- Email: [email protected]
Geographical pathology—East Africa
GEOGRAPHICAL
PATHOLOGY--EAST
AFRICA*
I. W. J. McADAM, O.B.E., F.R.C.S.E.
From Makerere College Medical School, Kampala, Uganda IN the past century the diseases which devastated the people in tropical Africa have been intensively investigated and in many instances the aetiology of the disease defined and a remedy supplied. This has been a great era for the bacteriologist and parasitologist. The tall trees in the jungle of tropical medicine have been felled and in the clearings there are emerging patterns of disease which were probably always present but nevertheless dwarfed into insignificance by the endemic infections. To take this metaphor farther, the clearing of tall trees in an African jungle allows the undergrowth to flourish. Who would have imagined fifty years a g o t h a t the beds occupied by patients suffering from typhoid, plague and smallpox would now be filled by patients with fractured limbs resulting from car accidents, strangulated hernias, gonococcal strictures of the urethra and tumours of the jaw. These conditions represent some of our major surgical problems in Uganda. Uganda is a country with a population of six million, made up of Asians, Europeans and Africans of mixed Bantu, Nylotic and Hamitic origin.
Although the Equator passes through the southern part of Uganda, the climatic conditions are influenced by the proximity of Lake Victoria and the height above sea level. Kampala is 3,800 feet above sea level and, although humid, the climate has been likened to a continuous Californian summer. Mulago, the University Teaching Hospital of Makerere (Fig. 1), has 900 beds and serves a population of about one million people who live in a thirty-mile radius of the town. It is the only specialist centre in the country and in this capacity receives patients from the whole of Uganda and beyond. The new hospital, which has accommodation for Africans, Asians and Europeans, provides an opportunity to observe the disease patterns which occur in various racial groups and also the changes which occur within a racial group when their conditions of life alter. The skeleton of this paper could take many forms, the bones consisting of the various factors which influence the disease pattern in a given area. Genetic and environmental factors and tribal customs represent a broad classification of the
FIG. l The new Mulago Hospital, the Teaching Hospital of Makerere University College. * Paper presented at the Central African Radiological Congress, Bulawayo, May, 1962 G(16)
193
194
CLINICAL
factors operating to produce some of the surgical conditions 1 have selected for discussion. GENETIC FACTOR There is a great temptation to ascribe the cause of disease which occurs with undue frequency in a given area to a genetic factor. This attitude is often superficial and may prevent the real cause being uncovered. Congenital deformities.--The congenital deformities are not in this controversial category. In the West Nile area, supernumerary digits on the fifth finger and toe occur with great frequency. In a high proportion of schoolchildren in the area a tell-tale scar can be seen over the medial aspect of the fifth finger. Congenitally short metatarsals, usually the fourth and often bilateral, are observed in a high proportion of Banyaruanda but are rarely observed in other tribes. Certain other congenital deformities are rarely seen. In the past sixteen years I have not seen a child with congenital dislocation of the hip. It may be that mild forms of this condition are cured by the abduction, flexion and lateral rotation of the hips by the mother carrying the child on her back for the first months of life. Hernia.--A more controversial problem is the varying incidence of hernia in East and Central Africa. Although the incidence of simple and uncomplicated hernias amongst Africans is not available for East and Central Africa, the figures for strangulated hernia have, however, been collected from the operation records of widely scattered hospitals. Table 1 shows that the incidence of strangulated hernia is fairly uniform throughout all the various territories except Uganda. The figures for Uganda are of great interest and require explanation. Table 2 gives the incidence of strangulated external hernia in the main district hospitals of Uganda in 1959. It also shows the incidence of strangulation per 100,000 population. It is appreciated that not all strangulated hernias are treated in the main district hospitals, but nevertheless few districts have more than one large hospital and the people in the area will travel a long distance to receive treatment for a condition which they realise carries a high mortality rate. Table 2 also indicates that the strangulated hernia is common along the northern shores of Lake Victoria and gradually becomes less common in the northern and western provinces where the incidence would appear to be somewhat similar to that in other parts of East and Central Africa.
RADIOLOGY TABLE 1 STRANGULATEDEXTERNAL HERNIA
INCIDENCE OF STRANGULATED EXTERNAL HERNIA EAST AND CENTRAL AFRICA. TWELVE-MONTH PERIOD 1960 TO 1961 Uganda
Mulago
.
164 516
Jinja Kenya
Nairobi
.
8
Tanganyika
Mbeya Bukoba
.
18 13
Nyasaland
N . Rhodesia
S. Rhodesia
10 5
Blantyre .
Zomba
.
Kitwe Broken H'iU
12 9
""
42 30
Salisbury• Bulawayo (Mpilo)
TABLE 2 STRANGULATEDHERNIA IN UGANDA INCIDENCE OF STRANGULATEDEXTERNAL HERNIA TREATED IN THE MAIN DISTRICT HOSPITALS OF UGANDA (1959)
District
Hospital
No. of patients treated
Incidence per 100,000 population
Mengo
Mulago
198
15
Masaka
Masaka
105
24
Busoga
Jinja
300
45
Bukedi-Bugisu
Mbale
136
18
Teso
Soroti
40
9
Lango
Lira
8
2
Acholi
Gulu
4
1
West Nile
Arua
9
2
Bunyoro
Hoima
15
12
Toro
Fort Portal
8
2
Ankole
Mbarara
1
0.2
Kigezi
Kabale
10
2
Jinja (Jinja Hospital, Uganda) being the area with the highest incidence, a brief analysis of the type of hernia producing strangulation is of interest. The high incidence of strangulated direct hernia and of femoral hernia is shown in Table 3 for Jinja Hospital and has also been noted in the Mulago figures, but whereas in Mulago only one in four strangulations occurs in a direct hernia, three in every five strangulations are due to a direct hernia in Jinja. The age incidence of direct
GEOGRAPHICAL
PATHOLOGY--EAST
TABLE 3 ANALYSIS OF THE TYPE OF STRANGULATED EXTERNAL HERNIA JINJA 1961
Oblique hernia
196
Direct hernia
272
Femoral hernia
.
48
TOTAL
516
TABLE 4 AGE INCIDENCE OF STRANGULATEDDIRECT HERNIA JINJA 1961
Under twenty
7
Twenty to thirty .
57
Thirty-one to forty
96
Over forty
112
TOTAL
272
TABLE 5 STRANGULATEDFEMORAL HERNIA INCIDENCE OF STRANGULATEDFEMORAL HERNIA IN EAST
AND CENTRAL AFRICA. TWELVE-MONTHPERIOD 1960 TO 1961
Uganda
Mulago . Jinja
Kenya
Nairobi
Tanganyika Mbeya Nyasaland
25 48 0
Bukoba
0 1
Blantyre Zomba
0 0
N. Rhodesia Kitwe Broken Iiill
0 0
S. Rhodesia Salisbury Bulawayo
1 0
hernias is shown in Table 4. It will be seen that over 60 per cent of these hernias are in patients under forty years of age. At operation the Sac in these direct hernias is found separate from and passes medial and above the cord, through the conjoint tendon. This hernia was described by Ogilvie in 1935 and he attributed the cause to an abnormal gubernacular mechanism. The age incidence lends support to this theory and suggests that a genetic factor is in part responsible for the high incidence of strangulated hernia in this area. Table 5 shows that over vast areas of East and Central Africa strangulated femoral hernia does not occur. A surgeon with sixteen years' experience
195
AFRICA
working in various parts of Kenya has stated that he has only seen two patients with strangulated femoral hernias and it would appear from the figures obtained from all the principal hospitals in Southern Rhodesia that only one strangulated femoral hernia was treated last year. An explanation for the high incidence of femoral hernia in Uganda and its virtual absence elsewhere may be found in the altered shape of the female pelvis. The Baganda women have a high incidence of obstructed labour due to a contracted pelvis. The angle of attachment of the inguinal ligament to the pelvis requires careful study as the shape and size of the femoral canal must in some way reflect the bony deformity of the pelvis. THE I N F L U E N C E S OF E N V I R O N M E N T It is appreciated that the sub-heading "Environmental Factors," if used in its widest context, would cover the whole subject under discussion, but a few diseases have been selected to illustrate that certain diseases occur amongst people living in a specific area (Table 6). The basis for classification within this group is that in most instances the Africans, Asians and Europeans are apparently equally susceptible and the common denominator therefore is their place of residence• Lymphoma.--Burkitt in 1958 described a previously unclassified tumour syndrome occurring in children (Fig. 2). In Uganda this accounts for more than all other malignant tumours in children added together. This is a bizarre condition. It is a lymphoid tumour but clinical involvement of peripheral lymph glands and spleen is exceptional. The fact that these tumours are widely distributed throughout the body might suggest dissemination through the blood-stream, but it is significant that the lungs are rarely involved. In fact, this tumour is believed to have a multicentric origin arising from primitive lymphoid cells. It is almost unknown before the age of two and it is rare after the age of fourteen. It has been found not only in Africans but also in TABLE 6 GEOGRAPHICAL PATHOLOGY PATHOLOGY INFLUENCED BY ENVIRONMENT
1. A tumour
The lymphoma syndrome
2. Infection A worm A mycobacterium A filaria A pyogenic organism
Helminthoma Mycobacteriam ulcerans Adenolymphocele Gonorrhoea
196
CLINICAL
RADIOLOGY
FIG. 3 Adult patient showing inguinal adenolymphocele.
FIG. 2 A boy aged eight with a malignant lymphoma involving the left maxilla.
Asians and Europeans who have been born and have lived in Africa. The distribution of the lesions is widespread. The commonest presentation is a tumour of the jaw. All four quadrants may be affected simultaneously and loose teeth is one of the earliest clinical features. The teeth are also displaced by the tumour and eventually fall out. In most areas of Uganda, a child presenting with a tumour involving the jaws, thyroid, testis, ovary, salivary gland or retroperitoneal tissues is more likely to be caused by a malignant lymphoma than by any other pathological process. The same applies to a patient presenting with paraplegia or a proptosis of an eye, Geographical distribution.--With the exception of a few patients in New Guinea, this tumour syndrome has not been reported from outside Africa. Burkitt is in the process of defining the geographical boundaries in Africa. The available information shows that the tumour occurs in a belt across tropical Africa which has a tail running down the south-east coast. It is rare in the Rhodesias and almost unknown in South Africa. The Highlands of Kenya a r e apparently immune as are the Highlands in the south-west of Uganda which, although they have a very dense population, have not as yet reported a patient suffering from this disease. This patchy distribution has led to an examination of the climatic conditions over the whole area.
It has been found that in all areas where the tumour occurs, the temperature does not fall below about 60 ° F. This explains why the Highlands of Kenya are excluded and also why the tumour spreads so far down the east coast and in the Rhodesias is only found in the low-lying valleys of the Zambezi, Luapula and Sabi Rivers. A clinical observation followed by a study of the geographical distribution has led to some exciting speculations on the cause of this tumour. The clinical manifestations are similar to those found in the experimentally produced polyoma, which is a virus induced tumour. This observation, and the apparent dependence on climatic factors, has suggested that some insect vector may be involved in the transmission of an infection which stimulates tumour formation. Adenolymphoeele.--This condition, shown in Figure 3, and described as an " adenolymphocele," may be mistaken by the uninitiated as an unusual hernia. One of the early indications of ageing in the normal male is the appearance of a pectoral fold below the breast and a similar sagging at or slightly below the inguinal ligament, which is evidence of loss of elastic tissue in the skin. The patient shown in Figure 3 is suffering from onchocerciasis, a filarias transmitted by a fly of the genus Simulium, which breeds in flowing water. This condition is widely distributed across tropical Africa. The microfilariae produce intense inflammatory reaction in the skin destroying the elastic tissue. This altered skin texture leads to the development of abnormal skin folds at the knee, axillae, groins and around the buttocks. The actual" lymphocele"
GEOGRAPHICAL
PATHOLOGY--EAST
AFRICA
197
in the groins contains lymph glands, and somewhat from these ulcers have come almost entirely from fibrotic subcutaneous tissues. It is u n c o m m o n to the district lying to the north and east of Kampala. find a concomitant elephantiasis of the scrotum Thirty out of the forty-five have come from a small or leg. area of low population density of less than fifty Mycobacterial skin ulcer.--Over the past three people per square mile, known as Buruli. The area years necrotising skin ulcers have been seen in is adjacent to the Nile, it has a low rainfall and forty-five patients at Mulago Hospital (Fig. 4). the vegetation is thorn scrub. These lesions have been described by Clancy et al No reason has yet been given for this high (196l). The patients are usually children who are prevalence within a small area. No one African in good general health. Ulcers have been present tribe is more prone to the disease than another. usually for six months or more before the child Members of nine African tribes and one Asian arrives at hospital, although we have seen com- girl (who have come to live in the area) have paratively early acute lesions within weeks of the developed this ulcer. The condition may affect onset of ulceration. A history of minor trauma is several members of the same family. sometimes obtainable. The injured area becomes Gonococcal stricture of urethra.--In Kampala, indurated and about three weeks later ulceration treatment of stricture of the urethra constitutes of skin starts and spreads at a variable rate, one of our greatest hospital problems. Two hundepending upon the virulence of the secondary dred and seventy-five patients were admitted to infection. Mulago last year with acute complications following The margins of the ulcer are irregular and the stricture of urethra. edge is characteristically undermined for anything These patients, varying in age from the early up to five centimetres. The floor of the ulcer twenties to the late sixties, arrive in hospital either contains foul smelling sloughs which separate with acute or chronic retention, a peri-urethral slowly leaving a fibrotic and somewhat callous abscess, a fistula, a local or mass extravasation of ulcer. Almost any part of the body may be affected. urine or with one of the less common complications The infection is not limited by the deep fascia and of this disease. All, however, have a fibrous tendons and bone may be destroyed, leaving a stricture in one of three sites : - grossly deformed and useless limb. 1. Along the perineal urethra distal to the bulb. The causative organism is an acid-alcohol fast 2. At the penoscrotal junction. mycobacterium which has been given the name of 3. At the meatus. mycobacterium ulcerans. The incidence of stricture of the urethra in the I f untreated, the ulcer tends to heal at one edge Kampala area is higher than in other areas of and break down at another. Healing is frequently Uganda, and still more striking differences are followed by recurrence months or years later. found when the wider field of East and Central These ulcers have been reported from Australia Africa is examined. and the Congo by MacCallum et al (1944) and Accurate figures of the incidence of acute Janssens et al (1959). In Uganda, patients suffering gonococcal infection are not available, suprapubic cystotomy has, however, been the standard treatment for the impassable or complicated gonococcal TABLE 7 SUPRAPUBIC CYSTOSTOMYFOR GOYOCOCCAL STRICTURE OF URETHRA. EAST AND CENTRAL AFRICA 1961
Fro. 4 Female aged twenty-eight with an ulcer on the posterior aspect of the leg showing scarring and healing at one end, undermining and extension at the other.
Uganda
Kampala.
Kenya
Nairobi Kisumu
12 3
Tanganyika
Bukoba Tabora
6 0
Nyasaland
Blantyre Zomba
0 0
N. Rhodesia Kitwe Broken tiill S. Rhodesia Bulawayo Salisbury.
275
4 0 8 24
198
CLINICAL RADIOLOGY
stricture of the urethra in the past. These operation figures are available over a wide area in East and Central Africa and are shown in Table 7. The problem in Uganda.--Gonococcal infection was widespread amongst the people in Uganda before the arrival of the European. It cannot be said that in Uganda it is a disease of civilisation, although it is accepted that with the breaking down of tribal institutions and urbanisation the spread of the disease has been accelerated. Gonorrhoea is thought to have been brought to Uganda by the Arab traders coming up the west coast of Lake Victoria from Tanganyika. It would appear from the operation figures for strictures of the urethra that the spread of gonorrhoea was mostly amongst the tribes living on the shores of Lake Victoria and also amongst the peoples living in the eastern regions of Uganda. The hospital problem of the treatment of stricture has already been stated, but the effect of gonorrhoea on the community is of still greater significance. The fertility rate in the various tribes in Uganda was published after the 1959 Uganda census and there is a negative correlation--where the incidence of stricture of the urethra is high, the fertility is low, and, conversely, where the incidence of stricture is low, the fertility is high (Griffith 1962). Many important deductions could be made if more facts were known. Does the frequency of complications of gonococcal infection have a direct correlationship with the incidence of acute infection ? It is difficult to imagine that in the peri-urban populations of the capital towns of Central and East Africa there is not a highinfection rate amongst the African populations--if this is the case, why so few urethral strictures outside Uganda? Is there another factor operating in the disease in Uganda ? A combination of population census and hospital records has brought to light problems in Uganda which must be solved, not only at individual patient level but also on a community basis. Helminthoma.--Helminthoma is the name suggested for an inflammatory lesion of the large gut produced by penetration of the gut wall by a worm of the hookworm family (Elmes and McAdam 1954). In 1954, six patients, three European, two Asian and one African, were admitted to hospital suffering from complications arising from this infection. The presenting clinical picture was different in each case. As examples--a twelve-year-old European child presented with a large abscess in the right iliac fossa and was mis-diagnosed as a perforated appendicitis; an adult male European developed sudden generalised peritonitis which was misdiagnosed as a perforated peptic ulcer but at
operation he was found to have a perforation of the transverse colon; an adult African male presented with an acute intestinal obstruction and was found to have an intussusception, the head of the intussusception being formed by a helminthoma. It was unfortunate in this patient that the lesion was mistaken for carcinoma and a hemicolectomy performed. Since June 1954 many more patients suffering from this condition have been seen. Helminthoma now figures as a definite possibility when we are confronted with an acute abdomen presenting with unusual signs and symptoms. The worm is normally parasitic in apes and monkeys, although it has been described in Uganda as being fairly common in sheep. Infection is believed to be by ingestion and cases have been reported in which penetration of the gut of the normal host has taken place producing intestinal nodules. TRIBAL CUSTOMS Carcinoma of the penis.--Thirty patients with carcinoma of the peni s were seen in Mulago Hospital last year. In the area which Mulago Hospital serves, circumcision is usually only performed to relieve a complication of phimosis. Amongst the Bagisu, who live in the eastern area of Uganda, circumcision is performed as a puberty ceremony. Marital sanctions are applied to the uncircumcised. The area hospital at Mbale has no record of carcinoma of the penis occurring in this tribe. It is known that carcinoma of the penis can occur in the circumcised if the operation is not performed in infancy. It would, however, appear that circumcision delayed until puberty also confers a high degree of immunity. Volvulus of the large bowel.--Volvulus of the large bowel has been seen in eighty-five patients in the three-year period 1958-60. The patients usually present one or more days after the onset of abdominal pain. There is gross distension which in many instances can be recognised as being asymmetrical. The pelvic colon is affected and in six of the eighty-five patients the strangulation had produced gangrene of the gut. Volvulus in Uganda is more commonly seen in the area in which strangulated hernia is also common--that is around the northern shore of Lake Victoria. Mr Fisher of Kitwe has made the observation that previously when the mines in Northern Rhodesia supplied the meals of coarsely ground maize, the incidence of volvulus was high. Later, this incidence
GEOGRAPHICAL P A T H O L O G Y - - E A S T AFRICA
fell when the policy was changed and fine ground maize was supplied to the mine employees who prepared their own meals. In Uganda, in the area Where volvulus occurs most frequently, the staple diet is the bulky, high residue green banana. Diet unfortunately is not the full explanation, because although there is a significantly high incidence in the area to which I have referred, only the male members of the population are affected. The female is almost immune. Volvulus is used as the last example quite intentionally. A number of diseases have been described in this paper and then catalogued into well-defined compartments. This is over-simplification. For example : - 1. Is there a genetic factor which leads to abnormally long mesenteries in people who develop volvulus ? 2. Is the cause of volvulus found in the environment in which the soil and climate allow bananas to grow with such profusion ? 3. Is the immunity which the women enjoy related to tribal customs which prevent them eating the same diet as the men ? Is it a single factor or is it a combination ? DISCUSSION Our medical institutions in Africa have a unique opportunity of making contributions fundamentally important to medical science. In the field of environmental medicine we have a vast experiment taking place, in which large populations are changing their way of life and also moving from their traditional homes to seek employment in urban areas, and by so doing altering their disease pattern. We are privileged observers. Sixteen years ago, sixty out of the 300 surgical beds in Mulago Hospital were filled with patients suffering from tropical ulcer. In M a y 1962 there were only five patients in hospital with tropical ulcer. Reference should be made to the diseases which figure prominently in our surgical text-books which are conspicuous by their almost total absence in our African practice:--Mulago Hospital, 1959: renal calculi 0; gallstones 0; perforated peptic ulcer 4.
199
It would appear irrational to investigate something which does not exist. It is, however, important to record as m a n y facts as possible about the normal physiology of the people amongst whom we work so that if and when the disease pattern changes we have an established base line from which to observe these changes. These observations can be made either in a laboratory, using highly speeialised equipment, or by the doctor in the course of his daily work, using his powers of observation. Fifteen years ago a surgeon on Mulago Hospital staff started a photographic record of unusual surgical conditions met with in our hospital practice. This hobby, which extended to keeping a catalogue of diseases, has led to a number of highly important discoveries. His success has been built on accurate clinical observation, a great deal of enthusiasm, a camera and a record file. In vast areas of Africa, the overworked clinician with inadequate financial support is unable to afford the luxury of a well-equipped laboratory, but he may well afford to contribute to the science of his subject by following this example. Aeknowledgements.--It is not possible to acknowledge the sources of information in detail. The observations referred to in this paper have been made by the surgical staff working in Mulago Hospital. Mr H. F. Lunn has been mainly responsible for the work on mycobacterial ulcers, Mr H. Griffith for the work on strictures of the urethra, Mr D. P. Burkitt for the observations on the lymphoma syndrome and, together with Dr E. H. Williams and Dr C. L. Nelson, for collecting information on disease patterns in Central and East Africa.
REFERENCES BURKITT, D. P. (1958). Brit. J. Surg. 46, 218. BURKITT,D. P. (1962). Brit. reed. J. 2, 1019. CLANCEY, J. K., DODGE, O. G., LUNN, H. F. & ODUORI, M. L. (1961). Lancet, 2, 951. ELMES, B. G. T. & McADAM, I. W. J. (1952). Ann. trop. Med. Parasit. 48, l.
FISHER,A. C. (1961). Personal communication. GRIFFITH,H. B. (1962). Personal communication. JANSSENS, P. G., QUERTINMONT,M. J., SIENIAWSKI,J. t~: GATTI, F. (1959). Trop. geogr. Med. 11, 293. MAcCALLUM, P., TOLHWRST,J. C., BUCKLE,G. & SISSONS, H. A. (1948). J. Path. Bact. 60, 93.
PATHOLOGY--EAST
AFRICA*
I. W. J. McADAM, O.B.E., F.R.C.S.E.
From Makerere College Medical School, Kampala, Uganda IN the past century the diseases which devastated the people in tropical Africa have been intensively investigated and in many instances the aetiology of the disease defined and a remedy supplied. This has been a great era for the bacteriologist and parasitologist. The tall trees in the jungle of tropical medicine have been felled and in the clearings there are emerging patterns of disease which were probably always present but nevertheless dwarfed into insignificance by the endemic infections. To take this metaphor farther, the clearing of tall trees in an African jungle allows the undergrowth to flourish. Who would have imagined fifty years a g o t h a t the beds occupied by patients suffering from typhoid, plague and smallpox would now be filled by patients with fractured limbs resulting from car accidents, strangulated hernias, gonococcal strictures of the urethra and tumours of the jaw. These conditions represent some of our major surgical problems in Uganda. Uganda is a country with a population of six million, made up of Asians, Europeans and Africans of mixed Bantu, Nylotic and Hamitic origin.
Although the Equator passes through the southern part of Uganda, the climatic conditions are influenced by the proximity of Lake Victoria and the height above sea level. Kampala is 3,800 feet above sea level and, although humid, the climate has been likened to a continuous Californian summer. Mulago, the University Teaching Hospital of Makerere (Fig. 1), has 900 beds and serves a population of about one million people who live in a thirty-mile radius of the town. It is the only specialist centre in the country and in this capacity receives patients from the whole of Uganda and beyond. The new hospital, which has accommodation for Africans, Asians and Europeans, provides an opportunity to observe the disease patterns which occur in various racial groups and also the changes which occur within a racial group when their conditions of life alter. The skeleton of this paper could take many forms, the bones consisting of the various factors which influence the disease pattern in a given area. Genetic and environmental factors and tribal customs represent a broad classification of the
FIG. l The new Mulago Hospital, the Teaching Hospital of Makerere University College. * Paper presented at the Central African Radiological Congress, Bulawayo, May, 1962 G(16)
193
194
CLINICAL
factors operating to produce some of the surgical conditions 1 have selected for discussion. GENETIC FACTOR There is a great temptation to ascribe the cause of disease which occurs with undue frequency in a given area to a genetic factor. This attitude is often superficial and may prevent the real cause being uncovered. Congenital deformities.--The congenital deformities are not in this controversial category. In the West Nile area, supernumerary digits on the fifth finger and toe occur with great frequency. In a high proportion of schoolchildren in the area a tell-tale scar can be seen over the medial aspect of the fifth finger. Congenitally short metatarsals, usually the fourth and often bilateral, are observed in a high proportion of Banyaruanda but are rarely observed in other tribes. Certain other congenital deformities are rarely seen. In the past sixteen years I have not seen a child with congenital dislocation of the hip. It may be that mild forms of this condition are cured by the abduction, flexion and lateral rotation of the hips by the mother carrying the child on her back for the first months of life. Hernia.--A more controversial problem is the varying incidence of hernia in East and Central Africa. Although the incidence of simple and uncomplicated hernias amongst Africans is not available for East and Central Africa, the figures for strangulated hernia have, however, been collected from the operation records of widely scattered hospitals. Table 1 shows that the incidence of strangulated hernia is fairly uniform throughout all the various territories except Uganda. The figures for Uganda are of great interest and require explanation. Table 2 gives the incidence of strangulated external hernia in the main district hospitals of Uganda in 1959. It also shows the incidence of strangulation per 100,000 population. It is appreciated that not all strangulated hernias are treated in the main district hospitals, but nevertheless few districts have more than one large hospital and the people in the area will travel a long distance to receive treatment for a condition which they realise carries a high mortality rate. Table 2 also indicates that the strangulated hernia is common along the northern shores of Lake Victoria and gradually becomes less common in the northern and western provinces where the incidence would appear to be somewhat similar to that in other parts of East and Central Africa.
RADIOLOGY TABLE 1 STRANGULATEDEXTERNAL HERNIA
INCIDENCE OF STRANGULATED EXTERNAL HERNIA EAST AND CENTRAL AFRICA. TWELVE-MONTH PERIOD 1960 TO 1961 Uganda
Mulago
.
164 516
Jinja Kenya
Nairobi
.
8
Tanganyika
Mbeya Bukoba
.
18 13
Nyasaland
N . Rhodesia
S. Rhodesia
10 5
Blantyre .
Zomba
.
Kitwe Broken H'iU
12 9
""
42 30
Salisbury• Bulawayo (Mpilo)
TABLE 2 STRANGULATEDHERNIA IN UGANDA INCIDENCE OF STRANGULATEDEXTERNAL HERNIA TREATED IN THE MAIN DISTRICT HOSPITALS OF UGANDA (1959)
District
Hospital
No. of patients treated
Incidence per 100,000 population
Mengo
Mulago
198
15
Masaka
Masaka
105
24
Busoga
Jinja
300
45
Bukedi-Bugisu
Mbale
136
18
Teso
Soroti
40
9
Lango
Lira
8
2
Acholi
Gulu
4
1
West Nile
Arua
9
2
Bunyoro
Hoima
15
12
Toro
Fort Portal
8
2
Ankole
Mbarara
1
0.2
Kigezi
Kabale
10
2
Jinja (Jinja Hospital, Uganda) being the area with the highest incidence, a brief analysis of the type of hernia producing strangulation is of interest. The high incidence of strangulated direct hernia and of femoral hernia is shown in Table 3 for Jinja Hospital and has also been noted in the Mulago figures, but whereas in Mulago only one in four strangulations occurs in a direct hernia, three in every five strangulations are due to a direct hernia in Jinja. The age incidence of direct
GEOGRAPHICAL
PATHOLOGY--EAST
TABLE 3 ANALYSIS OF THE TYPE OF STRANGULATED EXTERNAL HERNIA JINJA 1961
Oblique hernia
196
Direct hernia
272
Femoral hernia
.
48
TOTAL
516
TABLE 4 AGE INCIDENCE OF STRANGULATEDDIRECT HERNIA JINJA 1961
Under twenty
7
Twenty to thirty .
57
Thirty-one to forty
96
Over forty
112
TOTAL
272
TABLE 5 STRANGULATEDFEMORAL HERNIA INCIDENCE OF STRANGULATEDFEMORAL HERNIA IN EAST
AND CENTRAL AFRICA. TWELVE-MONTHPERIOD 1960 TO 1961
Uganda
Mulago . Jinja
Kenya
Nairobi
Tanganyika Mbeya Nyasaland
25 48 0
Bukoba
0 1
Blantyre Zomba
0 0
N. Rhodesia Kitwe Broken Iiill
0 0
S. Rhodesia Salisbury Bulawayo
1 0
hernias is shown in Table 4. It will be seen that over 60 per cent of these hernias are in patients under forty years of age. At operation the Sac in these direct hernias is found separate from and passes medial and above the cord, through the conjoint tendon. This hernia was described by Ogilvie in 1935 and he attributed the cause to an abnormal gubernacular mechanism. The age incidence lends support to this theory and suggests that a genetic factor is in part responsible for the high incidence of strangulated hernia in this area. Table 5 shows that over vast areas of East and Central Africa strangulated femoral hernia does not occur. A surgeon with sixteen years' experience
195
AFRICA
working in various parts of Kenya has stated that he has only seen two patients with strangulated femoral hernias and it would appear from the figures obtained from all the principal hospitals in Southern Rhodesia that only one strangulated femoral hernia was treated last year. An explanation for the high incidence of femoral hernia in Uganda and its virtual absence elsewhere may be found in the altered shape of the female pelvis. The Baganda women have a high incidence of obstructed labour due to a contracted pelvis. The angle of attachment of the inguinal ligament to the pelvis requires careful study as the shape and size of the femoral canal must in some way reflect the bony deformity of the pelvis. THE I N F L U E N C E S OF E N V I R O N M E N T It is appreciated that the sub-heading "Environmental Factors," if used in its widest context, would cover the whole subject under discussion, but a few diseases have been selected to illustrate that certain diseases occur amongst people living in a specific area (Table 6). The basis for classification within this group is that in most instances the Africans, Asians and Europeans are apparently equally susceptible and the common denominator therefore is their place of residence• Lymphoma.--Burkitt in 1958 described a previously unclassified tumour syndrome occurring in children (Fig. 2). In Uganda this accounts for more than all other malignant tumours in children added together. This is a bizarre condition. It is a lymphoid tumour but clinical involvement of peripheral lymph glands and spleen is exceptional. The fact that these tumours are widely distributed throughout the body might suggest dissemination through the blood-stream, but it is significant that the lungs are rarely involved. In fact, this tumour is believed to have a multicentric origin arising from primitive lymphoid cells. It is almost unknown before the age of two and it is rare after the age of fourteen. It has been found not only in Africans but also in TABLE 6 GEOGRAPHICAL PATHOLOGY PATHOLOGY INFLUENCED BY ENVIRONMENT
1. A tumour
The lymphoma syndrome
2. Infection A worm A mycobacterium A filaria A pyogenic organism
Helminthoma Mycobacteriam ulcerans Adenolymphocele Gonorrhoea
196
CLINICAL
RADIOLOGY
FIG. 3 Adult patient showing inguinal adenolymphocele.
FIG. 2 A boy aged eight with a malignant lymphoma involving the left maxilla.
Asians and Europeans who have been born and have lived in Africa. The distribution of the lesions is widespread. The commonest presentation is a tumour of the jaw. All four quadrants may be affected simultaneously and loose teeth is one of the earliest clinical features. The teeth are also displaced by the tumour and eventually fall out. In most areas of Uganda, a child presenting with a tumour involving the jaws, thyroid, testis, ovary, salivary gland or retroperitoneal tissues is more likely to be caused by a malignant lymphoma than by any other pathological process. The same applies to a patient presenting with paraplegia or a proptosis of an eye, Geographical distribution.--With the exception of a few patients in New Guinea, this tumour syndrome has not been reported from outside Africa. Burkitt is in the process of defining the geographical boundaries in Africa. The available information shows that the tumour occurs in a belt across tropical Africa which has a tail running down the south-east coast. It is rare in the Rhodesias and almost unknown in South Africa. The Highlands of Kenya a r e apparently immune as are the Highlands in the south-west of Uganda which, although they have a very dense population, have not as yet reported a patient suffering from this disease. This patchy distribution has led to an examination of the climatic conditions over the whole area.
It has been found that in all areas where the tumour occurs, the temperature does not fall below about 60 ° F. This explains why the Highlands of Kenya are excluded and also why the tumour spreads so far down the east coast and in the Rhodesias is only found in the low-lying valleys of the Zambezi, Luapula and Sabi Rivers. A clinical observation followed by a study of the geographical distribution has led to some exciting speculations on the cause of this tumour. The clinical manifestations are similar to those found in the experimentally produced polyoma, which is a virus induced tumour. This observation, and the apparent dependence on climatic factors, has suggested that some insect vector may be involved in the transmission of an infection which stimulates tumour formation. Adenolymphoeele.--This condition, shown in Figure 3, and described as an " adenolymphocele," may be mistaken by the uninitiated as an unusual hernia. One of the early indications of ageing in the normal male is the appearance of a pectoral fold below the breast and a similar sagging at or slightly below the inguinal ligament, which is evidence of loss of elastic tissue in the skin. The patient shown in Figure 3 is suffering from onchocerciasis, a filarias transmitted by a fly of the genus Simulium, which breeds in flowing water. This condition is widely distributed across tropical Africa. The microfilariae produce intense inflammatory reaction in the skin destroying the elastic tissue. This altered skin texture leads to the development of abnormal skin folds at the knee, axillae, groins and around the buttocks. The actual" lymphocele"
GEOGRAPHICAL
PATHOLOGY--EAST
AFRICA
197
in the groins contains lymph glands, and somewhat from these ulcers have come almost entirely from fibrotic subcutaneous tissues. It is u n c o m m o n to the district lying to the north and east of Kampala. find a concomitant elephantiasis of the scrotum Thirty out of the forty-five have come from a small or leg. area of low population density of less than fifty Mycobacterial skin ulcer.--Over the past three people per square mile, known as Buruli. The area years necrotising skin ulcers have been seen in is adjacent to the Nile, it has a low rainfall and forty-five patients at Mulago Hospital (Fig. 4). the vegetation is thorn scrub. These lesions have been described by Clancy et al No reason has yet been given for this high (196l). The patients are usually children who are prevalence within a small area. No one African in good general health. Ulcers have been present tribe is more prone to the disease than another. usually for six months or more before the child Members of nine African tribes and one Asian arrives at hospital, although we have seen com- girl (who have come to live in the area) have paratively early acute lesions within weeks of the developed this ulcer. The condition may affect onset of ulceration. A history of minor trauma is several members of the same family. sometimes obtainable. The injured area becomes Gonococcal stricture of urethra.--In Kampala, indurated and about three weeks later ulceration treatment of stricture of the urethra constitutes of skin starts and spreads at a variable rate, one of our greatest hospital problems. Two hundepending upon the virulence of the secondary dred and seventy-five patients were admitted to infection. Mulago last year with acute complications following The margins of the ulcer are irregular and the stricture of urethra. edge is characteristically undermined for anything These patients, varying in age from the early up to five centimetres. The floor of the ulcer twenties to the late sixties, arrive in hospital either contains foul smelling sloughs which separate with acute or chronic retention, a peri-urethral slowly leaving a fibrotic and somewhat callous abscess, a fistula, a local or mass extravasation of ulcer. Almost any part of the body may be affected. urine or with one of the less common complications The infection is not limited by the deep fascia and of this disease. All, however, have a fibrous tendons and bone may be destroyed, leaving a stricture in one of three sites : - grossly deformed and useless limb. 1. Along the perineal urethra distal to the bulb. The causative organism is an acid-alcohol fast 2. At the penoscrotal junction. mycobacterium which has been given the name of 3. At the meatus. mycobacterium ulcerans. The incidence of stricture of the urethra in the I f untreated, the ulcer tends to heal at one edge Kampala area is higher than in other areas of and break down at another. Healing is frequently Uganda, and still more striking differences are followed by recurrence months or years later. found when the wider field of East and Central These ulcers have been reported from Australia Africa is examined. and the Congo by MacCallum et al (1944) and Accurate figures of the incidence of acute Janssens et al (1959). In Uganda, patients suffering gonococcal infection are not available, suprapubic cystotomy has, however, been the standard treatment for the impassable or complicated gonococcal TABLE 7 SUPRAPUBIC CYSTOSTOMYFOR GOYOCOCCAL STRICTURE OF URETHRA. EAST AND CENTRAL AFRICA 1961
Fro. 4 Female aged twenty-eight with an ulcer on the posterior aspect of the leg showing scarring and healing at one end, undermining and extension at the other.
Uganda
Kampala.
Kenya
Nairobi Kisumu
12 3
Tanganyika
Bukoba Tabora
6 0
Nyasaland
Blantyre Zomba
0 0
N. Rhodesia Kitwe Broken tiill S. Rhodesia Bulawayo Salisbury.
275
4 0 8 24
198
CLINICAL RADIOLOGY
stricture of the urethra in the past. These operation figures are available over a wide area in East and Central Africa and are shown in Table 7. The problem in Uganda.--Gonococcal infection was widespread amongst the people in Uganda before the arrival of the European. It cannot be said that in Uganda it is a disease of civilisation, although it is accepted that with the breaking down of tribal institutions and urbanisation the spread of the disease has been accelerated. Gonorrhoea is thought to have been brought to Uganda by the Arab traders coming up the west coast of Lake Victoria from Tanganyika. It would appear from the operation figures for strictures of the urethra that the spread of gonorrhoea was mostly amongst the tribes living on the shores of Lake Victoria and also amongst the peoples living in the eastern regions of Uganda. The hospital problem of the treatment of stricture has already been stated, but the effect of gonorrhoea on the community is of still greater significance. The fertility rate in the various tribes in Uganda was published after the 1959 Uganda census and there is a negative correlation--where the incidence of stricture of the urethra is high, the fertility is low, and, conversely, where the incidence of stricture is low, the fertility is high (Griffith 1962). Many important deductions could be made if more facts were known. Does the frequency of complications of gonococcal infection have a direct correlationship with the incidence of acute infection ? It is difficult to imagine that in the peri-urban populations of the capital towns of Central and East Africa there is not a highinfection rate amongst the African populations--if this is the case, why so few urethral strictures outside Uganda? Is there another factor operating in the disease in Uganda ? A combination of population census and hospital records has brought to light problems in Uganda which must be solved, not only at individual patient level but also on a community basis. Helminthoma.--Helminthoma is the name suggested for an inflammatory lesion of the large gut produced by penetration of the gut wall by a worm of the hookworm family (Elmes and McAdam 1954). In 1954, six patients, three European, two Asian and one African, were admitted to hospital suffering from complications arising from this infection. The presenting clinical picture was different in each case. As examples--a twelve-year-old European child presented with a large abscess in the right iliac fossa and was mis-diagnosed as a perforated appendicitis; an adult male European developed sudden generalised peritonitis which was misdiagnosed as a perforated peptic ulcer but at
operation he was found to have a perforation of the transverse colon; an adult African male presented with an acute intestinal obstruction and was found to have an intussusception, the head of the intussusception being formed by a helminthoma. It was unfortunate in this patient that the lesion was mistaken for carcinoma and a hemicolectomy performed. Since June 1954 many more patients suffering from this condition have been seen. Helminthoma now figures as a definite possibility when we are confronted with an acute abdomen presenting with unusual signs and symptoms. The worm is normally parasitic in apes and monkeys, although it has been described in Uganda as being fairly common in sheep. Infection is believed to be by ingestion and cases have been reported in which penetration of the gut of the normal host has taken place producing intestinal nodules. TRIBAL CUSTOMS Carcinoma of the penis.--Thirty patients with carcinoma of the peni s were seen in Mulago Hospital last year. In the area which Mulago Hospital serves, circumcision is usually only performed to relieve a complication of phimosis. Amongst the Bagisu, who live in the eastern area of Uganda, circumcision is performed as a puberty ceremony. Marital sanctions are applied to the uncircumcised. The area hospital at Mbale has no record of carcinoma of the penis occurring in this tribe. It is known that carcinoma of the penis can occur in the circumcised if the operation is not performed in infancy. It would, however, appear that circumcision delayed until puberty also confers a high degree of immunity. Volvulus of the large bowel.--Volvulus of the large bowel has been seen in eighty-five patients in the three-year period 1958-60. The patients usually present one or more days after the onset of abdominal pain. There is gross distension which in many instances can be recognised as being asymmetrical. The pelvic colon is affected and in six of the eighty-five patients the strangulation had produced gangrene of the gut. Volvulus in Uganda is more commonly seen in the area in which strangulated hernia is also common--that is around the northern shore of Lake Victoria. Mr Fisher of Kitwe has made the observation that previously when the mines in Northern Rhodesia supplied the meals of coarsely ground maize, the incidence of volvulus was high. Later, this incidence
GEOGRAPHICAL P A T H O L O G Y - - E A S T AFRICA
fell when the policy was changed and fine ground maize was supplied to the mine employees who prepared their own meals. In Uganda, in the area Where volvulus occurs most frequently, the staple diet is the bulky, high residue green banana. Diet unfortunately is not the full explanation, because although there is a significantly high incidence in the area to which I have referred, only the male members of the population are affected. The female is almost immune. Volvulus is used as the last example quite intentionally. A number of diseases have been described in this paper and then catalogued into well-defined compartments. This is over-simplification. For example : - 1. Is there a genetic factor which leads to abnormally long mesenteries in people who develop volvulus ? 2. Is the cause of volvulus found in the environment in which the soil and climate allow bananas to grow with such profusion ? 3. Is the immunity which the women enjoy related to tribal customs which prevent them eating the same diet as the men ? Is it a single factor or is it a combination ? DISCUSSION Our medical institutions in Africa have a unique opportunity of making contributions fundamentally important to medical science. In the field of environmental medicine we have a vast experiment taking place, in which large populations are changing their way of life and also moving from their traditional homes to seek employment in urban areas, and by so doing altering their disease pattern. We are privileged observers. Sixteen years ago, sixty out of the 300 surgical beds in Mulago Hospital were filled with patients suffering from tropical ulcer. In M a y 1962 there were only five patients in hospital with tropical ulcer. Reference should be made to the diseases which figure prominently in our surgical text-books which are conspicuous by their almost total absence in our African practice:--Mulago Hospital, 1959: renal calculi 0; gallstones 0; perforated peptic ulcer 4.
199
It would appear irrational to investigate something which does not exist. It is, however, important to record as m a n y facts as possible about the normal physiology of the people amongst whom we work so that if and when the disease pattern changes we have an established base line from which to observe these changes. These observations can be made either in a laboratory, using highly speeialised equipment, or by the doctor in the course of his daily work, using his powers of observation. Fifteen years ago a surgeon on Mulago Hospital staff started a photographic record of unusual surgical conditions met with in our hospital practice. This hobby, which extended to keeping a catalogue of diseases, has led to a number of highly important discoveries. His success has been built on accurate clinical observation, a great deal of enthusiasm, a camera and a record file. In vast areas of Africa, the overworked clinician with inadequate financial support is unable to afford the luxury of a well-equipped laboratory, but he may well afford to contribute to the science of his subject by following this example. Aeknowledgements.--It is not possible to acknowledge the sources of information in detail. The observations referred to in this paper have been made by the surgical staff working in Mulago Hospital. Mr H. F. Lunn has been mainly responsible for the work on mycobacterial ulcers, Mr H. Griffith for the work on strictures of the urethra, Mr D. P. Burkitt for the observations on the lymphoma syndrome and, together with Dr E. H. Williams and Dr C. L. Nelson, for collecting information on disease patterns in Central and East Africa.
REFERENCES BURKITT, D. P. (1958). Brit. J. Surg. 46, 218. BURKITT,D. P. (1962). Brit. reed. J. 2, 1019. CLANCEY, J. K., DODGE, O. G., LUNN, H. F. & ODUORI, M. L. (1961). Lancet, 2, 951. ELMES, B. G. T. & McADAM, I. W. J. (1952). Ann. trop. Med. Parasit. 48, l.
FISHER,A. C. (1961). Personal communication. GRIFFITH,H. B. (1962). Personal communication. JANSSENS, P. G., QUERTINMONT,M. J., SIENIAWSKI,J. t~: GATTI, F. (1959). Trop. geogr. Med. 11, 293. MAcCALLUM, P., TOLHWRST,J. C., BUCKLE,G. & SISSONS, H. A. (1948). J. Path. Bact. 60, 93.