Clinical Radiology (2002) 57: 759767

doi:10.1053/crad.2002.0960, available online at http://www.idealibrary.com on

Case Reports
Chiari III Malformation: Antenatal MRI Diagnosis
R . L E E , K . S . TA I , P. W. C H E N G , W. M . L U I , F. L. C H A N
*Department of Radiology, Queen Mary Hospital, Hong Kong, China and
{Division of Neurosurgery, Department of Surgery, Queen Mary Hospital, Hong Kong, China

Chiari III malformation is a rare congenital malformation.

This report presents a case of third trimester MRI diagnosis
of Chiari III malformation and discusses use of SSFSE
(single shot fast spin echo) sequence to avoid motion artifact.

CASE REPORT
A 29-year-old Chinese lady was referred for magnetic resonance (MR)
examination of her fetus at the gestational age of 34 weeks and 4 days.
She presented late for antenatal care and received her rst antenatal
sonogram in her third trimester; this revealed a cystic mass arising from
the posterior cranial fossa. Antenatal MR examination was performed
for better characterization of the structural abnormalities. It was
performed with a 1.5 Telsa General Electric MR system (Milwaukee,
U.S.A.) using SSFSE (single shot fast spin echo) T2-weighted (TE93.5/
TR2099) and SPGR (spoiled gradient reversal) T1-weighted (TE1.4/
TR100) sequences. The SSFSE sequence was employed for rapid signal
acquisition. It utilized partial Fourier technique so that 50% of the lines
of K-space were acquired in one TR whereas the rest were interpolated.
It generated T2-weighted images promptly, with image acquisition time
reduced to a few hundred milliseconds, rendering sedation unnecessary
during the procedure. Being a single slice acquisition technique, SSFSE
thus limits maternal and fetal motion artifacts to the particular slice
during which motion takes place (Fig. 1). Levine et al. showed that
anatomical details were well visualized with the SSFSE technique in
fetuses of more than 20 weeks' of gestation [1].
The antenatal MR examination conrmed a 7  4  4 cm cystic
mass lesion at the low occipital and high posterior cervical region
(Fig. 2). It was of CSF signal intensity and there were multiple internal
septa. The cerebellum was low in position and appeared hypoplastic.
Associated upper cervical spinal bida and midline low occipital bony
defects were seen. Herniation of a small portion of the hypoplastic
cerebellum into the occipital cephalocele was noted. The body of the
corpus callosum was absent and there was colpocephaly. A long
syringohydromyelia was seen extending from the cervical to the midthoracic level. The prenatal diagnosis was Chiari III malformation.
A lower segment caesarean section was performed at 38 weeks
gestation, resulting in the birth of a baby boy weighing 2885 g. The
Apgar score was 9 at 1 minute and 10 at 5 minutes. The child's head
circumference at birth was 33.5 cm, falling between the 10th and 25th
percentiles [2].
Plain computed tomography (CT) and MRI examination of the
neonatal brain and cervical spine were performed on day one
(Figs 3, 4). They conrmed the prenatal diagnosis of Chiari III
Author for correspondence and guarantor of study: Dr. R. Lee,
Department of Radiology, Queen Mary Hospital, Room 11, Block H,
Pokfulam Road, Hong Kong SAR, China. Fax: 85228555479; E-mail:
raymandl@netvigator.com
0009-9260/02/$35.00/0

malformation: a cervico-medullary kink was present; the shape of the

fourth ventricle was distorted; there was partial agenesis of the corpus
callosum with preservation of the genu; interdigitation of the cerebral
gyri suggested hypoplasia or fenestration of the falx cerebri; a lacunar
skull was seen on the CT scout images; and there was mild scalloping
of the petrous bone as well as the clivus. Excision of the occipital
encephalocele with duroplasties was performed for the neonate. Glial
and meningeal tissues were noted in the resected specimen, in line with
a meningo-encephalocele.

DISCUSSION

The pathological ndings of the malformation were rst

described by Chiari in 1891 [3]. Chiari III malformation is
rare [4]. The largest series included nine patients reported
by Castillo et al. in 1992 [5]. Chiari III malformation is now
dened as a low occipital (below the inion) or high cervical
encephalocele in association with imaging features of Chiari
II malformation [5].
Pathogenesis of Chiari III malformation is believed to be
closely related to that of Chiari II malformation. According
to the theory of McLone and Knepper, Chiari II malformation is a consequence of the growth of a normal-sized
cerebellum within an abnormal, small posterior cranial
fossa, the latter being caused by arrested calvarial development. Continuous leakage of cerebrospinal uid (CSF)
through a neural tube defect is believed to be the primary
event [6]. Failure of endochondral bone induction results in
the low occipital and high cervical bony defect. It could be
the consequence of failure of complete neural tube closure
or incomplete fusion of the ossication centres. The
herniation of cerebellar tissue through the posterior
cephalocele could account for the distortion of the shape
of the fourth ventricle as well as the cervico-medullary kink
seen in our case. Surgically induced myeloschisis in chick
embryos has resulted in morphological changes similar to
those in Chiari malformation [7]. The deranged cerebrospinal uid dynamic is likely to be the underlying problem
[5,7] rather than a disturbance of mesenchymal development in early embryological life [8].
The encephalocele in our case was largely of CSF with
only small amount of cerebellar tissue. Large CSF spaces
were also identied in three encephaloceles in the Castillo
# 2002 The Royal College of Radiologists

760

CLINICAL RADIOLOGY

Fig. 1 An axial SSFSE T2-weighted (TE93.5/TR2099) MR image of

the fetus showing a small amount of glial tissue (arrow) herniating into
the cephalocele.

Fig. 3 Corresponding post-natal axial T2-weighted (TR5000/TE

101.4) MR image of the brain for comparison, conrming the
herniated glial tissues (arrow).

series [5] and this was also noted in the case reported by
Aribai et al. [9]. They did not directly communicate with the
ventricular system and were believed to be distorted and
enlarged basilar cisterns. The distortion of the CSF ow at
the foramen magnum was the likely cause of the thoracic
syringohydromyelia. Apart from the colpocephaly, the rest
of the ventricular system was not dilated in our case.
Colpocephaly was believed to be due to dysplasia of the
occipital lobe with the ventricular dilatation being a
secondary phenomenon. The predominantly CSF-containing cephalocele might act as a reservoir for the excessive
CSF. Hydrocephalus was also absent from the post-mortem
nding of the case presented by De Reuck and Theinpont
[3]. Hydrocephalus was likely a secondary event in Chiari
III malformation. Multiple septa were seen within the
predominantly cystic menigio-encephalocele, the aetiology
remaining to be elucidated. Septa were also noticed in the
syringohydromyelia cavity in the Chiari III malformation
seen by Kuharik et al. [10].

The treatment of encephalocele in Chiari III is mainly

surgical resection and re-establishment of the CSF ow
dynamic. The herniated brain tissue in the encephalocele is
believed to be non-functional. However, identifying the
position of the brain stem is crucial. Herniation of the
brainstem into the cervical encephalocele would contraindicate immediate operative closure. Snyder et el. reported
the method of CSF diversion with delayed surgical closure
at 30 months of age [11]. It is also esszential to identify the
position of the venous sinuses and preserve the posterior
fossa venous sinuses and deep cerebellar veins [12]. Ectopic
positioning of cerebral venous sinuses is not uncommon in
Chiari III malformation and was seen in 44% of the series
reported by Castillo [5]. MR studies serve to delineate these
anatomical derangements (Fig. 5). Overall, the prognosis of
Chiari III malformation is very poor [5]. Our patient has
had a VP shunt inserted for residual hydrocephalus after
the resection of the encephalocele. Follow up at 10-months
of age showed delayed gross motor function as well as

Fig. 2 A sagittal SSFSE T2-weighted (TE93.5/TR2099) MR view of

the fetus, showing the large encephalocele. Internal septations are seen
within the cephalocele (arrow).

Fig. 4 A reconstructed 3D CT image of the upper cervical spine and

lower occiput using surface-shaded display (SSD); it shows the bony
defect at the posterior element of the upper cervical spine.

CASE REPORTS

761

Fig. 5 Post-natal 2D TOF (time of ight) MR venogram shows the abnormal dural venous sinuses. The superior sagittal sinus appears divided. This
piece of information is important to the neurosurgeon, before the insertion of VP shunt.

delayed ne motor function. Social and language development were also retarded.
CONCLUSION

This case illustrates the usefulness of MR imaging in the

antenatal diagnosis of Chiari III malformation. Furthermore, we have demonstrated the usefulness of the SSFSE
sequence in antenatal MR examination of the fetus.
REFERENCES
1 Levine D., Barnes PD, Edelman R. State of the art: obstetric MR
imaging. Radiology 1999;211:609617.
2 Chiu MC. Paediatric Manual, 5th edn. Hong Kong, China: Hong
Kong University Press, 1991, 9.
3 De Reuck J, Theinpont L. Fetal Chiari's type III malformation.
Child's Brain 1976;2:8591.

4 Barkovich AJ. Paediatric Neuroimaging, 3rd edn. Philadelphia,

U.S.A.: Lippincott Williams and Wilkins, 2000, 327337.
5 Castillo M, Quencer RM, Dominquez R. Chiari III malformation:
imaging features. AJNR 1992;13:107113.
6 McLone DG, Knepper PA. The cause of Chiari II malformation: a
unied theory. Paediatric Neuroscience 1989;15:12.
7 Sim KB, Hong SK, Cho BK, Choi DY, Wang KC. Experimentally
induced Chiari-like malformation with myeloschisis in chick
embryos. J Korean Med Sci 1996;11:509516.
8 Rollins N, Joglar J, Perlman J. Coexistent holoprosencephaly and
Chiari II malformation. Am J Neuroradiol 1999;20:16781681.
9 Aribai ME, Gurcan F, Asian B. Chiari III malformation: MRI.
Neuroradiology 1996;38:184186.
10 Kuharik MA, Edwards MK, Grossman CB. Magnetic resonance
evaluation of paediatric spinal dysraphism. Paediatric Neuroscience
198586;12:213218.
11 Snyder WEIr, Luerssen TG, Boaz IC, Kalsbeck IE. Chiari III
malformation treated with CSF diversion and delayed surgical
closure. Paediatr Neurosurg 1998;29:117120.
12 Kannegieter LS, Dietrich RB, Pais MJ, Goldenberg TM. Paediatric
case of the day. Radiographics 1994;14:452454.

doi:10.1053/crad.2001.0905, available online at http://www.idealibrary.com on

Intravertebral Vacuum Phenomenon that Developed During

Transpedicular Biopsy
M . S I RVA NC I , C . DUR A N , O . AY DI NE R
Department of Radiology, Florence Nightingale Hospital, Kadir Has University School of Medicine, Istanbul, Turkey

The vacuum phenomenon (VP) is frequently seen in

degenerate intervertebral discs, but rarely within vertebral
Author for correspondence and guarantor: M. Sirvanci, Ayazma
mah. Velioglu sok. No: 9/7, Husnurat apt., Uskudar 81160, Istanbul,
Turkey. Fax: 212 296 52 23; E-mail: sirvanci@prizma.net.tr

bodies. About 120 cases of intravertebral vacuum phenomenon (IVP) have been reported in the literature, mostly as
case reports [15]. Historically, it has been suggested that
IVP is virtually pathognomonic of vertebral avascular
necrosis. However, recent studies have refuted this theory
and shown that it may be associated with osteoporotic