Textbook Of Gynecology. Dc Dutta’s

Chapter 32. Gynecologic Problems from Birth to Adolescence

INTRODUCTION

Those who are familiar with adult gynecology are often confused and embarrassed to deal with gynecologic problems of pediatric and adolescent group. The problems are peculiar to this particular period.

Pediatric and adolescent gynecology encompasses gynecologic diseases of children from birth to adolescence. It covers a spectrum of gynecological problems including congenital anomalies, problems due to infection (vulvovaginitis), precocious development, menstrual abnormalities and neoplasm. To prevent the problems of teenage pregnancy and sexually transmitted infections, contraceptive counseling has a place. Children are mostly accompanied by the parents for the problems.

For descriptive purpose, the entire span from birth to adolescence is arbitrarily divided into three phases. Each phase has got distinct problems of its own.

Neonates, toddlers and infants <5 years.

Premenarchal 5-11 years.

Perimenarchal to adolescence 12-18 years.

Gynecologists need specific communication skill considering the psychological and developmental milestones of the girl child and the adolescent.

NEONATES, TODDLERS AND INFANTS

CLITORAL ENLARGEMENT

Causes: Not infrequently, female babies are born with slightly enlarged clitoris. There is usually no evidence of intrauterine androgen stimulation of the child. Careful history taking reveals development of some degree of masculinizing features of the mother during pregnancy, probably due to increased adrenocortical activity (Fig. 32.1).

TABLE 32.1  COMMON CLINICAL PROBLEMS IN DIFFERENT PERIODS

Period

Common problems

< 5 years (Neonates, toddlers and infants)

 Diagnosis of sex at birth (Clitoral enargement)

 Genital crisis

 Labial adhesions

 Imperforate hymen

- Hydro or mucocolpos

 Ectopic anus

 Nipple discharge

5-11 years (Premenarchal)

 Vulvovaginitis in childhood

 Abnormal vaginal discharge

 Vaginal bleeding

 Precocious puberty (see p. 51)

 Trauma to the genital tract (Ch. 26)

 White lesion of the vulva (Ch. 17)

 Neoplasm (Ch. 23)

 

12-18 years (Perimenarchal to adolescence)

 Menstrual abnormalities (see p. 54)

 Delayed puberty (see p. 54)

 Hirsutism (see p. 571)

 Neoplasm (Ch. 23)

 Primary amenorrhea (p. 450)

 Leucorrhea (see p. 551)

 Congenital anomalies of the vagina (vaginal anomalies (see p. 41) and uterine anomalies (see p. 44).

 Intersex (see p. 439)

 Miscellaneous problems

Fig. 32.1 : Newborn baby with enlarged phallus due to adrenogenital syndrome causing confusion in the diagnosis of sex

Rarely, the enlargement may be one of the manifestations of intersexuality of type, female pseudohermaphrodite. This may cause confusion in the determination of sex at birth. The details have been mentioned in Chapter 27 (Fig. 27.1).

Management: In the idiopathic group, no treatment is required. However, an apparently female child with enlarged phallus and impalpable gonads requires at least estimation of serum 17-OHP, urinary 17 ketosteroid and chromosomal analysis to rule out congenital adrenal hyperplasia (see p. 440) (Fig. 32.1).

GENITAL CRISIS: It includes spectrum of disorders noticed within few days of birth. It is due to the effect of passive estrogenic stimulation, which has passed across the placenta from the mother. The presence of the estrogen so obtained produces changes in the endometrium and other target tissues such as breasts and cervical glands.

Bleeding per vaginum: It usually occurs within 10 days following birth; mostly blood stained but, at times, frank bleeding. This is due to decline in level of estrogen, which is unable to support the endometrium, resulting in withdrawal bleeding. There is no cause for concern.

Enlarged breasts: Due to effect of maternal estrogen and progesterone, there may be some development of duct and alveolar system of the breasts. Withdrawal of hormonal suppression of prolactin leads to discharge from the nipple called ‘witch’s milk’. No treatment is required; only assurance is enough.

Neonatal leucorrheaThis is due to excessive secretion of cervical mucus from the hypertrophied cervical glands under the influence of estrogen.

Labial adhesion (Adhesive vulvitis) is the condition when the Labia minora have adhered together.

Causes: Commonly, it is due to mild infection of the vulva which is favoured by lack of local defense due to absence of estrogen. There is denudation of the surface epithelium of the labia minora → adhesions.

The adhesions of the labia minora start from behind forward leaving a small opening at the foremost tip through which urine escapes out.

Rarely, it may be a manifestation of minor form of masculinization following maternal intake of androgen during pregnancy.

Diagnosis: The condition usually appears within 2-3 years of birth. The mother usually, anxious about the entity, brings to the notice of the physician for not visualizing the vaginal opening. The adhesions may cause difficulty in micturition or periodic attacks of urinary tract infection. There may be inflammation of the vestibule and vagina.

Examination reveals adhesions of labia minora obliterating the vaginal opening and, at times, even the external urethral meatus (Fig. 32.2A). A thin vertical line in midplane is pathognomonic for this adhesive vulvitis.

Confusion arises with:

Imperforate hymen

Agenesis of vagina Intersex.

In imperforate hymen and agenesis of vagina, the labia minora and external urethral meatus are clearly visible. In intersexuality, there is usually associated clitoral enlargement (see p. 440).

Fig. 32.2A: Labial adhesions in a TX-year-old girl

Fig. 32.2B: Separation of adhesions by fingers. Local application of estrogen ointment is also helpful

Treatment: Separation of the adhesions using fingers or by a probe is almost always effective (Fig. 32.2B). The raw area is treated with topical application of estrogen or any other antibiotic ointment to prevent reagglutination. Good perineal hygiene should be maintained.

There is a tendency of recurrence. If it does, in spite of repeated separations, it is better to await spontaneous cure at puberty because of high-level endogenous estrogen.

MUCO OR HYDROCOLPOS

Pathophysiology: There is imperforate hymen or a transverse vaginal septum just above the hymen. Due to excess estrogen stimulation acquired in utero from the mother, there is increased secretion of mucus or watery discharge from the cervical and uterine glands. If a large quantity of fluid is collected in the vagina, it produces hydro or mucocolpos. It may rarely be big enough to produce an abdominal swelling.

This is rarely met beyond 1 year of age because the uterine and vaginal transudation is not produced in sufficient quantity beyond that age.

Later on, the candidate may be the subject of hematocolpos → hematometra.

Clinical features

There are usually some urinary problems to the extent of retention of urine. Variable degrees of pain in lower abdomen are a constant feature.

Abdominal examination reveals a lower abdominal lump. Distended bladder makes the lump apparently bigger.

Vulval inspection reveals a tense bulge of the obstructing membrane which looks shiny. Rectal examination confirms the vaginal bulge.

TreatmentCruciate incision is enough to drain the pent- up mucus. Antibiotic is given. Head end is to be raised.

OVARIAN ENLARGEMENT: After the withdrawal of maternal estrogen, there is transient elevation of gonadotropins in neonates. This influence of elevated gonadotropins can stimulate to produce ovarian follicular cysts. These usually regress spontaneously. As such, the cysts need no surgery unless complicated. ECTOPIC ANUS: The anal canal may open either in the vestibule or in the vagina. The details have been described in Chapter 4.

PREMENARCHAL

VULVOVAGINITIS: The premenarchal girls are especially vulnerable to vaginal infection because of:

 Lack of or very low level of circulatory estrogen → lack of stratification of vaginal epithelium → lack of glycogen and absence of Doderlein’s bacillus → no acid formation → vaginal pH remains high, around 7.

 Inadequate perineal hygiene.

 Lack of protective pubic hair and fatty pads of labia majora.

As the vaginal infection is almost always associated with vulvitis, the terminology of vulvovaginitis is appropriate.

Causes: The infection may be due to nonspecific or specific organisms.

I. The nonspecific organisms (common)

The infection is polymicrobial in nature and it is difficult to pinpoint any particular one responsible for infection. The organisms are, however, of low virulence.

II. Specific

 Nongonococcal (common)

• Streptococcus • Chlamydia trachomatis

• E. coli • Gardnerella vaginalis

• Candida albicans

• Trichomonas vaginalis

 Gonococcus

III. Foreign body

IV. Threadworm infestation

V. Following systemic illness

• Viral infection like measles, chickenpox

• Juvenile diabetes

• Heres simplex

VI. Following antibiotic therapy

VII. Skin conditions

• Lichen sclerosus

• Psoriasis

• Eczema

Sources of infection

• Direct contact with infected person.

• Indirect from foreign body, infected towel or bath tub and intestinal infestation.

• Associated juvenile diabetes or antibiotic therapy favors monilial infection.

Symptoms

• Vaginal discharge: Purulent or blood-stained in the presence of foreign body.

• Pruritus or soreness in external genitalia.

• Painful urination.

• Vaginal bleeding.

Signs: Vulva becomes edematous and red or even ulcerated. Vaginal inspection using aural speculum reveals congested epithelium with pent-up discharge.

The offending foreign body may be detected. The examination may be done under anesthesia. It should be remembered that the vaginal epithelium in young girls looks red. Rectal examination is often helpful to detect the foreign body.

Investigations:

• Examination under anesthesia

• Vaginoscopy is needed to visualize the upper vagina for bleeding, foreign body or neoplasm. Specially designed speculum (Huffman Graves) is inserted into the vagina. For better visualization, water cystoscope (to wash away secretions, debris or blood) or laparoscope (8 mm) may be used.

Bacteriological examination of the discharge either by gram stain or hanging drop preparation or culture, to identify the causative organism (see p. 166).

• Smear from the anal area for detection of pin or threadworm.

• Stool examination may reveal the threadworm.

• Blood examination for estimation of sugar in suspected cases of juvenile diabetes.

• Urine for protein, sugar and culture study.

TreatmentAs the cause remains obscure in majority, the principles to be followed are:

• Vulvar hygiene—Proper wiping will reduce rectal flora in the vulvovaginal area.

Sitz baths are very helpful in relieving symptoms (baking soda in water).

Scrubbing the vulvar area is avoided.

To keep the local area dry.

To wear cotton undergarments.

Medication

• To reduce the overgrowth of pathogenic bacteria, Amoxicillin 20-40 mg/kg/day in 3 divided doses is effective.

• In refractory cases, estrogen locally as cream twice daily for 3 weeks is effective to improve the vaginal defense and to promote healing.

Specific therapy

• Trichomoniasis is treated by metronidazole (100 mg thrice daily for 10 days).

• Monilial infection is treated by local application of clotrimazole 1 percent cream.

• For gonococcal vaginitis (see p. 147).

• Associated systemic illness should be treated by intramuscular antibiotic therapy.

• Foreign body is to be removed followed by use of estrogen therapy.

• Helminth is eradicated by oral use of Albendazole.

LEUCORRHEA (See P. 551): As the puberty approaches, there may be excessive white discharge per vaginum. It is nonoffensive and nonirritant. It is due to excessive production of mucus from the cervical glands and increased transudation from the vaginal epithelium.

NEOPLASM: The neoplastic conditions encountered during this period are usually ovarian in origin and rarely from the cervix and vagina. The ovarian neoplasms (see p. 383) are malignant in about 25 percent. Germ cell tumors are common (70%). Granulosa cell tumor is estrogen-producing tumor and may cause precocious puberty. Mixed germ cell tumor is highly malignant and dysgerminoma is intermediary in position, provided the capsule remains intact. The benign tumors are cystic teratoma (30%) and epithelial tumors (see p. 291, 293).

Sarcoma botryoides: It should be remembered that the entity is most often present as early as 2 years of age (see p. 369).

VAGINAL BLEEDING—Common causes are:

• Precocious puberty/Isolated menarche.

• Foreign body in the vagina.

• Trauma/Infection.

• Sexual abuse.

• Neoplastic conditions (mentioned earlier).

• Leech bite.

• Prolapse of the urethral mucosa.

Leech bite: This is more prevalent in tropics where pond bath is quite common. The bleeding may, at times, be brisk and requires varying amount of blood transfusion. Bleeding usually stops spontaneously but may, at times, require hemostatic suture.

Prolapse of the urethral mucosa: it presents as a vascular swelling surrounding the external urethral meatus which bleeds easily. Prolapse may be partial or complete (entire 360° urethra). Treatment is conservative. Local application of estrogen cream is found helpful. Surgery is needed rarely when necrosis is present.

POSTMENARCHAL TO ADOLESCENCE

ADOLESCENCE

The period of life beginning with the appearance of secondary sex characters and terminating with cessation of somatic growth is described as adolescence. The problems during the period are:

 Menstrual disorders.

 Delayed puberty (see p. 54).

 Delayed manifestations of intersex.

 Hirsutism.

 Leucorrhea.

 Neoplasm.

MENSTRUAL DISORDERS: The neurohormonal mechanism essential for maintenance of normal menstruation takes some time (usually 2-3 years) to come to a normal balance. Till then, various types of menstrual abnormalities may occur, causing concern to the young girls or their parents (see p. 54).

Common Causes of Menstrual Irregularity

A. Hypothalamic-Pituitary-Ovarian (HPO) axis dysfunction

 Dysfunctional uterine bleeding (see p. 54)

 Stress (see p. 463)

 Obesity (see p. 573)

B. Endocrinopathies

 Thyroid dysfunction (see p. 456)

 PCOS (see p. 459)

 Prolactinoma (see p. 464)

 CAH (see p. 466)

Other Causes of Abnormal Vaginal Bleeding

C. Inflammatory

 Vulvovaginitis

 Endometritis

 PID

D. Traumatic

 Foreign body

 Sexual abuse

 Drug effects

E. Others

 Pregnancy (abortion problems)

 Bleeding disorders (Idiopathic thrombocytopenic purpura, see p. 185).

 Local—Polyps, neoplasms (see p. 285).

Management: Improvement of general health and assurance are enough in majority. It is expected that after a certain period of time, the menstrual cycles become normal with the onset of regular ovulation.

The unresponsive or problematic cases have been dealt with in appropriate chapters.

DELAYED MANIFESTATIONS OF INTERSEX: While majority of cases of intersex are diagnosed at birth, there are cases where the diagnosis is only revealed after puberty. These are:

 Mild degrees of congenital adrenal hyperplasia (see p. 440) with late manifestations of postpubertal hyperandrogenism (Fig. 33.4).

 Gonadal dysgenesis (see p. 441).

 Androgen insensitivity syndrome (see p. 443).

HIRSUTISM: Hirsutism is one of the manifestations of hyperandrogenism and often causes problems to the young girls. One should not forget to elicit iatrogenic cause of hirsutism following intake of androgenic steroids, corticosteroid or synthetic progestogens. The causes and management of hirsutism are discussed in p. 544.

LEUCORRHEA: Excessive normal vaginal secretion in this period may be due to:

 Relative hyperoestrogenic phase.

 Malnutrition and ill health.

 Congenital ectopy (erosion).

 Sexual excitement or masturbation.

 Vaginal adenosis.

VAGINAL ADENOSIS: Vaginal adenosis is present in about 30-50% of the teenagers who had DES exposure in utero. This is a benign condition. In these girls, the junction between the mullerian ducts and the sino-vaginal bulb may not be sharply demarketed. As the Mullerian elements invade the sino-vaginal bulb, remnants may remain as areas of adenosis in adult vagina. The columnar epithelium of the endocervix extends onto the ectocervix and also variable part of the vaginal fornices. There is thus copious vaginal secretion from the columnar epithelium. The pathology regresses spontaneously in due course of time. Rarely, it may progress to clear cell carcinoma (see p. 339).

Congenital ectopy producing copious discharge should be cauterized. In others, assurance and improvement of general condition cure the state.

Infective discharge during the period may be due to:

 Nonspecific infection following unhygienic use of menstrual pads or foreign body in the vagina.

 Specific infections such as Trichomonas vaginalis or monilial infection.

 New growths from the vagina or cervix.

The investigations and management have been mentioned earlier in this chapter.

NEOPLASM: Ovarian—Functional cysts which are rare in premenarchal period are quite common during this period. They are usually 6-8 cm in diameters and usually regress within 3-6 months. These are usually follicular cysts.

Commonest neoplastic cyst during this period is cystic teratoma (dermoid cyst). The others, though rare, are benign epithelial tumors, dysgerminoma, mixed germ cell tumor or androblastoma. Germ cell tumors constitute 50-75 percent of all ovarian neoplasms in this age group (see p. 381).

The patients usually come late with symptoms. Common symptoms are lump in the lower abdomen, acute pain abdomen or, at times, with retention of urine.

DiagnosisThe diagnosis is made by abdominal, bimanual vaginal or rectal examination. Ultrasound is an invaluable tool in the diagnosis of ovarian mass. CT, MRI may be needed in few cases of pelvic mass with uncertain diagnosis. Pre-operative work-up needs tumor markers estimation (serum CA125, a fetoprotein, hCG, inhibition, CEA and testosterone) in these adolescent girls.

Treatment: A suspected functional cyst (6-8 cm) may be observed for 3-6 months. Unilocular functional cysts usually resolve spontaneously. Surgical therapy is needed in cases where there are symptoms, masses that fail to resolve or masses with solid or multilocular appearance on ultrasound.

Laparoscopy is usually done when an adnexal mass appears to be benign. However, prompt laparotomy should be done when there are evidences of malignancy.

The surgery is usually conservative (ovariotomy or ovarian cystectomy) considering her future fertility and endocrine functions. Adolescent groups usually have borderline epithelial or germ cell tumors. In such a situation, the affected ovary is removed and a formal staging is done (fertility sparing surgery). Unilateral salpingo-oophorectomy is justified for most young girls with stage-I (Stage IA or IB) (see p. 380), Grade 1 or 2 disease. Germ cell tumors are highly responsive to chemotherapy (see p. 384). However, if the capsule is ruptured, radical surgery, i.e. total hysterectomy with bilateral salpingo-oophorectomy is to be done followed by radiotherapy or chemotherapy.

TABLE 32.2 COMMON CAUSES OF LUMP IN LOWER ABDOMEN

<5 years

— Hydro or mucocolpos

5-11 years

— Retention urine due to vulvovaginitis

— Pelvic neoplasm

12-18 years

— Pregnancy

— Retention of urine

— Ovarian tumor

— Hematometra

— Encysted peritonitis

A problem may arise when an apparently cystic epithelial benign ovarian tumor is removed, which ultimately proves malignant histologically. In such cases, in consultation with an oncologist,

MISCELLANEOUS PROBLEMS

chemotherapy followed by relaparotomy and removal of uterus with contralateral tube and ovary may be done. This should be followed by chemotherapy.

Alternatively, the patient may be treated with chemotherapy alone with follow-up.

Uterus—Sarcoma botryoides (see p. 369).

Vagina—Clear cell adenocarcinoma of the vagina (see p. 339).

SEXUALLY TRANSMITTED DISEASES (STDs)

STDs are increasing among the adolescents. The younger the age of first intercourse, the higher the risk for STDs. Chlamydia infection, though most common, other infections are HPV (see p. 323), HIV (see p. 153), Gonorrhea (see p. 147), Hepatitis B, Syphilis (see p. 148) and others (see Ch. 11). Impact on health due to STD complications include: cervical metaplasia, PID and its consequences (see p. 132).

Major problems faced with the management of adolescent STDs are denial of history and symptoms. Fear and social embarrassment cause delay or, at times, incomplete treatment.

Treatment

Sex education among the adolescent girls, practice of safer sex and maintenance of perineal hygiene are essential. Proper use of condoms protects STDs. Treatment of STDs among the adolescents is the same that for the adults (see p. 148).

ACNE: This is of concern due to cosmetic reason. The cause is due to excess androgen secretion by the adrenals. Assurance is enough. No treatment is required. It passes off spontaneously.

OBESITY: Obesity is best assessed by calculating body mass index (BMI). BMI is expressed (see p. 666) as weight (kg) divided by the height squared (M2). Ideal BMI should be between 20 and 24. BMI 25 or more is called over weight, Whereas BMI 30 or more is considered obese. Obesity increases the risk of cardiovascular (e.g. hypertension) and metabolic (e.g. diabetes) diseases (see p. 573).

Usually, it is due to overeating and constitutional. Rarely, it may be due to hypofunction of pituitary or manifestation of Cushing’s syndrome or PCOS (see p. 459) even at a younger age.

ABNORMAL HEIGHT: Apart from constitutional as found in premenarchal period, abnormal tallness is due to:

 Hypersecretion of the growth hormone from the anterior pituitary. This may be due to pituitary eosinophilic adenoma resulting in gigantism. The treatment is excision of the pituitary adenoma. If the features are well-established, it is, however irreversible.

 Primary ovarian failure—There is lack of endogenous estrogen → delayed closure of the epiphysis of long bones. There is associated unopposed action of the growth hormone from the anterior pituitary resulting in linear growth of the long bones (see p. 463).

KEY POINTS

Undue clitoral enlargement seen at birth may be due to intersexuality. It requires at least estimation of 17-OHP, 17 ketosteroid and chromosomal analysis. Levels of 17-OHP >800 ng/dL are virtually diagnostic of congenital adrenal hyperplasia (21-hydroxylase deficiency).

Genital crisis is due to hyperestrogenic state and includes bleeding per vaginum, enlarged breasts and neonatal leucorrhea (see p. 543).

Labial fusion is commonly due to infection and rarely a feature of intersexuality. Separation of the adhesions is effective using fingers or a probe (see p. 543).

> Muco or hydrocolpos is usually found within 1 year of age and results from imperforate hymen.

The childhood vulvovaginitis is mainly due to poor perineal hygiene. Vulvovaginitis in premenarchal period is mostly due to non-specific organisms and occasionally to specific gonococcal infection. Bacteriological examination should be carried out from the discharge prior to therapy.

Ovarian follicular cysts are common in adolescent girls and are usually self-limiting. An ovarian enlargement more than 5 cm needs to be investigated.

The neoplasm in premenarchal period is usually ovarian and, in about 25 percent, it is malignant. The common type is germ cell tumor (benign cystic teratoma, dysgerminoma, mixed germ cell tumor). The others are granulosa cell tumor or epithelial tumors. The benign tumors are epithelial tumors.

Sarcoma botryoides is most often observed before the age of 8 (see p. 369).

> In a tall girl—to achieve arrest of bone growth, estrogen therapy for 3-6 months may be effective.

The menstrual disorders in adolescent period are usually self-limiting and the hormones should not be used injudiciously. Assurance and improvement of health are enough in majority.

Vaginal adenosis is present in about 30-50 percent and is a benign lesion. Rarely, it progresses to clear cell carcinoma.

Commonest ovarian neoplasm in adolescence is benign cystic teratoma (see p. 293).

STDs are increasing among the adolescents. Sex education, use of condoms and treatment of STDs are essential (see p. 549).

> Obesity is best assessed by calculating body mass index (BMI) (p. 666).



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