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Intracranial haemorrhage in haemophilia in Nigerians
27)') TRANSACTIONSOF THE ROYAL SOCIETY OF TROPICAL MEDICINE AND HYGIENE. Vol. 66. No. 2. 1972.
I N T R A C R A N I A L H A E M O R R H A G E IN H A E M O P H I L I A IN NIGERIANS
E. M. E S S I E N AND A. ADELOYE Department of Haematology and the Neurosurgery Unit, Department of Surgery, University ~f Ibadan, N~eria Although comparatively few cases of classical haemophilia have been reported in Africans, experience of recent years from various parts of Africa shows that the disease may not be as rare as was formerly suggested (BoYLES and CURRIE, 1958; WINXROBe, 1967). In East Africa, FORBES, MACKAY and KHAN (1966) reported 5 cases in Africans from Kenya, and LOXHE (1968) described 8 cases encountered in Uganda between 1959 and 1967. TAYLOR et al. (1969) referred to a total of 11 African Kenyans who were treated with cryoprecipitate. In West Africa, where fewer cases have been reported, LINHARD et al. (1967) reported on 2 cases from Senegal, and most recently ESSIEN et al. (1970) described 6 Nigerians seen at University College Hospital (U.C.H.) Ibadan since 1966. The purpose of this paper is to draw attention to the unusually frequent occurrence of intracranial haemorrhage observed among the haemophiliacs in Nigeria. To date, of the 8 cases of classical haemophilia diagnosed in Nigerians ~.t U.C.H. Ibadan, 3 have had intracranial haemorrhage within the last 2 years. This observation is in striking contrast to the other series reported from East Africa in none of which intracranial haemorrhage was recorded either as a manifestation or complication of haemophilia. Report o f cases
Case 1 : Patient with incracranial ventricular haemorrh~e R.C., the first child of his parents, was circumcised at home at the age of 2 weeks without any untoward event. There is no family history of a bleeding tendency. The first manifestation of haemophilia was at the age of 4 months when he had meningitis and bled into the cerebral ventricles. He became severely anaemic in spite of repeated blood transfusion, and because of the rapid progression of his hydrocephalus, a ventriculo-peritoneal C.S.F. diversion using a Till-Wade valve was successfully performed (ADELOYE et al., 1969). After operation he bled profusely and investigation at this stage established that he had classical haemophili~,, his Factor V I I I level being 0.9" ~,. He was successfully managed with cryoprecipitate.
Case 2: Patient with subarachnoid haemorrhage J.F. was admitted at the age of 2 months with prolonged post-circumcision bleeding and severe pallor. Investigations revealed that he had classical haemophilia with Factor V I I I level of less than 1"i~. At the age of 6 months, he presented with neck stiffness and a lumbar puncture under cryoprecipitate cover revealed a uniformly blood-stained C.S.F. The authors acknowledge the helpful criticism of Professor L. Luzzatto of Ibadan and Dr. G. I. C. Ingram of St. Thomas's Hospital, London.
250
INTRACRAN_rAL HAEMORRHAGE IN HAEMOPHILIA IN NIGERIANS
A diagnosis of subarachnoid haemorrhage was made. He was successfully treated with transfusions of cryoprecipitate and plasma. He has since remained without any residual symptoms. Case 3: Patient with subdural haematoma
A.O., first presented with prolonged post-circumcision bleeding at the age of 2 months. His Factor V I I I level was 1.9°Z His older brother had bled to death after circumcision 2 years earlier. At the age of 3~ years he hit his head on the floor following a fall from a height of about 1~ feet. He had convulsive seizures, became drowsy, and developed a right fixed dilated pupil and decerebrate posture. Clinically, a right-sided intracranial surface clot was suspected. Bilateral parietal burr-holes were made and a right-sided subdural haematoma was drained successfully under cover of cryoprecipitate transfusion. Post-operatively, he was treated with transfusion of blood, plasma and cryoprecipitate. He made a complete recovery. Discussion
That haemorrhage can occur in the central nervous system in haemophilia is an old observation, the first reference to which was made by LANE (1840) ~ few years after HOPFF (1828), a pupil of Johann Schonlein, gave the name "haemophilia" to this life-long haemorrhagic diathesis. Such an event however is rare, the incidence varying from 1.6",, (FESSEY and MYENELL,1966) to 13.80,, (KERR, 1964). 3 of our 8 cases (38°i,) in Nigeria have had this complication within the last 2 years. In one patient (Case 2) this occurred spontaneously; in the other two, there was an element of trauma involved. 2 of the 3 patients (Cases 1 and 2) had episodes of violent coughing at the time of haemorrhage and this probably played some part in precipitating the bleeding into the central nervous system, if we assume that coughing happened to coincide with the "bleeding phase" in these haemophilic patients (KERR, 1963). Our incidence of 38%, is very high when compared with other reports. A much larger series than we have at present will however be needed for a more accurate assessment of the incidence of intracranial haemorrhage among haemophiliacs in our environment. Non-traumatic methods--skull x-ray, electroencephalography (E.E.G.), scanning techniques and the clinical history and findings are the most suitable aids to the diagnosis of haemorrhage into the C.N.S. in a haemophiliac (KERR, 1963). 2 of our 3 patients had lumbar punctures performed--one under cryoprecipitate cover, the other without when haemophilia had not yet been diagnosed. Both patients recovered, one with no residual symptoms. T h e other patient, who had Staphylococcus aureus meningitis, developed acute hydrocephalus and was successfully treated with cryoprecipitate and plasma infusions (ADELOYEet al., 1969). T h e risk of precipitating haemorrhage is minimised when cryoprecipitate is infused to raise the Factor V I I I level to 20-30°, before the lumbar puncture. Intracranial bleeding can involve any compartment of the intracranial cavity, and it is the most serious complication of the disease. In young haemophiliacs it occurs especially in relation to head injury. In the past, the mortality was as high as 70". (SILVERSTEIN, 1960) but this has fallen recently to about 20~'~, (DAVIESet al., 1966). The actual surgical procedures and problems in these patients are the same as in normal cases. The pre-operative diagnosis and the post-operative bleeding that may follow are the major problems of this complication. The use of cryoprecipitate is helpful in both directions; pre-operatively, the usual investigations are made feasible (DAVIES et al.,
E. M. ESSIEN AND A. ADELOYE
257
1966), and adequate Factor V I I I levels that can secure satisfactorv haemostasis can be achieved and maintained post-operatively. None of our 3 patients died from the intracranial haemorrhage which resulted from their bleeding diathesis. This emphasizes again the invaluable place of cryoprecipitate in the management of haemophilia.
Summary This paper reports the occurrence of intracranial haemorrhage in 3 Nigerians with classical haemophilia. As far as we are aware no instance of intracranial haemorrhage was mentioned in previous reports on haemophilia from other parts of Africa south of the Sahara, REFERENCES ADELOYE, A., SERIKI, O., LUZZATTO, L. & ESSIEN, E. M. (1969)..7. Xe~m~]. New'oswe. Psychiat., 32, 470. BOYLES, P. W. & CURRIE, J. (1958). Am. J. reed. Sci., 235, 452. DAVIES, S. H., TURNER, J. W., CUMMING, R. A., GII.LINGHAM, E. J.. GIRWOOD, R. H. & DARG, A. (1966). Br. reed. J., 2, 1627. ESSIEN, E. M., FOIAMI, A. O. & LUZZATTO,L. (1970). Trop. geogr. Med., 22, 403. FESSEY, B. M. & MEYNELL, M. J. (1966). Br. reed. J., 2, 211. FORBES, C. D., MACKAY,N. & KHAN, A. A. (1966). Trans. R. Soc. trop. Med. H3'g., 60, 777. HOPFF, F. (1828). Uber die Haemopkilie oder die erbliche Anlage su todlichen Blutungen. Inang Diss. Wurzburg. KERR, C. B. (1963). The Management of Haemophilia. Australasian Medical Publishing Ltd., N.S.W. - (1964). J. Neurol. Neurosurg. Psvchiat,, 27, 166. LANE, S. (1840). Lancet, 1, 185. LINHARD, J., GUICHENEY, A., PLASSART, H. & DIEBOLT, O. (1967). BM]. Soc. m~d. Air. noire Lang fr., 12~ 522. LOTHE, F. (1968). Trans. R. Soc. trop Med. Hyg., 62, 359. SlLVERSTEIN, A. (1960). Arcks. Neurol. Psvchiat., Chicago, 3, 141. TAYLOR, J. R., AHLUWALLA,N. S., MORRISON, C. J., KAVITI, J. M. & CARDWEI.L,([. 1.. (1969). E. Afr. reed. J., 46, 121.
I N T R A C R A N I A L H A E M O R R H A G E IN H A E M O P H I L I A IN NIGERIANS
E. M. E S S I E N AND A. ADELOYE Department of Haematology and the Neurosurgery Unit, Department of Surgery, University ~f Ibadan, N~eria Although comparatively few cases of classical haemophilia have been reported in Africans, experience of recent years from various parts of Africa shows that the disease may not be as rare as was formerly suggested (BoYLES and CURRIE, 1958; WINXROBe, 1967). In East Africa, FORBES, MACKAY and KHAN (1966) reported 5 cases in Africans from Kenya, and LOXHE (1968) described 8 cases encountered in Uganda between 1959 and 1967. TAYLOR et al. (1969) referred to a total of 11 African Kenyans who were treated with cryoprecipitate. In West Africa, where fewer cases have been reported, LINHARD et al. (1967) reported on 2 cases from Senegal, and most recently ESSIEN et al. (1970) described 6 Nigerians seen at University College Hospital (U.C.H.) Ibadan since 1966. The purpose of this paper is to draw attention to the unusually frequent occurrence of intracranial haemorrhage observed among the haemophiliacs in Nigeria. To date, of the 8 cases of classical haemophilia diagnosed in Nigerians ~.t U.C.H. Ibadan, 3 have had intracranial haemorrhage within the last 2 years. This observation is in striking contrast to the other series reported from East Africa in none of which intracranial haemorrhage was recorded either as a manifestation or complication of haemophilia. Report o f cases
Case 1 : Patient with incracranial ventricular haemorrh~e R.C., the first child of his parents, was circumcised at home at the age of 2 weeks without any untoward event. There is no family history of a bleeding tendency. The first manifestation of haemophilia was at the age of 4 months when he had meningitis and bled into the cerebral ventricles. He became severely anaemic in spite of repeated blood transfusion, and because of the rapid progression of his hydrocephalus, a ventriculo-peritoneal C.S.F. diversion using a Till-Wade valve was successfully performed (ADELOYE et al., 1969). After operation he bled profusely and investigation at this stage established that he had classical haemophili~,, his Factor V I I I level being 0.9" ~,. He was successfully managed with cryoprecipitate.
Case 2: Patient with subarachnoid haemorrhage J.F. was admitted at the age of 2 months with prolonged post-circumcision bleeding and severe pallor. Investigations revealed that he had classical haemophilia with Factor V I I I level of less than 1"i~. At the age of 6 months, he presented with neck stiffness and a lumbar puncture under cryoprecipitate cover revealed a uniformly blood-stained C.S.F. The authors acknowledge the helpful criticism of Professor L. Luzzatto of Ibadan and Dr. G. I. C. Ingram of St. Thomas's Hospital, London.
250
INTRACRAN_rAL HAEMORRHAGE IN HAEMOPHILIA IN NIGERIANS
A diagnosis of subarachnoid haemorrhage was made. He was successfully treated with transfusions of cryoprecipitate and plasma. He has since remained without any residual symptoms. Case 3: Patient with subdural haematoma
A.O., first presented with prolonged post-circumcision bleeding at the age of 2 months. His Factor V I I I level was 1.9°Z His older brother had bled to death after circumcision 2 years earlier. At the age of 3~ years he hit his head on the floor following a fall from a height of about 1~ feet. He had convulsive seizures, became drowsy, and developed a right fixed dilated pupil and decerebrate posture. Clinically, a right-sided intracranial surface clot was suspected. Bilateral parietal burr-holes were made and a right-sided subdural haematoma was drained successfully under cover of cryoprecipitate transfusion. Post-operatively, he was treated with transfusion of blood, plasma and cryoprecipitate. He made a complete recovery. Discussion
That haemorrhage can occur in the central nervous system in haemophilia is an old observation, the first reference to which was made by LANE (1840) ~ few years after HOPFF (1828), a pupil of Johann Schonlein, gave the name "haemophilia" to this life-long haemorrhagic diathesis. Such an event however is rare, the incidence varying from 1.6",, (FESSEY and MYENELL,1966) to 13.80,, (KERR, 1964). 3 of our 8 cases (38°i,) in Nigeria have had this complication within the last 2 years. In one patient (Case 2) this occurred spontaneously; in the other two, there was an element of trauma involved. 2 of the 3 patients (Cases 1 and 2) had episodes of violent coughing at the time of haemorrhage and this probably played some part in precipitating the bleeding into the central nervous system, if we assume that coughing happened to coincide with the "bleeding phase" in these haemophilic patients (KERR, 1963). Our incidence of 38%, is very high when compared with other reports. A much larger series than we have at present will however be needed for a more accurate assessment of the incidence of intracranial haemorrhage among haemophiliacs in our environment. Non-traumatic methods--skull x-ray, electroencephalography (E.E.G.), scanning techniques and the clinical history and findings are the most suitable aids to the diagnosis of haemorrhage into the C.N.S. in a haemophiliac (KERR, 1963). 2 of our 3 patients had lumbar punctures performed--one under cryoprecipitate cover, the other without when haemophilia had not yet been diagnosed. Both patients recovered, one with no residual symptoms. T h e other patient, who had Staphylococcus aureus meningitis, developed acute hydrocephalus and was successfully treated with cryoprecipitate and plasma infusions (ADELOYEet al., 1969). T h e risk of precipitating haemorrhage is minimised when cryoprecipitate is infused to raise the Factor V I I I level to 20-30°, before the lumbar puncture. Intracranial bleeding can involve any compartment of the intracranial cavity, and it is the most serious complication of the disease. In young haemophiliacs it occurs especially in relation to head injury. In the past, the mortality was as high as 70". (SILVERSTEIN, 1960) but this has fallen recently to about 20~'~, (DAVIESet al., 1966). The actual surgical procedures and problems in these patients are the same as in normal cases. The pre-operative diagnosis and the post-operative bleeding that may follow are the major problems of this complication. The use of cryoprecipitate is helpful in both directions; pre-operatively, the usual investigations are made feasible (DAVIES et al.,
E. M. ESSIEN AND A. ADELOYE
257
1966), and adequate Factor V I I I levels that can secure satisfactorv haemostasis can be achieved and maintained post-operatively. None of our 3 patients died from the intracranial haemorrhage which resulted from their bleeding diathesis. This emphasizes again the invaluable place of cryoprecipitate in the management of haemophilia.
Summary This paper reports the occurrence of intracranial haemorrhage in 3 Nigerians with classical haemophilia. As far as we are aware no instance of intracranial haemorrhage was mentioned in previous reports on haemophilia from other parts of Africa south of the Sahara, REFERENCES ADELOYE, A., SERIKI, O., LUZZATTO, L. & ESSIEN, E. M. (1969)..7. Xe~m~]. New'oswe. Psychiat., 32, 470. BOYLES, P. W. & CURRIE, J. (1958). Am. J. reed. Sci., 235, 452. DAVIES, S. H., TURNER, J. W., CUMMING, R. A., GII.LINGHAM, E. J.. GIRWOOD, R. H. & DARG, A. (1966). Br. reed. J., 2, 1627. ESSIEN, E. M., FOIAMI, A. O. & LUZZATTO,L. (1970). Trop. geogr. Med., 22, 403. FESSEY, B. M. & MEYNELL, M. J. (1966). Br. reed. J., 2, 211. FORBES, C. D., MACKAY,N. & KHAN, A. A. (1966). Trans. R. Soc. trop. Med. H3'g., 60, 777. HOPFF, F. (1828). Uber die Haemopkilie oder die erbliche Anlage su todlichen Blutungen. Inang Diss. Wurzburg. KERR, C. B. (1963). The Management of Haemophilia. Australasian Medical Publishing Ltd., N.S.W. - (1964). J. Neurol. Neurosurg. Psvchiat,, 27, 166. LANE, S. (1840). Lancet, 1, 185. LINHARD, J., GUICHENEY, A., PLASSART, H. & DIEBOLT, O. (1967). BM]. Soc. m~d. Air. noire Lang fr., 12~ 522. LOTHE, F. (1968). Trans. R. Soc. trop Med. Hyg., 62, 359. SlLVERSTEIN, A. (1960). Arcks. Neurol. Psvchiat., Chicago, 3, 141. TAYLOR, J. R., AHLUWALLA,N. S., MORRISON, C. J., KAVITI, J. M. & CARDWEI.L,([. 1.. (1969). E. Afr. reed. J., 46, 121.