ILIACUS A Common Complication of Haemophilia
Transcription
ILIACUS A Common Complication of Haemophilia
ILIACUS JOHN GOODFELLOW, From This but not paper the A Common Complication C. B. FEARN Nuffield describes exclusively, HAEMATOMA and D’A. Orthopaedic seeks and J. M. Centre to explain as a complication of Haemophilia and the MATTHEWS, Oxford Haemophilia a clinical syndrome of haemophilia. The by Bulloch and Fildes in 191 1 (Gunther 1924, 1935 ; Seddon 1953; Douglas and McAlpine of forty patients fifteen had suffered haemorrhage intramuscular We have Department We have . found of Orthopaedic Surgery for treatment had been treated elsewhere results from haemorrhage fourteen of the syndrome published cases since Miller and Taylor (1949) syndrome has usually been The following was known case history to suffer from is in many Christmas onset the pain became worse Two days later hypoaesthesia of this CASE ways disease then Lucia 1944 ; Weil 1946 ; Lyons is common in haemophiliacs: syndrome from and were examined by one into the iliacus muscle. ILLUSTRATIVE day after ofplasma. characteristically, features by marked flexion deformity nerve palsy. since the publication of Getting’s 1930 ; Tallroth 1939 ; Aggeler and 1956; Hall 1961). The syndrome studied by Davidson, Epstein, at least one such episode. The occurs main no less attributed into the psoas muscle, and Weil (1946) considered this the commonest haemorrhage in haemophilia. studied twenty-four patients, eleven of whom were admitted to the other thirteen the syndrome who only ENGLAND Centre which are the sudden onset of severe pain in the groin accompanied at the hip, followed by a mass in the iliac fossa and femoral Episodes of this sort have been reported in the literature case OXFORD, 1962 to of us. We 1962 a boy than to site of Nuffield 1965. The believe that REPORT typical. In October complained of pain in the left of sixteen hip. On the and he was admitted to hospital and given transfusions in the distribution ofthe left femoral nerve and weakness of the quadriceps muscle were noticed, and a mass was transferred to the Nuffield Orthopaedic Centre the same iliac and lumbar regions and held the left hip flexed to 30 30 degrees and there was a fairly free range of abduction, sensibility was impaired in the area of distribution of the felt in the left iliac fossa. He was day. He had severe pain in the left degrees. It could be flexed a further adduction and rotation. Cutaneous femoral nerve ; there was paralysis of the quadriceps muscle, and the patellar tendon reflex was absent. Deep in the left iliac fossa a tender mass was felt extending laterally along the iliac crest. In the past he had suffered numerous haemarthroses and two episodes of haematuria. Both knees and both elbows lacked full movement. Treatment-He of Christmas activity equivalent concentration the following controlled was factor from day. by large treated in bed (factor IX) concentrate to six litres nothing Pain doses of with normal to 1 18 per was the cent left thigh supported in flexion and were given by intravenous infusion. plasma, and of average persistent and severe of pethidine, after which raised normal. for the first it slowly his plasma Christmas A second twenty-four dose hours, diminished. 500 millilitres This was of For two was factor given on being poorly weeks he had intermittent discomfort, but the size of the mass in the iliac fossa remained unaltered. A month after the onset he got out of bed and the next day the pain returned and the mass became much larger. Two separate swellings could now be felt ; a rounded one filled the cavity of the 748 THE JOURNAL OF BONE AND JOINT SURGERY ILIACUS inner aspect of the ilium, ofthe psoas muscle. The nerve palsy was flexed, and further HAEMATOMA 749 and a fusiform one was felt medially, extending upwards combined swellings almost filled the lower left abdomen. complete. The Christmas patient factor was was put given. on a Robert The pain Jones rapidly frame in the The with subsided and line femoral the left in two hip weeks the mass was much smaller. A month later he was taken off the frame and the joints were mobilised in the swimming pool under cover of Christmas factor and plasma transfusions. At that time the mass had almost disappeared but the femoral nerve palsy was still complete. Fifteen months later the quadriceps femoris muscle had regained full power, but the patellar tendon reflex was was palpable The following mass absent. Sensory in the loss was limited to a small area over the patella and THE description of the SYNDROME syndrome is based on observations made on the twenty- have examined and on fourteen case histories published by other All but one of these patients suffered either from classical haemophilia or Christmas and had suffered previous bleeding. Most had had many haemarthroses, particularly knee. One patient, not a sufferer from a bleeding disease, developed four patients haematoma phenindione and syndrome Presenting started thigh. Onset the In none believe in the was than groin sudden this in one always was and of the Flexion therapy report anticoagulant that is spread in some the first of the bleeding the presenting to the patients intensity steadily increased until did severe trauma precipitate patients the onset of pain followed several twist complicating we occurring other symptom-Pain usually but and of with the diseases. syndrome. lumbar It region insidious or the in others, it was severe and constant. the haemorrhage, but in strenuous use or a minor limb. contracture of the hip-The hip was always held and usually in lateral rotation. The patients assumed posture in bed, lying curled up, often on the affected hips and knees flexed. Extension of the hip made but other movements were present sign distinguishes iliacus haematoma suppurative arthritis of the hip. in flexion a characteristic side, with the the pain worse if extension was avoided. from haemarthrosis or This acute Fenzoralnert’epalsy-Haemorrhage into the iliacus muscle is usually followed by femoral nerve palsy, but not always (Tallroth 1939, Weil 1946); we have seen one patient in whom an undoubted iliacus The haematoma thigh extent and of The shaded the the cases after did not cause nerve lesion development was of . nerve afterwards. nerve not the . compression. Nerve In detected until haematoma, many several but in . reported days or weeks our experience the onset of pain to the distribution usually is paralysed the degree complete. and the patellar in one of our loss sensory becomes of overlap tendon patients by adjacent well evidence cases accompanied 49 B, VOL. K the of other NO. lateral of nerve by 4, NOVEMBER surface lesion. an isolated 1967 with of us of the We believe femoral but the femoris Figure The skin thigh after that nerve the lateral two a iliacus palsy, “ the the area palpable is aboye crestand on the surof the iliacus which was wall tl the inguinal iliac ligament. muscle 1 shows the distribution area of total anaesthesia over palsy of the has examined area haematoma . quadriceps reflex absent. and is typical. nerves Lyons (1953) reported a patient as the femoral nerve. One anaesthesia The on leg anaesthesia. shows skin face marking or very soon of the femoral . and shaded abdominal . begins with palsy is limited compression nerve authors. disease, in the we iliacus an no abdomen. patella haemorrhage haematoma that was cutaneous haemophilic of cutaneous varied with always anaesthetic. nerve of the thigh as patients who had in the is in the it occasionally groin but great majority “ occurs without no of J. 750 GOODFELLOW, nerve compression at all, involved. Mass in the iliac Jbssa-There and C. B. D’A. that rarely was tenderness FEARN the AND lateral J. M. MATTHEWS cutaneous in the groin and nerve of the guarding thigh in the iliac may be fossa in all cases. In some the greatest tenderness was about the mid-inguinal point and slight fullness could be felt there. In others the tenderness was greatest in the iliac fossa next to the iliac crest. It was not possible to feel the mass in patients examined soon after the onset of symptoms the haematoma difficult. It always takes some time became palpable as to reach its full size, and after one or two days. “ guarding “ makes 60 65 palpation 11 10 9 8 Episodes 7 bleeding of 6 5 4 3 2 0 5 10 15 20 25 30 of thirty 45 50 55 70 (years) FIG. The age at onset 40 35 Age episodes in twenty-four receiving anticoagulant 2 patients. therapy. The patient over 65 years was The mass arises from the lateral wall of the pelvis and makes the normal concavity inner aspect of the wing of the ilium convex. In three of our patients the tumour iliac fossa and a second, discrete, fusiform tumour became palpable medial to the with a groove between the two. This second swelling was caused by blood distending psoas sheath. the the of filled first, the developed a few days after the onset of symptoms but the blood loss was never enough to cause shock. Constipation-Constipation, abdominal distension and colic occurred in most of our patients. Bowel sounds were present, but diminished, and two patients needed enemas. A degree of Anaemia-Anaemia constipation is to be expected in a patient in pain and lying motionless, but it is likely that a large iliacus haematoma has a more direct effect on the bowel. Ecchymosis-We have not seen skin discoloration in the vicinity of the haematoma, but Tallroth (1939) mentioned it. Age incidence-iliacus haematoma appears to be unique among the haemorrhagic accidents of the bleeding diseases as it occurs mainly in adolescents and young men (Fig. 2). It is strange THE JOURNAL OF BONE AND JOINT SURGERY ILIACUS that the are complication frequent so should and when arise in the haematomas so rarely intramuscular ANATOMICAL The walls syndrome of iliacus which muscle. the part to the inner lip ofthe margin of iliac the Over the upper psoas muscle the of the ten of life years at other when are common. sites haemarthroses CONSIDERATIONS described posterior transversalis. first is the result of haemorrhage into are formed by bone and fascia and which contains Figure 3 shows the iliac fascia in a fresh cadaver. at its margins to 751 HAEMATOMA crest inguinal and to the brim ligament where a closed compartment, the the femoral nerve and the The fascia is firmly attached ofthe true pelvis. it is continuous and lateral parts of the iliacus fascia is thin and translucent It is connected with the fascia muscle and over all but the lowest but it is particularly strong over iiiacus muscle cutaneousnerve of igh FIG. The the groove running the parts of the fascial Experiment-In injected When fluid occurred, was the into the VOL. similar and arching over cadaver it to the there were two The iliacus 49 B, NO. 4, of NOVEMBER 1967 transverse forms was found a dense nerve that are thicker and in Figure femoral cadaver. fibres progressively site the of a fresh the as it lies in the very large which distended of the iliacus it passes indistensible 4 shows quantities easily muscle by reinforced as funnel fibres bundles downwards of enclosing the lowest intermuscular of groove. water in its upper part a tense globular could be (Fig. 5). swelling palpated clinically (Fig. 6). When further fluid psoas sheath, which then filled up from below so that with a deep groove between them (Fig. 7). The psoas and in separate compartments except where they pass together the into swellings its fascia its fossa haematoma “ muscles lie therefore the thigh. In iliacus haematoma haemorrhage parts 3 iliac dissection of the psoas muscle into the substance iliacus thinner where at this sheath sheath injected left becomes muscles. in it “ overflowed forced eventually ligament of the muscles fascia of both a fresh the was into wall two The inguinal lower part these longitudinally. behind the between posterior and the occurs rounded into tumour the iliacus which muscle was felt causing distension of in all our patients. As 752 J. the small fibrous sheath related yet slow and distended (1939) on AND J. M. blood femoral MATTHEWS is forced nerve. a fusiform Should tumour which into He believed which occurs the bleeding was felt dense funnel continue, in three the of our of psoas patients. the genito-femoral nerve, though closely and therefore usually escape compression haemorrhage is usually associated record of a psoas abscess which this. sheath psoas FEARN of the thigh and the fascial envelope iliopsoas a single remarked of the distension D’A. distensible, the producing shown, found B. is not compresses lateral cutaneous nerve to the muscles, lie outside 3). As has been we have not Tallroth compartment below becomes The (Fig. iliacus tissue C. GOODFELLOW, with caused femoral nerve that it could be explained when a tuberculous abscess Psoas nerve palsy; compression. by the forms, relatively and that muscle iliacus muscle I Fibres FIG. The posterior cadaver. in the upper surrounded nerve and inflamed fascia is eroded and wall The of fascia part the right display over L4 muscle and iliac fossa dissected the of a fresh off the muscles femoral nerve lying by fat in the intermuscular groove. the iliacus and psoas muscles pass under a dense fibrous arch. ruptures long nerve 4 has been to arch.ng before the pressure The below within the abscess can compress the nerve. However, these considerations hardly explain the absence of nerve compression when the psoas muscle is the site of an acute non-tuberculous abscess as described by Zadek (1950). His six patients were found at operation to have tense abscesses within the psoas sheath ; yet none suffered a femoral nerve palsy. We described. believe The that the significant whether acute or chronic, which may overflow into explanation difference is to between is that the former the psoas sheath. be found an in the iliacus is primarily results of haematoma a haematoma THE JOURNAL the and experiment a psoas of the OF BONE AND iliacus JOINT just abscess, muscle SURGERY ILIACUS rise fluid Primary distension to compression, nerve at low of the psoas sheath, because the thin, 753 HAEMATOMA from whatever distensible cause, fascia would can be unlikely contain a large to give volume of pressure. FIG. Figure iliacus S FIG. 5-Injection of the psoas muscle. Figure 7-Further 6 FIG. sheath. Figure 6-Initial effect injection produces overflow of injection distension 7 of the sheath of the of the psoas sheath. PATHOLOGY The morphology of iliacus haematoma is shown in Figure 8. The specimen consists of the left half of the sacrum and a portion of the ilium of a patient of twenty-three years with haemophilia who developed bilateral iliopsoas haematomata shortly before his death (Hall 1961). At necropsy the haematomata were found to extend from the diaphragm to the inguinal ligament. fibro-osseous in front The cavity of the left haematoma communicated compartment which limits the haematoma the iliac fascia, are displaced forward by of It is difficult section clot. The to distinguish any structures that the femoral nerve confirmed In this patient iliacus muscle, destroyed the haematoma remnants of in the substance of the haematoma, but traversed the cavity, completely surrounded had ruptured which are seen into the compressed be supposed to compressing cases there was the nerve, no evidence that haemorrhage would that into cause ischaemic this occurred. the fascial ofthe muscle, result in addition yet in our which is so of spasm of the muscle; Later the flexion contracture in two was great distension of the fascial sheath and not to ischaemic contracture of the muscle, for although it persisted for many weeks, full extension was in every case when the haematoma finally resorbed and in no case was the power of flexion at the impaired. In nearly VOL. envelope the DIFFERENTIAL age and bowel and had become infected. against the ilium, was largely necrosis of the iliacus muscle, The striking flexion of the hip characteristic of the syndrome in the early stages is the of our patients the hip extended fully under anaesthesia. hip histological by blood by infection. It might due to contained regained with the lumen of the colon. The is well shown. The iliac vessels, lying the haematoma but not compressed. all patients the presence of three. it follows As has been mentioned, that the patient will 49 B, 4, NO. NOVEMBER 1967 of a severe DIAGNOSIS bleeding iliacus haematoma be a known sufferer diathesis is diagnosed before the occurs in adolescence and adult life and will almost always show some 754 J. GOODFELLOW, C. B. D’A. FEARN AND J. M. MATTHEWS evidence of haemorrhages in the past, a fact which should make misdiagnosis unlikely if the clinician is aware of the frequency of the complication. The occurrence of the syndrome in a patient undergoing anticoagulant therapy suggests that the complication should be borne in mind in acquired as well as hereditary coagulation defects. Acute haemarthrosis or suppurative arthritis of the hip bears a resemblance to this syndrome, but of a nerve haemophilia. lesion should the demonstration should Retroperitoneal be reserved of tenderness distinguish haematoma for bleeding and them. iliacus haematoma. The left half of suffering iliopsoas fascia. produces an entirely into the nerve compression When it occurs the diagnostic and loin, This but space different it is not like in the from is potentially clinical the well made space hip and the is uncommon presence in in haemophilia. The term which lies anterior to the 8 sacrum and large, ilium and A diffuse defined does not occur and there on the right side, iliacus error may have dangerous fossa, of the of 23 years of a patient haemophilia. very picture. tense the iliac haemarthrosis is a diagnosis commonly into the retroperitoneal FIG. An a mass Acute a true mass tumour retroperitoneal may be palpable, ofan iliacus is no flexion contracture haematoma may closely consequences (Fraenkel haemorrhage often extending haematoma. Femoral of the hip. mimic acute appendicitis 1957). If there is any evidence of femoral nerve compression (the earliest sign being hypoaesthesia of the skin over the patella) the possibility of appendicitis can be excluded. If nerve compression is absent, or if its onset is delayed, the diagnosis may be in doubt. Flexion deformity of the hip is more severe in iliacus palpated when in haematoma than is seen in acute appendicitis. in the early stages because of intense “ guarding doubt the iliac tumour is easily felt by examination lliacus “ of the under THE JOURNAL haematoma abdominal anaesthesia. OF BONE cannot muscles, AND JOINT be but SURGERY ILIACUS 755 HAEMATOMA TREATMENT The aim nerve damage, Pain that of treatment in the by correcting continues acute phase the clotting defect in spite of correction is to stop and bleeding and by immobilising of the clotting relieve pain the affected defect ceases and iliacus when lessen muscle. the patient is immobilised. The patient of human with haemophilia antihaemophilic is given globulin fresh if plasma frozen plasma or a more potent concentrate proves ineffective. The patient with Christmas disease is treated with plasma, which need not be fresh but should be less than a week old. A concentrate of Christmas factor is used in a few centres for control of persistent bleeding. Infusion should be continued for at least four days to allow some organisation of the clot (Biggs and Macfarlane 1962). Bed rest, with pillows to support the affected haemorrhage, but when bleeding continues and hip pain in flexion, is severe may be adequate immobilisation in slight should be complete. A modified Robert Jones frame has been used with success in several cases. Once the pain has subsided the flexion contracture diminishes, and its correction may be helped by light traction to the leg. Protective infusions may be given again for a few days when mobilisation and weight bearing are begun. When walking is allowed it is important to protect muscle has knee the knee regained with a caliper full power. is almost inevitable. disability from which damaged an iliacus it beyond We and to continue If this precaution this protection is omitted have many examined until recurrent patients the quadriceps haemarthrosis in whom haematoma was a disorganised knee repair during the months of quadriceps the femoris of the only from repeated paralysis. residual haemarthroses PROGNOSIS In the published unpredictable. showed cases Two patients a considerable haemorrhage. numbness muscle for but nerve In one the and twenty power was is recovery who even suffered immediately and was there less than excellent, patients, recovery a complete of femoral was in Medical severe iliacus treatment the only treatment. patient we have seen who escaped This experience suggests that avoided or at least function marred only and wasting of the Research developed a nerve palsy if treatment patients or while signs by has been 1953); showed a year a small three none or more of area quadriceps femoris Council scale. The prognosis if proper treatment is given. in hospital, it was possible to haemorrhage, haemorrhage he never institute in seventeen complete weakness 4 on the nerve recovery (Seddon 1930, Lyons 1924, 1935; Weil 1946); and two (Gunther In five not studied, McAlpine 1956). cases of iliacus haematoma patella. recovery of our made In fifteen over have improvement (Tallroth 1939, Douglas We have reviewed after we of nerve and the is given compression. This is only patient who got prompt at once nerve palsy may be diminished. SUMMARY I Haemorrhage femoral nerve into the is a common . 2. The iliacus haematoma thirty episodes occurring 3. The anatomy of the compression 4. Differential explained. diagnosis, one patient are 5. An instance presented. of iliacus fascial compartment which complication of haemophilia. syndrome is described and illustrated in twenty-four patients. iliopsoas fascia is described and the prognosis haematoma is recorded. VOL. 49 B, NO. 4, NOVEMBER contains 1967 and treatment occurring are discussed as a complication the iliacus from the mechanism and the muscle and authors’ of study femoral necropsy of anticoagulant the of nerve findings therapy in 756 .i. G000FELLOW, We wish clinical were to record our indebtedness manifestations treated under to Professor of haemophilia. in collaboration the direction both in care of Lord Mayor Treloar M. Heywood, the necropsy C. B. i)’A. the with J. Trueta Patients the Medical patients in preparation Orthopaedic Hospital the Council’s We of this are M. MATTHEWS inspiration Blood Our thanks given are to the study of Orthopaedic Research Rosemary several Dr has Coagulation to Dr us to examine Disabled Boys. The illustrations he Department grateful paper. who allowed Headmaster of the Alton School for findings of the patient he reported. J. to the Nuffield Research R. G. MacFarlane. AND for admitted of Professor and FEARN due to patients Unit, Biggs Mr M. R. Hall gave are the work of for Oxford, her advice E. S. Evans under of Surgery his care, of the and to Mr us permission to publish Mr David Drury of the Photography Department, by the Oxford Regional Blood Products of the Medical the Nuffield Orthopaedic Centre. The fresh frozen plasma referred to was supplied Blood Transfusion Service; the human anithaemophilic globulin concentrate by the Laboratory of the Lister Institute, and the Christmas factor concentrate by Dr E. Bidwell Research Council’s Blood Coagulation Research Unit, Oxford. REFERENCES P. M., and AGGELER, and Mental R., BIGGS, and W., Section and XIVa. C. S., DAVIDSON, of Forty Disease. Forschuizg, H. GUNTHER, M. : Eugenetics and R. (1961): (191 D., 1): Blood Haemophilia: In Laboratory G. MILLER, Journal, Surgery (‘oagulation: and Its Disorders, and F., Treasury of Humaz Inheritance. F. H. L. (1949): TAYLOR, Neurological in Haemophilia. Complicated Haemophilia Hemophilia Nerve. Les I. (1950) : Acute and Joint 241 et seq. p. 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