Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Pathophysiology | Etiology | Clinical features | Diagnosis | Laboratory | Radiology description | Prognostic factors | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Videos | Sample pathology report | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Steele J, Hecht JL. Maternal vascular malperfusion. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/placentauteroplacentalinsuff.html. Accessed March 22nd, 2023.
Definition / general
- Conditions in which the functional capacity of the placenta is impaired due to altered maternal blood flow to the intervillous space
- Clinical presentation ranges from fetal growth restriction to preeclampsia
- References: Arch Pathol Lab Med 2016;140:698, Pediatr Dev Pathol 2004;7:237
Essential features
- Conditions in which the functional capacity of the placenta is impaired due to altered maternal blood flow to the intervillous space
- 2 patterns: global (small placenta with villous malformation: accelerated villous maturation or distal villous hypoplasia) and segmental (villous infarcts from occluded spiral arteries)
- Gross features: infarction, infarction hematoma
- Microscopic features: accelerated villous maturation, distal villous hypoplasia, syncytiotrophoblastic knots, decidual arteriopathy or acute atherosis
Terminology
- Uteroplacental insufficiency
- Maternal vascular underperfusion
- References: Arch Pathol Lab Med 2016;140:698, Pediatr Dev Pathol 2004;7:237
ICD coding
- ICD-10: O36.5190 - maternal care for known or suspected placental insufficiency, unspecified trimester, not applicable or unspecified
Epidemiology
- Reported incidence range is broad: 8% of low risk nulliparous women to 32.8% of term placentas in an unselected cohort (Obstet Gynecol 2017;130:1112, Am J Obstet Gynecol 2017;216:411.e1)
- Higher incidence with:
- Preterm birth: 47.7 - 50.6% (Placenta 2016;48:56, Am J Obstet Gynecol 2017;216:411.e1)
- Small for gestational age (SGA) and preeclampsia: 47% (Obstet Gynecol 2017;130:1112)
- Black women: odds ratio = 1.14 - 1.58 (Placenta 2018;69:102
- Gestational diabetes: 30.5% (Placenta 2017;49:10)
Sites
- Placenta
Pathophysiology
- Global maternal vascular malperfusion:
- Begins early in pregnancy with a high recurrence in subsequent pregnancy
- Due to abnormal implantation with inadequate spiral artery remodeling, leading to erratic and heterogeneous blood flow (areas of underperfusion and areas of high velocity flow)
- Severity determines the spectrum of disease from growth restriction to preeclampsia (see also: preeclampsia)
- Segmental maternal vascular malperfusion:
- Represents acute / intermittent events, associated with thrombophilia or abruption
- Reference: Placenta 2009;30:473
Etiology
- Maternal vascular disease
- Diabetes
- Chronic hypertension
- Thrombophilia
- Smoking
- Drug abuse (especially cocaine, heroin and methamphetamine)
- Prior history of pregnancy with intrauterine growth retardation
- References: Arch Pathol Lab Med 2016;140:698, Pediatr Dev Pathol 2004;7:237
Clinical features
- Fetal growth restriction
- Preeclampsia
- Prematurity
- Stillbirth
- References: Arch Pathol Lab Med 2016;140:698, Pediatr Dev Pathol 2004;7:237
Diagnosis
- Second and third trimester ultrasound for placental size of the placenta, uterine artery Dopplers or fetal growth delay
- Maternal alpha fetoprotein levels (a fetal liver function test)
- Fetal nonstress test
- References: Arch Pathol Lab Med 2016;140:698, Pediatr Dev Pathol 2004;7:237
Laboratory
- Elevation of maternal serum alpha fetoprotein levels (a test of fetal liver function) (Prenat Diagn 1997;17:305)
- Elevation of maternal serum sFLT1/PlGF ratio (Ultrasound Obstet Gynecol 2018;52:631)
Radiology description
- Ultrasound may show fetal growth restriction and reversal of end diastolic flow on umbilical arterial Doppler (Clin Genet 2006;69:97)
- MRI is not routine but may show restricted diffusion (Radiology 2010;257:810)
Prognostic factors
- Risk of recurrence for severe global maternal malperfusion: 10 - 25%
Case reports
- 20 year old woman with placental infarct, preeclampsia and fetal intrauterine demise (Fetal Diagn Ther 2014;36:154)
- 33 year old woman with early intrauterine growth restriction at 22 weeks gestation and severe preeclampsia (Pulm Circ 2020;10:2045894020970056)
Treatment
- Close surveillance in subsequent pregnancy due to risk of recurrence (10 - 25%)
- Consider acetylsalicylic acid (ASA) therapy and early third trimester placental ultrasound
- Early delivery for growth restriction or poor uterine artery flow (J Obstet Gynaecol Can 2012;34:17)
- Consider screening mother for diabetes, thrombophilia, metabolic syndrome
Gross description
- Placenta that is small for gestational age (< tenth percentile of weight)
- Infarction or infarction hematoma, located away from periphery of the disc and of significant size (any infarction in preterm or > 5% of the disc at term)
- Thin umbilical cord (< 8 mm diameter near term), correlating with small disc
- Reference: Placenta 2014;35:696
Gross images
Microscopic (histologic) description
- Altered villous morphology (accelerated villous maturation):
- Villi are small, thin and elongated with increased syncytial knots
- Accelerated maturation should only be diagnosed based on examination of the villi adjacent to the maternal surface
- Appearance of accelerated maturation is identical to the normal appearance of the placental region under the fetal surface; this subchorionic zone serves as a good internal control
- Altered villous architecture (distal villous hypoplasia):
- More easily recognized before 32 weeks of gestation as a paucity of villi in relation to the surrounding stem villi (increased space around the villi)
- At lower power, this results in a prominence of large stem vessels all the way to the maternal surface
- In second trimester, there is a loss of the normal gradient of larger immature villi to small mature villi as one scans from mid placenta to maternal surface
- Syncytiotrophoblastic knots (aka Tenney-Parker change):
- Increase in nuclear clumping and basophilia of the multinucleated cells on the terminal villi
- Knots should generally only be reported if they are present in every 40x field (30% of the villi) or when prominent in a gestation under 36 weeks
- Reference tables for syncytial knots are available (Pediatr Dev Pathol 2010;13:305)
- Decidual arteriopathy
- Acute atherosis: collection of intimal foam cells; best seen in the arteries in placental bed biopsies but may extend into the decidual arteries
- Features often cited but not reproducible: islands of fibrin with extravillous trophoblasts; extensive increase in intervillous fibrin, chorion laeve pseudocyst; decidual necrosis
- Reference: Placenta 2014;35:696
Microscopic (histologic) images
Positive stains
- sFLT1 expression increased in hypoxic villi (those with accelerated maturation and syncytial knots)
Videos
Amsterdam terminology for placental lesions
General approach to diagnostic reporting
Clinical relationship to neonatal encephalopathy
Sample pathology report
- Singleton placenta at _ weeks gestational age; _ g (_ percentile):
- Small placenta with features of maternal vascular malperfusion
- Infarction, infarction-hematoma comprising _% of the disc
- Thin umbilical cord
- Accelerated villous maturation
- Distal villous hypoplasia
- Increased syncytiotrophoblastic knots
- Membranes with decidual arteriopathy
Differential diagnosis
- Incidental infarction:
- Any infarction at the disc edge or infarction comprising < 5% of the disc at term
- Features mimicking accelerated maturation:
- Small, widely spaced, elongated villi with increased syncytial knots (normally seen under the fetal plate and in a patchy distribution near the maternal surface in the region of venous drainage)
Additional references
Board review style question #1
A 34 year old woman who is pregnant with her third child and has 2 kids (G3P2002), with a history of gestational hypertension and diabetes, presents for delivery. Her placenta is sent for pathology. Gross examination reveals a placenta that is small for gestational age, with a 2.1 cm infarction within the central placenta (15% of placenta disc). What feature would be most expected on histopathologic examination?
- Chronic intervillositis
- Decreased syncytial knots
- Increased syncytial knots (Tenney-Parker changes)
- Maternal floor infarction
- Normal placenta
Board review style answer #1
C. Increased syncytial knots (Tenney-Parker changes)
Comment Here
Reference: Maternal vascular malperfusion
Comment Here
Reference: Maternal vascular malperfusion
Board review style question #2
Accelerated maturation is a feature of maternal vascular malperfusion. What histologic feature is associated with it?
- Decidual arteriopathy
- Decreased syncytial knots
- High placental weight
- Villlous sclerosis
Board review style answer #2