Cauda Equina Cavernous Angioma

Abstract

Cavernous angiomas are rare vascular lesions, most occurring supratentorially, with spinal cavernomas being even rarer. They have a varied magnetic resonance imaging (MRI) appearance which is usually not diagnostic. A 42-year-old man presented with progressive low backache for the past 5 years and was found to have a mild right ankle weakness. MRI showed an intradural extramedullary lesion at the L1 vertebral body level. Intraoperatively, a dark reddish-blue mulberry-like lesion was found attached to a nerve root that had to be sacrificed during the excision. Histopathology confirmed that it was a cavernous angioma and the patient had no added deficits postoperatively. Cauda equina cavernomas are extremely rare and hence not thought of as a differential preoperatively. They are relatively simple to remove but will mostly need the sacrifice of the adherent nerve root. Most cases do well postoperatively with stable deficits and some improving.

Keywords

cauda equina - spinal cavernoma - vascular lesions

Introduction

Cavernous angiomas are a rare vascular lesion in the central nervous system (CNS) with most occurring supratentorially. They are even rarer in the spine with most occurring in the vertebrae or extradurally.[1] Cauda equina cavernomas are the rarest with less than 50 case reports. They usually present with cauda equina syndrome and have no typical appearance on magnetic resonance imaging (MRI). We present our case of a cavernoma in the cauda equina followed by a review of the literature.

Case Report

History and Examination

A 42-year-old man presented with dull aching low back pain for the past 5 years which was progressive, aggravated at night, when lying down (more when turned to the left) with relief on standing or walking. He had difficulty standing on his toes and had a history of slippage of footwear on the right. On examination, he had weakness in plantar flexion of the right foot and dorsiflexion of the right large toe with no sensory deficits. Laboratory findings were unremarkable. MRI ([Fig. 1]) revealed an intradural extramedullary lesion at the L1 vertebral body level, 2 cm below the conus which was T1 isointense, T2 heterogeneous with mild homogenous enhancement. Under the presumption of being a schwannoma, the patient was taken up for surgical excision of the lesion.

Fig. 1 (A) T2 coronal showing a heterogeneous lesion. (B) T2 sagittal showing a hypointense intradural lesion at the L1 vertebral body level. (C) Homogeneous mildly enhancing lesion is seen.

Surgery

L1 and L2 laminectomy and midline durotomy were done. A 1.9 × 1.2 × 1.2 cm multilobulated, firm, vascular lesion with mulberry-like appearance was identified arising from the nerve root ([Fig. 2]). Extracapsular delineation of the lesion was done circumferentially. The nerve root cranial and caudal to the lesion was coagulated and cut on either side and the lesion was removed in toto. Water-tight dural closure was done. The patient had a smooth postoperative period with significant resolution of preoperative pain with no fresh deficits.

Fig. 2 (A) Intraoperative photograph after opening the arachnoid. (B) Lesion after en masse excision.

Histopathology

Examination under the microscope ([Fig. 3]) showed dilated cavernous blood vessels with organizing thrombi of varying stages surrounded by fibrous stroma and focal areas of calcification which was consistent with cavernous hemangioma.

Fig. 3 (A, B) Microscopic photograph (×100) of hematoxylin and eosin–stained slide of the lesion showing abnormally dilated vascular channels consistent with cavernous angioma.

Discussion

CNS cavernomas are a rare entity with an overall frequency of 0.41%[2] and spinal cavernomas being even rarer; 6 to 7% of spinal tumors are vascular lesions, of which 5 to 12% are cavernomas.[3] There are less than 50 case reports of cauda equina cavernomas ([Table 1]). Like cavernomas in other locations, in the cauda equina, there is a male predominance (1.7:1). The median age of diagnosis is 46 years with the youngest case seen in a 13- year-old girl.[4]

Table 1 Review of all previous case reports of cauda equina cavernomas with their demographic details, presentation, and MRI findings

Author	Year	Age	Sex	Level	Symptom	T1	T2	Contrast
Hirsch et al	1965	20	M	L2–3	SAH, pain, weakness, anesthesia, sphincter dysfunction	–	–	–
Pansini and Lo	1966	46	M	L2	Pain, anesthesia, sphincter/erectile dysfunction	–	–	–
Ueda et al	1987	28	M	L1–2	SAH, pain	Hyper	–	–
Pagni et al	1990	46	M	D12–L1	Low back pain + sciatica	–	–	–
Ramos et al	1990	67	F	L3	Sphincter dysfunction + difficulty in walking	–	–	–
Acciarri et al	1993	31	M	L2	Radicular pain	–	–	–
Bruni et al	1993	28	M	L2	SAH, headache, vomiting, neck stiffness	Hyper	–	–
Cervoni et al	1995	26	F	L1–2	SAH + sudden headache, lumbar pain	–	–	Enhancing
		32	M	L5	Left sciatica + left foot drop + left L5 hypoesthesia	–	–	Enhancing
Makino	1995	67	M	L2	Headache, unsteady gait	Hyper	Iso	Slightly enhancing
Moreno	1995	63	M		Low backache, sphincter dysfunction, sensory deficits, knee, and ankle reflexes diminished	Hypo	Hyper	–
Rao et al	1997	60	M	L1–3	Low backache, paresthesia, paraparesis	–	–	–
		65	F	L3–5	Sudden paraparesis after trivial injury, sensory deficit below L3	–	–	–
		35	F	D11–12	Sudden-onset paraparesis, sensory deficit below L3	–	–	–
		32	M	L2–3	Left sciatica	Hyper	Hypo	Enhancing
Mastronardi et al	1997	41	M	L5	Low backache, left ankle jerk diminished	Hyper slightly	Isointense
Duke et al	1998	49	F	L4	Low back pain	Hyper	Hyper	Not enhancing
Falavigna et al	2004	44	F	L4	Pain, anesthesia, sphincter/erectile dysfunction	–	–	Hetero
Crispino	2005	65	M	T12–L1	Due to coexisting cervical lesion	Iso	Hyper	Homogeneous
Miyake et al	2007	18	M	L1	Low back pain radiating to bilateral lower limbs	Iso	Hyper	Nonenhancing
Cecchi et al	2007	75	F	L3–4	Painful paresthesias	Iso–hypo	Iso–hypo	Heterogeneous
Caroli et al	2007	71	M		Low back pain and left leg radiation, left L4 hypoesthesia	Hyper	Hypo	Nonenhancing
Er	2007	67	M	T11–L1	Lower limb paresthesia, sphincter and bowel dysfunction, difficulty walking	Hyper	Hypo	Nonenhancing
Chun	2007	74	F	L3	Neurogenic claudication and lumbar tenderness	–	Hetero	Enhancing
Jin et al	2011	55	M	D12–L2	Headache, dizziness, SNHL	Iso	Hyper	No CE
Nie	2012	57	F	L1	Acute low back pain, lower limb weakness, sphincter dysfunction	High	Iso	Hetero
Popescu	2013	60	F	L4	Low back pain, bilateral radiculopathy, bilateral L4–S1 hypoesthesia	–	Hyper	–
Takeshima	2013	44	M	L2–3	Low back pain, bilateral sciatica, neck pain	Iso	Hypo	–
Yang et al	2013	42	M	L3	Low back pain, weakness	Iso–hypo	Iso–hyper	Mild
		31	F	L2–3	SAH, headache, right leg pain	Iso–hypo	Iso–hyper	Heterogeneously
		28	F	L1–2	SAH, bilateral leg pain	Iso–hypo	Iso–hyper	Heterogeneously
		27	M	L2	Low back pain, radiation to right, urinary disturbance	Iso–hyper	Iso–hypo	Mild
		23	M	L1–2	SAH, headache, bilateral leg pain, low backache	Iso–hyper	Iso–hypo	Heterogeneously
		49	F	L2–3	Right leg pain, numbness, urinary disturbance	Iso–hypo	Iso–hyper	Heterogeneously
		33	M	L2	Low back pain, right leg pain, numbness	Iso–hyper	Iso–hypo	Heterogeneously
		51	F	L1–2	Low back pain, bilateral leg pain, and numbness	Iso	Iso–hyper	Mild
		42	F	L2–3	Bilateral leg pain and numbness	Iso	Iso–hyper	Mild
		59	M	L3	Low back pain, left leg pain, and weakness	Iso to hyper	Iso to hypo	Mild
Mataliotakis	2014	79	M	L2–3	Back pain, neurogenic claudication, paresthesia, both lower limb weakness	Hetero	Iso	–
Kumar et al	2016	21	M	L3–4	Low backache, ascending sensory loss, weakness	Hypo	–	Enhancing
Rizzi et al	2016	49	F	L2	Low back pain, bilateral sciatica, paraparesis	Hyper	Hypo	Enhancing
Yaltirik et al	2016	13	F	L1	Back pain, right side EHL, and dorsiflexion weakness	Hyper	Hyper	Enhancing
Katoh et al	2017	36	M	L1	Sensorineural hearing loss, headache, and hydrocephalus	–	Iso	Nonenhancing
Golnari	2017	60	M	L2	Back pain radiating to left leg, headache		Hypo	Mild CE
Apotolakis et al	2018	77	M	L3	Low back pain	NA	NA	NA
Hughes et al	2021	50	M	L4	Bilateral leg weakness, bladder, and bowel dysfunction	Iso	Hyper	Enhancing

Abbreviations: CE, contrast-enhanced; EHL, extensor hallucis longus; MRI, magnetic resonance imaging; NA, not available; SAH, subarachnoid hemorrhage; SNHL, sensorineural hearing loss.

The most common presentation was with low back pain with or without radicular lower limb pain with some also presenting with lower limb weakness, paresthesias, or bowel/bladder dysfunction. Yang et al[5] pointed out the different patterns in the presentation of the patients with some experiencing a slow progressive course, while others had acute onset of symptoms. This may be due to the different pathophysiologies, as repeated bleeds, endothelial proliferation, and neoangiogenesis leading to slow progression while a large subarachnoid hemorrhage or an acute swelling of the lesion due to thrombosis in the acute setting. Subarachnoid hemorrhage was seen in 7 of the 46 patients who we reviewed.

There are two reports of patients presenting with headache and an unsteady gait who were found to have hydrocephalus which resolved after the removal of the lesion. Ramos et al[6] stated that the hydrocephalus was due to hyperviscosity of the cerebrospinal fluid (CSF) which must be either due to increased proteins in the CSF, slowed flow, or inflammation impeding its absorption. Jin et al[7] showed that headache resolved after lesion excision in a patient with superficial siderosis, impressing on the fact that the headache was due to the lesion.

MRI was the modality of choice in most case reports with no typical appearance and a wide range of differences as seen in [Table 1]. The presence of a hemosiderin ring may give a clue to the nature of the pathology but may not be specific since the blood products are degraded faster outside the blood–brain barrier. It should be known that ependymomas can also cause subarachnoid bleeds.[8] Contrast enhancement is also variable which may be related to which tissue the lesion arises from. MRI does not show flow voids and neither does angiography reveal cavernomas due to either thrombosis or poor flow.[9] Previously, myelogram used to be done which would show the location of a lesion but not its nature. In patients who have multiple bleeds, there may be features of superficial siderosis that can be easily missed.

Microscopically, there are large, dilated hyalinized vascular channels that often show thrombosis, perivascular hemosiderin deposition, and calcifications. Intralesional bleeds lead to the formation of cystic spaces that can be so severe that the lesion mimics a neurenteric cyst.[10] Five case reports showed nerve fibers within the lesion. Cavernomas of the spine can arise not only in the bone, extradural space, and intradural space but can also arise from the pia, be attached to the nerve root, within the nerve root, periradicular vessel, ligamentum denticulatum, or filum.

Bruni et al[11] pointed out that the lesions become symptomatic due to the dynamic mobility of the lumbar spine, with the squashing of the lesion within the canal. Enhi and Love[12] stated that these types of lesions develop after traumatic or other stimuli, while Willis et al considered it a hamartoma thinking of it as a congenital lesion.

All lesions in the case reports reviewed were symptomatic and were surgically excised en masse. The lesion was usually adherent to one nerve root as in our case necessitating the sacrifice of that root. Postoperatively, all had an excellent outcome with most improving after surgery and a few with stable deficits.

Conclusion

Cavernomas are very rare in the cauda equina and require a high index of suspicion to diagnose preoperatively. The exact pathophysiology of these lesions is yet to be elicited. After removal, the outcomes of these lesions are generally good.

Conflict of Interest

None declared.

• References

• 1 Popescu M, Titus Grigorean V, Julieta Sinescu C. et al. Cauda equina intradural extramedullary cavernous haemangioma: case report and review of the literature. Neurol Med Chir (Tokyo) 2013; 53 (12) 890-895
• 2 Flemming KD, Graff-Radford J, Aakre J. et al. Population-based prevalence of cerebral cavernous malformations in older adults: Mayo Clinic study of aging. JAMA Neurol 2017; 74 (07) 801-805
• 3 Ardeshiri A, Özkan N, Chen B. et al. A retrospective and consecutive analysis of the epidemiology and management of spinal cavernomas over the last 20 years in a single center. Neurosurg Rev 2016; 39 (02) 269-276 , discussion 276
• 4 Yaltirik K, Özdoğan S, Doğan Ekici I, Atalay B. Cauda equina cavernous hemangioma: very rare pediatric case. Childs Nerv Syst 2016; 32 (12) 2289-2291
• 5 Yang T, Wu L, Yang C, Deng X, Xu Y. Cavernous angiomas of the cauda equina: clinical characteristics and surgical outcomes. Neurol Med Chir (Tokyo) 2014; 54 (11) 914-923
• 6 Ramos Jr F, de Toffol B, Aesch B, Jan M. Hydrocephalus and cavernoma of the cauda equina. Neurosurg 1990; 27 (01) 139-142 . PMID: 2377272
• 7 Jin YJ, Chung SB, Kim KJ, Kim HJ. Spinal intradural extramedullary cavernoma presenting with intracranial superficial hemosiderosis. J Korean Neurosurg Soc 2011; 49 (06) 377-380 . Epub 2011 Jun 30. PMID: 21887400; PMCID: PMC3158485
• 8 Turgut M, Ak H, Özkara E. Filum terminale ependymoma with intratumoral and spinal subarachnoid hemorrhage. Surg Neurol 2006; 66 (06) 646-647
• 9 Cervoni L, Celli P, Gagliardi FM. Cavernous angioma of the cauda equina: report of two cases and review of the literature. Neurosurg Rev 1995; 18 (04) 281-283
• 10 Yang T, Wu L, Yang C, Xu Y. Cystic cavernous angioma of the cauda equina mimicking neurenteric cyst. Neurol India 2014; 62 (03) 339-340
• 11 Bruni P, Massari A, Greco R, Hernandez R, Oddi G, Chiappetta F. Subarachnoid hemorrhage from cavernous angioma of the cauda equina: case report. Surg Neurol 1994; 41 (03) 226-229
• 12 Ehni G, Love JG. Intraspinal lipomas: report of cases; review of the literature, and clinical and pathologic study. Arch NeurPsych 1945; 53 (01) 1-28

Publication History

Article published online:
27 September 2024