Pediatric and Adolescent Gynecology, An Issue of Obstetrics and Gynecology Clinics (The Clinics: Internal Medicine)

201 Pages·2009·4.89 MB·English

by  S. Paige Hertweck MD

PediatricandAdolescentGynecology
Foreword
WilliamF.Rayburn,MD,MBA
ConsultingEditor
This issue pertaining to Pediatric and Adolescent Gynecology (PAG), edited by
S. Paige Hertweck, MD, provides expert perspectives from a multidisciplinary team,
many of whom helped define pediatric and adolescent gynecology as a specialty.
An important clinical reference, this issue combines contemporary approaches to
diagnoses with the latest management advice to address gynecologic problems in
infants,children,andadolescents.
Gynecologicproblemsencounteredininfantsandchildren(genitaltrauma,intersex
disorders,mulleriananomalies)areuniquetotheseagegroupsandinvolvephysician
skillsdifferingfromthoseutilizedforadults.Becauseoftheseuniquecircumstances,
practicing obstetrician-gynecologists are often uncomfortable in evaluating and
managing these children. For this reason, pediatric gynecology was created as
anewspecialtyincorporatingtheexpertiseofgynecologists,pediatricians,urologists,
pediatricsurgeons,endocrinologists,andgeneticists.
The American College of Obstetricians and Gynecologists recommends that the
initial reproductive health visit to the obstetrician-gynecologist occurs as early as
age 13. This initial visit would not include a pelvic examination unless indicated by
the medical history. Instead, this encounter would provide an opportunity for the
obstetrician-gynecologist to begin a physician-patient relationship, counsel patients
and parents or guardians regarding health behaviors and dispel myths and fears. It
also will assist an adolescent in building trust into the health care system when she
hasaspecificneed.
Healthcareoftheadolescentfemaleshouldincludeareviewofnormalmenstrua-
tion,dietandexercise,healthysexualdecision-making,relationships,immunizations,
and injury prevention. Preventive counseling is beneficial for parents, guardians, or
other supportive adults, and can include discussions about physical, sexual, and
emotionaldevelopment;signsandsymptomsofcommonconditionsaffectingadoles-
cents;andencouragementoflifelonghealthchoicebehaviors.
This issue highlights differences between adolescents and adults about certain
gynecologicdisorders(endometriosis,polycysticovariansyndrome,abnormalcervical
cytology,menstrualdisorders,contraceptionandintrauterinedevices).Severalarticles
covertopicsemphasizedbytheAmericanCollegeofObstetriciansandGynecologists
ObstetGynecolClinNAm36(2009)xiii–xiv
doi:10.1016/j.ogc.2009.01.002 obgyn.theclinics.com
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xiv Foreword
CommitteeonAdolescentHealthCare.Therisksofexposuretoviolence,substance
use,sexuallytransmitteddisease,andunintendedpregnancythreatenthehealthand
well-beingofadolescents.Additionalresearchisneededtodeterminethebestcare
for adolescents, especially in defining optimal treatment. Unfortunately, there exists
confusion about adolescents participating in research because ofuncertainty about
the need for parental permission and what constitutes appropriate protection as
research subjects. We look to the distinguished group of contributors in this issue
tocarrythisagendaforward.
William F.Rayburn, MD,MBA
Departmentof Obstetricsand Gynecology
Universityof NewMexicoSchool ofMedicine
MSC10 5580;1University ofNewMexico
Albuquerque,NM87131-0001, USA
E-mail address:
[email protected]
PediatricandAdolescentGynecology
Preface
S.PaigeHertweck,MD
GuestEditor
Pediatric and adolescent gynecology (PAG) is a unique subspecialty of gynecology
that encompasses reproductive healthcare of young women under the age of 22.
The spectrum of conditions that a gynecologist may see present in young women
between the newborn period and adolescence is wide and varied. Many conditions
are an overlap between the fields of gynecology and pediatrics, pediatric endocri-
nology,hematology,urology,pediatricsurgery,dermatology,psychiatry,publichealth
medicineandgenetics.
AspastpresidentoftheNorthAmericanSocietyforPediatricandAdolescentGyne-
cology(NASPAG),IhavehadtheopportunitytointeractwithPAGexpertsacrossthe
UnitedStatesandCanada.ThisissueofObstetricsandGynecologyClinicsprovides
a collection of PAG topics presented by some of those physicians who have been
recognizedasexpertsineachchosenarea.Theseauthorshavebeenhand-selected
basedontheirexpertise,theirhistory,andtheirabilitytomakeacomplextopicunder-
standablebyallhealthcareproviders.
I am pleased to begin this issue with a written summary of one of my favorite
lectures given at NASPAG: My PAG Photo Album by Dr. Mary Anne Jamieson. In
this article, Dr. Jamieson provides a wonderful visual overview of PAG cases with
nuancesandclinical‘‘pearls’’toassistthereaderinprovidingthebesttreatmentfor
thepresentingcondition.Thisshouldstirthereaders’interestandenhancethereading
oftherestoftheissue.
Inthisissue,PAGissuesaddressedrangefromdisordersofsexualdevelopment,
including mu¨llerian anomalies, to those of genital trauma and urologic conditions
thatmaypresenttothegynecologist.Ahelpfulandpracticalarticle,‘‘TheAdolescent
SexualHealthVisit,’’beginsthelatterhalfofthisissue,andlikethearticlesonupdates
ofpolycysticovariansyndromeandcontraceptionintheadolescent,ithasanoverlap
with public health issues facing teens and our society today. The issue closes with
a look at hematologic conditions (both thrombophilias and bleeding disorders) that
presenttothegynecologist,anddysmenorrheaandendometriosisintheadolescent.
Eachauthor,asmentioned,wasselectedbecauseoftheirexpertiseintheirtopic,and
byreadingtheirarticles,youshouldgleanpracticalinformationforthoseyoungwomen
whomyouencounterwhohavePAGconditions.
ObstetGynecolClinNAm36(2009)xv–xvi
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xvi Preface
LetmeclosewithathankyoutoDrs.Jamieson,Allen,Breech,Laufer,Yerkes,Merritt,
Shafi, Burstein,Lara-Torre, Pfeifer, Kives,James, Dietrich, Yee,andTempleman for
their timely adherence to a tight deadline to facilitate publication in time for the
2009 Annual Clinical Meeting of NASPAG in San Antonio, Texas, and for the 2009
AnnualClinicalMeetingoftheAmericanCollegeofObstetriciansandGynecologists.
I would also like to acknowledge the noteworthy assistance of Carla Holloway
whosekindpersistence andpatientbutfirmdemeanor haskept usontrack. Sheis
acredittoherfield.Thankyou.
ForthosereaderswithfurtherinterestinPAG,pleaseconsidermembershiptothe
NorthAmericanSocietyforPediatricandAdolescentGynecology(NASPAG)oratten-
dance at one of NASPAG’s annual meetings. The mission of NASPAG is to provide
aforumforeducation,research,andcommunicationamonghealthcareprofessionals
whoprovidegynecologiccaretochildrenandadolescents.NASPAGisasmallsociety
ofapproximately400to500membersmakingitaverycollegialandinteractivegroup.
MembershipincludesasubscriptiontotheJournalforPediatricandAdolescentGyne-
cologyandaccesstoalistservetoassistyouwiththeabilitytoaskexpertsforadvice
inthecareofPAGpatients.
Ihopethat you find thisissue notonlyenjoyable, buthelpful asyou care forthis
unique type of gynecologic patient. We have a unique role as healthcare providers
tothisspecialpopulationastheirinteractionwithusisoftentheirintroductiontogyne-
cology. A proper diagnosis, management, and positive interaction with us can
empowertheseyoungwomentotakechargeoftheirownhealthcareandbestcare
forthemselves.
S. PaigeHertweck,MD
Departmentof Obstetricsand Gynecology
Universityof LouisvilleSchool ofMedicine
550 SouthJacksonStreet
Louisville, KY40202, USA
E-mail address:
[email protected]
A Photo Album
of Pediatric and
Adolescent Gynecology
MaryAnneJamieson,MD,FRCSC
KEYWORDS
(cid:2)Images (cid:2)Cases (cid:2) Dysmenorrhea (cid:2) Amenorrhea
(cid:2)Vulvardiseases (cid:2)Pelvicneoplasms (cid:2)Pelvicmass
(cid:2)Mullerianducts (cid:2)Pelvicpain
Duringthepastdecadeasthesolepediatricandadolescentgynecologysubspecialist
inatertiarycareacademiccenter,theauthorhashadtheprivilegeofcaringformany
patientswithmanyproblems,somecommonandsomeuniqueorchallenging.Often
thesepatientsandtheirfamilieshaveallowedtheauthortotakephotographs(protect-
ingtheiranonymity)withtheunderstandingthateducatinghealthcareprovidersisan
essential part of ensuring that other young girls who have similar conditions will
receivethecarethattheyneed.Thisarticlepresentsaselectionofimagesdepicting
pediatric and adolescent gynecologic conditions that, although not particularly
common,presentwitharelativelycommoncomplaintsuchasdysmenorrhea,pelvic
massorpain,genitalirritation,andamenorrhea.Healthcareproviderswhorecognize
theunusualunderlyingconditionwillsavepatientsandtheirfamiliesdays,weeks,or
even years of misdiagnoses, frustration, fear, or even pain and suffering. When
possible, references to useful publications/articles about the particular conditions
are provided. Textbooks on pediatric and adolescent gynecology also devote full
chapterstoeachofthesefourtopics.1–3
DYSMENORRHEA
Mostcasesofdysmenorrheainadolescentsrepresentprimarydysmenorrhea;thatis,
prostaglandin-mediated physiologic menstrual cramping associated with ovulatory
cycles.Becauseperimenarchalgirlsarenotalwaysovulatory,earlymenstrualcycles
often are irregular and painless. Girls who do experience primary dysmenorrhea
should get relief from properly administered non-steroidal anti-inflammatory drugs
(NSAIDS), and for those who still suffer, the combined use of oral contraceptives
andNSAIDSusuallysuffices.Whenthesestrategiesfail,orwhenseverepainaccom-
panies early cycles, other underlying conditions, such as obstructive Mu¨llerian
congenital anomalies (see the article by Breech in this issue), endometriosis (see
Pediatric&AdolescentGynecology,Obstetrics&Gynecology(andPediatrics),Queen’sUniver-
sity,Victory4,KingstonGeneralHospital,76StuartStreet,Kingston,Ontario,Canada
E-mailaddress:[email protected]
ObstetGynecolClinNAm36(2009)1–24
doi:10.1016/j.ogc.2009.01.004 obgyn.theclinics.com
0889-8545/09/$–seefrontmatterª2009ElsevierInc.Allrightsreserved.
2 Jamieson
thearticlebyTemplemaninthisissue),constipation,andpelvicadhesions,shouldbe
considered.4–18
Case1:ObstructedNon-CommunicatingUterineHorn(Figs.1A,2)
A12-year-oldcomplainedofpredominantlyright-sideddysmenorrheabeginningwith
menarche. She visited the emergency department with each of her first three
menstrualcycles.Aroutineultrasounddidnotidentify anyabnormality,buttheMRI
was classic for a non-communicating right-sided obstructed uterine horn adjacent
to a normal left hemi-uterus with patent outflow tract. After excision of the non-
communicating horn, the patient experienced only mild central dysmenorrhea that
respondedwelltoNSAIDS.Althoughthishornwasremovedbylaparotomy,laparo-
scopic excision is possible, depending on the junction and the surgeon’s comfort
andskill.Removalofthehorndidleaveabedofmyometriumontheleftuterusthat
required layered closure. Although the recommendation is controversial, the patient
was advised to discuss elective cesarean with any future obstetrics care provider
(seeCase4andreferenceslistedregardingmyomectomyandfutureobstetricuterine
rupture). (For a list of useful references, see the discussion of dysmenorrhea in the
previousparagraph.)
Case2:ObstructedHemi-VaginawithRightHematocolpos(Figs.1B,3–6)
A precoital 17-year-old girl did not experience dysmenorrhea until 3 years after
she began menstruating. She was thought to have primary dysmenorrhea, but
instead of improving with treatment with NSAIDS and an oral contraceptive pill,
the problem seemed to be worsening. The patient began to notice intermenstrual
B
A
= Ovary
C
Fig.1. (A)NoncommunicatingRuterinehorn(Case1).(B)ObstructedRHemivagina(Case2)–
often associated with Ipsilateral Renal Agenesis (OHVIRA). (C) Uterine Didelphys with
completelongitudinalvaginalseptum(Case3).
APhotoAlbumofPediatric,AdolescentGynecology 3
Fig.2. (Case 1) Non-communicating right uterine horn (RH) with unobstructed left hemi-
uterus (LH) with right and left ovaries (RO and LO, respectively) and normal left tube
(longarrow)withattenuatedhypoplasticrighttube(shortarrow)tofibriaremnant.
Fig.3. (Case2)Bulgingobstructedrighthemi-vaginawithhematocolpos(longarrow).Short
arrowindicatesthehymen.
Fig. 4. (Case 2) Evacuation of clotted hematocolpos from obstructed right-sided hemi-
vagina.
4 Jamieson
Fig.5. (Case2)Septum(S)excisioninprogress.
Fig.6. (Case2)Twocervices(left,LC,andright,RC)attheapexofthevagina.Oftenatleast
onecervixlooksalittleatypical.Notetheexcisionsutureline(arrow)istakenupashighas
possiblebetweenthetwocervices.
pelvic pressure and new-onset difficulty with tampon insertion. Although an MRI
was performed, the pelvic ultrasound (done transabdominally) and gentle single-
digit vaginal examination were enough to make the diagnosis of non-communi-
cating right-sided hemi-vagina. The vagina has a capacity to expand with the
slow accumulation of menstrual blood, and symptoms and therefore diagnosis
can be delayed several months to a couple of years. Especially because the left
side of this uterine didelphys and duplicated vagina are not obstructed, early
menstrual cycles can seem quite normal. The vaginal septum was excised in
the operating room under regional or general anesthetic (Figs. 3–6), and every
effort was made to wedge out the base to avoid leaving a ridge of uncomfortable
remnant. The lines of excision need to be oversewn with absorbable suture in
a hemostatic fashion. After this outpatient procedure the patient had no menstrual
or coital complaints, and a single tampon sufficed. (For a list of useful references,
see the earlier discussion of dysmenorrhea.)
Case3:UterineDidelphyswithCompleteLongitudinalVaginalSeptum(Figs.1C,7)
IncontrasttothepatientinCase2,thepatientshowninFig.7hasacompletelongi-
tudinalvaginalseptumandmustwearatamponinbothsidesunlessshechoosesto
APhotoAlbumofPediatric,AdolescentGynecology 5
Fig.7. (Case3)Introitalimageofcompletelongitudinalvaginalseptum(S)withrightand
leftvaginasidentifiedbyarrows.
havetheseptumremoved(performedinthesamefashionasthatdescribedinCase2).
Patientswhohavethisconditioncanhaveobstructedhemi-vaginaandipsilateralrenal
anomaly(OHVIRA)syndrome.13
Patientswhohaveacompletelongitudinalvaginalseptummostoftenhaveduplica-
tionoftheirMu¨lleriananatomy.Unlesstheyhavesometypeobstructionofmenstrual
outflowattheuterine/cervixlevel,theyexperienceonlyprimarydysmenorrhea.They
may be asymptomatic or may present with perceived tampon overflow, perceived
menorrhagia,difficultywithtamponinsertion,ordifficultywithintercourse.Sometimes
theypresenttotheemergencydepartmentafterintercoursehasresultedintearingof
the septum, which can bleed quite briskly and require urgent surgical repair (or
removal).Itoftentakesanastutecliniciantofindtheseptumduringthegynecologic
examination, but for patients in whom Pap smears and cervix swabs are indicated,
bothcervicesneedtobesampled.ThisparticularpatienthadtwoseparateMu¨llerian
systemsandhadatotaloftwopregnancies,oneineachhorn.Eachuteruswillhavean
ipsilateral fallopian tube and ovary. (For a list of useful references, see the earlier
discussionofdysmenorrhea.)
Case4:Leiomyoma(RareinThisAgeGroup)(Figs.8,9)
Aprecoital15-year-oldpresentedwithworseningdysmenorrheathatdidnotrespond
to standard medical therapies. A single-digit gynecologic examination was limited,
andarectalexaminationwas‘‘normal.’’Thetransabdominalpelvicultrasoundidenti-
fied an isolated 4- to 5-cm intramural fibroid, but in her age group this finding was
completely unexpected. After the myomectomy menstrual cycles were much more
tolerable. This surgery was performed by laparotomy, based on surgeon’s comfort
and skill, with meticulous hemostasis, layered closure, and minimal use of cautery.
Some surgeons would have chosen a laparoscopic approach. Although the recom-
mendation is controversial, the patient was advised that future childbirth probably
should be by elective cesarean section. The medical literature has several case
reportsandcaseseriesregardinglaparoscopicmyomectomyandtheriskofuterine
rupture during subsequent pregnancies. The reported rates of rupture are low, but
theytendtoberatesofprelaboruterinerupture,becausemanypatientsintheseries
doundergoelectivecesareansection.19–29