Otolaryngological complications of measles in West Africa By F. D. MARTINSON (Ibadan, Nigeria) IN some tropical developing countries like Nigeria, measles in children is usually regarded as serious since it is often lethal and carries a high incidence of major complications among survivors (Morley 1962; Hendrickse, 1964; Hendrickse et al., 1964). As in Europe, the otolaryngologist here sees numerous cases of upper respiratory tract infection and otitis media following measles but these on their own are of minor consequence compared with others, such as the severe pulmonary complications, laryngotracheo-bronchitis, meningitis and encephalitis or encephalomyelitis which appear frequently in otolaryngological clinics. These serious complications are of even greater significance here than in Western countries because of the low resistance of many children and also because there are very limited rehabilitative services for those who survive and are left with handicapping audiologic or neurologic sequelae. Almost peculiar to developing countries even in modern times are certain ulcerative lesions involving the head and face as well as the nasal, oral and pharyngeal cavities of children whose resistance has been previously lowered by poor states of nutrition. Sequelae of such ulcerations require the skills not only of the otolaryngologist but often also of the dental, facio-maxillary and plastic surgeons depending on the site affected, and the extent of destruction, disfigurement and impairment of function which result. The ulcerations usually commence during or after the desquamative stage of measles and seem to be of two types, a severe gangrenous destructive type and a mild non-gangrenous type limited to small areas. The gangrenous lesions The severe type, also known as cancrum or noma, commences as a small papule on the face or head or in the oral or nasal cavity. This soon becomes a rapidly spreading gangrenous ulcer destroying soft tissue and bone in its path. The extent and direction of spread before the destructive process is spontaneously arrested, varies considerably and is unpredictable. The deeper part of such a lesion usually covers a much wider area than the size of the visible superficial portion would suggest. Thus the eventual 631

F. D. Martinson degree of disfigurement or impairment of function ranges from slight to grotesque. In aural lesions, which might be referred to as 'cancrum auris', defects encountered have ranged from the loss of the lobule to loss of the pinna (Fig. i) and even to exposure of large areas of the infra-temporal region as well as of the middle and posterior cranial fossae. Nasal lesions (cancrum nasi) also vary from loss of an ala (Fig. 2) to destruction of the nose, palate and maxilla exposing the oro-nasal cavity and pharynx on one or both sides. However extensive the destruction, the tongue, in our experience has never been affected.

FIG. 1. Meatal Stenosis and Loss of most of a pinna following cancrum auris.

During the active stage of a typical case the child looks ill and except in a few cases looks obviously malnourished. It has, somewhere on the head or face, a fetid ulcer with greenish black necrotic edges and a central slough or a base of bare bone. Some oronasal lesions remain deep-seated in the nose or maxilla and the absence of an obvious external ulcer may cause difficulty in diagnosis. Thus a history of recent unilateral, foul, bloodstained nasal discharge may suggest among other things a foreign body, acute osteomyelitis or a Burkitt lymphoma, and the presence of an inflammatory facial swelling may strengthen the latter tentative diagnosis in this part of the world. In a few cases, brisk epistaxis which later proves to have originated from eroded ethmoidal or even internal maxillary 632

Otolaryngological complications of measles in West Africa artery may be the first symptoms for which the child is brought to hospital. Less dramatic indications of the erosive process in the nose and palate, are progressive nasal speech (rhinolalia operta) and regurgitation of fluids and feeds through the nose.

FIG. 2. Loss of columella and part of ala following cancrum nasi.

Similarly the nature of lesions commencing deeply in the external auditory meatus and spreading towards the middle ear may not be appreciated at first. The tendency of such lesions to form a sequestrum of large portions of the meatal wall places the facial nerve at risk. In these cases which may show no clinical evidence of facial paralysis the nerve has been found at operation lying, still intact, in such a sequestrum. In spite of the sites involved, clinical evidence of intracranial spread are surprisingly uncommon among patients who attend hospital for treatment. In one case removal of a large slough involving part of the bony and cartilaginous external auditory meatus and the post-aural area as far as about 2 cms behind the attachment of the pinna, was followed immediately by brisk haemorrhage from the superficial temporal vessels and from the sigmoid sinus. The latter as well as parts of the cerebellum and temporal lobe lay exposed in the resulting crater. The head of the mandible and about 2 cms of the ascending ramus were also visible. Surprisingly there were no signs of meningitis or other intracranial complication even 633

F. D. Martinson at this stage and adequate antibiotic treatment post-operatively prevented development of this complication later. Treatment of the ulcerative lesions consists of the administration of systemic antibiotics and sulphonamides and the improvement of the nutritional state. Surgical intervention is limited to the removal of sloughs and sequestra only since, as shown by Tempest (1966), excision of necrotic edges during the spreading phase is no guarantee against further extension of the gangrenous ulceration to its unpredictable limit. Vincent's organisms have been found most often in these gangrenous lesions, an observation also confirmed by Tempest (1966) in a study of over 250 cases in Nigeria. In the healed or arrested stage the child looks less ill but bears the scars and defects which indicate the site of previous ulceration. Palatal defects and severe trismus with the teeth tightly clenched, are common sequelae of cancrum oris, the trismus being due to fibrosis of the soft tissue between the angle of the mouth and the temporo-mandibular joint. In spite of this, the child soon learns to compensate for the impairment of function, a fact which explains the frequently observed long delays of 6 to 12 months or more before treatment is sought. One case of severe trismus, and he was unique, first presented for treatment about 15 years after the lesion had first developed. In spite of the disability and intra-oral deformity he appeared reasonably well-nourished, he had been through elementary school and for a long time taken an active part in a local chapel choir!! Later sequelae of intranasal lesions include middle ear infection, atrophic rhinitis, rhinitis caseosa, rhinolith, nasal and pharyngeal stenoses and palatal incompetence. These and other sequelae such as partial or complete loss of the nasal septum or the naso-antral wall, a saddle-shaped nose, or a defect or scarring of the pharynx or palate are findings which might each be diagnosed as end results of syphilis, yaws or a few other tropical conditions, if a history of cancrum were not given. Depending on the site and extent of destruction and the amount of disability produced, the management of the 'healed' stage varies in complexity. In a few cases when the scar is limited to the region of the masseter, or a fibrous band forms between the maxilla and the mandible causing trismus, it has been possible to detach the fibrous band from its attachment to the mandible and mobilize the jaw. The use of a simple jaw exerciser (Fig. 3) after this procedure has led to permanent and satisfactory function (Fig. 4). These cases, however, constitute a very small proportion of cases of trismus seen, and more extensive facio-maxillary or plastic surgery is usually necessary. A salivary fistula is not an uncommon complication. It can usually be corrected by inversion of the fistulous track. In about four cases (40 per cent of those treated) this did not prove successful and in these cases the affected gland was irradiated prior to closure of the fistula. 634

Otolaryngological complications of measles in West Africa

FIG. 3. Jaw exerciser in position after removal of scar and manipulation of Jaw.

FIG. 4. Another child four months after use of jaw exerciser.

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F. D. Martinson The non-gangrenous lesions Milder and non-gangrenous ulceration may involve the nostril and the adjoining area of the upper lip only, or the entrance to the external auditory meatus. The affected site is covered with granulations during the active stage and the underlying cartilage of the ala nasi or the tragus and external auditory meatus are the only firm tissues destroyed. Bone is not affected. Unlike the findings in the gangrenous lesions, the only organisms isolated have been staphylococci, non-haemolytic streptococci and some gram negative bacilli such as proteus, pyocyaneus and coliform organisms. Vincent's organisms have not so far been isolated. On healing the nostril is narrowed by collapse of the ala or by cicatricial stenosis. In aural lesions meatal stenosis develops the stenosing tissue varying from a partial 'web' to a thick occluding plug of fibrous tissue which occupies the caritaginous portion only of the external meatus. The pinna is only slightly if ever affected. Quite often at operation excision of the plug of fibrous tissue reveals a mass of soft cheesy material deep to which is a thin, but often unperforated tympanic membrane. The cheesy material sometimes ruptures and discharges into the post-auricular sulcus. Nasal and aural lesions may be present in the same patient and are sometimes bilateral. Corneal ulceration occurs and leads to a staphyloma or occasionally destruction of the eye. This familiar post-measles triad of ulcerations (in the ear, nose and cornea) may be present singly, all together or in any combination. Treatment during the ulcerative stage consists mainly of local dressings and the administration of antibiotics. Attempts to prevent stenoses have usually proved unsuccessful because of the difficulty of retaining short tubes or prosthesis inserted into the external ear or the nose. In the healed stage, such stenoses are surgically corrected and any associated or coincidental middle ear and nasal infection is also treated. Other complications of measles in various parts of the respiratory tract and oesophagus leading to stenoses and obstruction are also encountered. Laryngo-tracheal lesions (a) Although hoarseness with or without stridor may be due to a simple laryngitis, it may also be caused by ulceration of enanthemata which often appear in the laryngo-tracheal region. These ulcers heal leaving anything from a small symptomless scar on the false cords to distortion and stenosis of the laryngeal inlet, or formation of a thick fibrous 'web', each necessitating tracheostomy and prolonged surgical treatment as some glottic and subglottic stenoses often do. (b) A firm non-ulcerating swelling is sometimes seen occupying half or more of the circumference of the lumen of the subglottic area. Biopsy of this has shown non-specific inflammatory tissue only and the swelling has 636

Otolaryngological complications of measles in West Africa usually subsided in two to three weeks under hydrocortisone and antibiotic therapy. (c) Laryngeal papillomata, particularly in children under five years, are so often preceded by measles (nearly 50 per cent of 40 cases) that the association of these diseases is considered not to be coincidental. This association has also been referred to by Samakhvalova (1968). In one case seen here within the last three years the papilloma arose almost immediately after an anti-measles vaccination (Singh, 1971). Treatment is by repeated endoscopic removal whenever necessary and recurrences have been seen over periods varying from a few months to four or five years. In our experience of papillomas seen in young children (over 100 cases) only three have persisted to between the ages of 11 and 15 years having commenced at about the age of 5 years. In none of the three cases has the disease shown any sign of advancing during the past four years. (d) Laryngo-tracheo-bronchitis is usually more severe when it follows measles than it is when due to other causes but does not always require tracheostomy; intubation with a Jackson Rees tube is often enough to tide the patient over the crisis, but it has a drawback in that it is more difficult to clear obstructing membrane and thick secretions from it than from a tracheostomy cannula which has an inner tube. In half of these cases administration of antibiotics and hydrocortisone has proved effective whenever the patients have been seen reasonably early for treatment. (e) Recurrent laryngeal paralysis occurs but is uncommon. In ten years it has been encountered in six patients, in two of whom it was bilateral. Four improved in two to four months, and one, a bilateral lesion, still persists after three years. One defaulted after some improvement. (f) Pneumothorax and pneumomediastinum superimposed on a pulmonary infection have been observed by De Buse et al. (1970) and other authors as complications of measles. Here in Nigeria Ransome Kuti et al. (1968) recognized this complication in children and in a review by Familusi and Bohrer (1972), out of 33 cases of pneumothorax and pneumomediastinum in children found that 50 per cent developed after a measles. Some of these emergencies are sometimes hurriedly channelled to the Otolaryngologist as probable cases of respiratory (laryngeal) obstruction but the correct diagnosis can be made by careful examination and radiography. Mortality has been high in these cases. These lesions are due to rupture of an alveolus the site of which usually determines if a pneumothorax or pneumomediastinum will result. Ulceration of enanthemata in the trachea and bronchi sometimes perforates particularly if there is a degree of laryngeal obstruction and may well be the cause of these complications as well as of extensive surgical emphysema which has also been observed, but the latter has a better prognosis than the former. In one case the emphysema extended up to the 637

F. D. Martinson scalp and down to the ankles and in another the site of perforation was observed on endoscopy. Pharyngo-oesophageal lesions. Small non-gangrenous ulcerations and their scars in the oropharynx (not due to cancrum) cause little disturbance and are often discovered accidentally. In the hypopharynx or upper oesphagus the ulcer, considered to be also enanthematous in origin, may produce crescentic or annular strictures suggestive of small areas of corrosive oesophagitis, and in view of the prevalence of this condition at almost any age here, one may on casual inspection suspect it. Treatment of the oesophageal obstruction by repeated bouginage in the non-ulcerative stage has been successful in all of three cases seen. Auditory and Neurologic Complications. Not uncommonly labyrinthitis and intracranial infection may complicate suppurative otitis media which often follows measles. The former complication is sometimes also the result of cancrum auris, and in these cases irreversible unilateral or bilateral deafness is the natural outcome. Encephalitis and encephalomyelitis are unfortunately common. The otolaryngologist normally sees them only when deafness is severe or total, speech is lost and evidence of psychological changes have become apparent. At this stage he can merely assess the damage and recommend one of the limited rehabilitative services available. These intracranial complications have sometimes occurred without a history of a rash to suggest that measles might be the cause but as observed by Yassin et at. (1970) antibody examination or the appearance of the rash after the onset of the intracranial lesions, as occasionally happens, is a useful pointer to the aetiology. When the otolaryngologist eventually sees these cases, he has to consider other causes such as viral inner ear lesions, drugs and sickle cell disorder. Except in a few cases such a diagnostic exercise is merely of academic significance. Comment The practice in some Western countries of deliberately exposing a child to measles in the hope of achieving immunity at a time convenient to the family cannot be considered in parts of the tropics in which the infection produces a high mortality. The otolaryngologist here frequently sees complications of measles more varied and severe than those regularly seen in developed countries, but it is not possible to say what proportion of measles cases actually develop them. Some of these complications are reminiscent of diseases recorded in Western countries in the past among the under-privileged, and occasionally in recent times in conditions of privation and neglect (Eckstein, 1940; Linenberg et al., 1961). Although one of these diseases, noma (or cancrum), has followed a variety of severely debilitating diseases, measles is easily the most common precursor. It is 638

Otolaryngological complications of measles in West Africa noteworthy that in East Africa where measles is not as dangerous a disease as it is in West Africa (Tempest, 1966, quoting Fulmer) it does not feature in the aetiology of noma. The non-gangrenous ulcerations described earlier are not considered a milder form of the gangrenous lesion because the former are predictable in their extent of spread and have a different microbial aetiology. It is felt that the contributing factors are a combination of epithelial instability and increased vulnerability of the skin due perhaps to hypo-vitaminosis. In such a state rubbing or scratching an itching nose, external auditory meatus or an eye in which corneal desquamation has commenced, provides the trauma which starts off or worsens the ulceration. Among all the complications described the neuro-audiologic ones are not only permanent but also the most handicapping and are so far not amenable to surgical or medical therapy. Perhaps the anti-measles campaigns may mitigate the severity or frequency of complications but meanwhile improvement in standards of living and the health services, as well as the provision of suitable rehabilitative services, should go a long way to reduce the physical and psychological trauma inflicted by these diseases on both patient and parent. Summary In Nigeria as in West Africa generally measles is a serious condition with a high morbidity and mortality rate. Complications involve every section of Otolaryngology. Some of these are amenable to treatment by the otolaryngologist alone, while others need the skill of colleagues in other specialities. The irreversible neuro-audiological complications cause greatest concern to physician and parent and will continue to do so until the variety of available therapeutic, preventive and rehabilitative services are increased and improved. REFERENCES D E BUSE, P. J., LEWIS, M. G., and MUGERWA, J. W. (1970) Journal of Tropical

Paediatrics, 16, 197. ECKSTEIN, A. (1940) American Journal of diseases of Children, 59, 219. FAMILUSI, J. B., and BOHRER, S. P., (1972) Zectschrift Tropenmeo Parasitol, 23, 121. HENDRICKSE, R. G, (1964) Practitioner, 193, 146. , MONTEFIORE, D., SHERMAN, P. M., and VAN D E R WALL, H. M. (1964) British

Medical Journal, 5381, 470. LINENBERG, W. B., SCHMITT, J., and HARPOLE, H. J. (1961) Oral Surgery, 14, 1138.

MORLEY, D. C. (1962) American Journal of diseases of children, 103, 230. MORLEY, D., WOODLAND, M., and MARTIN, W. J. (1963) Journal of Hygiene (Cam-

bridge), 61, 115. RANSOME KUTI, O., VEIGA-PIRES, J. A., and AUDIT, I. S. (1968). Clinical

19, 47 SAMAKHALOVA, A. S. (1968) Vestnick Oto-Rhino-Laringologii, 30, 73. SINGH, S. P. (1971) Personal communication.

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YASSIN, M. W., GIRGIS, N. I., ABDEL, WAHAB K. E., EL MASRY, N. A., and ABU

EL ELA, A. (1970) Journal of Tropical Paediatrics, 16, 179. Dept. of Oto-Rhino-Laryngology, University of Ibadan, Ibadan, Nigeria.

Otolaryngological complications of measles in West Africa.

In Nigeria as in West Africa generally measles is a serious condition with a high morbidity and mortality rate. Complications involve every section of...
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