Pediatric & Adolescent Gynecology – A “How To” Approach (Didactic)
PROGRAM CHAIR Joseph S. Sanfi lippo, MD
PROGRAM CO-CHAIR Sara Brucker, MD
Heather Appelbaum, MD Marc R. Laufer, MD Robert K. Zurawin MD
Sponsored by AAGL Advancing Minimally Invasive Gynecology Worldwide
Professional Education Information
Target Audience This educational activity is developed to meet the needs of residents, fellows and new minimally invasive specialists 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
A Pediatric and Adolescent Gynecology Minimally Invasive Practice – A “How to” Approach J.S. Sanfilippo ...... 4
Minimally Invasive Surgery in the Pediatric and Adolescent Patient: Vaginoscopy, Hysteroscopy, Laparoscopy and Robotics R.K. Zurawin ...... 19
Pediatric and Adolescent Gynecologic Emergencies H. Appelbaum ...... 35
Endometriosis in Adolescents – The Beginning of the Story M.R. Laufer ...... 53
Surgical Approach to Müllerian Anomolies and Vaginal Agenesis S. Brucker ...... 64
Pediatric Gynecologic Oncology R.K. Zurawin ...... 72
Gynecologic Outcomes of Bariatric Surgery in the PAG Patient J.S. Sanfilippo ...... 78
Fertility Preservation – How and Why S. Brucker ...... 88
Cultural and Linguistics Competency ...... 94
PG 212 Pediatric & Adolescent Gynecology – A “How To” Approach (Didactic)
Joseph S. Sanfilippo, Chair Sara Brucker, Co-Chair
Faculty: Heather Appelbaum, Marc R. Laufer, Robert K. Zurawin
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 presented. 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 in fertility preservation when a young patient is faced with a diagnosis of cancer or other chronic debilitating disease. Counseling and discussion of options for future fertility will be succinctly presented. The problem of obesity is a recurrent concern for clinicians; evaluation, counseling and management of obesity continue to challenge the gynecologist. 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.
Learning Objectives: At the conclusion of this activity, the clinician 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.
Course Outline
8:00 Welcome, Introductions and Course Overview J.S. Sanfilippo
8:05 A Pediatric and Adolescent Gynecology Minimally Invasive Practice – A “How to” Approach J.S. Sanfilippo
8:30 Minimally Invasive Surgery in the Pediatric and Adolescent Patient: Vaginoscopy, Hysteroscopy, Laparoscopy and Robotics R.K. Zurawin
8:55 Pediatric and Adolescent Gynecologic Emergencies H. Appelbaum
9:20 Endometriosis in Adolescents – The Beginning of the Story M.R. Laufer
9:45 Questions and Answers All Faculty
9:55 Break
10:10 Surgical Approach to Müllerian Anomolies and Vaginal Agenesis S. Brucker
1 10:35 Pediatric Gynecologic Oncology R.K. Zurawin
11:00 Gynecologic Outcomes of Bariatric Surgery in the PAG Patient J.S. Sanfilippo
11:25 Fertility Preservation – How and Why S. Brucker
11:50 Questions & Answers All Faculty
12:00 Course Evaluation/Adjourn
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 Kimberly A. Kho* Frank D. Loffer, Executive Vice President/Medical Director, AAGL* Linda Michels, Executive Director, AAGL* M. Jonathan Solnik* Johnny Yi*
SCIENTIFIC PROGRAM COMMITTEE Ceana H. Nezhat Consultant: Ethicon Endo-Surgery, Lumenis, Karl Storz Other: Medical Advisor: Plasma Surgical Other: Scientific Advisory Board: SurgiQuest Arnold P. Advincula Consultant: Blue Endo, CooperSurgical, Covidien, Intuitive Surgical, SurgiQuest Other: Royalties: CooperSurgical Linda D. Bradley* Victor Gomel* Keith B. Isaacson* Grace M. Janik Grants/Research Support: Hologic Consultant: Karl Storz C.Y. Liu* Javier F. Magrina* Andrew I. Sokol*
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). Heather Appelbaum* Sara Y. Brucker* Marc R. Laufer* Joseph S. Sanfilippo* Robert K. Zurawin Consultant: Conceptus Incorporated, Ethicon Endo-Surgery, Hologic
Asterisk (*) denotes no financial relationships to disclose. Pediatric & Adolescent Gynecology Minimally Invasive Practice “A How To” Approach Disclosure
Joseph S. Sanfilippo, MD, MBA Professor OB, GYN, Repro Sciences I have no financial relationships University of Pittsburgh to disclose. Magee-Womens Hospital
4 5 PEDIATRIC PATIENT
. Patient Involved in History . Frog-legged Position . Knee-chest Position . “Show and Tell” . Low power Magnification . “Good job”
ADOLESCENT EXAM-PARADIGM PELVIC EXAM SHIFT
. TOOL KIT-ACOG . “Do You Use Tampons?” . First Exam . “Gynecologic Encounter” 13-15 Y/A – Collaborative with Primary Care Provider – Rapport with OB GYN – No Pelvic Exam – Followed By Annual Visits . Age 21 years of age Stewart F et al JAMA 2001;286:671 ACS, NIH, ACOG 2002
Tool Kit for Teen Care
. The Tool Kit for Teen Care is a comprehensive resource designed by the ACOG Committee on Adolescent Health Care to help every office care for adolescent patients.
6 SCREENING FOR SEXUALLY TRANSMITTED DISEASES
. Sexually Active Teens Should be Screened . “Urine screening Should be Considered When Teens are Reluctant to Have a Pelvic Exam” – Urine Ligase Chain Reaction-Less Expense than Cervical Cultures . “Vaginal Swab” Screening
Health Care for Adolescents ACOG 2002
Adolescence is Risky Business
. Latest: 63% Adolescents Sexually Active: Grade 12 . ACOG: First Adolescent Health Visit: 3-15 y/a – Development of Trust with Healthcare Provider . One if Four “Sexually Experienced: Adolescents Contract STI . Adolescent Pregnancy Rate: 39.1/1000 Adolescent Girls
Short M et al JPAG 2013;26:1
7 HISTORICAL PERSPECTIVE
. Late 1970’s Pediatric-sized Hopkins Rod Lens System “Telescopies” . TOOLS Of The TRADE . Instrumentation 2 mm “Pediatric Endoscope . Small Trocar 2.5 cm length . 3mm Diameter Instrumentation
PEDIATRIC MIS TECHNIQUE PEDIATRIC PATIENT
. Bladder Crede . Veress Needle vs. Open Technique . NG tube Placement . Ports Suture closed at End of Case-Thin . Insufflation Pressure Abdominal Wall Flow 0.5 L/minute – Separate Fascia and Skin Layers Pressure Setting . Post-op Reglan-Prevent Nausea Infant 6-8 mmHg Pediatric Age 8-10 mmHg . Transdermal Scope Patch > 8 y/a Adolescents 10-12 mmHg
8 SPECIFIC PROBLEMS YOU MAY ENCOUNTER
9 10 11 BREAST MASS-ADOLESCENT
. Ultrasound . Little if any Role for Mammography . Monitoring 1-3 cycles . FNA . Malignancy 0.2% of Carcinoma-Breast < 25 y/a – Incidence 0.1/1,000,000 per year . BSE Instruction
Simmons P in Pediatric & Adolescent Gynecology ed. Sanfilippo 2001 Saunders
WHAT DO YOU NEED TO MAKE THE DIAGNOSIS
. REVISED DIAGNOSTIC CRITERIA-PCOS ARE YOU UP TO DATE ON – 2003 CRITERIA PCOS? . Oligo-anovulation . Clinical or Biochemical Signs of Hyperandrogenism In the Adolescent? . Polycystic Ovaries And Exclusion of Other Etiologies (CAH, Cushing’s Syndrome)
Rotterdam ESHRE/ASRM PCOS Consensus Workshop Group Fertil Steril 2004’81:19
12 PCOS-ULTRASOUND Unique Problems for Adolescents
. Chronic Anovulation is Common . By Definition: . Menstrual Irregularities are Common – 12 or more Follicles @ – 40 to 50% Adolescents-Anovulatory Cycles 2-9mm in diameter . Ovulatory Cycles: and/or Increased – 23-35% Gyn Year 1 Ovarian Volume (>10mL) – 70-80% > Gyn Year 5 . Normal is” Menstrual Cycles 40-45days Until 2-3 years After Menarche
Carmina E, Lobo R et al AJOG Sept 2010
POLYCYSTIC OVARIAN SYNDROME in PCOS ADOLESCENCE OBSERVATIONS . In General 35 days-Upper Limit Normal in . Most Common Endocrinopathy Reproductive Age Women 98% of Adults-Prospective Data . Abnormalities Begin in Adolescence-If Not Earlier ? (Intra-uterine: IUGR-Low Birth Weight and PCOS development) . In Adolescents: 35-40 days in 66% . Rapid LH Pulse Frequency . Hyperinsulinemia . Irregular Menses Persisting 2 yrs After . Increased Androgen Production . Genetic: Autosomal Dominant ? (Single Gene Disorder) Menarche=Criteria to Diagnose PCOS in More Likely” Polygeneic Multifactorial Mode of Inheritance The Search for the “Candidate Gene”-more Than 50 Genes-Looked At Adolescents . CYP-11A-codes for P450 cholesterol side chain cleavage-rate limiting enzyme . Chromosome 19p13.2 Located Near Insulin Receptor “Looks Good” Carmina E, Lobo R et al AJGO Sept. 2010 Stewart D, et al J Clin Endocrinol Metab 2006;91:411 Franks S Reprod Biomed Online 2006;12:526
TESTS TO ORDER CHRONIC ANOVULATION-HIRSUTISM PCOS in ADOLESCENTS
. No Need for LH and/or FSH . Long-Term Problems: Metabolic Syn-DM-Type II-Infertility . Obtain . Polycystic Ovaries May or May Not be Present-Not in NIH definition PCOS – Total Testosterone . Ovarian Size-Max. Perimenarcheal Girl – DHEA-S . 10% Regularly Menstruation Adolescents-Polycystic – 17Hydroxyprogesterone (AM-Fasting) Appearing Ovaries – Prolactin . 40% Girls with Irregular Menses-Arrested Follicles-PCOS-like –TSH Phenotype with Abnormal 17-OHP Responses to GnRH analogue . Increased cytochrome P450c17alpha Activity In Both Ovary and Adrenal=Hyperandrogenemia Rotterdam ESHRE/ASRM PCOS Consensus Workshop Group Fertil Steril 2004’81:19
Blank S, et al Ann NY Acad Sci 2008;1135:76
13 SUSPECTING CHILD SEXUAL ABUSE
. Clinging Behavior . Nightmares . Excessive Masturbation “When In Doubt Check it Out”
DEFINING SEXUAL ABUSE
. Touching Over Child’s Clothing . Use Child for Sexual Gratification – Exhibitionism – Voyeurism – Child Pornography . Child Feels Betrayed, Confused
Britton, H et al Clin OG 1997; 40:226
14 THE NEW BUZZ WORD
. ATM – Abstinence ‘til Marriage
EMERGENCY CONTRACEPTION
WHERE IS THE FDA-Today?
15 ADOLESCENT’S USE of EMERGENCY CONTRACEPTION
. Washington State Pharmacists . 126 Teens Obtained ECP from Pharmacists . 20 item Questionnaire . If Pharmacy Access not Available 22% See if Pregnant – 58% Would See a Doctor . CONCL: ECP by Pharmacists-Efficacious
Sucato, G et al JPAG 2001;14:163
LARCs Long Acting Reversible Contraceptives
. 40% Unintended Pregnancies=Incorrect/Inconsistent What’s New? Use of Contraceptives . CHOICE Study – When Cost Removed >60% Adolescents Choose LARCs for Contraception – One Problem Adolescents/Young Adults Not Aware of Options . Off Label: Control of Uterine Bleeding Ongoing Research: Endometriosis Rx
Bryant, A et al JPAG 2013;25:347
CHRONIC PELVIC PAIN
. PQRST APPROACH CHRONIC PELVIC PAIN IN – Provocative-Palliative – Quality THE ADOLESCENT . Sharp or Dull – Radiation-Relief . Lower Extremity-Torsion – Severity – Timing
16 ENDOMETRIOSIS
. Youngest 8 y/a . Can Occur Before Menarche-Bx Proven . Clear-Reddish Lesions Not Brown-Black . Pain Cyclic or Acyclic . Adolescent-Chronic Pelvic Pain Incidence Endometriosis 25-38% Post-menarche 4-17% Females
Laufer M et al JPAG 2003;16:S3
APPENDECTOMY IN CHRONIC CHRONIC PELVIC PAIN PELVIC PAIN
. Retrospective Study 190 Women . Prophylactic Appendectomy . RESULTS: 154 Abnormal – Endometriosis – Carcinoid – Periappendicitis – Fibrous Obliteration – Lymphoid Hyperplasia Lyons T et al JAAGL 2001;8(4):542
APPENDECTOMY IN CHRONIC Training in Pediatric Adolescent Gyn PELVIC PAIN . Quality of Resident Education Markedly . CONCLUSION: Counsel Patients Effected by PAG Trained Faculty or Not Undergoing Operative Laparoscopy of the High Frequency of Abnormal Findings- – Fellowship Programs Nationwide Appendix – NASPAG (North American Society Pediatric Adolescent Gynecology) Education Committee . “Appendectomy Appears To Be A Resident Education Programs Worthwhile Consideration” . Short Curriculum . Long Curriculum Lyons T et al JAAGL 2001;8(4):542
17 JOIN US AT NASPAG
. Philadelphia, PA . April 24-26, 2014 . www.NASPAG.org
18 Disclosure Minimally Invasive Surgery in Consultant: Conceptus Incorporated, the Pediatric and Adolescent Ethicon Endo-Surgery, Hologic Patient
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
GYN Resident Experience
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
19 CREOG Objectives CREOG Objectives
Pediatric and Adolescent Gynecology Procedure Understand Understand and –“Understand the medical and surgical Perform treatment of pediatric gynecologic disorders” Laparoscopic X Hysterectomy TLH or –“Describe appropriate medical and surgical LSH Laparoscopy, diagnostic X treatments for patients with developmental and/or operative anomalies” Lysis of adhesions X –“Treat adolescent gynecologic disorders laparoscopic medically or surgically” (but only ovarian
diseases and masses, endometriosis) CREOG Educational Objectives Core Curriculum in Obstetrics and Gynecology, CREOG Educational Objectives Core Curriculum in Obstetrics and Gynecology, Tenth Edition, 2013 Tenth Edition, 2013
Point to Remember
In terms of surgery, especially endoscopic surgery:
Children are not “little adults”
Fundamentals Peritoneal Enty
• Optimal surgical outcome depends on the The sine qua non of laparoscopy surgeon’s knowledge of – Anatomy If you can’t safely enter the peritoneum, • Intimate, “autonomic” familiarity of pathologic conditions you can’t do ANYTHING and relevant anatomic structures – Technology If you can safely enter the peritoneum, you • TOTAL understanding of the surgical instruments can do EVERYTHING – Electromechanical principles – Troubleshooting ANY malfunction – Technique • Tissue handling • Visual and proprioceptive coordination
20 Challenges to Peritoneal Extent of the Problem Access ~ 4 million laparoscopies per year in the Childhood Obesity U.S. Previous abdominal surgery 0.5 - 3 percent of laparoscopic – Previous pediatric surgery procedures have complications related to – Previous laparoscopy !! peritoneal entry Adhesions to the umbilical undersurface occur Number of complications = ~ 60,000 per in 21.2% of adult patients who have year undergone a prior laparoscopy through an umbilical incision; 10.8% in children
Chapron C, Querleu D, Bruhat MA, et al: Surgical complications of diagnostic and operative Sepilian V Ku L, H, Liu C.Y., Phelps J “Prevalence of Infraumbilical Adhesions gynecological laparoscopy; a series of 29966 cases. Hum Reprod13(4):867–872, 1998 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
Decision Tree Consensus Guidelines
Umbilicus or Alternative Site? Middlesbrough Consensus - International Elevate abdominal wall? Collaborative Group met in 1999 – Hand elevation or towel clips? – Basic guidelines are still followed today1 Veress needle or Direct Trocar Entry? Bladed or Bladeless Trocar? Council of the Society of Obstetricians and 2 Optical trocar or non-optical trocar? Gynaecologists of Canada
1 Gynaecological Endoscopy, Volume 8, Issue 6 (p 403-406) 2 Vilos GA et al, J Obstet Gynaecol Can 2007;29(5):433–447
Umbilicus Safe Veress Needle Entry Comparison of elevation of the abdominal Thinnest point on abdominal wall wall Overlies vital bowel and vascular – Hand elevation structures – Towel clips placed 2 cm on either side of Frequent site of umbilical hernias with umbilicus hernia sacs and/or bowel contents – Towel clips placed at the edge of the Adhesions from prior surgery umbilicus Intraumbilical 2 cm lateral Hand elevation
Mean distance (cm) between parietal 6.85.143.5 peritoneum and underlying P<0.01 structures
Roy GM et al,.Safe Technique for Laparoscopic Entry into the Abdominal Caviry. J Am Assoc Gynecol Laparosc 8(4):519–528, 2001
21 2cm 2cm 3+cm 1+cm
Roy et al / JAAGL 2003 Roy et al / JAAGL 2003
Alternative Sites of Insufflation
Transuterine
Cul-de-sac
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 No 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 change! 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. Roy et al / JAAGL 2003
Primary Port Placement Basic Trocars
Optical entry Open entry Palmer’s Point
4 FB
Palmer R. Safety in laparoscopy. J Reprod Med 1974;13:1–5.
22 You’ve picked the site – now Optical Trocar Entry what? Critical principles: – Force of entry into abdominal wall 4-6 kg in reusable trocars 2-3 kg in shielded bladed retractable trocars and bladeless trocars1 – Insufflate to desired Pressure NOT Volume Increase to 20 – 25 mm Hg until all ports are placed, then reduce to 15 mm Hg2,3
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.
Caveat: Trauma to Ilioinguinal Secondary Port Placement and Iliohypogastric Nerves 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
Whiteside JL, Barber MD, Walters MD, Falcone T. Anatomy of ilioinguinal and iliohypogastric nerves in relation to trocar placement and low transverse incisions Am J Obstet Gynecol. 2003 Dec;189(6):1574-8
Combined View Operative Procedures
Open laparotomy Minimally invasive procedures – Laparoscopy – Hysteroscopy – Vaginoscopy
23 Gynecological Operations Gynecological Operations
Congenital Abnormalities Congenital Abnormalities Foreign body Foreign body Trauma Trauma Ovarian cysts Ovarian cysts Pelvic Pain/Endometriosis Pelvic Pain/Endometriosis Malignancies Malignancies Pelvic Inflammatory Disease Pelvic Inflammatory Disease Ectopic pregnancies Ectopic pregnancies
Congenital Abnormalities
Septae Duplications and defects of fusion Dysgenetic ovaries
Septate Uterus
Historical repair – Strassman procedure – Tompkins metroplasty Hysteroscopic management – Blind division with scissors – Lasers – Monopolar cautery in hypotonic solution – Bipolar cautery in normal saline
24 Duplications
Didelphys Defects of fusion – lateral – vertical Rudimentary horns – communicating – noncommunicating
Didelphys with Obstructed Uterus Didelphys Hemivagina
OHVIRA OHVIRA
25 OHVIRA Didelphys and Cervical Agenesis
Cloaca
Principles of Resection Meyer-Rokitansky (MRKH)
Preoperative radiologic evaluation Vaginal agenesis Laparoscopy/hysteroscopy/vaginoscopy Variable development of internal genitalia Adequate dissection to isolate blood Problems if viable endometrium – supply obstruction Midline plane MRI insufficient for diagnosis – need Proper instrumentation to insure minimal laparoscopy collateral tissue injury Port placement and number
26 Gynecological Operations
Congenital Abnormalities Foreign body Trauma Ovarian cysts Pelvic Pain/Endometriosis Malignancies Pelvic Inflammatory Disease Ectopic pregnancies
27 Gynecological Operations
Congenital Abnormalities Foreign body Trauma Ovarian cysts Pelvic Pain/Endometriosis Malignancies Pelvic Inflammatory Disease Ectopic pregnancies
Ovarian Cysts Functional Cyst
Functional Hemorrhagic Corpus Luteum Non-functional – Benign – Malignant
28 Torsion
Para-ovarian cysts Para-ovarian cysts
29 Benign Neoplasms
Dermoids
Tendency to leak, especially if thin, attenuated cyst wall Copious irrigation Watch for bilaterality Negligible risk of complications if spill occurs1
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
30 The Problem 40 35 The vast majority of adnexal masses are 30 benign 25
20 The vast majority of adnexal masses Cystectomy Oophorectomy treated by gynecologists result in 15 preservation of the ovary 10
The vast majority of ovarian masses 5 treated by pediatric surgeons end up with 0 salpingo-oophorectomy Benign Torsion Malignant Prenatal Tumors Tumors Cysts
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
Gynecological Operations
Congenital Abnormalities Foreign body Trauma Ovarian cysts Pelvic Pain/Endometriosis Malignancies ENDOMETRIOSIS Pelvic Inflammatory Disease Ectopic pregnancies
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 Gynecological Operations Sexually Transmitted Disease
Congenital Abnormalities Persistent vaginal discharge Foreign body Absence of foreign body Trauma Inconsistent history of sexual abuse Ovarian cysts Often negative cultures in ER or referring Pelvic Pain/Endometriosis physician’s office Malignancies Look for trauma to hymen, fourchette, but Pelvic Inflammatory Disease may be absent Ectopic pregnancies
Gynecological Operations
Congenital Abnormalities Foreign body Trauma Ovarian cysts Pelvic Pain/Endometriosis Malignancies Pelvic Inflammatory Disease Ectopic pregnancies
32 Remember
Children are not “little adults” They require special techniques and instrumentation
Laparoscopic Equipment Emergency Situations
Never need more than 5 mm scope Ectopic pregnancy 3 mm and 5 mm ports Pelvic inflammatory disease Special insufflation requirements in Uncontolled menorrhagia children less than 6 years old Undiagnosed vaginal bleeding Consider equipment for heating and – Sexual abuse humidifying insufflated environment – Foreign body Adhesion prevention after ALL non- infected procedures
Exception
Associated anomalies or medical Objective conditions that prohibit fertility – Congenital heart disease – Profound retardation – End-stage renal disease Maintain Reproduction – Acute life-threatening medical conditions Function
33 Summary
Minimally invasive surgical techniques are within the grasp of all pediatric and adolescent gynecologists It is not enough to have the proper instrumentation available. You must be comfortable with the use of all equipment Competent surgical team Adequate visualization KNOW YOUR ANATOMY AND EMBYROLOGY
34 Surgical Emergencies in Pediatric Gynecology
Heather Appelbaum, MD, FACOG Associate Professor, Hofstra NSLIJ School of Medicine Chief, Division of Pediatric and Adolescent Gynecology Department of Obstetrics and Gynecology Long Island Jewish Medical Center Steven and Alexandra Cohen Children’s Medical Center of New York
Disclosure Vulvovaginal bleeding
I have no financial relationships to disclose.
Hormonal True precocious puberty
Dermatologic Non gynecologic • Activation of the HPO axis • Includes secondary sexual characteristics
Vaginal Bleeding • Idiopathic • Cerebral lesions • Primary hypothyroidism
Traumatic Infectious
Structural
35 Evaluation of the adolescent vulva Evaluation of the prepubertal child and vagina • Lithotomy • Manual • Frog-legged • Vaginoscopy • Knee to chest • Colposcopy • Vaginoscopy • Colposcopy • Killian nasal speculum
Prepubertal vestibule
Normal hymenal variations Normal hymenal variations
36 The hymen Estrogen effect
• Crescentic • Annular • scalloped • Redundant • Imperforate • Cribiform • Microperforate • septated
Hormonal Traumatic injuries
Dermatologic Non gynecologic • Saddle injury • Accidental penetrating injuries • Accidental vaginal insufflation injuries • Crush or shear injuries Vaginal Bleeding • Animal or human bites • Burns • Sexual abuse
Traumatic • Coital injuries Infectious • Foreign body • Self manipulation Structural
Classification for genital injuries Vulvar blood supply in female children
Genital Extent of injury injury score I Isolated genital injury distal to hymen
II Isolated genital injury including hymen
III Isolated genital injury including vagina
IV Hymenal or vaginal injury plus partial tear of anorectum
V Vaginal injury plus complete tear of anorectum
Onen A, et al. Genital trauma in children: classification and management. Urology 2005; 65(5): 987
37 Mechanism of traumatic genital injuries Animal or human bites Straddle injuries Soft tissues of the vulva are compressed between an object and the boney pelvis resulting in ecchymoses, abrasions, laceratations, or hematomas • Irrigate with 1% povidine-iodine solution Accidental penetrating injuries Piercing of the vulva, vagina, urethra, bladder, anus, or rectum with a • Wound debridement sharp or pointed object • Antibiotic prophylaxis Accidental vaginal insufflation injuries • Tetanus vaccine for mammalian bites Pressurized water enters the vagina causing over distension of the vaginal walls resulting in tearing • Rabies immunization
Crush or shear injuries Crush injuries can result in fragments of bone penetrating into the vagina or lower urinary tract; shear injuries can result from rapid abduction of the lower extremities
Burns Assessment of vulvar injury
• Accidental • History • Inflicted • Chemical • Examination • Valsalva • Vaginal lavage • 2% lidocaine jelly
• Cool for 20 minutes with cold tap water • Vaginal lavage
Laceration Management of vulvar hematoma
• Exam under • Ice packs anesthesia • Bed rest • Possible diagnostic laparoscopy or • Foley catheter exploratory • Evacuation laparotomy • Gelfoam, Surgicel • Electro or chemical cautery • Suturing
38 Time required for nonhymenal Ecchymoses genital injuries to heal Type of injury Time of resolution Abrasions 2-3 days Edema 5 days Ecchymoses 2-18 days Labial hematoma 2-4 weeks Petechia 24 hours Blood blisters 30 days Superficial laceration 2 days Deep lacerations Surgical repair
McCannJ et al. Healing of nonhymenal genital injuries in prepubertal and adolescent girls; a descriptive study. Pediatrics 2007;120(5):1000-11.
Physical findings suggestive of sexual abuse Normal variants Highly suspicious findings Periurethral bands Deep posterior hymenal notching Intravaginal ridges Ano-genital warts Hymenal bumps/tags Vesicular lesions or ulcers Anterior hymenal notching 2cm anal dilation Septate hymen Urethral dilation with labial traction Acute laceration or ecchymosis Absence of posterior hymenal tissue Non-specific findings Culture confirmed STI Erythema of the vestibule Pregnancy Increased vascularity of vestibule Sperm Labial adhesions Friability of posterior fourchette Excoriations Petechiea Anal fissures
American Academy of Pediatrics Committee on Child Abuse and Neglect Forensic Evidence Collection Guidelines for the evaluation of sexual abuse of children • Clothing and debris collected • Head and pubic hair combings History Exam Labs Level of Reporting concern • Describe hematomas, ecchymoses, lacerations None Normal None None None • Scrape or swab dried secretions Behavior Normal None Variable Possibly • Bluemaxx lamp detection of semen None Non-specific None Low Possibly • Fingernail scrapings Nonspecific Nonspecific None Intermediate Possibly • Wet prep and urine for sperm, trichomonads • Serology for syphilis, HIV, hepatitis B Clear Normal None High Report • Vaginal, rectal and throat cultures none Specific None High Report • Urine or blood toxicology None Nonspecific Positive High Report
39 Prophylactic treatment for alleged Hormonal acute sexual assault Dermatologic Non gynecologic • Ceftriaxone 125mg IM • Metronidazole 2g PO
• Azithromycin 1g PO Vaginal Bleeding • Hepatitis B vaccine • Tetanus toxoid
• Levonorgestrel 0.75mg PO q12h X 2doses Traumatic Infectious
Structural
Foreign body Tumors
• Cervical or vaginal polyps • Hemangioma • Urethral prolapse • Sarcoma botyroides
40 Urethral prolapse
• Painless bleeding vulvar nodule • Caused by repetitive valsalva maneuvering • Treat with topical estrogen cream • Do NOT biopsy!
Hormonal
Dermatologic Non gynecologic
Vaginal Bleeding
Traumatic Infectious
Structural
Lichen sclerosis
• White, atrophic, parchment like skin, subepithelial hemorrhage
• Loss of demarcation of the labial structures, scarring of the clitoral hood, hour glass configuration involving the anus
• Symptoms including pruritis, bleeding, soreness, irritation, dysuria, dyschezia, constipation
• Treat with clobatosol 0.05% ointment bid
• 5% lifetime risk of squamous cell carcinoma
41 Vulvar ulcers Vulvovaginitis
• Viral syndrome • Poor hygiene • Beçhets • Vagina and anus in close proximity • Crohn’s disease • Lack of protective hair • Infectious ulcers • lack of estrogenization • Urinary reflux
Assessment and treatment for Staph infections vulvovaginitis • Vulvovaginal culture • Wet mount • Augmentin 40mg/kg/d divided tid X 10days • Clindamycin 25mg/kg/d divided tid X 10days
Candida Hormonal
Dermatologic Non gynecologic
Vaginal Bleeding
Traumatic Infectious
Structural
42 Estrogen withdrawal bleeding in the Endometrial response to estrogen neonate
80 70 60
vaginal 50 soy maturation 40 cow index (%) 30 breast 20 10 0 0 30 60 90 120 180 age (days)
Bernbaum JC, et al. Pilot studies of estrogen-related physical findings in infants. Environmental Health Perspectives: 2008
Physiologic neonatal ovarian cysts True precocious puberty
• Functional neonatal • Activation of the HPO axis ovarian cysts develop in utero in response • Includes secondary sexual characteristics to maternal hormones • Idiopathic • Clinically significant ovarian cyst occur in • Cerebral lesions 1/2,500 live female • Primary hypothyroidism births • Rupture can result in estrogen withdrawal bleeding
Precocious pseudopuberty Normal menstrual cycle • Estrogen causes proliferation of the • Gonadotropin-independent endometrium • Progesterone stabilizes the • Ovarian tumors endometrium • Adrenal tumors • Hormonal withdrawal causes lysosomal • Congenital adrenal hyperplasia membrane destabilization and leakage • McCune-Albright syndrome • Prostaglandins, proteases, and collagenases cause • Exogenous estrogens tissue breakdown and • Premature menarche vasoconstriction of blood vessels • Thrombin plugs spontaneously form
43 Dysfunctional uterine bleeding: Diagnostic tools The hormonal imbalance of adolescence
Estrogen breakthrough bleeding • History and physical exam • Estrogen causes proliferation of • ßHCG the endometrium • CBC with platelets • Anovulation results in persistent •PT/PTT estrogen and excessive growth of the endometrium • Liver and renal function tests • TSH/Prolactin • Disorganized destabilization of the endometrial lining • pelvic ultrasound
• Dysfunctional bleeding
Estrogen breakthrough bleeding
Dysfunctional uterine bleeding • Anovulation • Continuous OCPs • Estrogen breakthrough bleeding • Estrogen withdrawal bleeding • Progesterone breakthrough bleeding • Progesterone withdrawal
Treatment for dysfunctional Anovulation bleeding • Adolescence • Consider blood transfusion • Obesity • Short term estrogen therapy • Polycystic ovaries • Congenital adrenal hyperplasia – high dose conjugated estrogen 25mg IV q4h • Hypothalamic dysfunction X 24hours OR • Hyperprolactinemia – Conjugated estrogen 1.25mg or 2.0mg po qd • Hypothyroidism X 7days • Medications – OCP taper 4X4, 3X3, 2X2, 1X1 • Withdrawal • Maintain on OCPs or cyclic progestins
44 Dysfunctional uterine bleeding: Estrogen withdrawal The hormonal imbalance • Estrogen breakthrough bleeding • Metabolism of maternal hormones • Estrogen withdrawal bleeding • Ruptured ovarian cyst • Progesterone breakthrough bleeding • Missed OCPs • Progesterone withdrawal
Treatment for estrogen Dysfunctional uterine bleeding: withdrawal The hormonal imbalance • Add back estrogen • Estrogen breakthrough bleeding – high dose conjugated estrogen 25mg IV q4h • Estrogen withdrawal bleeding X 24hours OR • Progesterone breakthrough bleeding – Conjugated estrogen 1.25mg or 2.0mg po qd OR • Progesterone withdrawal – low dose estrogen 0.625mg po qd • Progestin maintenance or combined estrogen/progesterone maintenence
Treatment for progesterone Dysfunctional uterine bleeding: breakthrough bleeding The hormonal imbalance • Estrogen therapy • Estrogen breakthrough bleeding – Conjugated estrogen 1.25mg or 2.0mg po qd • Estrogen withdrawal bleeding OR • Progesterone breakthrough bleeding – low dose estrogen 0.625mg po qd • Progesterone withdrawal • Progestin • Withdrawal • Monophasic 30-35µg OCPs maintenecnce
45 Progesterone withdrawal Alternate treatments for bleeding menorrhagia • Normal menstrual cycle • Plasminogen activator inhibitors reduce • Provera challenge menstrual blood flow by 40-50% •OCPs • Hemostatic agents that correct abnormal platelet function reduce capillary bleeding • Levonorgesterol IUD • Antifibrinolytics
Hormonal Pregnancy Dermatologic Non gynecologic • Spontaneous abortion • Incomplete abortion Vaginal Bleeding • Threatened abortion • Ectopic pregnancy • Subchorionic hematoma Traumatic • Implantation Infectious
Structural
Systemic diseases Bleeding dyscrasias
• Blood dyscrasias • Von Willebrand’s disease • Liver disease • Prothrombin deficiency • Chronic renal disease • Platelet aggregation disorders • Systemic lupus • Leukemia erythematosus • Idiopathic thrombocytopenic purpura • endocrinopathies • hypersplenism • Infection
46 Medications contributing to Screening for coagulation disorders abnormal uterine bleeding • History • Anticoagulants • Physical exam •CBC •Steroids • Platelet count • Hormonal therapy • Complete metabolic panel • Herbal medications/natural supplements • Fibrinogen level • Platelet function •PT/PTT • Factor VIII level, vWF level, and ristocetin cofactor
Hemorrhagic cyst
Dermoid cysts Paraovarian cyst
Adnexal masses Adnexal masses
Adnexal torsion Malignancy
Simple cyst
Hemorrhagic cyst Twisting of the ovary on its
Dermoid cysts ligamentous supports results in Paraovarian cyst impedance of blood supply
Adnexal masses
Adnexal torsion Malignancy
Simple cyst
47 Factors predisposing to adnexal Ovarian and fallopian tubal torsion in children torsion
• Ovarian masses • 94% of cases associated with ovarian cyst or neoplasm, although normal ovaries may also tort • Elongated utero-ovarian ligament in prepubertal girls • May be associated with strenuous exercise or sudden increase in abdominal pressure • Persistant neonatal ovarian cyst • Neonates present with abdominal mass, feeding • Müllerian anomalies intolerance, vomiting, abdominal distension and irritability
Incidence and Trends of Pediatric Ovarian Symptoms associated with Torsion Hospitalizations in the US, 2000-2006 Guthrie, B., Adler, M, Powell, E. Pediatrics. 2010:125;532-538 ovarian torsion • Incidence of ovarian torsion in age group 1-20 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%) • 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 Transabdominal pelvic ultrasound ovarian torsion • Peripheral follicles with stromal edema
• Heterogenously enlarged ovaries
• Free fluid in the cul-de-sac
• A ratio of torsed adnexal volume to the normal adnexal volume greater than 20 is predictive of a mass inside the ovary
• Color flow Doppler can be appreciated in a torsed ovary
• Color flow Doppler can be absent in normal ovaries
48 The role of inflammatory Ovarian torsion markers in ovarian torsion
70 • Surgical emergency to preserve ovarian 60 functioning 50 pg/dl 40 P<0.001 Torsion • Must have high clinical index of suspicion 30 No torsion in patients with acute and/or intermittent, 20 variable abdominal pain and 10 nausea/vomiting 0 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
Surgical management of ovarian torsion • Diagnostic and therapeutic laparoscopy • Exploratory laparotomy What should you do with the • Ovarian preservation • Detorsion of ovary purple, black, and ugly ovary? •Cystectomy • Detorsion with second procedure cystectomy • Cyst aspiration • Ovarian bivalving • Oophoropexy • Oophorectomy
Assessing ovarian viability
• Color flow Doppler is not a reliable measure of DETORT THE OVARY! ovarian viability • Leukocytosis, fever, and signs of peritonitis may indicate irreversible damage to the ovary
• Macroscopic appearance is not a good indicator of viability
49 How long is too long to wait to Detorsion of the Ovary detorse the ovary? Study % recovery of ovarian function • The adnexa of rats were twisted for 36 hours or Oelsner et al, (1993) 91% (85/92) until they became bluish-black in appearance Mage et al., (1989) 94% (16/17) Shalev et al. (1995) 94% (49/52) Rody et al, (2002) 100%(1/1) • All ovaries that were torsed under 24 hours Aziz et al, (2004) 100%(14/14) showed no immediate or delayed evidence of Celik et al, (2005) 92%(13/14) Rousseau et. al. 100%(19/19) necrosis on histologic evaluation Levy et al. 100% (3/3) Pansky et al 88% (7/8) • Ovaries torsed for 36 hours showed immediate Cohen et al (1996) 100% (7/7) TOTAL 93% (211/227) 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
Is there a role for post operative ultrasound • Rule out malignancy How do we prevent • Assess viability recurrence?
• Serial ultrasounds are not indicated
Oophoropexy Oophoropexy
Pros Cons • Utero-ovarian ligament at the ovarian • May prevent recurrent torsion • Theoretical risk of impaired insertion is attached to the ipsilateral of a detorsed ovary blood supply uterosacral ligament using permanent • May prevent torsion with • Theoretical risk of peritubal polycystic ovaries adhesions suture • Elongated utero-ovarian • Insufficient data on future • Plication of the utero-ovarian ligament ligaments likely pose a higher fertility functioning risk of recurrence • Ovary can be sutured to the pelvic • May be prophylactically useful sidewall for the contralateral ovary following unilateral oophorectomy
50 Ovarian torsion key points
• Early intervention for ovarian torsion results in preservation of ovarian function, despite the gross appearance of the ovary
• Ultrasonographic appearance of the ovary is a useful Obstructed Müllerian tool for managing ovarian torsion, but color flow Doppler is not reliable Anomalies
• Oophoropexy of the detorsed adnexa or the contralateral ovary may be appropriate
Imperforate hymen Transverse Segmental vaginal septum vaginal agenesis Cyclic abdominal pain and Obstructed Müllerian Anomalies Pelvic Mass in Adolescents Think Mullerian Anomaly Functional rudimentary uterine horn OHVIRA
Cervical agenesis
Developmental embryology of Müllerian 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 and upper vagina.
51 Cyclic pelvic pain and Cyclic pelvic pain with normal amenorrhea menses • Imperforate hymen OHVIRA Functional • Uterine didelphys Rudimentary horn • Unicornuate uterus • Transverse vaginal septum • Obstructed hemivagina • Contralateral cervical • Ipsilateral renal and vaginal agensis • Segmental vaginal agensis agenesis
Obstruction to menstrual egress What should you do?
• Pelvic pain Do Don’t • Suppress menses • Perforate the dilated structure • Hematometria/hematocolpos • Provide analgesia • Decompress the • Attempt drainage bladder for urinary • Operate before the • Pyometria/pyosalpinx retention anatomy is clearly • Refer to specialist for defined surgical intervention • Endometrioisis
52 Endometriosis in Adolescents – Disclosure
The Beginning of the Story I have no financial relationships to disclose. Marc R. Laufer, M.D. Chief of Gynecology Children’s Hospital Boston Center for Reproductive Medicine Brigham & Women’s Hospital Director, Boston Center for Endometriosis Harvard Medical School
Objectives 15 year old with pelvic pain
• At the conclusion of this presentation the • Pain for 6 months • Seen in Gyn 2 days after participant will be able to: • Worse with period EW visit • Asked to start OCP for – identify common adolescent endometriosis lesions • Missing school • Called your office with dysmenorrhea and – Recommend treatment options for adolescents with increased pain ovarian cyst suppression endometriosis • Sent to EW and found to • US 4 weeks later: normal have 6cm ovarian cyst • Pain persists • Asked to follow up in Gyn
Medical Management of Pelvic Dysmenorrhea Pain/Dysmenorrhea
• Accompanies 20 - 90% of adolescent menstrual • Combined oral contraceptives (COC’s) cycles – Numerous overall benefits • Dysmenorrhea that significantly interferes with • Progestin-only OC’s may also be beneficial is function 1 - 3 days/month affects 5- 42% of special circumstances adolescents • If there is a persistence of pain on COC and • More likely be primary than secondary in origin NSAIDS then we need to proceed with a diagnosis
53 Don’t “normalize” symptoms
How do we decide when it is no longer • Dysmenorhea that fails to respond to dysmenorrhea and further evaluation conventional treatment is not a “normal” is required? occurrence • Women should not be led to believe that they simply have to endure pelvic pain because it is their lot in life to do so.
A young woman’s dreams Costs of Endometriosis
• Yearly total (direct & indirect) – Europe: 30 billion Euros – US: 22 billion dollars
Gao X, Outley J et al, Economic burden of endometriosis. Fertil Steril 2006;86:1561. Simoens S, Hummelshoj L, D’Hooghe T. Endometriosis: cost estimates and methodological perspective. Hum Reprod Update 2007;13:395. Fourquet J, Gao X, et al. Patients’ report on how endometriosis affects health, work, and daily life. Fertil Steril 2010;93:2424.
Data From the International Endometriosis: Etiologies Endometriosis Association Ballweg ML. J Pediatr Adolsc Gynecol 2003;16:S21-S26. • Sampson’s theory: retrograde menstruation • Hematogenous and/or lymphatic spread of endometrial • Registry of 4000 women with endometriosis • Most women see approximately 9 health care providers tissue prior to diagnosis • Metaplastic transformation • Most women have had symptoms that they associate • Immune system abnormality to ectopic endometrium with endometriosis since adolescence – 21% had severe pain under age 15 • Environmental exposures [Dioxin] – 17% age15-19 • Genetic predisposition – 12% age 20-24 – 50% 24 or younger
54 Endometriosis: Symptoms Endometriosis: Co-Occurrences
• None • Interstitial Cystitis • Pain: dysmenorrhea; localized pain; dyspareunia; • IBS noncyclic pain • Kidney/uteteral stones • GI: constipation, diarrhea, hematochezia, melena • Temporo-mandibular disorders • GU: hematuria, dysuria, frequency, urgency • Migraines • Pelvic Mass/Cyst [not usually seen prior to age 22] • Fibromyalgia • Subfertility/Infertility: Stage I to Stage IV • Vulvodynia
GI=gastrointestinal GU=genitourinary
Adoelscent Endometriosis: Adolescent Physical Exam Diagnosis
• What is the goal? – Rule out: • No diagnostic blood test • Obstructive anomaly • Ovarian mass • No diagnostic imaging study • Bimanual • History – May not be possible • Q-tip test • Physical Exam- A pelvic exam should not be • Recto-abdominal barrier for evaluation [patency, ? Mass] • Unlikely findings – Uterosacral nodularity • Trial of NSAIDs & CHT – Endometrioma • Laparoscopy for diagnosis AND surgical management
Incidence of Endometriosis Characteristics of Subjects With & Without in Adolescents With Pelvic Pain Diagnosed by Endometriosis
Laparoscopy (Laufer MR, Goitein L, Bush M, Cramer DW, Emans SJ. Prevalence of endometriosis in adolescent women with chronic pelvic pain not responding to conventional therapy. J Pediatr Adolesc Gynecol 1997;10:199-202.)
• 67% – Rock (1996) Patients with Endometriosis • 69% – Laufer (1997)* Characteristics N=32, Number (Percent) • 97% – Laufer (2012) Presenting Symptoms Acyclic and cyclic pain 20 (62.5) Acyclic pain 9 (28.1) Cyclic pain 3 (9.4) *data from early ’90s, optics now better, ie higher rates Gastrointestinal pain 11 (34.3) Urinary symptoms 4 (12.5) Irregular menses 3 (9.4) Vaginal discharge 2 (6.3)
55 Data From the International What’s the delay? A qualitative study of women’s experiences of reaching a diagnosis of Endometriosis Association endometriosis Ballweg ML. J Pediatr Adolsc Gynecol 2003;16:S21-S26. Ballard K, Lowton K, Wright J Fertil Steril 2006;86:1296-301. • Registry of 4000 women with endometriosis • Most women see approximately 9 health care providers prior to diagnosis • Significant delay in diagnosis [11.8y in US, 6.7 in UK] • Most women have had symptoms that they associate • Diagnostic laparoscopy after failure of first line with endometriosis since adolescence – 21% had severe pain under age 15 • Women suffer at physical, emotional and social – 17% age15-19 levels when they remain undiagnosed – 12% age 20-24 – 50% 24 or younger
Patients’ report on how endometriosis affects health, work, and daily life Fourquet J, Gao X, et al. Fertil Steril 2010;93:2424-8.
• 108 patients in Puerto Pico • Majority had symptoms starting 11-19 year of age There is no correlation between • Mean delay in diagnosis was 8.9 years amount of disease and pain. • Many patients consulted 5 or more physicians prior to diagnosis • Endometriosis impairs health-related quality of life
ASRM Staging Relates to Fertility Staging and Pain Are Unrelated Potential, Not Pain
Incidence: • Stage I: 40% • Stage II: 24% • Stage III: 24% • Stage IV: 12%
Fedele, et al. Stage and localization of pelvic endometriosis and pain. Fertil Steril. 1990;53:155-158.
56 Endometriosis in adolescents is a hidden, progressive and severe Endometriosis: disease that deserves attention, not just compassion Brosens I, Gordts S, Benagiano G. Hum Reprod 2013; 28:2026-31. Types of Lesions and Pain Author Year Subjects Age Range St I % St II % St III % St IV % Goldstein et al 1980 66 10-19 58 38 0 4 Vercellini et al 1989 18 11-19 67 33 0 0 • Clear 76% Davies et al 1993 36 13-20 28 22 19 31 • Red 84% Reese et al 1997 49 11-19 80 12 6 2 Laufer et al 1997 32 13-21 77 23 0 0 • White 44% Emmert et al 1998 37 11-19 92 8 0 0 Bai et al 2002 39 14-21 10 44 28 18 • Black 22% Ventolini et al 2005 28 12-18 14 39 43 4 Stravroulis et al 2006 11 13-20 45* 55^ • Pain perception 1-27 mm from lesion Vicino et al 2010 38 15-21 18 13 34 34 Roman 2010 20 14-20 40 45 5 10 Yang 2012 63 12-20 8 3 52 37 Demco L. JAAGL. 1998. *=St 1&2, ^=St 3&4
Immune Dysregulation and Inflammation in Endometriosis
Protective Immunity Chronic Inflammation Tariveridan N, Theoharides TC, et al. Semin Immunopathol 2007;29:193-210. American Society for Reproductive Medicine. Fertil Steril 1997;67:817.
Different appearances of endometriotic implants
57 Surgical Approaches Identification of Clear Lesions Laufer MR. Fertil Steril 1997;68:739-40. • use of liquid medium to improve visualization • Laparoscopy [laparotomy is not a failure] • improve magnification • Open laparoscopy vs. direct insertion • 3-dimensional effect • Resection or Ablation • Laser, scissors, harmonic scalpel, electrosurgery • Hysterectomy • Hysterectomy with bilateral oophorectomy
Surgical Excision vs Ablation Clear lesions & destruction [Wright J., Lotfallah H, et al. Fertil Steril 2005;83:1830-6.]
• Randomization at initial laparoscopy for CPP if Stage I or II diagnosed: ablation with cautery or excision • 24 patients with 12 in each group with 6 month follow up • Significant improvement in pain in both groups • No difference in either group • 1 patient in each group had worsening of symptoms
58 Plaque lesions & destruction Surgical Outcomes in Adults
Now we have published follow up in adolescents!
Medical Treatment for All Patients Medical Treatment Options in Adults • No surgical cure of endometriosis • Estrogen/Progestin • Treatment until childbearing is • Continuous [OCPs/Ring] complete or fertility is no longer • Progestins desired • Depo-Provera • Limit the life-time surgical • Aygestin [5-15mg/day] procedures • Danazol • Hysterectomy should not be a • GnRH agonists with add-back recommendation for an adolescent • Aygestin [5mg] • PremPro [.625/2.5] • Do Not Use OCPs for Add-back • GnRH-antagonists with add-back • Anti-estrogens or anti-progestins
Continuous Combination Progestins and Endometriosis Hormonal Therapy
• +/- ovulation inhibition • Decidualization • Decreased menstrual flow • Formulations – progestin only pill – Norethindrone acetate [5-15mg] Monophasic progestin dominant pill-21 – Depo-provera Disp# 4 packs [3 months] Sig: 1 po q D without breaks or placebos for Rx of endometriosis
59 Use of norethindrone acetate alone for postoperative suppression of endometriosis symptoms Kaser DJ, Missmer SA, Berry KF, Laufer M. J Ped Adol Gyn 2012;25:105-8. Results
• 194 patients with surgically confirmed endometriosis • 55.2% had no SE • Median age 18.9 years [10.2 – 41.9] • 92.2% had Stage I or II disease –16.1% wt gain • Pain 0-10 severity scale –9.9% acne • Bleeding 0-4 –8.9% mood swings 0 = no bleeding, 1 = spotting, 2 = irregular bleeding once monthly, 3 = weekly bleeding, 4 = daily bleeding • 64.7% reported lower pain scores • FDA approved dose: 5-15mg • 58.1% reported reduced bleeding • Self-titrate to amenorrhea and decreased pain
Bone Density in Adolescents Treated with a GnRH Agonist and Add-Back Therapy for Endometriosis DiVasta AD, Laufer MR, Gordon CC J Pediatri Adolesc Gynecol 2007;20:293-7.
• BMD at the hip normal in most adolescents with endometriosis receiving leuprolide and add-back therapy with norethindrone acetate Outcomes in Adolescents • 1/3 of subjects had clinically significant skeletal deficits (Z-score ≤ -1.0) at the spine – Greater than expected in normal population – Likely due to GnRH-a induced low-estrogen state • Duration of treatment not associated with BMD at hip or spine
The Effect of Standard Surgical-Medical Intervention on the Progression of Endometriosis Necessity of Post-op medication? Doyle JO, Missmer SA, Laufer MR. J Pediatri Adolesc Gynecol, 2009;22:257. Yeung P, Sinervo K, et al. Fertil Steril, 2011;95:1909-1912.
• 90 patients age 12-24 • 20 prospectively enrolled ages 12-19 • Laparoscopy for diagnosis & surgical treatment – Pre-op questionnaire • Medical treatment – Laparoscopy with “complete excision of endometriosis” – Continuous E/P [91%] – F/u questionnaire @ 1-2 yr intervals (median f/u 23 months) – LA + NA or Prempro [78%] – P only [12%] • No specific post surgical recommendation made to • 2nd laparoscopy due to pain participants regarding hormonal therapy • Trend towards disease progression not seen – May use for contraception or suppression “patient's choice” • Standard surgical-medical management retards disease progression
60 Results Conclusions • Author’s conclusions • 6/17 subjects OCP – “Careful/complete excision of endometriosis” – Duration 10-22 months improves quality of life • 1/17 subjects utilized GnRHa – Duration 6 months – Pain relief from surgery persist without medical • 8/17 subjects had recurrent therapy (up to 2 years) pain and underwent repeat laparoscopy. No • Problems: endometriosis appreciated – Pain persisted in 50% of patient regardless of [visually or histologically] operative findings – 50% of patients required a 2nd operation within 2 years due to pain – Does not address fertility concerns
Adolescent Endometriosis Management Endometriosis in adolescents is a hidden, • Need for Diagnosis/surgery progressive and severe disease that deserves – ACOG: “ After a comprehensive evaluation and and attention, not just compassion adequate trial of hormone therapy and NSAIDs, Brosens I, Gordts S, Benagiano G. Hum Reprod 2013; 28:2026-31. laparoscopy should be offered for diagnosing and treating presumed endometriosis” • Importance of early diagnosis • Surgical Treatment: excision/ablation of all visible lesions • Early intervention to avoid progression – Importance of adolescent lesions • Since there is no cure for the disease, post- operative medical therapy until child bearing is complete
Boston Center for Endometriosis • Biorepository and Clinical Database – $3 million gift - J. Willard & Alice S. Marriott Foundation – Additional $1.5 million for research • Development of a non-invasive diagnostic • New treatments on the way to a cure • Life long endometriosis data base from adolescence through adulthood [BCH & BWH] – Genetic predisposition, Environmental exposure, Diet, Long term follow up [% need for additional surgery, future fertility]
61 Current Research: Biorepository Clinical Data Base • Collect blood, urine, saliva, fluid from the pelvic cavity, and tissue from adolescents and adult women • Life long endometriosis data base with endometriosis – Genetic predisposition • “Fast track” evaluation and treatment • Development of a non-invasive diagnostic • How should we manage the daughter of a woman with a history of infertility, • New treatments on a way to a cure Stage IV endometriosis, and no pelvic pain? – Associated diseases – Environmental exposure – Long term follow up [% need for additional surgery, future fertility]
The Importance of Awareness Education and Life Long Support • If adolescent endometriosis can be kept from progressing with surgical destruction followed by medical therapy
• And if early diagnosis can save a woman’s fertility and prevent extreme physical and emotional pain
• Need to improve awareness – www.endometriosisassn.org – www.youngwomenshealth.org – www.bostoncenterendometriosis.org
Educating Professionals
www.youngwomenshealth.org
62 References Conclusions Gao X, Outley J et al. Fertil Steril 2006;86:1561. Simoens S, Hummelshoj L, D’Hooghe T. Hum Reprod Update 2007;13:395. Fourquet J, Gao X, et al. Fertil Steril 2010;93:2424. • Endometriosis does occur in adolescents Ballweg ML. J Pediatr Adolsc Gynecol 2003;16:S21-S26. Laufer MR, Goitein L, et al. J Pediatr Adolesc Gynecol 1997;10:199-202. • Early diagnosis to decrease pain & progression Reese KA, Reddy S, Rock JA. J Pediatr Adolesc Gynecol 1996;9:125. • Education and awareness is the key to decreasing pain, Ballard K, Lowton K, Wright J. Fertil Steril 2006;86:1296-301. Fourquet J, Gao X, et al. Fertil Steril 2010;93:2424-8. suffering and infertility Brosens I, Gordts S, Benagiano G. Hum Reprod 2013; 28:2026-31. Demco L. J Am Assoc Gynecol Laparosc 1998;5:241. Tariveridan N, Theoharides TC, et al. Semin Immunopathol 2007;29:193-210. American Society for Reproductive Medicine. Fertil Steril 1997;67:817. Laufer MR. Fertil Steril 1997;68:739-40. Wright J., Lotfallah H, et al. Fertil Steril 2005;83:1830-6. Kaser DJ, Missmer SA, Berry KF, Laufer M. J Pediat Adolesc Gynecol 2012;25:105-8. DiVasta AD, Laufer MR, Gordon CC. J Pediatri Adolesc Gynecol 2007;20:293-7. Doyle JO, Missmer SA, Laufer MR. J Pediatri Adolesc Gynecol, 2009;22:257. Yeung P, Sinervo K, et al. Fertil Steril, 2011;95:1909-1912.
63 Laparoscopic-assisted creation of neovagina Disclosure Surgical approach to Müllerian Anomalies and vaginal agenesis I have no financial relationships to disclose. Prof. Dr. S. Brucker
Frauenklinik Tübingen Universitäts-
Laparoscopic-assisted creation of neovagina Congenital Vaginal Agenesis
- Mayer - Rokitansky - Kuester - Hauser – Syndrom (Incidence 1: 4000 -5000) - AIS = Androgen Insensitivity Syndrom (Incidence 1: 25000)
Oppelt, Brucker: Hum Reprod. 2006 Mar
Laparoscopic-assisted creation of neovagina as complex as misleading
64 Laparoscopic-assisted creation of neovagina
Malpractice (n=163)
Imperforate hymen 15,4%
(Hymen incision 8 %)
Ovarian insufficancy 25,5%
TOTAL 40,9 %
3 „A“: asymptomatic, amenorrhoe, aplasia
Brucker et al: Geburtsh Frauenheilk 2009, 69: 568 - 575
Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina
Congenital complex malformation Different methods of creation of neovagina
Non-operative: Diagnostic: Dilatation of pseudohymenale membrane with dummies (Frank) Disadvantages: painful, duration, compliance, prolaps of neovagina History
Rectal / introitus ultrasound Operative creation of neovagina Digitale Palpation (vaginal, rectal) with transplantation - free skin / mash-graft (Mc.Indoe) Chromosome-analyses - bowl (Sigmoid, Ileum, Rectum: Schmid, Baldwin, Bryan) - peritoneum (Davydov) hormone profile Disadvantages: big operativ-technicale deal, wound healing Uro-MRT problems, shrinking, scars, need for lubrication, diag. laparoscopy smelling discharge
without transplantat: minimal-invasive surgical method
Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina McIndoe procedure with mash-graft Prolaps after self-dilatation
65 Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina
Prolaps after Sigmoid-vagina Management of vaginal agenesis (Tübingen)
Vaginal agenesis
Pre-operative Oncological procedure Operation
No Yes
Lsc.-ass. Mc Indoe Sigma Neovagina
Brucker: Geburtsh Frauenheilk 2005 Nov
Laparoscopic-assisted creation of neovagina Laparoscopic-assisted Neovagina Laparoscopic-assisted Neovagina: Application - Instruments Optimizing due to new traction device and vagino-abdominale Perforation without dissection of vesico-rectale space Thread Guide • straight Thread Guide - vagino-abdominale perforation Surgical steps • two Curved Thread Guides - peritonealization • 1.laparoscopic step: Pluggable segmented dummy Recto-vaginale palpation, simultaneouslly cystoscopy + laparoscopy • central hole for flowing off secretion (=diaphanoscopy), • determine of the exact length of the neovagina laparoscopic desicion of the perforation point • suprapubic catheter
• vaginal step: Traction dev ice perforation of vaginale membrane without dissection • no ripping off of the threads • stable direction of the tension • 2. laparoscopic step: • possibility of tighten equally both traction threads • no unintentional opening retroperitonally guidance of threads • smooth surface
• Fixation of traction device Dummies postoperative dummies, different sizes for individually use Gauwerky, Wallwiener: Arch Gynecol Obstet 1992; 252 (length 10 cm or 12 cm; diameter 2; 2,5 and 3 cm)
Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina
Brucker et al.: Geburtsh Frauenheilk 2004 / Gynecol Surg 2004
66 Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina Placement of traction device Results: optimized vs. conventional armentarium
new set old set
(n3 = 122) (n4a = 30) - ripping of threads 6 - slipping of traction device 10 -skin lesions non 5 - Luxation of dummy 0 - Dehiszenz Neovagina 1 Time of tension (days ) 4,8* 7,5*
*p = 0,0001
Infinitely variable and equally tighten of both traction threads • no tearing of the neovagina
Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina Results n=180: vagino- abdominale Perforation with vs non dissection of vesico-rectal space Long-time follow up
Results: With vrD No vrD No vrD Epithelialisation Length and sexual intercourse Old Set New Set dark: no sex, (n = 12) (n = 18) (n = 150) 1 4a 3 white: with sex 113* *p = 0,0559 93,5* *p < 0,0001 47,5* Operation Time (min) (75 – 155) (55 – 150) (20 – 114) passagere intraoperative 1 (0) 1 (0) 2 (0)
Via falsa: bladder (rectum) proportion
-Injury of A. Iliaca externa 0 0 1 length [cm] -Haematoma bladder (SPK) 0 1 2 -Bleeding after removing device 0 0 1
*p < 0,0001 4567891011 8,9 7,8 9,8 0.0 0.2 0.4 0.6 0.8 1.0 Post-op. length neovagina (cm) 13612<12 (6 -12 ) ( 6 – 12) (6 – 12,5) 13612>12 months months 11,7* 7,5* 4,8* Time of tension (Days ) (7 – 15)*p < 0,0001 (4 – 13)*p = 0,0001(2 – 7)
Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina Long-time follow up (n=135/152) Results - Sexual intercourse -
Interventionalsatisfaction Trail in Health (FSFI) Care Research Rate of regular intercourse BMBF-FKZ: 01GY1125
1
3
6 months
12
>12
0.00.20.40.60.81.0 Vaginal mucosa: normal in thickness. proportion PAS-reaction: broad intermediate cell zone with abundant cytoplasmic Satisfaction (FSFI) Sexual intercourse glycogen and a covering layer of narrow, superficial cells. no sexual intercourse IH reaction (cytokeratin 13): normal epithelial cells, squamos differentations
67 Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina
Translational research: Cause of MRKH-Syndrome Congenital malformation of the
• Detection of endometrium vagina • Defect of estrogen-receptor ( Ki 67- expression ) • Epigenetic factors: Overexpression ER-1 • labia synechia • septated hymen • imperforate hymen • vertical / longitudinal vaginal septum • vaginal aplasia - Mayer-Rokitansky-Kuester-Hauser-Syndrome -AIS
Rall, Brucker: Fertil Steril 2013 Nik-Zainal, Brucker: J Medical Genetics 2011 Brucker: Orphanet J Rare Diseases 2011
Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina Synechia of labia Congenital malformation of the vagina
• labia synechia • septated hymen • imperforate hymen • vertical / longitudinal vaginal septum • vaginal aplasia - Mayer-Rokitansky-Kuester-Hauser-Syndrome -AIS
Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina
68 Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina
Congenital malformation of the vagina
• labia synechia • septated hymen • imperforate hymen • vertical / longitudinal vaginal septum • vaginal aplasia - Mayer-Rokitansky-Kuester-Hauser-Syndrome -AIS
Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina Mucocolpos Haematocolpos
Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina Haematocolpos Congenital malformation of the vagina
• labia synechia • septated hymen • imperforate hymen • vertical / longitudinal vaginal septum • vaginal aplasia - Mayer-Rokitansky-Kuester-Hauser-Syndrome -AIS
69 Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina
Vaginal septum Haematokolpos – vertical vaginal septum
longitudinal septum reaching the Introitus problems with vaginal delivery / sex. intercourse Often combined with Uterus didelphys (Ut. bicornis, Cervix duplex) Septum in the upper third of vagina = lower birth rate (only 20%) High incidence of endometrioses due to retrograde monthly bleeding „paravaginal tumor“ due to second rudimentary vagina
Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina
Cervial aplasia and vaginal vertical septum Uterine malformations
Uterus bicornis
inhibitory malformation of muellerian duct 2 separated uteri uterus bicornis combined with cervix duplex = Uterus didelphys often combined with vaginal septum
Strassmann - metroplastic
Patients with symptoms: abortion, premature birth
Laparoscopic-assisted creation of neovagina Laparoscopic-assisted creation of neovagina
Uterus bicornis and myomectomy Cervial aplasia in 1 rudiment with haematometra
70 Laparoscopic-assisted creation of neovagina
Surgical approach to Müllerian Anomalies and vaginal agenesis
Prof. Dr. S. Brucker
Frauenklinik Tübingen Universitäts-
71 Consultant: Conceptus Incorporated, Ethicon Endo‐Surgery, Hologic
Robert K. Zurawin, MD Associate Professor Director Minimally Invasive Gynecologic Surgery Baylor College of Medicine Houston, Texas
Diagnostic tests Functional Ultrasonography Hemorrhagic Corpus Luteum If solid tumor: hCG, ‐fetoprotein, CA‐125 Non‐functional FSH, LH Benign testosterone Malignant If malignancy suspected: CXR, CT, LFT
Incidence increased due to greater detection rate Most masses are large when first detected All tumors seen in adults are seen in children BUT the because children/adolescents do not have relative incidence is different pelvic exams anovulatory cycles functional cysts Abdominal presentation due to small pelvic size Germ cell tumors > epithelial tumors Smaller lesions are usually detected only as an 1/10 ovarian tumors during childhood and adolescence incidental finding in the work‐up of pain are malignant 25‐40 percent of ovarian cancers in children are germ These are almost always functional cell tumors A solid ovarian mass in childhood is malignant until proven otherwise You W, Dainty LA, Rose GS, Krivak T, McHale MT, Olsen CH, Elkas JC. “Gynecologic malignancies in women aged less than 25 years.” Obstet Gynecol. 2005;105(6):1405 Hassan E, Creatsas G, Deligeorolgou E, Michalas S “Ovarian tumors during childhood and adolescence. A clinicopathological study” Eur J Gynaecol Oncol. 1999;20(2):124-6.
72 Only benign ovarian tumors are candidates for minimally invasive surgery Rare tumors of the uterus (sarcomas) are not suitable Ovarian Mass for minimally invasive surgery, except for diagnostic Symptoms purposes Size "Malignant" behavior Hormone-producing Rhabdomyosarcoma of the vagina (sarcoma botyroides) may be diagnosed and treated using Rupture Compression of Bowel infiltration Precocoious puberty Torsion bladder or bowel Ascites Virilization vaginoscopy Hemorrhage Menstrual irregularities Solid tumors (especially those >3 cm) should not be approached laparoscopically
Simple and small is good AFP Endodermal sinus tumors Embryonal carcinomas Mixed germ cell tumors Complex and large is bad CA-125 All epithelial tumors, especially serous Endometriosis, infections HCG Choriocarcinoma Embryonal carcinoma Mixed germ cell tumors
Premenopausal Ovarian Mass
Exclude Non-gynecologic Problem
LDH Dysgerminoma Simple on US, AND Solid or Complex on US, OR Mixed germ cell tumors < 6 cm > 6 cm, OR AND Elevated AFP/hCG/LDH, OR Testosterone Sertoli cell tumors Normal CA-125 Elevated CA-125 Leydig (hilus) cell tumors Observation for 6-8 weeks and Estradiol Adult granulosa cell Gonadotropin Suppression tumors, thecomas (OCP) Surgical Persistent Evaluation on US
73 Negative tumor markers Congenital Abnormalities No free peritoneal fluid Foreign body Unilocular –BUT remember that corpus luteum Trauma cysts can appear complex on U/S! Ovarian cysts Smooth capsule, no excrescences Pelvic Pain/Endometriosis Controversy about dermoids –they have Malignancies complex radiographic appearance, but may be Pelvic Inflammatory Disease approached laparoscopically Ectopic pregnancies
Turner’s syndrome/mosaic Any Y‐chromosome
Streak ovaries can be very attenuated Endo‐loop usually not practical –streak ovaries are not pedunculated Proximity to pelvic sidewall and ureter requires careful avoidance of collateral injury during dissection Attention to hemostasis
74 Teratomas Usually smooth with no excrescences Challenging to differentiate from benign tumors radiographically Rare free fluid Tumor markers variable False negative pathologic diagnosis is common Preponderance of neural elements suggestive of increased malignancy
O’Connor DM, Norris HJ: The influence of grade on the outcome of stage I ovarian immature (malignant) teratomas and the reproducibility of grading. IntJGynecolPathol 1994;13:283
Dysgerminoma Neuroblastoma Wilms’ tumor Rhabdomyosarcoma Lymphoma Leukemia Other nongenital tumors of the pelvis
75 Most common soft tissue sarcoma in children Genitourinary sites = 20% of primaries Usually localized to anterior vaginal wall Present as painless vaginal bleeding in pre‐ pubertal girls Poorer prognosis if extends to the cervix. MRI essential for adequate staging
Solomon LA, Zurawin RK, Edwards CL. J Pediatr Adolesc Gynecol 16:139-142. June 2003
76 Preserve reproductive and sexual function if possible If malignancy is suspected, perform a unilateral salpingo‐oophorectomy This may be performed laparoscopically if specimen can be removed intact Pursue staging and metastatic workup based on final permanent pathology
77 Disclosure
Gynecologic Outcomes of Bariatric I have no financial relationships to disclose. Surgery in Pediatric Adolescent Gynecologic Patient
Joseph S. Sanfilippo, MD, MBA University of Pittsburgh Magee‐Womens Hospital
BMI Categories: Underweight = <18.5kg/m2 Normal weight = 18.5–24.9 Overweight = 25–29.9 Obesity = BMI of 30 or greater Candidates for Gastric Surgery: BMI > 40 with co‐morbidity (DM‐ Obstructive Sleep Apnea‐HTN‐ Pseudotumor Cerebri BMI > 50
What is Obesity?
Clinical Terms Used to Describe Various Levels of Body Fat1
Normal Weight Overweight Obese Obese Extremely Obese (BMI* 18.5 to 24.9) (BMI 25 to 29.9) (Class I) (Class II) (Class III) (BMI 30 to 34.9) (BMI 35 to 39.9 ) (BMI 40 or more)
* BMI (Body Mass Index): A measurement of an individual’s weight in relation to height (kg/m2).
78 PATHOPHYSIOLOGY
• LEPTIN = satiety hormone – Produced by adipose tissue, regulates food intake / energy expenditure – Increased production and secretion correlates w/ amount of fat tissue – Targets CNS to reduce appetite and food intake – Deficiencies in leptin signaling / functioning obesity – Obesity = leptin resistance, dysfunctional energetic state
Health Consequences of Obesity
Major Health Other Health Consequences: Consequences:
• Type 2 diabetes • Asthma • Hypertension • Acid reflux (GERD) • Sleep apnea • Degenerative arthritis • High cholesterol • Polycystic ovarian • Heart disease syndrome (infertility) • Stress incontinence • Depression/anxiety • Gallstones • Some cancers
Who is “Predisposed” to Eating Disorders?
• Assess Family Environment‐School Environment Social Support • Abuse‐ Post traumatic Stress Disorder • Smoking Substance Abuse • Mood Disorders
79 Epidemic Rates Continue to Increase in the United States Comorbidities in adolescents seeking bariatric surgery 1990 1999 % of patients (n) Hyperinsulinemia 91% (n‐30) Sleep apnea 70% (n=23) 2008 Dyslipidemia 76% (n=25) Hypertension 36% (n=12) Polycystic ovary disease 12% (n=04) Diabetes 12% (n=o4) No data < 10% 10%‐14% 15%‐19% 20%‐24% 25%‐29% ≥30% Prevalence of obesity (body mass index-BMI ≥ 30) among US adults
Goals of Psychological Evaluation The Health Burden of Obesity Pre‐Operatively
• Ability to Consent‐Family Agreement/Support Endocrine Neurologic & Orthopedic & Cardiovascular Psychologic vs. Coercion Rheumatologic • Motivation‐Compliance • Knowledge regarding procedure(s) Dermatologic Renal • Post‐operative Expectations • Homework Between Appointments • Psychological Factor Analysis Ahead of Time Gastrointestinal Pulmonary Reproductive
Brown WV, et al. Am J Med 122:24-11, 2009
80 Open vs Laparoscopic
81 Important Prerequisites
• Preop Eval. – TSH T4 CMPAN PTH 25‐OH Vit D – Anthropometrics: Age onset of “Overweight” – Medical Co‐morbidities – Psychological Measures – Family History of Obesity Especially with co‐ morbidities: First & Second degree relatives – Surgical Procedure(s) Discussion – Risks‐Benefits‐Alternatives – Post‐op Recovery Time and Setting
Bariatric Surgery Options - Risks of Bariatric Surgery Adjustable Gastric Banding Advantages: Adjustable Banding Risks: •No stomach stapling, cutting, or intestinal rerouting •Low postoperative complication rates • Band erosion •Low malnutrition risk • Band slippage • Adjustable–customized for each patient • Infection of the band and •Reversible and/or port • Minimally invasive approach for all patients • Conversion to open procedure •Hospital stay is 1 day and 1 night for bleeding of other organ Disadvantages: injury •Slower initial weight loss than gastric bypass • Blood clots •Regular follow‐up critical for optimal results • Bleeding • Requires implanted medical device Adjustable banding •Less experience in the United States
Laparoscopic Adjustable Gastric Banding Bariatric Surgery Options – Adjustable Gastric Banding
82 Band adjustment
Band prolapse Band erosion
Tlcsurgery.com Tlcsurgery.com
83 84 Morbidly Obese 2 Year Old
Where Do We Draw the Line?
Morbidly Obese 2 Year Old OBSTETRIC COMPLICATIONS • Obesity increases risk of many complications of pregnancy • Greatest among morbidly obese • Saudi Arabia 2 y/a Failed Multiple Diets • Remains significantly increased for all women with BMI ≥ 30 • Sleep Apnea • International J of Case Reports 2013 • Surgery Successful – Dropped from 72 lbs to 52 lbs
TREATMENT OF OBESITY TO IMPROVE OBSTETRIC COMPLICATIONS REPRODUCTIVE OUTCOMES • Recommendation for weight loss before pregnancy prudent for young • Dietary and Lifestyle Changes women – First option for obese women with infertility • Some urge that BMI < 35 should be reached prior to conception – Intentional weight loss > 20lbs associated with 25% reduction in • All‐ cause mortality • Women in later reproductive years, postponing pregnancy to achieve • CV mortality weight loss must be balanced against risk of declining fertility with • Cancer mortality increasing age – Improvement in menstrual cycle regularity – Ovulatory function returns in majority of obese anovulatory women with PCOS after 5‐10% reduction – Improves insulin resistance – androgen levels, SHBG
• Anti‐Obesity Drugs • Surgery
85 SURGICAL CORRECTION SURGICAL CORRECTION • Musella et al. Effect of bariatric surgery on obesity‐ related infertility – Retrospective study, 110 obese infertile women • 69/110 (62.7%) – Evaluated effectiveness of bariatric surgery in improving achieved pregnancy fertility, assessing influence of age, surgical technique, co‐ morbidities (HTN, DM), weight loss, BMI (pre / post surgery) • 41/110 (37.3%) did not • BMI > 40 or BMI > 35 with serious co‐existing medical conditions • Procedures = gastric balloon placement, adjustable gastric banding, • BMI of women who sleeve gastrectomy, gastric bypass (laparoscopic) became pregnant • Followed for ≥ 2.5 years significantly lower than BMI among who did not conceive (p=0.001)
• Weight loss of >5 BMI strong predictor of pregnancy (OR 20.2)
OBESITY AND ART • Koning et al. 2012. Complications and outcome of assisted reproductive technologies in overweight and obese women. – Overweight / obese women seeking help for subfertility increasing – Overweight / obese women receive least‐fertility related services • New Zealand: BMI > 32 excluded from any fertility treatment • UK: almost all clinics with cutoff, but wide‐ranging (25‐40) • UK national guidelines: BMI > 29 be informed about lower pregnancy chances, but no official cutoff point for treatment
86 LAPAROSCOPIC SURGERY BENEFITS AND DISADVANTAGES BENEFITS DISADVANTAGES
Faster recovery Long specialty training required by the surgeon Less Pain Most surgeons cannot perform the Quicker return to work advanced procedures safely
Shorter Hospital stay Complex procedures require hundreds of cases before expertise Less chance of hernias and wound develops Infections Most surgeons trained in advanced Better Visualization laparoscopy were never trained in bariatric surgery Decreases stress on the immune system
Better cosmetic result
Minimal restrictions on activity
Candidates for bariatric surgery Bariatric Surgery Efficacy for Resolution of T2DM •BMI ≥ 40 or ≥ 35 with comorbidities Author Procedure Resolution • Understands and Pories et al 1995 Gastric Bypass 89% accepts risks Torquati et al 2005 Gastric Bypass 74% Schauer et al 2003 Gastric Bypass 82% • Willing and able to Sugerman et al 2003 Gastric Bypass 86% comply with diet, Dixon et al 2003 Lap Band 64% vitamins, & follow-up Gagner (unpublished) Sleeve Gastrectomy 65%
NIH Consens Statement 1991;9
“Questions”
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87 Disclosure
I have no financial relationships to Fertility preservation disclose. methods
Sara Brucker
Universitäts-Frauenklinik Tübingen
Problem situation Desire to conceive and cancer
Successful, aggressive treatments with very good prognoses for Cancer has no effect on fertility in 71% young cancer patients of female patients and 68% of male patients. (Shover 1999 und 2002; Zanagnolo 2005)
Life after treatment Fear of relapse 29% of breast cancer patients stated that the fear of a possible loss Quality of life, Long-term consequences of of fertility affected their decisions regarding their treatment. illness and treatment (Partridge 2004) future plans
DNA-damage by chemotherapy? Effective use of fertility preservation methods in Germany
• In 508 children of 235 patients who had received Establishment of Fertiprotekt 2006 chemotherapy with alkylating agents and/or radiotherapy in the abdominal area: no significant increase in genetic disorders • However: because of low patients numbers, an increase in the risk of genetic disease cannot be statistically excluded. (Meistrich et al., 2002, Am J Hum Genet)
Desire to conceive: wait 6 – 12 months as folliculogenesis takes ca. 6 months -Annual workshops -Definition of treatment recommendations -Recording of treatments -Expert evaluations
88 What happens to the ovary during chemotherapy? „Life cycle“ of the ovary
Leidenberger et al (2005): Klinische Endokrinologie für - Chemotherapy causes atresia of the follicle Frauenärzte, Springer-Verlag which has just entered the growth phase or is in the early stages of gonadotrophin - From 8 weeks of embryonic development: ca. 10,000.000 germ cells migrate into the undifferentiated gonads from the yolk sac and become stimulation. embedded in the gonadal cortex (= primordial follicle). Germ cells cease - Risk of development of ovarian insufficiency at this stage of development. is dependent on the number of follicles - At birth, around 90% of these primordial follicles have perished. - At puberty: still ca. 100,000 primordial follicles present in the ovary and therefore on the = reserve for the reproductive phase patient’s age. - Within ca. 40 years, between 400 and 500 egg cells are ovulated, the rest degenerate by apoptosis
What happens to the ovary during Which option for which patient? chemotherapy? • Oncological treatment • Partner status
(Block 1952, Gougeon 1994) • Time until first chemotherapy • Age < 35 versus > = 35 years Chemo • Likelihood of ovarian metastases • Hormone receptor status in breast cancer POF Menopause •Costs
Fertility preservation methods
Cryoconservation of fertilised Cryoconservation of egg cells after ISCI unfertilised egg cells Observation: Chemotherapy during prepubescence in girls does not always lead to subsequent POF
Cryoconservation of Ovarian protection using ovarian tissue GnRH-analogues Ovarian protection by downregulation of the ovaries before starting chemotherapy
89 Effectiveness of GnRH-cover:
GnRH-cover for ovarian protection Studies with proven effect Studies with no proved effect
-Beck-Fruchter et al. 2008: ZORO study: no reduction in the 12 studies (only 2 randomised): amenorrhoea rate with GnRH use -Chemo without GnRH-cover: POF in Follicle 59% of cases atresia -Chemo with GnRH-cover: POF in 9% of cases Increased Estradiol -Blumenfeld und von Wolff 2008 recruitment of Inhibin - Meta-analysis: primordial - POF rate 55.5% versus 11.1% follicles -Prospective study (Badawy et al. 2009) in 80 breast cancer patients: -After 8 months menstruation in the FSH GnRHa-group 89.6% of patients, in the control group 33.3%.
GnRH-analogues
Risks of GnRH-administration: Ovarian protection using GnRH -Menopausal side-effects analogues: -Reduction in bone density when GNRH analogues are used for >6 months -Reduction in oncological treatment effects with GnRH Recommendation: analogue use in estrogen receptor positive breast cancers. - Start: 2 weeks before chemo (Flare-up effect) - For early chemo start – additional use of an - However: no conclusive evidence for or against these antagonist hypotheses - End: 3 – 4 weeks after chemo Recommendation “AGO Mamma”: no GnRH in HR-pos. breast cancer
Fertility preservation methods Hormonal stimulation and ISCI procedure
Cryoconservation of fertilised Cryoconservation of egg cells after ISCI unfertilised egg cells
Follicular Cryoconservation of Ovarian protection using puncture ovarian tissue GnRH-analogues
Fertilisation (ICSI) Fertilised egg cell Cryoconservation
90 Antagonist protocol for stimulation „Classic“ antagonist protocol US Ovulation induction US Ovulation induction start in the second half of cycle
18mm 18mm GnRH-antagonists from day 6 of stimulation Follicle puncture Follicle puncture GnRH-antagonist daily, 0.25 mg/day GnRH-antagonist daily 0,25 mg/day
FSH / hMG 225 IE / day from day 2 of cycle Recombinant FSH 225 IE / day Period Period
Patient information before Fertility preservation methods hormonal stimulation for ISCI Cryoconservation of fertilised Cryoconservation of • Time required: ca. 14 days egg cells after ISCI unfertilised egg cells • Effect of hormonal stimulation with short-term high estradiol levels on breast cancer is unclear; probably no negative effect • Chances of pregnancy the same as for conventional IVF/ISCI methods • Cryoconserved fertilised egg cells can only be transferred with the partner’s consent • If overstimulation occurs, the start of Cryoconservation of Ovarian protection using ovarian tissue chemotherapy must be postponed if necessary GnRH-analogues Ovarian • Possibility of low response protection using • Information about the risks of follicular puncture GnRH-analogues • Financial situation (Letter can be provided for health insurance company)
Hormonal stimulation Fertility preservation methods
Cryoconservation of fertilised Cryoconservation of egg cells after ISCI unfertilised egg cells
Follicular Cryoconservation of puncture Disadvantage: Ovarian protection using ovarian tissue GnRH-analogues Significantly poorer pregnancy rates with cryoconserved unfertilised cells
Unfertilised oocyte Cryoconservation
91 Cryoconservation of ovarian tissue IVF Centre Tuebingen University Hospital for Women Appointment for counselling on fertility preservation 8 reported pregnancies after retransplantation of ovarian tissue up to now
Counselling on fertility preservation methods depending on tumour Problems: type, oncological treatment necessary and patient’s wishes -Risk of retransplantation of malignant cells – currently no retransplantation for leukaemia cases; future: in-vitro maturation of removed ovarian tissue possible? -Transplantation site: follicular genesis is disturbed in Hormonal stimulation GnRH Ovarian tissue for heterotopic transplantation; therefore orthotopic for egg cell retrieval protection +/- +/- cryoconservation transplantation is preferable (spontaneous pregnancies + / - IVF/ICSI are thereby possible )
Method: LSK: Atraumatic (no electrosurgical) resection of 1/3 – ½ Individual therapy, incorporated into the of the ovary, important: ovarian cortex (Storage in Bonn) oncological treatment concept
Tuebingen counselling results Tuebingen counselling results
>80 patients aged <40 years were counselled between November 2006 – Jan 2013
GnRH Hormonal Ovarian tissue Combination of No treatment stimulation for removal for methods Age group 21-30 years 31-40 years oocyte retrieval cryoconservation
Number 37 52 Total 20 15 34 18 16
Age distribution of the counselled patients
HR-positive HR-negative
Number 68 18
Hormone receptors Therapeutic decision after counselling
Method overview Take-home messages
Time needed Achievable pregnancy rates
- Treatment options are In vitro fertilisation ca. 2 weeks ca. 40% available (IVF/ICSI)
Cryoconservation of ca. 2 weeks ca. 40% - Accurately timed unfertilised oocytes planning is necessary Autologous transplantation ca. ½ week 2 births of conserved ovarian tissue (experimental)
GnRH-analogues not applicable
92 Thank you for your attention!
93 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.
~
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.
94