Anemia of Prematurity - Portal Neonatal

TREATMENT Section 6 of 10
Medical Care: The single most important factor to ensure proper treatment of the fetus with hydrops is
a precise and detailed diagnosis. Until the underlying pathophysiology is clearly understood and the
extent of the abnormalities leading the development of hydrops is defined completely, any attempt at
treatment is futile and potentially harmful.
• Once the underlying problems are understood completely, address the question of whether
the abnormalities present are compatible with life, whether fetal survival would be at the cost
of an unacceptably poor quality of life, and what the consequences may be for future
generations. Currently, parental involvement and guidance are fundamental requirements and
require full knowledge by the parents of all possible potential consequences.
• If the decision is made to continue the pregnancy, the next steps are to decide whether to
intervene with invasive fetal treatment(s) and to determine at what point preterm delivery
represents less risk for the fetus than continued gestation. Because major uncertainties about
these questions inevitably exist, regardless of the underlying cause(s), full parental
involvement is essential.
• Decisions about fetal treatment are inevitably uncertain because the necessary evidence is
not available. While many anecdotal approaches are found in the literature, no properly
designed clinical trials are available for the clinician concerned with evidence-based
management.
o Many treatment schemes exist; however, all are based on the biases and experiences of
the individual author(s). In such circumstances, treatment decisions are difficult,
particularly for the prudent clinician who requires evidence to balance risks against
benefits of a specific treatment.
o To further complicate the issue, spontaneous remission of the hydropic process has been
reported in hundreds of cases. Underlying causes in these cases include cardiac
arrhythmias, twin-to-twin transfusion syndrome, pulmonary sequestration, cystic
adenomatoid malformation of the lung, lysosomal storage diseases, cystic hygroma with
or without Noonan syndrome, both Parvovirus and CMV infections, placental
chorangioma, and idiopathic ascites or pleural effusions. Both clinician(s) and parent(s)
completely must understand that decisions at this point basically are uncertain and
arbitrary.
• Unproven high-risk treatments are easier to accept when they consist of procedures targeted
to correct the underlying pathophysiology leading to fetal hydrops. Thus, the most widely
accepted management schemes include fetal transfusion to correct anemia (regardless of
cause), drug treatments for cardiac arrhythmias, correction or reduction of space-occupying
lesions that impede cardiac venous or lymphatic return, and procedures designed to stop fetal
loss of blood, regardless of cause.
• Treatment reported for fetal arrhythmias has included doing nothing, administering drugs, and
immediate delivery.
o If fetal maturity permits, the most simple and direct approach is obviously delivery of the
affected fetus and direct neonatal treatment of the arrhythmia.
o When fetal immaturity prevents this approach, use of drugs has generally been accepted
as appropriate. However, whether this is justified is not supported by any evidence from
controlled clinical trials, and the frequency with which spontaneous cessation of the
arrhythmia and remission of the hydrops has been reported should promote more
skepticism and caution about fetal drug treatment than generally has been standard.
o Drugs have been administered to the mother (oral, intramuscular, intravenous), to the
fetus (intraperitoneal, intramuscular, intravenous via cordocentesis), and to both,
attempting to correct fetal arrhythmias.
o Even fetal pacing has been reported. As perhaps expected, the failures are infrequently
reported while the successes serve as topics for case or case-series reports (ie, reporting
bias). Such treatment is not without risk, partly consequent to the drugs used and not
uncommonly related to the mode of administration.
o Drugs used have included digitalis, furosemide, flecainide, verapamil, amiodarone,