Severe early onset IUGR

Dorothy Ting

Nepean Hospital

JE-Antenatal history

• 22yo primip BMI 31

• Regional centre, EDD 2/5/15

• A positive, serology NAD

• Cholecystectomy 12/40

• PMHx: depression, PCOS, tramadol use

• FHx: deletion on chromosome 20p3 (nephew+sister)

• Non smoker

JE-Antenatal history

• Low risk NT

• 20+4 US symmetrically small = 18/40. Normal FAS +amniotic fluid

• 21+2 Amniocentesis. Normal results

• 24+4 US EFW 383g = 20.5/40. Dilatation of right heart and fluid within bowel. REDF. Normal AFI.

• Self-referred to Nepean

JE-Antenatal history: first visit

• 25+5 EFW 383g REDF. Normal anatomy• 25+5 NICU meeting: steroids, ASA, clexane• Investigation: PET, thrombophilia, TORCH, HbA1c,

FGTT, TFT-NAD• 28+6 EFW 444g AFI 4.9 REDF, normal MCA+DV• 29+4 EFW 472g AFI 7.7 AEDF, normal MCA+DV.

Reduced FM on US.• 29+4 NICU meeting: surveillance and daily CTG• 29+6 NICU meeting: US MWF, daily CTG, CS 31-

32/40. Repeat PET Ix

JE-Antenatal US

JE-Antenatal history

• 30+4: EFW 494g AFI 11.8 AEDF, normal MCA and DV

• 30+6 and 31+0: Rescue steroids

• 31+1: Admission

• 31+2: MgSO4 loading

JE-Delivery

• 31+2: LSCS of LFI, respiratory effort notes

• Birthweight 630g

• Apgar 6 at 1min, 8 at 5min

• IPPV and suction

• Venous gas: pH 7.32, lactate 3

• Arterial gas: pH 7.21, lactate 2.9

• Pt discharge day 2 post op

JE-Placenta

• Weight 149g (3rd-5th centile)• Cord 5-8mm diameter• Variable appearance of villi- oedematous stroma,

syncytial knotting• Avascular sclerotic villi• Suggestive of fetal thrombotic vasculopathy• MCS no significant growth• Sent for FISH: Mosaic Monosomy X (36%), probe

for chromosome 16 not available

Challenging dilemma

• Extreme prematurity at diagnosis

• Early onset, severe IUGR

• Aetiology of IUGR

• Risks in utero vs perinatal

• Timing of delivery

• Surveillance

Severe early onset IUGR

• 0.4% of pregnancies• Varying outcomes

– EFW <501g, GA< 30/40 and AREDF: FDIU 53%, overall survival 14%, good intact survival

– TRUFFLE EFW<550g• Placental dysfunction is amenable to

intervention• Delivery

– 2% median survival gained per day between 24-27/40– GA 29+2 best predictor for intact survival

• Multinodal foetal surveillance

Foetal surveillance

Baschat AA. Arterial and venous Doppler in the diagnosis and management of early onset fetal growth restriction. Early Human Development 2005: 81: 877-887

Foetal surveillance and delivery

• Perinatal mortality is 5.5x higher if both FH and DV PI affected (SB 1/52 after)

• Monitoring: raised UA 2/52, brain sparing 1/52, AEDV 2x/52, 2-3x/7 raised DV PI

• Thresholds for delivery for 27/40 and EFW<500g vs 27-37/40 (DV, bPP, FHR)

• Steroids

Confined placental mosaicism

• At least two cell lines with different chromosomal complements

• Only placenta affected

• Occurs 1-2% viable pregnancies studied by CVS (50% confirmed in placenta)

• Mostly autosomal trisomy

Pathogenesis

Kalousek DK and Vekemans M. 1996. Confined Placental mosaicism. J Med Genet 33:529-533

Outcomes

• Conflicting• Likely depends on genomic imprinting,

number of placental cells, specific chromosome

• Association with idiopathic IUGR– 35% of CPM if high aneuploidy– 20% of idiopathic IUGR have CPM

• Prenatal loss-MC, FDIU, perinatal (22%)• Long term studies: short stature

Specific CPM

• Monosomy X: normal phenotype, (45X/46XX/46Xi(Xq), 45X/46XY, 45X/47XYY)

• Trisomy 16: severe IUGR, pre-eclampsia, fetal anomalies

• Other chromosome associated with IUGR 2, 3, 7, 8, 12, 13, 15, 18, 22