Kavernózní angiomy kaudy equiny jsou vzácné cévní malformace; v literatuře bylo popsáno pouze 15 případů. U většiny případů malformace adherovala k míšním kořenům kaudy equiny.
Popis případu: Je popisován případ 67letého muže s jednoměsíční historií bolesti dolní části zad vyzařující do oblasti levého ischiadiku, s exacerbací bolesti v posledním týdnu. Při neurologickém vyšetření byla zjištěna poklepová bolestivost nad lumbosakrální páteří, palpační citlivost nad levým ischiadikem, taktilní hypestezie v dermatomu L3–4 vlevo a chabá paréza levé dolní končetiny. Dále byl snížen reflex patelární a šlachy Achillovy vlevo. Magnetická rezonance odhalila nehomogenně se sytící masu obliterující míšní kanál v úrovni L2–3 a komprimující kaudu equinu. Masa, která adherovala ke kořenům a filum terminale, byla při operaci totálně resekována. Histopatologické vyšetření potvrdilo diagnózu kavernózní malformace (CM). Po operaci rychle ustoupila bolest, do šestého měsíce ustoupila i paréza a poruchy citlivosti.
Závěr: CM kaudy equiny je extrémě vzácná léze, která se může manifestovat bolestí dolní části zad a v ischiadické oblasti, neurologickým deficitem nebo subarachnoidálním krvácením. Předoperační diferenciální diagnóza oproti intradurálnímu tumoru pouze ze zobrazovacího nálezu, zejména u případů bez krvácení, není snadná. CM je léčitelná chirurgickou excizí. Je bezpečné excidovat filum terminale nebo izolovaný kořen v případě, že k nim CM pevně adheruje.
Y. Yi 1; D. Zhao- xia 2; Z. Dong 1; Y. Gang 1; T. Wen- yuan 1
Department of Neurosurgery, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China
1; Department of Intensive Care Unit, Xi’nan Hospital of The Third Military Medical University, Chongqing, China
Cesk Slov Neurol N 2009; 72/105(6): 575-579
Background: Cavernous angiomas of the cauda equina are rare vascular malformations; only 15 cases have been reported in the literature. Most cases described adherance to the spinal root of the cauda equina.
Case description: A 67‑year- old male patient presented with a 1-month history of lower back pain and left sciatica and a 1‑week history of exacerbated symptoms. On neurological examination the patient was found to be tender to percussion over the lumbosacral spine, had tenderness over the left sciatic nerve, left hypoesthesia on the L3– 4 dermatome with left flaccid lower monoparesis. In addition, he was found to have decreased patellar and Achilles’ reflexes on the left. Magnetic resonance imaging (MRI) revealed a heterogeneous enhancing mass obliterating the spinal canal at the L2– 3 level and compressing the cauda equina. On operation the lesion was found to adhere to the filum terminale as well as the roots, and was totally resected. Pathological examination confirmed the diagnosis of a cavernous malformation (CM). The patient’s pain was quickly resolved after the operation. He remains pain‑free with full recovery of motor function and hypoesthesia in the sixth postoperative month.
Conclusion: CMs of the cauda equina are extremely rare lesions that may present with lower back pain and sciatica, neurological deficit or subarachnoid haemorrhage. Preoperative differential diagnosis of intradural tumours is not easy in cases without haemorrhage from imaging alone. They can be successfully treated by surgical excision. It is safe to cut the filum terminale or a single root to which the lesion is adherent.
malformations (CMs), or cavernous angiomas, are rare vascular malformations
consisting of closely packed, large, sinusoid-esque
vascular channels without neural or glial elements . These lesions can occur
throughout the central nervous system, but they favour the cerebral zone .
Spinal variants are most frequently found in the vertebral body [3,4].
Approximately 3% of spinal haemangiomas are intradural, and these are usually
intramedullary. Only rare cases present with intradural-extramedullary
involvement . Although most CMs in intradural-extramedullary
space are to be found in the cauda equina , the number of cases is still
very small. To date, there have been only 15 surgically treated cases
reported (Table 1). We describe an unusual case of intradural cauda equina CM adherent
to roots and filum terminale. We discuss its clinical, radiological, and
surgical findings, and follow with a brief review of the pertinent
male patient, presenting with a 1-month
history of lower back pain radiating into the posterior part of the left leg
and a 1‑week history of
exacerbated symptoms accompanied by numbness of left lower extremity, was
admitted to the hospital. The pain was exacerbated on assuming a recumbent
position and worse at night. There was no loss of bladder or bowel sphincter
control, no fever and no onset of severe pain during the course.
Neurological examination revealed tenderness
to percussion over the lumbar spine and tenderness over the sciatic nerve. Left
tactile hypoesthesia on the L3–4 dermatome
was identified, and flaccid left lower paraparesis was present with 3/5 muscle
strength on plantar flexion of the left foot, difficulty in walking on tiptoe
and decreased patellar and Achilles’ reflexes on the same side. Left straight
leg raising test was positive. Rectogenital examination was normal.
Magnetic resonance imaging (MRI) revealed
a 20 × 18mm heterogeneous
enhancing mass obliterating the spinal canal at the L2–3 level
and compressing the cauda equina. The lesion exhibited a mixed signal on
both T1- and T2-weighted
images (Figure 1 a–c).
A bilateral L2 and partial
L3 laminectomy was performed with the tense dura opened under microscopic
magnification. A 20 × 18 × 13mm
dark-bluish mass was identified between the
adherent nerve roots, which had compressed the roots to the right side. After
sharp dissection of the adherence to the roots, we found that the mass remained
closely adherent to the filum terminale, which was encapsulated within the
lesion. The lesion appeared to have arisen from the filum terminale. The mass
was gross totally resected after cutting off the filum terminale but no
requirement to sacrifice any roots emerged. Opening the lesion in vitro,
various old capsular spaces and clotted blood of variable density consistent
with haemorrhage of various ages were observed. Histopathological examination
confirmed a CM with numerous small vessels and large, dilated sinusoidal
spaces lined with a single cell layer. The vascular channels were
immediately adjacent to one another, without intervening nervous tissue (Figure
2). The course of postoperative recovery was uneventful, and the patient’s pain
was immediately resolved following the operation. The patient’s motor strength
returned totally after three days. The patient was discharged after seven days
without pain. He remains pain‑free with full
recovery of motor function, normal sphincter function and stable left L3–4 dermatome
hypoesthesia in the sixth postoperative month.
of the literature
Table 1 provides
a summary of the 15 reported surgically treated cases of cauda equina
CMs, including the present report. The patients are in their third to eighth
decades of life. The ages of the patients are between 20 and 75 years,
with an average of 49.6 years (Table 1). There is a male predominance
(11 male and 5 female). The clinical symptoms were most often the
result of local compression of the nerve roots of the cauda equina.
Accordingly, low back pain with sciatica was the major symptom in twelve cases,
while two cases among them presented with only pain [7,8]. Sensorimotor deficit
was revealed among seven cases [6,9–13],
and three cases had only sensory deficit [14–16]. Five patients had loss of sphincter control [6,9–12]. Subarachnoid haemorrhage was observed in
four of the cases [4,14,17,18]. Two cases of cavernous angioma in the cauda
equina diagnosed on the basis of headache due to hydrocephalus were reported
[11,19]. Intraoperatively, all cases were found to be adherent to the nerve
roots, among which twelve adhered to a single root and four to more roots.
Additionally, one cavernoma was found to have invaded from extradural space and
to be adherent to the vertebral body ; one other lesion originated from
between roots . Adherence of the filum terminale to the lesions was
observed in only two cases , including the present one. Total excision was
achieved in 14 cases with preservation of neural tissue, and incomplete
resection was performed in two cases in which the lesion was tightly adherent
to the spinal cord [7,10]. Twelve cases recovered excellently postoperatively,
while exceptions included three cases with stable sensory deficit [7,9] and one
case with persistent erectile dysfunction .
Cavernous angiomas are vascular malformations composed
of abnormal, dilated and packed vascular sinusoidal channels without interposed
neural tissue or tumour tissue. Thus they have been classified as
a vascular malformation and termed cavernous malformation (CM). The most
common location of these vascular malformations is the supratentorial cerebral
parenchyma . Only 5–16% of CMs have been reported for the spine, usually
located within the vertebral bodies . The cauda equina nerve root is
a more rare location. CM of the cauda equina was first reported by Hirsch
et al in 1965 . The patient presented with pain, SAH, sensorimotor
deficit and sphincter dysfunction, and diagnosis was made through operation and
pathohistological study. Later, Pansini et al described a second case with
operative treatment and pathological diagnosis . Although these vascular
malformations are discovered most frequently because of the widespread use of
MRI, CMs of this location still remain uncommon lesions. A PubMed search
identified just 15 cases, including the present one (Table 1).
Although CMs belong to
one group of occult vascular malformations in terms of embryology and
aetiology, the causation of this lesion was not clear. CMs are well demarcated.
lesions. They may arise from blood vessels of the nerve roots, the inner
surface of the dura mater, and the pial surface of the spinal cord.
Accordingly, close adherence of the lesion to the spinal nerve roots or spinal
cord have been observed during operations. In all of the cases, the cavernoma
was adherent to single or multiple roots, and in three cases, the root was
encapsulated within the lesion. In the current case, we observed a CM
tightly adherent to the filum terminale as well as to multiple roots; we had to
cut off the filum terminale in order to resect the lesion totally. Reviewing
the literature, only one case with adherence to the filum terminale was
reported . The filum terminale is an extension structure of spinal cord
without neural tissue, and it is rare to observe vessels there. Therefore, we
assumed that the two latter CMs most probably originated from vessels near to
the filum terminale. Another possible explanation is that the lesions in these
two cases encased the structure.
deficit and sphincter dysfunction are common presentations for patients with
CMs [6,8,15]. However, they play only a minor role in the differential
diagnosis with other lesions of the cauda equina, such as schwannomas and
meningiomas. We should pay more attention to the five cases with SAH. The
clinical deterioration seen in these patients is thought to be secondary to
repeated haemorrhage within the lesion or subarachnoid spaces. This is the most
dangerous presentation among cases of CM. Given the tendency of these lesions
toward haemorrhage and neurological deterioration, and that acute compressive
injury to neural tissue is usually much more severe than the chronic, complete
surgical resection should be performed promptly. In the present case, although
there was no SAH to be observed clinically and operatively, haemorrhage within
the CM was identified by opening the lesion.
MRI is the imaging
modality of choice for diagnosing CMs [20,21]. The typical MRI features are
those of a well‑defined lesion with mixed signal intensity on both T1- and T2-weighted images. The
mixed signal demonstrates the subacute and chronic haemorrhage within the CM
. CMs are often surrounded by a hypointense ring on T2-weighted images arising
from haemosiderin deposition . Enhancement with Gadolinium is variable .
Spinal myxopapillary ependymomas often present as exophytic masses at the conus
and cauda equina; MRI scans show a homogeneous mass isointense on T1,
hyperintense on T2 and enhancement after gadolinium. Thus it is not very
difficult to distinguish these two entities preoperatively. However, among
those CMs lacking the characteristic surrounding lower signal ring, the
preoperative radiological diagnosis of CM is not easy, because
a heterogeneous lesion signal is also generated by other disorders, such
as spinal intradural tumours, spinal vascular pathologies, and infections.
Spinal angiography does not reveal these lesions . In the present case, we
found a round lesion with heterogeneous signal intensity on T1- and T2-weighted image and
significant enhancement after injection of contrast medium. However, we found
no phenomenon of hemosiderin deposition, probably because the haemorrhage was
only within the CM.
The treatment of choice
is surgical removal. The lesions are usually well demarcated and total excision
has proved possible in most cases, including the present one, even though the
lesions were closely attached to nerve roots. The most frequent surgical
finding is that the lesion adheres to the nerve roots [6,8]. Most of the
surgically treated cases have had excellent results, but in patients with
severe preoperative neurological deficits, such as sphincter dysfunction,
recovery was not complete [9,10]. Total excision was achieved in most of the
cases. Subtotal removal was performed in only two cases in which the cavernoma
had invaded the bone, dura and roots . In most cases, the nerve root was
spared when the lesions were removed, but sometimes this is not possible
because the lesion originates within the nerve root . In this event, even
when it proved necessary to cut the single root, the patient still made
a good recovery. In the present case, the cavernous angioma was adherent
to the filum terminale as well as multiple roots. A similar pattern of
growth is reported in only one other case . We preserved all the adhering
roots but cut the filum terminale because of its tight adherence to the lesion.
That it is safe to cut this structure is demonstrated by the good postoperative
CMs of the cauda equina are
extremely rare. They may initially present as low back pain, neurological
deficits, or as a subarachnoid haemorrhage. The clinical outcome depends
largely on the patient’s preoperative neurological status, and optimal treatment
is surgical resection. In those cases in which total resection proves difficult
because of tight adherence of a single root or the filum terminale to the
lesions, it is safe to sacrifice the filum terminale or a single root in
order to complete removal of the lesion.
Yan Yi, M.D. The First Affiliated Hospital of Chongqing Medical University Chongqing 400016 e‑mail:
Accepted for review: 10. 7. 2009 Accepted for publication: 21. 8. 2009
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