Cancrum oris in an adult caucasian female

British Journal of Oral and Maxiilofacial Surgery (1989) 27, 411-422 0 1989 The British Association of Oral and Maxillofacial Surgeons 0266-4356x/89/0027-0417/$10.00

CANCRUM

ORIS IN AN ADULT

CAUCASIAN

FEMALE

L. F. A. STASSEN,~. M.A., F.D.s.R.c.s., F.R.c.s., A. G. G. BATCHELOR,”F.R.c.s., J. S. RENNIE,“” Ph.D., B.D.s., F.D.s.R.c.P.s., M.R.C. Path. and K. F. Moos,? M.B., B.S., F.D.S.R.C.S., F.D.S.R.C.P.S., F.R.C.S. fCanniesburn Hospital, Bearsden, Glasgow G61 lQL, *St James’s University Hospital, Leeds L58 7TH and *“Department of Oral Medicine and Pathology, Glasgow Dental Hospital, Glasgow G2 3JZ Summary. This paper reports a case of cancrum oris in an adult Caucasian female who had no obvious pre-disposing cause. It highlights the difficulties in making this diagnosis in a part of the world unaccustomed to seeing the disease and it illustrates that early surgery and reconstruction can be undertaken. To our knowledge, this is the first published case of cancrum oris reconstructed with the use of micro-vascular free tissue transfer.

Introduction

Cancrum oris most commonly affects young black Africans (Emslie, 1963; Tempest, 1966; Adekeye & Ord, 1983) who are often immunocompromised (Tempest, 1966; Agnew, 1947; Bend1 et al., 1983) or ‘undernourished (Enwonwu, 1972). The most important predisposing causes have been reported as measles, scarlet fever, typhoid, syphilis, tuberculosis and the leukaemias (Stones, 1954). Emslie (1963) considers malnutritrion to be a secondary feature of the disease but most other authors believe malnutrition to be an important predisposing factor (Tempest, 1966; Enwonwu, 1972). The disease is rare in adults, unless they are compromised by a lymphoreticular disorder or other severe debilitating disease (Dawson, 1945; Adelsberger, 1946; Malden, 1985; Tempest, 1966; Bend1 et al., 1983), but Archer (1966) gives a short case report of an adult patient who developed cancrum oris following extraction of two teeth. This patient unfortunately rapidly succumbed to the disease and a predisposing factor was not elucidated. Cancrum oris has been classified into three types, labiomental, buccal and labiomaxillary. In the labiomental form, the lesion involves the gingivae and then extends into the bone and cheek. Clinical examination shows a painful red or dusky red lesion on the alveolus in the premolar or molar region. This enlarges, ulcerates and exposes underlying bone. In the other two types the lesion commences in the soft tissues at the commissure of the lips and only later invades the adjacent bone (Mead, 1946; Emslie, 1963). It is usually unilateral but may be bilateral (McGregor, 1958). The disease progresses rapidly leading to destruction of bone and soft tissues (Osler, 1901), with the intra-oral destruction of tissue being much greater than the apparent extra-oral disease (Tempest, 1966). The disease is frequently associated with marked trismus (Tempest, 1966; Adekeye & Ord, 1983; Adekeye et al., 1986), which is thought to be a consequence of involvement of the floor of mouth, cheek and the extra-capsular structures of the T.M.J., by the infiltrating necrotising and (Received 15 August

1988; accepted 3 October

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fibrosing process. The masseter, medial and lateral pterygoid and the temporalis muscles are only occasionally involved in this disease process. Clinically the disease appears to be infective in origin, with recurrent spikes of temperature and an associated mild leucocytosis (Osler, 1901; Archer, 1966). Microbiology has failed to identify a specific pathogen but the organisms that appear to be of greatest significance are Borrelia vincenti and Fusobacterium nucleatum (Fusiformis fusiformis or Bacillus fusiformis). Bacteroides melaninogenicus may also be present (Uohara & Knapp, 1967). The histopathological features are not specific but show areas of necrosis, a heavy mixed inflammatory infiltrate, peripheral fibrosis and widespread tissue destruction. The diagnosis is based on suspicion and once seen the condition is never again mistaken. Treatment may be divided into three parts: supportive, debridement and reconstruction. Supportive therapy includes rehydration, correction of anaemia and malnutrition and the use of parenteral antibiotics. .Generally the patient responds to this regimen and the lesion will start to demarcate. Debridement should be radical as the disease is always more extensive than is clinically apparent. An interval between debridement and reconstruction is usually advised to ensure that the necrotic process has been controlled. Reconstruction must be with well perfused, healthy tissue and therefore the use of distant tissue is recommended such as a delto-pectoral or a pectoralis major myocutaneous flap (Adekeye & Ord, 1983; Adekeye et al., 1986). When the disease progress was not arrested by rehydration and transfusion, the mortality was as high as 95% (Eckstein, 1940). The outcome of the disease has been strikingly improved by the use of antibiotics, especially penicillin and the mortality rate is now 8 to 10% (Tempest, 1966; Eckstein, 1940). Morbidity remains high and even with modern reconstructive methods there is extensive facial deformity. This case report records the unusual presentation of cancrum oris in a healthy white adult Caucasian female in a part of the world where the disease is very uncommon. Case report

A 63-year-old Caucasian female was referred in September 1985 to the Oral and Maxillofacial Unit, Canniesburn Hospital in a depressed, feeble and toxic state, firmly convinced she had cancer. She gave a history of requiring dental extractions 5 months previously, due to pain in her left lower molars. These extractions were complicated by pain, delayed healing and the development of a tumour like lesion on her left mandibular alveolus which eventually ulcerated. She had been thoroughly investigated elsewhere and at that time all haematological investigations had proved normal (Hb 13.1 g/dl, WBC 9.9X109/1, albumin 40 g/l), bacteriological investigation revealed no growth and histological examination showed a non-specific chronic inflammatory reaction. Despite several courses of antibiotics, the condition of the patient continued to deteriorate with progressive weight loss, depression, lethargy and the development of a dusky blue area affecting the left cheek. There was a history of recurrent spikes of temperature, sweating attacks with the patient losing approximately 10 kg in weight over the previous 5 months. Her medical history revealed that she had been taking prednisolone 5 mg and phyllocontin 225 mg daily for chronic obstructive airways disease and propranolol 40 mg b.d. for hypertension. The patient was a non-drinker and a non-smoker.

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On examination, the patient was thin, pale and pyrexial (39°C) with a 3x3 cm, full thickness, indurated, dusky blue lesion of the left cheek (Figs. 1 & 2). There was complete left labial anaesthesia. There was no, associated lymphadenopathy. The patient was edentulous and had marked scarring and induration of the floor of the mouth and left mandibular alveolus. There was moderate trismus with opening between the upper and lower alveolus limited to 2 cm. Ward temperature screening showed recurrent spikes of temperature (40°C) at irregular intervals. The patient was normotensive and haemotological investigations now showed a normochromic, normocytic anaemia (8.8 g/dl) and a mild neutrophilia (14.6~ lO’/l) with an ESR of 68 mm/h. Her albumin was 26 g/l. There was a raised a 1 and a 2 globulin level compatible with an acute-phase reaction. Radiographs revealed a moth-eaten appearance of the left premolar area of the mandible and isotope scanning showed increased uptake in this area but no other abnormalities. Repeated microbiology of tissue specimens from the lesion and multiple blood cultures revealed no causative organism, despite the with-holding of antibiotics for some time. Specific microbiology for actinomyces and unusual fungal infections was negative. Mycobacteria could not be identified and serology for syphilis was negative. There was no history of radiation to the area. The patient was taken to the operating theatre, 3 weeks after her admission, for a further biopsy and debridement of the area. At operation the commissure of the lip, which was necrotic and very friable, disintegrated. Postoperatively the patient was started on metronidazole, netilmicin and high-dose penicillin intravenously. For 3 days, after this limited debridement, the temperature remained normal but

Fig.

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Fig. 2 Figure Figure

l-Full-face

2-Lesion,

view of lesion.

as viewed

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the left side

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then reverted to recurrent spikes. The patient’s morale became very poor and her general condition had deteriorated. It was decided to treat her as an unusual case of cancrum oris, because of the clinical features and the absence of a specific histological or bacteriological diagnosis. Therefore a wide resection was carried out to include a major portion of the left cheek, commissure, left half of the lower lip and one third of the upper lip. A hemi-mandibulectomy excluding the condyle and posterior ramus was necessary. The floor of the mouth, the contents of the left submandibular triangle and the posterior portion of the maxilla were also removed (Fig. 3) and sent for histopathological examination. The post-operative course was marked by an immediate improvement. The patient became more alert, her temperature returned to and remained normal. The specimen consisted of a mandibular resection with overlying skin, lower lip and chin, measuring approximately 110X 70X 50 mm. After trimming and decalcification, blocks were paraffin processed and sections cut at 7 urn and stained with haematoxylin and eosin. Histological examination showed extensive destruction and replacement of the soft tissues by an intense mixed inflammatory infiltrate, in which lymphocytes, macrophages and neutrophil polymorphs were prominent. Necrosis, although present in areas, was not extensive. The most striking feature was the intensity of the inflammatory infiltrate and the replacement of muscle, minor salivary glands and fat by inflammatory cells and extensive fibrosis. In places some anatomical structures remained intact, particularly nerves and small arteries. In the mandible the advancing border of the lesion was asssociated with a prominent periosteal and endosteal new bone formation, but in many areas resorption with extension of the inflammatory process through the cortex was evident.

Fig.

Fig.

3 Figure Figure

3-Extent 4-Result

of resection. of reconstruction

4

CANCRUM

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A variety of special stains and an exhaustive search failed to identify the presence of bacteria, mycobacteria, fungi or uncommon parasitic infection. The histological features were considered entirely consistent with the clinical diagnosis of cancrum oris, but the alternative diagnosis of a malignant lymphoma, most likely histiocytic, was for some time considered. However referral to a specialised centre and immunopathological techniques failed to support this diagnosis and an inflammatory basis for the lesion was considered most likely. It had been intended initially to wait 5 to 6 weeks before attempting reconstruction but it was decided because of the vast improvement in the clinical state to proceed after only 1 week. A radial forearm flap was used to reconstruct the floor of the mouth, its artery and vein were anastomosed, end to end, to the facial vessels of the right side, as the fibrotic reaction surrounding the vessels on the left side made their patency suspect. A deep circumflex iliac artery flap was also raised with skin and iliac bone, the bone pattern being determined by a template taken at the start of the procedure. The iliac bone was wired into position to reconstruct the mandible and the soft tissue used to reconstruct the cheek. The deep circumflex iliac artery was anastomosed to the other end of the radial artery of the free flap, the vein was anastomosed to one of the venae commitantes. Both skin and bone were satisfactorily perfused by the deep artery. The superficial circumflex iliac vein was anastomosed to the remaining vena commitants. Her post-operative recovery was uneventful and the patient was sent home 2 weeks later, eating, drinking and speaking well. She has been followed up for 2 years and there has been no evidence of any recurrence of the disease (Fig. 4). Discussion

This case of cancrum oris in a Caucasian adult, with no obvious predisposing cause is unusual. It is possible that her initial pain was caused by a localised acute necrotising gingivitis, which was aggravated by the extraction of the molar teeth. The steroid dose, that was taken for her chest disease, was not considered to have induced a significant degree of immunodepression but it may have influenced the progress of the disease. There was no evidence of malnutrition. There was no history of a debilitating disease, or of radiotherapy to the area. Full haematological investigations at her initial presentation elsewhere were normal (6 weeks prior to her admission to Canniesburn). At operation the extent of the lesion including its involvement of the posterior part of the maxilla and the pterygoid region was surprising. The fibrosis, involving the floor of mouth and cheek, appeared to be the cause of the trismus. Her recovery following surgery was rapid. In view of the dramatic response to debridement, early reconstruction was undertaken. The diagnosis of cancrum oris was made late and was only confirmed by the clinical behaviour of the condition and its response to treatment. The histological features, although not diagnostic, are entirely consistent with a diagnosis of cancrum oris. The margins of the resection were only just clear despite the wide resection undertaken. Although there were several options available for reconstruction of the defect, it was.felt that the combination of two free flaps would provide the required soft and hard tissue. The iliac bone was chosen to give the length, height and width required to reconstruct the mandible and its associated soft tissue were used to reconstruct the cheek and lip. The bone taken from the ilium was cut using an accurate

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template. Bone from the radial forearm or a serratus anterior rib flap would have given satisfactory length but their shape and height were unsuitable for later denture reconstruction. The floor of the mouth also required reconstruction. Radial forearm free flaps are relatively easy to raise and have large vessels for anastomising, additionally they provide skin of a satisfactory thickness and pliability to allow more free tongue movements than other reconstructive methods. Delto-pectoral flaps are poor for intra-oral lining because of their length, fistula formation and the high incidence of tip necrosis of the flap. Forehead flaps are very satisfactory for reconstruction but the poor cosmetic result at the donor site makes it less acceptable. Pectoralis major myo-cutaneous or other such flaps, when used for floor of mouth reconstruction, are bulky, inflexible and can lead to feeding difficulties because of a tendency to funnel later, due to the pull of gravity and fibrosis of the flap. In this case the radial vessels were anastomosed to the facial vessels of the other side, which was possible because of the length of the radial vessels that can be raised. The radial vessels allow a through flow and as the flow was satisfactory the deep circumflex iliac artery and vein were anastomosed onto them. The use of one major flap piggy-backed onto another is open to question, but it was felt at the time that perfusion of both flaps was satisfactory. Had there been the slightest doubt about either anastomosis, it would not have been undertaken. This case presented great difficulties in diagnosis and management. The decision to treat it as a case of cancrum oris, we feel was correct. The decision to undertake such a large resection with no histological diagnosis was not an easy one and it was only because of the extraordinary response to excision of the lesion, that early reconstruction was undertaken. References Adelsberger, L. (1946). Medical observations in Auschwitz concentration camp. Lance-t 1, 317. Adekeye, E. O., Lavery, K. M. & Nasser, N. A. (1986). The versatility of pectoralis major and latissmus dorsi myocutaneous flaps in the reconstruction of cancrum oris defects in children and adolescents. Journal of Maxillofacial Surgery, 14, 99. Adekeye, E. 0. & Ord, R. A. (1983). Cancrum oris: Principles of management and reconstructive surgery. Journal of Maxillofacial Surgery, 14, 99. Agnew, R. G. (1947). Cancrum oris. Journal of Periodontology, 18, 22. Archer (1966). Gangrenous stomatitis following extraction of teeth, case report. In Oral Surgery. 4th Edition. p 740. W. B. Saunders Company. Bendl, B. J., Padmos, A., Harder, E. J. & McArthur, P. D. (1983). Noma: report of three adult cases. Australasian Journal

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Dawson, J. (1945). Cancrum oris. British Dental Journal, 79, 151. Eckstein, A. (1940). Noma. American Journal of Diseases of Children, 59, 219. Emslie, R. D. (1963). Cancrum oris. Dental Practitioner, 13, 481. Enwonwu, C. 0. (1972). Epidemiological and biochemical studies of necrotizing ulcerative gingivitis and noma (cancrum oris) in Nigerian children. Archives of Oral Biology, 17, 1357. Malden, N. (1985). An interesting case of adult facial gangrane (from Papua, New Guinea). Oral Surgery,

Oral Medicine,

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59, 279.

McGregor, M. (1958). Paediatrics in Western Nigeria. Archives of Disease in Childhood, 33, 277. Mead, S. V. (1946). Oral Surgery. 3rd Edition. p 366368. London: Henry Kimpton. Osler, Sir W. (1901). The Principles and Practice of Medicine. 4th Edition. p 444. Edinburgh & London: Young J. Pentland. Stewart, M. J. (1912). Observations on the histopathology of cancrum oris. Journal of Pathology and Bacteriology,

16, 221.

Stones, H. H. (1954). Oral and Dental Diseases. 3rd Edition, Chap. 32, 638-640. Edinburgh: Livingstone. Tempest, M. N; (1966). Cancrum oris. British Journal of Surgery, 53, 949. Uohara, G. I. & Knapp, M. J. (1967). Oral fusospirochaetosis and associated lesions. Oral Surgery, Oral Medicine,

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24, 113.