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Original Paper

Received: Novem ber 14, 2001


Pediatr Neurosurg 2002;36:225–228
Accepted: February 2, 2002

D orsal Bony Sept um :


A Split Cord M alf orm at ion V ariant
Kam il M elih Akay Yusuf Izci Alper Baysefer
Departm ent of Neurosurgery, Gulhane M ilitary M edical Academ y, Ankara, Turkey

Key Words fied theory that resolves the problem of the pathological
Dorsal bony septum W Split cord m alform ation W definition and clinical significance of such malforma-
Em bryological studies tions. Pang et al. [1] identified two types of SCMs accord-
ing to two easily identifiable features, i.e. dural invest-
ment of the hemicords and the nature of the midline mes-
Abstract enchymal derivates. Although this classification covers
Split cord m alform ations (SCM s) are rare spinal anom a- almost all types of SCMs, there are a few cases that cannot
lies and their classification is still a m atter of debate. be classified according to this system [3, 4].
There is no w idespread consensus on the em bryological This report consists of a review of two unusual SCM
basis of this entity. The unified theory, proposed by Pang patients. We have reviewed the medical records of these
et al. [Neurosurgery 1992;31:451–480], w as an attem pt to cases and emphasized the different features of the lesions
explain the em bryogenetic m echanism as a basic error which distinguish them from other cases.
occurring around the tim e w hen the prim itive neuroen-
teric canal closes. We report tw o unusual cases of SCM s
w ith a dorsally situated bony spur. We analyzed the Case Report s
radiological, clinical and surgical features of the lesions
and w ere not able to classify these cases according to the Case 1
A 7-year-old girl presented to our outpatient clinic with a tuft of
unified theory. Further em bryological studies should be hair on her lumbar region. She was the second child in her family and
conducted to elucidate the m echanism s of occurrence of the product of a normal full-term delivery. The physical and mental
these lesions, and the dorsal bony septum variant should development of the patient were normal. There were no neurological
be considered in SCM surgery. deficits. A plain X-ray film showed a bony spur at L4 level. Com-
Copyright © 2002 S. Karger AG, Basel puted tomography (CT) studies of the lumbar spine demonstrated a
dorsally located bony spur with a hypertrophied posterior arch
(fig. 1a). Magnetic resonance (MR) studies revealed two separate
hemicords in a single dural tube with a bony septum that was situated
Int roduct ion dorsally and indented the dural tube (fig. 1b, c). The conus extended
to the 4th lumbar vertebra. The preoperative electrophysiological
and urodynamic tests were in normal ranges.
Among cases of spinal dysraphism, split cord malfor-
The patient was operated under general anesthesia. She was
mations (SCMs) have a specific place in neurosurgery due placed in the prone position and a vertical midline incision was made
to the confusion regarding their embryogenesis and classi- between the L2 and S1 spinous processes. The spinous processes
fication. Pang et al. [1] and Pang [2] have proposed a uni- were exposed and laminectomies were performed from L3 to L5. The

© 2002 S. Karger AG, Basel Kamil Melih Akay, MD


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Fig. 1 . a Axial spinal CT scan image of case 1. Note the dural inden-
tation of the involved lamina. b T1-weighted axial MR imaging scan
of case 1. The dorsal bony septum arising from the posterior arch
does not have any anterior attachment. Although the spinal cord at
the involved level was seen as a single structure, surgical confirma-
tion of the lesion showed that it is split. c T2-(left) and T1-weighted
(right) sagittal MR imaging scans of case 1 showed the indentation of
the spinal cord by the dorsal bony septum at L4 level and the tethered
spinal cord. Also, note the widening of the lumbosacral spinal canal.

laminectomy procedure was difficult due to the posterior adherent walk at the end of her first year and her psychomotor development
bony spur and performed meticulously to avoid dural tears. The was normal. On her physical examination, only a rigid mass lesion
bony septum was removed and the dura was opened. The inner space due to sacral bone deformity was detected. Plain X-ray studies
of the dural tube was examined carefully to detect any fibrous or showed a bifid lamina and bony septum at S1 level. CT scan of this
cartilaginous septum lying between the bony septum and vertebral patient showed a dorsal bony septum indenting the dural tube at S1
body. There was no other spinal dysraphic lesion, but there were a level associated with sacral bone deformity (fig. 2a, b). MR studies of
couple of rootlets coming out from the medial aspect of the hemi- the patient revealed two hemicords in one dural tube, a bony septum
cords. Following untethering of the two hemicords and release of the situated dorsally at S1 level and the conus medullaris lying on the
filum terminale, which was assessed as normal, the dorsal dura was coccyx (fig. 2c, d). Normal results were obtained on electrophysiolog-
closed and the operation was terminated. The patient was discharged ical and urodynamic evaluation of the patient.
without neurological deficits. In the 18th follow-up month, the The patient underwent surgery. After a midline incision between
patient was doing well. L3 and S2 was made, laminectomies were performed at the level of
L4, L5 and S1. The bony septum and bone structures which caused
Case 2 the sacral deformity were removed and the dorsal dura was opened.
A 2-year-old girl presented to our outpatient clinic with the com- The structure of the hemicords was consistent with SCM. Medial
plaint of a rigid mass lesion on her sacral region since birth. She was rootlets were coming out from the dorsal medial aspect of the hemi-
the first child in her family and the product of a normal delivery. Her cords. The inner space of the dural tube was examined carefully and a
neurological examination was in the normal range. She had begun to fibrous ligamentous structure that was lying on the dorsal dura was

226 Pediatr Neurosurg 2002;36:225–228 Akay/Izci/Baysefer


Fig. 2 . a Axial CT scan image of case 2
showing the abnormal structure of the in-
volved spinal column. b Three-dimensional
spinal CT reconstruction of the spinal col-
umn of case 2 is shown. c T2-weighted axial
MR imaging scan of case 2. Note the unequal
splitting of the spinal canal by the dorsal
bony septum. d T2-weighted sagittal MR
imaging scan of case 2 shows the dorsally sit-
uated bony septum at S1 level, the widened
spinal canal and low conus medullaris with
the tethered spinal cord.

excised. The conus medullaris was meticulously released from the and maintains that the endomesenchymal tract causes all
dural attachments and the thick filum terminale. The neurological double cord malformations.
status of the patient was not changed after this procedure. She was
SCMs are classified as one of two types according to
discharged without complications. In the first follow-up period of 3
months, she could still walk without deficit. the unified theory; in type I, the hemicords are always
invested with individual dural sacs and the medial walls
of the sacs always ensheathe a rigid (bony or cartilaginous)
D iscussion midline spur, while in type II, the hemicords are always
within a single dural sac and the midline septum is always
Since Ollivier [5] first used the term ‘diastematomye- composed of nonrigid fibrous or fibrovascular tissues [1,
lia’ in 1837 to describe an abnormality of the spinal cord 2, 6]. Erşahin et al. [7] supported this theory with their
in which the dura is separated by a bony spike or a rigid study and also emphasized the presence of unusual forms
fibrous band, there have been many theories proposed by of this malformation [8].
authors to explain the embryologic basis of SCMs. Pang et Emura et al. [9] reported an experimental animal mod-
al. [1] and Pang [2] have called attention to the confusion el of SCMs produced by the surgical induction of a fistula.
regarding the classification of double cord malformations Even though the results of their study supported the
and proposed a unified theory to explain the embryoge- hypothesis that SCMs originate from an accessory neu-
netic mechanisms of all variants of SCMs. This theory is roenteric canal, it is not sufficient to explain the mecha-
founded on the presence of anomalous neuroenteric canal nisms of occurrence of all variations of SCMs.

Dorsal Bony Septum Pediatr Neurosurg 2002;36:225–228 227


Chandra and Mahapatra [3] reported a different case hin [4] and Chandra and Mahapatra [3]. These lesions
of dorsally situated bony spur in 1999 which could not be could not be classified as type I or type II SCMs according
explained by the unified theory, and they proposed a to the unified theory. Thus, we need a new hypothesis to
slight modification of the unified theory. They hypothe- explain these entities. Although Chandra and Mahapatra
sized two mechanisms responsible for the occurrence of [3] and Ersahin [4] tried to explain this situation with dif-
that rare lesion: (1) passage of an abnormal cell popula- ferent hypotheses, in our opinion, further embryological
tion dorsally and subsequent loss of contact with the ven- studies must be conducted to evaluate such hypotheses.
trally situated cell population; (2) migration of cells first From the surgical standpoint, a full en bloc laminecto-
around the hemicords and then passing between them in a my is not appropriate when operating on an occult spinal
dorsoventral direction. However, these hypotheses do not dysraphism defect, because of dorsal tethering lesions
explain whether this is an isolated event or an event tak- such as dorsal bands or sinus tracts. The potential pres-
ing place in all SCMs. ence of a dorsal bony septum may be accepted as another
Erşahin [4] reported another case of dorsally situated reason for carefully removing the laminae when operating
bony spur in 2000. He considered this malformation a on a case of occult spinal dysraphism.
type II SCM and the dorsal bony spur as one of the verte-
bral anomalies involving the lamina. Although the nature
of the lesion in our second case supports this notion Conclusion
because of the presence of sacral bone deformity in asso-
ciation with the dorsal bony spur, it is difficult to main- The classification of SCMs will continue to be a matter
tain that the lesion in our first case was a vertebral anoma- of debate in the future. Despite the unified theory of
ly involving the lamina. Furthermore, Katoh et al. [10] embryogenesis and classification for the accurate manage-
reported a case of type II SCM without a fibrous septum, ment of such malformations proposed by Pang et al. [1]
which is not explained by the unified theory. and Pang [2], much confusion still exists with respect to
In our two cases, we detected a bony spur situated dor- the classification of uncommon variations. Thus, more
sally with two hemicords in a single dural sac. In other experimental embryological studies are needed to eluci-
words, the lesions in our patients were consistent with date the unified theory. Finally, the dorsal bony septum
SCM. Our cases are similar to the cases reported by Erşa- variant should be considered in SCM surgery.

References

1 Pang D, Dias MS, Ahab-Barmada M: Split 4 Erşahin Y: An unusual split cord malforma- 8 Erşahin Y, Demirtaş E, Mutluer S, Tosun AR,
cord malformation. 1. A unified theory of em- tion. Pediatr Neurosurg 2000;32:109. Saydam S: Split cord malformations: Report of
bryogenesis for double spinal cord malforma- 5 Ollivier CP: Traité des maladies de la moelle three unusual cases. Pediatr Neurosurg 1996;
tions. Neurosurgery 1992;31:451–480. épinière. Paris, Mequignon-Marvis, 1837. 24:155–159.
2 Pang D: Split cord malformation. 2. Clinical 6 Dias MS, Pang D: Split cord malformations. 9 Emura T, Asashima M, Hashizume K: An ex-
syndrome. Neurosurgery 1992;31:481–500. Neurosurg Clin N Am 1995;6:339–358. perimental animal model of split cord malfor-
3 Chandra PS, Mahapatra RK: An unusual case 7 Erşahin Y, Mutluer S, Kocaman S, Demirtaş E: mation. Pediatr Neurosurg 2000;33:283–292.
of dorsally situated bony spur in a lumbar split Split cord malformations in children. J Neuro- 10 Katoh M, Hida K, Iwasaki Y, Koyanagi I, Abe
cord malformation. Pediatr Neurosurg 1999; surg 1998;88:57–65. H: A split cord malformation. Childs Nerv Syst
31:4–52. 1998;14:398–400.

228 Pediatr Neurosurg 2002;36:225–228 Akay/Izci/Baysefer


Copyright: S. Karger AG, Basel 2002. Reproduced with the permission of S. Karger AG, Basel. Further
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