PEDIATRIC AND ADOLESCENT GYNECOLOGY pptx

YOUNG ADOLESCENT 10–13 YEARS During this phase of development, the external genitalia continue to approach adult appearance.. Partial vaginal agenesis, usually only the lower one third,

The reproductive tract in pediatric and adolescent patients differsfrom that of the adult, requiring special techniques and equipmentfor examination The gynecologic problems addressed in childrenand adolescents may differ markedly from those of adult womenbut may be no less serious Both the anatomy and physiology ofthe reproductive tract will change from the hormone-stimulated state

of the newborn to the relatively estrogen-free state of the youngchild to the blossoming of womanhood during adolescence

if massaged Breast massage should be avoided to prevent infection

or continued milk production

At birth, the clitoris is prominent, with a clitoral index of

0.6 cm2(clitoral index length in centimeters  width in timeters) The labia minora are large and may protrude through bul-bous labia majora The hymen is prominent and red, protecting avagina that averages 4 cm long A whitish vaginal discharge of mu-cus and exfoliated cells with an acid pH may be prominent Theuterus may be enlarged (4 cm long), with cervical eversion present.The endometrium may slough and vaginal bleeding may occurwithin a few days after birth Parents can be reassured that the

cen-18

PEDIATRIC AND

ADOLESCENT GYNECOLOGY

bleeding will stop by 10 days of age The ovaries have not descended

from the abdomen and cannot be palpated if normal

YOUNG CHILD (UNDER 7 YEARS)

With little estrogen stimulation, the external genitalia have luted from birth The labia majora are flat, and the labia minora arethin, as is the hymen The clitoris is no longer prominent, but the

invo-clitoral index remains unchanged The mucous membranes are pink

and only slightly moist The diameter of the hymenal opening is

⬃0.4 cm The vagina is ⬃5 cm long, and its secretions have an kaline pH Vaginal fornices do not develop until puberty Therefore,the cervix is appositioned against the vaginal vault and is difficult

al-to see or palpate If seen, the cervical os is a small slit The gressed uterus does not return to the size of the newborn until 6 y.The ovaries have many follicles that decrease in number untilmenarche During this time, the ovaries begin their descent into thetrue pelvis

re-OLDER CHILD (7–10 YEARS)

As estrogen stimulation returns, the mons pubis thickens, the labiamajora fill out, and the labia minora become more rounded Thehymen thickens, and the opening enlarges to 0.7 cm The vaginalmucosa thickens, and the vagina elongates to 8 cm The body ofthe uterus enlarges primarily by myometrial proliferation The en-dometrium gradually thickens The ovaries enlarge and descendlower into the pelvis The follicles enlarge, although none will par-

ticipate in ovulation, then gradually regress in size Breast buds may appear.

YOUNG ADOLESCENT (10–13 YEARS)

During this phase of development, the external genitalia continue

to approach adult appearance Bartholin glands begin to produce

mucus immediately before menarche The hymenal opening larges to about 1 cm The vagina lengthens to adult size (10–12 cm),and vaginal secretions become acidic The vaginal fornices develop.The body of the uterus becomes twice as long as the cervix Theovaries descend further into the true pelvis Breast development

en-continues, with buds progressing to small mounds Other ary sex characteristics develop (pubic and axillary hair), the body

second-becomes more rounded, and the adolescent growth spurt begins

GYNECOLOGIC EXAMINATION

NEWBORN

Because internal examination usually is unnecessary and difficult

at this age, examination is usually limited to the external genitalia.

Assessment includes the overall appearance, and looking for alies in addition to ambiguity of sex differentiation An abnormal

anom-or enlarged clitanom-oris may suggest congenital adrenal hyperplasia Thehymen is inspected for patency (to rule out imperforate hymen orvaginal agenesis) Rectal examination may detect the cervix, butnormally no other reproductive organs will be palpable

CHILD

Avoiding the use of stirrups often enhances the child’s cooperation.

An adequate view of the genitalia can be obtained with the child inthe frog leg position (knees flexed, legs fully abducted) on the ex-amination table or in the mother’s lap Enlisting the child’s coop-eration is often facilitated by direct conversation and explanationduring the examination After a general examination, including in-spection and palpation of the breasts, attention may be directed togentle palpation of the abdomen Ovarian tumors in this age groupusually occur in the low to midabdomen

Evaluation of the external genitalia includes evidence of properhygiene as well as lesions of the skin, inflammation, tumors, exco-riations, or vaginal discharge The labia minora should be separateposteriorly Ascertaining the presence of a vaginal opening is usu-ally accomplished by direct visualization Digital rectal examina-tion must be gentle

If visualization of the upper one third of the vagina is sary (e.g., foreign body, abnormal bleeding, screening for in uteroDES exposure, or penetrating injury), a vaginoscope, cystoscope,

neces-or laparoscope may be used and examination under anesthesia may

be necessary In the younger child, a 0.5 cm instrument can be used.

In the older child, an 0.8 cm instrument usually can be passedthrough the hymenal orifice

YOUNG ADOLESCENT

At this age, the girl may be very sensitive about the changes inher body She should be an active participant in the history and

examination process She should be asked whether or not she wishes

her mother to be present, and a female assistant should be present

if the mother is not It is important to reassure her that she may beembarrassed or somewhat uncomfortable but that the examinationwill not be painful and her hymen will not be damaged Sufficienttime must be available to allow for an unhurried examination andfull explanation of each procedure

Explaining and teaching breast self-examination during thebreast examination helps to establish this preventive measure Stir-rups usually are accepted in this age group After examination ofthe external genitalia, the cervix and vagina may be inspected us-ing a long-bladed Huffman-Graves vaginal speculum If the hy-menal opening is of sufficient size, bimanual palpation may be ac-complished with a single finger in the vagina If not, the uterus andovaries may be palpated using the rectal approach

After the examination, it is crucial to discuss the findings with

the patient and address her concerns Patient–doctor ity should be maintained If there is some problem of which the par-

confidential-ents should be made aware (e.g., pregnancy), advising the patientand serving as a supportive advocate may assist her in the neces-sary communication(s)

CONGENITAL ANOMALIES OF REPRODUCTIVE TRACT TYPICALLY DIAGNOSED BEFORE MENARCHE

ABNORMALITIES OF THE HYMEN

There are so many normal variations in the appearance of the men (e.g., size and number of orifices, thickness) that essentially

hy-the only true anomaly is imperforate hymen The solid membrane

of the imperforate hymen is thought to be a persistent portion ofthe urogenital membrane formed whenever the mesoderm of theprimitive streak abnormally invades the urogenital portion of thecloacal membrane

Obstruction of the vaginal outlet by the imperforate hymen

causes a buildup of vaginal secretions, initially a mucocolpos, and later (postmenarche) a hematocolpos The mucocolpos may be seen

as a flat or mildly protruding, thin, shiny membrane The vagina isdistended and may fill the pelvis Sonography will distinguish be-tween this condition and vaginal agenesis Hematocolpos is diag-nosed in an amenorrheic adolescent with a bulging purplish red hy-menal membrane and distended vagina Blood may fill the uterus

(hematometra) and spill from the uterine tubes into the peritonealcavity.

Imperforate hymen is corrected surgically at the time of nosis In the newborn, the procedure involves simple excision with-

diag-out sutures In the postmenarchal patient, the membrane must beexcised or incised as sutured because simple incision and drainageare likely to result in spontaneous closure and recurrence of hema-tocolpos

In some cases, an apparently imperforate hymen has very tiny

openings and is termed microperforate hymen Treatment is

simi-lar to that for imperforate hymen A septate vagina may have a gle thick median ridge at the hymenal orifice separating the twohalves, leaving a double hymenal opening Surgical correction isnecessary if obstruction of vaginal drainage is evident or if it willinterfere with intercourse

sin-VAGINAVAGINAL SEPTUM

A vaginal septum may be transverse or longitudinal The transverse septum is the result of faulty canalization of the embryonic vagina

and may occur at any level Septa in the upper portion usually arepatent, whereas those in the lower portion of the vagina may be im-perforate and result in mucocolpos or hematocolpos Incompletesepta may be followed until menarche, when complete excision can

be performed more easily A complete transverse septum should be incised at diagnosis to allow drainage to occur until menarche, when

complete excision of the remaining septum along with the attacheddense, subepithelial connective tissue can be performed

A longitudinal vaginal septum results from improper fusion of

the distal ends of the mullerian ducts The septum is fibrous, with

an epithelial lining that divides the vagina into two There may be

an accompanying bicornuate uterus with one or two cervices.

Treatment is necessary only if there is obstruction of drainage fromone side of the vagina, if dyspareunia is present, or if it would in-

terfere with vaginal delivery Rarely, a double vagina, complete with

two separate muscle layers, occurs and may be accompanied bydouble vulva, bladder, and uterus

AGENESIS

Nearly all patients presenting with an absent vagina have one offour circumstances: the Mayer-Rokitansky-Kustner-Hauser syndrome,

Kallmann’s syndrome, male pseudohermaphroditism, and testicularfeminization In the two syndromes, the individuals are geneticallyfemale (46,XX), whereas in the latter two circumstances, the indi-viduals are genetically male (46,XY) Numerically, although still

rare, the Mayer-Rokitansky-Kustner-Hauser syndrome is by far and away the most common.

Vaginal agenesis when associated with absence of the cervixand complete or partial uterine absence (and possibly the uterinetubes) is the Mayer-Rokitansky-Kustner-Hauser syndrome (alsocalled the Rokitansky sequence) This condition is the result of em-bryologic failure of the mullerian ducts to make contact with theposterior portion of the urogenital sinus Defects of the urinary tract(45%) and spine (10%) are common, as is hearing deficiency Onexamination, a dimple is noted where the hymenal opening should

be, with the remainder of the external genitalia appearing normal.Imaging (sonography, CT, or MRI) usually confirms the absence

of, or rudimentary internal genitalia, with normal ovaries Almostall of Mayer-Rokitansky-Kustner-Hauser syndrome patients will

have a 46,XX karyotype, but male pseudohermaphroditism and ticular feminization must be ruled out via karyotypic documenta-

tes-tion Treatment of the Mayer-Rokitansky-Kustner-Hauser syndromepatient usually involves only the development of a neovagina (seebelow)

Kallmann’s syndrome (KS) is the association of olfactory deficit with irreversible, congenital gonadotrophin deficiency (IHH) There

are several variants, and it occurs in both sexes In at least one ofthe male syndromes there is spontaneous endogenous gonadotrophinsecretion recovery in later life (the Bauman variant) The nongonadalmanifestations of Kallmann syndrome vary: unilateral renal aplasia,coloboma of iris, deafness, midline anomalies, oculomotor apraxia,and Moebius anomalad Most (but not all) patients have low serumlevels of basal gonadotrophins, testosterone, or estrogen, and had apoor response to LHRH stimulation Gene mutations that affect hy-pothalamic, pituitary, and gonadal function include: three genes thatcause inherited hypogonadotropic hypogonadism, gene mutations forthe beta-subunits of FSH and LH have been characterized, and bothactivating and inactivating mutations have been identified for the go-nadotropin receptor genes Treatment includes exogenous sex hor-mone replacement and psychological support, with long-term follow-

up to ensure normal sexual development, normal bone mass, andpsychosocial outcome, with fertility induction when indicated

The evaluation of pseudohermaphroditism and testicular inization necessarily involves detailed psychologic evaluation for

fem-gender acceptance before initiating therapy Therapy must behighly individualized and is probably best performed in one of

the national centers experienced in dealing with these complexproblems.

The treatment of vaginal agenesis involves the creation of a vagina when the patient is contemplating sexual activity This can

be accomplished without surgery by having the patient use a series

of progressively larger dilators to exert pressure in the dimple where

the hymen should be for 20–30 min daily for several months If this

is unsuccessful, a vagina may be created surgically Currently, three surgical procedures are popular for creation of the neovagina: Abbe- McIndoe (performed vaginally using a split thickness skin graft over

a stint), Vecchetti (a combined vaginal and laparascopic approach), and the use of a portion of sigmoid (requires laparotomy and bowel

surgery)

The ovaries function normally in the Hauser syndrome and pregnancies have been reported by in vitrofertilization with use of a surrogate Transvaginal ovum recovery ismaterially easier in those cases where the neovagina was created

Mayer-Rokitansky-Kustner-by pressure

Partial vaginal agenesis, usually only the lower one third, is

believed to result from failure of the urogenital sinus epithelium toinvade the vagina at 4–5 months gestation The upper vagina, uterus,

and tubes are normal Visual examination externally is the same as total vaginal agenesis, but sonographic examination confirms the presence of internal genitalia Rectal examination may reveal a dis-

tended upper vagina (especially if postmenarchal), and renal alies may be present

anom-Treatment of partial vaginal agenesis requires drainage of the obstructed upper vagina, usually by creation of a lower vagina.

UTERUS

Most uterine anomalies are not diagnosed until after menarche less other abnormalities of the reproductive tract are present (seeFig 18-1 and Chapter 22)

un-URETHRA

Epispadias is the term used to describe the female urethra that opens cephalad to a bifid clitoris as the result of failure of normal fusion of the anterior wall of the urogenital sinus This may be accompanied

by exstrophy of the bladder and defects in the abdominal wall as well

as the pelvic girdle Urological reconstruction is performed in infancy,but gynecologic repair usually is delayed until adolescence

FIGURE 18-1. Congenital uterine abnormalities

(From B Pensky, Review of Medical Embryology Macmillan, 1982.)

GYNECOLOGIC DISORDERS IN PREMENARCHAL CHILDREN

stim-Nonspecific vulvovaginitis is a polymicrobial infection

associ-ated with disturbed homeostasis, usually secondary to poor hygiene

or foreign body Vulvovaginitis due to secondary inoculation sults from blood borne or contact inoculation of the vagina withpathogens infecting other areas of the body (e.g., urinary tract in-

re-fection, upper respiratory tract infection) Specific vulvovaginitis is primary infection by such organisms as Neisseria gonorrhoeae, Bacterial vaginosis, Treponema pallidum, and herpes simplex The vaginal discharge (mucopurulent or purulent) from acute

vulvovaginitis may be minimal or profuse If the thin mucous brane of the vulva or vagina is denuded, there may be a blood-tinged appearance to the discharge The odor may be very foul Thepatient may experience only mild discomfort or severe perineal pru-ritus and burning, with itching so intense that the child scratches toexcoriation with bleeding The inflamed area may burn when urinepasses over it, suggesting urinary tract infection (UTI) when indeedthe urinary tract is uninvolved In these cases, a clean-catch speci-men cannot diagnose UTI because leukocytosis and contaminationfrom the vagina are difficult to eliminate

mem-Examination of the perineal area reveals erythema or soreness that may be localized or extending to the anus and thighs A rectal examination is essential to evaluate the pelvic organs Vaginoscopy

should be performed if the infection is recurrent or refractory to ment, especially if a foul-smelling bloody discharge (associated withforeign body) is present The most common foreign body is toiletpaper, although various small objects, such as beads and toys, can

treat-be found Radiographs are not reliable for diagnosis treat-because mostobjects are not radiopaque Objects in the lower third of the vaginacan be flushed out with warm saline or removed with bayonet for-ceps, but vaginoscopy is necessary to ensure that no objects remainhigher Recurrent placement of foreign bodies is not unusual

URETHRAL PROLAPSE

When the urethral mucosa protrudes through the meatus, it forms

a hemorrhagic, tender vulvar mass A short course of estrogen cream

is therapeutic when there is no urinary retention and the mass is small If the mass is large or urinary retention is present (or both), surgical resection of the prolapsed tissue is required under anes-

thesia with postoperative urinary catheterization for 24 h

TRAUMA

Although most injuries to the genitalia of children are accidental,

an index of suspicion must be maintained to avoid missing evidence

of child abuse or sexual abuse The description of the accidentshould fit the injury produced

Injury to the vulva usually results in hematoma formation that

requires no specific therapy other than cold compresses, unless theurethra is obstructed or the hematoma is large and continuing to in-crease in size If the urethra is obstructed, the bladder must bedrained, usually by the suprapubic approach A large hematomashould be incised and drained, with ligature of the bleeding points.Continued bleeding necessitates packing with gauze for 24 h and

prophylactic antibiotics Radiographs of the pelvis may be

advis-able to rule out fracture

If the hymen is lacerated, bleeding may be minimal, but a

pen-etrating injury must be suspected and vaginoscopy performed even

if the patient is asymptomatic Although most vaginal injuries

in-volve the lateral walls with little bleeding and little pain, a lesion extending to the vaginal vault requires pelvic exploration to rule

out extension into the broad ligament or peritoneal cavity Smallintravaginal hematomas require no therapy Large intravaginalhematomas should be incised and drained, with ligature of the bleed-ing point

LICHEN SCLEROSUS

Lichen sclerosus (hypotrophic dystrophy) of the vulva is seen mostcommonly in postmenopausal women but may be seen in youngchildren Histologic findings are the same in both groups, with nomalignant potential in children Whitish plaques or papules are seen

no further than the middle of the labia majora and do not encroachinto the vagina Because this lesion is susceptible to infection andbruises easily, vulvar irritation, pruritus, dysuria, and bleeding fromscratching are typical

Treatment consists of good hygiene and short-term use of drocortisone creams to stop the pruritus and allow healing About

hy-80% will improve significantly with onset of puberty

LABIAL ADHESION

Labial adhesion, which is common in prepubertal children, is lieved to be related to the thinness of the skin over the labia minora

be-as the result of low estrogen levels Local irritation can lead to

scratching, with injury and adherence in the midline Most adhesionsare asymptomatic and undiagnosed unless interference with urina-tion occurs Dysuria, pruritus, irritation, and vulvovaginal infections

may result Rarely, total occlusion causes urinary retention Treatment of symptomatic adhesions consists of 7–10 days of Premarin cream once or twice a day If medical treatment is un-

successful, surgical separation may be necessary Recurrence iscommon until puberty, when spontaneous resolution will occur

GENITAL TUMORS

Although uncommon, about 50% of the genital tumors of childrenare either malignant or premalignant and must be considered whenany of the following findings are present: chronic genital ulcer, non-traumatic swelling of the external genitalia, tissue protruding fromthe vagina, abdominal pain or enlargement, bloody foul discharge,and premature sexual maturation

BENIGN TUMORS

The common benign genital tumors of children are teratomas, mangiomas, simple cysts of the hymen, retention cysts of the pa- raurethral ducts, granulomas, and condylomata acuminata Small

he-cysts usually require no therapy Larger he-cysts require excision andmarsupialization of the remaining wall to prevent recurrence Ter-atomas require surgical excision Capillary hemangiomas usuallyregress spontaneously, but cavernous hemangiomas may bleedextensively if traumatized and must be evaluated for removal orablation.

quired for diagnosis Six months of chemotherapy is followed by surgical removal, radical hysterectomy, and vaginectomy without

oophorectomy Further chemotherapy for 6–12 months follows If

the tumor cannot be removed, radiation therapy is given to shrink

the tumor

Other Malignant Tumors

Endodermal carcinoma, mesonephric carcinoma, and clear cell cinoma of mullerian origin (associated with in utero DES exposure)are seen in children or adolescents Virtually all genital tumors seen

car-in adult women have been reported car-in children, and the treatment

gonadotropin-of ovarian stimulation leads to the production gonadotropin-of estrogen The organ response to gradually increasing estrogens and finally prog-esterone determines the alterations that occur during adolescenceand result in puberty Although the age of onset of puberty is in-fluenced by genetic as well as environmental factors, the second-ary sexual changes leading to sexual maturity occur over 3–5 years

end-(most within 2–4 years), usually between ages 9–14.

Early in the process leading to puberty, the genital system dergoes marked alterations The external genitalia gradually assume

un-the adult appearance The vagina develops progressively thicker cosa and, while becoming more distinct from the cervix, reaches itsadult length (10–12 cm) It also is more distensible and progres-sively more moist and acidotic with the reappearance of lactobacilli.

mu-The uterine corpus enlarges to twice the length of the cervix, and the ovaries descend into the true pelvis.

Late premenarche is marked by accelerated somatic growth and often rapid changes in secondary sexual characteristics The

body habitus begins to assume more feminine characteristics,

with breast buds appearing and gradually increasing in size larche, breast development, is the earliest adolescent change to-

The-ward puberty, preceding regular ovulation by ⬃2 years Pubic

hair (pubarche) and axillary hair appear later The method of

clas-sifying adolescent secondary sexual development through puberty

proposed by Marshall and Tanner (Table 18-1) has become

widely accepted Although puberty is technically defined as thematuration of endocrine and gametogenic function to the point

of reproductive capability, it is not uncommon for menarche (thefirst menses) to be used nearly interchangeably This is unfortu-nate, for the first few cycles (generally up to a year) are usually

anovulatory The average age of menarche in the United States is 12.8 years.

TABLE 18-1

TANNER CLASSIFICATION OF ADOLESCENTDEVELOPMENT IN THE FEMALE

DISORDERS OF SEXUAL

MATURATION

ACCELERATED SEXUAL MATURATION(PRECOCIOUS PUBERTY)

Sexual precocity is defined as the onset of sexual maturation $2.5

SD earlier than the normal age (i.e., onset of secondary sexual

characteristics8 years or menarche 10 years) Accelerated

sex-ual maturation may be complete or incomplete, depending on

whether one or all of the secondary sexual changes are occurring

The work up is similar in both circumstances and is geared to determining whether there is gonadotropin production and if there

is a detectable underlying disorder causing the condition In the majority of cases there will be maturity of the hypothalamic– pituitary–ovarian axis.

A complete medical, family, and social history is essential bertal development staging (Tanner) and plotting growth provide

Pu-quantifiable data for comparison to the norm Complete physical examination and pelvic examination will assist in guiding the proper utilization of further diagnostic tests Frequently imaging (ultra- sound, CT, or MRI) will be useful Hormonal analyses (most com-

monly FSH, LH, and estradiol) are often useful In cases of early

pubertal development, an interval of observation assists to ensure

that sexual maturation is continually progressive, as some cases willspontaneously regress

there may be no change in bone growth and no estrogen effect umented in the vagina, girls with premature thelarche have signif-

doc-icantly higher estradiol levels than normal prepubertal girls Thus,

premature thelarche does not involve increased sensitivity of breasttissue to estrogens Breast biopsy should not be performed, and gen-

erally no specific therapy is indicated.

PREMATURE PUBARCHE

Isolated development of pubic or axillary hair before age 8 years

may be idiopathic and of no clinical significance However, this hairgrowth may be a sign of excess androgen production from an inbornerror of metabolism (congenital adrenal hyperplasia from steroid en-

zyme deficiency) or tumor Excess androgen production must be cluded before idiopathic premature pubarche is diagnosed.

ex-PREMATURE MENARCHE

Isolated cyclic vaginal bleeding before age 10 years is considered

premature menarche In the past, it was thought that estrogen els were not increased and that bleeding resulted from endometrial

lev-sensitivity to low level estrogens More recently, higher levels of estradiol have been identified Other causes of vaginal bleeding should be excluded There is no adverse effect on growth, future

fertility, or menstrual pattern No therapy is advocated

These young females experience the orderly process of puberty

at an earlier age than normal, typically close to the expected age

of puberty but possibly as early as 2–3 years of age On occasion,

CT scan of the brain may reveal a small hamartoma in the thalamus Precocious puberty may also be the result of other cen- tral nervous system (CNS) lesions (e.g., tumors, previous fractures, meningitis, or encephalitis) It is believed that irritation of the hy-

hypo-pothalamus begins the maturation early, but the overall process may

be very prolonged (years)

Treatment is directed toward the CNS lesion, if it is treatable.

In idiopathic cases of incomplete accelerated sexual maturation,complete accelerated sexual maturation, or cases without a treat-

able CNS condition, recent therapy has been gonadotropin ing hormone agonists (GnRHA) Currently, most authorities rec-

releas-ommend GnRHA utilization from diagnosis until the projected onset

of the normal adolescent growth spurt (11–12 years) The long-termuse of GnRHA has been supplemented in some trials by the use ofgrowth hormone to treat any potential retardation of attaining fullheight The combination, while promising for those of short stature,

is still under investigation The potential osteopenia associated withlong-term GnRHA use has been reported to respond to concurrentsupplemental calcium administration

Uterine bleeding during GnRHA treatment for precocious

pu-berty is common, and may be massive and recurrent However,most episodes resolve spontaneously and necessitate no furthertreatment Thus, pretreatment counseling concerning this potentialcomplication (to those being treated as well as to their families)may assist in avoiding unnecessary anxiety and achieving bettercompliance

Early endogenous estrogen production is most likely of ian origin Large follicular cysts, teratomas, granulosa cell tumors,

ovar-or cystadenomas of the ovary may either produce estrogen ovar-or

stimulate estrogen production Nonovarian estrogen may be duced by adrenal adenomas, but both are rare Autonomous secre-tion of gonadotrophin by a tumor is even more rare, but has beenreported

pro-The largest single association with precocious puberty in the

absence of gonadotropin production is the McCune-Albright drome This condition, consisting of polyostotic fibrous dysplasia,

syn-irregular cutaneous pigmentation, and precocious puberty, holds anunfavorable prognosis The precocious puberty usually occurs at avery early age and results in short stature from early epiphyseal clo-sure and pathologic fractures Many affected girls are infertile, with

menstrual abnormalities The cause is unknown, and no specific treatment is available Recently experimental therapy for this form

of gonadotropin independent precocious puberty (which is resistant

to therapy with GnRH analogues) has included ketoconazole.Additionally, some authorities recommend the addition of growthhormone

women Although hirutism poses an esthetic problem, it is usually

a progenitor of infertility and the polycystic ovarian syndrome.

The work up of hirsutism and hyperandrogenism frequently

in-cludes: testosterone, androstenedione, dehydroepiandrosterone phate, 17 alpha-hydroxyprogesterone (basal and after ACTH), luteinizing hormone, and FSH Imaging (i.e., pelvic sonography)

sul-may be useful Therapy will depend upon the results of the nostic work up (See p 721)

diag-Additionally, girls with precocious puberty are at increased risk for anovulation during late (but not early) adolescence onward At

particular risk are those with a low weight at birth and/or a high17-hydroxyprogesterone response to ACTH at prepubertal diagno-sis of accelerated sexual maturation

DELAYED SEXUAL MATURATION

As with accelerated sexual maturation, a complete history,

includ-ing a three generation pedigree, is the startinclud-ing point in the work up.The absence of onset of puberty beyond 2.5 SD of the normal age

is considered delayed Absence of thelarche by 13 years or che by 15 years warrants investigation, and evaluation may be

menar-initiated earlier if there is concern Patients with delayed sexual

maturation may be classified into one of three categories: delayed menarche with adequate secondary sexual development, delayed puberty with inadequate or absent secondary sexual development,

or delayed puberty with virilization Delayed puberty in females is

a rare condition The majority of delayed puberty is associated with

a genetic disorder or hypothalamic–pituitary–ovarian problem Ofcourse, anatomic abnormalities of the ovaries, uterus, or lower gen-ital tract are rare but important considerations

Obtaining a complete medical, family, and social history is sential, as are plotting growth and performing pubertal develop-mental staging (Tanner criteria) A complete physical examinationand pelvic examination will assist in guiding the proper utilization

es-of further diagnostic tests Frequently imaging (ultrasound, CT, or MRI) will be useful Hormonal analyses (most commonly FSH, LH, and estradiol) are often useful.

Complete workup and differential studies are described inChapter 25.

PREGNANCY IN CHILDREN

AND ADOLESCENTS

Precocious or juvenile pregnancy occurs in girls with precociouspuberty and has been reported at less than 6 years of age Mostcases involve sexual abuse or incest There is an increased inci-dence of premature onset of labor, pregnancy-induced hypertension,and spontaneous abortion If the patient is under 9 years of age, ab-normal labor occurs in ⬃50% and neonatal loss may approach 35%

Adolescent pregnancy is increasing at an alarming rate The

attitudes and expectations of the teenager regarding pregnancyand motherhood usually are far from realistic Prenatal care andnutrition often are suboptimal The incidence of cigarette smoking,drug abuse, and sexually transmitted disease is high Preeclampsia-eclampsia, premature delivery, and intrauterine growth retardationoccur more frequently in adolescents than in adult women, makingadolescent pregnancy, in general, high risk Although adolescentsmay have a lower rate of gestational diabetes mellitus than adults,

it still occurs Thus, the incidence of screening for gestational betes mellitus in adolescents may be modified by the risk of theirethnic group or other risk factors

dia-Perhaps the best hope for preventing or improving the outcome

of adolescent pregnancy lies in early sex education, conscientious contraceptive counseling, and emphasis on prenatal care.

BREAST PROBLEMS OF THE CHILD

OR ADOLESCENT

The child or adolescent presenting with a breast complaint is uated just as is the adult female (see Chapter 19) A thorough his-tory, a detailed physical examination, and a careful sonography issufficient to obtain the correct diagnosis in most cases Mammo-grams are difficult to interpret because of the dense glandular breasttissue and almost never reveal microcalcifications, even in the pres-ence of breast cancer

eval-Fortunately, breast carcinoma is very rare before age 20 Indeed,

if malignant tumors are seen in children or adolescents they aremore likely to be metastatic (or secondary) than primary to thebreast Thus, the spectrum of breast malignancy encountered in chil-dren or adolescents is composed of relatively uncommon tumors,

with rhabdomyosarcoma and hematolymphoid tumors being themost frequent Sarcomas may arise in cystosarcoma phyllodes, ormore rarely, from other structures Primary breast carcinoma must

Cys-Although surgical care of any lesion has a primary objective ofcomplete excision of the lesion, biopsies of the developing breastshould also aim to protect the breast bud, nipple, and areola Specialbreast surgical problems of the child or adolescent requiring extraor-dinary operative approaches are gynecomastia and macromastia

At some time in their lives nearly all women experience a problem with their breasts Although these afflictions range from totally be-

nign to highly malignant, the majority of problems are self-limited

or readily treatable (Table 19-1) Indeed, the most common lems encountered in multidisciplinary care settings are a question

prob-of a mass ( ⬃65%), pain (⬃15%), nipple problems (including

dis-charge, ⬃10%), or a question concerning family history or other symptoms ( ⬃10%) However, the very real specter of breast cancer

makes preventive care (screening), early detection, thorough uation and complete care of every case a compelling medicalnecessity

eval-Moreover, the high incidence and potential seriousness of

breast disorders combine to cause breast problems to be one of the most frequently encountered problems in health care for women.

Patient expectations are not confined to just answering concernsrelative to their chief complaint They also appreciate rapid andcomprehensive response(s) to detected problems, education, pre-vention, and a resource for learning more about breast problems.Thus, to meet the expectations of their patients, providers of fe-

male health care need a thoughtful, well-organized, and carefully conducted plan for management of breast diseases One measure

of not meeting patient expectations concerning breast problems

is that failure to diagnose breast cancer in a timely manner isthe most common reason for malpractice litigation in the UnitedStates

Diagnosis may be accomplished in breast problems by using

standard techniques: a careful history, physical examination of the breasts, documentation, and imaging (usually mammography,

possibly sonography) The history should establish how long theproblem has existed, whether any other changes have been ob-served, any risk factors, and whether there is a history of biopsy

or breast cancer The physical examination aims to identify thosefeatures distinguishing malignant from benign changes and shouldinvolve axillae and periclavicular areas as well as the breasts

19

DISEASES OF THE BREAST

547

TABLE 19-1

TYPES, INCIDENCE, AND PEAK YEARS OF BENIGN

BREAST DISEASES

Incidence Peak

Mammary ⬃50 30–50 Bilateral, painful,

masses with cyclic variability Fibroadenoma 2–3 20–40 Usually unilateral

(10% –15% bilateral), firm, discrete, solitary, nontender mass Intraductal 3–5 45–55 Bloody, serous,

discharge from

a duct without palpable mass Fat necrosis
5 Any age Half give a history

of trauma, ecchymosis, skin retraction, or local tenderness Mammary ⬃1 50–60 Tender, bilateral

nipple retraction, enlarged axillary glands Macromastia 0.1 Puberty Diffuse enlargement

with continued growth Galactocele Perilactation Cystic ductal

dilatation Breast abscess ⬃2 Lactation Unilateral, localized

inflammation, fever, pain, tenderness, segmental erythema,