Antenatal Treatment of Hydrocephalus
by Ventriculoamniotic Shunting
Fredric D. Frigoletto, Jr, MD; Jason C. Birnholz, MD; Michael F. Greene, MD
WE HAVE previously shown that
serial percutaneous cephalocenteses
could be accomplished safely with
continuous ultrasound guidance.1 This
method provides only transient relief
of elevated intracranial pressure be-
cause of prompt fluid reaccumulation.
For editorial comment
see p 2498.
It is apparent that a continuous form
of decompression will be required for
effective antenatal treatment of hy-
drocephalus. Percutaneous placement
of an indwelling ventriculoamniotic
shunt is described.
Report of a Case
A 24 1/2-week-old male fetus was referred
by his mother's obstetrician when an
ultrasound examination obtained to re-
solve an apparent size-date disparity at 23
weeks of gestation disclosed fetal hydro-
cephalus. The mother was a 34-year-old,
gravida 4 para 3. Had the option of
terminating the pregnancy been available,
it would have been unacceptable to both
parents. After a thorough discussion with
emphasis on the experimental nature of
the procedure, they requested that mater-
nal transabdominal ventriculoamniotic
shunt placement be attempted.
Further ultrasound examination con-
firmed symmetrical ventricular dilatation.
Communication between the lateral ven-
tricles at the level of the cavum septum
pellucidum was noted, but no other intra
cranial abnormalities were seen (Fig 1).
During the initial evaluation, a facial cleft
on the right side (Fig 2) was noted, but no
other anomalies of the spine, skeleton, or
From the Departments of Obstetrics-Gynecology
(Drs Frigoletto and Greene) and Radiology (Dr
Birnholtz), Harvard Medical School and Brigham and
Women's Hospital, Boston.
Reprint requests to Brigham and Women's Hospi-
tal, 75 Francis St, Boston, MA 02115 (Dr Frigolet-
to).
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head were seen. The eye-movement pat
tern was normal developmentally,2 and
body movements were brisk and without
observed seizure activity. A Silastic cathe
ter with a pressure valve was inserted
through a left temporoparietal entry site,
using a thin-walled, 13-gauge needle with
a technique previously developed for
transperitoneal intrauterine fetal transfu
sion.' Clear CSF was initially obtained on
needle entry into the left lateral ventricle.
The valve portion of the catheter jammed
in the needle, and the shunt was pulled out
of the ventricle and lost in the maternal
abdomen. A second shunt was placed
immediately and without evident compli
cation during needle withdrawal. The CSF
was blood tinged. Despite satisfactory
positioning of the shunt, serial ultrasound
studies during the next ten days demon
strated a slight but progressive increase in
ventricular size. A second shunt placement
was performed at 26 Vi weeks of gestation
through a posterior right parietal entry
site, 13 days after the first procedure,
using a simple piece of 16-gauge Silastic
tubing, 20 cm long, with several side holes
at one end. Subsequent examinations dis
closed an immediate and progressive
decrease in the relative size of the ventri
cles (Fig 3).
Ten days later, at 28 weeks of gestation,
the mother experienced mild irregular
uterine contractions and leaking amniotic
fluid. Amniotic fluid obtained at that time
disclosed a lecithin-sphingomyelin ratio of
2.5:1 and a saturated phosphatidyl choline
level of 1,350 mg/dL. Episodes of fetal
bradycardia to 80 beats per minute were
noted on ultrasound examination and con
firmed on fetal monitoring. These were
interpreted to represent fetal distress, and
concern for fetal CNS infection led to
emergency delivery by cesarean section. A
1,310-g male infant with Apgar scores of 8
and 9 at one and five minutes was deliv
ered. Umbilical vein blood-gas studies
showed pH 7.27; Po^ 26 mm Hg; and Pco
48 mm Hg. A facial cleft on the right side
through the lip and palate and into the
Fig 1.Cranial base view showing hydro-
cephalus. Cerebral mantle (arrowheads) is
compressed. Portion of choroid plexus (C)
crosses midline defect.
right orbit was noted. Dubowitz examina
tion results were consistent with the
estimated 28-week gestation. Clear CSF
dripped freely from the right-sided
(straight) catheter, but there was no flow
from the valved catheter on the left. Both
catheters were removed shortly after
delivery, and cultures of their tips showed
no growth. (The third catheter was found
at the time of cesarean section free within
the maternal peritoneal cavity.)
Because of hypoventilation and apnea,
the infant required endotracheal intuba
tion and mechanical ventilation with mod
erate oxygen supplementation within the
first few hours of life. Postnatal cranial
ultrasound examination (day 1) and x-ray
computed tomography (CT) (day 2) dem
onstrated moderate dilatation of the later
al ventricles (left slightly greater than
right) with discrete midline cleft. An EEG
(day 3) was also abnormal, with asymme
try with lower voltage and less well
formed activity on the right compared
with the left. Although there was only
minimal increase of lateral ventricular
size during the next 72 hours, the infant
required a ventriculoperitoneal shunt for
increasing hydrocephalus at 2 weeks of
age. During the observation period, there
was no ultrasound evidence of ventriculi-
tis" and no ultrasound or CT indication of

Fig 2.Coronal (frontal) face view. Lens of
right eye (arrowhead) is centrally positioned
within orbit. Facial cleft runs obliquely from
inferior medial orbital margin to mouth (ar
row).
Fig 3.Coronal view after catheter place
ment demonstrates normal lateral ventricles
(v) and normal mantle thickness.
intraventricular hemorrhage. After shunt
placement, the infant's course was marked
by the requirement for continued mechan
ical ventilation, persistent hyperbili-
rubinemia, several episodes of presumed
diabetes insipidus, and, finally, general
ized seizures that could not be controlled
medically. He had three courses of antibi
otics for suspected sepsis, although results
of all cultures, including those of blood
and CSF, were negative. The last set of
cultures was eight days before death. His
shunt continued to function adequately.
Karyotype was 46,XY. He died at 5V
weeks of age after cardiac arrest. Permis
sion for postmortem examination was
denied.
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Comment
Experimental studies with fetal
rhesus monkeys provide a rationale
for invasive management of fetal
hydrocephalus.5* When extreme pre
maturity precludes delivery and
definitive neonatal management, ven
triculoamniotic shunt catheter place
ment may be considered. This case
demonstrates the capability of
achieving continuous decompression
and prolonged shunt patency with
Silastic tubing.
Optimal physical characteristics
for the shunt device remain to be
developed. The insertion needle diam
eter must be minimized to decrease
the risk to the mother of premature
labor, amniotic fluid leak, uterine
trauma, or placental separation
(when a transplacental route must be
used) and to reduce the risk of fetal
brain trauma. A valve within the
catheter would prevent substantial
reflux of amniotic fluid into the ven
tricle, although inclusion of a valve
will increase the potential for shunt
failure and may preclude use of a
small-bore insertion needle.
The importance of amniotic fluid
reflux is uncertain. Intracranial pres
sure should be equal to or greater
than intra-amniotic pressure-limiting
reflux. Inasmuch as solute concen
trations and tonicity of CSF and
amniotic fluid differ, a chemical ven-
triculitis, choroiditis, or alteration of
CSF production might occur, al
though there is no indirect evidence
that either of those complications
occurred in our case. Antenatal shunt
placement may not be appropriate for
every case of hydrocephalus diag
nosed definitively in utero. We recom
mend the following guidelines for
case selection.
1. The hydrocephalus should be
detected sufficiently early that deliv
ery and postnatal shunting are not
realistic options.
2. The hydrocephalus should ap
pear as a simple obstructive variety
without associated major dysmorphic
brain development.
3. The hydrocephalus should not be
associated with other major malfor
mations that are themselves incom
patible with survival or that indicate
an irremediable malformation syn
drome.
4. Each pregnancy should be evalu
ated for chromosomal abnormalities
and associated neural tube defects
during initial workup. (Operative
treatment need not be delayed while
awaiting results.)
5. The ventricular dilatation
should be progressive.
6. Pretreatment evaluation should
include a multidisciplinary team's
consultation with physicians in peri-
natology, neonatology, ultrasonogra-
phy, neurosurgery, and genetics.
These guidelines may be difficult to
adhere to in practice because of tech
nical considerations and subjective
needs of the parents. Each case must
be considered individually, and de
tailed evaluation should be repeated
serially.
Many issues and concerns7 have
been raised since the recent reporting
of in utero surgery; these are similar
to those raised when intrauterine
fetal transfusion was pioneered in
1963* for erythroblastosis fetalis.
During the interim, diagnostic evalu
ation, indications for use, utility, and
safety have been refined. A compara
ble evolution may be anticipated for
other percutaneous intrafetal proce
dures.'' The technical capability for
percutaneous indwelling catheter
placement exists and may be consid
ered when a clinical condition war
rants intervention, where profes
sional resources and equipment are
available, and when parents are fully
involved in the decision-making pro
cess.
The Antenatal Hydrocephalus Shunt was pro
vided by Denver Surgical Developments, Inc.
Ann Stark, MD, reviewed the manuscript.
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