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1256 PART IV Obstetric and Fetal Sonography

because of the associated increased risk for hemorrhage, infection,

or malignancy. 131

NEURENTERIC CYST

Neurenteric cysts represent a posterior enteric remnant caused

by incomplete separation of the notochord from the foregut

during embryogenesis. hey typically occur in the posterior

mediastinum or in the spinal canal midline. he cysts can have

a septated or bilobed appearance. Associated spinal abnormalities

are typically present but diicult to identify prenatally. 132-135 MRI

is a useful adjunct in the further evaluation of the spine. Esophageal

duplication cysts should be considered in the diferential

of midline chest cysts.

PLEURAL EFFUSION

Any luid in the pleural space is abnormal. If suiciently large,

pleural efusion may result in mass efect, leading to compression

of the heart and hydrops as well as compression of the lungs,

resulting in pulmonary hypoplasia. 136

Primary pleural efusion is rare, with an incidence of up to

1 in 15,000 pregnancies. 137,138 It is most oten caused by chylothorax,

139 which results from defective development of the

lymphatic system. Any insult to the course of the thoracic duct

in the posterior mediastinum in the fetal chest can result in

chylous fetal pleural efusion. 140 his can be on either the right

or the let side, because the thoracic duct in the posterior

mediastinum crosses from right to let at the ith thoracic level.

Primary pleural efusion is suspected when the efusion is

unilateral or is much larger on one side than the other and when

no other signs of hydrops are present. Primary efusions are

characterized by having high pressures, resolving rapidly ater

thoracentesis and developing hydrops restricted to the upper

fetal body. 141

Primary chylothorax is associated with aneuploidy in 2% to

6% of cases. 142,143 By deinition, chylothorax in neonates is identiied

when luid contains more than 1.1 mmol/L triglycerides

with oral fat intake, with lymphocyte proportion exceeding 80%. 144

However, the fetus is fasting in utero, and the mean percentage

of lymphocytes in the blood of normal fetuses is normally greater

than 80%. 145 herefore these parameters do not apply in utero.

Secondary pleural efusion occurs in association with

aneuploidy (Turner syndrome or trisomy 21), infection (TORCH

[toxoplasmosis, rubella, cytomegalovirus, herpes simplex, HIV]),

genetic syndromes, and other structural malformations (CPAM,

BPS, lymphangiectasia, cardiac anomalies). 142-145 hese syndromes

typically present with bilateral pleural efusions (particularly in

fetuses with hydrops) 140 but occasionally with unilateral

efusion.

Pleural efusions appear on prenatal ultrasound as anechoic

luid collections in the pleural space, leading to the appearance

of the lung loating in the luid, surrounded by the chest wall

and diaphragm 146 (Fig. 36.10, Video 36.2). Small efusions appear

as an anechoic thin rim outlining the lungs and the mediastinum.

Larger unilateral efusions result in mass efect, causing mediastinal

shit and lattening or eversion of the diaphragm. If isolated

and small, pleural efusions have a benign course. If large with

mass efect, untreated fetal pleural efusion has a mortality rate

of 22% to 53%. 137,140,147-149

Small pleural efusions do not shit the mediastinum.

Pleural efusions associated with hydrops may be unilateral

or bilateral, oten beginning as unilateral collections that progress

bilaterally. Once hydrops develops (cardiac compression

from unilateral efusion), distinguishing between primary and

secondary efusion can be diicult. If mediastinal shit is visualized

in association with a small pleural efusion, a chest mass

such as a CDH, CPAM, or BPS should be sought. MRI may

help identify associated abnormalities not seen by ultrasound,

such as an inconspicuous CPAM or BPS. Depending on the

content of the pleural luid (lipid, protein, or blood products),

diferent signal intensities might be seen with MRI in both

T1- and T2-weighted images that may help establish the causal

diagnosis. 150

Prenatal pleural efusion has a variable natural course ranging

from spontaneous resolution to progressive development of

hydrops and polyhydramnios with high risk for perinatal morbidity

and mortality. 140 An efusion ratio can be obtained by

ultrasound (area of efusion divided by area of thorax) and

followed with serial examinations. If it elevates, it increases the

risk of evolution to hydrops, 141 but no cutof ratio has demonstrated

usefulness as a prognostic tool. 151

A fetal assessment for associated anomalies, including echocardiography,

maternal serology for infections, and fetal karyotyping,

should be performed. 141,152 Prognosis depends on the presence

of associated abnormalities, whether it is unilateral or bilateral,

the size of hydrothorax, and severity of mediastinal shit. A small

primary hydrothorax can have spontaneous resolution in 22%

of cases. 142 Unilateral efusions, spontaneously resolved efusions,

and those without mediastinal shit or hydrops have a 73% to

100% survival rate. 140,141

Progression from unilateral to bilateral efusions is associated

with impending hydrops. When large, bilateral, and with mediastinal

shit, the mortality rate increases to 52%. 140 With progression

to hydrops, the mortality rate increases to 62%. 140 Secondary

hydrothorax has a worse outcome. he mortality rate in these

cases can be up to 98% depending on size, progression to hydrops,

and underlying cause. 140

Pleural efusions are treated by drainage if the efusion is

isolated (or asymmetrical) without additional anomalies, typically

in fetuses at risk for developing hydrops (i.e., those with severe

mediastinal shit or with other signs of hydrops already present). 63

Typically, a pleural efusion is drained with a single stick procedure.

153 If it recurs, placement of a thoracoamniotic shunt is

an option. 154 A series of 65 fetuses showed thoracoamniotic shunts

to have a high survival rate in nonhydropic fetuses of 93%, with

survival of 86% in the hydropic group. 4 Risks of prenatal thoracoamniotic

shunts include fetal hemorrhage, blockage or migration

of the shunt, and placental abruption or preterm labor. In cases

of large efusions, drainage immediately before delivery can assist

in airway management at birth. A 2007 systematic review of

prenatal intervention for isolated primary pleural efusion without

hydrops suggested that survival rates ater prenatal intervention

are as high as 60%. 138

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