- Email: [email protected]
Correction of Lip Defect Caused by Noma
Arumsari Asian J Oral MaxillofacMangundjaja, Surg 2002;14:245-249. CASE REPORTS
Correction of Lip Defect Caused by Noma Sunardi Mangundjaja, Asri Arumsari Department of Oral Surgery, Faculty of Dentistry, Padjadjaran University, Bandung, Indonesia
Abstract This report presents a 20-year-old woman undergoing correction of an upper lip deformity secondary to noma. The noma infection was acquired at the age of 3 years, and the defect was left untreated until the age of 12 years, when the patient had an operation at a suburban clinic. She was dissatisfied with the result and came to the Department of Oral Surgery, Padjadjaran University in Indonesia 8 years later, where the correction was achieved in 3 stages. The first operation was performed to bring down the commissure of the lip and to restore normal philtral height. The second operation was performed to achieve the normal position of the lip commissure and to complete the restoration of lip length. Both operations were performed using general anaesthesia. The third operation was a small procedure to correct a minor defect of the lip angle using local anaesthesia. Key Words: Noma, Infection, Lip
Introduction Noma is also known as cancrum oris, gangrenous stomatitis, and necrotising stomatitis.1-6 The origin of the term is the Greek word noma, which means “to devour”.2 Noma is an acute, fulminating, toxic, rapidly progressive gangrenous process of the oral and facial tissues, usually seen in debilitated and poorly nourished children.2,4 Typically, noma presents in children aged between 2 to 10 years1 — an extensive series of 69 Nigerian children between the ages of 2 and 7 years has been reported.4 The most common predisposing factors include malnutrition, dehydration, poor oral hygiene, recent illness, malignancy, and immunodeficiency disorders, including AIDS. Commonly associated illnesses include measles, scarlet fever, and tuberculosis, and leukaemia is one of the more frequently associated malignancies.1-8 Noma is clinically characterised by a rapid, progressive course beginning as a small, painful, red spot or vesicle on the attached gingiva. A necrotic ulcer rapidly forms and exposes underlying bone. Correspondence: Asri Arumsari, Department of Oral Surgery, Faculty of Dentistry, Padjadjaran University, Bandung, Indonesia. E-mail: [email protected]
Asian J Oral Maxillofac Surg Vol 14, No 4, 2002
Stomatitis develops as the lesion extends outward in a cone-like fashion, causing painful cellulitis of the lips and cheeks. The overlying skin becomes inflamed, oedematous, dark, and necrotic. Within a short period, sloughing of the soft tissues occurs, eventually exposing the underlying bone, teeth, and deeper soft tissues.1-6 The odour arising from the gangrenous tissues is foul.4 In many instances, the infection begins as an acute necrotising ulcerative gingivitis (ANUG), and some clinicians believe that noma is merely an extension of the same process. The microbiological findings of patients with noma almost always show infection with Pseudomonas aeruginosa,1,2 often combined with Escherichia coli, Klebsiella species, or Staphylococcus species.1 The inflammatory reaction is initiated by anaerobic bacteria, among which are found fusospirochaetal organisms, such as Borrelia vincentii and Fusobacterium nucleatum (Fusiformis fusiformis or Bacillus fusiformis). Bacteroides melaninogenigus may also be present.2,5,7 Treatment of an infected noma requires the use of appropriate antibiotics and correction of inadequate nutrition, hydration, and electrolyte imbalance. Debridement of the wound and gross necrotic areas is recommended. However, aggressive removal is 245
Correction of Lip Defect Caused by Noma
contraindicated because it does not stop the extension of the process and compounds the reconstruction problems. Necrotic bone is left in place and reconstruction should be delayed for 1 year to ensure complete recovery.1,2 The goals of correction of the residual defect are primarily to improve function and aesthetics. Reconstruction surgery consists of replacing lost tissue by transplanting similar tissue. Complete reconstruction is seldom a single-stage surgery but rather a series of staged procedures.9 The desired soft tissue reconstruction should provide viable tissue bulk with a vascular network. If the vascularity is maintained, the reconstruction tissue will remain supple and heal into the recipient tissue bed with minimal change in dimension.9 Reconstruction with facial flaps is most successful when based laterally or inferiorly, with incisions following normal skinfolds and lines of expression. The disadvantages of these regional flaps include the need for additional incisions and elevation of tissues on the face with resultant scarring.9,10 The feasibility of corrective surgery without the use of distant and complex flaps is unusual. a
Case Report A 20-year-old woman was referred to the Department of Oral Surgery, Padjadjaran University in Indonesia, due to deformity of her right upper lip. When she was 3 years old, she had initially been treated at the department for acute noma infection. After control of the infection, the residual defect was left untreated until she was 12 years of age, when she underwent an operation in a suburban clinic. The secondary deformity of her right upper lip consisted of distortion of the level of the lip, resulting in the lip commissure being almost in contact with the alar base. In addition, the distances of the left and right commissures from the midline were unequal, with the right side shorter than the left side. The lower lip was intact but distorted by the pull of the shortened upper lip on the right side (Figure 1a). Surgery was planned in 2 stages. The first corrective stage was intended to bring down the lip commissure and rotate the philtral column. The second stage was to complete the restoration of the lip length and achieve a normal location for the lip commissure. It was planned to perform both procedures with general anaesthesia. To bring down the lip commissure and restore normal lip length, a rotational flap from the cheek next to the ala nasi was used (Figures 1b and 2a). Excision of the scar from the previous operation was done and dissection performed to reduce the tension in the defect. An inferiorly-based rotation flap of the cheek b
Figure 1. Lip deformity secondary to noma. (a) Before surgery; (b) diagram of deformity with planned rotational flap (stage 1 procedure).
246
Asian J Oral Maxillofac Surg Vol 14, No 4, 2002
Mangundjaja, Arumsari
a
a
b
b
c
Figure 2. (a) Diagram of flap after rotation into recipient site (stage 1 procedure); (b) after first-stage surgery.
(next to the commissure) rotated the philtrum, but the length of the right lip remained short (Figure 2b). The second operation was performed 5 months after the first. To achieve a normal location for the lip commissure, a pedicled flap similar to a Limberg flap was used. To create the vermillion at the defect area, buccal mucosal advancement flaps were utilised (Figure 3). Asian J Oral Maxillofac Surg Vol 14, No 4, 2002
Figure 3. Diagram of stage 2 procedure. (a) The outline of the skin excision. The lower lip was separated from the upper lip; (b) the new commisure created by using pedicle flaps from the mucosa; and (c) after second stage surgery.
247
Correction of Lip Defect Caused by Noma
17 years because of the classic noma-related problem of poverty. As she reached puberty, the patient demanded cosmetic treatment and was then supported by her brother and sister to access the services.
Figure 4. The final result.
Adjustment of the vermillion border was performed 5 months after the second stage using local anaesthesia (Figure 4).
Discussion Noma is typically found in relatively underdeveloped countries, especially those in which malnutrition or protein deficiencies are prevalent.1,2,4,6 No cases of active noma have been seen at the Department of Oral Surgery, Padjadjaran University, for more than a decade. The improvement in child health in Indonesia following efforts such as immunisation and an integrated health service for children has reduced factors predisposing to noma infection. Prior to the discovery of antibiotics, the mortality rate associated with noma infection approached 90% to 95%, but today this is less than 10%.1,4 The morbidity of this infection, however, remains significant, and facial disfigurement with effects on growth and development is not rare.1,2 Penicillin and metronidazole are the first-line antibiotics for necrotising stomatitis.2 This patient survived the infection, but the defect was not properly treated for 248
The first stage of reconstruction was to bring down the lip angle and to rotate the philtrum column. The planned procedure utilised Wright’s facial landmark to mark the new location for the lip angle and a horizontal lip line.10 By using a rotational flap from the cheek, the philtrum was rotated to the same height as the normal side. Since the base of the flap was next to the lip angle, the procedure to relocate the lip angle was delayed. The second stage procedure, to put the lip angle in a normal position, utilised a singlepedicle flap similar to that described by Limberg.9,11-14 This is a simple procedure for opening the mouth, but excess mucosal bulk in the lip angle is common.10 Although additional revision surgery is generally suggested after at least 1 year, the second procedure was performed only 5 months later because the procedure was simple. The unfavourable dimple was corrected later using local anaesthesia. The cosmetic result for this patient is not perfect, and further minor surgery could be performed to achieve the best result. However, the patient and her family are satisfied and no further surgery is planned. This patient was fortunate in the sense that her deformity was correctable by relatively simple procedures without resorting to complex flaps. This is probably due to early control of the original infection, thus limiting the extent of tissue destruction and tissue loss, which is frequently associated with noma.
References 1. Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and maxillofacial pathology. Philadelphia: WB Saunders Company: 1995:155-156. 2. Topazian RG, Goldberg MH. Oral and maxillofacial infection. 3rd ed. Philadelphia: WB Saunders Company; 1994:417-422. 3. Archer WH. Oral and maxillofacial surgery. Volume 12. 5th ed. Philadelphia: WB Saunders Company; 1975:1673-1675. 4. Shafer WG, Hine MK, Levy BK. A textbook of oral pathology. WB Saunders: Philadelphia; 1983:358-359. Asian J Oral Maxillofac Surg Vol 14, No 4, 2002
Mangundjaja, Arumsari
5. Robbins, SL. Pathologic basis of disease. Philadelphia: WB Saunders Company; 1974:882. 6. Regezi JA, Sciubba J. Oral Pathology, clinicalpathologic correlation. 2nd ed. Philadelphia: WB Saunders Company; 1993:47-48. 7. Reinhart P, Philipsen HP. Colour atlas of dental medicine — oral pathology. New York: Thieme; 2000:31. 8. Tyldesley WR, Field EA. Oral medicine. 4th ed. New York: Oxford University Press; 1995:29. 9. Fonseca RJ, Walker RV, Betts NJ, Barber HD. Oral and maxillofacial trauma. Volume 2. Philadelphia: WB Saunders Company; 1997: 1101-1110.
Asian J Oral Maxillofac Surg Vol 14, No 4, 2002
10. Strauch B, Vasconez L, Hall-Findlay EJ. Grabb’s encyclopedia of flaps. 2nd ed. Philadelphia, Lippincot-Raven; 1998:641-647. 11. Smith JW, Aston SJ. Grabb and Smith’s Plastic surgery. Part 1. 4th ed. Philadelphia Little, Brown and Company; 1991:44-65. 12. Burian F. The plastic surgery atlas 1. London: Butterworths; 1967:34-49. 13. Swartz SI, Shires GT, Spencer FC Storer EH. Principles of surgery. 3rd ed. New York: McGrawHill Book Company; 1979:2061-2065. 14. Converse JM, Little JW. Reconstructive plastic surgery. Volume 2. Head and neck. Philadelphia: WB Saunders Company; 1964:853-855.
249
Correction of Lip Defect Caused by Noma Sunardi Mangundjaja, Asri Arumsari Department of Oral Surgery, Faculty of Dentistry, Padjadjaran University, Bandung, Indonesia
Abstract This report presents a 20-year-old woman undergoing correction of an upper lip deformity secondary to noma. The noma infection was acquired at the age of 3 years, and the defect was left untreated until the age of 12 years, when the patient had an operation at a suburban clinic. She was dissatisfied with the result and came to the Department of Oral Surgery, Padjadjaran University in Indonesia 8 years later, where the correction was achieved in 3 stages. The first operation was performed to bring down the commissure of the lip and to restore normal philtral height. The second operation was performed to achieve the normal position of the lip commissure and to complete the restoration of lip length. Both operations were performed using general anaesthesia. The third operation was a small procedure to correct a minor defect of the lip angle using local anaesthesia. Key Words: Noma, Infection, Lip
Introduction Noma is also known as cancrum oris, gangrenous stomatitis, and necrotising stomatitis.1-6 The origin of the term is the Greek word noma, which means “to devour”.2 Noma is an acute, fulminating, toxic, rapidly progressive gangrenous process of the oral and facial tissues, usually seen in debilitated and poorly nourished children.2,4 Typically, noma presents in children aged between 2 to 10 years1 — an extensive series of 69 Nigerian children between the ages of 2 and 7 years has been reported.4 The most common predisposing factors include malnutrition, dehydration, poor oral hygiene, recent illness, malignancy, and immunodeficiency disorders, including AIDS. Commonly associated illnesses include measles, scarlet fever, and tuberculosis, and leukaemia is one of the more frequently associated malignancies.1-8 Noma is clinically characterised by a rapid, progressive course beginning as a small, painful, red spot or vesicle on the attached gingiva. A necrotic ulcer rapidly forms and exposes underlying bone. Correspondence: Asri Arumsari, Department of Oral Surgery, Faculty of Dentistry, Padjadjaran University, Bandung, Indonesia. E-mail: [email protected]
Asian J Oral Maxillofac Surg Vol 14, No 4, 2002
Stomatitis develops as the lesion extends outward in a cone-like fashion, causing painful cellulitis of the lips and cheeks. The overlying skin becomes inflamed, oedematous, dark, and necrotic. Within a short period, sloughing of the soft tissues occurs, eventually exposing the underlying bone, teeth, and deeper soft tissues.1-6 The odour arising from the gangrenous tissues is foul.4 In many instances, the infection begins as an acute necrotising ulcerative gingivitis (ANUG), and some clinicians believe that noma is merely an extension of the same process. The microbiological findings of patients with noma almost always show infection with Pseudomonas aeruginosa,1,2 often combined with Escherichia coli, Klebsiella species, or Staphylococcus species.1 The inflammatory reaction is initiated by anaerobic bacteria, among which are found fusospirochaetal organisms, such as Borrelia vincentii and Fusobacterium nucleatum (Fusiformis fusiformis or Bacillus fusiformis). Bacteroides melaninogenigus may also be present.2,5,7 Treatment of an infected noma requires the use of appropriate antibiotics and correction of inadequate nutrition, hydration, and electrolyte imbalance. Debridement of the wound and gross necrotic areas is recommended. However, aggressive removal is 245
Correction of Lip Defect Caused by Noma
contraindicated because it does not stop the extension of the process and compounds the reconstruction problems. Necrotic bone is left in place and reconstruction should be delayed for 1 year to ensure complete recovery.1,2 The goals of correction of the residual defect are primarily to improve function and aesthetics. Reconstruction surgery consists of replacing lost tissue by transplanting similar tissue. Complete reconstruction is seldom a single-stage surgery but rather a series of staged procedures.9 The desired soft tissue reconstruction should provide viable tissue bulk with a vascular network. If the vascularity is maintained, the reconstruction tissue will remain supple and heal into the recipient tissue bed with minimal change in dimension.9 Reconstruction with facial flaps is most successful when based laterally or inferiorly, with incisions following normal skinfolds and lines of expression. The disadvantages of these regional flaps include the need for additional incisions and elevation of tissues on the face with resultant scarring.9,10 The feasibility of corrective surgery without the use of distant and complex flaps is unusual. a
Case Report A 20-year-old woman was referred to the Department of Oral Surgery, Padjadjaran University in Indonesia, due to deformity of her right upper lip. When she was 3 years old, she had initially been treated at the department for acute noma infection. After control of the infection, the residual defect was left untreated until she was 12 years of age, when she underwent an operation in a suburban clinic. The secondary deformity of her right upper lip consisted of distortion of the level of the lip, resulting in the lip commissure being almost in contact with the alar base. In addition, the distances of the left and right commissures from the midline were unequal, with the right side shorter than the left side. The lower lip was intact but distorted by the pull of the shortened upper lip on the right side (Figure 1a). Surgery was planned in 2 stages. The first corrective stage was intended to bring down the lip commissure and rotate the philtral column. The second stage was to complete the restoration of the lip length and achieve a normal location for the lip commissure. It was planned to perform both procedures with general anaesthesia. To bring down the lip commissure and restore normal lip length, a rotational flap from the cheek next to the ala nasi was used (Figures 1b and 2a). Excision of the scar from the previous operation was done and dissection performed to reduce the tension in the defect. An inferiorly-based rotation flap of the cheek b
Figure 1. Lip deformity secondary to noma. (a) Before surgery; (b) diagram of deformity with planned rotational flap (stage 1 procedure).
246
Asian J Oral Maxillofac Surg Vol 14, No 4, 2002
Mangundjaja, Arumsari
a
a
b
b
c
Figure 2. (a) Diagram of flap after rotation into recipient site (stage 1 procedure); (b) after first-stage surgery.
(next to the commissure) rotated the philtrum, but the length of the right lip remained short (Figure 2b). The second operation was performed 5 months after the first. To achieve a normal location for the lip commissure, a pedicled flap similar to a Limberg flap was used. To create the vermillion at the defect area, buccal mucosal advancement flaps were utilised (Figure 3). Asian J Oral Maxillofac Surg Vol 14, No 4, 2002
Figure 3. Diagram of stage 2 procedure. (a) The outline of the skin excision. The lower lip was separated from the upper lip; (b) the new commisure created by using pedicle flaps from the mucosa; and (c) after second stage surgery.
247
Correction of Lip Defect Caused by Noma
17 years because of the classic noma-related problem of poverty. As she reached puberty, the patient demanded cosmetic treatment and was then supported by her brother and sister to access the services.
Figure 4. The final result.
Adjustment of the vermillion border was performed 5 months after the second stage using local anaesthesia (Figure 4).
Discussion Noma is typically found in relatively underdeveloped countries, especially those in which malnutrition or protein deficiencies are prevalent.1,2,4,6 No cases of active noma have been seen at the Department of Oral Surgery, Padjadjaran University, for more than a decade. The improvement in child health in Indonesia following efforts such as immunisation and an integrated health service for children has reduced factors predisposing to noma infection. Prior to the discovery of antibiotics, the mortality rate associated with noma infection approached 90% to 95%, but today this is less than 10%.1,4 The morbidity of this infection, however, remains significant, and facial disfigurement with effects on growth and development is not rare.1,2 Penicillin and metronidazole are the first-line antibiotics for necrotising stomatitis.2 This patient survived the infection, but the defect was not properly treated for 248
The first stage of reconstruction was to bring down the lip angle and to rotate the philtrum column. The planned procedure utilised Wright’s facial landmark to mark the new location for the lip angle and a horizontal lip line.10 By using a rotational flap from the cheek, the philtrum was rotated to the same height as the normal side. Since the base of the flap was next to the lip angle, the procedure to relocate the lip angle was delayed. The second stage procedure, to put the lip angle in a normal position, utilised a singlepedicle flap similar to that described by Limberg.9,11-14 This is a simple procedure for opening the mouth, but excess mucosal bulk in the lip angle is common.10 Although additional revision surgery is generally suggested after at least 1 year, the second procedure was performed only 5 months later because the procedure was simple. The unfavourable dimple was corrected later using local anaesthesia. The cosmetic result for this patient is not perfect, and further minor surgery could be performed to achieve the best result. However, the patient and her family are satisfied and no further surgery is planned. This patient was fortunate in the sense that her deformity was correctable by relatively simple procedures without resorting to complex flaps. This is probably due to early control of the original infection, thus limiting the extent of tissue destruction and tissue loss, which is frequently associated with noma.
References 1. Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and maxillofacial pathology. Philadelphia: WB Saunders Company: 1995:155-156. 2. Topazian RG, Goldberg MH. Oral and maxillofacial infection. 3rd ed. Philadelphia: WB Saunders Company; 1994:417-422. 3. Archer WH. Oral and maxillofacial surgery. Volume 12. 5th ed. Philadelphia: WB Saunders Company; 1975:1673-1675. 4. Shafer WG, Hine MK, Levy BK. A textbook of oral pathology. WB Saunders: Philadelphia; 1983:358-359. Asian J Oral Maxillofac Surg Vol 14, No 4, 2002
Mangundjaja, Arumsari
5. Robbins, SL. Pathologic basis of disease. Philadelphia: WB Saunders Company; 1974:882. 6. Regezi JA, Sciubba J. Oral Pathology, clinicalpathologic correlation. 2nd ed. Philadelphia: WB Saunders Company; 1993:47-48. 7. Reinhart P, Philipsen HP. Colour atlas of dental medicine — oral pathology. New York: Thieme; 2000:31. 8. Tyldesley WR, Field EA. Oral medicine. 4th ed. New York: Oxford University Press; 1995:29. 9. Fonseca RJ, Walker RV, Betts NJ, Barber HD. Oral and maxillofacial trauma. Volume 2. Philadelphia: WB Saunders Company; 1997: 1101-1110.
Asian J Oral Maxillofac Surg Vol 14, No 4, 2002
10. Strauch B, Vasconez L, Hall-Findlay EJ. Grabb’s encyclopedia of flaps. 2nd ed. Philadelphia, Lippincot-Raven; 1998:641-647. 11. Smith JW, Aston SJ. Grabb and Smith’s Plastic surgery. Part 1. 4th ed. Philadelphia Little, Brown and Company; 1991:44-65. 12. Burian F. The plastic surgery atlas 1. London: Butterworths; 1967:34-49. 13. Swartz SI, Shires GT, Spencer FC Storer EH. Principles of surgery. 3rd ed. New York: McGrawHill Book Company; 1979:2061-2065. 14. Converse JM, Little JW. Reconstructive plastic surgery. Volume 2. Head and neck. Philadelphia: WB Saunders Company; 1964:853-855.
249