Post on 04-Jul-2015
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Gynecologic Problems of Childhood
Nelson’s Club
April 2011
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Neoplasms
• Most common gynecologic neoplasm found in children
of ovarian originusually presents as an abdominal mass• Ovarian neoplasmsconstitute 1% of all childhood malignancies8% of all malignant and abdominal tumors
in children
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Neoplasms
• Ovarian neoplasms10-30% of the ovarian neoplasms
operated on during childhood or adolescence are malignant
• Other common neoplasms:Paraovarian tumorsUterine neoplasms
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Neoplasms
• Vagina or vulva may also be the site of a benign or malignant lesion in children
• Cervical dysplasia may occur in adolescents
• Chemotherapy, especially with alkylating agents is associated with germ cell damage in the postpubertal ovary
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Neoplasms
• Prepubertal ovary, on the other hand, is markedly resistant to chemotherapeutic damage of germ cells
• Survivors of childhood cancer who previously underwent abdominal or gonadal irradiation have an increased rate of spontaneous abortions
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Ovaries
• Neonatal and Pediatric Ovarian cystso most often clinical manifestations of
ovarian tumors are abdominal pain, mass, or both
• Teratoma – the most common neoplasm in adolescents; are usually benign
-Calcification on abdominal roentgenogram is often a hallmark of a benign teratoma
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Ovaries
• Ovarian adenomas are the second most common benign tumor
• Ovarian cancers are extremely uncommon in children, but are responsible for 1:500 ovarian malignancies; affect only 4% of women
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Ovaries
Dysgerminoma
- The most common germ cell tumor
- Other germ cell tumors are malignant teratomas, endodermal sinus tumors, embryonal carcinomas, mixed cell neoplasms, and gonadoblastomas
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Ovaries
Immature teratomas and endodermal sinus tumors
- more aggressive malignancies than dysgerminomas
- occur in a significantly higher proportion of younger girls (<10 yr of age)
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Ovaries10-yr survival rates:
73% for epithelial carcinomas
44% for sex cord stromal tumors
73% for dysgerminomas
33% for malignant teratomas
39% for endodermal sinus tumors
25% for embryonal carcinomas
30% for other germ cell neoplasms
100% for gonadoblastomas
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OvariesDysgerminomas usually are associated
with XY gonadal dysgenesisGerm cell tumors - associated with
positive alpha-fetoprotein, human chorionic gonadotropin (HCG), and chorioembryonic antigen
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OvariesTreatment:
surgical excision followed by postoperative chemotherapy; radiotherapy
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Ovaries
Embryonal Carcinoma
-accounts for 6% of pediatric ovarian neoplasms and 8% of all germ cell tumors
-presents with progressive increase in abdominal pain and distention
-highly malignant with an average age of detection between 13-15 yr
-60% of patients have endocrinologic signs and symptoms
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Ovaries
Embryonal Carcinoma
-60% of patients have endocrinologic signs and symptoms (precocious pseudopuberty, abnormal vaginal bleeding, and hirsutism)
-produce HXG and alpha-fetoprotein
-Treatment involves unilateral salpingo-oophorectomy for stage I disease
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Ovaries
Endodermal Sinus Tumor
-are the most lethal of all ovarian cancers
-median age of presentation is 19 yr
-alpha-fetoprotein as a useful tumor marker
-tumors are radiosensitive
-may be treated surgically if they are stage I, unilateral salpingo-oophorectomy is performed
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Ovaries
Choriocarcinoma
-Primary choriocarcinoma of the ovary is extremely rare and often fatal
-0.6% of germ cell tumors are choriocarcinomas
Mixed Germ Cell Tumor
-4% of all ovarian neoplasms in children
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OvariesSex Cord Stromal Tumor- 5% of ovarian neoplasms- granulosa cell tumor is the most common- Isosexual precocity and occasionally
virilization may be observed in the juvenile variety
- characteristic histologic features: modular architecture, follicle formation, microcysts, cell necrosis, and increased mitotic activity
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OvariesSertoli-Leydig Cell Tumor
- also a sex cord stromal tumor; accounts for 2% of pediatric ovarian tumors
- rare in prepubertal girls
- presents with evidence of virilization
- Treatment:unilateral salpingo-oophorectomy for stage I disease
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OvariesOvarian Follicular Cysts
- occur from birth to puberty and usually disappear spontaneously
- UTZ:cyst usually presents as a nonechogenic area, frequently larger than 20 mm at its greatest diameter; diffuse swelling of the ovarian parenchyma and follicular enlargement of the cortical zone are also noted
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OvariesPolycystic Ovarian Syndrome (PCOS)
- presents with menstrual dysfunction and signs and symptoms of androgen excess often at the time of puberty
- menstrual irregularity can range from amenorrhea to oligomenorrhea
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OvariesFunctional and Hemorrhagic Ovarian Cysts- cysts are an integral part of follicular
development during the menstrual cycle- Following ovulation, it is termed a corpus
hemorrhagicum- are often symptomatic and best evaluated by
ultrasonography- Monophasic oral contraceptives can facilitate
suppression of functional cysts
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OvariesOvarian Torsion- a complication that should always be
considered in the differential diagnosis of ovarian tumors or abdominal pain in a female patient
- often presents with intermittent sharp abdominal pain, radiates down the ipsilateral extremity
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OvariesOvarian Torsion- when unilateral torsion is diagnosed,
oophoropexy (plication) of the contralateral adnexa may be indicated
- Incidence in one series was 25% (16 of 63 benign ovarian masses)
- often be evaluated and managed with the use of Doppler flow studies and laparoscopy
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OvariesOvarian Torsion
-underlying cause: associated ovarian cyst or neoplasm
-managed conservatively
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OvariesAutoamputation of the Ovary
- presents as a small, calcified, free-floating mass associated with absent adnexa
- may be asymptomatic
- hypothesized that antenatal or subclinical ovarian torsion leads to necrosis, calcification, and separation of the adnexa from its blood supply
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OvariesJuvenile Granulosa Cell Tumors of the Ovary
(JGCT)- 1-2% of all ovarian tumors- median age of presentation is 7.6 yr, with a range of
6 mo-17.5 yr- Majority presents with abdominal distention and
sexual precocity- better prognosis with multidrug chemotherapy- neurotoxicity, especially ototoxicity, and bone
marrow depression are serious complications
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Cervix• prevalence of dysplasia and carcinoma in situ is
18.8/1,000 for those 15-19 yr of age• biopsy-proven cases of all grades of cervical
intraepithelial neoplasia (CIN) in the teenage population have a prevalence of 13.3/1,000
• Bethesda Classification System with the Papanicolaou (Pap) smear is widely used for diagnosis
• overall frequency of abnormal Pap smear results in the adolescent population is 3.8%
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Cervix• 1% of all abnormal Pap smears are associated
with CIN• Papillomavirus infectionand altered vaginal flora
are consistent findings in patients with CIN• Colposcopic examination is essential when CIN
is diagnosed in an adolescent• Other pathologic abnormalities:cervical polyps
and mixed mesodermal tumors
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UTERUS (BENIGN AND MALIGNANT TUMORS OF THE UTERINE CORPUS)
Adenocarcinoma of the corpus - are in children and adolescents- frequent presenting sign: vaginal bleeding not
associated with sexual precocity- Treatment: hysterectomy, with removal of the
ovaries, followed by adjunctive radiotherapy or chemotherapy or both, depending on the operative findings
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UTERUS (BENIGN AND MALIGNANT TUMORS OF THE UTERINE CORPUS)
Mixed mesodermal tumors and leiomyomas of the uterus
-included in the differential diagnosis of a pelvic mass in an adolescent
Leiomyosarcoma
-extremely rare, has also been noted in an adolescent
-abnormal vaginal bleeding usually is present
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Vagina
Gartner duct (mesonephric) cyst
- a common vaginal wall abnormality
- usually is an incidental finding; requires no specific therapy
- excision may be necessary if there is associated dyspareunia
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Vagina
Paramesonephric (müllerian) duct cysts- often become symptomatic at menarche when the
cavity fills with menstrual blood- Women exposed to diethylstilbestrol (DES) in
utero have a high incidence of adenosis of the vagina and cervix
- potential reproductive abnormalities:infertility, habitual abortion, and tubal and uterine cavity abnormalities
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Vagina
Clear cell adenocarcinoma of the vagina and cervix
-rare sequela of DES exposure in uteroSarcoma botryoides
- vaginal carcinoma that occurs primarily in pediatric patients
- best treated by surgical excision
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Vulvovaginal and Müllerian Anomalies
Imperforate Hymen
- presents with primary amenorrhea; secondary sex characteristics are normal
- requires surgical interventionCongenital Absence of the Vagina (Mayer-
Rokitansky-Küster-Hauser Syndrome)
-incidence: 1/4,000 to 1/5,000 births
-etiology is unknown
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Vulvovaginal and Müllerian Anomalies
Congenital Absence of the Vagina (Mayer-Rokitansky-Küster-Hauser Syndrome)
-characterized by primary amenorrhea, normal vulva, duplication anomaly of the uterus, attenuated fallopian tubes, normal ovaries, normal female karyotype and phenotype
-associated anomalies: renal and skeletal
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Vulvovaginal and Müllerian Anomalies
Congenital Absence of the Vagina (Mayer-Rokitansky-Küster-Hauser Syndrome)
- treatment: delayed until the patient is ready to be sexually active; frank dilators, combined laparoscopic vulvar procedure, McIndoe procedure involving a split-thickness skin graft placed in the vagina
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Incomplete Vertical Fusion of the Vagina
-Transverse and longitudinal vaginal septa represent failure of complete canalization of the vagina
-presents with amenorrhea and cyclical pain, which is a result of cryptomenorrhea
-usually asymptomatic until puberty, hydrometrocolpos may occur in children
-most common site of the septum is between the middle and upper thirds of the vagina
-patients have a functional uterus, although their fertility is often compromised
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Incomplete Vertical Fusion of the Vagina
-prognosis is worse for higher obstructions
-increased incidence of endometriosis secondary to retrograde menstruation
-treatment:surgical resection of the obstruction from below
-anastomosis of the upper and lower segments should be attempted to prevent stenosis
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DISORDERS OF LATERAL FUSION
• Include anatomic variations of nonobstructive longitudinal septum
• obstructed hemivagina- associated with a didelphic uterus and often with a pelvic mass
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UTERINE ABNORMALITIES
• Incidence ranges from 1/100 to 1/1,000• may present with primary amenorrhea or with
irregular or even regular menses• pregnancy wastage and infertility may cause the
first suspicion of a uterine anomaly• diagnosis should include a pelvic ultrasound
examination, renal tract assessment, and skeletal inspection for anomalies
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CONGENITAL ATRESIA OF THE UTERINE CERVIX
• extremely rare anomaly often presents at puberty with cryptomenorrhea, amenorrhea, and pelvic pain
• associated with significant renal anomalies in 5–10% of patients
• complete absence of a cervix but a palpable uterus is found
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COMPLETE VULVAR DUPLICATION
• rare congenital anomaly presents in infancy
• consists of 2 vulvas, 2 vaginas, and 2 bladders, a didelphic uterus, a single rectum and anus, and 2 renal systems
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LABIAL HYPERTROPHY
• surgical revision may be indicated if there are symptoms of discomfort or bulging is noted when wearing tight-fitting garments
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CLITORAL ABNORMALITIES
• Agenesis of the clitoris is rare
• Clitoral duplication- associated with pelvic organ abnormalities, including agenesis of other genital tract structures and bladder exstrophy
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HYMENAL ABNORMALITIES
• often associated with mucocolpos
• Other abnormalities include cribriform or stenotic hymen
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CLOACAL ANOMALIES
• represent a common urogenital sinus into which both urethral and anal orifices exit
• the single opening (cloaca) requires surgical correction
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Special Gynecologic Needs
• Mentally Handicapped Children
-face several gynecologic issues including varying levels of understanding of reproduction, sexuality, and contraception
-perineal hygiene is also of concern
-cyclical behavioral changes (crying, self-abusive behavior, and tantrums) prior to menstruation are common
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Special Gynecologic Needs
• Mentally Handicapped Children
-changes are presumed to result from pain or cramps as 65% of the patients responded to nonsteroidal anti-inflammatory drugs (NSAIDs)
-treatment with NSAIDs unsuccessful: birth control pills and depo-medroxyprogesterone acetate improved behavior in 40–66% of patients
-SSRIs are also effective
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Special Gynecologic Needs
• Mentally Handicapped children
-sexual abuse is a major concern
-as many as 25% of mentally impaired women have been sexually assaulted
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Gynecologic Imaging
• Ultrasonography is a key screening tool, enabling appropriate diagnosis in patients presenting with ambiguous genitalia, ovarian or uterine masses, primary amenorrhea, and abdominal or pelvic pain
• Ovarian cysts and hydrocolpos or hydrometrocolpos are the most common abnormalities noted in neonates
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Gynecologic Imaging
• Hydrocolpos- dilatation of the vagina, which usually is associated with accumulation of serous fluid or urine (if there is a urogenital sinus)
• Hydrometrocolpos- dilatation of both the uterus and the vagina; may also be associated with vaginal or cervical atresia, stenosis, or an imperforate hymen
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Gynecologic ImagingTABLE 556-1 -- Normal Ovarian and Uterine Dimensions
OVARY• Birth• 15 mm long, 3 mm wide, 2.5 mm thick Ovarian volume 0.7 cm3[*]• Postpuberty• 22.5–50.0 mm in length 1.5–3.0 cm in width; 0.6–1.5 cm in
thickness Ovarian volume 1.8–5.7 cm3[*]• UTERUS• Neonate• Length 2.3–4.6 cm Anteroposterior diameter 0.8–2.2 cm• Infant to 7 yr of age• Length 2.5–3.3 cm Anteroposterior diameter 0.4–1.0 cm• Postpuberty
• Length 6 cm