The severity of psychosocial and functional morbidity among facially disfigured untreated noma cases in Ethiopia.

OBJECTIVES: Noma is a facially disfiguring disease that affects the oral cavity and midface structures. If left untreated, the disease is fatal. Noma causes severe cosmetic and functional defects in survivors, leading to psychiatric and social problems. However, there are limited data on psychosocial and functional sequelae associated with this disease. This cross-sectional study aimed to investigate psychosocial and functional morbidity among facially disfigured untreated Noma cases. Study participants were volunteer patients diagnosed with noma and awaiting surgery at two noma treatment centers in Ethiopia. A questionnaire derived from the APA's DSM-5, the DAS59, and the Appearance Anxiety Inventory protocol was used to measure the psychosocial and functional morbidity of the cases between September 16 and October 10, 2022. RESULTS: A total of 32 noma cases (19 women and 13 men) awaiting the next surgical campaigns were involved in the study. Study participants reported severe social (Likert score = 2.8) and psychological (Likert score = 3.0) morbidity. Functional limitation was moderate (Likert score = 2.9). This study has shown that psychosocial and functional morbidity in untreated noma cases in Ethiopia is substantial. Therefore, policymakers, clinicians, and researchers need to pay sufficient attention to providing adequate health care and preventing the occurrence of the disease in the long term.

Airway Management Through a Facial Defect Resulting From Noma (Orofacial Gangrene): A Case Report.

At a rural district hospital in Burundi, a 3-year-old severely malnourished girl with Noma presented to the operating room for placement of a gastrostomy tube. The child had a large left-sided facial defect as well as trismus. After induction of general anesthesia, the anesthesia provider was unable to open her mouth. Given that the hospital has no fiberoptic bronchoscope, direct laryngoscopy and intubation were performed directly through her facial defect. After securing the airway, the surgery proceeded without difficulty.

Outcomes at 18 mo of 37 noma (cancrum oris) cases surgically treated at the Noma Children's Hospital, Sokoto, Nigeria.

BACKGROUND: Noma is a rapidly progressing infection of the oral cavity frequently resulting in severe facial disfigurement. We present a case series of noma patients surgically treated in northwest Nigeria. METHODS: A retrospective analysis of routinely collected data (demographics, diagnosis and surgical procedures undergone) and in-person follow-up assessments (anthropometry, mouth opening and quality of life measurements) were conducted with patients who had surgery >6 mo prior to data collection. RESULTS: Of the 37 patients included, 21 (56.8%) were male and 22 (62.9%) were aged >6 y. The median number of months between last surgery and follow-up was 18 (IQR 13, 25) mo. At admission, the most severely affected anatomical area was the outer cheek (n = 9; 36.0% of patients had lost between 26% and 50%). The most frequent surgical procedures were the deltopectoral flap (n = 16; 43.2%) and trismus release (n = 12; 32.4%). For the eight trismus-release patients where mouth opening was documented at admission, all had a mouth opening of 0-20 mm at follow-up. All patients reported that the surgery had improved their quality of life. CONCLUSIONS: Following their last surgical intervention, noma patients do experience some improvements in their quality of life, but debilitating long-term sequelae persist.

Case Report: Malignant Transformation of Noma: Repair by Forearm Flap.

Noma is a progressive infectious disease manifested by a necrotic ulcer of the mouth and face. It usually occurs in poor, malnourished children, with about 30,000-140,000 cases each year and a low survival rate. The exact cause of noma remains unclear, but bacterial infection has been postulated to be a major cause of this disease. Antibiotics and improved nutrition could help inhibit the progression of noma, but most patients still require oral surgery because of the bacterial infection-induced tissue damage. In this study, we report an unusual case of a noma patient whose facial lesion developed a malignancy. The necrotic tissue and infectious area were surgically resected, and a forearm flap was used to repair the patient's facial defect. This case aimed at increasing people's awareness of tropical diseases such as noma.

Facing Africa: Describing Noma in Ethiopia.

Noma affects the most marginalized communities in the world, beginning as oral ulceration and rapidly progressing to orofacial gangrene. With a mortality rate estimated to be as high as 90% and with very few able to access treatment in its active phase, very little is understood about the disease. This retrospective review of patients treated by Facing Africa for deformity and functional impairment secondary to noma between May 2015 and 2019 highlights some of the difficulties encountered by those afflicted. Eighty new patients with historical noma defects were identified and were seen over the course of nine surgical missions, with notes providing valuable geographical, socioeconomic, and psychosocial information. The mean self-reported age of onset was 5 years and 8 months, with a median time of 18 years from onset to accessing treatment. Before intervention, 65% covered their face in public, 59% reported difficulty eating, 81% were unhappy with their appearance, and 71% experienced bullying. We aimed at emphasizing the significant burden, both psychologically and physically of noma, demonstrating the disparity between recent decades of progress in the well-being of Ethiopians in general and the access to health care and mental health support for some of those most in need.

Twenty-Five Years of Experience with the Submental Flap in Facial Reconstruction: Evolution and Technical Refinements following 311 Cases in Europe and Africa.

BACKGROUND: The submental flap is a pedicled island flap with excellent color match for facial reconstruction. The flap can be raised with muscle, submandibular gland, or bone and can be transposed to reach defects up to two-thirds of the face. The authors report the primary author's (D.M.) experience of 25 years using the submental flap from its original description to most recent technical evolutions in both Europe and Africa. METHODS: This is a retrospective study including all patients with facial defects who underwent reconstruction using a submental flap between 1991 and 2016. This study included the use of all four variations of the submental flap: platysmal, digastric, extended, and superextended. The authors report technical adaptations and complications encountered. RESULTS: The authors performed 311 facial reconstructions using submental flaps: 32 platysmal, 133 digastric, 91 extended, and 45 superextended variations. In conjunction with these reconstructions, the authors performed 10 osteocutaneous submental flaps and two free flaps. The authors report two cases of total flap necrosis (0.6 percent) and 28 minor complications, including 23 cases of distal skin necrosis (7 percent), one reversible mandibular facial nerve palsy (0.3 percent), and three hematomas (1 percent). CONCLUSIONS: The submental flap has proven to be a reliable flap for head and neck reconstruction. The four technical modifications described use varying amounts of soft tissue to replace tissue lost and can include vascularized bone from the mandibular margin. This flap exemplifies Gillies' principle of "replacing like with like" and should be discussed as an alternative to free tissue transfer in facial reconstruction, especially in settings where resources are limited. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Noma surgery.

OBJECTIVE: Noma is a devastating and destructive disease of the face for which there is a dearth of information regarding surgical options. Herein, we describe the facial deformities and patient characteristics in a patient population affected by noma and the surgical approaches used in treatment. METHODS: Retrospective case series of a Doctors Without Borders (Médecins Sans Frontières) intervention at Sokoto Children's Noma Hospital in northern Nigeria, the highest-volume noma hospital in the world. RESULTS: Twenty-two procedures were performed on 18 patients with noma, 44% of whom were children. The majority of patients (n = 10, 55.5%) were made aware of surgical care through a Doctors Without Borders outreach program. Patients' reasons for seeking care included functional (61.1%, n = 11), appearance (61.1%, n = 11), and social stigma (66.7%, n = 12). The majority (83.3%, n = 15) had lip involvement. Four patients (22.2%) underwent staged flap procedures including prelamination, flap delay, or pedicle division. The mean duration of surgical procedure was 87 minutes (range 5-306 minutes). The minor complication rate was 16%. There were no major complications or deaths. CONCLUSION: Noma is a mutilating disease of the face that occurs in settings of extreme malnutrition. A total of 55.5% of noma patients were made aware of surgical care through outreach programs. The majority of noma patients seek care to improve function (61.1%) and appearance (61.1%), and to address social stigma (66.7%). A total of 83.3% of noma patients had lip involvement. Facial reconstructive surgeons must rely on principles of congenital, traumatic, and oncologic deformity repair while focusing on safe, reliable procedures for low-resource settings. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:96-99, 2019.

The surgical management of extra-articular ankylosis in noma patients.

Extraarticular ankylosis occurs frequently in children suffering from noma sequelae. Over 20years of operating on these patients, we observed high recurrence of mouth opening limitation. We therefore progressively changed our surgical strategy. This retrospective study compares the impact of different parameters (types of surgery, noma type, physiotherapy compliance, age and sex) on immediate and long-term mouth opening. It includes a series of 121 patients with extraarticular ankylosis operated on between 1990 and 2015. Soft tissue reconstruction evolved from local and pedicled flaps to large free flaps. Mouth opening was performed by bone-bridge excision, sometimes associated to contralateral coronoidectomy. Mouth opening technique including bilateral coronoidectomy with free flap reconstruction was the only independent factor for significantly better immediate mouth opening with a mean increase of 8.7mm [95% confidence interval (CI) 4.3-13.1, P<0.001) and this effect was maintained in the 3years of follow-up. Another positive factor related to long-term results was excellent physiotherapy, while noma type 4 was a negative factor. Recurrence remains problematic in the management of noma sequelae. If physiotherapy and long-term follow-up cannot be offered, patients should not be operated on, because if limitation of mouth opening recurs, oral feeding may become impossible when a facial defect has been reconstructed.

Microvascular reconstruction of facial defects in settings where resources are limited.

The surgical treatment of defects caused by noma is challenging for the surgeon and the patient. Local flaps are preferred, but sometimes, because of the nature of the disease, there is not enough local tissue available. We describe our experience of free tissue transfer in Ethiopia. Between 2008 and 2014, 34 microsurgical procedures were done over 11 missions with the charity Facing Africa, predominantly for the treatment of defects caused by noma (n=32). The mean duration of operation was 442 minutes (range 200 - 720). Six minor wound infections were treated conservatively and did not affect outcome, a return to theatre was required in 4 patients with wound infections and one with a haemorrhage; 2 flaps failed and 2 partially failed, one patient developed an oronasal fistula, and one had an infection at the donor site that required a repeat graft. In settings where resources are limited, free flaps can be used when local tissue is not available and they cause less morbidity than pedicled tissue transfer.

Total reconstruction of the nose in settings where resources are limited.

We describe a series of 8 patients who had total reconstruction of the nose during 4 separate missions to Ethiopia. The aetiology was noma (n=3), assault (n=2), acid burn (n=1), squamous cell carcinoma (n=1), and a sequela of meningococcal septicaemia (n=1). Reconstruction was with forehead flaps when adequate tissue was available (n=6) and with radial forearm flaps when it was not (n=2). Some reconstructive approaches require procedures to be done in stages, and in settings where resources are limited, difficulties with the continuity of care and provision for the management of complications, must be overcome.

[Child care management in maxillofacial humanitarian mission]. / Prise en charge de l'enfant en mission humanitaire maxillofaciale.

Our practice in a humanitarian (or crisis) context differs from what we experience in daily practice. There are several reasons for this. First, the diseases encountered are sometimes unfamiliar, such as sequelae of noma, or the presentation of familiar diseases may be unusual, such as facial malformations seen at a late stage. Secondly, these missions take place in developing countries, and consequently, evaluation and anticipation of possible malnutrition should be considered, especially because facial diseases themselves may be responsible for nutritional problems. Lastly, conditions are often difficult, occurring in an unusual environment, and we sometimes have to face communication and equipment problems. The goal of our work, based on a 15-year experience (in Bamako and Mopti with the Association "Santé et Développement", and in Ouagadougou with the organization "Les enfants du noma") and the analysis of literature, is to point out these features and maybe to be helpful to others.

Reconstruction of complex oro-facial defects using the myocutaneous sub-mental artery flap.

Oro-facial defects require reconstruction that provides suitable colour match and texture. Moreover inner and outer cheek lining and bulk are key considerations. In cases of severe oro-facial infections concomitant mandibular abnormality, for example trismus, can mandate the need for tissue to obturate mandibular defects. We assessed the use of the myocutaneous sub-mental artery flap (MSA) in non-oncological patients with such defects. Twenty two consecutive patients were included in this case series. All patients were survivors of Cancrum Oris (NOMA). Demographic details, nutritional status and co-morbidities were recorded. Defects were classified according to the tissues destroyed; cheek, mandible, oral cavity, lip(s), nose and eye(s). Simultaneous procedures carried out were recorded. The surgical anatomy of the MSA is described. All patients had composite defects of the cheek and oral cavity plus another local anatomical structure. Adjunct procedures such as trismus release were carried out in 18/22 patients. Four patients required a return to theatre. There was no trismus recurrence observed. No flap losses were incurred. The MSA is a robust flap with minimal incidence of major complications. The MSA negates the need for microsurgical tissue transfer. Furthermore the MSA provides adequate bulk to obturate these defects. Future applications of the MSA may include complex oro-facial oncological defects.

Special article: airway management in reconstructive surgery for noma (cancrum oris).

Noma (cancrum oris) is a disease of poverty and malnutrition, which predominantly affects children younger than 10 years in developing countries. Although the majority of sufferers die of sepsis at the time of the initial infection, or of subsequent starvation due to severe trismus and an inability to eat, a small minority of patients survive and require reconstructive surgery for severe facial scarring and deformity. These patients present significant problems to the anesthesiologist with regard to airway management. We present a series of 26 patients undergoing primary and subsequent reconstructive surgery, with particular focus on airway management. We show that airway management, while challenging, can be performed safely and successfully by using individualized airway plans but may require advanced techniques and equipment. Traditional tests focusing on the anterior/superior airway are helpful in assessing patients with facial deformity due to noma.

First experiences with simultaneous skeletal and soft tissue reconstruction of noma-related facial defects.

Noma victims suffer from a three-dimensional facial soft-tissue loss. Some may also develop complex viscerocranial defects, due to acute osteitis, chronic exposure, or arrested skeletal growth. Reconstruction has mainly focused on soft tissue so far, whereas skeletal restoration was mostly avoided. After successful microvascular soft tissue free flap reconstruction, we now included skeletal restoration and mandibular ankylosis release into the initial step of complex noma surgery. One free rib graft and parascapular flap, one microvascular osteomyocutaneous flap from the subscapular system, and two sequential chimeric free flaps including vascularized bone were used as the initial steps for facial reconstruction. Ankylosis release could spare the temporomandibular joint. Complex noma reconstruction should include skeletal restoration. Avascular bone is acceptable in cases with complete vascularized graft coverage. Microsurgical chimeric flaps are preferable as they can reduce the number and complexity of secondary operations and provide viable, infection-resistant bone supporting facial growth.