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Title: Diasmatomyelia (split cord malformations)

Our Experience

Article Type: Clinical Research Paper

Keywords: Key words .Diasmetomeylia .Split cord malformations .Spinal dysraphism
Corresponding Author: Dr. Umar Farooq, MBBS,MS(Neurosurgery)
Corresponding Author's Institution: Pakistan Institute of Medical Sciences
First Author: Umar Farooq, MBBS,MS(Neurosurgery)
Order of Authors: Umar Farooq, MBBS,MS(Neurosurgery); Sami u Rehman, MBBS, MS (Neurosurgery);
Nawaz Khan, MBBS, MS (Neurosurgery); Khaleeq u Zaman, MBBS,FRCS(SN),FCPS NEUROSUGERY
Abstract: Abstract
Object. Diasmetomeylia (split cord malformations) are rare anomalies of the spine. Total 18 cases were
studied prospectively at author's center during period of one and half year.
Methods. Patient's demographic profile, symptoms and signs, imaging studies, operative findings,
complications, and outcomes were assessed prospectively.
The mean age of the patient's was 6.33 years (male/female 3/1). Type I was seen in 7 cases
(27.77%) and type II was seen in 11 cases (60.11%). Dermal manifestation were seen in all 18 cases
(100 %); hypertrichosis 14 cases (77.77%), dimple 5 cases (27.77%), dermal sinus in 4 cases (22.22%)
subcutaneous lipoma 5 cases (27.77%). Orthopedic deformity in 9 cases (50%). Neurological deficit
present in 15 cases (83.33%). Asymptomatic were 3 cases (16.66%).All patients were treated
surgically. Outcome 15 cases improved neurologically (87.5%), 1 case (12.5%) no improvement
occurs. Two cases were lost to follow up. Complication in 4 cases (22.22%), 2 cases (11.11%) surgical
site infections and urinary tract infection occurs in 2 cases (11.11%).
Conclusions. The authors present 18 cases in one and half year. The patients with dermal
manifestations should be referring for neurosurgical evaluation, prompt management before
developing any other manifestations. Awareness program for pediatric physicians and general public
should be done for better outcomes. Especially Parents should be counseled regarding folic acid
supplement in future. A multicenter large study carried out to answer the challenges.
Key words .Diasmetomeylia .Split cord malformations .Spinal dysraphism

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*Clinical Guidelines

TABLE 1. GUIDELINES FOR THE REPORTING OF CLINICAL RESEARCH DATA IN THE JOURNAL
OF PEDIATRIC SURGERY
Methods:
Reported

Not
Applicable

Reporting Detail
The number and practice type of all institutions where cases were performed
The number of surgeons who actually operated in the study (& relative number of cases for each)
The prior experience of participating surgeons in performing the reported intervention
The precise timeline during which all patients were treated in the study(i.e. Jan 1995 to March
1998)

1
2
25 years

January 2013 to
June 2014

A clear description of how patients were selected into the study. This should include relevant
inclusion and/or exclusion criteria.

Included were
having first
surgery all oother
were excluded
NA
18
Yes standard
criteria
yes
Yes for TYPE I
and TYPE II

The number of eligible patients at the study sites excluded during the timeline of the study
A clear description of the study population from which the patients were selected
A clear description of the relevant diagnostic criteria used to identify cases
A clear description of critical aspects of operative technique and perioperative care
Statement as to whether any attempts were made to standardize operative technique or perioperative
care (and how this was accomplished)

Results:
Reported

Not
Applicable

yes
yes
NA
YES
Lost to follow up

Reporting Detail
The range and mean of all relevant demographic and baseline variables
The range and median (not mean) for length of follow-up reporting
Relevant outcome variables are presented with appropriate measures of range and variability
(i.e. standard deviation)
Methods for measuring outcomes of interest are clearly described
Statement regarding whether any data is missing (and how missing data is addressed in the analysis
of outcome variables)
Number and appropriate details regarding all complications

YES

Additional Details for Studies Reporting More Than One Treatment Group (e.g. Controls):
Reported

Not
Applicable
NA
NA
NA
NA
NA
NA
NA

Reporting Detail
Mean and range for all relevant demographic and baseline variables for all treatment groups
The range and median (not mean) for length of follow-up reporting for each treatment group
A precise timeline during which all patients were treated for each group
Outcome variables being compared between groups are presented with appropriate measures of
variability (e.g. standard deviation)
Measures to type II error (P-values) for comparison statistics are presented with actual values if
P=.01 or larger (e.g. P=NS and P<.05 are not acceptable)
A description of how patients were selected into each treatment group
A statement is made as to whether the same surgeons operated on patients from different treatment
groups

Manuscripts concerning clinical research should follow a uniform set of reporting guidelines. The guidelines, listed above, were developed from
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*Title Page

Diasmatomyelia (split cord malformations)

Our Experience
Umar Farooq, Sami Ur Rehman, Muhammad Nawaz,
Prof. Khaleeq u Zaman.

*Abstract

Diasmatomyelia (split cord malformations)

Our Experience
Umar Farooq, Sami Ur Rehman, Muhammad Nawaz,
Prof. Khaleeq u Zaman.
Department of Neurosurgery, Pakistan Institute of medical sciences,
Islamabad, Pakistan.

Abstract

Object. Diasmetomeylia (split cord malformations) are rare anomalies of the

spine. Total 18 cases were studied prospectively at authors center during period of
one and half year.

Methods. Patients demographic profile, symptoms and signs, imaging studies,

operative findings, complications, and outcomes were assessed prospectively.
The mean age of the patients was 6.33 years (male/female 3/1). Type I
was seen in 7 cases (27.77%) and type II was seen in 11 cases (60.11%). Dermal
manifestation were seen in all 18 cases (100 %); hypertrichosis 14 cases (77.77%),
dimple 5 cases (27.77%), dermal sinus in 4 cases (22.22%) subcutaneous lipoma 5
cases (27.77%). Orthopedic deformity in 9 cases (50%). Neurological deficit
present in 15 cases (83.33%). Asymptomatic were 3 cases (16.66%).All patients
were treated surgically. Outcome 15 cases improved neurologically (87.5%), 1
case (12.5%) no improvement occurs. Two cases were lost to follow up.
Complication in 4 cases (22.22%), 2 cases (11.11%) surgical site infections and
urinary tract infection occurs in 2 cases (11.11%).

Conclusions. The authors present 18 cases in one and half year. The
patients with dermal manifestations should be referring for neurosurgical
evaluation, prompt management before developing any other manifestations.
Awareness program for pediatric physicians and general public should be done for
better outcomes. Especially Parents should be counseled regarding folic acid
supplement in future. A multicenter large study carried out to answer the
challenges.

Key words .Diasmetomeylia .Split cord malformations .Spinal dysraphism

. Neurosurgery

*Manuscript
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Diastematomyelia (split cord malformations): Our experience

Clinical article
Umar Farooq, Sami Ur Rehman, Muhammad Nawaz, Khaleeq UZ Zaman.
Department of Neurosurgery, Pakistan Institute of Medical Sciences, Shaheed Zulfiqar
Ali Bhutto Medical University, Islamabad, Pakistan.
Object. Diastematomyelia (split cord malformations) are rare anomalies of the spine.
Total 18 cases were studied prospectively at authors center during period of one and a
half year.
Methods.Patients demographic profile, symptoms and signs, imaging studies, operative
findings, complications, and outcomes were assessed prospectively. The mean age of the
patients was 6.33 years (male: female 3:1). Type I was seen in 7 cases (27.77%) and
type II was seen in 11 cases (60.11%). Dermal manifestations were seen in all 18 cases
(100 %); hypertrichosis 14 cases (77.77%), dimple 5 cases (27.77%), dermal sinus in 4
cases (22.22%) subcutaneous lipomas in 5 cases (27.77%). Orthopedic deformity in 9
cases (50%). Neurological deficit present in 15 cases (83.33%). Neurologically intact
were 3 cases (16.66%). All patients were treated surgically. Outcome 15 cases improved
neurologically (87.5%), 1 case (12.5%) no improvement occurred. Two cases were lost to
follow up. Complications in 4 cases (22.22%), 2 cases (11.11%) surgical site infections
and urinary tract infection in 2 cases (11.11%).
Conclusions. The authors present 18 cases in one and a half year. The patients with
dermal manifestations should be referred for neurosurgical evaluation, prompt
management before developing any other manifestations. Awareness program for
pediatric physicians and general public should be done for better outcomes. Especially
Parents should be counseled regarding folic acid supplementation in future.
Key words. Diastematomyelia. Split cord malformations .Spinal dysraphism
plit cord malformations are rare congenital
anomalies in which the cord is split over a
portion of its length to form double neural
tubes or two hemicords in a single dural
sheath. Many of the affected children are
asymptomatic at birth, but neurological
deterioration occurs mostly within the first 2 to 3
years of life due to tethering of the cord by
tissues that pass through it (bone spurs or fibrous
bands) or by a thick terminal filum. We analyzed
18 patients with SCM surgically treated at our
center. We studied the clinical and imaging
profiles of these patients, as well as their
surgical outcomes and complications.

Clinical Material and Methods

Patient Population

A total of 18 patients with SCM were

treated surgically between January 2013 and
June 2014. Each patients age, sex, clinical
features, imaging studies, operative details,
associated bone and soft-tissue anomalies,
complications, and surgical outcome were noted
or evaluated in detail prospectively from
January2013 to June2014. All patients with a
diagnosis of SCM were treated surgically, even
if they were asymptomatic and neurologically
intact. The follow-up duration of these patients 2
weeks, 3 months and six months.
The mean age of the patients with
neurological deficits was 6.33 years, whereas
asymptomatic patients presented at a mean age
of 0.7 years. There were 12 male patients
(66.66%) and 6 female patients (33.33%).

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Table 1. Clinic-al picture and number of the

patients affected
Skin stigmata

18 (100%)

Spina bifida

4 (22.22%)

Scoliosis

2(11.11%)

and the two dural tubes were converted to one

by suturing lateral margins of both dural tubes
at the midline. The terminal filum was cut in all
cases involving tethered or low-lying cords
through the same incision.
Type II SCM.

Orthopedic lower
limbs deformity

9 (50%)

Sensory, motor and

autonomic

15 (83.33%)

Trophic ulcers

3 (16.66%)

Pain: Back and lower

limbs

15 (83.33%)

For patients with Type II SCM, the

laminectomy or laminotomy was performed at
the lower end and upper end of the split. The
dura was opened and the fibrous septum or
arachnoid adhesions were excised (associated
lesions tethering the spinal cord were also
corrected at the same time).The terminal filum
was also cut to minimize the traction on the
conus medullaris.
Results
Clinical Features

Imaging Studies
Magnetic resonance imaging of the
spine (for type and level of split, level of the
conus medullaris, thickness of the terminal
filum, presence of other associated tethering
elements, and syringomyelia) was performed,
along with a screening MR imaging study of the
brain and craniovertebral junction to look for
hydrocephalus, Chiari malformation, and
syringomyelia if symptoms were present. Plain
x-ray films of the spine were obtained to assess
for kyphoscoliosis and vertebral or rib
anomalies.

The presenting symptoms can be classified as

skin stigmata,spina bifida aperta, scoliosis or
kyphoscoliosis, musculocutaneous deformities
of the lower limb, and sensorimotor and
autonomic deficits (Table 1).
Table 2. Neurological deficit and number of
patients affected
Motor

15 (83.33%)

Sensory

15 (83.33%)

Autonomic

10 (55.55%)

Sensory, motor and

autonomic

10 (55.55%)

Neurologically intact

3 (16.66%)

Surgical Procedures
Type I SCM.
A careful laminectomy or laminotomy
was performed around the attachment of rigid
septum. A laminotomy was used in patients with
well-formed laminae or in those who needed
exposure of the cord above or below the
dysraphic spine. The spur was then dissected
extradurally between the two dural sleeves and
removed piecemeal by using small rongeurs.
Fibrous adhesions were excised and dural
sleeves resected. The dura mater was opened

Motor deficits consisted of weakness and

atrophy of the limbs and gait disturbances were
present in 15 patients (83.33%), whereas sensory
complaints in 15 patients (83.33%) were mainly
in the form of dysesthetic pain, hypesthesia, and
trophic ulcers. Bladder and bowel disturbances
were noted in 10 patients (55.55%), the majority

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of whom presented with symptoms of urinary

tract infections. Their symptoms included
urinary frequency and urgency, a feeling of
incomplete voiding, poor voluntary control and
urge, and stress incontinence (frequent dribbling
of urine that increased on crying). Three patients
(16.66%) had no neurological deficits (Table 2).
Table 3. Cutaneus markers and number of
patients affected
Hypertrichosis

14 (77.77%)

Dimple

8 (44.44%)

Dermal Sinus

6 (33.33%)

Subcutaneus lipoma

5 (27.77%)

Some form of cutaneous markers was found in

all 18 patients, and hypertrichosis was the most
common skin manifestation, being present in
14(77.77%) patients (Table3). Of the orthopedic
anomalies of the lower limb seen in 9 patients,
congenital talipes equinovarus was the most
common (5 patients). Shortening of one limb
was seen in 2 cases (11.11%), pes planus (flat
foot) in 2 cases (11.11%), and scoliosis 2 cases
(11.11%; Table 4).
Table 4. Orthopedics deformity: total 9 cases
Talipes Equinovarus

5 (27.77%)

Scoliosis

2 (11.11%)

Pes planus

2 (11.11%)

No orthopedics
deformity

9 (50%)

Imaging Findings

Plain x-ray films of the spine and chest revealed

a number of deformities of the vertebrae and
ribs. Scoliosis or kyphoscoliosis was present in 2
patients (11.11%) on imaging. Magnetic
resonance imaging revealed lesion causing
tethering in 15 cases (83.33%). The cord was
low lying in 12 cases (66.66%), whereas Chiari
malformation was not noted. The lumbar and
thoracolumbar regions were the most common
sites involved (in 4 [22.22%] and 14 patients
[77.77%], respectively), and the cervical region
was not involved (Table 5).The breakdown of
patients with Type I and Type II SCM was 7
(31.66%) and 11 (69.13%), respectively.
Table 5. Site of SCM and number of patients
affected
Type I

7 (38.88%)

Type II

11 (61.11%)

Cervical

0 (0%)

Dorso-lumbar

10 (55.55%)

Dorsal

4 (22.22%)

Lumbar

4 (22.22%)

Intraoperative Findings
There were four lumbar SCMs and 14
thorocolumbar SCMs in our series. In seven
patients the spur arose dorsally from the surface
of the neural arch. Various other lesions causing
tethering were observed, including a thick or
tight terminal filum, intradural lipoma, dermoid
or dermal sinus tract. Myelomeningoceles
manqu arising from the paramedian dorsal
surface of the hemicords projecting dorsally and
caudally from their point of origin from the cord
and attaching or penetrating the dura caudally
were noted in 5 (77.42%) of 7 cases of SCM
Type I and in 9 (81.81%) of 98 cases of Type
II. These bands constitute an important cause of

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tethering and whenever encountered they were

divided from their visible dural connections so
that hemostasis was ensured. The ventral surface
of the cord was inspected in all cases to look for
any paramedian ventral nerve roots, but none
was found in the present series. The cord was
detethered through an upper midline incision at
the site of a bone or fibrous spur and a thick,
tight terminal filum was excised through a lower
incision. Type II SCM is characterized by a
fibrous or membranous spur causing the split
and leading to two hemicords in a single dural
sleeve. The dura was opened and the fibrous
septum or arachnoid adhesions were excised
(associated lesions tethering the spinal cord were
also corrected at the same time). The dura mater
was opened and the two dural tubes were
converted to one by suturing lateral margins of
both dural tubes at the midline.

patients, none suffered

deficits.
.
Table 6. Outcome

fresh

neurological

Parame
ter

Num
ber of
cases

Impro
ved

Deterior
ated

Unchan
ged

Motor

15

14

Nil

Sensor
y

15

14

Nil

Sphinct
ers

10

Nil

Lost to follow up: 2 patients

Post complications in 4 patients (22.22%):
2 cases of UTI (11.11%)

Patient Outcome
All of the patients were followed up 2
weeks after discharge and again at 3- to 6months intervals. Of the symptomatic patients,
14 (77.77%) showed improvement in motor
power, 14 (77.77%) of 15 improved with regard
to sensory symptoms, and 9 (90%) of 10
regained continence. Significant healing of
trophic ulcers was seen in 1 (50%) of 2 patients.
Neurological status was static in 1 (5.55%) of 18
patients. No patients showed neurological
deterioration. Neurological deficits improved
gradually. Three patients had permanent deficits,
and the one did not improved after 6 months of
follow up. Two patients were lost to follow up.
(Table 6). Postoperative complications were
present in 4 patients (22.22%) and two patients
(11.11%) had wound infections. All of these
issues were managed conservatively, and the
wounds healed well. Urinary tract infections
developed in 2 patients (11.11%). There were no
operative deaths. Among the symptomatic

2 cases of SSI (11.11%)

Discussion
Despite the well-accepted unified theory
of embryogenesis proposed by Pang, et al., 14
significant confusion exists. These authors
hypothesized that all SCMs result from the basic
error in formation of the accessory neurenteric
canal between the yolk sac and amnion, which is
subsequently invested with mesenchyme to form
the endomesenchymal tract that splits the
notochord and the neural plate. The common
embryological error in SCM involves the
formation of an abnormal fistula through the
midline embryonic disc that maintains
communication between the yolk sac and
amnion and makes possible continued contact
between the ectoderm and endoderm. This
abnormal fistula necessarily leads to regional
splitting of the notochord and the overlying
neural plate. The formation of the abnormal
fistula is the crucial step, and further
developments around the fistula occur
subsequently. As the result of adhesion between
the ectoderm and endoderm, the surrounding

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multipotential mesenchyme condenses around

the fistula to form an endomesenchymal tract
that not only permanently bisects the notochord
but also forces each overlying hemineural plate
to neurulate against its own hemicord. The basic
malformation therefore consists of two
heminotochords and two hemineural plates
separated by a midline tract containing
ectoderm, mesenchyme, and endoderm.14 Further
evolution of this basic form into the full-grown
malformation depends on the ability of the
heminotochords and hemicords to heal around
the endomesenchymal tract, the developmental
fates of the three germ elements within the tract,
the
variable
extent
to
which
the
endomesenchymal tract persists, and the
interaction between the heminotochord and the
hemineural plates during neurulation. Unlike the
case of the endoderm, some derivatives of the
mesenchymal component almost always persist
in the midline cleft of the mature SCM. Meninx
precursors possess a dual fibrogenic and
sclerogenic capability and are the likely source
of midline bone, apart from formation of the
dural tubes. Because normal arachnoid tissue is
derived from the inner lining of the primitive
meninx after the formation of the dura, the side
of the median dura facing the hemicord will
form half of a complete arachnoid tube that
ultimately surrounds each hemicord, whereas the
side of the median dura facing the midline forms
the bone spur. The extent of disappearance of
the endomesenchymal tract and the subsequent
interaction of primitive meninx with the midline
tract remnant are probably genetically
controlled, but further research needs to be done
for this hypothesis to be substantiated.
Uncommon congenital anomalies of the spinal
cord, SCMs are seen more often in female
patients: Pang, et al., 14 reported a female/male
ratio of 1.5:1, and we found in our series (1:3).
Our patients usually presented in two peaks: the
first between 2 and 5 years of age and the
second in a rapid growth phase between 12 and
16 years of age. The mean age of our patients
was 6.33 years; however, the mean age for
neurologically intact patients was 0.7 years.
Four patients in our study were adults older than
16 years of age. Thoracolumbar and lumbar
regions were the most common sites.2, 7,
11,14
Cervical and cervicodorsal SCMs are rare,3,4

and we recorded no incidence. As in other

series, we observed skin lesions frequently in
patients with SCM, hypertrichosis being the
most common. One or more skin manifestations
were present in 14 patients (77.77%).
Asymmetric weakness of the lower
limbs was the most common neurological
deficit and was present in 14 cases (77.77%).
Sphincter disturbances are often described in
patients with SCM.1, 6, 9,11,12,14. The bladder and
bowel disturbances we saw in 55.55% of our
patients are easily explained by their delayed
referral to our center. In 1992, Pang, et al14,
recommended surgical treatment even for
asymptomatic adults who led an active lifestyle.
Miller, et al., 13 noted neurological deterioration
in patients with SCM who did not undergo
surgery. In our study, the average age of
symptomatic patients was higher than that of
asymptomatic ones. Ersahin, et al.,5 also
observed that the
age
and duration of
symptoms in patients with deficits were
significantly greater than in those without
deficits. The risk of neurological deficits
developing increases with age because of
progressive tethering of the spinal cord. 6, 8, 11The
deficit also depends on associated anomalies
such as syringomyelia and on associated
lipomeningomyeloceles and congenital tumors
such as dermoids and epidermoid lesions.
Overall, the outcome also depends on anomalies
of other systems.6, 7,10,14. The particular SCM I
subtype is also an important factor determining
neurological outcome. According to our
experience, Type I is the easiest to address
surgically with the least chance of deterioration
because of the significant space above and
below the bone spur. On the other hand, surgery
is much more difficult in patients with SCM
Type II because of the lack of space for
manipulation above and below the lesion.
Hence, chances of postoperative deterioration
are maximal in Type II SCM. Most surgeons
agree that early surgery is the key issue in the
management of SCM; however, although many
authors recommend prophylactic surgery 6, 11,14
others may not agree because of the risk of
neurological deterioration. Nonetheless, the fact
remains that once a neurological deficit appears,
there is a low chance of complete recovery. In

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the present series, 15 patients had motor deficits

and 14 (77.77%) of them showed varying
degrees of improvement. Sensory improvement
was seen in 77.77% of patients with
preoperative sensory disturbances. The chances
of autonomic improvement are lower still,
reportedly in the range of 15 to 40%.2, 7, 11. In the
present series, autonomic disturbances improved
in 38.88% of patients. These observations
emphasize the role of surgery in neurologically
intact patients to prevent future neurological
deterioration.

Conclusions
Split cord malformations are rare
anomalies of the spinal cord, and we present the
largest prospective series so far reported in the
world literature. The risk of neurological deficits
developing increases with age. We recommend
prophylactic surgery for all asymptomatic
patients with SCM. Cutaneous markers should
be an important pointer for investigations for
occult spinal dysraphism, which was present in
100% of the patients in our series. All of the
patients with congenital and progressive
scoliosis should be screened with MR imaging.
The entire neuraxis should be evaluated and any
tethering lesions found should be simultaneously
treated at the time of the first surgery. Surgery
must be planned meticulously according to the
SCM type. Parents, pediatricians and
gynecologists should be counseled regarding
use folic acid during pregnancy.
References
1. Chandra PS, Kamal R, Mahapatra AK: An
unusual case of a dorsally situated bony spur
in a lumbar split cord malformation.
PediatrNeurosurg 31:4952, 1999
2. Colak A, zcan OE, Erbengi A:
Diastematomyelia in pediatric age. A
retrospective study of 15 cases. J
PediatrNeurosci 4:296300, 1988
3. David KM, Copp AJ, Stevens JM, Hayward
RD, Crockard HA: Split cervical spinal cord

with Klippel-Feil syndrome: seven cases.

Brain 119:18591872, 1996
4. Dias MS, Pang D: Split cord malformations.
NeurosurgClin N Am 6:339358, 1995
5. Ersahin Y, Mutluer S, Kocaman S,
Demirtas E: Split spinal cord malformations
in children. J Neurosurg 88:5765, 1998
6. Goldberg C, Fenelon G, Blake NS, Dowling
F, and Regan BF: Diastematomyelia: a
critical review of the natural history and
treatment. Spine 9:367372, 1984
7. Gower DJ, Del Curling O, Kelly DL Jr,
Alexander E Jr: Diastematomyelia40-year
experience. Pediatric Neurosci 14:9096,
1988
8. Guthkelch AN, Hoffman GT: Tethered
spinal
cord
in
association
with
Diastematomyelia. SurgNeurol 15:352354,
1981
9. Humphreys RP, Hendrick EB, Hoffman HJ:
Diastematomyelia. ClinNeurosurg 30:436
456, 1983
10. Jindal A, Kansal S, Mahapatra AK: Split
cord malformation with partial eventration
of diaphragm. Case report. J Neurosurg 93
(2 Suppl):309311, 2000
11. Jindal A, Mahapatra AK: Split cord
malformationsa clinical study of 48 cases.
Indian Pediatr 37:603607, 2000
12. Keim HA, Greene AF: Diastematomyelia
and scoliosis. J Bone Joint Surg Am
55:14251435, 1973
13. Miller A, Guille JT, Bowen JR: Evaluation
and treatment of diastematomyelia. J

Bone Joint Surg Am 75:13081317,

1993
14. Pang D, Dias MS, Ahab-Barmada M:
Split cord malformation: Part I: a unified
theory of embryogenesis for double
spinal cord malformations. Neurosurgery
31:451480, 1992

1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
22
23
24
25
26
27
28
29
30
31
32
33
34
35
36
37
38
39
40
41
42
43
44
45
46
47
48
49
50
51
52
53
54
55
56
57
58
59
60
61
62
63
64
65

*References

References
1. . Neurosurgery 31: Chandra PS, Kamal R, Mahapatra AK: An unusual case of a dorsally
situated bony spur in a lumbar split cord malformation. PediatrNeurosurg 31:4952, 1999
2. Colak A, zcan OE, Erbengi A: Diastematomyelia in pediatric age. A retrospective study of 15
cases. J PediatrNeurosci 4:296300, 1988
3. David KM, Copp AJ, Stevens JM, Hayward RD, Crockard HA: Split cervical spinal cord with
Klippel-Feil syndrome: seven cases. Brain 119:18591872, 1996
4. Dias MS, Pang D: Split cord malformations. NeurosurgClin N Am 6:339358, 1995
5. Ersahin Y, Mutluer S, Kocaman S, Demirtas E: Split spinal cord malformations in children. J
Neurosurg 88:5765, 1998
6. Goldberg C, Fenelon G, Blake NS, Dowling F, and Regan BF: Diastematomyelia: a critical
review of the natural history and treatment. Spine 9:367372, 1984
7. Gower DJ, Del Curling O, Kelly DL Jr, Alexander E Jr: Diastematomyelia40-year experience.
Pediatric Neurosci 14:9096, 1988
8. Guthkelch AN, Hoffman GT: Tethered spinal cord in association with Diastematomyelia.
SurgNeurol 15:352354, 1981
9. Humphreys RP, Hendrick EB, Hoffman HJ: Diastematomyelia. ClinNeurosurg 30:436456, 1983
10. Jindal A, Kansal S, Mahapatra AK: Split cord malformation with partial eventration of
diaphragm. Case report. J Neurosurg 93 (2 Suppl):309311, 2000
11. Jindal A, Mahapatra AK: Split cord malformationsa clinical study of 48 cases. Indian Pediatr
37:603607, 2000
12. Keim HA, Greene AF: Diastematomyelia and scoliosis. J Bone Joint Surg Am 55:14251435,
1973
13. Miller A, Guille JT, Bowen JR: Evaluation and treatment of diastematomyelia. J Bone Joint

Surg Am 75:13081317, 1993

14. Pang D, Dias MS, Ahab-Barmada M: Split cord malformation: Part I: a unified theory of
embryogenesis for double spinal cord malformations451480, 1992

Table/Figure Legends

Table 1. Clinic-al picture and number of the patients affected

Skin stigmata
Spina bifida
Scoliosis
Orthopedic lower limbs deformity
Sensory, motor and autonomic
Trophic ulcers
Pain: Back and lower limbs

18 (100%)
4 (22.22%)
2(11.11%)
9 (50%)
15 (83.33%)
3 (16.66%)
15 (83.33%)

Table 2. Neurological deficit and number of patients affected

Motor
Sensory
Autonomic
Sensory, motor and autonomic
Neurologically intact

15 (83.33%)
15 (83.33%)
10 (55.55%)
10 (55.55%)
3 (16.66%)

Table 3. Cutaneus markers and number of patients affected

Hypertrichosis
Dimple
Dermal Sinus
Subcutaneus lipoma

14 (77.77%)
8 (44.44%)
6 (33.33%)
5 (27.77%)

Table 4. Orthopedics deformity: total 9 cases

Talipes Equinovarus
Scoliosis
Pes planus
No orthopedics deformity

5 (27.77%)
2 (11.11%)
2 (11.11%)
9 (50%)

Table 5. Site of SCM and number of patients affected

Type I
Type II
Cervical
Dorso-lumbar
Dorsal
Lumbar

7 (38.88%)
11 (61.11%)
0 (0%)
10 (55.55%)
4 (22.22%)
4 (22.22%)

Table 6. Outcome
Parameter
Motor
Sensory
Sphincters

Number of cases
15
15
10

Improved
14
14
7

Lost to follow up: 2 patients

Post complications in 4 patients (22.22%):
2 cases of UTI (11.11%)
2 cases of SSI (11.11%)

Deteriorated
Nil
Nil
Nil

Unchanged
1
1
3

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