Original Paper

Pediatr Neurosurg 2012;48:6–12 Received: March 5, 2012

Accepted after revision: May 21, 2012
DOI: 10.1159/000339850
Published online: July 21, 2012

Endoscopic Choroid Plexus Coagulation in Infants

with Hydranencephaly or Hydrocephalus with a
Minimal Cortical Mantle
David I. Sandberg a Parthasarathi Chamiraju b Garrett Zoeller b Sanjiv Bhatia b
John Ragheb b
a
Division of Neurological Surgery, Departments of Pediatric Surgery and Neurosurgery, University of
Texas-Houston, Children’s Memorial Hermann Hospital, and Mischer Neuroscience Institute, Houston, Tex., and
b
Division of Neurological Surgery, Miami Children’s Hospital and University of Miami Miller School of Medicine,
Miami, Fla., USA

Key Words Three of 4 patients who had a successful endoscopic proce-

Hydranencephaly ⴢ Choroid plexus coagulation ⴢ Third
ventriculostomy ⴢ Endoscopic management
Abstract
Background/Aims: This study evaluates endoscopic cho-
roid plexus coagulation, in conjunction with third ventricu-
lostomy when technically feasible, as a strategy to treat pa-
tients with hydranencephaly or hydrocephalus with a mini-
mal cortical mantle. Methods: We retrospectively reviewed
patients with hydranencephaly (n = 4) or hydrocephalus with
the cortical mantle !1 cm in maximal thickness (n = 4) who
underwent endoscopic choroid plexus coagulation from
2007 to 2010. Endoscopic third ventriculostomy was per-
formed simultaneously when technically feasible (in 3 of 8
patients). Endoscopic management was considered success-
ful if a shunt was not subsequently required. Results: Endo-
scopic management was successful in 4 of 8 patients (50%)
who did not require a shunt over a median follow-up period
of 15 months (range 2.5–24). Four patients (50%) failed endo-
scopic management and required a shunt 1 month to 11
weeks after endoscopic surgery. One patient died 4 days af-
ter shunt placement due to pneumonia and renal failure.
dure ultimately died of causes that were likely unrelated to
hydrocephalus (pneumonia in 2 patients and sudden infant
death syndrome in 1 patient). Besides failure to control hy-
drocephalus adequately in 4 patients, there were no addi-
tional complications noted after endoscopic surgeries. Con-
clusion: Endoscopic choroid plexus coagulation can enable
some infants with hydranencephaly or massive hydrocepha-
lus to avoid a ventriculoperitoneal shunt.
Copyright © 2012 S. Karger AG, Basel
Introduction
Hydranencephaly is defined as total or near total ab-
sence of the cerebral hemispheres with an intact cranium
and meninges. Typically, the brainstem, thalamus and
cerebellum are spared, and patients usually have progres-
sive macrocephaly [1]. While a majority of patients with
hydranencephaly die during their first 2 years of life due
to various complications, others survive well into early
childhood and beyond [2]. Cerebrospinal fluid (CSF) di-
version is typically offered for patients with progressive
macrocephaly, a full fontanelle, or other signs and symp-

© 2012 S. Karger AG, Basel David I. Sandberg, MD

1016–2291/12/0481–0006$38.00/0 Division of Neurological Surgery, University of Texas-Houston
Fax +41 61 306 12 34 6431 Fannin Street, Suite 6.264
E-Mail karger@karger.ch Accessible online at: Houston, TX 77030 (USA)
www.karger.com www.karger.com/pne Tel. +1 713 500 7285, E-Mail David.I.Sandberg @ uth.tmc.edu
toms of elevated intracranial pressure as a palliative
means of maintaining a more normal head size and fa-
cilitating care of the child by the patient’s family.
Shunt placement in children with hydrancencephaly
or hydrocephalus with a thin cortical mantle is associated
with many complications. Despite the fact that CSF fills
the greater portion of the supratentorial intradural space,
proximal shunt malfunction may occur repeatedly in
these patients. The commonly reported complications of
shunting such as malfunction, CSF leak, infection, dis-
connection, and skin breakdown lead to a shunt failure
rate approaching 50% in the first year after shunt place-
ment [3]. Patients with massive hydrocephalus with a thin
cortical mantle are also prone to subdural hematomas af-
ter shunting, as bridging veins can be disrupted with de-
compression of the ventricles.
Coagulation of the choroid plexus, first reported by
Dandy [4] in 1918, has been revisited in recent years in
conjunction with endoscopic third ventriculostomy as a
means of treating hydrocephalus endoscopically and
avoiding a shunt and its associated complications. While
endoscopic procedures to treat hydrocephalus often have
a lower success rate in infants and young children than in
older children and adults [5, 6], Warf [7] reported in a
large series of African children that adding coagulation
of the choroid plexus to endoscopic third ventriculosto-
my can greatly increase this success rate. Two previous
publications have focused on endoscopic choroid plexus
coagulation specifically in patients with hydranencepha-
ly [5, 8]. While both of these studies described success
using this approach, the number of patients was small
(n = 3 and 4, respectively). In 2010, Malheiros et al. [9]
performed a trial randomizing 17 patients with hydran-
encephaly or near hydranencephaly to endoscopic cho-
roid plexus cauterization versus ventriculoperitoneal
shunting. Eight of 10 patients (80%) randomized to endo-
scopic treatment avoided subsequent CSF diversion. We
add to this existing literature by reporting our results
when performing endoscopic choroid plexus coagula-
tion, combined with third ventriculostomy when techni-
cally possible, in a series of 8 patients with hydranenceph-
aly or massive hydrocephalus with the cortical mantle
!1 cm.
Patients and Methods
After Institutional Review Board approval was obtained from
the University of Miami (IRB study number 20091035), we retro-
spectively reviewed medical records from 72 infants ! 2 years of
age who underwent endoscopic procedures for hydrocephalus at
Endoscopic Choroid Plexus Coagulation
Miami Children’s Hospital and Jackson Memorial Hospital. Of
these 72 patients, we identified 8 patients between 2007 and 2010
who underwent endoscopic choroid plexus coagulation for hy-
drancencephaly (n = 4) or massive hydrocephalus with the corti-
cal mantle !1 cm in maximal thickness (n = 4). These 8 patients
form the basis of the current study.
Patient characteristics are listed in table 1. Seven patients were
born at full term, and 1 patient was born premature (at 25 weeks
gestation). Four were males and 4 were females. On computed to-
mography (CT) and/or magnetic resonance imaging (MRI) stud-
ies, 4 patients had hydranencephaly and 4 patients had massive
hydrocephalus with the cortical mantle !1 cm in maximal thick-
ness. One of the latter patients was also noted to have a Dandy-
Walker malformation. In all patients, surgery was performed due
to macrocephaly and clinical signs of elevated intracranial pres-
sure such as fullness of the fontanelle and splayed sutures. Five
patients underwent endoscopic surgery within the first 2 weeks of
life. Two patients underwent surgery at 2 months of age; one of
these was a premature infant who had a reservoir placed first for
management of progressive macrocephaly. One patient had sur-
gery at 9 months of age after not being offered treatment for pro-
gressive macrocephaly at another institution due to her presumed
poor overall prognosis.
All patients underwent endoscopic coagulation of the choroid
plexus using a rigid endoscope (Aesculap or Karl Storz). An inci-
sion was made at the lateral aspect of the fontanelle, and addi-
tional bone was removed from around the coronal suture as need-
ed. After opening the dura, the endoscope was introduced either
directly into the ventricle (in patients with hydranencephaly) or
through a thin layer of cerebral cortex (in patients with a thin cor-
tical mantle). All visible choroid plexus was coagulated using bi-
polar and/or monopolar cautery. In 3 patients, endoscopic third
ventriculostomy was performed simultaneously using standard
techniques. In the remaining 5 patients, endoscopic third ven-
triculostomy was not performed because of fused thalami or oth-
er abnormal ventricular anatomy as judged by the neurosurgeon.
Endoscopic procedures were considered successful if subsequent
CSF diversion via shunting was not required. A shunt was placed
subsequently if, despite the endoscopic procedure, progressive
macrocephaly was noted along with other signs of elevated intra-
cranial pressure such as a full fontanelle and/or splayed cranial
sutures.
Results
Endoscopic management was successful in 4 of 8 pa-
tients (50%) who did not require a shunt over a median
follow-up period of 15 months (range 2.5–24). Besides
failure to control hydrocephalus adequately in the 4 pa-
tients who required a shunt, there were no additional
complications noted after endoscopic surgery. Specifical-
ly, there were no infections, CSF leaks, pseudomeningo-
celes, new neurological deficits, or any other recognized
complications. Three of 4 patients who had a successful
endoscopic procedure ultimately died of causes that were
not believed to be related to hydrocephalus (pneumonia
Pediatr Neurosurg 2012;48:6–12 7
Table 1. Patient features, imaging findings, surgical procedures, and results for patients with hydranencephaly or near hydranenceph-
aly managed endoscopically

Pa- Age at time of Sex Imaging findings Surgical procedure performed Results
tient endoscopic (CT and/or MRI)
surgery

1 2 days male hydrocephalus with cortical mantle endoscopic choroid plexus coagulation shunt required 1 month
<1 cm; Dandy-Walker malformation postoperatively
2 9 months female hydranencephaly endoscopic choroid plexus coagulation no subsequent CSF diversion
and third ventriculostomy required; died 23 months
postoperatively of pneumonia
3 1 day female hydranencephaly endoscopic choroid plexus coagulation no subsequent CSF diversion
and third ventriculostomy required; died 8 months
postoperatively of pneumonia
4 1 day male hydrocephalus with cortical mantle endoscopic choroid plexus coagulation shunt required 11 weeks
<1 cm and third ventriculostomy postoperatively
5 2 months male hydranencephaly endoscopic choroid plexus coagulation no subsequent CSF diversion
required over 2-year follow-up
period
6 9 days male hydrocephalus with cortical mantle endoscopic choroid plexus coagulation shunt required 1 month
<1 cm postoperatively
7 2 months female hydrocephalus with cortical mantle endoscopic choroid plexus coagulation no subsequent CSF diversion
<1 cm required; died 10 weeks
postoperatively of sudden infant
death syndrome
8 5 days female hydranencephaly endoscopic choroid plexus coagulation shunt required 1 month
postoperatively; died of aspiration
pneumonia and renal failure 4 days
after shunt placement

in 2 patients 8 and 23 months postoperatively, respec-
tively, and sudden infant death syndrome 10 weeks post-
operatively in 1 patient). Four patients (50%) failed endo-
scopic management and required a shunt 1 month to 11
weeks after endoscopic surgery. One of these 4 patients
died 4 days after shunt placement due to pneumonia and
renal failure when care was withdrawn by the patient’s
family.
Only 1 of 3 patients (33.3%) who underwent endoscop-
ic third ventriculostomy in conjunction with choroid
plexus coagulation required subsequent CSF diversion,
while 3 of 5 patients (60%) who underwent choroid plex-
us coagulation alone required subsequent CSF diversion.
Also, only 1 of 4 patients (25%) with hydranencephaly re-
quired subsequent CSF diversion after endoscopic sur-
gery, while 3 of 4 patients (75%) who had hydrocephalus
with a thin cortical mantle failed endoscopic surgery and
required shunt placement. The small number of patients
in this series precluded meaningful statistical analysis
comparing these groups.
Three illustrative cases (one failure and two successes)
are presented below to further highlight patient features
in this series.
Case Example of Failed Endoscopic Choroid Plexus
Coagulation
Patient No. 1 is a 2-day-old boy who was born at full
term with a full fontanelle and massive macrocephaly.
His head circumference was 56 cm at birth. A CT scan
demonstrated massive hydrocephalus with a thin cortical
mantle (fig. 1a) and an associated Dandy-Walker malfor-
mation (fig. 1b). An endoscopic choroid plexus coagula-
tion was performed. Simultaneous third ventriculostomy
was not performed because it was judged not to be feasible
based upon the abnormal ventricular anatomy. One week
postoperatively, the fontanelle was sunken and the head
circumference was 44 cm. However, the head circumfer-
ence progressively grew and the fontanelle became fuller
over the subsequent 3 weeks, and a ventriculoperitoneal
shunt was placed 1 month after the initial endoscopic sur-
gery. Over the following 5 years of follow-up, the patient
has required 6 shunt revisions.
Case Example of Successful Avoidance of Shunt
Patient No. 2 is a 9-month-old girl who was born at full
term and was treated at an outside hospital before presen-
tation to our institution at 9 months of age. At the outside

8 Pediatr Neurosurg 2012;48:6–12 Sandberg /Chamiraju /Zoeller /Bhatia /

       

Ragheb  
Fig. 1. a Non-contrast CT scan demon-
strating massive ventriculomegaly with a
thin cortical mantle in patient No. 1.
b Non-contrast CT scan in patient No. 1
demonstrating associated Dandy-Walker
malformation. a b

Color version available online

Fig. 2. a Photo of the abdomen of patient
No. 2 demonstrating massive abdominal
hernia despite four previous surgeries and
gastrostomy tube. b Non-contrast CT scan
from patient No. 2 demonstrating hydran-
encephaly. a b

hospital, she underwent four abdominal surgeries for a
giant abdominal hernia. Despite these surgeries, she still
had a large abdominal hernia and a gastrostomy tube at
presentation to our institution (fig. 2a). She was noted on
a CT scan to have hydranencephaly (fig. 2b). Despite the
fact that she had a very full fontanelle and progressive
macrocephaly, no intervention was offered for hydro-
cephalus at the outside institution due to her perceived
overall poor prognosis. Her complex abdomen made her
a poor candidate for a ventriculoperitoneal shunt. An en-
doscopic choroid plexus coagulation and third ventricu-
lostomy was performed. Her head circumference was 48
cm preoperatively and 46 cm 2 months postoperatively
with minimal growth thereafter. She died of pneumonia
23 months postoperatively. She did not require subse-
quent CSF diversion during the 23 months of follow-up
after endoscopic surgery until her death.
Second Case Example of Successful Avoidance of
Shunt
Patient No. 3 is a 1-day-old girl who was born at full
term. Her head circumference was 43 cm at birth, and her
fontanelle was full. An MRI scan demonstrated hydran-
encephaly (fig. 3a). An endoscopic choroid plexus coagu-
lation and third ventriculostomy were performed. No
subsequent CSF diversion was required. Preoperatively,
her head circumference was 43 cm. Six months postop-
eratively, at the time of her last neurosurgical follow-up,
her head circumference was 39 cm and a CT scan showed
overriding sutures and concave frontal bones (fig.  3b).
Two months after this visit (8 months postoperatively),
the patient died of pneumonia.

Endoscopic Choroid Plexus Coagulation Pediatr Neurosurg 2012;48:6–12 9

Fig. 3. a Axial T2-weighted MRI scan from
patient No. 3 at presentation demonstrat-
ing hydranencephaly. b Non-contrast CT
scan in patient No. 3 six months postop-
eratively demonstrating overriding su-
tures and concave frontal bones. a b
Discussion
Hydranencephaly is defined as complete or near com-
plete absence of the cerebral hemispheres with an intact
cranium, brainstem and meninges [1, 10, 11]. CSF fills the
supratentorial space which is normally inhabited by the
cerebral hemispheres. When hydranencephaly coexists
with hydrocephalus secondary to aqueductal stenosis, it
is typically accompanied by rapid enlargement of the
head both in utero and after birth [12]. The etiology of
hydranencephaly is unclear, but an infectious cause, a
vascular event, or a genetic abnormality have all been
postulated [13]. Prognosis in patients with hydranen-
cephaly is poor, and many of these patients die early in
childhood either because of pneumonia or various other
complications [1, 10, 11]. However, survivors well beyond
this time point are common, and if CSF diversion is not
offered, care of these children is complicated by massive
macrocephaly and resulting scalp pressure ulcers and
other complications.
In another subset of infants with hydrocephalus, the
supratentorial cranial compartment is filled almost com-
pletely with CSF, but a very thin cortical mantle is pres-
ent. In some of these patients, the cortical mantle is so
thin that it is difficult to discern on imaging studies. Out-
comes for this specific subset of infants are not as well
defined in the neurosurgical literature, but most patients
are developmentally delayed. In both patients with hy-
dranencephaly and hydrocephalus with a thin cortical
10 Pediatr Neurosurg 2012;48:6–12
mantle, numerous shunt revisions may be required after
shunt placement, and these revisions are associated with
significant potential morbidity. When the cortical mantle
is absent or thin, CSF leak and subsequent skin break-
down and/or meningitis may result. Moreover, while in-
tuitively it would seem that proximal catheter obstruc-
tion would be uncommon when a catheter is situated
within a supratentorial compartment filled with CSF,
proximal catheter obstruction may still be observed fre-
quently in this patient population. Due to the morbidity
and the expense associated with shunting and its compli-
cations in this challenging group of patients, alternative
means of CSF diversion are desirable.
Choroid plexus coagulation to control hydrocephalus
was first described by Dandy [4] in 1918. This procedure
was performed by an open technique and had a high mor-
tality rate, ranging from 43 to 46%. Endoscopic choroid
plexus coagulation was first described in 1934 by Putnam
[14] and achieved satisfactory control of hydrocephalus in
only 5 out of 22 patients. With advances in endoscopy and
optics facilitating better visualization, endoscopic coagu-
lation of the choroid plexus has been revisited as an op-
tion in treating patients with hydrocephalus. The largest
published series describing endoscopic choroid plexus
coagulation was reported in 2005 by Warf [7]. In 550 Af-
rican children !1 year of age, combining choroid plexus
coagulation with endoscopic third ventriculostomy had a
greater success rate than when endoscopic third ventricu-
lostomy was performed alone. The majority of patients in
Sandberg /Chamiraju /Zoeller /Bhatia /
Ragheb  
this study had postinfectious hydrocephalus, and subsets
of patients with hydranencephaly or massive hydroceph-
alus with a thin cortical mantle were not identified with-
in this large cohort.
Only three previous publications review the results of
choroid plexus coagulation in patients with hydranen-
cephaly and/or near hydranencephaly. In 1981, Albright
[8] reported that endoscopic coagulation of the choroid
plexus successfully controlled elevated intracranial pres-
sure in 2 of 3 patients with hydranencephaly. In 2002,
Wellons et al. [5] retrospectively compared results be-
tween 9 patients with hydranencephaly who underwent
shunt procedures and 4 patients who underwent coagula-
tion of the choroid plexus via craniotomy. These authors
reported a lower incidence of reoperation and readmis-
sion to the hospital in the patients who underwent cho-
roid plexus coagulation. In the largest published series to
date, Malheiros et al. [9] reported a prospective, random-
ized trial comparing endoscopic choroid plexus cauter-
ization with ventriculoperitoneal shunting in 17 patients
with hydranencephaly or near hydranencephaly. Eight of
10 patients (80%) randomized to endoscopic treatment in
this study avoided subsequent CSF diversion.
In all three previously reported publications described
above for this patient population, choroid plexus coagula-
tion was performed without attempted simultaneous
third ventriculostomy. Based upon the impressive results
of Warf [7] when combining these two procedures, our
approach has been to combine endoscopic third ventricu-
lostomy with choroid plexus coagulation when the intra-
ventricular anatomy is favorable. In this series, combin-
ing the two procedures was only possible in 3 of 8 patients
due to fused thalami or difficulty identifying normal
landmarks for safe third ventriculostomy. While the
small number of patients in this series precludes statisti-
cal analysis, it is at least worth noting that only 1 of the 3
patients (33.3%) who underwent the combined procedure
required subsequent shunting, while 3 of 5 patients (60%)
who underwent choroid plexus coagulation alone re-
quired a shunt. A larger number of patients would be re-
quired to determine if combining the two procedures
truly leads to a higher success rate than when choroid
plexus coagulation is performed alone.
It is also worth noting that only 1 of 4 patients (25%)
with hydranencephaly required subsequent CSF diver-
sion after endoscopic surgery, while 3 of 4 patients (75%)
who had hydrocephalus with a thin cortical mantle failed
endoscopic surgery and required shunt placement. Again,
the number of patients in this series was too small to per-
form meaningful statistical analysis assessing this find-
ing. Because hydranencephaly and near hydranencepha-
ly are relatively uncommon, it would likely require a
study involving multiple centers to obtain statistically
significant data.
One can conclude from this study as well as the previ-
ously reported publications on this patient population
that endoscopic choroid plexus coagulation, with or
without simultaneous third ventriculostomy, is a safe
procedure with a low complication rate. Patients in this
population have a high mortality rate from causes such
as pneumonia which are not attributable to hydrocepha-
lus. However, long-term survivors with hydranencephaly
and near hydranencephaly are frequently observed in
modern pediatric neurosurgical practices. While a sig-
nificant proportion of patients, 50% in our series, still
ultimately require a shunt, those who avoid a shunt are
spared the significant potential morbidity associated
with shunting.
In conclusion, endoscopic choroid plexus coagulation
is a reasonable alternative to ventriculoperitoneal shunt-
ing in patients with hydranencephaly and near hydran-
encephaly. Performing simultaneous endoscopic third
ventriculostomy may be beneficial when allowed by fa-
vorable ventricular anatomy. Because hydranencephaly
and near hydranencephaly are relatively uncommon, a
multi-center collaborative study would be useful to deter-
mine optimal management in these patients.

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