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US of the Pediatric Female Pelvis1


Reviews and Commentary 

Harriet J. Paltiel, MD
This review presents the normal and pathologic develop-
Andrew Phelps, MD2
ment of the gonads and genitourinary tract and addresses
the role of ultrasonography in the diagnosis and manage-
ment of gynecologic disorders of the pediatric pelvis, in-
cluding ambiguous genitalia, prepubertal bleeding, pri-
mary amenorrhea, pelvic mass, and pelvic pain.

© RSNA, 2014

Online supplemental material is available for this article.

1
 From the Department of Radiology, Boston Children’s
Hospital and Harvard Medical School, 300 Longwood Ave,
Boston, MA 02446. Received August 15, 2012; revision
requested September 10; revision received April 1, 2013;
accepted April 15; final version accepted June 6. Address
correspondence to H.J.P. (e-mail: harriet.paltiel@
childrens.harvard.edu).
2
Current address: Department of Radiology and
Biomedical Imaging, Benioff Children’s Hospital, University
of California, San Francisco.

q
 RSNA, 2014

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Review for Residents: US of the Pediatric Female Pelvis Paltiel and Phelps

T
he most common indications for computed tomography (CT) and mag- the endometrial lining undergoes cycli-
imaging of the pelvis in girls in- netic resonance (MR) imaging are gen- cal changes associated with the men-
clude ambiguous genitalia, prepu- erally reserved for further characteriza- strual cycle (9).
bertal bleeding, primary amenorrhea, tion of congenital malformations or
pelvic mass, and pelvic pain. Ultrasonog- tumors (1–4).
raphy (US) is the main imaging modality Normal Development of the Gonads and
used in the investigation of these disor- Reproductive Tract
ders with cross-sectional modalities; Technique Development of the female reproduc-
The pediatric uterus, vagina, and ovaries tive tract involves cellular differentia-
are optimally imaged when the pa- tion, migration, fusion, and canalization
Essentials tient has a full bladder. All patients are with probable apoptosis (programmed
therefore asked not to void in the hour cell death). This complex and inte-
nn Anomalies of the reproductive
prior to undergoing imaging and are grated sequence of events is associated
tract may manifest at different
encouraged to drink fluids; teenagers with many opportunities for abnormal
stages of life; most abnormalities
are asked to drink 16 ounces. development and structural anomalies.
of the external genitalia are ob-
Transabdominal and linear-array US Anomalies of the reproductive tract
vious at birth, whereas obstruc-
transducers are generally adequate for may manifest at different stages of life.
tive and nonobstructive lesions
most pelvic US examinations. A trans- Most abnormalities of the external gen-
of the reproductive tract may
perineal approach is useful in young italia are obvious at birth, whereas ob-
be apparent at birth or only
girls with urogenital malformation, hy- structive and nonobstructive lesions of
later: during childhood, at pu-
drometrocolpos, labial mass, or anal the reproductive tract may be apparent
berty, in adolescence, or in
atresia (5) (Fig 1). In sexually active at birth or only later: during childhood,
adulthood.
adolescents, a transvaginal approach is at puberty, in adolescence, or in adult-
nn In patients with ambiguous geni- used to complement the transabdomi- hood (10).
talia, US is used to determine nal examination. In the setting of a
the location of the gonads and complex congenital anomaly, genitogra-
the presence of a uterus. phy performed with water-soluble con- Structural Anomalies of the
nn US is used to assess ovarian size trast material in conjunction with US is Reproductive Tract
and morphology and to confirm often helpful in identification and char- Reproductive tract anomalies may re-
or exclude a postpubertal ap- acterization of the vagina, urogenital sult from agenesis and/or hypoplasia or
pearance of the internal geni- sinus, or cloaca. abnormalities related to lateral fusion,
talia; US can be used to diagnose vertical fusion, or resorption. Although
an estrogen-secreting adrenal or various classifications of these anom-
Normal Anatomy alies have been proposed, each has its
ovarian tumor and to monitor
the effects of medical or surgical The size and shape of the uterus and limitations, and none is comprehensive.
treatment. ovaries are age dependent and subject to The American Society for Reproductive
nn Ovarian tumors in the pediatric hormonal influence. Maternal and pla- Medicine, or ASRM, system separates
population are usually benign, cental hormones result in a relatively the anomalies into groups with similar
with cystic teratoma accounting large size of the neonatal uterus (Fig 2) clinical manifestations and prognosis
for more than 90% of all benign and ovaries, compared with their size (Table 1, Fig E1 [online]). Vaginal
ovarian tumors; most cystic tera- later in infancy, where they remain rel- anomalies are not addressed. The Va-
tomas range from 5 to 10 cm in atively stable until the first growth spurt gina Cervix Uterus Adnexa–associated
diameter, have a complex ap- occurs at 7 to 8 years of age (6). Mature Malformations classification includes
pearance, and generally contain follicles may be documented within the abnormalities not found in the ASRM
fewer than 50% soft-tissue ele- ovary at all ages, owing to the secre- classification (Table 2).
ments by volume. tion of follicle-stimulating hormone (7,8)
(Fig 3). The prepubertal cervix is equal
nn In the setting of adnexal torsion,
to or greater in thickness than the uter-
the affected ovary is always
ine fundus, and the endometrial lining Published online
enlarged, with a median volume 10.1148/radiol.13121724  Content codes:
is relatively inconspicuous (Fig 4). Uter-
12 times that of the contralateral
ine length varies, on average, from 2.5 Radiology 2014; 270:644–657
normal ovary.
to 4 cm, with a thickness less than or
nn US is a critical tool in the evalua- equal to 1 cm. Prepubertal ovarian vol- Abbreviations:
tion of pelvic pain in girls, in- DSD = disorders of sex development
ume is slightly less than 1 cm3. During
cluding those suspected of having PCOS = polycystic ovarian syndrome
puberty, the uterine fundus elongates
acute appendicitis. and thickens and is larger than the cervix; Conflicts of interest are listed at the end of this article.

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Review for Residents: US of the Pediatric Female Pelvis Paltiel and Phelps

Figure 1  Table 1
Classification of Müllerian Anomalies
according to the American Society for
Reproductive Medicine System
Anomaly
Type Classification

Type I “Müllerian” agenesis or hypoplasia


A. Vaginal (uterus may be normal or
 exhibit a variety of malformations)
B. Cervical
C. Fundal
D. Tubal
E. Combined
Type II Unicornuate uterus
A. Communicating (endometrial cavity
Figure 1:  Images depict transducer placement and normal female anatomy at transperineal US.  present)
(a) Sagittal diagram shows placement of a linear-array transducer on the perineum. (b) Corresponding B. Noncommunicating (endometrial
sagittal transperineal US image. R 5 rectum, U 5 urethra, V 5 vagina.  cavity present)
C. Horn without endometrial cavity
D. No rudimentary horn
Type III Uterus didelphys
Figure 2  Figure 4 
Type IV Uterus bicornuate
A. Complete (division down to
 internal os)
B. Partial
Type V Septate uterus
A. Complete (septum to internal os)
B. Partial
Type VI Arcuate
Type VII Diethylstilbestrol -related anomalies
Figure 2:  Sagittal US image of the neonatal A. T-shaped uterus
uterus demonstrates that the anteroposterior diam- B. T-shaped uterus with dilated horns
eter of the cervix (C) is greater than that of the C. Uterine hypoplasia
fundus (F). The endometrial cavity is visible as a
Figure 4:  Sagittal US image of the uterus in a Note.—Reprinted, with permission, from references 11–13.
thin, echogenic stripe in the center of the uterus
7-year-old girl shows a cervix (C) that is comparable
(arrow).
in thickness to the fundus (F).

Figure 3  other associated abnormalities. A di- misdiagnosed initially (15). US shows


agnosis can be assigned at birth, when fluid distension of the vagina and a
a bulge from the associated hydroco- lesser degree of uterine distension.
colpos and/or mucocolpos is present, Echogenic debris within the fluid is
owing to vaginal secretions related to due to mucous secretions in neonates
maternal estrogen stimulation. If not (Fig 5) and blood in postmenarcheal
diagnosed at birth, the mucus will be girls. Spillage of secretions through the
resorbed, and the bulge will resolve. fallopian tubes may lead to ascites.
After the onset of menarche, the ado- Uterine and vaginal anomalies may be
lescent patient may be asymptomatic diagnosed in the neonatal period in girls
or have a history of cyclical abdominal who are undergoing evaluation of multi-
and/or pelvic pain (10). A diagnosis of ple congenital anomalies or in adoles-
Figure 3:  Sagittal US image of an ovary in an
11-month-old infant shows normal follicles (*). imperforate hymen can usually be cence, during investigation of amenor-
made on the basis of physical examina- rhea, mass, and pelvic and/or abdominal
tion alone. However, in many patients pain. In the young child, an initial US
An imperforate hymen is the most a complete physical examination is not study will usually suffice until puberty, at
common anomaly of the female repro- performed at birth, and, frequently, which time MR imaging is indicated for a
ductive tract. There are usually no cases in symptomatic adolescents are more complete assessment. In the ado-

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Table 2 lescent patient, MR imaging is generally


performed after identification of abnor-
Vagina Cervix Uterus Adnexa–associated Malformations Classification mal US findings to conduct a complete
Anatomic Subgroup and Classification Definition evaluation of the genitourinary tract.
During normal development of the
Vagina (V) uterus, a resorption failure in the sep-
 0 Normal tum between the two embryological mül-
 1a Partial
lerian ducts results in a septate uterus.
 1b Complete hymenal atresia
The external surface of the uterus has
 2a Incomplete septate vagina , 50%
either a normal configuration or a flat,
 2b Complete septate vagina
 3 Stenosis of the introitus
broad fundus, and there are two endo-
 4 Hypoplasia
metrial cavities. The septum can be par-
 5a Unilateral atresia tial or complete, in which case it extends
 5b Complete atresia to the internal cervical os. There is usu-
 S1 Sinus urogenitalis (deep confluence) ally a single cervix (Fig 6). A bicornuate
 S2 Sinus urogenitalis (middle confluence) uterus results from partial nonfusion of
 S3 Sinus urogenitalis (high confluence) the müllerian ducts. The uterine horns
 C Cloacae are not fully developed. The external
  1 Other surface is deeply indented, and the cen-
 # Unknown tral myometrium may extend to the level
Cervix (C) of the internal cervical os (bicornuate
 0 Normal unicollis) or external cervical os (bicor-
 1 Duplex cervix nuate bicollis) (Fig 7). Complete nonfu-
 2a Unilateral atresia and/or aplasia sion of both müllerian ducts leads to a
 2b Bilateral atresia and/or aplasia
didelphys uterus. The individual horns
  1 Other
are fully developed and almost normal in
 # Unknown
size. Two cervices are inevitably present.
Uterus (U)
A longitudinal or transverse vaginal sep-
 0 Normal
 1 Arctuate
tum may be noted. Didelphys uteri have
 1b Septate , 50% of the uterine cavity the highest association with transverse
 1c Septate . 50% of the uterine cavity vaginal septa, but septa may be observed
 2 Bicornuate with the other müllerian duplication
 3 Hypoplastic uterus anomalies, as well. A unicornuate uterus
 4a Unilaterally rudimentary or aplastic is a single horned uterus with a single
 4b Bilaterally rudimentary or aplastic round ligament and fallopian tube that
  1 Other occurs when there is complete or near-
 # Unknown complete arrested development of one
Adnexa (A) müllerian duct. In most of these pa-
 0 Normal tients, arrest is incomplete, and a con-
 1a Unilateral tubal malformation, ovaries normal tralateral rudimentary horn with or
 1b Bilateral tubal malformation, ovaries normal without functioning endometrium is pre-
 2a Unilateral hypoplasia and/or gonadal streak (including tubal sent. The unicornuate uterus communi-
  malformation, if appropriate)
cates with a single cervix and a normal
 2b Bilateral hypoplasia and/or gonadal streak (including tubal
vagina. The rudimentary horn may or
  malformation, if appropriate)
may not communicate with the devel-
 3a Unilateral aplasia
oped uterine horn. US may demonstrate
 3b Bilateral aplasia
  1 Other
two uterine horns of different sizes. No
 # Unknown treatment is required, unless functional
Associated malformation (M) endometrium within a noncommunicat-
 0 None ing horn leads to hematometra or endo-
 R Renal system metriosis (10,16,17).
 S Skeleton A transverse vaginal septum results
 C Cardiac from a fusion and/or canalization failure
 N Neurologic in the müllerian ducts and embryological
Table 2 (continues) urogenital sinus. In these patients, the
external genitalia appear normal. A

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Table 2 (continued) Figure 8 

Vagina Cervix Uterus Adnexa–associated Malformations Classification


Anatomic Subgroup and Classification Definition

  1 Other
 # Unknown

Note.—Reprinted, with permission, from reference 14.

Figure 5  Figure 7 

Figure 5:  Imperforate hymen. Sagittal US image


of the pelvis in a 1-day-old neonate shows a mark-
edly distended vagina (V) filled with echogenic fluid.
A portion of the cervix is visible superiorly (arrow). Figure 7:  Bicornuate uterus in an 11-year-old
girl with severe dysfunctional uterine bleeding.
Transverse US image shows two uterine horns (*)
Figure 6  separated by a central indentation (arrowhead) (see Figure 8:  Transverse vaginal septum. (a) Sag-
Movie 2 [online]). ittal US image of the pelvis in a 12-year-old girl
with normal external genitalia shows marked disten-
sion of the uterus and upper vagina with blood and
complete vaginal septum may be located secretions. A hypoechoic crescent of tissue at the
in the upper (46%), middle (40%), or most inferior aspect of the dilated upper vagina
represents the vaginal septum (arrow). (b) Sagittal
lower (14%) vagina (18). The vagina is
transperineal US image demonstrates a collapsed
short or is a blind-ending pouch. The
distal vagina (*). The septum is not depicted on this
septa are generally less than 1 cm in
view. B 5 bladder, R 5 rectum, U 5 urethra, V 5
thickness and extend completely or in-
dilated proximal vagina.
completely across the vagina (Fig 8).
There is often a small central or eccen-
tric perforation. Patients may present mary amenorrhea, cyclic or chronic ab-
with mucocolpos in infancy or childhood, dominal or pelvic pain, or a pelvic mass.
hematocolpos in adolescence, and/or The usual treatment is hysterectomy.
pyohematocolpos due to ascending infec- Müllerian aplasia, also known as vag-
tion that occurs through the small perfo- inal agenesis and Mayer-Rokitansky-
Figure 6:  Septate uterus. Transverse US image of ration. It is clinically important to iden- Küster-Hauser syndrome, is the congen-
the uterus in a 16-year-old girl with pelvic pain and tify a cervix at imaging examination to ital absence of the vagina in association
severe menstrual cramping demonstrates two sepa- differentiate between a high vaginal sep- with variable müllerian duct anomalies.
rate endometrial cavities (*). The external myome- tum and cervical atresia, since treatment The incidence is approximately 1 in
trial contour is normal (see Movies 1a and 1b and prognosis differ (10). 5000 female births (17). There is usually
[online]). Cervical atresia is rare and occurs in associated cervical and uterine agenesis,
association with absence of the upper although about 7%–10% of patients
vagina. Patients may present with pri- have a normal but obstructed uterus, an

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Review for Residents: US of the Pediatric Female Pelvis Paltiel and Phelps

Figure 9  have normal secondary sexual charac-


teristics. However, a vaginal orifice is
absent. Transperineal US is useful in
documenting absence of the distal va-
gina and measuring the distance be-
tween the proximal vagina and the per-
ineal surface, particularly in an
intraoperative setting (10) (Fig 9).

Ambiguous Genitalia
Although a child born with ambiguous
genitalia is a rare event, the potential
psychological damage to the patient
and his or her family that is caused by
a delayed or incorrect diagnosis war-
rants the need for some familiarity of
the underlying origins and the initial
Figure 9:  Distal vaginal atresia in a 15-year-old girl with no vaginal orifice. (a) Sagittal US approach to diagnosis and management
image of the pelvis demonstrates a dilated uterus and upper vagina filled with blood and secre- on the part of healthcare providers.
tions. (b) Sagittal transperineal US image used to measure the distance (between calipers) from Any deviation from the normal appear-
the perineum to the obstructed, fluid-filled vagina. There is no vaginal tissue distal to the ance of the genitalia, such as labial fu-
obstruction. sion or clitoromegaly, should prompt
immediate investigation.
Table 3 In 2006, the Lawson Wilkins Pediat-
ric Endocrine Society and the European
DSD Definitions Society for Pediatric Endocrinology or-
Disorder Definition ganized an International Consensus
Conference on Intersex and published
Sex chromosome DSD 45,X and 46,XY mixed gonadal dysgenesis; 46,XX and a consensus statement proposing the
  46,XY chimerism term disorders of sex development
46,XX DSD (chromosomally female) Defect in ovarian development; disorder leading to high levels (DSD) to indicate congenital conditions
 of androgens (congenital adrenal hyperplasia most common);
with atypical development of chromo-
abnormality of müllerian ducts (dysgenesis or hypoplasia),
somal, gonadal, or anatomic sex (19).
uterus, or vagina
The DSDs are classified as sex
46,XY DSD (chromosomally male) Defect in testicular development; disorder of androgen synthesis
chromosome DSD (karyotype is not
 or action; syndrome associated with defects in genital
development (cloacal anomaly, genetic syndrome, vanishing
the usual 46,XX or 46,XY); 46,XX
testis syndrome, congenital hypogonadotropic hypogonadism)
DSD (chromosomally female); and
46,XY DSD (chromosomally male)
(Table 3). Karyotyping, hormonal
analysis, and imaging evaluation are
obstructed septate uterus, or a rudimen- formed to confirm the presence of nor- required for proper characterization of
tary uterus with functional endometrium mal ovaries, to characterize any mülleri- these disorders.
(17). Auditory, renal, and skeletal anom- an tissue, and to determine renal US is used to determine the location
alies occur in a substantial proportion of presence, position, and morphology. of the gonads and the presence of a
patients. Affected individuals have a nor- Distal vaginal atresia occurs when uterus and to assess the adrenal glands
mal female 46,XX karyotype with nor- the lower portion of the vagina fails to for diffuse enlargement or masses. A
mal ovarian hormonal and oocyte func- develop from the urogenital sinus and retrograde genitogram, obtained by in-
tion. At physical examination, patients fibrous tissue replaces the absent distal jecting contrast material through the
exhibit normal secondary sexual charac- vagina. The uterus, cervix, and upper urogenital orifice (Fig 10), and/or a
teristics and a normal perineum. The vagina are normal. Affected individuals voiding cystourethrogram will delineate
hymen is usually present, along with a usually present with primary amenor- the anatomy of the urethra, vagina (if
small distal vaginal pouch or vaginal rhea and may develop cyclical or chronic present), urogenital sinus, and occa-
dimple, since the hymen and distal va- pain and a pelvic or abdominal mass as sionally the cervix. In the setting of a
gina are both derived embryologically the upper vagina becomes distended cloacal anomaly, delineation of the con-
from the urogenital sinus. US is per- with blood and secretions. Patients nections of the urinary, genital, and

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Review for Residents: US of the Pediatric Female Pelvis Paltiel and Phelps

Figure 10  Figure 11 

Figure 10:  Sagittal oblique image from retrograde


genitography in a 2.5-month-old infant with a clo-
acal malformation. A Foley catheter was placed into
the perineal opening, and the balloon was inflated on
the perineum (arrow). Contrast material injected
through the catheter fills the vagina (V) and rec-
tum (R). A small amount of contrast material outlines
the endocervical canal (arrowheads).

gastrointestinal tracts to the cloaca can


also be ascertained (20,21).
The most common cause of sex
chro­mosome DSD is 45,X (Turner
syndrome), caused by absence of part
or all of one of the X chromosomes.
Monosomy X is the most common.
Associated abnormalities include Hashi-
moto thyroiditis, congenital heart
disease, renal anomalies, diabetes,
and skeletal abnormalities. Charac-
teristic physical features include short Figure 11:  Adrenal hyperplasia in a 6-month-old infant with clitoromegaly. (a, b) Transverse US images
stature, low-set ears, webbed neck, demonstrate markedly enlarged, cerebriform right (a) and left (b) adrenal glands (between arrows). (c) Sagittal
and broad chest. Gonadal dysfunction US image of the pelvis shows a dilated, fluid-filled vagina and uterus. (d) Sagittal image from voiding
manifests as amenorrhea and steril- cystourethrography shows a catheter coiled within the bladder. There is reflux of urine into the vagina that
ity. US demonstrates a juvenile ap- communicates with the urogenital sinus (arrow) via a short channel (arrowhead). B 5 bladder, L 5 liver,
pearance of the uterus, and the LK 5 left kidney, RK 5 right kidney, S 5 spleen, U 5 uterus, V 5 vagina.
ovaries may not be visualized (Fig E2
[online]).
Most cases of 46,XX DSD are A cloacal malformation occurs as a to duplication of the uterus and proxi-
caused by congenital adrenal hyperpla- result of interruption of the normal dif- mal vagina. The urogenital sinus per-
sia. In these patients, the adrenal ferentiation of the urinary, genital, and sists, and the hymen and lower vagina
glands are enlarged, and there is mas- gastrointestinal tracts. Cloacal malfor- do not form appropriately. Drainage of
culinization of the lower urogenital mations occur exclusively in females, pooled urine through the vagina is gen-
tract. Reflux of urine into the vagina with an incidence of 1 in 40  000 to erally necessary to prevent bladder out-
and uterus in patients with a urogeni- 50 000 newborns. Failure of the urorec- let obstruction, and the gastrointestinal
tal sinus can lead to hydrometrocol- tal septum to form or fuse properly tract is decompressed with a colostomy.
pos. The uterus and ovaries are nor- during embryological development re- Many of these children have additional
mal (Fig 11). Rarer causes of sults in incomplete separation of these congenital anomalies and often require
masculinized but chromosomally nor- tracts with a common confluence, the multiple reconstructive operations.
mal females include maternal andro- cloaca, and a single perineal orifice. Detailed diagnosis usually requires
gen ingestion in early pregnancy and a There is usually an associated fusion genitography and/or voiding cystoure-
masculinizing ovarian tumor. failure in the müllerian ducts that leads thrography (Fig 12), as well as con-

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Figure 12  Figure 13  menarche without other signs of pu-


berty (22).
In central precocious puberty, acti-
vation of the hypothalamic-pituitary-
gonadal axis with secretion of gonado-
tropin-releasing hormone by the
hypothalamus leads to isosexual pu-
bertal development. Although the
cause of central precocious puberty in
most patients (.80%) is idiopathic, le-
sions of the hypothalamus, pituitary
gland, or adjacent structures have
been identified at CT and MR imaging.
Central nervous system abnormalities
that lead to precocious puberty can oc-
cur at any time during childhood, al-
though they are more common in
younger children. Some authors there-
fore recommend that all girls younger
than 8 years of age who have progres-
sive central precocity undergo central
nervous system imaging (23). Pro-
longed exposure to sex steroids from
any source can also trigger central pre-
cocious puberty (22).
In gonadotropin-independent (pe-
Figure 13:  Adrenocortical carcinoma in a 6-year- ripheral) precocious puberty, serum
old girl, with early development of pubic hair, axillary gonadotropin levels are low. An ovar-
odor, clitoromegaly, and hypertension. (a) Sagittal ian cyst or tumor, or, occasionally, an
US image of the pelvis shows a peripubertal appear- adrenal tumor, may produce estrogen
ance of the uterus (between cursors), with relative autonomously. These lesions often
prominence of the uterine fundus (F). (b) Sagittal US produce high levels of estrogen, and
image of the right adrenal gland depicts a large, pubertal development may progress
Figure 12:  Images in a 4-day-old neonate with solid adrenal mass. The child died several years later more rapidly than in children with cen-
cloacal malformation. (a) Transverse US image of of metastatic disease. tral precocity. Girls exposed to topical
the pelvis shows duplicated vaginas filled with fluid or ingested androgen or estrogen can
and debris (*). (b) Sagittal image from voiding cys- flicted trauma, or trauma resulting also develop signs of precocity, such as
tourethrography shows a catheter in the bladder (B). from physical or sexual abuse), and a virilization or breast development
Contrast material has refluxed into both vaginas (V), vaginal mass. (22). US is used to assess ovarian size
as well as into the rectum (R), which communicates and morphology and to confirm or ex-
with the cloaca in the midline, between the dupli- Precocious Puberty clude a postpubertal appearance of the
cated vaginas. (Image courtesy of Jeanne S. Chow, The definition of precocious puberty in internal genitalia. US can be used to
MD, Boston Children’s Hospital and Harvard Medical North American girls is somewhat con- diagnose an estrogen-secreting adrenal
School, Boston, Mass.) troversial. However, the traditional or ovarian tumor and is used to moni-
definition of breast or pubic hair devel- tor the effect of medical or surgical
trast material–enhanced examination opment in girls younger than 8 years of treatment (Fig 13).
of the distal limb of the colostomy age is still used by most endocrinolo-
(10,20). gists. Precocious puberty may be cen- Vaginal Foreign Body
tral and gonadotropin dependent in Toilet paper and fibrous material from
origin or peripheral and gonadotropin clothing and carpets are the most
Prepubertal Bleeding independent in origin. There are also common vaginal foreign bodies. Other
Vulvovaginitis is the most common origin incomplete forms of precocious pu- causes include self-exploration and
of prepubertal bleeding. Other underly- berty, including premature thelarche sexual abuse. Patients may present
ing abnormalities include precocious pu- (breast development), premature with vaginal bleeding, discharge, uri-
berty, vaginal foreign body, genital pubarche (adrenarche; pubic or axil- nary symptoms, and abdominal or pel-
trauma (accidental trauma, self-in- lary hair), and isolated premature vic pain. With US, both radiopaque

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Review for Residents: US of the Pediatric Female Pelvis Paltiel and Phelps

Figure 14  Figure 15  Figure 16 

Figure 16:  Vaginal rhabdomyosarcoma in a


16-month-old girl with a mass protruding through
the introitus. Sagittal US image of the pelvis dem-
onstrates a large soft-tissue mass within the vag-
inal lumen (between arrowheads). The uterus (U)
appears normal.

dysgenesis (50%); hypothalamic hy-


pogonadism (20%); absence of the
Figure 15:  Fibroepithelial polyp in a 17-year- uterus, cervix, and/or vagina (15%);
old girl with a protruding vaginal mass. (a, b) transverse vaginal septum or imper-
Sagittal transperineal US images demonstrate a forate hymen (5%); pituitary disease
solid mass (arrows) protruding through the vag- (5%); and other disorders (polycystic
inal lumen (V), anterior to the rectum (R). ovarian syn­ drome [PCOS]; congeni-
tal adrenal hyperplasia; androgen in-
Figure 14:  Images of a vaginal foreign body in a
vulvar, or perineal mass. The tumor sensitivity) (5%) (32). US is usually
4-year-old girl. (a) Sagittal US image demonstrates has a bimodal distri­bution, with the the initial imaging study performed
a tubular foreign body (arrow) indenting the pos- first peak between 2 and 6 years of in the evaluation of these patients.
terior wall of the bladder (B). (b) Anteroposterior age and the second between 14 and The need for additional imaging stud-
supine radiograph shows a screw in the midpelvis 18 years of age. The embryonal and ies is dictated by the associated ab-
that was removed at vaginoscopy. botryoid subtypes are the most com- normalities.
mon. Tumors metastasize to the liver, PCOS is the most common endo-
lymph nodes, lung, and bone (Fig 16, crinopathy in premenopausal women,
and nonradiopaque objects can appear Fig E4 [online]). US is often the first affecting 6%–8% of females of repro-
echogenic. Distal acoustic shadowing imaging modality used in the work up ductive age (33). Despite its high prev-
can be present, and there may be a of girls with vaginal rhabdomyosar- alence, the diagnostic criteria and
slight indentation on the posterior coma. MR imaging or CT is required treatment are not universally agreed
bladder wall (2,24) (Fig 14). to assess the full extent of pelvic upon. The Androgen Excess Society
disease, and chest CT is performed to criteria for PCOS include (a) hyperan-
Vaginal Masses determine the presence of pulmonary drogenism (hirsutism and/or hyperan-
Benign vaginal masses include cysts and metastases. drogenemia) and (b) ovarian dysfunc-
polyps (Fig 15, Fig E3 [online]). Malig- tion (oligoanovulation and/or polycystic
nant masses are rare and include rhab- ovaries at US) (34). The diagnosis of
domyosarcoma, endodermal sinus tumor, Primary Amenorrhea PCOS in young adolescents is espe-
and clear cell carcinoma (25–29). Fewer than 10% of girls have menar- cially challenging, since transient oligo-
Vaginal rhabdomyosarcoma arises che before age 11, while 90% have menorrhea and mild hyperandrogen-
in the anterior wall adjacent to the begun to menstruate by age 13.75 emia are common in the first few years
cervix. Uterine involvement occurs years (30,31). Absence of menarche after the onset of menarche.
secondary to direct extension of tu- by the age of 15 years is termed “pri- The sonographic features of PCOS
mor from the vagina. Patients may mary amenorrhea.” Causes of pri- are variable; the ovaries may appear
have vaginal bleeding and a vaginal, mary amenorrhea include gonadal normal or enlarged, with multiple

652 radiology.rsna.org  n  Radiology: Volume 270: Number 3—March 2014


Review for Residents: US of the Pediatric Female Pelvis Paltiel and Phelps

Figure 17  Figure 18  rhagic ovarian cysts reflect the age of
the blood: Fresh blood is hyperechoic
relative to the ovarian parenchyma,
older blood has a heterogeneous ap-
pearance, and a blood clot undergoing
lysis becomes anechoic.
In the prepubertal child, ovarian
cysts may be functional or nonfunctional.
Functional cysts develop as a result of
gonadotropin stimulation of the ovary,
with a decreased incidence in early
childhood after the neonatal period and
an increase as puberty is approached.
Figure 17:  Polycystic ovarian syndrome in an
Some functional cysts will be hormonally
11-year-old girl with secondary amenorrhea.
active and cause gonadotropin-indepen-
Sagittal US image of the right ovary (volume, 20.5
mL) demonstrates multiple, small intraparenchy- dent (peripheral) precocious puberty,
mal follicles with a predominantly peripheral which manifests as premature breast de-
distribution. velopment or vaginal bleeding. Nonfunc-
tional ovarian cysts also occur in child-
hood and will appear as nonresolving
small follicles (Fig 17, Fig E5 [online]). cystic masses. Both types of cyst often
The Rotterdam US criteria define poly- appear as an asymptomatic abdominal
cystic ovaries as the presence of 12 or mass or increasing abdominal girth
more follicles measuring 2 to 9 mm in noted by a parent or physician. Other
diameter or an ovarian volume of more modes of presentation include chronic
than 10 mL in at least one ovary, as abdominal pain, intermittent pain from
measured at transvaginal US (35). torsion without complete compromise of
Since most adolescent girls will un- the vascular supply, or acute, severe
dergo transabdominal US and not Figure 18:  Prenatal ovarian cyst complicated by pain that mimicks appendicitis or perito-
transvaginal US, a determination of torsion. (a) Prenatal US image shows a simple ovarian nitis from hemorrhage, torsion, or per-
the number of ovarian follicles is diffi- cyst (between cursors). A large mass was palpable at foration. Nonspecific symptoms, such as
cult, and volume measurements are birth. (b) Sagittal postnatal US image obtained at 10
nausea, vomiting, bloating, urinary
days of age shows a cystic structure filled with layer-
generally used as the main diagnostic frequency, or retention, may also occur.
ing debris extending out of the pelvis and into the
criterion. US is used to document cyst size and to
abdomen (arrow). Ovarian torsion was subsequently
determine if a lesion is a simple cyst, a
identified at surgery. RK 5 right kidney.
complex cyst, or a solid mass. Simple
Gynecologic Pelvic Masses cysts or cysts with debris suggestive of
Gynecologic masses in girls include ovar- tion by maternal and fetal gonadotro- hemorrhage are managed conserva-
ian cyst, hematocolpos, primary ovarian pins. Ovarian cysts are often displaced tively, with follow-up imaging examina-
neoplasm, metastatic ovarian disease, into the abdomen, where they may be tions performed after 4–8 weeks to doc-
primary vaginal and uterine neoplasms, palpable and are simple or complex at ument regression. If the cyst persists at
and pregnancy. US. Most are asymptomatic, and spon- follow-up but the US features are reas-
taneous regression occurs after birth. suring, continued observation is appro-
Ovarian Masses Regression usually occurs within 3–4 priate. When a prepubertal simple ovar-
Ovarian masses include nonneoplastic months but can take as long as a year ian cyst persists, increases in size to
cysts and benign and malignant tumors. (36). Serial US studies are performed more than 5 cm in diameter, or is symp-
Most tumors in the pediatric population to document regression. tomatic, then surgery should be consid-
are benign and of germ cell origin, un- The development of hemorrhage ered (39,40). Simple ovarian cysts and
like adults, where epithelial neoplasms within an ovarian cyst in utero or post- hemorrhagic corpus luteum cysts are
predominate. natally has a high association with long- common in postpubertal girls.
term ovarian loss, generally thought to
Ovarian Cyst be due to torsion (Fig 18), although Ovarian Tumors
Fetal ovarian cysts are occasionally de- there has been some speculation in the Ovarian tumors may arise from germ
tected at antenatal sonography. They literature that the underlying origin cells, stroma, or surface epithelium. Most
are usually unilateral, and most are may be related instead to ovarian dys- occur in the 2nd decade of life, with
thought to result from ovarian stimula- genesis (37,38). US features of hemor- teratoma being the most common type.

Radiology: Volume 270: Number 3—March 2014  n  radiology.rsna.org 653


Review for Residents: US of the Pediatric Female Pelvis Paltiel and Phelps

Teratomas are composed of elements of Figure 19  Figure 20 


all three germ cell layers: 90% are clas-
sified as mature or cystic, and 10% are
classified as immature (containing em-
bryonic neural elements) or malignant.
Ectodermal components predominate
in cystic teratomas, which are also known
as dermoid cysts (41).
Cystic teratoma accounts for more
than 90% of all benign ovarian tumors.
It is usually unilateral, although 10%–
20% are bilateral. Most tumors range
from 5 to 10 cm in diameter. The US
appearance depends on the relative Figure 19:  Ovarian dermoid cyst in a 14-year-old Figure 20:  Granulosa theca cell tumor in a
amounts of sebum, serous fluid, cal- girl. Sagittal US image of the left adnexa demon- 9-year-old girl with breast development, vaginal
strates a complex mass that contains both cystic bleeding, and abdominal pain. Sagittal US image
cium, hair, and fat within the lesion
(arrows) and solid, echogenic (*) components. demonstrates a large, solid mass of the abdomen
(29,42,43). Most tumors have a complex
and pelvis that contained cystic foci.
appearance and usually contain fewer
than 50% soft-tissue elements by vol-
ume (Fig 19, Fig E6 [online]). serous and mucinous cystadenoma,
Malignant ovarian neoplasms are most of which are benign (47). Serous vic inflammatory disease, ectopic preg-
rare, accounting for only 1%–2% of all cystadenoma is usually a large, unilocu- nancy, and appendicitis.
malignant neoplasms in children youn- lar, thin-walled cystic mass that may
ger than 17 years of age (44–46). Ma- contain thin septations and papillary Adnexal (Ovarian and/or Tubal) Torsion
lignant germ cell tumors usually occur projections; approximately 20% are bi- Partial or complete rotation of the
in postmenarcheal girls and most often lateral. Mucinous cystadenoma gener- ovary on its vascular pedicle compro-
manifest as an asymptomatic pelvic or ally occurs as a large, multiloculated mises venous and lymphatic drainage,
abdominal mass. They are typically cystic mass. Papillary projections are as well as arterial inflow. Torsion is
larger than 10 cm in diameter at the identified less frequently, and it is less nearly always unilateral, and pathologic
time of diagnosis. Intraabdominal likely to be bilateral in comparison to findings range from massive edema to
spread involves the lymph nodes and serous cystadenoma (29,42). parenchymal necrosis. Ovarian torsion
liver. Dysgerminoma is the most com- Although little has been reported on in neonates and premenarcheal girls is
mon ovarian malignancy in childhood; the subject of metastatic ovarian tu- a rare event because most cases are re-
10% are bilateral. Malignant teratoma mors in children, the ovaries may serve lated to the presence of an ovarian cyst,
contains more than 50% soft-tissue ele- as a sanctuary for leukemia (48), and which is uncommon in this population.
ments by volume. Other germ cell tu- metastatic spread from neuroblastoma, However, torsion of a normal adnexum
mors may be homogeneous and echo- Burkitt lymphoma (49), colon cancer, can occur. Hypermobility due to lax
genic at US or complex with cystic and rhabdomyosarcoma (50) has been supporting ligaments is thought to ac-
areas, due to hemorrhage (29,41–43). documented. Metastatic ovarian in- count for torsion of the normal adnex-
Sex cord stromal tumors are low- volvement is often asymptomatic, and um. The peak incidence of adnexal
grade malignancies that arise from gran- diagnosis is only made at autopsy (51). torsion occurs in adolescence and
ulosa theca cells and Sertoli cells of the young adulthood (53).
embryonic gonad. They are diagnosed Pregnancy The affected ovary is always en-
most often in prepubertal girls and are Pregnancy should be considered in the larged, with a median volume 12 times
usually asymptomatic. Granulosa theca differential diagnosis of a pelvic mass that of the contralateral normal ovary
cell tumor may cause isosexual precoc- in girls 9 years of age and older. There (54). Multiple mildly enlarged (8–12
ity due to estrogen production (Fig 20, is an increased risk of complications mm), peripherally located follicles are
Fig E7 [online]). Sertoli-Leydig cell tu- in pediatric pregnancy, including toxe- the only relatively specific sign of tor-
mor may cause virilization as a result of mia, preeclampsia, placental abruption, sion, believed to be caused by transuda-
androgen production. At US, these tu- laceration, and need for cesarean sec- tion of fluid as a result of vascular con-
mors appear as heterogeneous masses tion (52). gestion (Fig 21). A ratio of torsed
with cystic foci. Metastases are unusual adnexal volume to normal adnexal vol-
but may occur on the peritoneal surfaces ume that is higher than 20 is predictive
or within the liver (29,41–43). Pelvic Pain of an associated ovarian mass, whereas
Epithelial tumors are rare before Causes of pelvic pain include adnexal a ratio of less than 20 is predictive of
puberty; the most common types are torsion, hemorrhagic ovarian cyst, pel- absence of an ovarian mass (54).

654 radiology.rsna.org  n  Radiology: Volume 270: Number 3—March 2014


Review for Residents: US of the Pediatric Female Pelvis Paltiel and Phelps

Figure 21  Figure 22  Figure 23 

Figure 21:  Adnexal torsion in a 5-year-old girl


with acute onset of abdominal pain and nausea and
a palpable pelvic mass. Color Doppler US image of
the right adnexa shows an enlarged, avascular ovary
with multiple peripheral follicles. Right ovarian vol-
ume was 27 mL, and left ovarian volume was 1.3
mL. There was no associated right ovarian mass at
surgery.

Pelvic Inflammatory Disease


Pelvic inflammatory disease affects girls
of reproductive age and is usually due to
an ascending sexually transmitted infec-
tion. Common causative organisms in- Figure 22:  Pyosalpinx in an 18-year-old woman
clude Neisseria gonorrheae and Chla- with pelvic inflammatory disease. (a) Transvaginal US
mydia trachomatis. The diagnosis is image shows a dilated, thick-walled fallopian tube
generally established clinically on the (arrow) abutting the ovary (O). The tube is filled with
basis of fever, pelvic pain, tenderness, echogenic material. (b) Transvaginal color Doppler Figure 23:  Perforated appendicitis in a 3-year-
and vaginal discharge (55). US image demonstrates mural hyperemia of the old girl. (a) Sagittal US image of the right lower
US is useful in identifying complica- fallopian tube. quadrant of the abdomen shows a well-circum-
tions of pelvic inflammatory disease, in- scribed, heterogeneous collection with an echogenic
cluding pyosalpinx and tubo-ovarian ab- focus at its base, associated with distal shadowing
scess. USc findings depend on the stage cents who have the highest reported (arrow) in keeping with an appendicolith. (b) Color
of the inflammatory process: Findings death rate. The diagnosis should be con- Doppler US image depicts prominent peripheral
are minimal, if any imaging abnormal- sidered in the presence of pelvic pain, an hyperemia, which is compatible with an abscess.
ities are detectable early in the course of abnormal serum b-human chorionic go-
infection. Pyosalpinx appears as either a nadotropin level, irregular vaginal bleed- ending structure that demonstrates a gut
thick-walled, fluid-filled tubular structure ing, and a missed menstrual period (57). signature and an outer diameter larger
or as an oval or round mass with low-level than 6 mm (58). Intraluminal stones (ap-
echoes due to the presence of purulent Acute Appendicitis pendicoliths), periappendiceal inflamma-
debris (Fig 22). There is a spectrum of Acute appendicitis is the most common tion, and abscesses are also readily de-
ovarian involvement, including enlarge- pediatric surgical emergency. Patients tected (Fig 23, Fig E8 [online]).
ment and increased ovarian echogenici- typically present with right lower quad- Advantages of US for diagnosis relative
ty; tubo-ovarian complex, consisting of rant pain, tenderness, and leukocytosis. to CT include lack of ionizing radiation;
an abnormal matting together of the Symptoms of acute appendicitis overlap no need for contrast material adminis-
ovary and fallopian tube; and tubo-ovari- with a number of other gastrointestinal tration or sedation, with the attendant
an abscess, result­ing in a complex intra- conditions, including mesenteric adenitis risks; and lower cost.
ovarian mass (56). and Crohn disease, as well as acute gyne- In our practice, we will initially per-
cologic conditions. form a US examination in patients sus-
Ectopic Pregnancy The appendix is optimally imaged pected of having acute appendicitis.
A complete discussion of ectopic preg- with US by using a high-frequency trans- Cross-sectional imaging with CT or MR
nancy is beyond the scope of this article. ducer and a graded-compression tech- imaging is reserved for those patients
However, it is important to note that it nique. The inflamed appendix is depicted in whom the appendix is not visualized
occurs on rare occasion in young adoles- as a noncompressible, tubular, blind- at US, for patients in whom the US study

Radiology: Volume 270: Number 3—March 2014  n  radiology.rsna.org 655


Review for Residents: US of the Pediatric Female Pelvis Paltiel and Phelps

is equivocal and there is a strong clini- 8. Cohen HL, Shapiro MA, Mandel FS, Shapiro sex disorders. Pediatrics 2006;118(2):e488–
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nancial activities related to the present article: 25(Suppl 1):S60–S61.
none to disclose. Financial activities not related 13. The American Fertility Society classifications
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