J. Neurol. Neurosurg. Psychiat., 1957, 20, 276.

NEUROLOGICAL MANIFESTATIONS IN HAEMANGIOMAOF THE VERTEBRAE

BY

H. ASKENASY and A. BEHMOARAM

From the Neurosurgical Department, Beilinson Hospital, Petah Tikva, Israel

Haemangioma of the vertebrae is believed to beextremely rare and of little clinical importance,because most often it is detected as an incidentalradiological finding in patients examined for com-plaints thought to be related to other diseases.However, haemangiomata of the vertebrae havebeen found on examination in large necropsy seriesin a much larger proportion than suggested byclinical experience. Topfer (1928), in Schmorl'sInstitute, reported an incidence of haemangioma of119% in a study of 2,154 spinal columns and laterJunghanns (1932) found this lesion in 10% of10,000 necropsies.

Since Perman (1926) emphasized the radiologicalfeatures characteristic of haemangioma of thevertebrae, there has been increasing interest in thislesion, particularly when it was realized that it maybe responsible for a wide variety of symptoms andsigns ranging from tenderness and mild pain overthe diseased vertebrae to complete transversemyelopathy. Furthermore, neurological disturbancesoccur more frequently in association with haeman-gioma of the vertebrae than appears from theliterature, and since we started performing syste-matic radiological examinations in patients referredto our out-patient clinic for back pain we foundhaemangioma of the vertebrae in a comparativelyshort time in another three patients who were notadmitted to hospital.The indications for surgical treatment of patients

with signs of cord compression are still a. matterof dispute. Opinion has been divided ever sinceNattrass and Ramage (1932) and Ferber and Lampe(1942) insisted that radiotherapy only should beused in all cases of vertebral haemangioma. Wetherefore present eight cases of haemangioma of thevertebrae seen during the last few years in theDepartment of Neurosurgery of the BeilinsonHospital.The salient features of these patients are

summarized in Table I. Our patients experiencedvarious back, shoulder, arm, chest, abdominal, and

sciatic pains. Some were thought to have cardiac,joint, or abdominal disease. Others exhibitedneurological disturbances.

The Two Clinical GroupsIn accordance with the manifestations, patients

with symptomatic haemangioma of the vertebraeare classified into two clinical groups: (1) Patientswith symptoms only; (2) patients showing signs ofroot and/or spinal cord compression in additionto their complaints.

Group 1.-Tenderness or pain are the leadingsymptoms of this group, in most cases probablydirectly attributable to irritation by the lesion or theinnervation of the bone itself; however, they mayalso be early manifestations of root involvement.

Tenderness over the diseased vertebra may bespontaneous or elicited on palpation, percussion,or movement. This complaint is far from beingcharacteristic of the lesion but whenever encounteredradiographs of the region should be taken.

Pain may be spontaneous or elicited; it may belocalized or diffuse, continuous, or, more often,remittent; it may be mild or very severe. Sometimesthe pain has a radicular distribution.The symptoms depend on the level of the bone

lesion. Patients with cervico-dorsal lesions maycomplain of stiff neck, suboccipital pain, pain in thearms and in the praecordial region. Most often,however, a manifesting haemangioma of the verte-brae is found in the dorsal spine and then thecommon symptoms are of intercostal neuralgia,abdominal or back pain. Low back and sciaticpain, sacralgia, and pain radiating to the inguinalregion are seen in thoraco-lumbar and lumbarlesions. It is significant that in quite a number ofpatients, whose complaints had been labelled for along time as nephrolithiasis, gastric or duodenalulcer, pancreatitis and cholelithiasis, haemangiomaof the dorsolumbar vertebrae was found on radio-logical examination. Improvement or recovery

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NEUROLOGICAL SIGNS OF VERTEBRAL HAEMANGIOMA

TABLE I

CLASSIFICATION OF EIGHT PATIENTS WITH HAEMANGIOMA OF THENEUROLOGICAL MANIFESTATIONS

VERTEBRAE WITH

X-raySeries Main Clinical Duration Various Clinical Diagnosis

SexAge ~~~~of Diagnoses before and yeo Treatment ResultNo.es Sex Age FeaturesSymptoms Admission Site of graphy

Lesion

Group I: Patients with Symptoms OnlyI M 41 Attacks of low ab- 11 years Nephrolithiasis L, Not done X-ray irradia- Good; free from pain

dominal pain, right Lumbar interverte- tionflank and right in- bral discguinal region

Later, low back pain2 F 48 Attacks of abdominal 10 years Nephrolithiasis Di Negative X-ray irradia- Good; free from pain

pain, later low back Cholelithiasis tionand sciatic pain. Multiple disc lesions L,Gradual aggrava- Prolapsed interverte-tion with remissions bral disc

3 F 45 Attacksofrightflank, 11 years Nephrolithiasis D, Negative X-ray irradia- Unsatisfactory; al-abdominal, right Chronic pancreatitis tion though much im-thoracic pain. Pro- Duodenal ulcer proved, she stilltracted course with Intercostal neuralgia D,o complains of diffuseremissions pain 3 years after

first course of radio-therapy

Group 11: Patients with Signs of Root and Spinal Cord Compression4 M 53 Progressive spastic 12 years Spinal tumour, mye- D3 Negative X-ray irradia- Treatment not yet

paraparesis w i t h lography negative D4 tion for the completedsensory distur- 12 years ago, disc Ds first time afterbances; back and lesions- orthopae- D,2 p r e s e n tright sciatic pain. dic treatment. L, examinationCondition stable for Myocardial infarc- and diagno-the last 8 years tion 8 years ago sis (1956)

Multiple sclerosis5 F 33 Insidious onset with 1I years Multiple sclerosis D6 Partial Laminectomy Satisfactory; no fur-

weakness of right Spinal tumour block X-ray irradia- ther progress ofleg, gradual aggra- Haemangioma of the D, tion neurological disable-vation with remis- vertebrae ment observed aftersions. Since 4 operation. Gaitmonths m a r k e d more stable. Re-paraparesis w i t h ferred for radio-sensory and sphinc- therapy on August 8,ter disturbances 1956

6 F 20 Sudden onset during Neurological distur- D, Complete Laminectomy Excellent; completesleep, paraplegia, bances due to preg- stop X-ray irradia- recoverylevel of loss of sen- nancy. Transverse tionsation and double myelopathy due tosphincter inconti- acute compressionnence of the spinal cord

7 F 65 Insidious onset and 1 j years Lateral sclerosis D, Complete Laminectomy Excellent; completegradual deteriora- stop (removal of recoverytion without remis- subperiostalsions: paraparesis haemangio-and sphincter dis- ma)turbances X-ray irradia-

tion8 M 39 Insidious onset but 5 months Spinal tumour D, Complete Laminectomies Very good: except for

rapid progression, D4 stops at (D,-D6 and some weakness ofspastic paraparesis, D D3 and L,-L,) right foot, no com-sphincter distur- D: Li a X-ray irradia- plaints. Returned tobances, and impo- L, tion physical labourtence

from the pain after irradation of the bone lesionprovides, in these patients, convincing proof thathaemangioma of the vertebrae was the offender.

Case I.-A 48-year-old woman complained for 10 yearsof severe epigastric pain radiating to the inguinal regions,together with nausea and vomiting at their peak. At theonset complaints were localized on the right side only.She had been in hospital and treated for nephrolithiasisfor two months. However, no radiological or laboratoryevidence of renal or ureteral calculi was found. After a

remission of one and a half years she again developedsevere epigastric pain radiating now to both inguinalregions. On admission to hospital again, although thecholangiogram was negative, a tentative diagnosis of

cholelithiasis was made and the patient was treatedaccordingly. She was reported much improved on

discharge from hospital. Until five years before admissionto our department she was not severely ill, but nonethe-less she suffered occasionally mild to moderate discom-fort localized in the region of the xyphoid process andthe epigastrium. Five years before admission she againhad a severe attack of abdominal pain radiating tothe back.Three years ago, on re-examination for similar com-

plaints, spondylo-arthritic degeneration of the dorso-lumbar spine and mild scoliosis were found. Ortho-paedic treatment was instituted. Since then she hadsymptoms only after effort. One month before admissionthe patient was suddenly taken with very severe abdominal

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H. ASKENASY AND A. BEHMOARAMand low back pain, referred along the right sciatic nerve.She was admitted to the Department of Neurosurgery onJanuary 2, 1955, with the provisional diagnosis of multi-ple disc lesions with, possibly, a prolapse in the lumbarregion.On admission, the physical examination showed mild

tenderness on palpation over the low dorsal and upperlumbar spine. The overlying skin appeared normal. Theneurological examination was entirely negative. Thecardiovascular system, the lungs, and abdomen showedno abnormalities. The blood pressure was 140/95 mm.Hg. Routine laboratory investigations were normal. Theblood sedimentation rate was 7/14 mm. in one hour(Westergren). Radiographs of the dorso-lumbar spinedisclosed slight scoliosis and spondylo-arthritis. Theeleventh dorsal and the second lumbar vertebral bodieshad a reticular appearance with the paralleling andhypertrophy of vertical trabeculae pathognomonic forhaemangioma.On lumbar puncture, the cerebrospinal fluid was clear

and normal in every respect. The jugular compressiontest was negative. The patient was discharged onJanuary 10 and referred for x-ray irradiation centred onthe diseased vertebrae. She was seen in the out-patientclinic after the completion of radiotherapy. Untilexamined, for the last time, one year later she had beenentirely free of complaints.

This case illustrates symptomatic haemangiomaof the spine with subjective complaints only.Nephrolithiasis, cholelithiasis, and, finally, spondylo-arthritis with disc prolapse were successivelydiagnosed over a period of 10 years. The course ofthe illness was characterized by remissions but witha general trend towards aggravation. There wasnoticeable improvement after radiotherapy and thepain disappeared.

Group 2.-The second group comprises patientsexhibiting symptoms and signs of root or spinalcord compression or of both. At the onset theremay be only tenderness or pain as in the first group,neurological signs developing later with the growthof the lesion. However, in most patients of thisgroup, the onset of motor weakness of the legs withparaesthesiae is insidious, and, later on, ascendingneurological disturbances develop. The lattermay be gradual and slow or dramatic. Sphincterdisturbances and impotence are usually seen in moreadvanced stages of cord compression but they mayappear early. Predominant involvement of thelateral and, less frequently, of the dorsal columnsof the spinal cord is characteristic. Such a distribu-tion of cord lesions does not depend solely on directcompression but is probably due to stress forcesexerted on the lateral columns of the cord resultingfrom stretching of the anchoring dentate ligamentsby the backward pressure on the cord (Kahn, 1947).In some cases complete transverse myelopathy

develops with paraplegia and double sphincterincontinence.

Case 2.-A 20-year-old woman was admitted to theNeurosurgical Department of the Beilinson Hospital onAugust 3, 1951, with the diagnosis of tumour of thespinal cord.She had been entirely healthy until July 20, 1951, when

she awoke during the night with lancinating pain anddiscovered that both her legs were paralysed. Fourdays later (July 24, 1951) she was delivered of a normalchild. When admitted to the medical department ofanother hospital (July 29, 1951), flaccid paraplegia,double sphincter incontinence, and anaesthesia from theD12 dermatome downwards were found. A lumbarpuncture was done and complete blockage of the sub-arachnoid space was found on the jugular compressiontest. The Pandy reaction was positive, cells 10/3, protein150 mg. %. Radiographs of the spine had not been taken.On admission to our department (August 3, 1951), the

examination showed neurological signs of transversemyelopathy at the level D12 as detailed above. Thegeneral examination was negative. The blood pressure

FIG. 1.-Antero-posterior view of the dorsal spine, showing thenet-like reticular aspect, with hypertrophy of vertical trabeculaeof DI,, characteristic of haemangioma of the vertebrae. Theconcave borders of the vertebral body are lost; the pedicles arehardly recognizable.

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FIG. 2.-Lateral view of the spine, showing vertical trabeculation ofthe body, its posterior border bulging into the spinal canal; thepedicles and arches are involved in the lesion.

was 100/70 mm. Hg. The blood sedimentation rate was24/75 mm. in one hour. Other routine laboratoryexaminations were normal.

X-ray films of the dorsal spine showed a reticularappearance with gross trabeculation and expansion of thecontours of the eleventh dorsal vertebra, typical ofhaemangioma (Fig. 1). The pedicles and the laminaewere involved by the lesion as seen on lateral views(Fig. 2). There was a complete stop at the level of thebone lesion on myelography (August 4, 1951).At operation on the same day (laminectomy D0O-D12),

there was profuse bleeding on removing the spinousprocesses and the arches. Bleeding was controlled withbeeswax. On completion of the laminectomy, the cord,which was slightly humped over the bulging posteriorsurface of the vertebra, recovered normal pulsation.Histological examination of the removed bone showed ahaemangioma of the vertebra (Prof. J. Casper).The post-operative course was uneventful. The patient

was ambulatory on the thirteenth day after operation(August 18, 1951); however, she needed support in dailywalking practice. She was referred for radiotherapy onSeptember 9, 1951.The patient came periodically to the out-patient clinic;

good progress was noted and when seen for the last time

in 1953 she was leading a normal life and the neuro-logical examination was negative.

In this case of vertebral haemangioma in apregnant woman, signs of transverse myelopathydeveloped suddenly four days before delivery.

Massive neurological disturbances of suddenonset are certainly an unusual development inhaemangioma of the vertebrae. Collapse of thevertebral body due to destruction by haemangiomais rare (Bell, 1955).

Neurological signs progressed slowly in reportedcases with collapse of the vertebral body due tohaemangioma: over a period of 16 years in Muth-man's case (quoted by Bell, 1955), eight years inSandahl's (quoted by Bell, 1955), one year in that re-ported by Globus and Doshay (1929), and more thansix months in Bell's (1955) patient. But if the richvascularity of the lesion is borne in mind the opera-tive mechanisms of acute cord compression in hae-mangioma of the vertebrae becomes intelligible.Vascular lesions, and in particular those located with-in the spinal canal or protruding into it, are boundto produce severe disturbances when they suddenlyincrease in size. Moreover, it is known that signsbecame aggravated during the night in intraspinalspace-occupying lesions due to physiological venousstasis and congestion of the epidural spinal venousplexus during sleep. Congestion is further enhancedby increased blood volume and raised intra-abdominal pressure in advanced pregnancy, andthese factors probably favoured the sudden onset ofacute cord compression during sleep in Case 2.

Case 3.-A 65-year-old woman was admitted to theDepartment of Neurosurgery on September 22, 1952, foroperation on a suspected tumour of the mid-thoracicspinal cord.Her complaints had started one and a half years before

admission with weakness of the right leg. She tiredeasily and needed support for walking. Six months beforeshe experienced numbness and pricking of the right sole,later involving the right foot and then the other leg inthe same sequence. A month later she noticed that shedid not feel her stockings, she " walked on wool ".Ascending disturbances of sensation developed graduallywith advancing paraparesis. A week before admissionshe complained of severe back pain and difficulty inurination.

Neurological examination on admission showedspastic paraparesis, more pronounced on the right sidewith hyperactive tendon jerks, right ankle clonus, andbilateral Babinski signs. Hypoaesthesia for light touch,cold and heat, hypalgesia, and loss of position sense werefound from the D6 dermatome increasing downwards.Anaesthesia and analgesia of the soles and lateral sidesof both feet were noted and there was hyperalgesia andhyperaesthesia in a zone corresponding to D5-D6.Physical and routine laboratory examinations were

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FIG. 3.-Surgical specimen of subperiostal haemangioma measuring2-21 cm.

negative. The blood pressure was 160/100 mm. Hg.The blood sedimentation rate was 10/16 mm. (Wester-gren). On lumbar puncture, clear cerebrospinalfluid under normal tension was obtained. The Pandyreaction was negative, cells 4/3, protein 28 mg. andglucose 65 mg. per 100 ml. The Mastix and Kahn testswere negative. The jugular compression test showed aslow elevation and a retarded drop of the cerebrospinalfluid column suggestive of incomplete obstruction of thesubarachnoid space.

Straight radiographs of the spine disclosed slighttrabezulation of the fifth vertebral body; its contour,however, was normal. The pedicles and the laminaeappeared normal. A tentative diagnosis of haemangiomaof the vertebrae was proposed but the possibility of acoexistent intraspinal tumour was entertained. Myelo-graphy (October 2, 1952) showed a complete stop at D5.At operation on the same day (laminectomy D4-D6), theexposed spinal cord bulged over a dark, red-bluishepidural mass protruding from the posterior surface ofthe fifth dorsal vertebrae. The tumour was well delineated,friable, and bled profusely when incised. After carefullateral retraction of the cord the tumour was easilyremoved. There was severe bleeding which was con-trolled with bone wax. The mass measured 2 to 2j cm.(Fig. 3). Histologically (Prof. H. Casper) it showed thestructure of a haemangioma.The post-operative course was uneventful. The patient

was discharged from hospital on October 17, 1952, andreferred for radiotherapy. She was seen for the last timein the out-patient clinic in 1954 when the neurologicalexamination was negative, except for slight hypoaesthesiaof the right foot.

The gradual progression of signs, without remis-sion, in this case was suggestive of a spinal tumour.On operation an intraspinal subperiostal haeman-gioma was found. A subperiostal haemangioma ofthe vertebrae may attain relatively large proportionsbefore causing radiologically recognizable alteration

of the vertebral structure. In such cases intraspinaltumour is diagnosed and after positive myelographysurgery is invariably undertaken. Globus andDoshay (1929) and later Karshner, Rand, andReeves (1939), who added another case to thepreviously reported 10 such cases treated surgically,emphasized the most promising results from opera-tion on haemangioma of the vertebrae in thislocation.

Case 4.-A 39-year-old male manual worker wasadmitted to the Department of Neurosurgery onJanuary 13, 1955, with the diagnosis of spinal tumour.He had been entirely well until five months before

admission to hospital, when he experienced numbness ofthe right foot. He massaged his leg because it " feltfrozen ". A few days later dysaesthesiae and weaknessdeveloped in both legs; these complaints became gradu-ally worse and after a week he began to suffer back painand uight sciatic pain. Walking became unsteady andhe could not climb stairs. Two months before admissionhe developed sphincter disturbances with difficulty onurination and he became impotent.

FIG. 4.-Antero-posterior view of the spine. Vertical trabeculationand " expanding " of the D3, D4, Ds, and D8 vertebral bodiesis visible. Note the " spindle-shaped " dense shadow around D8,radiologically suggesting a cold abscess.

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The physical examination showed spastic paraparesiswith hyperactive tendon reflexes, except for an absentright patellar reflex; bilateral Babinski's sign and bilateralankle clonus were noted but the abdominal reflexes werenot diminished and they were equal. Superficial sensationfor light touch, cold and heat, and pin prick wasdiminished from D7 downwards. There was almostcomplete loss of superficial and deep sensation correspond-ing to L2, L3, and L4, and associated muscle weaknessand noticeable wasting of the muscles of the thigh. Thepatient's gait was spastic and ataxic. The generalexamination did not reveal any abnormality. The bloodpressure was 140/70 mm. Hg. Routine laboratoryexaminations gave normal results. The cerebrospinalfluid was slightly xanthochromic, and tension was low.The Pandy test was positive, cells 28/3 (lymphocytes),protein 110 mg. per 100 ml.; Mastix and Kahn tests werenegative. Straight x-ray films showed scoliosis of thedorso-lumbar spine with gross modifications of D3, D4,D5, D8, and L2 vertebrae, typical for haemangioma. Inaddition a spindle-shaped dense shadow around the eighthdorsal vertebra was seen. This finding seemed suggestiveof a cold abscess (Fig. 4). However, the intervertebraldiscs appeared intact and there were no historical, clinical,or radiological data supporting the diagnosis of tuber-culosis. A complete stop at the level of the secondlumbar vertebra was demonstrated on lumbar mye-lography. A lesion at that level could account for theradicular syndrome of the right leg, and possibly for thesphincter and sexual disturbances, but it did not explainthe complete neurological picture, so myelography bythe cisternal route was supplemented. This showedanother stop, in the standing position, at D4.Decompressive laminectomy was performed at both

levels cn the same day: first D2-D6 and then Ll-L3.There was severe bleeding and haemostasis took time.The spinal canal was inspected after complete controlof the bleeding. The cord, which was paler than normaland slightly humped at the upper operation site, recoverednormal pulsation.The post-operative course was uneventful. The neuro-

logical disturbances gradually became less severe. Thelevel of sensory impairment receded to D9. From theeleventh day after operation the patient walked withsupport and did not complain about pain. The controlmyelographic examination performed in view of thelesion at D8, showed freely flowing contrast medium alongthe length of the spinal canal. The patient was discharged16 days after operation for x-ray irradiation centred onboth operative fields and on the eighth dorsal vertebra.He was periodically re-examined in the out-patientclinic. One year after operation his gait was steadywithout support, the reflexes were still active, but hewas free from pain and had resumed his former occu-pation.

In this case of multiple haemangioma of thespinal column (D3, D4, D5, D8, L2), there developedprogressive neurological manifestations of rathershort duration. Bailey and Bucy (1929) andSchlezinger and Ungar (1939) pointed out thatinvolvement of the pedicles and the arches by the4

lesion is responsible for the greater incidence andearlier onset of neurological disturbances. Althoughin the present case the arches and the pedicles of thevertebrae were noticeably involved, neurologicaldisturbances seem to have developed in a ratheradvanced stage of the process. In view of theextensive osseous lesions the manifestations in thiscase were unimpressive when compared to those inCase 7, and in particular, with the dramatic onsetand massive neurological picture in Case 6(Table I).The radiological finding of a spindle-shaped

shadow around the eighth dorsal vertebra is ofinterest. The haemangioma had obviously extendedto the paravertebral soft tissues, thus giving theradiological picture of a cold abscess. Br0beck(1950) reported that a similar finding was en-countered in Sheid and Buckhardt's case. However,as pointed out, the lack of involvement of inter-vertebral joints should help to differentiate thelesion from suspected tuberculosis of the spine.

DiscussionAmong the haemangiomata, those arising in

proximity to the central nervous system, that is,within the cranial cavity or in the spinal canal, areof special importance. It is easy to understand thatsevere disturbances may result from an even rela-tively slight constriction of the lumen of the spinalcanal. Haemangiomata of the skin, the liver, andthe spleen, for instance, may attain enormousproportions without causing serious functionaldamage, while a small haemangioma, either bycompressing the spinal cord or through secondaryvascular disturbances, may bring about seriousneurological disability.Haemangiomata of the vertebrae are benign,

expansive, dark red vascular growths. They aremade up of cavities which may form cavernousbodies when sufficiently large. The cavities are linedwith endothelial cells. The haemangioma does notmetastatize, but with growth it displaces the normalbone marrow, which partially degenerates intofatty tissue. Most often it involves the vertebralbody, but it may also spread to the pedicles and thearch (Cases 4, 6, 8 in Table I). The lesion maydevelop initially in the arch, the pedicle, and thespinous process; it may infiltrate the paravertebralsoft tissues (Case 8); it may finally erupt into thespinal canal developing subperiostally (Case 7).Br0beck (1950) cites the case of Jacobovici in whichthe angioma spread into the soft tissues of the backin the form of a prominent pulsating subcutanousgrowth. In the course of its development thehaemangioma erodes the bony trabeculae of thespongiosa. Additional changes in the cancellous

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bone are produced by ischaemia due to stasis andthrombosis in the large channels and cavities of thevascular lesion. Local ischaemia is known to causeosteolytic and secondary reparatory, osteoblasticactivity in the bone.The structure and pattern of bone depend in part

on its vascularization. As long ago as 1905 Gerb-hardt considered that the architecture of bone islargely determined by its vascular system. Weiden-reich (1923), quoted by Trueta (1956), noticed thatthe lamellae of cancellous bone are always arrangedaround spaces containing vessels and follow theirdirection. Recently Trueta (1956), in research onosteoarthritis, has found suggestive evidence thatrealignment of the trabecular pattern is also deter-mined by the ability of the spongiosa to orientate itstrabeculae along the lines of forces of tension andpressure. It seems, therefore, that the force ofgravitation acting in man, in the erect position, isresponsible for buttressing the vertical trabeculaein haemangioma of the vertebrae, which in their turnare responsible for the pathognomonic " verticaltrabeculation" seen on radiological examination.It is understandable, moreover, that the load-supporting dorsal and, particularly, lumbar, verte-brae undergo more readily these specific modifica-tions. On the other hand, in haemangioma of thecervical vertebrae, where the call for vertical but-tressing is less, as well as in the pedicles, the arches,and the spinous processes of the vertebrae, whosemain function is not to bear weight, the pattern ofrearrangement is manifested on the x-ray plate asthe " soap-bubble effect " or the " sun-burst"trabeculation, radiating from a common centre.The tendency of haemangioma to occur in the

vertebrae more often than in other sites of the bodyis believed to be related to the extensive venousplexus in the mesenchymal tissue of the embryo,which later form the vertebral column. Hanson(1926) pointed out that the frequent occurrence ofcongenital haemangiomatous new growths may beexpected to result from the extremely rich venousplexuses of the embryo. Its remnants in the adultvertebra have been demonstrated in the form ofcanals traversing the vertebral body, visible onradiography during the first two decades of life.Similarly, the rich venous network of the diploe isconsidered a reasonable explanation for the notinfrequent occurrence of haemangioma of the skull(Schlezinger and Ungar, 1939).The majority of the growths were situated in the

dorsal column (Table I). The site of predilectionof the lesion in cases complicated by neurologicaldisturbances was mid- and lower dorsal and in thehigher lumbar regions in this series, whereas Globusand Doshay (1929), Schlezinger and Ungar (1939),

and Ghormley and Adson (1944) pointed to themid-dorsal (D3-D5) location of haemangioma asmore likely to produce neurological manifestations.Lumbar haemangioma of the vertebra is more oftenseen in asymptomatic cases. Involvement of thecervical column seems to be rare. Junghanns (1932)reported an incidence in women of haemangiomaof the spine of 12'5% as compared with 8-9% in men.The age incidence on diagnosis ranges from pubertyto a fairly advanced age, as, for example, 65 yearsin Case 7. Br0beck (1950) cites Zdansky for havingreported the oldest patient, 64 years of age. Althoughthe overall incidence of haemangioma of thevertebrae was found to be below 5% in persons lessthan 30 years old, approximately 50% of the cases,which were accompanied by neurological distur-bances referable to compression myelopathy,occurred in the lower age group (Schlezinger andUngar, 1939). In our material the youngest patientwas aged 20 (Case 6). The average age incidenceof 43 years in the present series places them in theolder age group, in disagreement with the acceptedbelief that severe neurological complications occurmostly in younger patients.Of great diagnostic value is the radiological

examination of the clinically suspected region of thespine. However, x-ray examination of the entireskeletal system is highly recommended in such cases.Perman (1926), Bailey and Bucy (1929), and Bucyand Capp (1930) described the radiological featuresof haemangiomata of the vertebrae, characterizedby reduction of bone density between denserhypertrophied vertical trabeculae. Perman (1926)maintained that the radiological picture is patho-gnomonic of the disease. However, infrequently theradiological findings may raise diagnostic difficulties.In Case 8, for example, the spindle-shaped delineatedshadow, due to the spread of the lesion in theparavertebral tissues, was similar to the picture seenin Pott's disease and suggested the presence of aparavertebral cold abscess. Osseous lesions en-countered in malignant tumour metastases, giantcell tumour, osseous lymphogranulomatosis, myelo-matosis, Kiimmel's and von Recklinghausen'sdiseases may lead to diagnostic difficulties.

It should be pointed out that two cases from thishospital of haemangioma of the vertebra with symp-toms only were treated erroneously for perforatedduodenal ulcer and for chronic pancreatitis. In bothpatients haemangioma of the vertebrae was laterfound incidentally on abdominal radiologicalexamination.The neurological manifestations due to compres-

sion myelopathy caused by haemangioma of thevertebrae are comparable, generally with thoseproduced by any other type of extradural tumour,

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NEUROLOGICAL SIGNS OF VERTEBRAL HAEMANGIOMA

although Ghormley and Adson (1944) maintainedthat they " probably resemble more the symptomsproduced by a metastatic lesion of the spinal cord ".Bilateral neurological signs were reported as aregular feature resulting from the almost symmetricalinvolvement of the cord by the lesion (Schlezingerand Ungar, 1939). This is not verified in our series.The neurological manifestations in the early stageof the disease were invariably unilateral except inthe patient with a sudden onset of paraplegia(Case 6).A clinical course with remission of neurological

manifestations is characteristic for vascular lesions.Alpers and Pancoast (1932) report a case of hae-mangioma with varying symptomatology in whichsubarachnoidal block, demonstrated at first byQueckenstedt's test, could not be confirmed on asecond examination. This would suggest the possi-bility that there may be a certain amount ofexpansionof the haemangioma due to filling of the blood spaces.Such an explanation seems acceptable in the caseof a subperiostal growth of the lesion. However,considering the rigid bony structure confining thelesion when it is within the vertebral body, variationsin symptoms during the course of the disease aremore likely to be related to venous stasis andcongestion of the epidural venous plexus, and sub-sequent disturbance in cerebrospinal fluid circulation.The problem of the correct treatment of patients

exhibiting neurological disturbances due to haeman-gioma of the vertebrae is still unsettled. Bailey andBucy in 1929 clearly stated that the only treatmentof any avail in this disease when there are signs ofcompression of the spinal cord is laminectomy. Theyadvised radiation of the lesion only after decom-pression of the cord. They pointed out that theoperation is difficult and may be fatal on accountof uncontrollable bleeding.

In view of the danger involved in surgery and thereported high mortality rate (20-25 %), Nattrassand Ramage (1932), Ferber and Lampe (1942),Lindqvist (1951), and others have employed deepx-ray therapy as the " primary and sole method oftreatment ". Although the value of radiotherapyhas been conclusively demonstrated in haeman-gioma of the vertebrae, one cannot easily be recon-ciled with such an attitude when the cord is com-pressed. The use of radiotherapy alone in such casesseems not only inadequate, as control radiographsshow little or no demonstrable improvement inthe bony lesion, but it is hazardous to the patientbecause the cord may be irreparably damaged beforeany beneficial effect from irradiation can be antici-pated.

In cases where the haemangioma has not spread

to the arches and the spinous process the operativeprocedure itself does not carry undue risks. Thepossibility of such a spread should be judiciouslyevaluated before operation. Experience has shownthat with modem anti-shock and blood replacementtherapy, the surgery of haemangioma of the verte-brae is relatively safe. Decompressive laminectomywas carried out by us five times in four patients,one of whom (Case 8) was operated on at two sites,dorsal and lumbar, at the same session without illeffect. There was no mortality and none of thepatients were shocked. The post-operative coursewas uneventful and the patients were ambulatoryin the third week after operation. The end-resultswere good (Table I).

In conclusion we favour the following attitudetowards patients with symptomatic haemangioma ofthe vertebrae:

(1) Patients with only subjective complaintsshould be irradiated.

(2) Patients exhibiting neurological signs only ofroot compression should be irradiated.

(3) Patients exhibiting progressive neurologicalsigns of cord compression should undergo mye-lography. In the presence of a block of the sub-arachnoid space, immediate decompressive laminec-tomy should be carried out, supplemented byradiotherapy.

SummaryEight cases of haemangioma of the vertebrae with

neurological manifestations were observed in aperiod of six years. Four of these are presented indetail. The cases are classified into two mainclinical groups: those with symptoms only and thosewith objective neurological signs.

In the first group, root involvement is heldresponsible for a variety of complaints which oftensimulated acute or chronic medical and surgicalconditions. In these patients haemangioma of thevertebrae was incidentally discovered on radio-logical examination. Improvement after radiotherapysupported the diagnosis of symptomatic haeman-gioma of the vertebrae.The second group of patients exhibited objective

neurological signs of root lesion or of cord com-pression. Five examples of spinal cord compressiondue to haemangioma of the vertebrae are given.Four of these were operated on after myelographicconfirmation.The authors believe that the neurological com-

plications are not due solely to compression butare enhanced by congestion, bleeding, thrombosis

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H. ASKENASY AND A. BEHMOARAM

within the lesion in combination with stasis ofblood flow in the epidural venous plexus, andsecondary disturbances of the cerebrospinal fluidcirculation. Aggravation of the clinical manifesta-tions may be favoured by various factors. Endocrinedisturbances and elevated intra-abdominal pressurein advanced pregnancy, together with physiologicalnocturnal venous stasis, are believed to have con-

tributed in one case to the sudden onset ofparaplegia.The authors rally to the opinion that operation is

the treatment of choice in cases of haemangiomaof the vertebrae with myelographic confirmation ofcompression of the cord. Decompressive laminec-tomy should be supplemented by radiotherapy.Surgery in the hands of the authors carried no

mortality.

REFERENCESAlpers, B. J., and Pancoast, H. K. (1932). Surg. Gynec. Obstet.,

55, 374.Bailey, P., and Bucy, P. C. (1929). J. Amer. med. Ass., 92, 1748.Bell, R. L. (1955). J. Neurosurg., 12, 570.Brobeck, 0. (1950). Acta. radiol. (Stockh.), 34, 235.Bucy, P. C., and Capp, C. S. (1930). Amer. J. Roentgenol., 23, 1.Ferber, L., and Lampe, J. (1942). Arch. Neurol. Psychiat. (Chicago),

47, 19.Ghormley, R. K., and Adson, A. W. (1944). J. Bone J. Surg., 23, 887.Globus, J. H., and Doshay, L. J. (1929). Surg. Gynec. Obstet.,

48, 345.Hanson, R. (1926). Arch. chir. scand., 60, 309.Junghanns, H. (1932). Arch. klin. Chir., 169, 321.Kahn, E. A. (1947). J. Neurosurg., 4, 191.Karshner, R. G., Rand, C. W., and Reeves, D. L. (1939). Arch.

Surg. (Chicago), 39, 942.Lindqvist, I. (1951). Acta radiol. (Stockh.), 35, 400.Nattrass, F. J., and Ramage, D. (1932). J. Neurol. Psychopath.,

12, 231.Perman, E. (1926). Acta chir. Scand., 61, 91.Schlezinger, N. S., and Ungar, H. (1939). Amer. J. Roentgenol.,

42, 192.Topfer, D. (1928). Frankfurt Z. Path., 36, 337. Quoted from

Schlezinger and Ungar.Trueta, J. (1956). Lancet, 1, 585.

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guest. Protected by copyright.

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eurosurg Psychiatry: first published as 10.1136/jnnp.20.4.276 on 1 N

ovember 1957. D

ownloaded from