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von Hippel-Lindau disease and pregnancy: what an obstetrician should know.
[von hippel-lindau disease]
von
Hippel-
Lindau
disease
is
an
autosomal
dominant
genetic
disorder
that
presents
with
a
variable
constellation
of
symptoms
and
signs
due
to
formation
of
hemangioblastomas
and
cysts
.
We
address
the
management
of
pregnancies
associated
with
this
disorder
from
an
obstetric
perspective
.
A
26
-
year
-old
known
to
have
type
2
A
von
Hippel-
Lindau
disease
with
a
significant
surgical
history
secondary
to
disease
manifestations
was
managed
from
early
pregnancy
to
delivery
.
A
literature
review
to
determine
the
best
evidence-based
care
from
an
obstetric
perspective
,
addressing
patient
management
preconceptionally
,
in
the
antepartum
period
(
including
antepartum
surgical
care
)
,
during
labor
and
delivery
(
including
type
of
analgesia
/
anesthesia
,
mode
of
delivery
)
,
and
the
postpartum
period
,
as
well
as
contraception
,
was
performed
.
A
pregnant
woman
with
von
Hippel-
Lindau
disease
should
be
managed
in
conjunction
with
a
multidisciplinary
team
.
Routine
clinical
and
radiological
imaging
should
be
continued
within
safe
parameters
of
pregnancy
.
Mode
of
delivery
,
analgesia
/
anesthesia
,
and
postpartum
care
(
including
birth
control
)
should
be
determined
based
on
each
patient
's
disease
phenotype
and
individual
risk-
to
-benefit
ratio
on
a
case-
by
-case
basis
.
