FETAL MEDICINE

INTRO TO EMBRYOLOGY

Implantation and Placental Development o Implantation occurs ~day 6: blastocyst emerges from zona pelucida and begins to adhere to uterine wall (mechanistic details unknown) Inner cell mass is nearest to attachment site o Trophoblast development of 2 cell types: 1) Syncytiotrophoblast: very invasive, eats into uterine wall 2) Cytotrophoblast o Blastocyst sinks entirely into uterine wall and begins to receive all nourishment from maternal tissue rapid growth

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Abnormal Implantation o Normal implantation: occurs at anterior/posterior wall of uterus o Placenta previa: results from implantation at internal os o Extrauterine/Ectopic Tubal Ectopic Pregnancy 95% of ectopics Most common site: fallopian tube 95% Cause: fertilized ovum stopped for unknown reasons in the tubal lumen o No cause found for many ectopics Problem: unsatisfactory site for implantation b/c thin walled tube unable to sustain trophoblastic invasion bleeding and/or rupture o Embryo cannot survive Risk factor: pelvic inflammatory disease Peritoneal cavity: rectouterine pouch Within ovary Placental Development o Components which will develop into placenta 1) Endometrium in secretory phase: maximum thickness following ovulation Glands: corkscrew shape & producing glycogen and lipids, which are the main source of nutrients later Under influence of estrogen and progesterone from corpus luteum and 1

trophoblast 2) Trophoblast 3) Fetal tissue forms chorionic villi which grow into maternal blood sinuses o Stages of Development Double layered trophoblast invades endometrium and erodes capillaries Lakes of maternal blood form: lacunae Chorionic villi surround lacunae and have following endocrine functions: Secretion of HCG Secretion of HCS: stimulates lactogenesis Secretes estrogens and progesterone

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Early Stages: Villi surround the chorion Later 2 sides form: Chorion frondosum: fetal side Chorion leave: maternal side

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o 2 Circulations: do NOT mix Endothelial barrier w/ trophoblast outside, which gets very thin as fetus develops Villi rupture Rh incompatablity

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Decidua = functional layer of endometrium o Deciduas basalis: covers chorion frondosum 2

o Deciduas capsularis: anembryonic pole and degenerated as anembrionic pole joins opposite uterine wall o Decidua parietalis: lines anembrionic uterine wall o Amnion and chorion fuse membrane which eventually ruptures during pregnancy Fetal Development o Inner cell mass differentiates into 2 cell layers Hypoblast: inner layer, primitive endoderm Epiblast: outer layer, primitive ectoderm o Cells develop fluid filled cavities divided by blilaminar disk = embryonic disk Cells above disk form amniotic membrane and space o Development of body axis and gastrulation Primitive streak: appears in epiblast 13-14 days post-fertilization Gastrilation: cells in epiblast migrate into groove, detach and drop off to lie between the epiblast and hypoblast layers trilamenar disk Mesoderm: new middle layer formed during gastrulation Visceral mesoderm = splanchnic o Neural tube Begins formation around day 17-18 Ectoderm along the axis begins to thicken to form neural plate Plate begins to fold & the folds on either side come into contact with each other and fuse neural tube. Closure begins in the middle of the embryos back & then extends cranially and caudally until zipped up around day 28 Developmental Problems o Spina bifida: failure of complete closure of neural tube o Lung agenesis - lung buds fail to form right or left bronchi - unilateral or bilateral - rare o Respiratory distress syndrome - in premature babies, surfactant production may be inadequate. The lungs may collapse & endoderm surfaces damaged. About 6000 premature babies in the UK suffer from this and 33% die. Surfactant either from human or animal sources also synthetically produced improves survival rate. o Tracheo-oesophageal fistula - an abnormal connection between the oesophagus and trachea (1;100,000 births) This is commonly associated with oesophageal atresia - blind ending of the oesophagus (1;3000 for the combined condition). Baby gags as the milk enters lungs, respiratory distress

THE PLACENTA Anatomy o Fetal surface: 2 membranes inner amnion and outer chorion o Umbilical cord: 2 arteries and 1 vein surrounded in Whartons jelly and covered by connective tissue Arteries divide over the surface of the placenta as chorionic vessels which then divide into the placental tissue and supply the placental villi o Villi bathed in maternal blood that flow from spiral arteries into intervillous space and out through uterine veins o Hemomonochorial membrane: consists of fetal epithelium, connective tissue and syncytiotrophoblast and functions to separate fetal and maternal blood o Maternal surface: made of 15-20 lobules = cotyledons Cotyledon: hundreds of frond-like structures called villi that are richly supplied by capillaries that drain back into chorionic veins to the umbilical vein o Abnormal Anatomy Velamentous insertion of the cord: cord inserts at the margin rather the center of placenta Seccenturiate lobe: an extra lobe of placenta which is separate from the main organ Usually a communicating vessel runs in membranes btw placenta and lobe Vasa previa: communicating vessel runs across cervix, can bleeding in labor or w/ AROM rapid fetal death Physiology o Gas exchange across hemomonochorial membrane o Fetal Blood flow Arteries carry deoxy-blood and vein carries oxygenated from mother to fetus Low resistance to blood flow in the placenta gives umbilical arteries characteristic waveform with good flow of blood during diastole QuickTime and a Wave can be seen in umbilical artery dopplers in TIFF (Uncompressed) decompressor are needed to see this picture. early pregnancy Abnormal wave: may indicate impaired placental invasion/function and may be early predictor of IUGR or pre-eclampsia Powerful constriction of umbilical vessels occurs post partum to cease circulation between the fetus and the placenta Cord blood: can be used just after delivery to determine fetal blood group and acid base status pH: 7.28 0.15 pO2: 1510 kPa pCO2: 4515kPa Base excess: 74 mmol/L Fetal blood compared to maternal blood Larger RBCs (MCV) Higher [Hb]: 18 vs 12g/100mL Higher oxygen saturation: 80% vs 40% Lower oxygen partial pressure (compensated for by high affinity HbF) Maternal Blood flow Maternal flow to uterus is 100-150mL/kg/min w/ 80-85% going to the placenta in late pregnancy Q = 1000mL/min by end of pregnancy Affected by posture: Pressure of the uterus on the IVC in the supine position obstructs venous return from the uterus Completely obstructed during peak of strong contractions: Venules in the 4

myometrium are completely occluded by surrounding muscle fibers pre-eclampsia: important component in the pathophysiology of PET in o Endocrine Functions -HCG: stimulates corpus luteum in early pregnancy and may control progesterone metabolism in later pregnancy Estrogens: stimulate growth of maternal tissues including breasts and uterus Progesterone: dampens down intrinsic uterine activity produced by corpus luteum until 8-10w then by placenta Human placental lactogen (HPL): influences glc metabolism and insulin resistance and may initiate lactation o Filtration Involved in water and ion transfer which contributes to volume of amniotic fluid Pathology o Abruption: sudden bleed behind the placenta causing it to separate from the uterine wall Complications: fetal hypoxia and/or death, severe maternal bleeding o Placenta previa: placenta covering cervix Diagnosed after 32w in most cases o Placental accreta/percreta: placenta invaded into/through myometrium into another organ Complications: massive hemorrhage requiring hysterectomy Risk factors: any condition interfering w/ the decidua (infection, previous curettage, previous section), chronic endometritis A/w placenta previa and Ashermans syndrome (intrauterine adhesions) o Small placenta: may cause w/ intrauterine growth restriction and pre-eclampsia, trophoblast invasion has been inefficient smaller more resistant spiral arteries less efficient nutrient and oxygen exchange fetal growth May be detectable early via umbilical artery Doppler abnormal wave form o Oligohydramnios: may be caused by placental failure amniotic fluid production o Molar pregnancy: abnormal placental development grape-like mass, may be complete or incomplete Snowstorm appearance on US Complications: severe hyperemesis, thyrotoxicosis, early onset pre-eclampsia, haemorrhage, persistent trophoblastic disease, choriocarcinoma

FETAL ABNORMALITIES Goal: to identify from a large population a smaller group of patients that have an increased risk of a disorder and to offer that smaller group a more specific diagnostic test Screening vs diagnosis o Screening test: estimates risk of disease presence, usually simple and w/o major risk o Diagnostic test: confirms presence/absence of disease, often more invasive w/ significant risks/complications Advantages of Prenatal Diagnosis o Patient reassurance when normal o Allows time for preparation when abnormal o Avail of subspecialty consultation o Avail of pregnancy termination o Delivery at tertiary-care facility o Optimise mode of delivery o In-utero therapy Should NOT be assumed that all patients undergoing prenatal diagnosis will request a termination of pregnancy if they receive an abnormal result What is screened for antenatally: major birth defects present in 2-3% of all births o Mendelian defects o Polygenic/multifactorial conditions o Teratogenic conditions o Chromosomal disorders 1:160, most either autosomal trisomy or sex chr abnormalities Specific conditions screened for: o Chr abnormalities Trisomy 21 (Downs) Trisomy 18 (Edwards) Trisomy 13 (Pataus) Triploidy Sex Chromosome Abnormalities 45X: Turner Syndrome, XXY: Klinefelters, XYY, XXX Balanced Translocations o Familial genetic disorders: CF, DMD, Huntingtons o Disease in high risk populations: Tay Sachs in Ashkenazi Jews -thal in SE Asians -thal in Mediteranians Sickle cell o Structural fetal anomalies Congenital heart disease Neural tube defects Abdominal Wall defects Genitourinary defects Lung Disorders o Congenital infection Toxoplasma CMV Rubella 6

Chickenpox Parvovirus B19 Syphillis Listeria Pretest counseling addresses following issues: o Risk of affected fetus o Nature and consequences of problem o Risks and limitations of procedures to diagnose problem o Time limitation of reporting o Failed or inconclusive reporting Types of Screening Tests o Serum screening o Ultrasound o Chorionic Villus Sampling Performed at 11 to 14 weeks results available in 2 days Risk of loss 1/100 (1%) Transabdominal / transcervical approach with ultrasound guidance Sample of placental cells removed to test for karyotype, FISH or PCR o Amniocenteis Performed after 15 weeks gestation Risk of loss (miscarriage rate) 0.1% to 0.5% Continuous US guidance used to ensure needle doesnt hit fetus Sample of amniotic fluid removed for karyotype, FISH or PCR (results in 2d) Screening for Chr Abnormalities common cause of morbidity and mortality o Involves combo of biochem and US markers o Performed in 1st and 2nd trimesters now more emphasis on Downs screening in 1st o Should be voluntary and only provided after pre-test counseling of benefits and limitations o Downs is most common serious abnormality 1:660 live births Increased risk with advancing maternal age o 1 in 1,000 risk at 20 yrs, increases to 1 in 20 at 45 yrs o Limiting screening to just those older than 35 years will only detect 30% of Down Syndrome so screening should be made available to all patients who want it, irrespective of age o 50% have major cardiac or GI abnormalities and most have IQ <70 Serum screening o Normal Pregnancy: -hCG 20% per week and PAPP-A 65% per week o 75% (60-70% on quiz) of cases of T21 can be diagnosed by combination of age and maternal serum markers in the second trimester for a 5% false positive rate: o Decreased MS-AFP o Decreased unconjugated estriol (uE3) o Increased hCG o Increased inhibin-A o This test is referred to as the second trimester Quad screen st 1 Trimester o Biochemical screening o Maternal blood sample taken at 10-14weeks o Measure free hCG and PAPP-A protein -hCG 2x in Downs, PAPP-A 60% QuickTime and a

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o US screening o Nuchal translucency: normal space seen on US btw back of fetal neck and overlying skin o NT space chance of Downs o Combined screening o Combining maternal age and NT and serum screening in 1st trimester identifications of 87% of affected fetuses w/ a 5% false positive rate 2nd Trimester: US 30% of DS have major structural defect visible by mid-trimester o Atrial septal defect/Ventricular septal defect 40-50% o Duodenal atresia 4% o Omphalocele o Ventriculomegaly o Cystic hygroma o Other soft markers, aka red flags for DS o Nuchal fold thickness o Short femur o Renal dilatation o Hypoplasia of middle phalanx 5th finger (Clinodactyly) o Sandal gap between toes of feet o Echogenic bowel (?)

o Edwards Syndrome: trisomy 18 Prevalence = 1:3000 Lifespan: hours days US features: single umbilical artery, IUGR, strawberry head, choroid plexus cysts, hyrocephalus, micrognathia, nuchal edema, CHD, NTD Amniocentesis: confirms prenatal dx

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o Patau Syndrome: trisomy 13 Prevalence = 1:5000 Prognosis: poor 50% die in hours-days, most my 6mo US features: holoprosencephaly, facial abnormalities, microcephaly, CHD, omphalocele, polydactyly

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o Amniocentesis: confirms prenatal dx

o Turner Syndrome: 45XO Prevalence = 1:3000, not related to maternal age US features: cystic hygroma, generalized edema, cardiac defects Neonatal features: short stature, coarctation of aorta, streak/rudimentary ovaries, usually have normal IQ o Kleinfelters Syndrome: 47XXY 8

Prevalence = 1:2000 Phenotype: tall male w/ sparse facial hair, gynecomastia, azoospermia, occasionally IQ Commonest cause of male hypogonadism and usually diagnosed during infertility Ix

Structural Abnormalities o Congenital Heart Disease Prevalence 8/1,000 live births Most common congenital abnormality Overall prevalence likely higher as many cases of CHD are not apparent until later in life 33% are associated with abnormal karyotype 45% also have extracardiac malformations CHD responsible for 20% -35% infant deaths 90% CHD cases occur without identifiable risk factor in the general population, and are therefore mostly unpredictable Only 10% CHD cases occur in a high risk group: o Family history of CHD o Maternal medical disease (e.g. Diabetes) o Teratogen exposure (e.g. Lithium) o Genetic syndromes (e.g. DiGeorge) o Fetuses with chromosomal abnormalities o Fetuses with extracardiac abnormalities Best screening test: 4 chamber heart view at 18-22w o 90% detection rate in high risk population o 50% detection rate in low risk population o Allows Dx of: AV defects, VSD, hypoplastic LR ventricle, Ebsteins anomaly, pericardial effusions, cardiac tumors o Limitations: o Performance can depend on the gestational age, maternal body habitus, fetal position, amniotic fluid volume, and experience of the sonographer. o Certain malformations can be present with a normal appearing 4-chamber view, such as transposition, tetralogy of Fallot, double outlet right ventricle, coarctation. o Most experts now add views of the right and left cardiac outflow tracts to the routine exam to maximise detection of these abnormalities Management of CHD o Detailed Ix for extracardiac malformations: present in up to 50%, impact Px o Fetal karyotyping offered: 1/3 will have chr abnormality o Pre-natal counseling w/ sub-specialists and expert medical team for delivery o Early dx may help optomize either delivery timing or location (tertiary vs comm.) o Neural Tube Defects Incidence: 3:1000 (USA is 1:1000) Cause: failure of closure of neural fold at 26-28d Risk factors: none in 90%, others include folate, antiepileptic drugs Dx: 90% detectable by US at 16-20 weeks (midtrimester) Recurrence risk: 2-3% Types: spina bifida, anencephaly, encephalocele QuickTime and a TIFF (Uncompressed) decompressor o Anencephaly: skull vault and cerebral cortex absent are needed to see this picture. o Dx on midtrimester US in 100% o Open spina bifida o 1/3 neonatal death o 2/3 Survive w/ long term disability: LL paralysis, bladder/bowel 9

dysfunction, hydrocephalus, clubfoot, scoliosis, meningitis, mental retardation Dx: Midterm (18-20W) US in 90%: lemon sign (frontal bone scalloping), banana sign (cerebellum pulled down) Maternal serum: FP levels from 15-20w onward Management Sonographic surveillance indicated to confirm appropriate fetal growth and to rule out development of polyhydramnios (complicates over 50% of cases and is associated with preterm delivery) Karyotyping: rule out trisomy via amniocentesis Prenatal consult w/ sub-specialist to prepare for delivery and care Eg: perinatologist, neonatologist, pediatric neurologist and possibly also a pediatric neurosurgeon NTD dx shouldnt affect timing of delivery In severe NTD elective c-section should be offered Prevention 0.4mg Folic acid 3mo before and continuing 12w after conception to risk by 70% 4.0mg recommended is previous NTD Screening: 1) Elevated msAFP a/w NTD and abdominal wall defects Detects 80% of all open NTDs: 80% of SB and 90% anencephaly 2) US amniocentesis

o Abdominal Wall Defects Omphalocele: incomplete return of abd contents to abd cavity in early pregnancy herneation of bowel or liver through base of QuickTime and decom TIFF (Uncompressed) a are needed to see this pictu umbilical cord (hernia contained in membrane) o Incidence: 0.1% - w/ maternal age o A/w chr and cardiac abnormalities: amniocentesis usually recommended o W/o other abnormalities outcome is good Gastroschisis: herniation of abd contents to R of umbilical cord, w/ free-floating bowel not contained by membrane o No associated chr abnormalities amniocentesis not indicated QuickTime and a TIFF (Uncompressed) decomp o Early transfer to pediatric surgeon after birth for closure are needed to see this pictu o Prognosis: 10% neonatal mortality from bowel ischemia, complications of IV feeding o GUT Abnormalitites Renal Dysplasia Cystic Kidney Disease Hydronephrosis/Pyelectasis: unilateral (80%) or bilateral renal pelvic dilation o A/w postnatal UTI and reflux nephropathy o All started on ABics after dx and followed up w/ renal US nostnatally Posterior Urethral Valves: mucosal folds obstructing bladder neck

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o M>F o Causes anhydramnios renal damage and poor often fatal prognosis Potters Syndrome: pulmonary hypoplasia, limb deformity o Pathogenesis: bilateral renal agenesis extrememe oligohydramnios Potters sequence o Amniotic fluid important for alveolar maturation, esp 16-22w absence hypoplasia death o Diaphragmatic Hernia: Defect allows stomach, bowel liver to enter thoracic cavity pulmonary hypoplasia QuickTime and a TIFF (Uncompressed) decompre are needed to see this picture 15-30% a/w chr abnormality, often w/ other structural abn Survival: 50% - determining factor is presence of liver in chest
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US AND FETAL WELL BEING 1st Trimester US o Confirm viability and location of pregnancy exclusion of Ectopic o Confirm dates by measuring crown-rump length (CRL) o Exclude multiple gestation o CRL: Incomplete Miscarraige Irreg Uterine Sac: missed MC
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o 2nd Trimester: 18-22weeks o Detailed fetal anatomical survey to exclude malformation o Confirm pregnancy dating by measuring biparietal diameter (BPD), head circumference (HC) and femur length (FL) o Placental location, esp in women w/ vaginal bleeding to exclude placenta previa, or in women w/ previous section to exclude abnormal placentation (eg accreta)

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3rd Trimester o Estimation of fetal weight (EFW): biparietal diameter (BPD), HC, FL and abdominal circumference (AC) o Also for fetal well being, confirmation of presentation and placental location and a TIFF QuickTimesee this picture. TIFFQuickTime and a decompressor QuickTime and a are (Uncompressed) decompressor (Uncompressed) picture. needed to TIFF (Uncompressed) decompressor are needed to see needed to see this are this picture. o

Antepartum Fetal Surveillance o Methods available: Maternal perception of fetal activity: kick counts/charts 11

 Cardiotocography (CTG) = non-stress test (NST) in N America Biophysical profile (BPP) Doppler studies o Who to test: examples Postdates pregnancy: >40 0/7 weeks Chronic HT IDDM IUGR Decreased fetal activity Maternal disease, eg chronic renal disease, SLE, etc o Maternal Perception of Fetal Activity Should be recommended routinely from 28 weeks in all pregnancies Inexpensive and sensitive 3rd trimester: normoxic, healthy fetus spends 10% of its time making gross body movement Use of a fetal kick counting chart (e.g. Cardiff count-to-ten chart) can reduce perinatal mortality (PNM) from 8 to 2 per 1,000 In high risk patients who report diminished fetal movement, up to 60% of fetuses will be compromised o CTG/NST Top line: FHR, Bottom line: pressure transducer tracing of uterine activity Accelerations of fetal heart rate (FHR) are associated with fetal normoxia (wellbeing) 90% of fetal movements (FM) are associated with in fetal heart rate (FHR) Criteria for a reassuring CTG: 2 accelerations of 15bpm for 15sec twice in 20 min o Called Reactive CTG o No accelerations = Nonreactive CTG v suspicious for fetal hypoxia o Reactivity is gestational age dependent, typically only expected after 28 weeks gestation Should be significant beat-to-beat variability in the heart rate tracing (should not be flat line!) Late accelerations are non-reassuring Any abnormality in CTG should be reviewed promptly, and a decision needs to be taken as to whether it is safe to leave the fetus in utero. If a non-reassuring tracing: confirmatory test to exclude significant hypoxia must be done promptly (such as a fetal scalp sampling), or delivery must be expedited Normal CTG:

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Abnormal/Non-reassuring CTG: no accelerations present sign of fetal hypoxia

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Early decelerations and variability (first not worrying, second sign is) QuickTime and a TIFF (Uncompressed) decompressor are needed to see this picture. Non-reassuring: Late decelerations, sign of hypoxia 2 to uteroplacental insufficiency QuickTime and a TIFF (Uncompressed) decompressor are needed to see this picture.

Abnormal CTG: variable decelerations indicating cord compression not worrying unless repetitive and deep

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o Biophysical Profile/Score (BPP/BPS) Fetal hypoxia decreased CNS-regulated activities, altered heart rate patterns, decreased fetal breathing movement, decreased body movement and poor fetal tone. These can be quantified by ultrasound Four parameters: 2 points each 2 if present and 0 if absent (never give 1) Fetal tone Fetal gross body movement Fetal breathing movement Amniotic fluid volume (CTG: modified profile gives additional 2pts for reactive CTG for total of 10) Scores less than 6/8 are suspicious for fetal hypoxia and must be promptly evaluated for a decision on delivery versus continued in utero management On average only 8 minutes required to complete the scan and score 8/8 Test valid from 28 weeks onwards 13

Normal BPP score is very reassuring - false negative rate of only 0.7 / 1,000 i.e. less than 1 patient in every 1,000 who are given a score of 8/8 will have a fetus that is hypoxic False positive rate: 40-100% (3% were abnormal) Specific schedule / frequency of BPP is dictated by particular clinical need e.g. IDDM or postdates testing - twice weekly BPP

o Contraction Stress Testing (CST) Rarely used now Based on the observation that late decelerations were associated with stillbirth and low Apgars Method: oxytocin infusion, fetal heart response to induced contractions is studied If late decelerations are noted, this is a positive CST and implies high likelihood of fetal hypoxia requiring delivery Used as a second-line test to further evaluate an abnormal CTG Mostly replaced now by BPP o Doppler US Should be performed for IUGR, not required in normal fetal growth, routine anomaly US scan, 1st/2nd/3rd trimester US screening Umbilical artery blood flow: towards the placenta, with peak velocity during fetal cardiac systole, and slower velocity during fetal cardiac diastole Ratio of systolic to diastolic flow (S/D ratio) is used to quantify how well blood is flowing to the placenta Diastolic flow (leading to a larger S/D ratio) is reflective of increased placental resistance due to placental pathology (e.g. abruption, chronic hypertension, etc) Absent end diastolic flow (AEDF) or reversal of end diastolic flow (REDF) in umbilical artery is a strong marker for significant hypoxia in the fetus

Normal waveform: reassuring of fetal well being, esp in IUGR fetus

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AEDF: can occur in IUGR, may require close monitoring QuickTime and a TIFF (Uncompressed) decompressor are needed to see this picture. REDF: sign of fetal compromise, most cases suggests need for prompt delivery
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ABNORMAL FETAL GROWTH Small for Gestational Age (SGA) o Definition: fetus has failed to achieve a specific biometric or estimated fetal weight by a specific gestational age o Two Types: 1. Fetal growth restriction (FGR) or intrauterine growth restriction (IUGR) Definition: fetuses that have failed to achieve their growth potential. Better to use term FGR or IUGR rather than SGA prenatally, as it is difficult to know for sure that a fetus in utero is simply constitutionally small 2. Fetuses that are constitutionally small o Quantification: SGA cut-off is 10th percentile so 10% of population are SGA, 2/3 of which have FGR Low birth weight: <2500g Very low birth weight: <1500 o Associated risks: Birth hypoxia Neonatal complications Impaired neurological development o Causes of SGA Physiological: Small parental size Ethnicity Past hx of SGA fetus Pathological: Maternal o Infxn: CMV, rubella, etc o Smoking, drug abuse o HT or chronic illness Placental o Multiparity Fetal o Malformations o Chr abnormalities o Diagnosis Abd palpation + symphyseal-fundal height: limited accuracy, detects 30% of SGA US measurement of: AC, HC, FL, BPD calculation of EFW (estimated fetal weight) Serial US 2 weeks apart o Fetal Growth Restriction 1) Symmetrical FGR: HC and AC both <10th centile, amniotic fluid is normal Often have chr abnormalities or infected in-utero Often pathological insult occurring in early pregnancy 2) Asymmetrical FGR: due to utero-placental insufficiency High HC:AC b/c AC restricted while head growth preserved Fetal CO redistributed away from kidneys limbs and gut towards brain Head develops and abdominal growth amniotic fluid (fluid late event) A/w severe maternal anemia, smoking, etc Morbidity and Mortality Intrauterine: o Intrauterine fetal demise 15

o Oligohydramnios o Fetal hypoxia Neonatal o Hyperbilirubinemia o Hypoglycemia o Meconium aspiration o Polycythemia o Pulmonary hemorrhage o Pneumothorax o 6x Perinatal mortality Infant: o Physically small o Delayed neuro development Adult: risk of cardiovascular disease and DM Management Assess for chr defects: 20% w/ both AC and EFW <5th centile have chr defect o Percent >20 if there are other structural abnormalities, normal amniotc fluid and normal umbilical artery doppler Surveillance of fetal well being o Umbilical artery Doppler in high risk fetuses o If abnormal US scan but normal Doppler fetus is likely normally small Monitor as OP w/ US for growth every 2 weeks, w/ aim to deliver at 37w or earlier if distress o If abnormal Doppler admit w/ close monitoring via CTG and biophysical profile A/REDF a/w perinatal morbidity and mortality Give steroids if <36w Abnormal CTG or BPP are indications for c-section delivery Deliver in unit w/ optimal neonatal unit (consider in-utero transfer w/o appropriate facilities)

Large for Gestational Age (LGA) o Definition: birth weight >4000g = macrosomia o Risk Factors: DM Post term pregnancy Maternal obesity Multiparity Excessive weight gain o Diagnosis Serial clinical exams US EFW >90th centle or AC>90th centile for gestational age o Morbidity Prolonged labor Risk of c-section Shoulder dystocia o Management: controversial Screen for gestational DM Aim to minimize maternal and fetal trauma at delivery Non-DM mother: no evidence for prophylactic induction of labour Doesnt reduce the risk of caesarean section or shoulder dystocia 16

DM mother: no evidence to support elective caesarean section of infants whose EFW > 4.25Kg o Management of Shoulder Dystocia 50% of shoulder dystocia occurs in fetuses of normal weight so difficult to prevent In certain diabetic patients with EFW > 4,500g planned c-section may be appropriate b/c distribution of truncal obesity in the fetus of a diabetic mother increases the risk of shoulder dystocia PRETERM LABOR AND PREMATURE RUPTURE OF MEMBRANES Preterm Labor (PTL) = labor occurring before 37w from the 1st day of the LMP o Labor 20-24weeks referred to as threatened miscarriage o Diagnosis: regular contractions + cervical change + cervical dilation o PTL which ends in preterm delivery accounts for majority of perinatal morbidity in normallyformed infants

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o Incidence: 11% of all live births 1/3 are due to spontaneous PPROM 1/3 are iatrogenic (i.e. deliberate medical decision to deliver early) 1/3 are idiopathic (i.e. no cause found) o Causes of Prematurity Iatrogenic: Pre-eclampsia IUGR Maternal disease necessitating delivery Spontaneous Preterm Delivery PTL PPROM Cervical incompetence o Risk Factory for PTL Non-pregnancy related o Low socio-economic group 17

o Extremes of age o Poor nutritional status (as reflected by either maternal weight <50 kg or inadequate weight gain in pregnancy) o Smoking o Drug abuse Pregnancy-related Multiple pregnancy PPROM Uterine anomalies History of preterm delivery in prior pregnancy Placenta praevia Placental abruption Polyhydramnios Medical complications of pregnancy Intrauterine infection

o Predicting Preterm Delivery Cervicovaginal swab at 22-35w for a fetal-derived protein known as fibronectin (fFN) Positive swab suggests increased risk preterm delivery Negative fFN excludes 94% cases preterm delivery Transvaginal ultrasound to measure cervical length Short cervix (<25mm) predicts 75% cases preterm delivery o Prevention Good prenatal care may play role, but data limited No proven preventative strategies Progesterone supplementation may prevent preterm delivery in certain high risk cases o Pathophysiology of PTL Actinomyosin: contractile protein in myometrial smooth muscle Tocolytics (drugs that stop uterine contractions) cause uterine relaxation by decreasing intracellular free calcium Oxytocin responsiveness depends on receptor concentration and increases with gestational age Oxytocin receptor antagonist, such as atosiban {tractocile} may have a role in treating preterm labour Prostaglandins cause cervical ripening and uterine contraction o Management of PTL 1. Administer antenatal corticosteroids Regimens: o Dexamethasone 6mg 12 hourly IM x 4 OR: o Betamethasone 12mg IM 24hrly x 2 doses (preferred b/c PVLM) Value: o Reduction in RDS [OR 0.5] o Reduction in neonatal mortality [OR 0.6] o Reduction in IVH [OR 0.5] o Reduction in NEC and PDA o No effect on: uterine contractions, fetal growth/size, maternal blood glc Benefit occurs after 24 hours, and lasts up to 7 days No role for repeating steroid courses increased risks 18

2. Tocolysis: controversial role may temporarily delay delivery until steroids complete or patient transferred to tertiary level facility with appropriate NICU some units do not use this Limited role: prolong gestation for administration of corticosteroids and transfer to a tertiary care centre Has not been shown to improve overall perinatal outcome: role is controversial Examples: atosiban (oxytocin receptor antagonist), nifedipine (Ca channel blocker), ritodrine or terbutaline ( -adrenergic agonist), magnesium sulphate (competitive antagonist to calcium), indomethacin (interferes with PG synthesis) 3. Transfer to tertiary level facility with NICU Delivery in PTL if preferable vaginal 4. Role of antibiotics Infection plays an aetiolgoic role in preterm labour in at least 15 -20% of cases o Organisms: GBS, listeria, mycoplasma, bacteroides, ureaplasma Only role for antibiotics is when PPROM occurs: no benefit of antibiotics in prevention of PTL when membranes are still intact o Some evidence that the treatment of bacterial vaginosis in 2nd trimester may decrease the likelihood of PTL/PPROM in high-risk women Main role in PTL is penicillin for GBS prophylaxis 5. Role of Cervical Cerlage: Prophylactic or therapeutic suturing of cervix Multiple trials have failed to show benefit There "may" be a role for cerclage in select high-risk cases in which a short cervix is found on transvaginal ultrasound o e.g. may be a benefit of cervical cerclage in women with cervical length less than 25mm if patient has a history of previous preterm delivery or other high risk factors

o Neonatal Complications of PTL Respiratory distress syndrome (RDS) Necrotising enterocolitis (NEC) Intraventricular haemorrhage (IVH) Periventricular leukomalacia (PVLM) Sepsis Patent ductus arteriosus (PDA) Preterm Premature Rupture of Membranes o Terminology: Preterm Premature ROM: rupture of the chorioamniotic membranes before 37w Incidence: 1-3% of all pregnancies Premature ROM: rupture of the membranes prior to the onset of labor, may occur at term Prolonged ROM: membrane rupture >24h before delivery o Diagnosis of PPROM History: gush of fluid, constantly wet Physical exam: sterile speculum exam, pooling of fluid in posterior vaginal fornix Confirmatory tests: Ferning: cervical mucous shows a broad fern pattern on microscopy vs narrow fern pattern of amniotic fluid Vaginal swab pH: Nitrazine sticks turn blue in presence of amniotic fluid Cervico-vaginal fetal fibronectin positive in presence of amniotic fluid Intraamniotic instillation of indigo carmine dye with demonstration of blue dye on a vaginal tampon confirms PPROM (only use in very special situations as 19

needs invasive test i.e. amniocentesis) o Causes of PPROM Spontaneous / Idiopathic (most cases) Infection: Chlamydia, GBS, Bacteroides Smoking Placental abruption PPROM in prior pregnancy Incompetent Cervix Multiple Pregnancy (twins / triplets etc) Iatrogenic (after amniocentesis) o Management of PPROM Must balance risk of prolonging pregnancy to fetal maturity vs risk of fetal infection & sepsis w/ continued pregnancy (later gestation: the benefit of 1st while risk of 2nd ) Intrauterine infection (20% - 60% of PPROM cases) Placental abruption (5% - 15% of PPROM cases) Cord compression leading to fetal compromise Fetal demise (1% - 2% of PPROM cases) Prolong gestation for as long as it is safe to do so Monitor for signs of intrauterine infection Maternal signs of infection o Pyrexia o Tachycardia o Uterine tenderness o Preterm labour o Foul smelling vaginal fluid Fetal signs of infection o Fetal tachycardia o Non-reactivity / reduced variability on CTG / variable decelerations o Alteration in biophysical profile Loss of breathing movements Decrease in gross body movements Serum markers of infection Ultrasound markers of fetal compromise & Altered biophysical profile (BPP) CTG abnormalities Amniotic fluid tests o Glucose low o WCC high o Gram stain/culture positive o C-reactive protein >20mg/L Promote lung maturity by administering antenatal corticosteroids Optimize the time and mode of delivery All cases of PPROM should be delivered by 37 weeks, as there is no benefit to continuing pregnancy beyond this time, while there is considerable risk Some centres deliver all cases of PPROM by 34 weeks, but this is not universally accepted (timing of delivery of PPROM between 34 and 36 weeks is therefore individualized between centres) Delivery should be arranged immediately in the setting of PPROM if fetal lung maturity has been documented Delivery should be arranged immediately in the setting of PPROM if there is any 20

evidence of fetal compromise or infection, even without evidence of fetal lung maturity Role of Antibiotics When PPROM occurs at 24 to 32 weeks gestation, 7 day course of Ampicillin plus Erythromycin significantly prolongs pregnancy and is associated with improved perinatal outcome OPD Management: most doctors manage PPROM as an inpatient because of risks of sudden preterm delivery or deterioration in fetal status

o Mi-trimester PPROM: 16-25w Rare occurrence, but associated with poor overall perinatal outcome In absence of infection overall survival is 50-75% Stillbirth rate 3.8 - 21.7% (compared with 0-2% at 30-36 weeks) Incidence of infection with earlier gestation at time of occurrence of PPROM 50% deliver within a week, 20% continue for over a month after occurrence of PPROM Pulmonary hypoplasia is a major complication when PPROM occurs at <26 weeks PPROM post amniocentesis has a better outcome (with higher likelihood of cessation of leakage) compared with spontaneous PPROM

ANTENATAL HEMORRHAGE Classification o Threatened Miscarriage - Vaginal bleeding associated with an intrauterine pregnancy up to 24 weeks gestation o Antepartum Haemorrhage - Vaginal bleeding from 24 weeks until the onset of labour In some countries the cut-off to differentiate the above mentioned conditions is 20 weeks, rather than 24 weeks o Intrapartum Haemorrhage - Vaginal bleeding from the onset of labour until the end of the 2nd stage of labour o Postpartum Haemorrhage Vaginal bleeding from the third stage of labour until the end of the puerperium (6 weeks postpartum) Causes o 1. Placenta Praevia: 30% o 2. Placental Abruption: 20% o 3. Local Causes: 5% o 3. Unclassified : 45% Placenta previa o Definition: placenta that covers in whole or in part the internal cervical os o Incidence: 1 in 20 at 24 weeks, 1 in 200 at 40 weeks Increased among multiparous patents: nulliparus 0.2%, multiparous 0.5% o Types: localization determined by distance from intoernal os to placental border Marginal or partial: the leading edge of the placenta reaches or barely covers the internal cervical os Complete: the placenta is completely centrally covering the internal os Low-lying: the leading edge of placenta is within 2 cm of the internal os

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 o Risk factors Prior caesarean section Increased Parity Advanced maternal age Previous evacuation of retained products of conception (ERPC) / dilatation and curettage (D&C) Multiple pregnancy Smoking o Clinical presentation Painless vaginal bleeding which can be unprovoked or occurs post coitus or following uterine contractions First episode of haemorrhage usually not severe & typically painless Often asymptomatic: Incidental diagnosis seen on routine obstetric ultrasound Physical signs Uterus soft and non tender Fetal heart rate is usually normal Typically high presenting part or malpresentation, because head cannot descend into pelvis Major patient safety note: Never attempt to do a vaginal exam in any patient with APH who might have placenta praevia. (Always safer to check a prior scan report first, or just repeat the scan.) o Diagnosis Clinical suspicion from history and exam findings Ultrasound: placental localisation. Measurement of the distance of the lowermost placental edge from the internal cervical os. Low lying placenta is within 2cm of the internal os. Most precise diagnosis is using transvaginal ultrasound (TVUS), which must be performed with an empty bladder. Cannot accurately diagnose or exclude praevia with TA US or with a full bladder (TVUS is not contraindicated in placenta praevia) The large arrow is pointing at the placenta which is centrally covering the internal cervical os (which is shown by the 2 small arrows)

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o o Complications 22

Maternal Hemorrhage Placenta accreta (15%) Hysterectomy Death Fetal Preterm birth IUGR: rare b/c placental function usually normal Death o Management 1) Focused history 2) Initial exam: maternal and fetal 3) Immediate management if presenting bleeding previa IV Line (14G x 2) FBC/Coag/X-match 4 units IV Fluids O Negative blood (be prepared to use O negative blood for transfusion if life threatening haemorrhage and cross-matched blood not available yet) Call for senior help (obstetric and anaesthesia) and notify NICU 4) Immediate delivery indicated if: Severe life-threatening maternal haemorrhage regardless of gestational age Evidence of non-reassuring fetal testing regardless of gestational age Advanced gestational age 34-36 weeks. o If patient has already received antenatal corticosteroids and has recurrent significant bleeds, 34 weeks is a reasonable threshold for delivery. If only minor bleed, and is not recurrent and steroids have not been completed yet, it may be reasonable to hold off on delivery until 36-37 weeks 5) Expectant management depends on: Ongoing risk of placenta praevia: sudden unpredictable major / life-threatening haemorrhage Gestational age at delivery: directly related to perinatal mortality o If < 35 weeks, steroids should be given o Inpatient hospital admission from viability (24 - 26 weeks) Outpatient management is acceptable only in very exceptional / stable circumstances in women who have never had significant vaginal bleeding and live close to the hospital with good immediate family supports Repeat ultrasound at 36 weeks to confirm if still praevia Aim for delivery at 37w or before if start substantial / recurrent vaginal bleeding Delaying c-section until 39-40 weeks is risky, with minimal perinatal benefit

Morbidly Adherent Placenta o Placenta accreta: abnormal attachment of the placenta to the uterine wall (decidua) such that the chorionic villi invade beyond the endometrium and directly into the myometrium. Possible Cause: primary deficiency of or secondary loss of decidual elements (decidua basalis). Classification: 3 grades based on pathologic assessment of myometrial invasion by the chorionic villi: Accreta - chorionic villi in contact with myometrium (80% of cases)

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Increta - chorionic villi invade into myometrium (15% of cases) Percreta - chorionic villi invade into serosa or beyond (5% of cases) Associations: history of prior cesarean section, uterine instrumentation, fibroid surgery, or prior placenta praevia. Rarely, abnormal attachment is seen in the absence of prior surgery and in the absence of placenta praevia Prior to C-section: patient w/ suspected should be consented for possibility of hysterectomy, massive hemorrhage intrapartum and need for blood transfusion Pre-op assessment may require MRI

Placental Abruption: premature separation of a normal sited placenta o Incidence: 1/150 deliveries o Recurrence risk: 5-15% o Classification Revealed hemorrhage: blood tracks down between membranes and uterine wall and results in obvious vaginal bleeding Conceled hemorrhage: blood remains inside uterine cavity o Risk factors Chronic hypertension / preeclampsia Abdominal trauma Cocaine use QuickTime and a TIFF (Uncompressed) decompressor Smoking are needed to see this picture. Prolonged PROM High parity Abruption in prior pregnancy o Symptoms: severe abdominal pain, backache PV bleeding o Classical signs: Shock fainting Woody uterus: firm, tender and does not relax Fetus difficult to palpate inaudible fetal heart Irritable uterus o Diagnosis Clinical: must exclude previa and other causes of hemorrhage Confirmed by demonstration post-delivery of retro-placental clot indenting placental substance US: small role in dx, more for fetal assessment and to out rule previa ALWAYS rule out previa 1st o Management Early delivery is vital Mother must be stabilised IV Line: FBC, Group and x match 4 units, Coagulation screen Continuous CTG If baby is alive and gestation is not too early as to make fetal survival unlikely, delivery should be by emergency caesarean section If fetus already dead usually better to aim for a vaginal delivery & amniotomy should be performed to hasten this o Complication: coagulopathy Coagulopathy = decreased fibrinogen level, decreased platelets & raised fibrin degradation products Occurs in 30% of cases with placental abruption 24

Cause: Release of thromboplastins from the damaged placenta DIC Hypovolaemia can result in multisystem failure Expert haematological opinion is advised Increased risk of postpartum haemorrhage and haemorrhage at time of caesarean section when coagulopathy present

Local causes of APH o Cervical pathology: cancer, ectropion cervicitis, foreign body o Check date of last smear and take new smear if indicated

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Vasa previa o Definition: vessels of the umbilical cord run in the fetal membranes and cross the internal cervical os Coexists with velamentous insertion of the cord in which veins traverse the membranes before they come together into the umbilical cord Incidence: 0.1% or less o Presentation: intrapartum hemorrhage in early labor w/ ROM Fetus: rapid fetal haemorrhage, fetal bradycardia and fetal death if delivery is not accomplished immediately In contrast to placenta praevia, it is fetal blood that is being lost and therefore will not take much blood loss before fetus is compromised or dies Kleihauer test or an Apt test can be performed to confirm that the blood is in fact fetal blood, though rarely done b/c baby is usually delivered by emergency caesarean section due to life-threatening fetal compromise o Diagnosis: confirmed post-natally on placental and membrane exam
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Rhesus Isoimmunization o All rhesus negative women should receive anti-D injection if they have an APH, or an event that might cause a concealed abruption (? trauma) o A kleihauer test estimates the volume of fetomaternal haemorrhage and allows the appropriate dose of anti D to be calculated

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MULTIPLE GESTATION Incidence: o Twins: 1 in 30 births o Higher order multiples: 1 in 500 births Approx 7,000 triplet births per year in US o Increasing in last 10years Advancing maternal age effect: higher likelihood of spontaneous multiple ovulation More widespread use of assisted reproduction techniques for infertility (ART) 8% - 15% risk of multiples with ovulation induction 25% - 50% risk with IVF (depending on how many embryos replaced) Types of Twinning o 2/3 twins are dizygotic: Non-identical / fraternal twins Variable incidence depending on age, race, parity, medications 3/1,000 < 20 years old 14/1,000 > 35 years old o 1/3 twins are monozygotic: Identical twins Incidence constant (4/1,000) o Zygosity is an embryological term, which clinicians in general do not use, as it is difficult to be certain of zygosity based on ultrasound information alone. o Chorionicity and amnionicity are terms more commonly used by clinicians in practice

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Twin Placentation o All dizygotic twins must be dichorionic o All dichorionic twins must, by definition be also diamniotic. o Similarly, all monoamniotic twins must, by definition, be also monochorionic o Monozygotic twins: 30% are dichorionic: Embryo divides within 2-3 days fertilization 70% are monochorionic / diamniotic: Embryo divides between days 3 and 8 1% are monochorionic / monoamniotic: Embryo divides between days 8 and 13 1/50,000 are conjoined twins: Embryo divides after days 13 15 o Importance of Chorionicity: Certain complications are seen only in monochorionic twins o Optimal time to determine chorionicity: 8-11weeks, hard to see placentas and membranes later Dichorionicity Can be confirmed by twins of different sex If same gender look for separate placentas (one anteriorly and one posteriorly in uterus) or a thick dividing membrane between the two sacs Twin Peak Sign: placental tissue visible in base of dividing membrane

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Role of US o Diagnosis of multiple gestation 100% of multiple gestations should be correctly diagnosed antenatally with a policy of routine ultrasound Up to 10%-15% multiples will not be diagnosed until in labor if ultrasound not routinely performed in all pregnancies o Vital for determining chorionicity o Detection of fetal anomalies: 80%-90% anomaly detection rate with routine ultrasound in twins o Evaluation of fetal growth Always compare fetal sizes in multiple gestations, and if > 20% growth discordance, this may be abnormal o Confirmation of fetal well-being BPP and Doppler (umbilical and middle cerebral artery) studies allow confirmation of adequate oxygenation of each fetus Antepartum Management o Very high risk for preterm labour and preterm delivery o No role for elective cervical cerclage, inpatient bed rest, or prophylactic tocolysis o Cervical length (using trans-vaginal scan) is a useful tool for predicting preterm delivery: If cervix <25mm at 24 weeks, 8-fold increased chances of delivery before 32 weeks

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o Fetal fibronectin (using special vaginal swab) may be useful tool to predict preterm delivery: If positive at 28 weeks, 10-fold increased chances of delivery before 32 weeks However, cervical length and fibronectin assessments are more useful for their negative predictive values than their positive predictive values (i.e. if +/abnormal, most patients will still NOT deliver early, while if they are negative there is then a very high degree of confidence that the patient will NOT deliver early) o Interventions limited: caution w/ multiple tocolytics & multiple courses of prophylactic steroids o Surveillance for: Preeclampsia necessary as preeclampsia more common in multipes: More likely atypical or severe preeclampsia and more likely to be earlier onset No interventions known to prevent preeclampsia Other medical problems: Gestational diabetes, UTI, acute fatty liver Fetal growth in all cases: Dichorionic twins should have scans every 4 weeks to evaluate fetal growth Monochorionic twins should have scans every 2 weeks 27

BPP / Dopplers only required if IUGR or significant growth discordance

Intrapartum Management o Deliver all twins by 38 to 39 weeks Probably safer to deliver monochorionic twins by 34-36 weeks o Deliver all triplets by 36 to 37 weeks o Most standard labour management interventions are also applicable in twins Can utilize prostaglandins for induction, and oxytocin for augmentation Vaginal birth after cesarean section {VBAC} also safe in carefully selected cases o IV access is mandatory / epidural recommended for all twins undergoing vaginal delivery o Electronic fetal monitoring mandatory for both fetuses (CTG) o Deliver in (or near) operating theatre, with anaesthetist back-up o Availability of intrapartum ultrasound useful to determine the position and presentation of the second twin) o Vertex-Vertex Twins: Most common presentation: 40% - 45% of all twins will be vertex-vertex when in labour Vaginal delivery possible for most such cases No evidence that routine cesarean delivery for all such twins is beneficial Following vaginal delivery of twin A, should rapidly confirm presentation of second twin (either by vaginal exam or scan) Management of twin (B) delivery is then based on heart rate tracing, but in general should expedite delivery o Vertex-Nonvertex Twins Vertex-breech / vertex-transverse presentations when in labour, occurs in 35%-40% Twin A can be delivered vaginally, while mode of delivery of twin B depends on: Size of B / concordance in size with A / presence of staff skilled in breech extraction Standard singleton breech criteria also apply (e.g. flexed head) Options for delivery of twin B are vaginal breech delivery / external cephalic version (ECV) from breech to vertex / cesarean section Breech extraction of second twin (twin B) after vaginal delivery of twin A is likely safe for fetuses that are >1,500g. However, there are insufficient data to confirm safety of this if < 1,500g Assisted breech / total breech extraction OK o Nonvertex First Twin Breech-vertex / breech-breech presentations occur in 15%-20% of twins Fear of interlocking chins if breech-vertex twins delivered vaginally Almost all such cases are therefore delivered by elective cesarean o Higher Order Multiples Safe vaginal delivery of triplets possible but practical difficulty of 3 or more fetal hearts to monitor c-section delivery in most cases Problems of Monochorionic twins o Twin-Twin Transfusion Syndrome (TTTS) Definition: unequal sharing of blood-flow across the single shared placenta between both fetuses Incidence: 10-20% of monochorionic gestations Pathogenesis uncertain: presence of AV shunt? Donor fetus: sends much of its blood supply to its co-twin failure to thrive Anemic, hypo-perfused

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 Smaller size / growth restricted / hypovolemic Oligohydramnios / stuck Small bladder Recipient Fetus: receives a large amount of blood from its co-twin fluid overload Plethoric, hyper-perfused Larger size / hypervolemic Polyhydramnios Heart failure / tricuspid regurgitation Diagnosis Confirm monochorionic twin pregnancy: Same sex / single placenta Discordant fluid: Oligohydramnios / polyhydramnios sequence Discordant growth: 20% discrepancy in size, measure HC, AC and CRL Abnormal umbilical arterial Dopplers: AEDF more common in donor twin Treatment Reduction Amniocentesis: US-guided needle drainage of as much amniotic fluid as possible from around the recipient fetus o Repeat the procedure, possibly every few days, or as often as needed, to maintain normal amniotic fluid volume o Unclear how this therapy helps: Reduce pressure from recipient sac on stuck twin May allow increase in perfusion of stuck twin Probably reduces uterine overdistension, thereby reducing chances of preterm delivery o Does not prevent neurologic sequelae if one twin dies 10% - 20% incidence severe brain injury in this situation Likely occurs because of sudden flow of blood from remaining live fetus, across the placenta, into the dead fetus, at the moment of death of one fetus. This sudden flow of blood may be enough to cause death of the remaining fetus, or may be sufficient to transiently drain blood from brain of surviving fetus Laser Ablation o Percutaneous / ultrasound guided 2 mm fetoscope Laser fiber placed in recipient sac Placental surface near dividing membrane examined QuickTime and a AV shunt vessels near membrane visualized TIFF (Uncompressed) decompressor are needed to see this picture. Selective photocoagulation of these communicating vessels o Amniotic fluid reduction performed at same time o More invasive than amniocentesis, but results in definitive treatment of underlying pathology o Recent randomized trial suggests laser is better treatment option than amniocentesis Mortality Rate of TTTF: 60% to 100% for both fetuses if untreated

o Monoamniotic Twins Only 1% of monochorionic gestations are also monoamniotic (i.e. both fetuses sharing the same amniotic sac) Prenatal diagnosis confirmed by ultrasound with failure to visualize dividing membrane and also clearly entangled umbilical cords > 50% perinatal mortality rate 29

Mortality unpredictable due to sudden cord compression or occlusion Intensive fetal surveillance required from time of viability (24 26 weeks) Hospitalization and daily fetal monitoring (CTG) If variable decelerations present, continuous fetal heart rate monitoring required Deliver if testing is non-reassuring Elective delivery, after steroids, at 34 weeks Vaginal delivery possible, but cesarean likely

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