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Anemia of Prematurity - Portal Neonatal

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o Karyotyping is always indicated if history or ultrasound results reveal a constellation <strong>of</strong><br />

findings consistent with a chromosomal aberration or if maternal or family history is<br />

suggestive.<br />

� Chromosome studies are indicated whenever initial diagnostic studies have failed to<br />

identify with certainty a specific cause for the fetal hydrops.<br />

� Chromosomal analyses may be performed on desquamated fetal epithelial cells in AF,<br />

fetal tissue biopsy samples, or placental (fetal tissue) biopsy samples.<br />

� An increase in AFP has been observed in almost 1 in 10 (8.4%) genetic<br />

amniocenteses; fetal mortality exceeded 1 in 10 (14%) when such AFP elevations<br />

occurred. Evidence <strong>of</strong> fetomaternal bleeding is present in 3 <strong>of</strong> 4 chorionic villous<br />

samplings. Thus, careful weighing <strong>of</strong> benefit versus risk must be made whenever<br />

direct invasive diagnostic methods are considered.<br />

o To obtain more precise information concerning fetal status, direct fetal sampling by<br />

cordocentesis (or periumbilical sampling) has been used with increasing frequency.<br />

� Acidemia, hypoxemia, and hypercarbia are found in most studies <strong>of</strong> fetal acid-base<br />

balance and blood-gas status obtained at time <strong>of</strong> direct fetal treatment. These results<br />

are nonspecific and anticipated and, while they may be <strong>of</strong> use in immediate<br />

management, are unlikely to be <strong>of</strong> value in longer-term care <strong>of</strong> the fetus with hydrops.<br />

� Analyses <strong>of</strong> serous effusion fluids (pleural, pericardial, or ascitic, most commonly)<br />

have been <strong>of</strong> surprisingly little value. For example, lymphocyte counts considered<br />

characteristic <strong>of</strong> congenital chylothorax when found in the newborn infant have been<br />

observed in pleural effusions from fetuses with CMV disease.<br />

� Serologic tests for specific infections, hemoglobin or hematocrit measurements,<br />

platelet counts, white cell counts and morphologic analyses, specific enzyme<br />

analyses, and karyotyping are just a few <strong>of</strong> the more common measurements<br />

obtained. While this information may be invaluable in specific cases, use <strong>of</strong> such<br />

invasive methods on a routine basis carries significant risks.<br />

� Fetal sampling by cordocentesis is followed by significant bradycardia in almost 1 in<br />

20 samplings (3.8%); <strong>of</strong> those with such complications, almost two thirds die (61.5%).<br />

o Elevated AF alkaline phosphatase has been observed in association with fetal hydrops<br />

due to Turner syndrome; while likely to be a nonspecific finding, further study is<br />

necessary.<br />

• The fetal biophysic pr<strong>of</strong>ile has been demonstrated to be abnormal in severe fetal hydrops.<br />

o Cardiotocographic records obtained 12 hours prior to fetal death demonstrate absence <strong>of</strong><br />

short-term and long-term variability, absence <strong>of</strong> tachycardia, presence <strong>of</strong> late<br />

decelerations, and terminal bradycardia.<br />

o Sinusoidal heart-rate patterns have been observed consistently in hydrops associated with<br />

severe fetal anemia related to isoimmunization and fetomaternal hemorrhage.<br />

Imaging Studies:<br />

• Once the possibility <strong>of</strong> fetal hydrops is considered or suspected, sophisticated and complete<br />

fetal imaging studies are an initial absolute necessity. Hydrops is defined by the presence <strong>of</strong><br />

serous effusion(s) in a fetus with subcutaneous tissue edema. Some authors have<br />

distinguished the presence <strong>of</strong> a single effusion (pleural, peritoneal) as an entity distinct from<br />

hydrops; however, recent evidence suggests that an isolated effusion <strong>of</strong>ten (if not usually)<br />

progresses to overt fetal hydrops. Exceptions appear to be isolated chyloperitoneum/ascites<br />

(usually associated with obstructive uropathy and, thus, not true hydrops) and pleural or<br />

peritoneal effusions that regress spontaneously (see Treatment). Thus, careful and complete<br />

imaging is required to establish the diagnosis and the extent <strong>of</strong> the hydrops.<br />

• Equipment employed must be capable <strong>of</strong> providing high-resolution images at large depth.<br />

o Linear array transducers are commonly used; however, sector scanners provide better<br />

views <strong>of</strong> the heart and many other structures.<br />

o Range-gated Doppler capability is optimal for functional physiologic assessments. Use<br />

<strong>of</strong> high-frequency transvaginal 2-dimensional and pulsed-wave/color Doppler flow<br />

mapping particularly has been promising.<br />

• The initial imaging study may provide important clues concerning the origin <strong>of</strong> the fetal

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