Keywords
posttubercular complication - syringomyelia - arachnoiditis - spinal shunt - flexible
endoscopy
Introduction
Syringomyelia is a rare pathological condition characterized by the presence of cystic
cavities within the spinal cord. The etiopathogenesis is still unclear but it can
rarely develop as a complication of tuberculous meningitis (TBM), even after successful
chemotherapy. Postinfectious arachnoiditis alters cerebrospinal fluid (CSF) flow into
the spinal cord with the possible formation of enlarging cavitation that could lead
to a severe myeloradicular compression.[1] Post-TBM syrinx incidence rate is related to the prevalence of TBM in the community,
and has been rarely described in literature with only 30 cases reported so far.[2]
[3]
[4]
[5]
[6]
[7]
[8] A precise diagnosis is mandatory to help the physician to adopt the appropriate
management and conservative treatment is not effective for post-TBM syringomyelia.[9] Early surgery is highly recommended before the establishment of gross neurological
deficits or in case that spine magnetic resonance imaging (MRI) highlights increased
size and extension of the cavity. Surgery can immediately arrest the progression of
syrinx with the rapid improvement of symptoms. Shunting procedures are advocated when
reestablishment of the CSF circulation is impossible.[1] However, the choice of treatment should be case tailored. In 2000 Kaynar et al described
a case of posttubercular syringomyelia treated with a syringosubarachnoid shunt with
a silastic “T” tube insertion. The syrinx relapsed after 2.5 years, due to arachnoid
thickening and formation of new adhesions and the patient underwent a reintervention
with placement of a syringoperitoneal shunt.[4] Iwatsuki et al in 2014 described a new modified surgical approach in order to minimize
the postoperative recurrence of syrinx, when treating syringomyelia associated with
spinal adhesive arachnoiditis: partial arachnoid dissection and syrinx-far distal
subarachnoid shunt placement.[10] In this report, we describe a case of a symptomatic posttubercular dorsolumbar syringomyelia.
After an extensive review of post-TBM syringomyelia cases, we decided to treat the
patients with a double approach: endoscopic arachnolysis and an s-shaped syringosubarachnoid shunt placement.
Case Presentation
A 25-year-old African male patient was admitted to our infectious diseases department
complaining headache, fever, confusion, and a mild weakness of the left hand. After
serological tests and brain and spine MRI, a diagnosis of tubercular meningoencephalitis,
with right frontal tuberculoma, was made. Antitubercular therapy was administered
and the patient progressively recovered and was discharged.
After 2 months, he came back complaining paraparesis with hypoesthesia below T5 level
and bladder dysfunction. Spine MRI scans showed dural enhancement all along the spinal
cord and highlighted a lesion, of 6.5 mm of diameter, at T5-T6 level. In T5-T8 levels
the spinal cord appeared edematous with an enlarged centromedullary canal. The patient
underwent a T5-T7 laminectomy to obtain the specimen and release the pressure on the
spinal cord. The syrinx was not considered for an elective surgical treatment. Steroids
and monoclonal antibodies were administered for 3 months with symptoms improvement.
At the time of discharge, the patient complaints of mild paraparesis with complete
recovery of bladder function.
After 4 months, he was readmitted for worsening of paraparesis. Spine MRI scans documented
enlargement of the syringomyelic cavity in the thoracolumbar tract.
The patient underwent spine computed tomography scans with the evidence of an almost
complete interruption of CSF flow below the level of D7, site of the previous laminectomy.
CSF analysis was negative for mycobacterium and cells count documented high proteins
levels (107 mg/dL). At this point, it was decided to proceed with the surgical treatment
of the syrinx. A double approach was performed: the creation of a syrinx-subarachnoid
shunt and the endoscopic navigation of the adjacent subarachnoidal spaces.
Neurophysiological monitoring was set up to evaluate motor and sensory changes in
evoked potentials of lower limbs' muscles (abductor halluces brevis, anterior tibialis,
vastus lateralis) and of the anal sphincter. A single level laminectomy was performed.
The dura mater and the thickened arachnoid were opened on midline to expose a swollen
but still pulsating spinal cord. Myelotomy was performed with forceps and scissors.
Clear and transparent pressured CSF flew out. The spinal cord immediately appeared
more relaxed allowing the exploration of the posterior subdural space. At that point,
a 2.5 mm diameter flexible endoscope (STORZ, KARL STORZ GmbH & Co. KG) was set up
and handled with a free-hand technique. Particular attention was paid on avoiding
excessive pressure on the spinal cord. Endoscopic navigation, driven upward and downward,
showed postinfectious adhesions. The fibrous septa encountered were quite easily dissected,
carefully pushing the endoscopy 20 cm in both directions. The endoscope was pushed
till L5 level, to clearly visualize and debride the nervous roots ([Fig. 1]). The anterior and lateral subdural spaces were not explored. Furthermore, we tried
to introduce the endoscope through the myelotomy, but the narrow stomy did not allow
the insertion. Thanks to the direct visualization of the subarachnoid space it was
possible to unleash the arachnoid adherences and get an accurate arachnolysis, both
in cranial and caudal spaces. At this point we shaped a silicon-made cannula (35 mm
length and 3 mm outer diameter) in an S italic form. An s-shaped spindle was inserted into the cannula and then was boiled in sterile water
for 1 minute to mold it ([Fig. 2]). The top of the cannula was placed through the stomy into the syrinx with the lower
part leaning on the posterior face of the spinal cord. Once checked the proper functioning
of the shunt, we fixed it with a drop of fibrin glue ([Fig. 3]).
Fig. 1 (A) Midline sagittal T2-weighted preoperative spine MRI showing syringomyelia. (B) Endoscopic imaging: the endoscope through the perimedullar fibrous septae consequent
the arachnoiditis. (C) Endoscopic evidence of the fibrous adherences stretched between the cord and the
arachnoid layer. (D) Endoscopic view of the roots of the cauda equina and the fibrous septae between
them. MRI, magnetic resonance imaging.
Fig. 2 Picture of the silicon shunt and its measures.
Fig. 3 Intraoperative imaging. (A) Evidence of thickened spinal arachnoid layer. (B) Posterior view of the enlarged spinal cord. (C) A 6-mm myelostomy opened on the posterior midline. (D) The shunt in its definitive position. It is evident the decompression of the spinal
cord after the drainage of the syringomyelic cavity.
No disturbances in motor evoked potential or hemorrhagic complications occurred during
endoscopic navigation. Neurophysiological monitoring showed improvement of the motor
evoked potentials after the opening of the dura mater and after the emptying of the
syrinx ([Fig. 4]). Postoperative follow-up was characterized by a significant improvement of the
legs' strength. Early after surgery, the patient was able to walk alone with support
and 15 days later he only complaint about a slight weakness in both legs (more evident
in the right leg). After 40 days, spine MRI scans showed the reduction of the diameter
of the syrinx (3 mm), the improvement of the CSF flow, and the unchanged position
of the subdural shunt ([Fig. 5]).
Fig. 4 Motor evoked potentials (MEP) screenshots. Comparison between MEP before the dural
opening (up) and MEP after the drainage of the syrinx (down): improvement of the conductivity
is particularly evident while monitoring left vastus lateralis.
Fig. 5 (A) Midline sagittal T2-weighted preoperative spine MR image of the syrinx. (B) Midline sagittal T2-weighted image from the spine MRI performed 40 days after the
operation showing the reduction of the syrinx diameter. The device is barely evident
in the lower part of the syrinx. (C) Same image as in B where the position of the silicon shunt has been artificially
enhanced. MR, magnetic resonance; MRI, magnetic resonance imaging.
Discussion
Syringomyelia is a rare condition that affects 8.4 out of 100,000 people in the general
population[1] and is defined as a cystic formation within the spinal cord. The etiology is still
unclear but is often associated with traumatic events, infectious processes, hindbrain
herniation-syndromes (Chiari-like) and occasionally craniovertebral junction malformations.[11]
[12]
[13]
[14] In the adult 50% of cases are associated with Chiari I malformations and the remainder
are of acquired nature: whereas 25% are associated with spinal cord trauma or postinfectious
arachnoiditis.[15] Tuberculosis may affect the vertebrae (Pott disease) and rarely nonosseous structures
of the spine.[8] Posttubercular adhesive arachnoiditis is a rare late complication of tubercular
meningitis and its association with syrinx formation[15]
[16]
[17] is even rarer. Some authors believe that the initial stage of syringomyelia formation
in the case of spinal arachnoiditis could be an intramedullary cystic degeneration
caused by ischemia as consequence to a circulatory disturbance in the pia-arachnoid
layers. The blockage of CSF pathways around the spinal cord contribute to the formation
of intramedullary cystic cavities.[9] Recent studies described how the interstitial edema caused by the pia-arachnoid
scarring has a major role in the development of syringomyelia in spinal arachnoiditis.[1] Disturbances in venous circulation may interfere with CSF absorption and lead to
excessive fluid intake from interstitial spaces. This process could expand intramedullary
microcystic lesions and eventually form a syrinx.[18]
CSF diversion procedures as syringe-subarachnoidal, syringe-pleural,[20] and syringe-peritoneal shunt, were widely used in the past, with the evidence of
early improvement of symptoms.[11]
[12]
[21]
[22] Unfortunately long-term effectiveness has been recently questioned because of the
high rate of failure or the poor long-term outcome[19] observed in approximately half of the treated patients.[9] Aghakhani et al reported a considerable number of recurrences (73%) and aggravation
of symptoms (40%), after the shunting procedure, for a mean follow-up of 86 months.[21] Syringe-subarachnoid shunt is the treatment of choice when shunting with the peritoneal
space is not feasible,[4] but also syringe-subpleural shunt have shown a good efficacy for the resolution
of the syrinx and it also proved to be useful as rescue procedure in a patient with
syringomyelia refractory to the restoration of CSF.[23]
[24]
[25]
[26] A potential issue related to the creation of a channel linking the syrinx to the
subarachnoid space could be the developing of flow reversal into the syrinx in particular
during Valsalva maneuvers. Furthermore, in tuberculous arachnoiditis, the high protein
content of the CSF and the lack of a pressure head between the syrinx and subarachnoid
space often lead to blockage of the shunt on a long-term basis. Some authors used
a T-tube for syringe-subdural shunts. This technique presented some limitations: the
development of rotational forces, due to the shunt shape, can damage the spinal cord;
the removing of a T-tube, without cord injury, is more difficult in the case of complications
such as obstruction, infection, or cord compression ([Table 1]).[6]
Table 1
Review of literature: Cases of the syrinx in post-TB arachnoiditis with treatment
and outcome. For a complete overview of treatment modalities, we also reported two
cases of posttraumatic syrinx formation
Authors
|
Age and sex
|
Localization
|
Etiopathogenesis
|
Preoperative symptoms
|
Treatment
|
Follow-up
|
Outcome
|
Complication
|
Posttraumatic iatrogenic degenerative
|
Infection
|
Motor symptoms
|
Sensory symptoms
|
Shunt dislocation
|
Resolution
|
Recurrence of syrinx
|
Sharma et al[8]
|
23, M
|
Dorsal
|
|
MT
|
Asymmetrical wasting of thenar and hypothenar muscles of both the hands and bilateral
foot drop + dissociative sensory loss below C5 level with sparing of posterior column
sensations
|
Full antitubercular chemotherapy
|
nd
|
NI
|
|
Yes
|
No
|
Bhagavathula Venkata et al[13]
|
54, F
|
From C4 to C6
|
Yes
|
|
Wasting of the right forearm + paresthesias in all four limbs + impaired pain and
temperature sensation in the right upper limb
|
Decompressive laminectomy
|
3–6 mo
|
NI
|
SI
|
|
Yes
|
No
|
Iwatsuki et al[10]
|
52, F
|
From C7 to T7
|
Yes
|
|
Progressive gait disturbance and sphincter deficiency
|
Far distal SSS + laminectomies + arachnolisis
|
1 y
|
MI
|
SI
|
No
|
Yes
|
Asymptomatic large pseudomeningocele
|
73, F
|
From T8 to L 1
|
Yes
|
|
Progressive gait disturbance and numbness in lower limbs
|
No
|
Yes
|
No
|
Kim et al[6]
|
54, M
|
L1
|
Yes
|
|
Impairment of motion and position sense on his right side + motor weakness of the
right upper extremity
|
SSS with a T-tube
|
6 mo
|
MI
|
SI
|
No
|
Yes
|
No
|
Khalid et al[5]
|
4, M
|
From T1 to T6
|
|
MT
|
Acute flaccid paralysis
|
Laminectomy + midline myelotomy
|
|
MI
|
SI
|
|
Yes
|
no
|
Ramanathan et al[7]
|
52, F
|
From C5 to D6
|
|
MT
|
Quadriparesis + spasticity a + respiratory failure and bladder + sensory loss could
not be assessed at that time
|
Conservative treatment
|
nd
|
MI
|
SI
|
|
Yes
|
No
|
Gul et al[3]
|
21, M
|
From T11 to L1
|
|
MT
|
Spastic paraparesis + T10 sensory level + neurogenic bladder
|
SPtS
|
1 mo, 6 mo, 2 y
|
MI
|
NI
|
No
|
Yes
|
No
|
Ersoy et al[2]
|
19, M
|
From C1 to T6
|
|
MT
|
Quadriparesis
|
SPtS
|
18 mo
|
NI
|
No
|
No
|
No
|
Ohato et al[28]
|
47, M
|
From C2 to T2
|
|
MT
|
Weakness both hands with muscle atrophy + hypalgesia below the level of T4 Sphincter
tone depressed. Spastic bladder
|
Arachnolysis
|
1 y
|
MI
|
SI
|
|
Yes
|
No
|
Kaynar et al[4]
|
30, F
|
From T3 to T9
|
|
MT
|
Spastic paraparesis + loss of bladder and bowel control
|
SSS with a T-tube
|
2.5 y
|
MI
|
nd
|
No
|
Yes
|
Yes
|
Abbreviation: F, female; M, male; MI, motor improvement; MT, mycobacterium tuberculosis;
nd, not described; NI, no improvement; SI, sensory improvement; SPtS, syringoperitoneal
shunt; SSS, syringosubdural shunt.
Several authors proposed arachnolysis to resolve the spinal cord tethering and blockage
of the subarachnoid space caused by adhesions. Arachnolysis, without CSF shunting,
demonstrated to have excellent results in postadhesive arachnoiditis, regardless of
the etiology of the syringomyelia, and a significantly longer symptom-free period
before recurrence.[27]
[28] In literature, there is no clear consensus about the optimal surgical extent of
arachnolysis. Klekamp advocates the use of duraplasty to increase the size of the
arachnoid space and to limit tethering, combined with the reduction of bleeding into
the arachnoid space.[29] Nevertheless, in case of treatment failure, CSF diversion could be the last treatment
option via any of the aforementioned shunting methods.[25] However, the surgical outcome following broad arachnoid dissection and syrinx-subarachnoid
shunts are limited by the risk of surgical damage to the spinal cord and the postoperative
formation of new adhesions.[30] In their multivariate regression analysis, Ghobrial et al showed that CSF diversion
did not have a significant impact on the outcome and that arachnolysis was the only
surgical treatment to have a significant effect to prevent recurrences and improve
the outcome ([Table 2]).[27]
Table 2
Review of recent case series of postinfectious syringomyelia and treatment modalities
Authors
|
Patients (N)
|
Sex
|
Age (median)
|
Localization
|
Etiopathogenesis
|
Preoperative symptoms
|
Treatment
|
Follow-up
|
Outcomes
|
Tumoral
|
Malformative
|
Posttraumatic/iatrogenic
|
Infectious
|
Unknown
|
Motor symptoms
|
Sensory symptoms
|
Shunt dislocation/complication
|
Resolution of syrinx
|
Recurrence of syrinx
|
Reoperation
|
Fan et al[23]
|
26
|
M 16
F 10
|
41.5
|
From C to T levels
|
|
12
|
6
|
5
|
3
|
nd
|
SPS
|
16–53 mo
|
25 MI, 1 NI
|
|
1 shunt infection
|
24
|
No
|
1
|
Soo et al[22]
|
5
|
M 1
F 5
|
42.4
|
From C to T levels
|
|
|
2
|
|
4
|
Intractable pain + motor weakness
|
Silastic wedge SSS
|
3–36 mo
|
5 MI (3–6 of postarachnoiditis)
|
|
No
|
Yes
|
No
|
|
Isik et al[19]
|
44
|
|
9.1
|
nd
|
|
32
|
|
|
12 (primary medullar cavitation)
|
nd
|
21 PCF + SPS and 21 only SPS
|
1–17 y
|
6 NI, 5 MD, 39 MI
|
NI
|
2 shunt migration, 1 misplacement, 1 tethering, 1 CSF overdrainage
|
|
|
4 + (3 SPS only required PCFD)
|
Mauer et al[33]
|
28
|
M 15
F 13
|
42
|
nd
|
|
|
6
|
1 (bacterial meningitis)
|
21
|
Progressive neurological deterioration
|
Endoscopic arachnolysis
|
1 wk–2 y
|
1 MD
|
1 SD
|
nd
|
18/22
|
04/28
|
3
|
Oluigbo et al[20]
|
22
|
M 14
F 5
|
48
|
nd
|
2 (hemangioblastoma)
|
5
|
5
|
1 (bacterial meningitis)
|
6
|
nd
|
LPS + PCFD for CM
|
3–51 mo (3 lost follow-up)
|
5 MI (3–6 of postarachnoiditis)
|
nd
|
No
|
2
|
nd
|
6 with SPS or SSS
|
Colak et al[29]
|
8
|
M 5
F 3
|
32
|
C
|
|
CM
|
nd
|
nd
|
nd
|
nd
|
PCFD + SSS
|
12 mo
|
MI
|
7 SI, 1 NI
|
No
|
|
|
|
Koyanagi et al[9]
|
15
|
M 6
F 9
|
46.9
|
From C to T levels
|
|
|
4
|
6 MT + 3 ndd
|
2
|
13 tetraparesis + 2 paraparesis (5 complete motor and sensory paralysis of legs)
|
10 SPtS, 3 SSS, 2 VPS
|
1 mo–10 y
|
9 MI, 1 NI, 5 MD
|
2 SD, 13 SI
|
No
|
9
|
5 unchanged or enlarged syrinx
|
4 SPtS + 2 LPS + 2 SSS
|
Abbreviations: C, cervical; CSF, cerebrospinal fluid; CM, Chiari malformation; F,
female; LPS, lumbo-perotonea shunt; M, male; MD, motor deterioration; MI, motor improvement;
nd, not described; NI, no improvement; ndd, not define diagnosis; PCFD, posterior
cranial fossa decompresion; SD, sensory deterioration; SI, sensory improvement; SPS,
sub-pleural shunt; SPtS, syringoperitoneal shunt; SSS, syringosubdural shunt; T, thoracic;
VPS, ventriculoperitoneal shunt.
Endoscopic examination of the spinal cord, cord surface, and syrinx cavities have
been reported in the past decades in cadaveric studies, before clinical endoscopic
intervention. Endoscopic examination of intradural arachnoid cyst and syrinx have
been described as a less invasive procedure than the standard surgical approach[31] but on the basis of the current understanding of the pathophysiology of syringomyelia
it is not considered an effective treatment.[16] Huewel et al support that the use of a flexible neuroendoscope may have the possibility
to perforate the septa which subdivide the cavity under visual control. In their studies,
they reported 11 cases of septate syringes, which have been operated using a flexible
neuroendoscope.[32]
[33] Endoscopes could be safely inserted and approached to the lesions under direct vision,
avoiding blood vessels and nerve roots on the spinal cord surface. Also, Endo et al
described the use of a flexible endoscope to inspect spinal cord and the subarachnoid
space and in some patients, the resection of arachnoid adhesion was attempted.[34] The use of a flexible endoscope with a diameter bigger than 2.5 mm presents high
risk of injury of the spinal cord or the spinal vessels by the rear parts of the instrument,
which cannot be seen by the surgeon.[16]
Conclusion
In the reported case of posttubercular syringomyelia not responsive to antitubercular
therapy and with progressive debilitating deficits we adopted a surgical strategy
consisting in syringe-subarachnoid shunting and endoscopic arachnolysis using a flexible
endoscope. We observed that shaping the shunt on an S-italic form allowed an easier
placement, an increased stability of the implant and an easier revision procedure
in case of complications. Furthermore, we support that the endoscope can allow a wider
arachnolysis, both cranially and caudally, in comparison to that executed with a microscope.
The combination of endoscopic arachnolysis and syringe-subdural shunt proved to have
the synergistic effect needed to resolve the syrinx and restore the CSF flow in selected
case of posttubercular syringomyelia.