Child's Nervous System

, Volume 34, Issue 5, pp 883–891 | Cite as

Management of split cord malformation in children: the Lyon experience

  • Beuriat Pierre-Aurelien
  • Di Rocco Federico
  • Szathmari Alexandru
  • Mottolese Carmine
Original Paper

Abstract

Introduction

Split cord malformation (SCM) is a rare congenital spinal abnormality. Clinical presentation varies. Other congenital defects can be associated. Management is surgical.

Material and methods

We retrospectively reviewed all our SCM cases and reported our experience for its management. From 1990 to 2014, 37 patients were operated. Five situations lead to the diagnosis (orthopedic disorders (n = 8), orthopedic and neurological disorders (n = 16), pure neurological disorders (n = 5), no symptoms except cutaneous signs (n = 7), antenatal diagnosis (n = 1)). Scoliosis was the most common associated condition. The level of the spur was always under T7 except in one case. There were more type I (n = 22) than type II (n = 15) SCM.

Results

Patients with preoperative neurological symptoms (n = 21) were improved in 71.4%. Five out of nine patients that had preoperative bladder dysfunction were improved. Eleven patients needed surgical correction of the scoliosis.

Conclusions

For us, the surgical procedure is mandatory even in case of asymptomatic discovery in order to avoid late clinical deterioration. In any case, the filum terminale need to be cut in order to untether completely the spinal cord. In case a surgical correction of a spinal deformity is needed, we recommend a two-stage surgery, for both SCM type. The SCM surgery can stop the evolution of the scoliosis and it may just need an orthopedic treatment with a brace.

Keywords

Diastematomyelia Diplomyelia Split cord malformation Surgery Tethered cord syndrome Bone spur 

Abbreviations

CSF

Cerebro spinal fluid

CT scan

computed tomography scanner

DM

Diplomyelia

DSM

Diastematomyelia

MM

Myelomeningocele

MRI

Magnetic resonance imaging

SCM

Split cord malformation

US

Ultrasound scan

Introduction

Diastematomyelia (DSM) is a rare congenital spinal abnormality that was first reported in the literature by Ollivier in 1837 [1], sometimes also identified in literature as diplomyelia (DM). In 1992, Pang et al. proposed a new classification, the split cord malformation (SCM), with two types (I and II). According to the authors, type I SCM consists of two hemicords, each contained within its own dural sac and separated by a dura-sheathed rigid osteocartilaginous median septum. In type II SCM, the two hemicords are housed in a single dural tube and separated by a non-rigid fibrous median septum [2].

The embryological origin of DSM has been the subject of a long debate and several theories have been propounded [2, 3, 4, 5, 6, 7]. In 1992, Rilliet, in his fine study on chick embryo, concluded that DSM was a developmental disorder of the notochord and not of the neural plate [7]. Pang elaborated on the embryological mechanism of Bremer and updated the existence and persistence in the malformation of an accessory neurenteric canal [2] (Fig. 1a–e).
Fig. 1

Drawing that represents the embryological theory of the split cord malformation according to Pang theory. a During the second week of gastrulation with a two-layered structures (1 amnionic sac, 2 epiblast, 3 hypoblast, and 4 yolk sac); b gastrulation period during which cells of the epiblast migrate and ingress (black arrows) through the primitive groove to become the endoderm and mesoderm (1 neural plate, 2 primitive groove, 3 ectoderm, 4 mesoderm, and 5 endoderm); c primary neurulation which failed because of an ecto-endodermal adhesion (4) (1 ectoderm, 2 neural plate, 3 somite, 4 ecto-endoderm adhesion, 5 future notochord, 6 endoderm); d primary neurulation which failed due to an ecto-endodermal adhesion which will induce the creation of an accessory neurenteric canal and the formation of two notochords (1 neural crest, 2 neural plate, 3 somite, 4 hemi notochord, 5 accessory neurenteric canal, 6 endoderm; black arrows represent the neural folding and brown arrow represents the invagination of the accessory endoderm/neurenteric canal); e the result of the presence of this accessory neurenteric canal is the creation of two neural tubes (2) (1 cutaneous ectoderm, 2 hemi neural tubes, 3 endomesenchymal tract, 4 hemi notochords, 5 mesenchyme, 6 endoderm)

Not exceptionally, SCM may remain longly asymptomatic until accidentally recognized. In most cases, however, it is suggested by cutaneous markers [8], namely hypertrichosis with the typical faun tail that, in our experience, is a pathognomonic sign or may present with a combination of neurological, orthopedic, or urological signs and symptoms of variable severity. Other congenital malformations such as myelomeningocele (MM) or lipomyelomeningocele may be associated in a few subjects.

Usually, the diagnosis is made the pediatric age. Females outnumber male [9].

The management is controversial in asymptomatic accidentally recognized subjects while the surgical correction is preferred in symptomatic cases. The surgical correction consists of removing the bone spur that separates the two hemicords in order to relieve the pressure on the adjacent nervous and vascular structures with the aim of reducing or curing the neurological deficits in symptomatic patients or at least preventing their progression. In children presenting with progressive scoliosis or kyphosis, the main aim of the operation is to stop the evolution of the spinal deformities. It is debated whether the malformation needs to be corrected in asymptomatic subjects or in children with non-progressive spinal deformity, although several authors consider the surgical correction mandatory even in these cases [9, 10, 11].

In this article, we retrospectively review all SCM cases dealt within our department from 1990 to 2014.

Patients and methods

All the cases of patients who underwent SCM surgery in our department between 1990 and 2014 were considered for this retrospective analysis. The study was approved by the local ethical committee. All patients’ data for this retrospective study were anonymized; therefore, the study was exempted from the need for patient consent. Patients’ gender, age at diagnosis, clinical presentation, associated conditions, type of the malformation (type I or II), spur level, age at operation, surgical modality, post-operative complications, and outcome (orthopedic, neurological, and urinary tract) were collected.

Demographic data are resumed in Table 1. There were two peaks of incidence at 8 and 15 years.
Table 1

Demographic data

Number of patient

37

Female/male

25/12

Mean age at diagnosis (years)

6

Age range

1 month–16 years

Patient under 2 years old

11

SCM type I/type II

22/15

At presentation, 16 children complained of a combination of orthopedic and neurological disorders, eight had only orthopedic disorders, and five pure neurological disorders. In seven subjects, the diagnosis was made merely on the grounds of cutaneous stigmata (Fig. 2a–c). In the remaining case, the malformation had been revealed antenatally by a routine ultrasound (US) scan (Fig. 3). Out of the 29 patients with orthopedic and/or neurological disorders, 11 showed cutaneous signs.
Fig. 2

Cutaneous hallmarks. a The classical hypertrichosis; b pigmentation anomalies with hypertrichosis; c the classical hypertrichosis with a dimple

Fig. 3

A US scan showing the bony spur (antenatal diagnosis)

The clinical presentation at diagnosis is resumed in Table 2 and cutaneous signs in Table 3; scoliosis was the most frequent presenting sign (Fig. 4) followed by foot disorders and MM. Preoperative imaging revealed other spinal-associated lesions in eight cases: a lipoma in five cases and a dermal closed fistulous tract in three cases.
Table 2

Clinical presentation at diagnosis

Clinical presentation

Number of patient (%)

Orthopedic disorders

 Scoliosis/kyphoscoliosis

18 (49)

 Foot disorders

8 (22)

 Trophic disorders

3 (8)

Neurologic disorders

 Myelomeningocele

7 (19)

 Bilateral leg paresis

6 (16)

 Unilateral leg paresis

4 (11)

Urologic disorders (MM excluded)

Bladder dysfunction

9 (24)

Table 3

Description of the cutaneous signs at diagnosis

Skin hallmarks

Number of patient (%)

Hypertrichosis

12 (32)

Dimple

3 (8)

Subcutaneous lipoma

3 (8)

Pigmentation anomaly

1 (3)

Fig. 4

3D reconstruction of a spine CT scan showing scoliosis in a type I split cord malformation

The level of the spur was always under T7 except in one case (Fig. 5). The spur was in the thoracic in 19 cases and in the lumbar region in 18 cases. The most affected level was L1 (n = 7), followed by T12 (n = 5) and T11, and L4 (n = 4 each).
Fig. 5

Bar graph representing the spur level

All patients underwent the surgical correction immediately after diagnosis, even when asymptomatic. The infant with antenatal diagnosis was operated at 1 month of age because he suffered two episodes of urinary infection that were attributed to bladder dysfunction. There were no differences in clinical presentation between patients with type I and type II malformation.

Eleven patients needed surgical correction of the scoliosis that was carried out in all of them only after having removed the malformed bone spur or fibrous band separating the two hemicords. The remaining seven subjects with scoliosis could be managed with a brace.

Postoperative clinical controls were made at 3 and 6 months after the surgical correction and then, every year, every 2 years and every 5 years, depending on the clinical evolution. Postoperative spinal MRI or CT studies were carried out at 3 months and then yearly throughout the entire postoperative follow-up. For the vesical function evaluation, the necessity of complementary examination was accessed by our urologist. Patients were referred to the orthopedist in order to follow up the spine growth and to check for scoliosis if not present at diagnosis. In the cases where the scoliosis was present at diagnosis, the orthopedist’s follow-up consisted in a yearly outpatient consultation with a full spine X-ray. Follow-up ranged from 2 to 20 years (mean 10.2 years).

Surgical management

Under general anesthesia, all the patients (symptomatic and asymptomatic) underwent to the correction of the malformation either in prone or genupectoral position. After a midline incision (approximately 10–15 cm) centered on the level of the abnormality, a subperiosteal exposure of the spinal process and lamina was performed. Then, a laminectomy or laminotomy (at least four laminae) was done in order to expose the spur. Laminotomy was preferred in young children.

For type I, the bony spur was gently dissected subperiosteally from the dura matter under microscopical view. It was then very carefully removed. Some little veins and arteries at its insertion on the posterior aspect of the vertebral body needed to be coagulated with bipolar forceps (see video, supplement digital content 1, which shows the surgical technique to remove the bony spur). The coagulation was further assured by the apposition of bone, compressed with cottonoid on a dissector paying particular attention at avoiding any trauma on the hemicords. After that, each dural sac was opened in a spindle-shaped manner in order to obtain one single dural sac wide enough to avoid any contact between the dura and the spinal cord and the roots. The two medial parts were sutured together, with a continuous non-resorbable suture in order to form the anterior wall of the single dural sac. For such a maneuver, sometimes was necessary to eliminate the excess of the membrane to obtain a good closure. Then, the two lateral parts of the two dural sacs were sutured together with a continuous non-resorbable suture as well to form. The posterior wall of the dural sac (see video, supplement digital content 2, which shows the surgical technique to reconstruct a single dural sac and Fig. 6a–d).
Fig. 6

a A drawing that represents the anatomical situation after the removal of the bony spur. We can see the dura mater that is split into two different dural sacs. b A drawing that represents the line of the dura matter incision (yellow line). c A drawing that represents the anatomical situation after the opening of the dura mater. We can see the two hemi spinal cords. d A drawing that represents the different part of the dura mater that should be closed together in order to recreate a single dural sac

For type II, the dura mater was opened through a midline incision. The non-rigid fibrous spur was detached from its dural insertion on the inner surface of the anterior dural wall (Fig. 7). All adhesions to the roots were detached. The dural sac was closed with a watertight continuous non-resorbable suture.
Fig. 7

Surgical picture under microscopic view of a type II split cord malformation. Note the two hemicords (X) within a single dural sac and the non-rigid fibrous spur (S) attached to the dura

The filum terminale was cut at the lower lumbar level and sent for anatomopathological examination. Macroscopically, this structure was tight with an abnormally fibrotic consistence. When present, other associated abnormalities such as lipomas or dermal sinuses were removed as well in order to minimize the risk of retethering. If the lesion was too far from the SCM, a second incision was performed.

No postoperative wound drain was used, and the patient is allowed to get up the day after the surgery in function of pain (no postoperative mobilization restriction).

No perioperative neurophysiological monitoring was used because it was not available at the time of the surgeries.

Results

Immediate outcomes

We did not have any perioperative mortality, surgical complications, and blood transfusion.

Postoperative complications

Postoperative complications were transient. We observed three cases (8.1%) of pseudomeningocele that were resolved conservatively with compression dressing and diuretic medication for 1 week. Four children cases (10.8%) complained of transitory motor worsening. There were two wound infections (5.4%) without meningitis that were treated with standard antibiotics.

Late outcomes

In no case, there was regrowth of the bony spur at the late MR or CT control.

Neurological outcome

The neurological long-term outcomes were overall good. The 16 patients with no preoperative neurological symptoms were not worsened and had stable neurological condition. In the group of patients with preoperative neurological symptoms (n = 21), 15 (71.4%) were improved, 5 (23.8%) stabilized, and 1 (4.8%) worsened. Generally, motor deficit improvement was observed after the first 6 postoperative months and continued during the first 2 years after the operation.

Vesico-sphincterian outcome

The long-term urinary function outcome was good as well. No patient who did not have preoperative bladder dysfunction worsened. If we excluded the seven MM patients, five out of nine patients with preoperative bladder dysfunction were improved clinically, three were stabilized, and one worsened during the follow-up period.

Discussion

Embryology and associated conditions

The actual incidence of DSM is difficult to estimate as this rare congenital malformation has been described in literature under different definitions and often underreported or only mentioned as an additional finding of other more common malformative conditions such as MM or lipomyelomeningocele. Indeed, for many years, DSM was distinguished from DM as they were considered to represent two different pathological conditions. DM was regarded to be a spinal cord abnormality characterized by a real duplication of the cord, contained in a unique dural sac, in which each hemicord had its own paramedian and lateral nerve roots. On the other hand, the term DSM was reserved to cases where the spinal cord was divided into two hemicords, each one being contained in its own dural sac. In 1992, Pang published a unified theory in which the distinct definitions of DSM and DM were abandoned in favor of the unique denomination of SCM [2]. The author propounded that all cases of SCM had the same embryological origin and that the presence of one or two dural sacs, the presence or absence of paramedian nerve roots, the presence of a rigid osteocartilaginous median septum, or the presence of a non-rigid fibrous median septum did not imply a different ontogenetic error. The unified theory of Pang has gained a wide favor in recent times after a long debate that was characterized by the proposal of several theories, namely the split notochord syndrome theory, the accessory neurenteric canal theory, the hydromyelic theory, the twinning theory, the overgrowth theory, and the neuroschistic blebs theory [2, 3, 4, 5, 6, 7]. By examining the findings observed in his patients and taking into account the embryologic development, Pang concluded that the SCM originates from a splitting of the notochord in agreement with the experimental conclusion of Rilliet [2, 7]. According to Pang’s theory, the splitting of the notocord is caused by the formation of adhesions between the ectoderm and the endoderm leading to an accessory neurenteric canal that condenses an endomesenchymal tract which divides the notochord finally resulting the formation of two hemineural plates [2]. The two types of SCM described by Pang, according to the presence of a spur that can be osseous cartilaginous or fibrous [2, 9]. Three different positions of the placode in SCM are described: the first one in which the placode is terminal, the second one in which the placode is not terminal, and the third in which a fibrous band travels with the fibrous septum to the base of the meningeal outpouching with the placode at the level of the malformation [12, 13]. In our series, the neural placode was situated caudally to the level of the split cord in all cases, and we never saw spinal roots on the median side of the hemicord. SCM is associated with other neural tube defects such as MM in 5 to 36%. In our series, seven patients (18.9%) had associated MM, five (13.5%) had a lipoma, and three (8.1%) had a dermal closed fistulous tract.

An accurate radiological examination is mandatory in SCM. We carried out a MRI study in all cases where this diagnosis was evocated. The preoperative radiological work-up with spinal MRI and a spina CT scan (Fig. 8a–c) is very important as the surgical technique to untether the spinal cord is different for SCM type I and II and, in case of associated MM, and depends on the placode localization too. In the case of MM, if the malformation is situated rostrally, it can be treated during the same surgical procedure. When the antenatal diagnosis is done [14, 15] of an isolated SCM, the prenatal counseling can reassure the parent that the neuropsychological development will not be worsen because of the malformation. In our series, if we exclude MMC patients, all others had a normal neuropsychological development.
Fig. 8

a Axial T2-weighted MRI sac showing a type I split cord malformation with a bony spur splitting the spinal cord into two hemicords within two separated dural sac. b 3D reconstruction of a spine CT scan showing a complete bony spur in a type II split cord malformation. c Axial view of a spine CT scan showing an incomplete bony spur in a type II split cord malformation

In case of associated MM, we proceeded to repair this malformation within the first 48 h of life to decrease the risk of infectious complications, while the surgical correction of SCM, when located far from the placode, was delayed of a few months. Similarly, delayed surgical correction was performed in case of an associated lipomyelomeningocele even when recognized at birth. In this last instance, however, attention was paid to not postpone the operation excessively late. Actually, in our experience, the surgical treatment of a lipomyelomeningocele was less risky during the first year of life than in older age when the increased adherence of the abnormal fat tissue to the nerve roots make the surgical manipulation more tedious and dangerous in case of a dermal closed fistulous tract; it must be resected during the correction of the SCM.

Concerning the spur, in our series, it was located in the lumbar region in the great majority of the cases and always inferior to T7. We have not observed cervical spur as others [16]. The cervical localization is mostly accompanying Klippel-Feil syndrome that results from a more severe mesenchymal trouble with fusion of the vertebral bodies [5].

Split cord malformation and tethered cord

The tethering of the spinal cord in SCM depends on the spur (bony or fibrous) which impedes the up and down movement of the spine and on the nearly always association with a fatty or tight filum terminale as we proved in our series by systematic anatomopathological examination of this structure. This dual cause of spinal cord tethering should then be managed possibly in the same operation [17].

Surgical technique and indications

The timing and technique of SCM correction were and still are a subject of debate. It is generally accepted that early surgery leads to better results so that some authors will advocate prophylactic surgery [11, 12, 16, 18, 19]. Other authors, however, recommend a more careful attitude because of the surgical risk, especially in still asymptomatic children [20]. Nevertheless, such a cautious attitude should be weighted taking into account the possibility of non-reversible neurological deficit or progression of an associated scoliosis. Actually, in 78% of our patients the diagnosis of SCM was due to an actual clinical progression that suggests the evolutive nature of the malformation in the great majority of cases. A further demonstration of the risk of a wait and see policy is provided by many reports of the literature related to patients becoming symptomatic in adult life [12, 21]. In this series, we adopted a prophylactic approach which appears to be justified by the results we achieved. None of our asymptomatic children (n = 7) were damaged by the operation. All but one patient with neurological deficit at the time of surgery were stabilized or improved. Only one patient who had already a neurological deficit at the time of the treatment presented a worsening with an incomplete recovery. Among patient with neurological deficit and scoliosis, the surgery did worsen the neurological status. Nearly 90% of patients with sphincterian disorders before the surgery (n = 9) were either stabilized or improved, a result which further supports the hypothesis that the neurosurgical treatment prevents the natural worsening of such a function in SCM.

The surgical treatment of SCM is technically demanding and requires an expert surgeon to avoid spinal cord traumatism during procedure. The malformation should be approached from the nearest intact superior and inferior spinal cord segment moving towards the area of the bone spur or splitting fibrous band slowly. In SCM type I, the bony spur must be removed before opening the dura mater. The dissection of the spur from the dura mater must be done with extreme caution in order not to damage both hemicords. When the bony spur is removed, the dura mater is opened on both sides and suspended. Then, the goal is to recreate only one dural sac. The margins of the internal sheet are sutured on the midline in front of the two hemicords to achieve a water-tight suture of the anterior wall of the unique sac to be created. The posterior wall is finally reconstructed suturing the lateral margins above the two hemicords. We do not use artificial dural patch because, in our experience, it is large enough to avoid the use of this material to recreate a large perimedullary space in order to reduce the risk of late retethering unlike in the surgery of conus lipomas. We consider this maneuver relevant to prevent the re-formation of the bone spur which was propounded to depend on an osteogenic activity of the dura mater in contact with the base of the removed spur. Indeed, we have never observed a regrowth of the bony spur when it is removed completely as others had [22].

In case where the SCM is near the filum, this structure is sectioned from the lower aspect of the operative field prior suturing the posterior wall of the dural sac. If the SCM is far from the filum terminale, a second additional incision at the L5-S1 level is required.

Concerning the risk of postoperative deficits and their relationships to the type of SCM, some authors have reported a major incidence in case of type I [11]. Even if our surgical results were not different in the two types of SCM, we agree on the major surgical difficulties when dealing with type I SCM. Furthermore, the level of the spur can condition the surgical risk with an increased neurological risk in higher location.

Scoliosis management

Besides sensorimotor deficits and vesico-sphincter anomalies, SCM is often associated with orthopedic abnormalities, namely scoliosis, which often are progressive especially when there is a malformation of vertebrae like a butterfly vertebra. About one third of our children presented with a progressively worsening scoliosis. In cases, surgical correction of the scoliosis is needed; we strongly recommend untethering the spinal cord both at the level of the SCM and the filum prior to the orthopedic procedure in order to prevent neurological complications. Also, neuroimaging studies of cervicocranial junction should be obtained before the operation on the spine in order to exclude a possible, though rare, Chiari type I malformation which also may require a preventive correction. Even though some authors do not consider the treatment of SCM necessary prior to the surgical management of scoliosis [23, 24], we prefer to avoid further constraints and straining forces on an untreated malformation; the blood supply of which could be already in a precarious equilibrium and unable to tolerate further ischemic insult such as those that may result from a mechanical stretching of the spine.

Furthermore, if the correction of the spine deformation must be made, we recommend a two-stage surgery as described by Winter in 1974 [25]. First of all, the complication rate described for the one-stage procedure is quite high [23, 24]. In particular, the longer duration of the one-stage procedure might enhance the risk of infection that is already favored by the use of osteosynthesis materials. Also, it should be emphasized that adopting the two-stage procedure, the clinical response to the initial operation to correct the SCM may be so favorable that the subsequent orthopedic correction on the scoliosis might not be necessary anymore as we noticed in 40% of our cases. Moreover, the correction of the spinal deformity in a one-stage procedure may be lower than in a two-stage one [23]. One other argument against the two-stage surgery is that most of the time, the age at surgery is low and therefore the decision to perform the surgery for the scoliosis is not taken. Once again, some patient will improve their scoliosis after the tethering; therefore, our orthopedic team follow the patient closely after the surgery to see if the deformation improves or not.

Conclusions

SCM is a rare and complex congenital abnormality which is difficult to understand from an embryological point of view. In our opinion, surgical treatment is mandatory, and we recommend early surgery to prevent clinical evolution, especially vertebral deformation and neurological and vesico-sphincter deficit. Most of the time, surgery helps symptomatic patients improving their clinical evolution. We recommend always completing the surgery of the malformation with the filum terminale section. In case of severe scoliosis, we recommend a two-stage surgery. To finish, it is important to remember that patients with SCM generally have a good neuropsychological development. This point is of tremendous importance when the diagnosis is done before birth in order to provide good prenatal counseling to parents.

Notes

Compliance with ethical standards

Conflict of interest

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper. This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors

Supplementary material

381_2018_3772_MOESM1_ESM.mov (249.5 mb)
supplement digital content 1 surgical technique to remove the bony spur (0:02: dissection of the spur; 0:14 coagulation of the veins and arteries; 0:23: removal of the spur) (MOV 255506 kb)
381_2018_3772_MOESM2_ESM.mov (343.4 mb)
supplement digital content 2 surgical technique to reconstructed a single dural sac (0:01: opening of the dura; 0:10 closure of the dura; 0:31: one single dural sac is obtained) (MOV 351658 kb)

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Beuriat Pierre-Aurelien
    • 1
  • Di Rocco Federico
    • 1
  • Szathmari Alexandru
    • 1
  • Mottolese Carmine
    • 1
  1. 1.Department of Pediatric NeurosurgeryHôpital Femme Mère Enfant, Hospice Civil de LyonLyon CedexFrance

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