85.143.5 peritoneum and underlying P<0.01 3+cm structures

Roy GM et al,.Safe Technique for Laparoscopic Entry into the Abdominal Caviry. J Am Assoc Gynecol Laparosc 8(4):519–528, 2001 Roy et al / JAAGL 2003

19 2cm 2cm No 1+cm change!

Roy et al / JAAGL 2003 Roy et al / JAAGL 2003

Alternative Sites of Insufflation Anterior Abdominal Wall

Transuterine

CulCul--dede--sacsac

Left upper quadrant

Sanders RR, FilshieGM. Transfundal induction of pneumoperitoneum prior to laparoscopy. J Obstet Gynaecol Br Cmwlth 1994;107:316-7 Morgan HR. Laparoscopy: induction of pneumoperitoneum via transfundal puncture. Obstet Gynecol 1979;54:260–1 Wolfe WM, Pasic R. Transuterine insertion of Veress needle in laparoscopy. Obstet Gynecol 1990;75:456–7 Neely MR, McWilliams R, Makhlouf HA. Laparoscopy: routine pneumoperitoneum via the posterior fornix. Obstet Gynecol 1975;45:459–60 vanLith DA, van Schie KJ, Beekhuizen W, duPlessis M. Cul-de- sac insufflation: an easy alternative route for safely inducing pneumoperitoneum. IntJGynaecolObstet1980;17:375–8.

Primary Port Placement Primary Port Placement

Palmer’s Point

4 FB

Location of deep and superficial vessels of the anterior abdominal wall. Palmer R. Safety in laparoscopy. J Reprod Med 1974;13:1–5. Blue circles indicate recommended locations for trocar placement

20 Basic Trocars Basic Trocars

Optical Entry Open entry Optical entry Open entry

You’ve picked the site – now Optical Trocar Entry what?what? Critical principles: – Force of entry into abdominal wall 44--66 kg in reusable trocars 22--3 kg in shielded bladed retractable trocars and bladeless trocars11 – Insufflate to desired Pressure NOT Volume Increase to 20 – 25 mm Hg until all ports are placed, then reduce to 15 mm Hg2,32,3

1Corson SL, Batzer FR, Gocial B,etal. Measurements of the force necessary for laparoscopic entry. JReprodMed 1994;34:282-4 2Richardson RF, Sutton CJG. Complications of first entry: a prospective laparoscopic audit. Gynaecol Endosc 1999;8:327–34. 3Phillips G, Garry R, Kumar C, Reich H. How much gas is required for initial insufflation at laparoscopy? Gynaecol Endosc 1999;8:369–74.

Secondary Port Placement Combined View

ALWAYS place secondary ports under direct visual guidance Use least amount of force, smallest diameter, and least traumatic puncture Avoid critical structures in anterior abdominal wall

21 Bermuda Triangle

Inferior Epigastic Vessels Medial Umbilical Ligament

Round Ligament

Slide courtesy of Andew I. Brill, MD

Operative Procedures Gynecological Operations

Congenital Abnormalities Open laparotomy Foreign body Minimally invasive procedures TraumaTrauma –– Laparoscopy Ovarian cysts – Hysteroscopy Pelvic Pain/Endometriosis – Vaginoscopy Malignancies Pelvic Inflammatory Disease Ectopic pregnancies

Gynecological Operations Congenital Abnormalities

Congenital Abnormalities Foreign body TraumaTrauma SeptaeSeptae Duplications and Ovarian cysts defects of fusion Pelvic Pain/Endometriosis Dysgenetic ovaries Malignancies Pelvic Inflammatory Disease Ectopic pregnancies

22 Septate Uterus

Historical repair – Strassman procedure – Tompkins metroplasty HHtysteroscop ic management – Blind division with scissors – LasersLasers – Monopolar cautery in hypotonic solution – Bipolar cautery in normal saline

Duplications Uterus Didelphys

DidelphysDidelphys Defects of fusion – laterallateral –– vertical ltil Rudimentary horns – communicating – noncommunicating

23 Didelphys with Obstructed OHVIRAOHVIRA Hemivagina

OHVIRAOHVIRA OHVIRAOHVIRA

Didelphys and Cervical Agenesis

24 CloacaCloaca Principles of Resection

Preoperative radiologic evaluation Laparoscopy/hysteroscopy/vaginoscopy Adequate dissection to isolate blood suppllly Midline plane Proper instrumentation to insure minimal collateral tissue injury Port placement and number

MeyerMeyer--RokitanskyRokitansky (MRKH)

Vaginal agenesis Variable development of internal genitalia Problems if viable endometrium – obtbstruc tibttition MRI insufficient for diagnosis – need laparoscopy

25 Laparoscopic Davydov

Dysgenetic Ovaries

Turner’s syndrome/mosaic Any Y-Y-chromosomechromosome

Principles of Excision

Streak ovaries can be very attenuated EndoEndo--looploop usually not practical – streak ovaries are not pedunculated PiittliidlldProximity to pelvic sidewall and ure ter requires careful avoidance of collateral injury during dissection Attention to hemostasis

26 Gynecological Operations

Congenital Abnormalities Foreign body TraumaTrauma Ovarian cysts Pelvic Pain/Endometriosis Malignancies Pelvic Inflammatory Disease Ectopic pregnancies

27 Gynecological Operations Ovarian Cysts

Congenital Abnormalities Foreign body FunctionalFunctional TraumaTrauma Hemorrhagic Corpus Luteum Ovarian cysts NonNon--functionalfunctional Pelvic Pain/Endometriosis – Benign Malignancies – MalignantMalignant Pelvic Inflammatory Disease Ectopic pregnancies

Functional Cyst

Torsion

28 ParaPara--ovarianovarian cysts

Cavitation Dissection ParaPara--ovarianovarian cysts

29 DermoidsDermoids

Tendency to leak, especially if thin, attenuated cyst wall Copious irrigation Wat ch f or bil a tera lity Negligible risk of complications if spill occursoccurs11

Mecke H, Savvas V. Laparoscopic surgery of dermoid cysts--intraoperative spillage and complications.Eur J Obstet Gynecol Reprod Biol 2001 May;96(1):80-4

The Problem

The vast majority of adnexal masses are benign benign The vast majority of adnexal masses treated by gynecologists result in preservation of the ovary The vast majority of ovarian masses treated by pediatric surgeons end up with salpingosalpingo--oophorectomyoophorectomy

40 Gynecological Operations 35 30 Congenital Abnormalities 25 Foreign body 20 Cystectomy TraumaTrauma OhOophorect omy 15 Ovarian cysts 10 Pelvic Pain/Endometriosis 5 Malignancies 0 Benign Torsion Malignant Prenatal Pelvic Inflammatory Disease Tumors Tumors Cysts Ectopic pregnancies Cass DL, Hawkins E, Brandt ML et al: Surgery for Ovarian Masses in Infants, Children and Adolescents: 102 Consecutive Patients Treated in a 15-year Period. J Pediatr Surg 36:693-699, 2001

30 Principles of Adhesiolysis

Careful attention to anatomy, especially ureters and great vessels Mini mum therma l energy Consider adhesion prevention barrier ENDOMETRIOSIS

Laparoscopic Appearance

Implants seen in adolescents are not typical of what is seen in adults Adolescents have clear vesicles, white implants, small hemorrhagic or petechial spots of the pelvic peritoneum Endometriosis found microscopically on biopsy of normal appearing peritoneum in 6% of patients (Nisolle FertilSteril 1990;53:984)

31 Cul de sac

Bullous lesion

Visible Endometriosis

Peritoneal Surface

Uterosacral ligament

Peritoneal window

32 33 Gynecological Operations

Congenital Abnormalities Foreign body TraumaTrauma Ovarian cysts Pelvic Pain/Endometriosis Malignancies Pelvic Inflammatory Disease Ectopic pregnancies

34 Gynecological Operations

Congenital Abnormalities Foreign body TraumaTrauma Ovarian cysts Pelvic Pain/Endometriosis Malignancies Pelvic Inflammatory Disease Ectopic pregnancies

Sexually Transmitted Disease

Persistent vaginal discharge Absence of foreign body Inconsistent history of sexual abuse Often negative cultures in ER or referring physician’s office Look for trauma to hymen, fourchette, but may be absent

Gynecological Operations

Congenital Abnormalities Foreign body TraumaTrauma Ovarian cysts Pelvic Pain/Endometriosis Malignancies Pelvic Inflammatory Disease Ectopic pregnancies

35 Laparoscopic Equipment

Never need more than 5 mm scope RememberRemember 3 mm and 5 mm ports Special insufflation requirements in children less than 6 years o ld Children are not “little adults” Consider equipment for heating and They require special techniques humidifying insufflated environment and instrumentation Adhesion prevention after ALL nonnon-- infected procedures

Emergency Situations

Ectopic pregnancy Pelvic inflammatory disease ObjectiveObjective Uncontolled menorrhagia Undiagnosed vaginal bleeding – Sexual abuse Maintain Reproduction – Foreign body FunctionFunction

ExceptionException Emergency Endometrial Ablation

Associated anomalies or medical Failure of hormonal therapy conditions that prohibit fertility Unstable condition preventing emergency – Congenital heart disease hysterectomy –– Profound retardation FilFailure o fbllf balloon compression ––EndEnd--stagestage renal disease – Acute life-life-threateningthreatening medical conditions

36 The Use of Thermal Balloon Ablation SummarySummary

3 pediatric/adolescent patients Minimally invasive surgical techniques are within Medical conditions: the grasp of all pediatric and adolescent gynecologists – SepsisSepsis It is not enouggph to have the proper –– UtlldbldiiJhh’WitUncontrolled bleeding in a Jehovah’s Witness instrumentation available. You must be – Diffuse pulmonary arterial stenosis comfortable with the use of all equipment Competent surgical team Adequate visualization KNOW YOUR ANATOMY AND EMBYROLOGY

Zurawin RK and Pramanik S, Endometrial balloon ablation as a therapy for intractable uterine bleeding in an adolescent. J Pediatr Adolesc Gynecol. 2001 Aug;14(3):119-21

37 I have no financial relationships to disclose.

Heather Appelbaum, MD Chief, Division of Pediatric and Adolescent Gynecology Director , Disorders of Sex Development Program The Steven and Alexandra Cohen Children’s Medical Center Associate Professor, Obstetrics and Gynecology Hofstra Northshore LIJ School of Medicine

 Gonads 1. Define normal and abnormal embryologic  Paramesonephros development of the reproductive structures  Mesonephros 2. Identify the level of obstruction for different  Metanephros Müllerian anomalies  Urogenital sinus  Sinovaginal bulb 3. Apply appropriate diagnostic and therapeutic strategies for treating hematocolpos

 Initially, male and  6 weeks ––11stst identifiable when they elongate female embryos have caudally and cross the metanephric ducts both mesonephric and medially to meet in the midline. paramesonephric ducts.  7 weeks – The urorectal septum develops and  The paired mesonephric separates the rectum from the urogenital sinus. ducts connect the  12 weeks – The caudal portion of the ducts fuse metanephros kidneys to the cloaca.cloaca.the to form the uterovaginal canal which inserts into the urogenital sinus.

38  The 2 müllerian ducts are initially composedcomposed of solid tissue and lie side by side. 1. Menstrual egress  Internal canalization of each duct produces 2 channels divided by a septum that is resorbedin a cephalad direction by 20 weeks. 2. Sexual intercourse  The cranial, unfused portions develop into the fimbria and fallopian tubes.tubes. 3. Fertility  The caudal, fused portions form the uterus and upper vagina.vagina 4. Pregnancy 5. Delivery

 Septated ,cribiform, or microperforate hymen  Persistant urogenital sinus  Longitudinal vaginal septum/duplicated vagina  Uterine didelphys  Bicornuate uterus  Uterine septum  Unicornuate uterus  Müllerian agenesis

 Rudimentary horns  Pelvic pain

 OHVIRA  Hematocolpos/Hematometria/Hematosalpinx

 Segmental vaginal agenesis  Pyometria/pyosalpinx

 Transverse vaginal septum  Endometrioisis

 Cervical atresia or cervical agenesis

39 • Pelvic pain

• Dysmenorrhea

• Abnormal bleeding

• Pelvic mass Think Obstructed Müllerian Anomaly

• Pregnancy complications

 Hydrocolpos/mucocele at birth  Hematocolpos at puberty  Untreated imperforate hymen can cause retrograde menstrual flow resulting in endometriosis  Hymenectomy should be delayed until puberty

 Characterizes the number and nature of the Müllerian structures

 Identifies cervical and vaginal anatomy

 Degree of uterine fusion in duplicated systems is delineated

 Associated urinary malformation are identified

40  The prevalence of a rudimentary uterine horn is 1/100,000

Cavitary or non-cavitary?  48% of rudimentary horns do not have a cavity

Functional or non-functional?  Most cavitary rudimentary horns do not have functional endometrium (Fedele, 1990)

 7-10% functional  Nonfunctional  MRI or pelvic uterine horns do not ultrasound identifies require surgical  Retrograde menstruation endometrial stipe or intervention  Hematosalpinx hhttiematometria  Pelvic abcess  Risk of ectopic  Endometriosis pregnancy  Hematometria  Laparoscopic removal of obstructed non-  Ectopic pregnancy communicating functional horn

 Ectopic pregnancy in a non-communicating  Transperitoneal migration of a sperm or tube of a rudimentary horn (Pokoly, 1989) fertilized ovum can result in ectopic pregnancy

 Uterine rupture between 10-20 weeks of  Ruptured tubal pregnancy on the same side as gestation (Tufail, 2007) the rudimentary horn (Handa, 1999)

 Functional cavitated rudimentary horns have a  Tubal or rudimentary horn pregnancies were higher ectopic and lower live birth rate than found only in women with unicornuate uterus those with a rudimentary horn and no cavity and rudimentary horn with a cavity (Heinonen, (Heinonen, 1997) 1997)

41  Uterine didelphys  Laparoscopic ressection of rudimentary horns  Obstructed resulted in successful pregnancies in infertile hemivagina patients with unicornuate uteri with non- communicating rudimentary horns (Giatras,  Ipsilateral renal 1997) agenesis

 Removal of the rudimentary horn may enhance reproductive performance of the unicornuate uterus (Fedele, 1987)

 Uterine didelphys  Obstructing vaginal septum  Unilateral cyclic or constant pelvic pain with normal menses  MRI or pelvic ultrasound identifies hematometrocolpos and normal uterus

Anterior vagina Posterior vagina distended with collapsed blood

 Uterine didelphys Do Don’t  Obstructed hemiuterus  Suppress menses  Perforate the dilated resulting in  Provide analgesia structure hematometrocolpos  Decompress the  Attempt drainage  Distal hemivaginal agenesis/non- bladder for urinary  Operate before the communicating retention anatomy is clearly longitudianal vaginal  Refer to specialist for defined septum/unilateral surgical intervention imperforate vagina

42  Semilunar convex  Greater than 1 cm distance between the upper incision made in most distal aspect left upper and lower vaginal tract anteriolateral wall of the hemivagina  Transvaginal surgical repair  Septum excised with  Segmental graft needle point cautery  Vaginal stenting  Sequential reapproximation of  Vaginal pull-through vaginal mucosa with 3.0  Serial dilation combined with other techniques vicryl to the level of 0.5cm from the cervices  Diagnostic laparoscopy not necessary

 Vertical fusion/obstructive Surgical technique Surgical Technique defect  Preoperative imaging is  Hematocolpos acts as tissue essential to rule out complex  Urogenital sinus fails to expander defects develop the lower vagina Lower vagina  Thin septae should be ressected  Preop mechanical dilation 14% followed by a primary end-to-  Mullerian structures remain can decrease the thickness of end anastomosis of the upper and lower vagina normal the segment  Thick septa should be ressected  Middle Vagina Presents with primary  Crescentric incision at the with a pull through and amenorrhea and cyclic or 40% circumfrential Z-plasty vaginal dimple Upper vagina reanastomosis technique or with constant pelvic pain 46%  Probes, dilators, transrectal a graft  Ultrasound shows ultrasound guide dissection  Distension of upper vagina with hematometria or menstrual blood or preoperative  Once vaginal mucosa dilation of the lower vagina may hematocolpos identified, vaginal pull decrease the thickness of the septum  MRI can delineate the through to the introitus or thickness of the segment and interpose graft confirm the presence of a cervix

 Transvaginal ressection with reapproximation of upper and lower vaginal mucosa  Preoperative dilation to thin the septum  Z plasty may minimize post operative stenosis

43  Utero-vaginal Canalization  Overall-low fertility  Transvaginal or Transabdominal Approach  High incidence of endometriosis-adhesive disease  Create Ostium From Uterus to Vagina  Pregnancy case reports  Stent with/without Grafting  IVF-Transmyometrial Embryo Transfer  Endocervical Gland Presence-Better Prognosis  GIFT  Gestational Carrier  Restenosis Rate High: 40-60%  Spont. Preg following Uterovaginal Anastomosis-  Risk Infection-Peritonitis-Sepsis-Death Graft Reconstruction  Fertility Success Remains Low

Fujimoto V et al AJOG 1997;177(6):1419

 Overall Incidence Uterine Anomalies 0.5%  8 patients with cervical atresia DeliveriesDeliveries  Cervical-vaginal fistula created  88--CervicalCervical Atresia-Atresia-Pelvic PainPelvic Pain  No Pregnancies  CervicalCervical-- VVilaginal FiFitlstula Creat tded  Hysterectomy is the treatment of choice  No Pregnancies  Hysterectomy Treatment of Choice Rock J et al Int J Ob 1984;22:231

Rock J et al Int J Ob 1984;22:231

 UteroUtero--vaginalvaginal Canalization  OverallOverall--LowLow Fertility  Transvaginal or Transabdominal Approach  EndometriosisEndometriosis--AdhesiveAdhesive Disease  Create Ostium From Uterus to Vagina  Case Reports Pregnancy  Stent with/without Grafting  IVFIVF--TransmTransmyyyometrial Embryo Transfer  Endocervical Gland PresencePresence--BetterBetter Prognosis  GIFTGIFT  Restenosis Rate High: 4040--60%60%  Gestational Carrier  Risk Infection-Infection-PeritonitisPeritonitis--SepsisSepsis--DeathDeath  Spont. Preg following Uterovaginal AnastomosisAnastomosis--  Fertility Success Remains Low Graft Reconstruction Fujimoto V et al AJOG 1997;177(6):1419

44  Retrograde menstrual flow with obstructive  Hemi-hysterectomy is recommended for a anomalies cavitated functional rudimentary horn  Early restoration of outflow tract can limit  No conclusive evidence exists to warrant endometrioisis excision of a non-cavitated rudimentary horn  Ceolomic metaplasia may play an additional role  The level of the obstruction should dictate the operative approach

 Obstructive congenital anomalies of the vagina should be approached transvaginally by septal ressection or vaginoplasty  Appropriate imaging is essential to assess the level of obstruction

 Hysterectomy is the treatment of choice for cervical agenesis  The level of the obstruction should dictate the operative approach

 Hemi-hysterectomy is recommended for a cavitated functional rudimentary horn  Laparoscopic hysterectomy is recommended for a cavitated functional rudimentary horn, and may be indicated for cervical agenesis  No conclusive evidence exists to warrant excision of a non-cavitated rudimentary horn  No conclusive evidence exists to warrant excision of a non-cavitated rudimentary horn

45 Consultant: Ethicon Endo‐Surgery, Ethicon Women's Health & Urology, Conceptus Incorporated, CONMED Corporation, UpToDate

Robert K. Zurawin, MD Associate Professor Director, Minimally Invasive Gynecologic Surgery Department of Obstetrics and Gynecology Baylor College of Medicine Houston, Texas

Classical Latin – vulva was “the womb” ‐ Latin – “the sheath of a sword” or “scabbard” Celsus, De medicina, IV, 1.2 ‐ Julius Caesar, Gallic Wars V, 44.8 Sanskrit – ulva Roman farming – “leaf sheath of an ear of wheat”

46 Clitoris –hypertrophy Vagina Vulva Imperforate hymen/microperforate hymen Congenital labial fusion Transverse vaginal septum Acquired labial agglutination Duplication Hypertrophy of labia minora and majora AiAgenesis Prolapse of urethral mucosa Wolffian duct remnants Hemangioma Garnter’s duct cyst Herlyn‐Werner syndrome

True hermaphroditism Ambiguous Genitalia Female pseudohermaphroditism Female pseudohermaphrodites Male pseudohermaphroditism Androgen abnormality/insensitivity Androgeng insensitivityy (testicular feminization) Labial Hypertrophy Mixed gonadal dysgenesis Female Genital Mutilation Chromosomal abnormalities with vulvovaginal Type I Clitoridectomy anomalies Type II Clitoridectomy and labial excision Type III Modified infibulation Type IV Total infibulation

47 48 Two of them are “labia majora” One of them is: Labium majus

Two of them are “labia minora” One of them is: Labium minus

49 Type I — Partial or total removal of the clitoris and/or the prepuce (clitoridectomy). When it is important to distinguish between the major variations of Type I mutilation, the following subdivisions are proposed: Type Ia, removal of the clitoral hood or prepuce only; Type Ib, removal of the clitoris with the prepuce. Type II — Partial or total removal of the clitoris and the labia minora, with or without excision of the labia majora (excision). When it is important to distinguish between the major variations that have been documented, the following subdivisions are proposed: Type IIa, removal of the labia minora only; Type IIb, partial or total removal of th e cl itoris and th e llbabia minora; Type IIc, partial or tota l remova l o f t he cl itoris, th e labia minora and the labia majora. Note also that, in French, the term ‘excision’ is often used as a general term covering all types of female genital mutilation. Type III —Narrowing of the vaginal orifice with creation of a covering seal by cutting and appositioning the labia minora and/or the labia majora, with or without excision of the clitoris (infibulation). Type IIIa, removal and apposition of the labia minora; Type IIIb, removal and apposition of the labia majora. Type IV —All other harmful procedures to the female genitalia for non‐medical purposes, for example: pricking, piercing, incising, scraping and cauterization.

50 51 Masses Ectopic ureter Prolapsed ureterocele Bladder exstrophy VVilaginal cystt Obstructed hemivagina Cloacal and urogenital sinus abnormalities

52 Urinary tract Unilateral renal agenesis with obstructed hemivagina Ectopic or duplicated ureter Exstrophy of the bladder Intestinal tract Ectopic or imperforate anus Rectovaginal fistula

Vagina Imperforate hymen/microperforate hymen Transverse vaginal septum Duplication AiAgenesis Wolffian duct remnants Garnter’s duct cyst Herlyn‐Werner syndrome

53 Greek – hymēn – “the thin skin or membrane covering the brain and heart” ‐Aristotle hymenoptera ~ “membrane wing” wasps Hymen was the god of marriages The Greek wedding song was a “hymenaios”

54 Disorders of : Agenesis Hypoplasia Vertical fusion (canalization abnormalities resulting from abnormal contact with the urogenital sinus Lateral fusion (duplications) Resorption (septa)

55 56 Hydatid of Morgagni cyst Cysts of the broad ligament Gartner’s canal (duct)

Frank dilator therapy McIndoe “Traditional” skin graft procedure Vecchietti Traction using flexible dilator placed at introitus Davydov Combined vaginal and laparoscopic approach

57 Septae Duplications and defects of fusion Dysgenetic ovaries

Historical repair Strassman procedure Tompkins metroplasty Hysteroscopic management Blind division with scissors Lasers Monopolar cautery in hypotonic solution Bipolar cautery in normal saline

Didelphys Defects of fusion lateral vertical Rudimentary horns communicating noncommunicating

58 Preoperative radiologic evaluation Laparoscopy/hysteroscopy/vaginoscopy Adequate dissection to isolate blood supply Midline plane Proper instrumentation to insure minimal collateral tissue injury Port placement and number

59 Minimally Invasive Surgery DisclosureDisclosure

for AdnexalMasses and I have no financial relationships to Adnexal Torsion in Children: disclose.disclose. a conservative approach

Heather Appelbaum, MD, FACOG Associate Professor, Hofstra University Medical School Chief, Division of Pediatric and Adolescent Gynecology Department of Obstetrics and Gynecology Long Island Jewish Medical Center Steven and Alexandra Cohen Children’s Medical CenterCenter

ObjectivesObjectives Adnexal masses Review the preoperative assessment of adnexal masses Physiologic Benign Malignant Other in children and adolescents Neoplasm Neoplasm Assess when ovarian preservation is indicated in Follicular cyst Mature teratoma Immature Ectopic children with adnexal masses teratoma pregnancy Corpus Luteal endometrioma Dysgerminoma Obstructive Review minimally invasive surgical techniques for cyst Mullerian approaching large adnexal masses in children anomaly DiagnoseDiagnose,, treat and prevent adnexal torsion in children Ruptured Serous Granulosa cell Paraovarian and adolescents. ovarian cyst cystadenoma tumor cyst Mucinous Lymphoma Torsion cystadenoma Tubo-ovarian abcess Hematosalpinx

Incidence of ovarian masses by Prenatal/neonatal ovarian cell typecell type cystscysts Non neoplastic 46.2%46.2% ovarian torsion 15.115.1%% corpus luteal cyst 14.2% 1:2,500 live female births paraovarian cyst 10.4% hematohemato//hydrosalpinxhydrosalpinx 1.9%1.9% Follicular cysts develop in response to ovotestisovotestis 0.90.9%% maternal hormones BenignBenignBenign neoplasticneoplastic 44. 34433%% mature teratoma ((dermoiddermoid)) 39.6%39.6% Differenti al diagnos is inc lu des congen ita l cystadenoma 2.6% urogenitalurogenitalanomalies,anomalies, messenteric or Malignant neoplastic 9.4%9.4% omental cysts, volvulusvolvulus,, intestinal dysgerminoma 3.83.8%% immature teratoma 1.9% duplication or urachal cysts yolk sac tumor 0.9% 0.9% granulosa cell tumor 1.9% 90% spontaneous resolution by three sertolisertoli--LeydigLeydig cell tumor 0.90.9%% monthsmonths Cass et al., Surgery for Ovarian masses in infants, children, and adolescents: 102 consecutive patients treated in a 15 year period. Journal of Pediatric Surgery 36(5)2001: 693-699

60 Complications associated Management of with prenatal/neonatal cysts prenatal/neonatal ovarian cysts Hemorrhage Serial ultrasounds at birth and q4q4--6weeks6weeks RuptureRupture Spontaneous regression occurs in most Torsion and necrosis casescases Incarceration in inguinal hernia SilitSurgical interven tiftion for pers itttistent cysts, Respiratory distress symptomatic cysts, cysts increasing in size, or cysts >5cm with ovarian Labor dystocia preservation

Reference needed

Ovarian cysts in Management of ovarian cysts infants/infants/prepubertalprepubertalgirls in prepubertal girls Rare phenomenon Serial ultrasounds Hormonally active cysts can cause precocious puberty Hormonal evaluation 2626%%% assympttittitomaticcaabdom ina llbdi masses are malignant <1% cysts with abdominal pain are 90% resolve spontaneously malignantmalignant Surgical intervention for persistent, large, or symptomatic cysts

Conservative management for Management of benign ovarian cysts in postpubertalpostpubertal/adolescent/adolescent ovarian cystscysts adolescents Follicular cysts are <3cm simple cysts and will Serial ultrasounds invariably self resolve

Functional cysts <6--10cm should be managed AnalgesicsAnalgesics conservatively unless symptomatic

Serial Hct Laparoscopic cystectomycystectomy for persistent, large, or symptomatic cysts Hormonal therapy Hormonal therapy will suppress further cyst formationformation

61 Complications from ovarian Case #1#1Case cystscysts Torsion Torsion L.H. is a 10y2m old with intermittent right Rupture Rupture lower quadrant pain of variable intensity Hemorrhage over the last 15 hours Urinary tract obstruction Prior to coming to the ED, her pain was severe and she was nauseated AortoAorto--cavalcaval shunting On arrival to the ED her pain had Incarceration of inguinal hernia improved and she felt much better Respiratory distress

Case #1#1Case Transabdominal pelvic ultrasound

She was afebrile, with normal vital signs Right ovary 6.4 X 4.8 X 4.0 cm with a 4.2cm Tanner staging B1PH1 simple cystsimple cyst Left ovary 1.4 X 1.7 X 1.3cm Abdomen was soft with tenderness in the Prepubertal uterus rihtlight lower qua dran t. There was no rebound or guarding. There were no palpable masses Pelvic examination was not performed

What is her diagnosis? Ovarian Torsion

Twisting of the ovary on its ligamentous supports results in impedance of blood supplysupply

62 Factors predisposing to adnexal Ovarian and fallopian tubal torsion in children torsiontorsion

Ovarian masses Elongated uteroutero--ovarianovarian ligament in prepubertal girlsgirls

Persistent neonatal ovarian cyst May be associated with strenuous exercise or sudden increase in abdominal pressure Polycystic ovaries Neonates present with abdominal mass, feeding intolerance, vomiting, abdominal distension and MMüüllerianllerian anomalies irritabilityirritability

Incidence and Trends of Pediatric Ovarian Symptoms associated with Torsion Hospitalizations in the US, 20002000--20062006 Guthrie, B., Adler, M, Powell, E. PediatricsPediatrics. 2010:125;5322010:125;532--538538 ovarian torsion Incidence of ovarian torsion in age group 11--2020 yo is 4.9/100,000 Stabbing pain (70%) Nausea and vomiting (70%) 58% of cases of ovarian torsion in children are associated with benign masses Sudden and sharp pain in the lower abdomen (59%)(59%) Less than 0.5% of ovarian torsion cases were associated Pain radiating to back, flank, or groin (51%) with malignant neoplasm Peritoneal signs (3%) Fever (<2%) There were no cases of venous thromboembolism Up to Date, January 2009

The role of ultrasound in Loss of normal ovarian ovarian torsion paranchyma Peripheral follicles with stromal edemaedema

Heterogenously enlarged ovaries

Free fluid in the cul--dede--sacsac

A ratio of torsed adnexal volume to the normal adnexal volume greater than 20 is predictive of a mass inside the ovaryovary

Color flow Doppler can be appreciated in a torsed ovary

63 Enlarged ovary crosses the Whirl pool sign midlinemidline

The role of inflammatory Surgical management of ovarian markers in ovarian torsion torsiontorsion Diagnostic and therapeutic laparoscopy 70 Exploratory laparotomy 60 Ovarian preservation 50 Detorsion of ovary Cystectomy pg/dl 40 Torsion P<0.001 Detorsion with second procedure cystectomy 30 No torsion Cyst aspiration 20 Ovarian bivalving 10 Oophoropexy 0 Oophorectomy IL-6 IL-8 E-selectin TNF

Daponte, et al. Novel serum inflammatory markers in patients with adnexal mass who had surgery for ovarian torsion. Fertility and Sterility 85(5)2006: 1469-72

Assessing ovarian viability

Color flow Doppler is not a reliable measure of What should you do with the ovarian viability purple, black, and ugly ovary? Leukocytosis, fever, and signs of peritonitis may indicate irreversible damage to the ovary

Macroscopic appearance is not a good indicator of viabilityviabilityof

64 Detorsion of the Ovary Case #1:Post operative ultrasound

StudyStudy % recovery of ovarian function 2.7 X 1.4 X 0.9 cm prepubertal uterus Oelsner et al, (1993) 91% (85/92) Mage et al., (1989) 94% (16/17) Left ovary is 2.6 X 1.5 X 1.4cm Shalev et al. (1995) 94% (49/52) Rody et al, (2002) 100%(1/1)100%(1/1) Right ovary is 2.4 X 1.8 X 1.0cm Azi z et al , (2004) 100%(14/14) Celik et al, (2005) 92%(13/14) Rousseau et. al. 100%(19/19) Levy et al. 100% (3/3)100% (3/3) Pansky et al 88% (7/8)(7/8)88% Cohen et al (1996) 100% (7/7)100% (7/7) TOTAL 93% (211/227)

How long is too long to wait to Ovarian torsion detorse the ovary? The adnexa of rats were twisted for 36 hours or Surgical emergency to preserve ovarian until they became bluishbluish--blackblack in appearance functioningfunctioning

All ovaries that were torsed under 24 hours showed no immediate or delayed evidence of Mus tht have hi hihliilidgh clinical index o f susp iiicion necrosis on histologic evaluation in patients with acute and/or intermittent, variable abdominal pain and Ovaries torsed for 36 hours showed immediate nausea/vomiting and delayed adnexal necrosis

Taskin et. Al, The effect of twisted ischemic adnexa managed by detorsion on ovarian viability and histology: An ischemic reprofusion rodent model. Human Reproduction 1998;13: 2823

Oophoropexy

UteroUtero--ovarianovarian ligament at the ovarian How do we prevent recurrent insertion is attached to the ipsilateral ovarian torsion? uterosacral ligament using permanent suturesuture Plication of the uteroutero--ovarianovarian ligament Ovary can be sutured to the pelvic sidewallsidewall

65 Oophoropexy Ovarian torsion key points Early intervention for ovarian torsion results in ProsPros ConsCons preservation of ovarian function, despite the gross May prevent recurrent torsion Theoretical risk of impaired appearance of the ovary of a detorsed ovary blood supply

May prevent torsion with Theoretical risk of peritubal Ultrasonographic appearance of the ovary is a useful polycystic ovaries adhesionsadhesions tool for managing ovarian torsion, but color flow Doppler is not reliable Elongated uteroutero--ovarianovarian Insufficient data on future ligaments likely pose a higher fertility functioning risk of recurrence Oophoropexy of the detorsed adnexa or the contralateral ovary may be appropriate May be prophylactically useful for the contralateral ovary following unilateral oophorectomy

Case #2#2Case

8y3mo with incidental finding of nonnon--tendertender abdominal mass on physical examination by pediatrician

Complex ovarian masses Ultrasound showed 11cm complex cystic ovarian mass

MRI sh owe d 101X8410.1 X 8.4 x 717.1 cm lo bu la te d cys tic mass with septations with a solid tubular component arising from the left ovary, a normal prepubertal right ovary measuring 1.9 x1.3 x 0.7cm and normal prepubertal uterus measuring 2.7 X 1.5 X 1.0cm, a small amount of free fluid in the pelvis

Tumor markers (AFP, HCG, inhibin, LDH) normal

Case #2 MRI Factors affecting surgical approach: laparotomy vs. laparoscopy? Risk of malignancy

Size and feasibility

Spillage and adhesion prevention

recurrence

66 Factors affecting ovarian Decision tree for ovarian preservation vs. oophorectomy enlargement

Risk of malignancy Ovarian enlargement Ratio of volume of neoppplasm to volume of OiOvarian mass <75 mm Ovarian mass >75mm normal ovarian tissue Predominantly cystic Predominantly solid Normal tumor markers Elevated tumor markers

BilateralityBilaterality Laparoscopy Laparotomy Peritoneal fluid sampling Peritoneal fluid sampling, staging and cystectomy Cystectomy with frozen section or oophorectomy Risk of ipsilateral or contralateral recurrence

Clinical findings suggesting Ultrasonographic benign vs. malignant mass characteristics of the ovary BenignBenign MalignantMalignant Benign pattern UnilateralUnilateral Bilateral Simple cyst without internal echoes Cystic Cystic SolidSolid Simple cyst with scattered echoes MobileMobile FixedFixed Central dense round echoes SmoothSmooth IrregularIrregular Thin or thick multiple linear echoes No ascitesNo ascites AscitesAscites Slow growing Rapid growth Malignant pattern Age <35yoAge <35yo Age >35yoAge >35yo Cystic echoes with papillary or indented mural parts Associated Irregular thick septations and solid parts endocrinopathy Heterogeneous component with irregular cystic part Completely solid with homogeneous component

Characteristics of benign tumors Predicting malignancy of the ovary Predominantly cystic Age Masses > 8cm <8cm tumor size Elevated tumor markers Predominantly solid Normal tumor markers Precocious puberty Palpable mass

Oltmann et. Al, Can we preoperatively risk stratify ovarian masses for malignancy? Oltmann et. Al, Can we preoperatively risk stratify ovarian masses for malignancy? Journal of Pediatric Surgery (2010) 45, 130130--134134 Journal of Pediatric Surgery (2010) 45, 130130--134134

67 Recommendations from Serum marker Associated tumor AFP Serum tumorendodermal markers Children’s Oncology Group sinus,embryonal carcinoma,mixed germ cell LDH choriocarcinoma, Tumor markers should be evaluated embryonal carcinoma, preoperatively mixed germ cell, HCG dysgerminoma, mixed germ cell Surgical intervention should evaluate the extent of disease, maximize complete tumor ressection, CA125 epithelial tumors spare uninvolved reproductive organs CEA serous cystadenocarcinoma, mucinous Incomplete surgical staging is upgraded and cystadenocarcinoma chemotherapy is advised Inhibin granulosa-theca cell tumor

Which complex ovarian masses Staging requirements require staging? Pelvic washings Neoplastic ovarian masses in children have 1010-- 20% risk of malignancy Visual inspection of contralateral ovary, pelvic Tumor markers are positive in only 54% of viscera, omentum, and peritoneal surface children with an ovarian malignancy Palpation of lymph nodes Ovarian masses greater than 8 cm

Prepubertal age range Removal of intact specimen with clean margins

Risk of rupture according to surgical procedure and surgical approach Tumor spillage Spillage upstages a malignant tumor Laparoscopy 92.8%92.8% P<0.001P<0.001 neccessiatating chemotherapy Laparotomy 36.8%36.8% P<0. 001P<0001001 Adhes iAdhiions Cystectomy 92%92% P<0.001P<0.001 Oophorectomy 14%14% Chemical peritonitis 00--0.2%0.2% P<0.001P<0.001

Templeman et al. The management of mature cystic teratomas in children and adolescents: a retrospective analysis. Human Reproduction 15(12) 2000:26692000:2669--7272

68 Outcomes for malignant tumors What is the result of ovarian treated with ovarian salvation preservation? 33--4%4% recurrence rate for mature teratomas and 2.6% recurrence for immature teratoma with 2.6% for immature teratomas ovarian preservation No recurrence 4.7y follow up after 00--18%18% recurrence rate for low malignant chthithththemotherapy without oophorectomy for pottiltttilttential tumors immature teratoma n=8 Recurrence with oophohorectomy plus 9.6%9.6%--14%14% recurrence in stage IA, grade 11--22 chemotherapy 22% recurrence for borderline ovarian tumors 9090--100%100% survival rate with chemotherapy +/+/-- treated with cystectomy only n=22 oophorectomy Beiner et al. Cystectomy for immature teratoma of the ovary. Gynecology Oncology. 2004; 93(2): 381381--44

Mini laparotomy with unilateral oophorectomy Is there a safe compromise between laparoscopy and lappyarotomy?

Complex ovarian masses key pointskey points Preoperative risk assessment for malignancy will help determine surgical approachapproach Children have a higher risk of ovarian malignancy than reproductive age women Negative tumor markers do not rule out malignancy

69 DisclosureDisclosure

Endometriosis in Adolescents •• I have no financial relationships to disclose.disclose. A Whole Different Ball Game

Joseph S. Sanfilippo, MD, MBA University of Pittsburgh School of Medicine

ENDOMETRIOSIS FIRST ObjectivesObjectives REPORTEDREPORTED •• Upon Completion of this Lecture the •• von Roikitansky 1860 Participant Will Understand: •• Sampson Variable Appearance –– Endometriosis is a Premenarchal Disease Endometriotic Implants 1920 ––FamilyFamily History is Associated with Incidence of •• Path Should be Obtained Endometrioma > 30% vs. 7.6% in Adults 3cm. – Importance of Multisystem Evaluation with •• Peritoneal Lesions: Chronic Pelvic Pain – Papular or Vesicular with Serous or Hemorrhagic Content

THEORIES OF CAUSATION

•• Retrograde Menstruation: “Sampson” •• Coelomic Metaplasia •• Lymphatic Metastasia •• Vascular Metastasis •• Iatrogenic Dissemination •• CellCell--mediatedmediated or Immunologic Defects Gleisher N et al OG 1987:70:115

70 PATHOPHYSIOLOGY

•• Defective Immune Surveillance •• “A Local PelvicPelvic--InflammatoryInflammatory Process” •• Peritoneal Macrophages --Their Secretory Products, Cytokines, Neovascularization – Cytokines: TNF alpha, Interleukins, Chemokines

PREMENARCHEAL

•• Two Premenarcheal Girls 12 y/a & 13 y/a •• Retrospective Review 67 Adolescents •• Emory University 19921992--19941994 WHERE DO WE BEGIN? •• Average Duration of Symptoms: 2.4 years –– LLtfLaparoscopy or Laparotomy for Pelvic Pain •• 49 Patients (73%) Endometriosis •• Majority Stage I (ASRM Class.) Adolescence – “Superficial Red Lesions” •• Stage III (6.1%) and Stage IV (2%) with NO Outflow Tract Obstruction – Age 12 Stage I No Mullerian Anomaly – Age 13 Cervical Dysgenesis Hematometra

Reese K et al J Pediatr Adolesc Gynecol 1996;9(3):125

Endometriosis in PremenarchalGirls Incidence of Endometriosis Without Uterine Anomalies in Adolescents •• Five premenarchal girls with chronic •• 2525––38%38% of adolescents with (>6 mos)mos) pelvic pain chronic pelvic pain •• Negative gastrointestinal evaluation ––JJ Reprod Med 1989;34:827 •• All had laparoscopic biopsy proven –– Clin Ex ppp ObstetObstetGGyyynecol 1999 ;;;26:76 endometriosis and ablative treatment •• 5050--70%70% of adolescents with pelvic •• All had marked improvement in pain pain not controlled with OCPs and •• Two had repeat laparoscopy 6 and 8 years later for pathologically confirmed NSAIDsNSAIDs endometriosis J Pediatr Adolesc Gynecol 1991996;9:125 J Pediatr Adolesc Gynecol 1997;10:199 Marsh EE, et al. Fertil Steril 2005;83:758.

71 Age of First Pelvic Symptoms Symptoms of Endometriosis

40 in Adolescents (n=49) 35 •• Cyclic pain (67%) 30

25 •• Noncyclic pain (39%)

20 Reggyistry I •• Dyy()smenorrhea (100%) 15 Registry II •• Gastrointestinal symptoms (67%) 10 •• Abdominal pain (58%) 5

0 •• Referred pain (31%) <15 15-19 20-24 25-29 30-34 35-39 40-44 >45 No Sx Davis GD, et al. J Adolesc Health 1993;14:362 Ballweg ML. J Pediatr Adolesc Gynecol 200316:S21.

What Are You Likely to Find in Adolescents?

Stage I or II “Atypical Red Lesions”

Diagnosis: Standard Technique and Systematic Investigation •• Panoramic view •• Vesicouterine peritoneum •• Anterior and posterior uterus •• CulCul--dede--sacsac and sigmoid •• Left ovarian fossa, ovary and tube •• Right ovarian fossa, ovary and tube •• Appendix and upper abdomen

72 Appearance of Endometriotic Implants in Adolescents Lesion type % Check out Peritoneal Pockets RedRed 82 Pigmented (Black/Blue) 76 Adolescents: Vesicular (Clear) 4141 Red Lesions "Flamelike"Flamelike"" "Polyps" WhiteWhite 6 or "Vesicles“ SuperficialSuperficial 98 Peritoneal pocket 18 Batt R J Pediatr Adolesc Gynecol 2003;16:337 Deep 12

Reese KA, et al. J Pediatr Adolesc Gynecol 1996;9:125.

Location of Superficial Endometriosis in ENDOMETRIOSIS & MAYERMAYER--ROIKATANSKYROIKATANSKY-- Adolescents (N=36) KUSTERKUSTER--HAUSERHAUSER SYN. Location Superficial Deep (>3mm) •• 20 y/o known Dx MRKH Syn. Broad ligament 26 (73%) - •• Increasing Pelvic Pain •• Operative LaparoscopyLaparoscopy--EndometriosisEndometriosis as Red CulCul--dede--sacsac 25 (69%) 11 (31%) Polypypypoid Lesions OvaryOvary 20 (56%) 7 (19%) •• (Stage I)(Stage I) UterosacralUterosacralligament 12 (33%) 21 (58%) •• Pelvic Kidney •• Implication: CoelomicMetaplasia Theory for Rectum 11 (31%) 7 (19%)(19%)7 EtiologyEtiology--EndometriosisEndometriosis Peritoneal pocket 6 (17%) - Bladder - 4 (11%) MokMok--LinLin E, LauferM et al JPAG 2009

Davis GD, et al. J Adolesc Health 1993;14:362

Dilute Vasopressin & Endometrioma’s

Less Coagulation Required Preservation of Ovarian Follicular ActivityActivity

73 Management Options Laparoscopic Treatment of Endometriosis in Teenagers •• SurgicalSurgical •• 31 patients (13(13--2020 years old) underwent ––ConservativeConservative laparoscopy for chronic pelvic pain unresponsive ––CorrectionCorrection of MMüüllerianllerian anomaly to NSAIDs and/or OCPs •• MedicalMedical •• Endometriosis found in 11 (36%) ––NSAIDsNSAIDs –– Stage I/II , N=5 ––OralOral contraceptives – Stage III, N=6 ––ProgestinsProgestins •• 3 with Stage I/II and 5 with Stage III were “pain ––GnRHGnRH agonists free or greatly improved” following surgery •• Combination Surgical/Medical •• 1 with Stage I and 1 with Stage III reported •• Alternative therapies for pain “partial improvement”

Stavroulis AI, et al. Eur J Obstet Gynecol Reprod Biol 2006;125:248

Tip of the Iceburg QuestionQuestion:: Can visualization of the endometriotic lesion accurately determine the depth of infiltration?

Overview Depth of Infiltration

30 Cornillie et al QuestionQuestion:: 25 Martin et al 20 % How deep are most 15 endldometriotic implants? 10 5

0

m m m m m m m m m m m

m m m m m m m m m

1 2 3 4 5 6 7 8 9 0m 0m

1 1

>

25% – 48% lesions are >5mm deep

(Cornillie et al. Fertility & Sterility, June 1990) (Martin et al. J of Gyn Surg, 1989)

74 Limitations of Fulguration Bipolar Instruments

LigaSure Sealing System Monopolar Bipolar destroys tissue destroys tissue • Continuous bipolar waveform at its tips between forceps

PK (PlasmaKinetic) Sealing System • Pulsed bipolar waveform • Allows cooling off pd between bursts of energy, reduces drying www.valleylab.com of tissue at contact point, and results in less sticking

Thermal Spread of Bipolar Excision of Endometriosis Laparoscopic Excision of Endometriosis: a randomized, Electrosurgery placeboplacebo--controlledcontrolled trial. (Abbott et al, Fertility & Sterility Oct. 2004)2004)

• 39 pts w/ any stage of Endometriosis

• Randomized to: Immediate group vs. Delayed Group L/S#1 excision staging L/S#2 (6mo) staging excision

• 6 & 12 month post-op evaluation  Thermal spread beyond the bipolar tips is <5mm.

Carbonell et al. J Laparoendoscopic & Advanced Surgical Techniques. 2003;13(6):377- 380.

GnRHagonist and Bone Mineral Density GnRH Agonists •• Indicated in > 16 y/a •• Monitor at 66--8mo.8mo. Then Every 2 yrs. For: Teens > 16 y/a •• All on Calcium & Vit D & Norethindrone Add:Add: Acetate 5mg/d (Add Back) CalciumCalcium •• Skeletal Defects: Spine NOT Hip (n=50) VitVit.. D. •• Bone Health Ctr by Gyn Program @ Add Back: Norethindrone Children's Hosp. Boston 1995-1995-20052005

Divasta A et al J Pediatr Adolesc Gynecol 2007;20:293s

75 E ffect of Effect of GnRH on Bone Density Effect of GnRH With AddAdd--BackBack on Bone Density

Author (Date) DrugLumbar spine BMD ∆∆(%)(%) 6 mos RX Post RX Author (Date) DrugLumbar spine BMD ∆∆(%)(%) 6 mosRX Post RX (6 mos)mos) Fukushima (1995)Buserelin --10.8%10.8%-8.1% -8.1% (6 mos) Edmond (1994) Goserelin --3.7%3.7%-2.1% -2.1% Revilla (1995) Triptorelin --2.9%2.9%-1.0% -1.0% (6 mos) 25 uggg EE22//g/g5 mg MPA -2.3%-2.3%-1.6% -1.6%

Dawood (1995) Leuprolide -14.0% -14.0%-3.3% -3.3% (1 yr) Howell (1995) Goserelin --4.1%4.1%-1.9% -1.9%

25 ugE 22/5 mg MPA -2.3%-2.3%-1.6% -1.6% Paoletti (1996) Goserelin --4.0%4.0%-6.0% -6.0% (6 mos) Moghissi (1996) Goserelin --4.1%4.1% - Taga (1996) Nafarelin --3.3%3.3%-2.2% -2.2% (6 mos) 0.3 mg CEE/5 mg MPA -2.0%-2.0% - 0.625 mg CEE/5 mg MPA --1.5%1.5% -

Lubiance JN, et al. J Reprod Med 1998;43:164

WHAT WORKS ?

•• NSAIDsNSAIDs ––COXCOX 2 –Selective–Selective Drugs •• OCPsOCPs--ContinuousContinuous ? Depending on EtiologyEtiology •• Neuropathic Analgesics – Tricyclic Antidepressants – SSRIsSSRIs •• Muscle Relaxants or Spasmolytics •• GnRHagonists

MULLERIAN ANOMALIES & OUTFLOW OTHER THERAPIES TRACT OBSTRUCTION

•• SPRMs (Selective Progesterone Receptor •• Incidence: 0.1-0.1-3.8%3.8% AdolescentsAdolescents--PelvicPelvic Pain Modulators) •• Presentation •• Aromatase Inhibitors •• Incomplete Obstruction •• IntraIntra--uterineuterine Contraceptive Systems •• Imperforate Hymen Diagnosis & Management •• Transverse Vaginal Septum

76 Are You Interested in the Genetics?Genetics?

If there is a Family History of ENDOMETRIOSIS IN Endometriosis ADOLESCENTS KEY POINTS •• Adolescent with Chronic Pelvic Pain Refractory to •• Multisystem Evaluation: GIGI--GUGU MusculoskelMusculoskel--PsychPsych--GynGyn Medical Therapy •• Prevalence: 47% – 30% Incidence vs 7.6% in Adults •• 2525--39%39% Present with Chronic Pelvic Pain •• Genetics:Genetics: •• Premenarchal Disease – Two Loci: Chrom. 10q26 & 20p13 •• ClCoelomi iMc Metapl liasiaa--EbEmbryoni iMllic Mullerian R ests •• Significance in Future Diagnosis or Predisposition •• Polygenic Multifactorial Mode of Inheritance to Endometriosis Screening •• Doctor Visits: > 5 VisitsVisits5 •• Diagnosis: 9.28 yrs from Onset of Symptoms Roman J Adolesc Endometriosis New Zealand Aust NZJ Obstet Gynecol •• Pain: Cyclic or NonNon--cycliccyclic 2010;50:179 •• Red Implants: Most Common (Clear Polypoid and White) Trolar S et al Am J Hum Genet 2005;77:365 •• Outflow Tract Obstruction: Stage IV Endometriosis

ACOG: Comm on Adolesc Health 2009

ENDOMETRIOSIS IN ADOLESCENTS KEY POINTS PAIN EVALUATION

•• Failure to Respond to NSAIDs OCPs •• Empiric Treatment: GnRHag •• If Pain Subsides=Diagnosis Endometriosis •• GRHGRHGnRHag > 16 y/ /16/a •• Add back: Norethindrone 5 mg/d •• 2% Femoral Neck bone Loss •• 5% Trabecular Bone Loss •• Majority of Bone Mass Growth Achieved by 20 y/a ACOG: Comm on Adolesc Health 2009

77 CHRONIC PELVIC PAIN CHRONIC PELVIC PAIN MULTISYSTEM APPROACH •• Definition: > 6 months6 months •• GIGI •• Prevalence: 38/1000 Females Aged 1515--7373 •• GUGU •• PQRST Approach •• GYNGYN – Provocative & Palliative •• MUSCULOSKELETAL – Quality of Pain •• PSYCHOLOGICAL/PSYCHIATRIC – Radiation & Relief – Timing

Ectopic Endometrial Glands/StromaGlands/Stroma CHRONIC PELVIC PAIN

•• Endometriosis •• AdhesionsAdhesions LUNGS •• Ovarian Mass

SKIN •• IBSIBS--ConstipationConstipation •• Inflammatory Bowel Disease BOWEL •• Musculoskeletal •• Psychosomatic BLADDER www.merck.com/media

Alternative Therapies for Pain LABORATORY ASSESSMENT •• Correct Diagnosis –– Role of LaparoscopyLaparoscopy--SystemsSystems Approach First •• Cognitive and behavioral •• CBCCBC –– Guided imagery •• Pregnancy Test –– Progressive muscle relaxation –– Biofeedback •• Sed Rate RateSedRate –– HiHypnosis •• Cervical Cultures •• Physical Therapy •• TriggerTrigger--PointPoint Injections or Peripheral Nerve •• Plain Film Abdomen (Stool) BlocksBlocks •• Pelvic Ultrasound –– Sacral Nerve Stimulation ? •• Acupuncture (Pediatrics 2000;104:941) •• Role of Laparoscopy •• InternetInternet •• Support Team

78 Endometriosis: Difficult to Dx

••An Educational Program from Irregular Capillary Lesions Black Powder -Burned Lesions ACOG and NASPAGNASPAG ••Include 32 clinical cases in Pediatric and Adolescent Gynecology

White Scarred Lesions Hemorrhagic Lesions Vesicular Lesions

..Online at http://sales.acog.org or http://www.naspag.org/store.cfm ..By phone at 800800--762762--ACOGACOG

79 Disclosure

Minimally Invasive Surgical Approach • I have no financial relationships to to Vaginal Agenesis disclose.

Heather Appelbaum, MD, FACOG Associate Professor, Obstetrics and Gynecology Hofstra NSLIJ School of Medicine Chief, Division of Pediatric and Adolescent Gynecology Steven and Alexandra Cohen Children’s Medical Center of New York

OBJECTIVE Müllerian agenesis Mayer 1. Review normal and abnormal embryologic Described vaginal dysgenesis (1829) development of the Müllerian structures and the urogenital sinus Rokitansky Further characterized vaginal agenesis (1938) 2. Describe different minimally invasive surgical approaches to creating a neovagina Küster 3. Compare the advantages and disadvantages of Identified associated renal anomalies (1910) minimally invasive surgical vs. non-surgical approaches to creating a neovagina Hauser Differentiated vaginal agenesis from androgen insensitivity (1961)

Associated defects

• Occurs in 1 in 4,000 to 5,000 live female • 30-50% have associated renal births anomalies • Variable Müllerian duct development • Assoc itdiated s kltlkeletal an d au ditory • 10% obstructed uterus anomalies

• 90% fibromuscular bilateral uterine remnants • 5% of anorectal malformations have associated vaginal agenesis

80 Developmental embryology of MMüllerianüllerian Duct Development the reproductive tract • The 2 Müllerian ducts are initially composed of solid • Gonads tissue and lie side by side. • Paramesonephros • Internal canalization of each duct produces 2 • Mesonephros channels divided by a septum that is resorbed in a cephalad direction by 20 weeks. • Metanephros • Urogenital sinus • The cranial, unfused portions develop into the fimbria and fallopian tubes. • Sinovaginal bulb • The caudal, fused portions form the uterus, cervix, and upper vagina.

Female genital tract MMüllerianüllerian Duct Development development • Müllerian duct progression • 6 weeks ––MMüüllerianllerian ducts elongate caudally and cross the metanephric ducts medially to meet in the midlinethe midline • Wolffian duct regression •• 7k7 weeks –– The urorect al sept um d evel ops and separates the rectum from the urogenital sinus • Renal development • 12 weeks – The caudal portion of the ducts fuse • Cloacal differentiation to form the uterovaginal canal which inserts into the urogenital sinus.

MayerMayer--RokitanskyRokitansky--KüsterKüster-- Vaginal agenesis Hauser Syndrome • Failure of the sinovaginal bulbs to develop • Congenital absence of the uterus and and form the vaginal plate vagina • May be caused by improper induction of • Karyotype 46,XX the sinovaginal bulbs from the neighboring • Normal ovaries uterovaginal primordium. • Normal secondary sex characteristics • Hymenal fringe is usually present along • Normal external genitalia with a small vaginal dimple because they • Vaginal dimple proximal to hymen are both derived embryologically from the • Associated renal agenesis or malformation urogenital sinus.

81 Androgen insensitivity

• 46, XY • Coital incidental dilation • Serial intermittent mechanical self dilation • Female body habitus, breast development • Continuous mechanical dilation and external genitalia – Vecchietti procedure • Short bli n d end vagi na – Balloon vaginoplasty • No Müllerian structures • Graft vaginoplasty – Split thickness skin graft • Absent axillary or pubic hair growth – Buccal mucosa – Bowel – Peritoneum • Williams labial flap vulvovaginoplasty

Mechanical intermittent self dilation: Frank or Ingram method Technique • Dilators of gradual sizes Advantages Disadvantages are used to create a vaginal space • Non surgical • Requires months to • Dilator is place by the approach years patient at the vaginal dimple • 85-90% success • Poor compliance • Pressure is applied for two rate • Inadvertent urethral or hours daily • Alternatively, sit on a rectal dilation bicycle seat, lean slightly forward with the mold in place in a pushing manner for 20 minutes 3 times a day Ingram JM. The bicycle seat stool in the treatment of vaginal agenesis and stenosis: a preliminary report. Am J Obstet Gynecol 1981;140:867-73.

Laparoscopic assisted creation of a neovagina • Modified Vecchietti procedure • Laparoscopically placed traction dilator device applies continuous pressure • Modified Davydov procedure resulting in invagination of the vaginal mucosa • Dilator remains in situ for 7-10 days on • Laparoscopic assisted bowel graft continuous traction vaginoplasty • Post procedural intermittent maintenance dilation with functional vagina after six • Modified balloon vaginoplasty weeks

82 The Vecchietti procedure Anatomical landmarks

• 1965 Vecchietti devised a traction device

• 1992 modified Vecchietti device for laparoscopic application

• 2004 new traction device and segmented dummy (Brucker, et al, 2004)

• 2009 modified Vecchietti instrument set FDA approved for use in the US

OR preparation Laparoscopic suture placement

Placement of the graduated dilator Placement of contralateral suture

• Sutures attached to graduated dilator are threaded through eye at the tip of the dissector and then carried through the rectovesicular space

• Post dilator placement requires cystoscopy and proctoscopy to confirm integrity of bladder and rectum

83 Affixing the device Post operative care

• Hospitalization for pain management 5-7 days • Adjust traction device q48hours • Device removed after 7- 10 days • Maintenance dilation with estrogen cream and rigid dilator twice weekly • Regular intercourse

Surgical outcomes Surgical complications n=71 • Mean duration of surgery 47.5 minutes • 11% developed UTI • Mean hospital stay 8.6 days • 2.8% accidental perforation of the bladder • Mean post operative vaginal length 9.6 cm • 1.4% bladder hematoma • Mean 3 month and 6 month vaginal length • 1.4% urethral necrosis 10.7cm • 1.4% vaginal synechiae • Epithelialization of vagina after 10.1 • 1.4% granulation tissue months • No rectal lesions

Brucker et al, Neovagina creation in vaginal agenesis: development of a new laparoscopic Brucker et al, Neovagina creation in vaginal agenesis: development of a new laparoscopic Vecchietti-based procedure and optimized instruments in a prospective comparative Vecchietti-based procedure and optimized instruments in a prospective comparative interventional study in 101 patients. Fertility and Sterility, 90 (5), 2008 interventional study in 101 patients. Fertility and Sterility, 90 (5), 2008

Patient satisfaction Long term outcomes

5 cases over 3 years reported satisfactory intercourse with • 110 patients underwent the laparoscopic improvement in self-confidence, self-esteem, general modified Vecchietti technique well being1

8 patients over 9 years median vaginal length was 1. 5cm2 • Followed at 1 month , 3 month , 6 month – Satisfactory intercourse (7.8/10) and 12 month postoperative – 4/6 had minor pain with sexual activity – 7/8 would have the procedure again • Vaginal length, Schiller’s test, quality of sexual intercourse assessed by Rosen’s 1. Kaloo et al., Laparoscopic-assisted Vecchietti Procedure for creation of a neovagina Australia and New Zealand Journal of Obstetrics and Gynaecology, 2002 Female Sexual Function Index (FSFI)

2. Keckstein, et al., Long-term outcome after laparoscopic creation of a neovagina in patients with Mayer-Rokitansky- L. Fedele, S. Bianchi, G. Fontino,et al. The laparoscopic Vecchietti’s modified technique in Rokitansky Kuster-Hauser syndrome by a modified Vecchietti procedure Obstetrical and Gynecological Survey, 2008 syndrome: anatomic, functional, and sexual long-term results. Am J Obstet Gynecol 2008; 198: 377

84 Advantages to the Vecchietti Long term outcomes procedureprocedure • Anatomic success was obtained in 104/106 • Minimally invasive (98%) patients • Functional success was obtained in 103/104 • Functional vagina created in (()99%) with no si gnificant difference in desire, approximately one week arousal, and satisfaction • Vaginoscopy showed 90% iodine-positive vaginal type epithelium • No long term post operative complications • Vaginal biopsies showed normal glycogen-rich normal squamous epithelium • Good long term sexual satisfaction

L. Fedele, S. Bianchi, G. Fontino,et al. The laparoscopic Vecchietti’s modified technique in Rokitansky syndrome: anatomic, functional, and sexual long-term results. Am J Obstet Gynecol 2008; 198: 377

Davydov technique

Surgical technique Postoperative care • Peritoneum is laparoscopically • Vaginal mold left in situ for six weeks mobilized • Functional vagina after six weeks must be • Crescentric incision at main ta ine d by in term itten t dila tion or vaginal dimple to the level of the peritoneum regular intercourse • Peritoneum pulled through to perineum and closed at the apex of the neovagina in a purse-string fashion

Surgical outcomes Laparoscopic bowel colpoplasty n=18 • 16/18 sexually active Surgical technique • Segment of bowel is mobilized on vascular • 14/16 sexually satisfied pedicle • 2/16 dyspareunia • End-to-end anastomosis of the bowel • Trochar site enlarged to 3cm to allow for distal • 0/18 vaginal stenosis end of graft to be exteriorized • 1/18 rectovaginal fistula • Distal end of graft closed in a purse string • Dissection of the rectovaginal space under • Vaginal length 6-9cm laparoscopic guidance • 0/18 vault prolapse • Tension free anastomosis to introitus with interrrupted circumfrential sutures

Soong, YK et al. Results of modified laparoscopically assisted neovaginoplasty in 18 patients with congenital absence of vagina. European Society for Human Reproduction and Embryology 11: 1996

85 Laparoscopic intestinal graft Outcomes laparoscopic sigmoid vaginoplasty colpoplasty Advantages Disadvantages n=7 • Most successful redo • Intestinal complications procedure • Not really minimally • Mean operative time=312 (220-450) min • Excellent option for invasive • Mean blood loss=decease in Hb=3.6g/dl patients with combined • Introital stenosis anorectal malformations • Mean hospital stay=7.7 days and vaginal ageneisis • LkhLeukorrhea • Adequate vaginal length • Mean vaginal length=11.5 cm (7-15cm) • Natural lubrication • Introital dilation required=2/7 • Early coitus • Lack of shrinkage •UTI=1/7 • ? minimally invasive • Vulvar hematoma=1/7

Darai, E et al. Anatomic and funcitonal results of laparoscopic-perineal neovagina construction by sigmoid colpoplasty in women with Rokitansky’s syndrome. Human Darai, E et al. Anatomic and funcitonal results of laparoscopic-perineal neovagina Reproduction 18(11): 2003 construction by sigmoid colpoplasty in women with Rokitansky’s syndrome. Human Reproduction 18(11): 2003

Satisfaction Balloon vaginoplasty • Laparoscopic suction irrigator used to apply downward • 4/7 sexually active pressure through rectovesicular space to level of vaginal dimple • 0/4 dyspareunia • Cannula used to guide needle intraperitoneally through • 3/4 completely satisfied the rectovesicular space and then exited out abdominally • Silk sutures affixed to 18g foley catheter • 7/7 satisfied with surgical outcome • Foley carried through intraperitoneal space to exit at the vaginal fovea • Inflate balloon with 15cc saline • Sutures removed and countertraction applied • Serial inflation of balloon

El Saman, AM. Enhancement balloon vaginoplasty for treatment of blind vagina due Darai, E et al. Anatomic and funcitonal results of laparoscopic-perineal neovagina to androgen insensitivity syndrome. Fertility and Sterility. Feb 2011 construction by sigmoid colpoplasty in women with Rokitansky’s syndrome. Human Reproduction 18(11): 2003

Surgical outcomes A look into the future of n=6 treatment for MMüüllerianllerian agenesis • 1/6 urethral injury • 1/6 rectal injury • Controlled studies with quality indicators • Neovagina 9-12cm comppgaring anatomical and functional • 5/6 sexual active outcomes of different approaches to • 90% sexual satisfaction vaginal agenesis are needed

• A look toward future human uterus transplantation (Green Journal, June 2012) El Saman, AM et al Modified balloon vaginoplasty: the fastest way to create a natural neovagina. American Journal of Obstetrics and Gynecology 2009

86 References References • Seong YK, et al. Results of modified laparoscopically • Brucker et al. Neovagina creation in vaginal ageneiss: development of a new laparoscopic Vecchietti-based assisted neovaginoplasty in 18 patients with congenital procedure and optimized instruments in a prospective absence of vagina. Europ Soc Hum Reprod Embryol comparative interventional study in 101 patients. Fert 1996;11(1): 200-203 Ster 2008;90(5) • Darai E, et al. Anatomic and functional results of • Fedele L, Bianchi S, Frontino G. The laparoscopic Veccietti’s modified technique in Rokitansky syndrome; laparoscopic-perineal neovagina construction by sigmoid anatiftiltomic, funtional, and sexual ll long-tltterm results. AJAm J colltilpoplasty in women w ithRkitk’dith Rokitansky’s syndrome. Hum Obstet Gynecol 2008; 198: 137- Reprod 2003; 18(11): 2454-9 • Fedele L, et al. Creation of a neovagina by Davydov’s • El Saman AM, et al. Enhancement balloon vaginoplasty laparoscopic modified technique in patients with Rokitansky syndrome. Am J of Ob Gynecol 2010; for treatment of blind vagina due to androgen 202(33): e1-6 insensitivity syndrome. Fert Ster 2011;95(2): 779-84 • Allen L, et al. Psychosexual and functional outcomes • El Saman AM, et al. Modified balloon vaginoplasty; the after creation of a aneovagina with laparoscopic fastest way to create a natural neovagina Am J Ob Davydov in patients with vaginal agenesis. Fert Ster 2010; 94(6): 2272-6 Gynecol 2009:546e1-6

87 DisclosureDisclosure

Preserving Fertility in •• Consultant: Ethicon EndoEndo--Surgery,Surgery, Ethicon Women's Health & Urology, Conceptus Adolescents with Cancer Incorporated, CONMED Corporation, UpToDateUpToDate Robert K. Zurawin, MD Director, Minimally Invasive Gynecologic Surgery Baylor College of Medicine Houston, Texas

Our case… Ms. BH HodgkinHodgkin’’s lymphoma •• The most common childhood cancer between Ms. BH is an 18yo G0 AAF with PMH the ages of 1515--1919 years old. significant for Hodgkin’’s Lymphoma, HTN, •• Incidence higher in females vs males •• Defined histopathologically by the presence of DM who initially presented to the Pedi Gyn clonal malignant Hodgkin/Reed --Sternberg (HRS) clinic in 09/2006 prior to initiation of cells with a variable cellular infiltrate. chemotherapy for discussion of her fertility •• EpsteinEpstein--BarrBarr virus (EBV) infection is associated with HL and can be detected in HRS cells… 2525-- 50% of classical HL in developed countries are EBV positive. •• Most common pediatric type is ““nodular sclerosingsclerosing”” type.

HodgkinHodgkin’’s lymphoma Cancer diagnosis •• Most children with HL present with painless lymphadenopathy, usually cervical, •• 4% of all people newly diagnosed with cancer supraclavicular, axillary, or, less often, inguinal – are younger than 35 years old (40,000 per feel rubbery. year).year). •• Theyyypy also have many nonspecific systemic •• 11--2%2% of all people newly diagnosed with cancer symptoms including fatigue, anorexia, and are younger than 19 years old (12 ,000 per weight loss. year).year). – Fewer than 20 percent of chchildrenildren with HL have the •• Most common ““young people”” cancers: classic fever and night sweats that are seen in adults. HodgkinHodgkin’’s lymphoma, leukemia, melanoma, •• Twenty years after diagnosis of HL, the cervical cancer, breast cancer. cumulative incidence of second malignancies •• In 2010, it is estimated that 1/250 adults will be was 7.6%.was 7.6%. childhood cancer survivors. – Breast cancer, thyroid cancer, and soft tissue sarcomas were the most common ones.

88 Infertility risk InfertilityInfertility •• Rates of permanent infertility depends greatly •• Risk of infertility is associated with 3 key aspects of on many factors… cancer theapy: patient gender and age, type of •• In females, infertility can be related to chemotherapy used, whether or not radiation is used decreased available primordial follicles or, (where and what dosage). alterations in blood supply yppy available to the •• Grea tes t in fer tility r is k is assoc iat e d w ith ch emoth erapy reproductive tract, or disruptions in the using alkylating agents: cyclophosphamide, isofosfamide, ““normalnormal”” anatomic locations of the reproductive nitrosoureas, cholorambucil, muphalan, busulfan, organs, or disruptions in hormone production. procarbazine. Causes accelerated oocyte apoptosis…. •• Resumption of menses is NOT an indicator of •• Radiation risk highest with any type of abdominal pelvic fertility, as patients typically believe. radiation, but also with cranial! •• And retained fertility immediately after treatment does not mean a normal duration of fertility (aka – increased risk of POF)

A refresher… Possible treatment effects

•• The number of oocytes that females have •• Immediate infertility at birth is FIXED at approximately 11--22 •• Increased risk of miscarriages million.million. •• Premature ovarian failure – 6 month old: 700,000 – Menopause before age 40 – 7 years old: 300,000 – Childhood Cancer SurvSurvivivoror Study showed us – 37 years old: 25,000 that 8% had POF overall and those who – 50 years old: 1,000 received radiation to the pelvis or abdomen had a 30% chance of developing POF.

Bone marrow transplant Abdominal/Pelvic radiation

•• Bone marrow transplant is associated with •• In one case series, 71% of treated prepre--pubertalpubertal a >90% risk of POF secondary to pre-pre- girls failed to enter into puberty, and 26% of the therapy whole body radiation. cases that did experience puberty had POF (rad doses all between 2000 --3000 cGy)cGy)3000 •• Only 9 reports of return of ovarian •• Ovarian tissues have a LD value of 600 cGy function in a population of 144 patients 5050 •• It has become apparent that ovaries which are studied (all < 25 years old). located outside of radiation field continue to function much more normally than direct or indirectly radiated ovaries (volume dependent).

89 Degree of risk Degree of risk

Lower risk ( 20%): High risk ( 80%): • ABVD (doxorubicin/bleomycin/vinblastin/dacarbazine) • Hematopoietic stem cell transplantation with cyclophosphamide/total body • CHOP 4-6 cycles (cyclophosphamide/doxorubicin/vincristine/prednisone) irradiation or cyclophosphamide/busulfan • CVP (cyclophosphamide/vincristine/prednisone) • External beam radiation to a field that includes the ovaries • AML therapy (anthracycline/cytarabine) • CMF, CEF, CAF 6 cycles in women age 40 and older (adjuvant breast cancer • ALL therapy (multi-agent) therapy with combinations of cyclophosphamide, methotrexate, fluorouracil, • CMF, CEF, CAF 6 cycles in women less than 30 (adjuvant breast cancer doxorubicin, epirubicin) therapy with combinations of cyclophosphamide, methotrexate, fluorouracil, doxorubicin, epirubicin) Intermediate risk: • AC 4 in women less than 40 (adjuvant breast cancer therapy with • CMF, CEF, CAF 6 cycles in women age 30-39 (adjuvant breast cancer therapy doxorubicin/cyclophosphamide) with combinations of cyclophosphamide, methotrexate, fluorouracil, doxorubicin, epirubicin) Very low or no risk: • AC 4 in women age 40 and older (adjuvant breast cancer therapy with Vincristine, Methotrexate, 5-fluorouracil doxorubicin/cyclophosphamide) Unknown risk: Taxanes, Oxaliplatin, Irinotecan, Monoclonal antibodies (trastuzumab, bevacizumab, cetuximab),Tyrosine kinase inhibitors (erlotinib, imatinib)

Risk assessment HodgkinHodgkin’’s Lymphoma

•• Typical chemotherapy regimen consists of: chlorambucil, vinblastine, procarbazine, prednisolone. –– Risk of gonadal failure in men –– 86%86% – Risk of gonadal failure in women – 50%50% •• Alternative regimen (without alkylating agent): adriamycin, bleomycin, vinblastine, dacarbazine

Fertility preservation options Embryo cryopreservation •• Depends on the age, diagnosis, type of •• Obviously requires a partner... treatment needed, whether or not the patient •• After puberty has a partner… •• Delay of cancer treatment: 2-2-66 weeks •• Live birth rates depend on the patient’’s age and number •• Surveys have found that at least 50% of all men of embryos able to be cryopreserved (usually about 1010-- and women treated with cancer during their 25% per embryo). reproductive ages do NOT recall ever having •• Pt must be willing to undergo ovarian hyperstimulation discussed the issue of fertility with their with daily hormone injections x 2 weeks and travel to multiple u/s appts to eval follicles prior to collection oncologist and many of those that DO report procedure.procedure. having had such a discussion felt that their •• The most established technique for fertility preservation concerns were not appropriately addressed. in women.in women. •• Cost: approx $8,000 per cycle, $350 per year storage •• Psychologic counseling also should be offered as fees.fees. a part of such discussions…

90 Oocyte cryopreservation Ovarian tissue cryopreservation (investigational) (investigational) •• No partner/donor required. •• Before or after puberty •• After puberty •• Clearly not suitable if cancer is suspected to have metastasized to ovarian tissue. •• Same time commitment,,, same cost. •• Still in early stages … case reports only; as of •• Definitely investigational… small case September 2012, only twenty live births series and case reports; as of 2005, 120 reported.reported. deliveries reported, approximately 11--3%3% •• ReRe--implantationimplantation can restore hormonal function live births per thawed oocyte (3(3--44 times •• Cost: >12,000 lower than standard IVF)

Donnez J, et al. ”Live birth after transplantation of frozen-thawed ovarian tissue after bilateral oophorectomy for benign disease” Fertil Steril 2012;98:720–5.

Strategies to preserve fertility in female Gonadal shielding during radiation cancer patients through freezing therapy (studied)

•• Only selectively possible. •• Expertise IS required to ensure that shielding does not interfere with other nearby areas (unwanted increases in dosing, etc). •• No additional cost.

Kim. Fertility preservation. Fertil Steril 2006;85:1–11

Ovarian transposition/oophoropexy Oophoropexy (studied)(studied) •• Same day, outpatient procedure (minimal time commitment compared to others) •• The ovaries and their attached vascular •• Helps to prevent radiation damage, specifically, supply from the ovarian vessels are and should be done two weeks or less ppprior to brought out of the pelvis and sutured therapy initiation to prevent dislocation. lateral and above the psoas muscle to get •• May need rere--positioningpositioning later, or IVF. them out of the field of radiation. •• Large cohort studies and case series suggest ––SomeSome authors recommend using permanent approximately 50% chance of success due to suture for this procedure and not dividing any altered ovarian blood flow and scattered of the attachments. radiation.radiation.

91 Trachelectomy Oophoropexy (studied)(studied) •• Obviously for young cervical cancer cases… •• Limited to early stage cases only •• Requires inpatient admission and usually about 6 weeks of recovery prior to treatment initiation. •• Expertise may be lacking •• Would require cerclage to maintain future pregnancy.pregnancy.

Ovarian suppression with GnRH analogs or antagonists (investigational) The ethics question •• Chemotherapy protective. •• After puberty. •• Deciding to pursue or forgo potential fertility sparing procedures/treatments requires a high level of decision •• Given before and during treatment with chemo to making capacity and is very often made by the child’’s stop ““ovarian activity”” and theoretically protect the parents.parents. adnexa at the cellular level . •• Is parenth ood somethi ng tha t these c hildren wou ld even •• Reduces risk of POF from about 58% to 3%, want in the future? especially when cytotoxic alkylating agents are •• It is hard to take out the personal bias of parents from used.used. this decision… too unfocused and dreaming of grandchildren someday vs too focused on saving the •• Give agonist for immediate treatment initiation and patient now. Parental judgement may not reflect the OK to give antagonist if can delay treatment for patientpatient’’s own best interest in the future. approx 4 weeks. •• Cost: approximately $500/mo (monthly injections)

Back to our case…

The patient denied sexual activity or being in a stable relationship. It was clear, however, that she desired to have children What would you do? in the future but she was wary of operations and was told that she needed to start her chemotherapy soon…

92 What we did… What we did…

We offered her Lupron for suppression Long discussion (2 hours) was had with during her chemotherapy and she the patient and her mother regarding the accepted this option. She continued it and patientpatient’’s wishes for future fertility –it– it was did very well –– chemo ended 11/2006. decided that she would undergo surgery for oophoropexy in preparation for her But, then, pelvic lymphadenopathy was need for localized pelvic therapy. noted… oncology plan: local radiation.

Unresolved issues in human ovarian Pregnancy after cancer treatment transplantation •• Pregnancy may be complicated with an overall •• Patient selection and exclusion criteria increased risk of organ impairment, especially of •• Optimization of freeze-freeze-thawthaw protocols the heart, lungs, and uterus (consider testing •• Optimal graft site(s) prepre--conceptually).conceptually). •• Quality of oocytes matured in a graft •• There is evidence that ppgregnancy may increase •• Efficacy of transplantation for restoration of the risk of worsening cardiac ejection fraction in fertilityfertility women treated with doxorubicin for childhood cancer,150 and uterine or totaltotal--bodybody irradiation •• Safety issues appears to increase the risk of miscarriage, •• IschemiaIschemia--reperfusionreperfusion injury prematurity and low birth weight. •• Prospects for in vitro follicle culture •• Also, if the patient will be using IVF, the •• Long term adverse effects on offspring increased risk of multiple gestation worsens these above risks/considerations. Kim. Fertility preservation. Fertil Steril 2006;85:1–11

The progeny Additional parenting options

•• Aside from hereditary genetic syndromes, •• IVF with donor eggs or embryos however, there is scant evidence that a history – Expensive (10,000 per cycle) of cancer, cancer therapy, or fertility •• SurrogacySurrogacy interventions increases the risk of problems in ––ExpensiveExpensive (20(20--100,000)100,000) the progeny. •• AdoptionAdoption •• Birth defects of progeny of cancer survivors carry the same overall risks of birth defects as ––ExpensiveExpensive (5(5--35,000)35,000) the general population(2population(2--3%).3%). – Personal h/o cancer CAN be prohibitive with waiting period, etc

93 ASRM Ethics Committee Statement ASRM Ethics Committee Statement

•• 11.. Physicians should informinform cancer patients about •• 5. Parents may act to preserve fertility of cancer patients options for fertility preservation and future reproduction who are minors if the child assents and the intervention prior to treatment. is likely to provide net benefits to the child. •• 2. Theeo only yestabs establish ed m etodsoethods of f etertili typesety preserv atoation •• 66ecsestuctossoudbegeaboutte. Precise instructions should be given about the are sperm cryopreservaticryopreservationon in men and embryo disposition of stored gametes, embryos, or gonadal cryopreservation in women. tissue in the event of the patipatientent’’ss death, unavailability, •• 3. Experimental procedures such as oocyte or ovarian or other contingency. tissue cryopreservation shouldshould be offered only in a •• 7. Preimplantation genetic diagnosis to avoid the birth of research setting with IRB oversight. offspring with a high risk of inherited cancer is ethically •• 4. Concerns about the welfare of resulting offspring acceptable. should not be cause for denying cancer patients assistance in reproducingreproducing..

Latest Information

•• ASRM resources on Fertility Preservation –– https://www.asrm.org/topics/detail.aspx?id=4 5555 THE END!END!THE

Any questions?

94 CULTURAL AND LINGUISTIC COMPETENCY

Governor Arnold Schwarzenegger signed into law AB 1195 (eff. 7/1/06) requiring local CME providers, such as the AAGL, to assist in enhancing the cultural and linguistic competency of California’s physicians (researchers and doctors without patient contact are exempt). This mandate follows the federal Civil Rights Act of 1964, Executive Order 13166 (2000) and the Dymally-Alatorre Bilingual Services Act (1973), all of which recognize, as confirmed by the US Census Bureau, that substantial numbers of patients possess limited English proficiency (LEP).

US Population California Language Spoken at Home Language Spoken at Home

Spanish English Spanish

Indo-Euro English Indo-Euro Asian Other Asian

Other 19.7% of the US Population speaks a language other than English at home In California, this number is 42.5%

California Business & Professions Code §2190.1(c)(3) requires a review and explanation of the laws identified above so as to fulfill AAGL’s obligations pursuant to California law. Additional guidance is provided by the Institute for Medical Quality at http://www.imq.org

Title VI of the Civil Rights Act of 1964 prohibits recipients of federal financial assistance from discriminating against or otherwise excluding individuals on the basis of race, color, or national origin in any of their activities. In 1974, the US Supreme Court recognized LEP individuals as potential victims of national origin discrimination. In all situations, federal agencies are required to assess the number or proportion of LEP individuals in the eligible service population, the frequency with which they come into contact with the program, the importance of the services, and the resources available to the recipient, including the mix of oral and written language services. Additional details may be found in the Department of Justice Policy Guidance Document: Enforcement of Title VI of the Civil Rights Act of 1964 http://www.usdoj.gov/crt/cor/pubs.htm.

Executive Order 13166,”Improving Access to Services for Persons with Limited English Proficiency”, signed by the President on August 11, 2000 http://www.usdoj.gov/crt/cor/13166.htm was the genesis of the Guidance Document mentioned above. The Executive Order requires all federal agencies, including those which provide federal financial assistance, to examine the services they provide, identify any need for services to LEP individuals, and develop and implement a system to provide those services so LEP persons can have meaningful access.

Dymally-Alatorre Bilingual Services Act (California Government Code §7290 et seq.) requires every California state agency which either provides information to, or has contact with, the public to provide bilingual interpreters as well as translated materials explaining those services whenever the local agency serves LEP members of a group whose numbers exceed 5% of the general population.

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If you add staff to assist with LEP patients, confirm their translation skills, not just their language skills. A 2007 Northern California study from Sutter Health confirmed that being bilingual does not guarantee competence as a medical interpreter. http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2078538.

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