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Cancrum Oris (Noma): The Role of Nutrition in Management
Journal of the American College of Clinical Wound Specialists (2016) -, -–-
1 2 3 4 5 6 7 8 Q1 9 10 11 12 Q2 13 14 15 16 Q9 17 18 19 Q3 20 21 Q4 22 23 24 25 26 27 28 29 30 31 32 33 Q5 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61
CASE STUDY
Cancrum Oris (Noma): The Role of Nutrition in Management Elroy Patrick Weledji, BSC, MSC, MBBCHBAO, FRCS (Edin)*, Sylvia Njong, MD Department of Surgery, Faculty of Health Sciences, University of Buea, Cameroon KEYWORDS: Cancrum oris (noma); Nutrition
Abstract Cancrum oris (noma) is caused by the spirochete Borrelia vincenti in association with anaerobic bacteria, commonly a member of the fusobacteria. The disease occurs in deprived and undernourished individuals with poor hygiene. We present a case of clinically diagnosed cancrum oris in a 2-year old child in sub-saharan Africa that was conservatively managed by wound care and hyperalimentation. As the underlying factor and cause of mortality from noma is the immune depression of malnutrition, the role of active nutrition or hyperalimentation in the management of noma is extremely important. Ó 2016 Elsevier Inc. All rights reserved.
Main lesson: The role of active nutrition or hyperalimentation in the management of cancrum oris (noma) is extremely important.
Case Report A 2-year old African girl presented with a painful swelling in the submental region of 4 days duration. This developed from an ulcer at the corner of her right upper lip that had occurred a week earlier. The swelling progressed rapidly and was associated with intermittent fever. On
Competing interests: None. Funding: None declared. Ethical approval: Written informed consent from the patient was granted to write and publish the paper and associated images. Guarantor: Prof Marcelin NGOWE NGOWE, Dean of the Faculty of Health Sciences, University of Buea. Contributorship: EPW is the surgeon who contributed to the arrangement, writing, editing and literature search; SN was the primary care physician and contributed to the literature search. Conflict of interest: None. * Corresponding author. P.O. Box 126, Limbe, S.W.Region, Cameroon. E-mail address: [email protected]
examination the patient was acutely malnourished and weighed 10 kg. She was pale, pyrexic (temperature 239 C), tachycardic and tachypnoiec. Extraoral examination revealed a tender 4 cm submental swelling, which was fluctuant warm with an erythematous base. There was mild crusting of the overlying smooth and shiny skin and a central punctum (Fig. 1). There were no skin lesions involving the palms and soles suggestive of yaws. The initial presentation was suggestive of a submental abscess with a differential diagnosis of a cellulitis. Investigations showed anemia (hemoglobin level of 7.6 g/dl), a leukocytosis (WBC 22 ! 109), a raised erythrocyte sedimentation rate (ESR 35 mm/h) and her HIV serology was negative. Following incision and drainage of the abscess and parenteral amoxycilline-clavulinic acid 80 mg/kg/24 h in 2 divided doses, the ulcer rapidly extended to the submandibular region, the corner of the mouth and to the preauricular region. This was managed from the second day of admission, with daily wound debridement, irrigation with hydrogen peroxide, saline and 0.2% chlorhexidine, parenteral antibiotherapy (amoxicillin-clavulanic acid 80 mg/ kg/24 h in 2 divided doses, metronidazole 7.5 mg/kg every 8 h s for 7 days, and optimized treatment of acute malnutrition with starter (F75) and catch-up feed (F100)
2213-5103/$ - see front matter Ó 2016 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jccw.2016.08.003
CRP 5.4.0 DTD - JCCW62_proof - 29 August 2016 - 4:20 pm - ce
62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 96 97 98 99 100 101 102 103 104 105 106 107 108 109 110 111 112 113 114 115 116 117 118 119 120 121 122 123 124 125 126 127 128
Journal of the American College of Clinical Wound Specialists, Vol -, No -
print & web 4C=FPO
129 130 131 132 133 134 135 136 137 138 139 140 141 142 143 144 145 146 147 148 149 150 151 152 153 154 155 Q6 156 157 158 159 160 161 162 163 164 165 166 167 168 169 170 171 172 173 174 175 176 177 178 179 180 181 182 183 184 185 186 187 188 189 190 191 192 193 194 195
196 197 198 199 200 201 202 203 204 205 206 207 208 209 210 211 212 213 214 215 216 217 218 219 220 221 222 223 224 225 226 227 228 229 230 231 232 233 234 235 236 237 238 239 240 241 242 243 244 245 246 247 248 249 250 251 252 253 254 255 256 257 258 259 260 261 262
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2
Figure 1
Cancrum oris (noma).
(Table 1). The feed were locally made with the contents as shown in the table. After 1 week of treatment the evolution was remarkable and the wound healed by the end of the second week (Fig. 2) (Table 2).
Discussion We describe an acutely ill 2-year old African girl with severe protein deficiency that preceded a clinically diagnosed cancrum oris (noma). ‘Noma’ from the Greek word ‘nomien’ means to devour. Cancrum oris (noma) affects children between 2 and 16 years of age with a peak incidence at ages 1–4 years after cessation of breast feeding. The global incidence is quite high, at 140,000 new cases per year but has disappeared in the developed world. It usually occurs in children who are recovering from a debilitating illness, commonly measles and may coincide with the period of linear growth retardation in
Table 1
Spirochatal infections.9
Disease
Organism
Syphilis Bejel (endemic non-venereal syphilis) Yaws Pinta Leptospirosis (Weil’s disease) Canicola fever Louse-borne relapsing fever Tick-borne relapsing fever Cancrum oris Lyme disease Rat-bite fever
Treponema pallidum Treponema pallidum variant Trepenoma pertenue Treponema carateum Leptospira icterohaemorrhagiae Leptospira canicola Borrelia recurrentis Borrelia duttonii Borrelia vincenti Borrelia burgdorferi Spirillum minus Streptobacillus monoliformis
Figure 2 Wound healed by week 2 following conservative treatment.
deprived children.1–7 It is caused by the sprochaete Borrelia vincenti in association with anaerobic bacteria commonly a member of the fusobacteria. Rapidly progressive gangrenous destruction of the inner aspect of the mouth and cheek occurs, which may continue to progress even after treatment with penicillin.1,8 The impaired feeding in the sick child with the oral lesion and deformity will further exacerbate the malnourished condition. The mortality rate from the complications, septicemia, severe dehydration and rarely hemorrhage is as high as 70–90% if not promptly treated, and probably reflects the underlying poor physical state of the patient.3–6 Malnutrition leads to dysregulated immunity with the release of cytokines. Vitamin A and zinc deficiencies are characterized by diminished cellmediated immunity, early breakdown in the integrity of epithelial tissue and pathologic alterations of the oral mucosa. These predispose B. vincenti infection in association with anaerobic bacteria. The massive release of anaerobic bacterial proteolytic enzymes causes a necrotizing fasciitis.1,8 The etiology is interestingly similar to ‘tropical ulcer’ usually due to fusiforms and B. vincenti following minor trauma, especially in malnourished young people in the tropics.9 The differential diagnosis include spirochetal infections due to Treponema (syphilis, bejel, yaws and pinta) and Borrelia (the relapsing fevers, tropical ulcer) (Table 1)9 Yaws caused by Treponema pertenue spreads by direct contact, usually in children where the organism again enters through damaged skin. However, dissemination of the organism leads to papular skin lesions containing treponema involving the palms and soles. The gross destruction and disfigurement particularly of the skull and facial bones occur from late yaws bony lesions.9 The other Borrelia infections are characterized by a relapsing fever with the spirochetes demonstrated microscopically in the blood during the febrile illnesses (Table 1). Plasma-free aminoacid profiles in noma patients were consistent with the syndrome of protein-energy
CRP 5.4.0 DTD - JCCW62_proof - 29 August 2016 - 4:20 pm - ce
Weledji and Njong
Cancrum oris and nutrition
263 Q7 Table 2 Nutrition recipe. 264 265 Initial feeding with locally Catch-up feeding with 266 made F75 (cereal-based starter) locally made F100. 267 1000 ml water 1000 ml of water 268 1 egg 80 g of dried 269 270 skimmed milk 271 25 g dried skimmed milk 60 g vegetable oil 272 70 g sugar 50 g sugar 273 125 g cereal 1 amp kcl 274 27 g vegetable oil 1 amp Nacl 275 Q8 1 amp Kcl 276 1 amp Nacl 277 278 279 280 281 malnutrition (PEM).1,2,6,7 Most of the children with noma 282 showed very marked depletion of plasma retinol, zinc and 283 ascorbate, and, hence the need for their supplementation.1 284 285 The management of noma requires a multidisciplinary 286 team approach. In the acute stage of cancrum oris (noma) 287 the role of surgery is a minor one: wound care. Wound 288 care entails topical irrigation with hydrogen peroxide, sa289 line and 0.2% chlorhexidine to deslough the necrotic tissue. 290 291 Adequate hydration, correction of electrolytes and vitamin 292 deficiencies with provision of sufficient nutritional support 293 is essential. Antibiotics will arrest or retard the progression 294 into septicemia.1,3,7 Late stage treatment may require plas295 tic/reconstructive surgery of the extensive facial ulceration, 296 297 but this can be avoided with adequate nutrition therapy as 298 in this case.10 In patients who survive noma, and develop 299 a mutilated and disabled face (trismus, leakage of saliva, 300 impaired speech), reconstructive surgery may improve their 301 fate significantly. Treatment consists of excision of all scar 302 303 304 305 306 307 308 309 310 311 312 313 314 315 316 317 318 319 320 321 322 323 324 325 326 327 328 329
3 tissue, correction of the trismus and closure of the tissue defects with local, pedicled or free flaps.11
Acknowledgments We acknowledge the photographs from the Muea Health centre.
References 1. Enwonwu CO, Falkler WA Jr., Phillips RS: Noma (cancrum oris). Lancet. 2006;368:147–156. 2. Berthold P: Noma: a forgotten disease. Dent Clin North Am. 2003;47: 559–574. 3. Barrera J, Connor MP: Noma in an Afghani child: a case report. Int J Pediatr Otorhinolaryngol. 2012;76:742–744. 4. Masipa JN, Baloyi RA, Khammissa G, Altini M, Lemmer J, Feller L: Noma (Cancrum Oris): a report of a case in a young AIDS patient with a review of the pathogenesis. Head Neck Pathol. 2013;7(2):188–192. 5. Phiri R, Feller L, Blignaut E: The severity, extent and recurrence of necrotizing periodontal disease in relation to HIV status and CD41 T cell count. J Int Acad Periodontol. 2010;12:98–103. 6. Idigbe EO, Enwonwu CO, Falkler WA, et al: Living conditions of children at risk for noma: Nigerian experience. Oral Dis. 1999;5:156–162. 7. Ogbureke KU, Ogbureke EI: NOMA: a preventable ‘‘scourge’’ of African children. Open Dent J. 2010;4:201–206. 8. Falkler WA Jr., Enwonwu CO, Idigbe EO: Microbiological understandings and mysteries of noma (cancrum oris). Oral Dis. 1999;5:150–155. 9. Farthing MJG, Rolston DDK: Infectious diseases and tropical medicine. In: Kumar Parveen J, Clark Michael L, editors. Clinical Medicine. Balliere Tindall, 2000. 10. Marck KW, de Bruijn HP: Surgical treatment of noma. Oral Dis. 1999; 5:167–171. 11. Valadas G, Leal MJ: Cancrum oris (noma) in children. Eur J Paed Surg. 1998;8:47–51.
CRP 5.4.0 DTD - JCCW62_proof - 29 August 2016 - 4:20 pm - ce
330 331 332 333 334 335 336 337 338 339 340 341 342 343 344 345 346 347 348 349 350 351 352 353 354 355 356 357 358 359 360 361 362 363 364 365 366 367 368 369 370 371 372 373 374 375 376 377 378 379 380 381 382 383 384 385 386 387 388 389 390 391 392 393 394 395 396
1 2 3 4 5 6 7 8 Q1 9 10 11 12 Q2 13 14 15 16 Q9 17 18 19 Q3 20 21 Q4 22 23 24 25 26 27 28 29 30 31 32 33 Q5 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61
CASE STUDY
Cancrum Oris (Noma): The Role of Nutrition in Management Elroy Patrick Weledji, BSC, MSC, MBBCHBAO, FRCS (Edin)*, Sylvia Njong, MD Department of Surgery, Faculty of Health Sciences, University of Buea, Cameroon KEYWORDS: Cancrum oris (noma); Nutrition
Abstract Cancrum oris (noma) is caused by the spirochete Borrelia vincenti in association with anaerobic bacteria, commonly a member of the fusobacteria. The disease occurs in deprived and undernourished individuals with poor hygiene. We present a case of clinically diagnosed cancrum oris in a 2-year old child in sub-saharan Africa that was conservatively managed by wound care and hyperalimentation. As the underlying factor and cause of mortality from noma is the immune depression of malnutrition, the role of active nutrition or hyperalimentation in the management of noma is extremely important. Ó 2016 Elsevier Inc. All rights reserved.
Main lesson: The role of active nutrition or hyperalimentation in the management of cancrum oris (noma) is extremely important.
Case Report A 2-year old African girl presented with a painful swelling in the submental region of 4 days duration. This developed from an ulcer at the corner of her right upper lip that had occurred a week earlier. The swelling progressed rapidly and was associated with intermittent fever. On
Competing interests: None. Funding: None declared. Ethical approval: Written informed consent from the patient was granted to write and publish the paper and associated images. Guarantor: Prof Marcelin NGOWE NGOWE, Dean of the Faculty of Health Sciences, University of Buea. Contributorship: EPW is the surgeon who contributed to the arrangement, writing, editing and literature search; SN was the primary care physician and contributed to the literature search. Conflict of interest: None. * Corresponding author. P.O. Box 126, Limbe, S.W.Region, Cameroon. E-mail address: [email protected]
examination the patient was acutely malnourished and weighed 10 kg. She was pale, pyrexic (temperature 239 C), tachycardic and tachypnoiec. Extraoral examination revealed a tender 4 cm submental swelling, which was fluctuant warm with an erythematous base. There was mild crusting of the overlying smooth and shiny skin and a central punctum (Fig. 1). There were no skin lesions involving the palms and soles suggestive of yaws. The initial presentation was suggestive of a submental abscess with a differential diagnosis of a cellulitis. Investigations showed anemia (hemoglobin level of 7.6 g/dl), a leukocytosis (WBC 22 ! 109), a raised erythrocyte sedimentation rate (ESR 35 mm/h) and her HIV serology was negative. Following incision and drainage of the abscess and parenteral amoxycilline-clavulinic acid 80 mg/kg/24 h in 2 divided doses, the ulcer rapidly extended to the submandibular region, the corner of the mouth and to the preauricular region. This was managed from the second day of admission, with daily wound debridement, irrigation with hydrogen peroxide, saline and 0.2% chlorhexidine, parenteral antibiotherapy (amoxicillin-clavulanic acid 80 mg/ kg/24 h in 2 divided doses, metronidazole 7.5 mg/kg every 8 h s for 7 days, and optimized treatment of acute malnutrition with starter (F75) and catch-up feed (F100)
2213-5103/$ - see front matter Ó 2016 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jccw.2016.08.003
CRP 5.4.0 DTD - JCCW62_proof - 29 August 2016 - 4:20 pm - ce
62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 96 97 98 99 100 101 102 103 104 105 106 107 108 109 110 111 112 113 114 115 116 117 118 119 120 121 122 123 124 125 126 127 128
Journal of the American College of Clinical Wound Specialists, Vol -, No -
print & web 4C=FPO
129 130 131 132 133 134 135 136 137 138 139 140 141 142 143 144 145 146 147 148 149 150 151 152 153 154 155 Q6 156 157 158 159 160 161 162 163 164 165 166 167 168 169 170 171 172 173 174 175 176 177 178 179 180 181 182 183 184 185 186 187 188 189 190 191 192 193 194 195
196 197 198 199 200 201 202 203 204 205 206 207 208 209 210 211 212 213 214 215 216 217 218 219 220 221 222 223 224 225 226 227 228 229 230 231 232 233 234 235 236 237 238 239 240 241 242 243 244 245 246 247 248 249 250 251 252 253 254 255 256 257 258 259 260 261 262
print & web 4C=FPO
2
Figure 1
Cancrum oris (noma).
(Table 1). The feed were locally made with the contents as shown in the table. After 1 week of treatment the evolution was remarkable and the wound healed by the end of the second week (Fig. 2) (Table 2).
Discussion We describe an acutely ill 2-year old African girl with severe protein deficiency that preceded a clinically diagnosed cancrum oris (noma). ‘Noma’ from the Greek word ‘nomien’ means to devour. Cancrum oris (noma) affects children between 2 and 16 years of age with a peak incidence at ages 1–4 years after cessation of breast feeding. The global incidence is quite high, at 140,000 new cases per year but has disappeared in the developed world. It usually occurs in children who are recovering from a debilitating illness, commonly measles and may coincide with the period of linear growth retardation in
Table 1
Spirochatal infections.9
Disease
Organism
Syphilis Bejel (endemic non-venereal syphilis) Yaws Pinta Leptospirosis (Weil’s disease) Canicola fever Louse-borne relapsing fever Tick-borne relapsing fever Cancrum oris Lyme disease Rat-bite fever
Treponema pallidum Treponema pallidum variant Trepenoma pertenue Treponema carateum Leptospira icterohaemorrhagiae Leptospira canicola Borrelia recurrentis Borrelia duttonii Borrelia vincenti Borrelia burgdorferi Spirillum minus Streptobacillus monoliformis
Figure 2 Wound healed by week 2 following conservative treatment.
deprived children.1–7 It is caused by the sprochaete Borrelia vincenti in association with anaerobic bacteria commonly a member of the fusobacteria. Rapidly progressive gangrenous destruction of the inner aspect of the mouth and cheek occurs, which may continue to progress even after treatment with penicillin.1,8 The impaired feeding in the sick child with the oral lesion and deformity will further exacerbate the malnourished condition. The mortality rate from the complications, septicemia, severe dehydration and rarely hemorrhage is as high as 70–90% if not promptly treated, and probably reflects the underlying poor physical state of the patient.3–6 Malnutrition leads to dysregulated immunity with the release of cytokines. Vitamin A and zinc deficiencies are characterized by diminished cellmediated immunity, early breakdown in the integrity of epithelial tissue and pathologic alterations of the oral mucosa. These predispose B. vincenti infection in association with anaerobic bacteria. The massive release of anaerobic bacterial proteolytic enzymes causes a necrotizing fasciitis.1,8 The etiology is interestingly similar to ‘tropical ulcer’ usually due to fusiforms and B. vincenti following minor trauma, especially in malnourished young people in the tropics.9 The differential diagnosis include spirochetal infections due to Treponema (syphilis, bejel, yaws and pinta) and Borrelia (the relapsing fevers, tropical ulcer) (Table 1)9 Yaws caused by Treponema pertenue spreads by direct contact, usually in children where the organism again enters through damaged skin. However, dissemination of the organism leads to papular skin lesions containing treponema involving the palms and soles. The gross destruction and disfigurement particularly of the skull and facial bones occur from late yaws bony lesions.9 The other Borrelia infections are characterized by a relapsing fever with the spirochetes demonstrated microscopically in the blood during the febrile illnesses (Table 1). Plasma-free aminoacid profiles in noma patients were consistent with the syndrome of protein-energy
CRP 5.4.0 DTD - JCCW62_proof - 29 August 2016 - 4:20 pm - ce
Weledji and Njong
Cancrum oris and nutrition
263 Q7 Table 2 Nutrition recipe. 264 265 Initial feeding with locally Catch-up feeding with 266 made F75 (cereal-based starter) locally made F100. 267 1000 ml water 1000 ml of water 268 1 egg 80 g of dried 269 270 skimmed milk 271 25 g dried skimmed milk 60 g vegetable oil 272 70 g sugar 50 g sugar 273 125 g cereal 1 amp kcl 274 27 g vegetable oil 1 amp Nacl 275 Q8 1 amp Kcl 276 1 amp Nacl 277 278 279 280 281 malnutrition (PEM).1,2,6,7 Most of the children with noma 282 showed very marked depletion of plasma retinol, zinc and 283 ascorbate, and, hence the need for their supplementation.1 284 285 The management of noma requires a multidisciplinary 286 team approach. In the acute stage of cancrum oris (noma) 287 the role of surgery is a minor one: wound care. Wound 288 care entails topical irrigation with hydrogen peroxide, sa289 line and 0.2% chlorhexidine to deslough the necrotic tissue. 290 291 Adequate hydration, correction of electrolytes and vitamin 292 deficiencies with provision of sufficient nutritional support 293 is essential. Antibiotics will arrest or retard the progression 294 into septicemia.1,3,7 Late stage treatment may require plas295 tic/reconstructive surgery of the extensive facial ulceration, 296 297 but this can be avoided with adequate nutrition therapy as 298 in this case.10 In patients who survive noma, and develop 299 a mutilated and disabled face (trismus, leakage of saliva, 300 impaired speech), reconstructive surgery may improve their 301 fate significantly. Treatment consists of excision of all scar 302 303 304 305 306 307 308 309 310 311 312 313 314 315 316 317 318 319 320 321 322 323 324 325 326 327 328 329
3 tissue, correction of the trismus and closure of the tissue defects with local, pedicled or free flaps.11
Acknowledgments We acknowledge the photographs from the Muea Health centre.
References 1. Enwonwu CO, Falkler WA Jr., Phillips RS: Noma (cancrum oris). Lancet. 2006;368:147–156. 2. Berthold P: Noma: a forgotten disease. Dent Clin North Am. 2003;47: 559–574. 3. Barrera J, Connor MP: Noma in an Afghani child: a case report. Int J Pediatr Otorhinolaryngol. 2012;76:742–744. 4. Masipa JN, Baloyi RA, Khammissa G, Altini M, Lemmer J, Feller L: Noma (Cancrum Oris): a report of a case in a young AIDS patient with a review of the pathogenesis. Head Neck Pathol. 2013;7(2):188–192. 5. Phiri R, Feller L, Blignaut E: The severity, extent and recurrence of necrotizing periodontal disease in relation to HIV status and CD41 T cell count. J Int Acad Periodontol. 2010;12:98–103. 6. Idigbe EO, Enwonwu CO, Falkler WA, et al: Living conditions of children at risk for noma: Nigerian experience. Oral Dis. 1999;5:156–162. 7. Ogbureke KU, Ogbureke EI: NOMA: a preventable ‘‘scourge’’ of African children. Open Dent J. 2010;4:201–206. 8. Falkler WA Jr., Enwonwu CO, Idigbe EO: Microbiological understandings and mysteries of noma (cancrum oris). Oral Dis. 1999;5:150–155. 9. Farthing MJG, Rolston DDK: Infectious diseases and tropical medicine. In: Kumar Parveen J, Clark Michael L, editors. Clinical Medicine. Balliere Tindall, 2000. 10. Marck KW, de Bruijn HP: Surgical treatment of noma. Oral Dis. 1999; 5:167–171. 11. Valadas G, Leal MJ: Cancrum oris (noma) in children. Eur J Paed Surg. 1998;8:47–51.
CRP 5.4.0 DTD - JCCW62_proof - 29 August 2016 - 4:20 pm - ce
330 331 332 333 334 335 336 337 338 339 340 341 342 343 344 345 346 347 348 349 350 351 352 353 354 355 356 357 358 359 360 361 362 363 364 365 366 367 368 369 370 371 372 373 374 375 376 377 378 379 380 381 382 383 384 385 386 387 388 389 390 391 392 393 394 395 396