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Anemia of Prematurity - Portal Neonatal

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most commonly thrombocytopenia, is found in approximately the same proportion<br />

<strong>of</strong> cases. Tumor size by sonography has not been demonstrated to be an<br />

independent prognostic factor; however, solid, highly vascular tumors lead to<br />

hydrops more <strong>of</strong>ten than those with a more cystic, less vascular structure.<br />

o Since chromosomal abnormalities and life-threatening anomalies are rare with<br />

sacrococcygeal sequestration, early diagnosis and aggressive fetal treatment are<br />

particularly important with this condition. While bloody AF secondary to rupture <strong>of</strong><br />

the highly vascular teratoma is not uncommon, diagnosis in most cases has been<br />

made only after hydrops has developed.<br />

o Early routine fetal imaging may be the only way in which early diagnosis can be<br />

made in this condition; however, the low incidence <strong>of</strong> sacrococcygeal teratoma<br />

may preclude cost-effective screening for this condition. Elevated concentrations <strong>of</strong><br />

alpha-fetoprotein (AFP) and/or acetylcholinesterase in AF have been found to<br />

accompany fetal sacrococcygeal teratoma, but the invasive sampling and low<br />

specificity appears to preclude these tests as routine screening procedures. While<br />

placental chorioangiomas are common (present in approximately 1% <strong>of</strong><br />

pregnancies), large vascular tumors with cardiovascular and hematologic<br />

consequences are very uncommon. When present, the pathophysiology is<br />

remarkably similar to that found with fetal sacrococcygeal teratomas. Diagnosis<br />

and techniques for early intervention are also similar.<br />

• Bronchopulmonary sequestration is a condition in which abnormal vascular supply and<br />

misplacement <strong>of</strong> a portion <strong>of</strong> the lung may lead to torsion <strong>of</strong> the affected lobes, pr<strong>of</strong>ound<br />

obstruction <strong>of</strong> lymphatic and venous return, and tension hydrothorax. This sequence <strong>of</strong><br />

events leads to fetal hydrops in perhaps one third <strong>of</strong> such cases. Although drainage <strong>of</strong> the<br />

hydrothorax, definitive diagnosis using color Doppler imaging, and fetal angiography have<br />

been described, and though fetal surgical excision <strong>of</strong> the affected portion <strong>of</strong> the lung may<br />

improve survival in this condition, nearly two thirds <strong>of</strong> these cases fail to be diagnosed<br />

before fetal death or birth occurs.<br />

• Cystic adenomatoid malformation <strong>of</strong> the lung may also lead to hydrops by mass<br />

compression <strong>of</strong> venous return. Because this condition is seldom associated with other<br />

malformations or with chromosomal abnormalities and because fetal surgical maneuvers<br />

have demonstrated considerable promise with some forms <strong>of</strong> the disorder, early and<br />

precise diagnosis using fetal imaging techniques is <strong>of</strong> critical importance. Pulmonary<br />

capillary-alveolar development is abnormal in this condition, and 3 degrees <strong>of</strong> severity,<br />

described initially by Stocker, have been used to predict prognosis.<br />

o Type I: The fetus with large (>2 mm), isolated cysts seldom develops hydrops, and<br />

spontaneous remissions have been reported. Drainage or excision <strong>of</strong> individual<br />

cysts has also been reported with generally favorable outcomes.<br />

o Type II: Poorer prognosis is associated in the fetus with smaller (

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