HORNER'S SYNDROME DUE TO AN OSTEOCHONDROMA OF ...

SIMPSON: HORNER 'S SYNDROME [ Canad. M. A. J.L Aug. 1948, vol. 59

HORNER'S SYNDROME DUE TO ANOSTEOCHONDROMA OF THE FIRST RIB*

J. F. Simpson, B.A., M.D., M.SC.

Toronto, Ont.

H ORNER 'S syndrome, a relatively uncommonclinical entity, is caused by a lesion involv-

ing the cervical sympathetic chain or thesympathetic pathways in the brain stem or

spinal cord. The syndrome is characterized byhomolateral ptosis, miosis, and enophthalmus.Less frequent findings are vasodilatation andanhidrosis. Other manifestations mentionedare injection of the conjunctiva, initraoeularhypotonia and diminutioni in the size of thenares. Stine and Draper' consider that de-

pigmentation of the iris and possibly cataractmay subsequently develop.The ptosis, which is only partial, (pseudop-

tosis) is due to paralysis of Muller's musele,the involuntary component of the levatorpalpebrwe superioris. The constricted pupil,which as a rule does not react to light, is dueto paralysis of the dilator pllpillw. Enoph-thalmus, caused by paralysis of the orbicu-laris oculi is not always appareint but accentu-ates the narrowing of the palpebral fissure andthe ptosis and allows the lower lid to rise a little.Although this syndronme is cominmoinly asso-

ciated with the name of Johann FriedrichHorner, who described it in 1859, it was previ-ously described by Edward Selleck Hare in 1838and therefore might more properly be calledHare's svndrome. Hare,2 while house surgeon

of the Stafford CouInty General Infirmary, de-scribed eye changes in a case of a small tumourof the inferior triangular space. This tumourlay on the 3rd and 4th nerves of the plexusand also inavolved the symnpathetic and itslowest cervical ganglion. Though not familiarwith previous experimenits, he noted the de-struction of sympathetic tissue amid eye changesbut did not comment on their relationship.Fulton3 feels it is reasonable to associate thissyndrome with Horner's5 name but Horner didnot discuss the etiology of his own case and

neglected to correlate his clinical findimigs tothose cases previously described by physiolo-gists and clinicians.According to Fulton, de Petit, a French

surgeon, first recorded in 1727, the relation-

* Fronm the Division of Tuberculosis Prevention,Onl ario Department of Health, Toronto.

ship, in dogs, between the cervical sympatheticchain and the eye. Subsequent workers con-

firmed this observation. In 1839, John Reid,an Edinburgh physiologist, drew attention toHare's work, and concluded that eye changesfollowiing injury to the cervical sympatheticnerve occurred immediately and were perma-nent. Further observations concerning theeffect of the sympathetic nerve OIn the pupilwere made by Biffi (1846) and Ruete (1847).In 1851 Budge and Waller showed that thesympathetic fibres arose from dorsal segmentsI and II of the spinal cord and that section or

injury of the fibres at their origin had the samiie

effect as section of the nerve in the neck.Claude Bernard in 1852 made the first completestudy of section and stimulation of the cervicalsyiipathetic.

The basic facts having been discovered, mostof the literature since 1869 has been mainlyconcerined with elaboration of details.

ANATOMY AND PHYSIOLOGYRecent literature indicates that the chief centre for

the autonomic, nervous system lies in the floor of thethird ventricle, from which the fibres pass downwardby the posterior longitudinal bundle into the medullathrough the ciliospinal centre and out the first few dorsalsegments.The sympathetic fibres running to the head and neck,

heart and upper extremity, pass upward thrIough thestellate ganglion. This ganglioin, which is actually acombination of the inferior cervical and first thor.acicsympathetic ganglion, lies ainterior to the hcad of thefirst rib and extends upward to the angle between thevertebral and inferior thyroid arteries and the subelavianartery. The lower pole of the stellate ganglion joinsthe second thoracic ganglion which lies in firont of themedial extremity of the first iintercostal space and ex-tends to the upper border of the second ril). Cephalad,the sympathetic chain crosses the inferior thyroid arteryopposite the sixth cervical vertebra, and at about thislevel, joins the middle cervical ganglion. Fibres fromthe latter ganlglion ascend to the superior cervical gang-

lion, which is placed opposite the second and third cervi-cal vertebrae.

Segmental branches from the stellate ganglion passto the sympathetic trunk as white rami, or preganglionicfibres, and proceed cranially. After relay in the variousganglia they are distributed as grey rami or postgangli-onic fibres to the whole of the corresponding side of thehead, neck and upper extremity. They supply the bloodvessels, salivary, mucous, sweat and sebaceous glandsand the erectores piloruin. Visceral branches from thestellate nucleus are derived from the ciliospinal centrewhich is situated in the grey matter of the C. VIII seg-

ment. Preganglionic fibres to the eye traverse thestellate ganglion, synapse with cells of the superior cervi-cal ganglion and the postganglionic fibres travel withthe internal carotid artery and so reach the cavernous

plexus. They supply the extrinsic unstriped muscle ofthe orbit (Muller's muscle) and the dilator fibres of thepupil.

Interiuption of these sympathetic fibres, whether pre-

ganglionic or postganglionic, results in Horner 'ssyndrome.

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ETIOLOGYAnatomically, Horner's syndromie m,ay origi-

nate at the following levels of the sympatheticchain: (a) central, including the ponis andmedulla, (b) spinal, including the spinal roots,and (c) peripheral. Certain lesions, such as

infiltrating intrathoracie or spinal cord tu-mours, injuries, etc., may be both peripheraland spinal and other lesions, like syringo-myelia, may be both central and spinal. Insome cases, no apparent cause may be found.Central.-Any lesion (e.g., brain tumour) of

the lower portion of the medulla may invadethe oculopupillary centre aned cause Horner'ssynedrome. The syndrome may also be pro-

duced by a lesion of the intramnedullary sympa-

thetic pathway. Not uneommonily reported isthe "'lateral medullary syndrome" usually due

to occlusion of the medial braneh of the pos-

terior inferior cerebellar artery. The lattersyndrome, according to Stead et al.,4 is notalways typical but may be accompaniied by a

homolateral Horner 's syndrome. Alpers5 statesthat thrombosis of the vertebral artery may

produce a similar clinical picture but Horner'ssyndrome is always present.

Spinal.-Hornier's syndrome may be due tolesions of the spinal cord or roots. It is not a

cardinal sign of syringomyelia but is the mostimportant of the less frequent signs of this dis-ease and is due to involvement of the cilio-spinal centre. Other spinal causes are, trau-matic injuries to the spine or roots, extra- or

intra-medullary tumours and inflammatory or

degenerative lesions of the ciliospinal centre.Peripheral.-Peripheral lesions involving the

cervical sympathetic trunk are the most com-

mon cause of Horner's syndrome. Alexander6

states that the most frequent operative nerve

accident that results from exposing and resect-ing the main phrenic nerve and the accessory

phrenic roots is Horner's syndrome. It is due

to the sympathetic trunk "being infiltrated bythe local anaesthetic, bruised by a retractor or

pinched by a hamostat". It is not common,however, and only permanent if the sympa-thetic nerve is mistaken for the phrenic nerve

and divided. A few cases of Horner's syn-drome, following thoracoplasty or extrapleuralpneumonolysis, have been reported. These are

due to injury to the stellate ganglion or sympa-thetic chain and are frequently permanent.According to Paley7 8 cases of the syndrome

have followed intrapleural pneum onolysis.These are considered to be causedl by directinjury to the ganglion or its communiieations,by heat radiating from the cautery or bydamage to a misplaced sympathetic chain,particularly if separation is in the extr.apleuralplane.Although any type of apical lung neoplasm

nay produce Horner's synidromiie, it is more

commonly associated with malignanit apicaltumours. Pancioast, in 1924, first derew atten-tion to radiologically demonstrable apical tu-mours, accompanied by homolateral brachialplexus iinvolveimient, Hormer's synidroitie, localdestruction of bones ancd infiltrationi of softtissue. These tumnours, originally called''superior pulmonary sulcus tumours'", becauseof their locality, are now conisidered bronchialcarcainomata and over sixty cases have beenreported. The Hormer's synidrome may be pre-

ceded by irritative sympathetic phenomenaand, according to Ray8 becomes coiiplete onlyif the sympathetic chain is destroyed at or

above the first thoracic gaiiglioii. Other apicalneoplasms, primary or seconidary, mi<ay cause

Horner's syndlrome. Apparently beniign apicaltumours are potentially maligniant and biopsyis often the only sure method of diagnosis.Very fewr cases of Horner's syndrome asso-

ciated with goitre have been repoorted. Ac-cording to Blackwell9 who reviewed the litera-ture in 1944, and reported one case, the patientssought advice because of ptosis or thyroidgland enlargement. Contrary to popularbelief, the majority of these cases are due tothyroid adenoma, and not carcinomi-a.A cervTical rib may occasionally affeet the

superior cervical chain by pressure aned pro-

duce Horner's syndrome. It may appear as

a feature of Klumke 's paralysis. Generally,however, the more severe cases result fromlesions of the seventh and eighth cervical andthe first thoracic nerve roots.

Other rare causes of Horner's syndrome are:

faulty injection of the scalenus anticus for therelief of brachial plexus pain; injuries; pres-

sure from enlarged glands; aneurysms andtumours in the cervical region.

CASE HISTORYA white female, aged 34, first came to our attention

following a routine examination, in September, 1946.She had no complaints. Physical examination revealedptosis and miosis of the left eye, accompanied by slightenophthalmos and elevation of the lower lid, resulting

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SIMPSON: HORNER 'S SYNDROME [Canad. M. A. J.LAug. 1948, vol. 59

in a smaller palpebral aperture (Fig. 1). The reactionof the left pupil to light and accommodation wassluggish. The left eye was hazel in colour and the righteye was brown. A hard bony mass was visible andpalpable in the left supraclavicular fossa, extendingabout one inch above the clavical and from the sternalend of the clavicle to the posterior border of the sterno-cleidomastoideus (Fig. 2).

Radiological studies revealed a left apical mass, about8 em. in its greatest diameter, and extending from be-

yond the posterior aspects of the vertebral bodies halfway to the clavicle. The radiological picture displayedirregular calcification and the clearly demarcated outermargin suggested a cartilaginous shell with early calcifi-cation (Figs. 3 and 4).

The clinical and x-ray findings suggested a diagnog.:sof Horner 's syndrome due to an osteochondroma, ap-parently originating from the posterior third of theleft first rib. Although there was no evidence of

malignancy the consensus was that removal of the tumourshould be attempted.

The patient recalled that at the age of seven, shehad a "gnawing" pain in the left arm. The pain ex-tended from the shoulder to the finger tips and was

accompanied by difficulty in extending the fingers. Atthe age of fourteen, following pneumonia, miosis wasfirst noticed. Ptosis and colour changes in the eyegraidually developed; the ptosis at first being noticed

only when she was tired. At the age of seventeen, sheconsulted a physician because of pain in the arm and theeye changes.1o0 11 At that time the hard mass, then

tender, was first discovered clinically and confirmed

radiologically (Fig. 5). A course of radiation therapywas given, but as no change was noticed, it was dis-continued.

The patient served as a Lieutenant in the W.R.C.N.S.from April, 1943 to March, 1946. The ocular changesand the mass in the left apex were noted both on in-duction and discharge examinations. On re-examinationin December, 1946, and January, 1947, the left pupilwas regular in outline and was 2 mm. in diameter. The

right was 4 mm. in diameter. Intraoeular pain on pressure was equal on both sides. The ciliospinal reflex wasabsent on the left side. A few drops of 2% cocainewere instilled into each eye at intervals of 10 minutes.The right pupil dilated but there was no change in theleft pupil, indicating that the miosis was due to a lesionof the cervical sympathetic (Fig. 6). To estimate theextent of anhidrosis, Minor 's method was used and 1/12grain of pilocarpine injected hypodermically. Thegeneral response was normal and no marked changes inperspiration noted, except that the patch of Minor'ssolution in the right palm became purple sooner thanthat in the left. No pupillary changes were apparent.

Operative report.-July 8, 1947 (Dr. Robert M.Janes). At operation it was necessary to resect about4 inches of the second rib from the transverse processesforward and the extrapleural space was entered at thislevel. Pleura was stripped down to expose the underaspect of the tumour. As the pleura came away fromthe tumour it was torn in one place on the rough surface.This was sutured. Gradually a complete extra-periostealremoval of the tumour was obtained. It was interestingthat by pressure alone but not infiltration there was adeep groove in the lateral aspect of the first and secondvertebrae. The sympathetic trunk was identified as itdisappeared underneath the tumour. The wound wasclosed without drainage and the air from the accidentalpneumothorax aspirated. Patient was not unduly upset.

Pathological report (Professor W. L. Robinson).-Grossly, a large, rough, nodular, stony hard mass,measuring 8 x 6 x 5 cm., protrudes from the concaveedge of the posterior surface of the first left rib, andis very firmly attached to the rib edges. One aspectof the mass presents a deep, densely-red surface, whilethe other surface has a nodular shiny, yellow-pink ap-pearance (Fig. 7). The cut surface of the tumour masspresents a marbled appearance and is surrounded by an

indefinite margin of cancellous bone (Fig. 8). Micro-scopically, numerous sections present large normal areasof trabecular bone, with abundant interstitial bonemarrow. Contiguous to these normal areas is the tumour

Fig. 1. (September, 1946).-Ptosis and miosis of theleft eye with smaller palpebral aperture. Fig. 2. (Sep-tember).-A protruding mass is present in the leftsupraclavicular fossa. Fig. 3. (October).-An antero-posterior view of the thorax, showing the left apicalmass which displays irregular calcification. Fig. 4.(March, 1947).-A postero-anterior oblique view of thethorax which indicates the posterior position of the mass.(Courtesy of Toronto Hospital, Weston.) Fig. 5. (July,1931).-An antero-posterior view of the left upperthorax. (Courtesy of Dr. H. A. Rawlings, Vancouver,B.C.) Fig. 6. (January, 1947).-After instillation of 2%cocaine into each eye, the right pupil dilated. Thei-ewas no change in the left pupil.

Fig. 7.-The tumour mass firmly attached to thepostero-concave surface of the first left rib, showingits nodularity and investing capsule. Fig. 8.-Cut sur-face of the tumour mass, presenting the marbled tumourarea surrounded by an indefinite margin of cancellousbone.

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tissue, made up of conglomerate masses of trabeculatedbone tissue and cartilage. The latter tissue shows somedegenerative changes. The bone and cartilage cells areall quite well differentiated and show no evidence ofmalignancy. Diagnosis, osteochondroma.

This case was followed for eight monthsafter the operation. There is no clinical orradiological evidence of recurrence of thetumour. The left pupil now reacts less slug-gishly to light and there is a delayed reactionto cocaine.

COMMENTSOsteochondromata of the ribs are rare.

Andrus,12 in 1934, reporting from the ChestClinic Registry found that, of 117 proved chesttumours, there were no osteochondromata andonly 4 chondrosarcomata or myxochondro-sarcomata. According to Harper,13 of 250,000tumours registered at the Mayo Clinic up to1919, only one rib chondroma had been found,and from all sources up to 1939, only 60 caseshad been reported. Due to the rarity of thelesion and the too frequent inclusion of mal-ignant forms, he felt that no single authorcould draw conclusions from personal observa-tions. Harper quotes Geschickter and Cope-land as considering that chondromata, osteo-chondromata, myxochondromata and osteomataoriginate from pre-cartilaginous connectivetissue and represent different stages of thesame pathological process. A chondroma mayossify or undergo myxomatous degeneration,developing into a myxochondroma or a chon-dromyxosarcoma. Most authors agree thatchondroma and osteochondroma are potentiallymalignant and therefore, when diagnosed,early and complete removal is advocated.Cartilaginous tumours tend to undergo malig-nant change with age and incomplete removalincreases the risk of malignancy. Janes,14 in1939, reported 8 primary rib tumours; one ofwhich was benign osteochondroma of the ninthrib and another a chondroma of the third rib.He states that rapid development of the tumoursuggests sarcomatous degeneration, but thatthe only sure method of diagnosis is removal,and histological examination.Eyes of different colour are not uncommon

but are usually congenital and noted early inlife. A later colour change is rare and usuallyassociated with chronic iridocyclitis. Numer-ous observers have reported differences inintraocular vascularity in Horner's syndrome

but depigmentation of the iris is apparentlyquite uncommon.

SUMMARYA case of Horner's syndrome caused by an

osteochondroma of the left first rib is pre-sented. This is a rare entity and to our knowl-edge, a similar case has not been described inmedical literature. The syndrome was firstnoticed when the patient was fourteen yearsof age. Although the tumour was definitelyidentified three years later, the history ofbrachial plexus pressure symptoms at the ageof 7 years suggests that it may have developedearly in life. Enlargement of the tumourprobably occurred at puberty and gave rise tothe syndrome.The tumour was removed when the patient

was 34 years of age. Radiologically it had re-mained practically unchanged for 17 years andapparently there were no significant clinicalchanges in this interval. There was no evi-dence of malignancy and the postoperativecourse was uneventful.Eight months after removal there was evi-

dence of some regeneration of the sympatheticnerve fibres involved.

The author is grateful to Drs. G. C. Brink and A. G.Hill, of the Ontario Department of Health, for theirco-operation in the investigation of this case; to variousradiologists for their aid in diagnosis and to Drs. C. R.Messecar, A. Forsberg and C. H. Rorabeck, of the Divi-sion of Tuberculosis Prevention, for their help in thepreparation of this paper.

REFERENCES1. STINE, G. H. AND DRAPER, P.: Rocky MountaiJn Med.

J., 42: 504, 1945.2. Editorial: J. Am. M1. AsS., 134: 957. 1947.3. FULTON, J. F.: Arch. Surg., 18: 2025, 1929.4. STFAD, E. A. JR., EBERT, R. V., ROMAND, J. AND

WARREN, J. V.: Arch. Neurol. 6 Psychiat., 48: 92,1942.

5. ALI'ERS, B. J.: Clinical Neurology, F. A. Davis Co.,Philadelphia, p. 436, 1945.

6. ALEXANPER, J.: The Collapse Therapy of PulmonaryTuberculosis, Charles C. Thomas, Springfield, 1937.

7. PALEY, A.: J. Thoracic Surg., 16: 298, 1947.8. RAY, B. S.: Surg., Gyn. &6 Obst., 67: 577, 1938.9. BLACKWELL, C. C.: Mil. Susrg., 95: 219, 1944.

10. MCNIcoL, J.: Personal communication.11. RAWLINGS, H. A.: Personal communication.12. A.NDsus, W. D.: J. Thoracic Surg., 4: 236, 1934-35.13. HARPER, F. R.: J. Thorac Susrg., 9: 132, 1939-40.14. JANFS, R. M.: J. Thoracic Surg., 9: 145, 1939-40.

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