1968, Br. J. Radial.
, 41, 844-847
The split notochord syndrome
By F. G. O. Burrows, F.R.C.S., F.F.R.,* and J. Sutcliffe, M.R.C.P., F.F.R.
Hospital for Sick Children, Great Ormond Street, London, W.C.1.
{Received May, 1968)
Developmental lesions of foregut origin associated
with cervical or thoracic vertebral anomalies have
been described by a number of writers including
Veeneklaas (1952), McLetchie, Purves and Saun-
ders (1954), Fallon, Gordon and Lendrum (1954),
Beardmore and Wiglesworth (1958), Neuhauser,
Harris and Berrett (1958), and Nathan (1959).
In many of the cases reported, vertebral abnormali-
ties have been of relatively minor degree, some being
demonstrable only by tomography. A cleft verte-
bral column with wide separation of the two halves
and associated enteric malformations is a much more
unusual condition and reports of such a major
developmental error are infrequent. Saunders (1943)
reporting a personal case, found 36 others in the
literature and observed that only two of these
(Adelmann, 1920; Bell, 1923) were described in the
English language. Saunders' paper is of great in-
terest in that it provides an excellent description of
the morbid anatomy of this condition.
His case was a female infant who presented with
a pad of mucous membrane in the lumbar region;
in the lower part of the mass there was a fistula
through which meconium passed. A catheter in-
troduced through the fistula emerged at the anus.
Active treatment was apparently not possible and
the child died at the age of five months. The autopsy
examination was performed meticulously. The spine
was normal down to T 7. T 8-T 12 vertebral bodies
were irregularly developed. Below this level, the
spine was split and the halves were widely separated,
rejoining at S 2. On each side of the cleft there was a
laminar ridge formed by fused half-arches. The
spinal cord also was normal down to T 7; below this
the cord and its central canal increased in diameter,
then bifurcated at the upper end of the vertebral
cleft into unequal divisions. In both, the central
canal was dilated, reducing each "cord" to a thin
shell of nervous tissue. The right division gave off a
single laterally directed set of nerves; the left gave
off both a lateral and a medial set. A loop of colon
lay between the halves of the split lumbar spine,
communicating with the posterior fistula. There was
also an anomaly of rotation, the caecum lying in the
left iliac fossa.
* Present address: The Children's Hospital, Birmingham.
844
In most of the other cases reviewed by Saunders,
clefts involved the cervical and thoracic spine. In
some there was a division of the entire spinal col-
umn; a few showed splitting of the lumbar region
alone. The degree of alimentary tract involvement
in each case was noted and as might be expected,
derivatives of foregut and mid-gut were usually
involved. The related part of the alimentary tract
did not in every case open or present itself dorsally.
In some it simply lay within the cleft; in others, a
"neurenteric" connection (a diverticulum or strand
or both) passed from it to the cleft. Saunders regard-
ed the term "vertebral cleft" as a convenient des-
cription for this abnormality. He considered that the
expression "anterior spina bifida" should be em-
ployed only when the vertebral body alone is affec-
ted. When there is a concomitant posterior defect,
the case could be spoken of as "anterior and pos-
terior spina bifida", or "combined spina bifida".
Rosselet (1955) described a male pseudo-herma-
phrodite who died shortly after birth. In this case
also there was combined anterior and posterior spina
bifida below the level of the 12th thoracic vertebra,
the halves of the split spine rejoining in the lower
sacral region. There were no coccygeal vertebrae.
Among many other congenital anomalies, the child
had a myelo-meningocoele in the thoraco-lumbar
region and rectal mucosa protruded through the
lower part of the spinal defect.
A remarkable series of five cases was described
by Bentley and Smith (1960), four of which were
examples of combined anterior and posterior spina
bifida: three affecting the lumbo-sacral spine and
one the thoracic. In one patient the spine appeared
to be normal. Three cases had posterior enteric
sinuses, that is to say, blind enteric tubes opening
on the dorsum. In a fourth case, a posterior sinus
may have communicated with the intestinal tract,
since there were recurrent E. colt infections after
excision. The fifth patient had a complete posterior
enteric fistula; a catheter passed through the dorsal
opening entered the rectum.
Smith (1960) suggested a useful classification of
the associated enteric anomalies. He described them
collectively as dorsal fistula remnants. Of this group,
a congenital dorsal enteric fistula represents per-
sistence of an entire tract between the gut and the

The split notochord syndrome
dorsum. A dorsal enteric sinus is the remains of the
posterior part of the tract and forms a blind exter-
nal opening. Dorsal enteric cysts (derived from the
intermediate portion) may be prevertebral, post-
vertebral or intraspinal. A dorsal enteric diverti-
culum is due to persistence of the anterior part of
the tract as a blind communication with the bowel.
Case report
R.S., born 14.1.57, the daughter of Italian parents living
in this country, was first seen at the Hospital for Sick Child-
ren, Great Ormond Street, in October, 1958.
History A large meningocoele had been present in the
lumbar region since birth; this was covered with skin with
the exception of a raw area around a small opening in the
mid-line. There was normal function of the right leg but
paralysis of the left. She had good control of micturition
and defaecation and anal sphincter tone was normal. She
was not seen at this hospital again until May, 1962 when she
was referred because she had begun to pass faeces through
the dorsal opening. In the intervening period she had re-
ceived treatment at Addenbrookes Hospital, Cambridge
for her paralysed left leg and was able to walk quite well
with two tripods and a caliper.
Clinical examination at this time (May, 1962) showed an
alert and intelligent child with a large lumbar meningocoele
(Figs. 1 and 2). In the mid-line, slightly below the centre of
FIG. 1. FIG. 2.
Clinical photographs of the patient at the age of six years,
showing the large lumber meningocoele and wasting of the
paralysed left leg. The opening of one fistula is just visible,
near the centre of the meningocoele
845
NOVEMBER 1968
the swelling, there were two small fistulous openings which
discharged faeces intermittently. There was severe paralysis
of the left leg, producing a flail limb with sensory deficit,
lowered skin temperature and peripheral cyanosis. No other
significant abnormality was found.
Radiological investigation (Dr. B. C. H. Ward). Plain films of
the spine showed fusion of the 12th thoracic and 1st lumbar
vertebrae. L 2 was wide and partially separated into two
hemi-vertebrae (Fig. 3). From L 3 downwards the vertebrae
were cleft, the halves remaining separate as far as the lowest
sacral segment where they appeared to rejoin. There were
no coccygeal vertebrae. In addition there was a dysplasia of
the left hip joint which had a wide shallow acetabulum.
Further investigation. Water soluble contrast medium given
orally demonstrated the colon clearly but a fistulous com-
munication could not be seen.
Barium sulphate suspension injected into the external
opening of the fistula outlined a tubular passage which
passed between the two halves of the vertebral column (Fig.
4). This passage communicated with a structure in the
abdomen, which resembled colon; however, no communica-
tion with the colon which had been outlined with less dense
water-soluble medium given by mouth, could be demonstra-
ted. 28.2.63. Laparotomy was performed by Mr. G. H. Mc-
Nab. The abdomen was opened by a left paramedian inci-
sion. There was a duplication of the pelvic colon, attached to
the latter by a connection 20 cm above the peritoneal reflec-
tion. The proximal blind end of the duplication contained a
cystic swelling which was adherent to colon. The distal end
of the duplication passed downwards and backwards and
disappeared into the fossa between the two halves of the
lumbo-sacral spine. The duplicated segment was dissected
out and excised, being divided above at the site of its
connection with the pelvic colon and below, flush with the
lumbo-sacral defect. The remaining segment of duplicated
bowel extending into the meningocoele was oversewn and
pushed retroperitoneally.
Her post-operative recovery was uneventful. The faecal
discharge from the fistula ceased almost immediately.
Report on the excised specimen (Dr. Barbara Ockenden)
"Naked eye examination. A segment of collapsed and re-
constructed large intestine 11 cm long by up to 2-5 cm in
diameter (Fig. 5). 2 cm from one end there is a junction with
another stump of large intestine 1 cm long. At the blind up-
per end the intestine is adherent to a firm round lump of
tissue (2-5xl-5xl-5 cm) which on section is seen to contain
a cyst with smooth white lining and clear but slightly viscid
content. There is no connection between the cyst and the
intestinal lumen which ends blindly at this point.
"Histology. Sections from the duplicated segment of colon
show that it is mainly lined by flattened, rather atrophic,
large intestinal epithelium; there is a muscularis mucosae
but the main muscle layers are thin or absent. At its apex
there is a non-communicating cyst lined by very flattened
gastric-type epithelium, in places reduced to a single layer
of columnar cells without crypts or villi. Some of the epi-
thelium of the bowel adjacent to the cyst is also of the gas-
tric type. There is a focal chronic infiltration in the serosa
which is somewhat oedematous. Intramural ganglia are
present in adequate numbers in those parts where normal
muscle layers are present.
"The appearances are those of colonic duplication and
enteric cyst, both lined by ectopic gastric mucosa. In ad-
dition there is an enteric canal lined by colonic mucosa
which extends back to the sacrum, from which it was
divided."
Further progress. The child has remained well. There is
occasional clear mucoid discharge from the sinus opening.
Because of a flexion contracture of the left knee a supra-
condylar osteotomy was performed in 1964 followed by a
Steindler operation and tenotomy of the Achilles tendon.
When seen again in 1967 she was making good progress and
was walking well with a caliper. Her meningocoele has not
been excised.
 VOL. 41, No. 491
F. G. O. Burrows and J. Sutcliffe

FIG. 3. FIG. 4.
FIG. 3. Antero-posterior film showing the cleft lumbo-sacral spine with separation of the two halves.
The lowest sacral segment where the halves rejoined is not shown in this view.
FIG. 4. Lateral view showing water-soluble contrast medium in the colon and barium sulphate sus-
pension in the posterior enteric fistula and duplicated segment of colon. The communication between
the latter and the colon proper was small and not demonstrated by radiological means.

FIG. 5.
Appearance of the excised specimen. On the left is the cyst
lined with gastric epithelium and adjacent to it the com-
munication with the colon. The segment which entered the
meningococle is on the right.
846
DISCUSSION
It is not proposed to discuss the embryology of
this condition at any length, but mention must be
made of three possible mechanisms which have
been suggested by various writers to explain the
origin of this type of abnormality.
The first is the accessory neurenteric canal theory
of Bremer (1952). The neurenteric canal is present
in the early embryo as a temporary communication
between the yolk sac and amniotic cavity (Arey,
1965). The final location of the remains of this
structure is at the tip of the coccyx. Bremer sug-
gested that any connection between the gastro-
intestinal tract and mid-line dorsal structures above
the coccyx was due to the previous existence of an
accessory neurenteric canal. He stated "experimen-
tally anomalous embryos have been produced,

The split notochord syndrome
showing a normal head and tail end, but with the
back between separated into halves by a long median
cleft, through which the underlying yolk bulged.
Essentially this cleft is a magnified neurenteric canal,
but since it does not pass through the primitive
knot it is an accessory neurenteric canal; the dorsal
intestinal fistula is of this type."
The second hypothesis, advocated by Veeneklaas
(1952) and later by Fallon et al. (1954) is that a
diverticulum of entoderm may be withdrawn from
the primitive foregut by the notochord and that
this may form a cyst or similar lesion and prevent
normal development of vertebral bodies. To quote
Fallon " . . . if, however, the withdrawal of the noto-
chord elements drew with it some of the adjacent
endodermal lining, there is not only the possibility
envisaged above of interference with the formation
of the spinal column, but also of various abnormali-
ties that could arise directly from this traction of the
endodermal cavity backwards into the mesoderm.
From such a pouch then could develop the redupli-
cations and communicating cysts of the alimentary
tract."
Thirdly, there is the "split notochord" theory
which was developed by Feller and Sternberg (1929)
and was supported by Saunders (1943) among others.
Feller and Sternberg suggested that a primitive
node cell rest persisted in the mid-line, producing a
notochordal cleft which caused the vertebral centra
to be laid down in independent halves. In favour of
this hypothesis it is noted that duplication of the
notochord was described by Johnston (1931) in an
11 mm embryo. In discussing Johnston's case,
Frazer (1931) also mentioned that he himself had
observed a split or double notochord in a human
embryo. In Frazer's words, "the notochord was di-
vided far back into two. The two chords passed for-
ward side by side and there was broadening of the
neural tube opposite the division of the notochord."
Saunders pointed out that in his own case there was
a nucleus pulposus between the hemivertebrae at
all levels on both sides of the cleft, which could be
accepted as evidence of notochordal duplication.
Comparison of the relative merits of these theories
is beyond the scope of this paper. It must be ob-
served, however, that the split notochord theory
847
NOVEMBER 1968
offers a more reasonable explanation for those cases
in which combined spina bifida involves a large
number of vertebral segments and particularly
those with splitting of the entire vertebral column
and a wide communication between the dorsum and
the gastro-intestinal tract. It appears therefore that
there is some justification for referring to this type
of anomaly as the "split notochord syndrome", a
term which may be preferred to "combined an-
terior and posterior spina bifida with dorsal intes-
tinal fistula".
ACKNOWLEDGMENT
This child was under the care of the late Mr. George
McNab.
ABSTRACT
A case is presented illustrating combined anterior and
posterior spina bifida in the lumbo-sacral region, accom-
panied by a dorsal intestinal fistula which opened externally
on the surface of a meningocoele. When the child was five
years of age, faeces began to discharge from the cutaneous
opening. A year later, operative treatment was undertaken
and the intra-abdominal portion of the fistulous tract was
excised.
Three of the theories concerning the embryology of this
condition have been mentioned. It is thought that sagittal
splitting of the notochord at an early stage of development
offers the most rational explanation and we feel that in des-
cribing the condition it is justifiable to use the expression
"split notochord syndrome", a term accepted by other
writers.
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