The Turkish Journal of Pediatrics
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Endoscopic Treatment of Cavum Vergae Cyst: Case Report and Review
Nurperi Gazioğlu, Ali Metin Kafadar, Bashar Abuzayed
Department of Neurosurgery, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey
|A 3.5-year-old boy was admitted with febrile convulsion for the first time.
The patient had a medical history of in vitro fertilization (IVF) and premature
delivery in the 21st week of pregnancy. Further, he suffered from neonatal
germinal matrix hemorrhage and intraventricular hematoma (IVH). The patient
was treated in the pediatric intensive care unit. Cranial magnetic resonance
imaging (MRI) showed resorption of the hematoma with the presence of
cavum vergae (CV) variation. The new brain MRI revealed progression of
the cavum to a large CV cyst compared with the previous imagings, causing
pressure upon both lateral ventricles. Endoscopic fenestration of the lateral
walls of the cyst with lateral ventricles was performed. The postoperative
period was uneventful and the patient was discharged the next day. Followup
MRI one year after surgery showed normal ventricle size, shrinkage of
the cyst and cerebrospinal fluid (CSF) flow between the cyst and the lateral
ventricles. This is an interesting demonstration of the progression over years
of a CV anatomical variation to a large CV cyst in a premature birth case
that experienced germinal matrix hemorrhage.|
cavum vergae cyst, intraventricular hemorrhage, neuroendoscopy,
|First described by the Italian anatomist Vergae
in 1851, cavum vergae (CV) -also called the
sixth ventricle- is an intraventricular cyst
located between the corpus callosum above
and the commissure of the fornix below. It is
found frequently in preterm infants and term
neonates, and most disappear soon after birth.
However, persistent CV is detected in 15%
of the asymptomatic adult population. Some
cases become symptomatic with expanding
cysts causing obstruction of cerebrospinal fluid
(CSF) flow through the foramen of Monro,
leading to increases in intracranial pressure
(ICP) and hydrocephalus. Symptomatic
cases require surgical intervention, which
includes open surgical procedures, ventriculoperitoneal
shunting, stereotactic fenestration,
and neuroendoscopic cysto-ventriculostomy-.
Today, endoscopic cyst wall fenestration is the
method of choice in many neurosurgical centers.
The technique is minimally invasive, permits
direct visualization of the cyst wall and avoids
the need to place a shunt. While the incidence
of the symptomatic CV is rare, endoscopic
treatment of these cysts is even more rarely
reported in the literature-.
In this report, the authors present a case of
progressively enlarging CV cyst in a child,
who was successfully treated by endoscopic
fenestration of the cyst, together with a brief
review of the literature on this issue.|
|Case Presentation |
|A 3.5-year-old boy was admitted with febrile
convulsion for the first time. The patient had
a medical history of in-vitro fertilization (IVF)
and premature delivery in the 21st week of
pregnancy. During the follow-up in the neonatal
intensive care unit, the patient suffered
from neonatal germinal matrix hemorrhage
and intraventricular hematoma (IVH). The
hematoma was observed and follow-up brain
magnetic resonance imaging (MRI) showed
resorption of the hematoma and the presence of
a CV cyst (Fig. 1A, B). Thereafter, the patient
was followed in the pediatric neurology clinic
with a regular physiotherapeutic program and
MRI (Fig. 1C, D).
Seizure intervention was done with intravenous
phenobarbital and the patient was unconscious
for 20 minutes in the postictal period. Physical
examination and chest X-ray revealed upper
respiratory tract infection (URTI). Neurological
examination after the postictal period showed
general minimal motor retardation with
more significant spastic paresis of the lower
extremities. Mental and language development
were found to be normal. Brain computerized
tomography (CT) scan and brain MRI revealed
a large CV cyst compared to the cyst volume in
the previous imagings, causing pressure upon
both lateral ventricles (Fig. 1 E-H). Surgical
intervention was planned after completing the
antibiotic trial and resolving the infection.
The patient was operated under general
anesthesia. The endoscopic approach was
performed through the right frontal burr
hole, and the endoscope was introduced
into the right lateral ventricle. The cyst was
identified and was found covering the foramen
of Monro. The right wall of the cyst, which
was covered with a thin ependymal layer, was
fenestrated just posterior to the septal vein.
The cyst was entered and the opposite (left)
wall was identified and also fenestrated. The
procedure was completed without complication.
The postoperative period was uneventful and
the patient was discharged the next day. The
last follow-up MRI one year after the surgery
showed normal ventricle size with shrinkage
of the cyst and communication of the CSF
between the cyst and the lateral ventricle
(Fig. 1 I-L).
|Cavum vergae (CV) is considered a normal
variant that usually does not require surgical
treatment. However, some cases become
symptomatic by causing obstruction of CSF flow
through the foramen of Monro, leading to various
symptoms including headache, papilledema,
emesis, loss of consciousness, and visual and
sensorimotor disturbances caused by increased
ICP and hydrocephalus-. CV is classified
as a non-communicating or communicating
cyst, depending on whether or not the cyst
communicates with the cerebral ventricular
system. The communicating type is the most
common and is considered to be asymptomatic.
In contrast, non-communicating cyst, which
could originally be asymptomatic, may enlarge,
block the foramen of Monro, and cause
symptomatic hydrocephalus. A communicating
cyst may become a non-communicating cyst
through various mechanisms. For example,
the cyst might secrete fluid, possibly through
the presence of migrated ependymal cells-.
The check valve phenomenon between the
cyst and the subarachnoid cavity may be
implicated based on immunohistochemical
analysis. The involvement of minor head
injury was also implicated in the expansion of
these cysts. However, the exact mechanism
of CV expansion remains uncertain, since
most reported cases were only identified
after becoming symptomatic. In our case, the
presence of CV can be related to the prematurity
of the patient, complicated by IVH.
| ||Fig. 1. Brain MRI of our case in different life periods
demonstrating progressive expansion of the cyst and the
postoperative results. A-B: The first MRI during followup
in the neonatal ICU showing the resolved IVH and
formation of CV. C-D: The second MRI at the age of 1
year showing the classical CV with minimal expansion
compared to the first imagings. E-H: The third MRI at
the age of 3.5 years showing the massive expansion of
CV compressing the lateral ventricles bilaterally. I-L:
Postoperative MRI 1 year after the operation showing
regression of CV, decompression of the lateral ventricles
and the communication of CSF flow between the cyst
and the lateral ventricles (arrows).|
An enlarging cyst may obliterate the foramen
of Monro and/or the aqueduct of Sylvius and
create intermittent hydrocephalus that causes
symptoms, therefore justifying surgery. In these
reports, the enlargement of the cavum has been
attributed to a one-way valve mechanism.
Moreover, the absence of secretory ependymal
lining in these cavities has been proven by
many autopsy series,. Oteruelo suggested
a different mechanism and claimed that fluid
travels passively from the ventricle into the
cavum through the septal lamina by a pressure
gradient in the absence of any communication.
Resorption of the fluid is performed by septal
capillaries and veins. In several reports, it was
claimed that the underlying pathophysiological
mechanism of the symptoms is an intermittent
obstruction of the foramen of Monro that
results in raised ICP, and not the compression
of surrounding structures, that causes limbic
dysfunction,,-. Of interest, a dilated cavum
septum pellucidum has been associated with
psychiatric disorders, mainly schizophrenia-.
On the other hand, Bodensteiner et al. found
that CV alone does not identify individuals
at risk for cognitive disorders. In our case,
the patient was found to have normal mental
status but delayed motor development. This
was probably secondary to the history of
neonatal IVH and the pressure of the enlarging
cyst upon the surrounding neural tissue. Due
to the variable developmental outcomes, it
is important to make the correct diagnosis
of CV, which may be confused with cavum
septum pellucidum. CV is a cavity within the
septum pellucidum, and is located posterior
to an arbitrary vertical plane formed by the
columns of the fornix. On the other hand,
cavum septum pellucidum consists of two thin
translucent leaves that extend from the anterior
part of the body, the genu and the rostrum of
the corpus callosum, to the superior surface of
the fornix, forming the medial wall of the lateral
ventricles. The other differential diagnoses
include a dilated third ventricle, aneurysm of
the vein of Galen and an arachnoid cyst.
Bronstein and Weiner first reported prenatally
diagnosed cases of cava septum pellucidum et
vergae in 1992. These authors reported eight
cases diagnosed by midtrimester transvaginal
ultrasonography. Among these cases, associated
anomalies were detected in five fetuses. As a
result, they concluded that prenatal detection
of cavum septum pellucidum et vergae should
be followed by a detailed search for associated
anomalies. Sahinoglu et al. reported
ultrasonographic prenatal diagnosis of three
fetuses with dilated CV. One of these fetuses
had ventriculomegaly and lumbar meningocele.
Another patient required placement of a
stereotactic cystoperitoneal shunt at six months
of age because of intracranial hypertension and
progressive enlargement of the CV. The third
infant remained asymptomatic.
Historically, CV cysts have commonly been
treated by craniotomy with cyst fenestration
or removal or by cystoperitoneal shunting.
Cystoperitoneal shunting has proven to be a
safer, simpler and more effective treatment
method than open surgery, and is considered
to be the best treatment of a large cyst in an
infant or a young child. However, complications
related to the shunting procedure such as
obstruction, infection, hemorrhage, and lifelong
shunt dependence are still not uncommon
and should be considered.
With the high failure rate of shunts and the
high morbidity rate after craniotomy-based
approaches, such as postoperative neurological
deficits, seizures, subdural hygroma, and
meningitis, there is a trend toward minimally
invasive procedures. With the advances in
neuroendoscopy and the large experience
gained over the last 20 years, many articles
advocate the advantages of this procedure
to manage certain intracranial pathologies,
with identification of the indications and
surgical techniques-. The neuroendoscopic
approaches have gained in popularity and
have been favored for the treatment of these
lesions. This was supported by reports that
documented the high success rate (71%-
81%) of the neuroendoscopic treatment of
intraventricular cysts. Furthermore, seeking
more precise localization of the intraventricular
cyst to reduce the failures and complications,
a combination of neuroendoscopy with
stereotaxy,,, or neuronavigation system
application has been reported. Now, the
role of neuroendoscopy is standard for the
treatment of hydrocephalus by endoscopic
third ventriculostomy, aqueductoplasty,
intracranial arachnoid cyst fenestration,,
intraventricular and pineal tumor biopsy or
removal, and colloid cyst removal. The
neuroendoscopic approaches are considered
safe techniques, especially in the hands of
highly experienced surgeons in specialized
centers, permitting direct visualization of the
cyst wall and avoiding the need for a shunt.
The endoscopic surgical technique includes
cysto-ventricular fenestration to achieve
communication between the cyst and both
lateral ventricles. This is done by fenestration
of both lateral walls of the cyst, as shown in
our case. However, the failure and complication
rates still range between 2% and 7%. In
addition to technical failures, the most common
complications are CSF leakage, intraventricular
hemorrhage, intraparenchymal hemorrhage,
subdural hygroma, and hematoma. These
complications were not seen in our case.
1. Rossitch E Jr, Wilkins RH. Developmental midline
intracranial cysts. In: Wilkins RH, Rengachary SS (eds).
Neurosurgery (2nd ed) Vol. 3. New York: McGraw-Hill;
2. Fratzoglou M, Grunert P, Leite dos Santos A, Hwang P,
Fries G. Symptomatic cysts of the cavum septi pellucidi
and cavum vergae: the role of endoscopic neurosurgery
in the treatment of four consecutive cases. Minim
Invasive Neurosurg 2003; 46: 243–249.
3. Lancon JA, Haines DE, Raila FA, Parent AD,
Vedanarayanan VV. Expanding cyst of the septum
pellucidum: case report. J Neurosurg 1996; 85: 1127–
4. Chiu CD, Huang WC, Huang MC, Wang SJ, Shih
YH, Lee LS. Navigator system-assisted endoscopic
fenestration of a symptomatic cyst in the septum
pellucidum — technique and cases report. Clin Neurol
Neurosurg 2005; 107: 337–341.
5.. Kafadar A, Abuzayed B, Kucukyuruk B, Cetin E,
Gazioglu N. Intracranial migration of bone dust after
intraventricular neuroendoscopy complicating acute
hydrocephalus and removal of bone dust: case report.
Neurosurgery 2010; 67: E503-504.
6. Nishijima Y, Fujimura M, Nagamatsu K, Kohama
M, Tominaga T. Neuroendoscopic management of
symptomatic septum pellucidum cavum vergae cyst
using a high-definition flexible endoscopic system.
Neurol Med Chir (Tokyo) 2009; 49: 549-552.
7. Cohen AR. Expanding cava septi pellucidi and cava
vergae in children: report of three cases. Neurosurgery
1998; 42: 674-675.
8. Neciga EG, Peralda AG, Polaina M, Sureda B, Bautista
J. Cyst of septum pellucidum and Korsakoff’s psychosis.
Eur Neurol 1989; 29: 99–101.
9. Rosin E, Grosskopf D, Perre J. Morphology and
immunohistochemistry of a symptomatic septum
pellucidum cavum vergae cyst in man. Acta Neurochir
1997; 139: 366–372.
10. Nishimoto H, Wada T, Kuroda K, et al. [A case of the
development of cavum vergae after head trauma]. No
Shinkei Geka 2003; 31: 297–301. (Japanese)
11. Behrens P, Ostertag CB. Stereotatic management
of congenital midline cysts. Acta Neurochir 1993;
12. Oteruelo FT. On the cavum septi pellucidi and the
Cavum vergae. Anat Anz 1986; 162: 271–278.
13. Sencer A, Sencer S, Turantan I, Devecioğlu O.
Cerebrospinal fluid dynamics of the cava septi pellucidi
and vergae. Case report. J Neurosurg 2001; 94: 127-
14. Miyamori T, Miyamori K, Hasegawa T, Tokuda K,
Yamamoto Y. Expanded cavum septi pellucidi and
cavum vergae associated with behavioral symptoms
relieved by a stereotactic procedure: case report. Surg
Neurol 1995; 44: 471-475.
15. Donauer E, Moringlane JR, Ostertag CB. Cavum vergae
cyst as a cause of hydrocephalus, “almost forgotten”
? Successful stereotactic treatment. Acta Neurochir
1986; 83: 12–19.
16. Kwon JS, Shenton ME, Hirayasu Y, et al. MRI study
of cavum septum pellucidi in schizophrenia, affective
disorder and schizotypal personality disorder. Am J
Psychiatry 1998; 155: 509–515.
17. Bodensteiner JB, Schaefer GB, Craft JM. Cavum
septi pellucidi and cavum vergae in normal and
developmentally delayed populations. J Child Neurol
1998; 13: 120–121.
18. Bronstein M, Weiner Z. Prenatal diagnosis of dilated
cava septi pellucidi et vergae: associated anomalies,
differential diagnosis, and pregnancy outcome. Obstet
Gynecol 1992; 80: 838–842.
19. Loeser JD, Alvord EC. Agenesis of the corpus callosum.
Brain 1968; 91: 553–570.
20. Sahinoglu Z, Uludogan M, Delikara MN. Prenatal
sonographic diagnosis of dilated cavum vergae. J Clin
Ultrasound 2002; 30: 378–383.
21. Krauss JK, Mohadjer M, Milios E, Scheremet R,
Mundinger F. Image-directed stereotactic drainage
of the symptomatic cavum septi pellucidi et vergae.
Neurochirurgia (Stuttg) 1991; 34: 57-71.
22. Ciricillo SF, Cogen PH, Harsh GR, Edwards MS.
Intracranial arachnoid cysts in children: a comparison
of the effects of fenestration and shunting. J Neurosurg
1991; 74: 230-235.
23. Schroeder HW, Wagner W, Tschiltschke W, Gaab MR.
Frameless neuronavigation in intracranial endoscopic
neurosurgery. J Neurosurg 2001; 94: 72-79.
24. Cohen AR. Endoscopic ventricular surgery. Pediatr
Neurosurg 1993; 19: 127-134.
25. Hopf NJ, Grunert P, Fries G, Resch KD, Perneczky A.
Endoscopic third ventriculostomy: outcome analysis of
100 consecutive procedures. Neurosurgery 1999; 44:
26. Tamburrini G, D’Angelo L, Paternoster G, Massimi L,
Caldarelli M, Di Rocco C. Endoscopic management of
intra and paraventricular CSF cysts. Childs Nerv Syst
2007; 23: 645-651.
27. Tirakotai W, Schulte DM, Bauer BL, Bertalanffy H,
Hellwig D. Neuroendoscopic surgery of intracranial
cysts in adults. Childs Nerv Syst 2004; 20: 842-851.
28. Hellwig D, Bauer BL, Riegel T, Schmideck HH, Sweet
WH. Surgical management of intracranial arachnoid,
suprasellar, and Rathke’s cleft cysts. In: Schmidek HH,
Sweet WH (eds). Operative Neurosurgical Techniques:
Indications, Methods, and Results (4th ed) Vol. 1.
Philadelphia, PA: Saunders; 2000: 513-532.
29. Gumprecht H, Trost HA, Lumenta CB. Neuroendoscopy
combined with frameless neuronavigation. Br J
Neurosurg 2000; 14: 129-131.
30. Schroeder HW, Gaab MR. Endoscopic aqueductoplasty:
technique and results. Neurosurgery 1999; 45: 508-
31. Luther N, Cohen A, Souweidane MM. Hemorrhagic
sequelae from intracranial neuroendoscopic procedures
for intraventricular tumors. Neurosurg Focus 2005;
32. Gazioglu N, Kafadar AM, Tanriover N, Abuzayed B,
Biceroglu H, Ciplak N. Endoscopic management of
posterior fossa arachnoid cyst in an adult: case report
and technical note. Turk Neurosurg 2010; 20: 512-
33. Schroeder HW, Gaab MR. Neuroendoscopic approach
to lesions in the pineal region. Clin Neurol Neurosurg
1997; 99(Suppl): S39—S44.
34. Decq P, Le Guerinel C, Brugières P, et al. Endoscopic
management of colloid cysts. Neurosurgery 1998; 42:
35. Peretta P, Raggazi P, Galarza M, et al. Complications
and pitfalls of neuroendoscopic surgery in children. J
Neurosurg 2006; 105(Suppl): 187-193.
36. Schroeder H, Oertel J, Gaab MR. Incidence of
complications in neuroendoscopic surgery. Childs
Nerv Syst 2004; 20: 878-883.
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