Pediatric Gynecology

Ambiguous genitalia: look for adrenogenital syndrome and congenital adrenal hyperplasia, which usually are due to 21-hydroxylase deficiency (90% of cases). Patients are female; males with this disease show precocious sexual development. Patients with 21-hydroxylase deficiency have salt-wasting (low sodium levels), hyperkalemia, hypotension, and elevated 17-hy-droxyprogesterone. Treat with steroids and IV fluids immediately to prevent death. No patient with ambiguous genitalia should be assigned, a gender until the work-up is complete. A karyotype must be done.

Important points:

1. Any child with a "bunch of grapes" protruding from her vagina probably has sarcoma botryoid.es, a malignant tumor

2. Premature or precocious puberty is usually idiopathic but may be caused by a hormone-secreting tumor or central nervous system disorder, which must be ruled, out. By definition,

Che patient must be younger than 8 (9 for males). Treat underlying cause or, if idiopathic, treat with gonadotropin-releasing hormone analog to prevent premature epiphyseal closure and to arrest or reverse puberty until appropriate age.

3. Most cases of vaginitis or vaginal discharge are nonspecific or physiologic. But look for foreign body, sexual abuse (especially with sexually transmitted disease), or candidal infection (as a presentation of diabetes; measure serum glucose and/or check for glycosuria).

4. Imperforate hymen: patient of menarche age with hematocolpos (blood in vagina) that cannot escape (hymen bulges outward). Treatment is surgical opening of the hymen.

5. Vaginal bleeding in the neonate is usually physiologic as a result of maternal estrogen withdrawal and resolves by itself.

Important points:

1. The .most common cause of secondary amenorrhea is pregnancy. Aimys do a pregnancy test first when a patient presents with amenorrhea. Pregnancy also must be ruled out as a cause of primary amenorrhea.

2. A woman may say that she is taking oral contraceptives and still be pregnant. No contraception is 100% effective, especially when you factor in poor compliance.

3. Signs of pregnancy: amenorrhea, morning sickness, Ilegar's sign (softening and compressibility of die lower uterine segment), Chadwick's sign (dark discoloration of the vulva and vaginal walls), linea nigra, chloasma, auscultation of fetal heart tones, visibility of gestational, sac and/or fetus on ultrasound, uterine contractions, weight gain, and palpation/ballottemetiL of fetus.

4. Give all pregnant patients folate to prevent neural tube defects. Ideally, all women of reproductive age should take folate, because it is most effective in the first trimester when most women do not know that they are pregnant. Iron is often given routinely to prevent anemia.

5. Macrosomia (or positive history in previous children) is caused by maternal diabetes meliitus until proved otherwise.

Routine laboratory tests in a pregnant patient:

1. Pap smear: give to every patient at first visit, unless she had a normal Pap smear in past 6 months.

Z. Urinalysis: at first visit and every visit (screen for preeclampsia and bacteriuria; not a good screen for diabetes meliitus).

3. Complete, blood, count: at first visit to see if the. patient is anemic (pregnancy may aggra vate it).

4. Blood type, Rh type, and antibody screen: at first visit (for identification of possible isoimmunization.).

5. Syphilis test: at first visit (mandated in most states) and subsequent visi ts if the patient is at high risk.

S. Rubella antibody screen: in the absence of a good vaccination history, obtain at: first visit (otherwise not needed).

7. Glucose screen: at first visit if the patient has risk factors for diabetes mellitus (obesity, family history, age >30 years); otherwise, do at 24—28 weeks. Screen with fasting serum glucose and serum glucose 1 or 2. hours after an oral glucose, load.

8. Serum alpha-fetoprotein (AFP) or triple screen: between 1 6-20 weeks for older or other high-risk patients. Positive triple screen (low AFP, low estriol, and high human chorionic gonadotropin [HCG]) means likely Down syndrome.

9. Hepatitis B serology, tuberculous skin test, HIV test, Chlamydia sp. and gonorrhea cultures, and ultrasound are used only when the patient has a suggestive history' or risk factors. If asked , you should do Chlamydia sp. and gonorrhea cultures for any pregnant teenager.

Important points:

1. At every prenatal visit, listen for fetal heart tones and evaluate uterine size for any size/date discrepancy. Uterine size is evaluated by measuring the distance from the symphysis pubis to the top of the fundus in centimeters. Between roughly 20-35 weeks, the mea surement in cm should equal the number of weeks of gestation. A discrepancy greater than 2-3 cm is called a size/date discrepancy, and ultrasound should be done to evaluate further. Possible explanations include intrauterine growth retardation and multiple gestation.

2. At 12 weeks' gestation, the uterus enters the abdomen; at roughly 20 weeks, it reaches the umbilicus.

3. Between 16 and 20 weeks, ultrasound is most accurate at estimating fetal age (using the biparietal diameter),

Hydatidiform mole: in a sense, the products of conception become a tumor. Look for preeclampsia before the third trimester; an HCG that does not return to zero after delivery or abortion or that rapidly rises during pregnancy; first- or second-trimester bleeding with possible expulsion of "grapes;" uterine size/date discrepancy ; and/or a "siiofr-stonn" pattern on ultrasound. Complete moles are 46 XX (all chromosomes from the father) and have no fetal tissue; incomplete moles are usually 69 XXY and contain fetal tissue. Gross appearance suggests a bunch of grapes. Treat with uterine dilatation and curettage, then follow HCG until it falls to zero. If HCG does not fall to zero or rises, the patient has either an invasive mole or choriocarcinoma; in either case, the patient needs chemotherapy (usually methotrexate or actinomycin D),

Intrauterine growth retardation (IUGR): defined as size below the tenth percentile for age. The causes are many and are best understood in broad terms as caused by one of three factors: maternal (e.g., smoking, alcohol or drugs, lupus erythematosus), fetal (e.g.,TORCH infections, congenital anomalies) or placental (e.g.. hypertension, preeclampsia), TORCH infections consist of toxoplasmosis, other (congenital syphilis and viruses), rubella, cytomegalovirus, and herpes simplex virus. Do ultrasound on all patients who have a size/date discrepancy greater than 2-3 cm or risk factors for pregnancy problems (e.g., hypertension; diabetes mellitus; renal disease; lupus erythematosus; cigarette, alcohol, or drug use; history of previous problems). Ultrasound parameters measured for IUGR determination include biparietal diameter, head circumference, abdominal circumference, and femur length.

Eva! nation of fetal well-being:

1. Nomtress test (NST): with the mother resting, fetal heart rate tracing is obtained for 20 minutes. A normal strip has at least two accelerations of the heart rate, each of which is at least 15 bpm above baseline and lasts at least 15 seconds. This is the first screening test to evaluate fetal well-being; it is often done in the context of a biophysical profile.

2. Biophysical profile (BPP): includes lour measurements: ■ NST (see above).

0 Amniotic fluid index (API): measures vertical pockets of amniotic fluid (in cm) in each of the four quadrants.The sum of the highest vertical pocket in eacfi quadrant is used to determine whether oligohydramnios or polyhydramnios is present (AFI < 5 cm ~ oligohydramnios, API > 25 cm = polyhydramnios).

m Fetal breathing movements: fetus should have at least 30 breathing movements in 10 minutes.

«Fetal movements: fetus should have at least three body movements (e.g., flexion, body rotation) in 10 minutes.

Note: If the fetus scores low on the BPR the next test is the contraction stress test. With high-risk pregnancies (e.g., IUGR, diabetes mellitus, hypertension, alcohol or drug use, postterm pregnancy, history of problem pregnancies, maternal or physician concern), the BPP often is done once or even twice a week until delivery.

3. Contraction stress test (CST): a test for uteroplacental dysfunction. Give oxytocin, and monitor the fetal heart strip. If late decelerations are seen, on the fetal heart strip with each contraction, the test is positive, and usually a cesarean section is done.

Note: In women with antiphospholipid antibodies and previous problem pregnancies, low-dose aspirin may help in subsequent pregnancies. Normally, aspirin and other NSAIDs should be avoided in pregnancy; use acetaminophen instead.

Postterm pregnancy: > 42 weeks' gestation. Generally, if gestational age is known to be accurate, labor is induced (e.g., by oxytocin) if the cervix is favorable. If the cervix is not favorable or the dates are uncertain, do twice-weekly NST and BPP. At 43 weeks, most authorities advise induction of labor or cesarean section. Both prematurity and postmaturity increase perinatal morbidity and mortality. Prolonged gestation is common in association with anencephaly and placental sulfatase deficiency.

Normal pregnancy changes: nausea and vomiting (morning sickness), amenorrhea, heavy (possibly even painful) feeling of the breasts, increased pigmentation of the nipples and areolae (and Montgomery tubercles), backache, linea nigra, chloasma, striae gravidarum, mild ankle edema, heartburn, and increased frequency of urination.

Alpha-fetoprotein levels:

a Low AFP = Down syndrome, fetal demise, or inaccurate dates.

a High. AFP = neural tube defects (e.g., anencephaly, spina bifida), ventral wall defects (e.g., omphalocele, gastroschisis), multiple gestation, or inaccurate dates.

m If AFP or triple screen is positive (at 16-20 weeks), the patient should undergo amniocentesis (also done at S 6-20 weeks) for a definitive diagnosis of chromosomal disorders (cell culture) or neural tube defects (amniotic fluid AFP).

Chorionic villus sampling (CVS): can be done at 9-12 weeks (earlier than amniocentesis) and generally is reserved for women with previously affected offspring or known genetic disease. CVS gives women the advantage of first-trimester abortion if a fetus is affected. It is associated with a slightly higher miscarriage rate than amniocentesis and. cannot detect neural tube defects.

Teratogenic agents (see table, top of next page)

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Important points:

1. Hyperglycemia and diabetes mellitus cause cardiovascular anomalies, cleft lip/palale, caudal regression, neural tube defects, left colon hypoplasia, and macrosomia (early dia betes) or microsomia (long-standing diabetes).

Z Radiation (> 5 cGy) causes IUGR, central nervous system defects, eye malformations, and future malignancies (especially leukemia).

3. Drugs that are generally safe in pregnancy: acetaminophen (not NSAIDs or aspirin), penicillin, cephalosporins, erythromycin, nitrofurantoin, 11, blockers, antacids, heparin, hydralazine, methyldopa, labetaiol, insulin, docusate.

4. Most TORCH intrauterine fetal infections can catise mental retardation, microcephaly, hydrocephalus, hepatosplenoraegaly, jaundice, anemia, low birth, weight, and/or IUGR:

■ Toxoplasma gondii: look for exposure to cats; specific defects include intracranial calcifications, chorioretinitis.

a Other: varicella zoster (limb hypoplasia and scarring of the skin.) and syphilis (rhinitis, saber shins, Hutchinson's teeth, interstitial keratitis, skin lesions).

m Rubella: worst in first trimester (some authorities recommend abortion if the mother contracts rubella in the first trimester), Always check antibody status on first visit if the patient has a poor immunization history. look for cardiovascular defects (patent ductus arteriosus, ventral septal defect), deafness, cataracts, and microphthalmia.

h Cytomegalovirus: most common; look for deafness, cerebral calcifications, microphthalmia.

sa Herpes: look for vesicular skin lesions (with positive Tzanck smears), history of maternal, herpes lesions.

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Note: With all in utero infections that can cause problems with the fetus, the mother may be asymptomatic (subclinical infection) and the infant may even be asymptomatic at birth, only to develop symptoms later (e.g., learning disability, mental retardation).

5. In untreated HIV-positive patients, transmission to the fetus occurs in roughly 25% of cases. With prenatal zidovudine (AZT) treatment for the mother and administration of AZ,T to the infant for 6 weeks after birth, HIV transmission is reduced to roughly 10%, A nomnfected infant may still be HIV-positive on testing because maternal antibodies can cross the placenta. Within 6 months, the test reverts to negative. HIV-positive mothers should not breast-feed because milk can transmit virus to the infant.

0. When the mother has genital herpes simplex, delay the decision of whether to do a cesarean section until t he mother goes into labor. If at the time of true labor she has lesions ofHSV, do a cesarean section. If at the time of true labor the mother has no HSV lesions, deliver vaginally.

7. If the mother has hepatitis B, give the infant, the first hepatitis B vaccine shot and hepatitis B immunoglobulin at birth.

8. If the mother gets chickenpox in the last 5 days of pregnancy or first 2 days after delivery, give the infant varicella zoster immunoglobulin,

9. In pregnancy, treat chlamydial infection with erythromycin (not tetracycline).

10. Signs of placental separation-, fresh show of blood from vagina; umbilical cord lengthens; the fundus rises and becomes firm and globular.

11. After a cesarean section with classical (vertical) uterine incision, the patient must have cesarean sections for all future deliveries because of the increased rate of uterine rupture. After a cesarean section with a lower (horizontal) uterine incision, the patient may deliver future pregnancies vaginally.

12. For the first several days after delivery, it is normal to have some discharge (lochia), which is red on the first few days and gradually turns to a white or yellowish-white color by day 10.. If the lochia is foul-smelling, suspect endometritis.

Breast-feeding:

1. If a woman does not want to breast-feed, prescr ibe tight-fitting bras, ice. packs, and analgesia. Bromocriptine and estrogens or oral contraceptives also may be used to suppress lactation.

2. If a woman does breast-feed, watch for mastitis, which usually develops in the first 2 months of breast-feeding. Breasts are red, indurated, and painful; often the patient has a low-grade fever. Staphylococcus aureus almost always is the cause. Treat by stopping breast feeding; obtain milk for culture and sensitivity; and begin antibiotic (penicillinase-resistant penicillin such as cloxacillin) for 7-10 days while awaiting culture results. Evaluate infant, for staphylococcal colonizat ion if given the option. If the breast is fluctuant, the condition may have progressed into an abscess, in which case incision and drainage are needed.

3. Breast-feeding is contraindicated in patients with HIV or hepatitis B and in patients who use the following: benzodiazepines, barbiturates, opiates, alcohol, caffeine or tobacco (in large amounts), antithyroid medications, lithium, chloramphenicol, anticancer agents, or ergot and its derivatives (e.g., methyscrgide).

Important points:

1. Epidural anesthesia is the preferred method in obstetric patients. General anesthesia involves a higher risk of aspiration and resulting pneumonia, because the gastroesophageal sphincter is relaxed in pregnancy and most patients have not been NPO, Spinal anesthesia can interfere with the mother's ability to push and has a higher incidence of hypotension than epidural anesthesia.

2. Treat asymptomatic bactcriuria in pregnancy (20% of patients develop cystitis and/or pyelonephritis if untreated because progesterone decreases the tone of the ureters and the uterus compresses the ureters.

3. Treat group B streptococcal (GBS) carriers only during labor and delivery. For example, if the patient is GRS positive at 2.6-28 weeks, wait until labor and give ampicillin.The goal of treatment is to prevent neonatal sepsis and endometritis.

4. If a woman has tuberculosis in pregnancy (positive purified protein derivative [PPD] test and suspicious chest x-ray, plus a positive sputum culture), treat as you would any other patient. If the patient is a known recent PPD converter or has additional risk factors (such as HIV positivity or household contact with an active case of tuberculosis), treat with isoniazid like a nonpregnant patient. Make sure to give the mother vitamin B6 with isoniazid to prevent nutritional defect in her and the fetus. Avoid streptomycin, which may cause deafness and nephrotoxicity in fetus.

5. Marijuana and lysergic acid diethylamide (I.SD) have not been confirmed as teratogens.

Preeclampsia: look for hypertension (in patients with preexisting hypertension, blood pressure should increase by > 30/15 mmHg over baseline); urinalysis with 2+ or more proteinuria; oliguria; swelling or edema of hands and/or face; headache; visual disturbances; and HKI.LP syndrome (hemolysis, elevated liver enzymes, low platelets). Preeclampsia often involves right upper quadrant and epigastric pain and develops i.n the third trimester. The main risk factors (in order of importance) are chronic renal disease, chronic hypertension, family history, multiple gestation, nulliparity, age > 40 (although the classic case is a young woman with her first child), diabetes mellitus, and black race.Treatment is delivery if the patient is at term. If the patient is premature and has mild disease, treat hypertension with hydralazine or labetalol and bed rest. Observe the patient carefully. If the patient has severe disease (oliguria, mental status changes, headache, blurred vision, pulmonary edema, cyanosis, HELLP, blood pressure > 160/110 mmHg, or progression to eclampsia [seizures]), deliver regardless of gestational age because both mother and infant may die.

a Mild ankle edema is normal in pregnancy, but severe ankle edema or hand edema is likely to be preeclampsia.

■ If preeclampsia symptoms develop before the third trimester, think of hydatiform mole and/or choriocarcinoma.

» Hypertension plus proteinuria in a pregnant patient is preeclampsia until proved, otherwise.

■ Preeclampsia plus seizures = eclampsia. Eclampsia can be prevented by regular prenatal care. Catch it in preeclamptic stage, and treat appropriately.

■ Use. magnesium sulfate for eclamptic seizures (also lowers blood pressure). Toxic effects include hyporeflexia (first sign of toxicity), respiratory depression, central nervous system depression, coma, and death.

■ Do not rerneasure very high blood pressure in a pregnant patient. Err on the safe side; assume that it represents preeclampsia and start treatment.

» Do not try to deliver the infant until the mother is stable (do not do a cesarean section while the mother is having a seizure).

a Preeclampsia and eclampsia cause uteroplacental insufficiency, IUGR, fetal demise, and increased. maternal morbidity and mortality.

a Preeclampsia and eclampsia are not risk factors for future development of hypertension or end-organ effects of hypertension .

Important points:

1. The top causes of maternal mortality are pulmonary embolism, pregnancy induced hy pertension, and hemorrhage (most texts say in that order).

2. When a postpartum mother develops shortness of breath, tachypnea, chest pain, hypoten sion, and/or disseminated intravascular coagulation, think of amniotic fluid (A.F) pulmonary embolism.

8. Oligohydramnios -- - AF <: 500 ml, AF index < S. Causes include JUGR, premature rap lure of membranes, postmaturity, and renal agenesis (Potter's disease). Oligohydramnios may cause fetal problems such as pulmonary hypoplasia, cutaneous and skeletal abnormalities due to ((impression, and hypoxia due to cord compression.

4. Polyhydramnios AF > 2000 ml, AF index > 25. Causes include maternal diabetes met litus, multiple gestation, neural tube defects (anencephaly, spina bifida), GI anomalies (omphalocele, esophageal atresia), and hydrops fetalis. Polyhydramnios may cause post partum uterine atony with resultant postpartum hemorrhage and maternal dyspnea (overdistended uterus compromising pulmonary (unction).

Normal Labor

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