Service evaluation of patients with orofacial granulomatosis and patients with oral Crohn's disease attending a paediatric oral medicine clinic.
Orofacial granulomatosis (OFG) This is the occurrence of persistent diffuse facial swelling affecting the lips and cheeks with the key histopathological feature of deep non-necrotising/non-caseating granulomas [Wiesenfeld et al., 1985]. It is an uncommon condition of unknown prevalence [Kolho et al., 2011]. However the reported incidence in children and young adults is increasing [Challacombe, 1997; Leao et al., 2004], probably secondary to greater awareness by clinicians. A slight female predominance has also been reported [Leao et al., 2004].
The presentation of both conditions can be widely variable [Mignogna et al., 2003] with multiple orofacial manifestations occurring at different times [Al-Johani et al., 2009]. These include diffuse swelling of the lips and cheeks, cobblestone mucosa with fissuring and hyperplastic folds, mucosal tags, aphthous or linear non-healing ulcers, angular cheilitis, granular gingivitis and glossitis related to haematinic deficiency [Wiesenfeld et al., 1985].
The aetiology of OFG is uncertain, but a number of causes have been implicated such as genetic, allergic, infective and immunological factors [Tilakaratne et al., 2008; Grave et al., 2009]. Allergic triggers that may lead to a delayed hypersensitivity reaction include dental restorative materials, toothpastes and foods, in particular those containing cinnamon and benzoates [Patton et al., 1985; Endo and Rees, 2007; Tilakaratne et al., 2008].
OFG is a clinical diagnosis of exclusion [Kramer et al., 1980]. Generally OFG is confirmed by lesional biopsy with evidence of non-caseating granulomatous inflammation [Endo and Rees, 2007]. An extensive review of the literature reveals there may be a considerable overlap with OFG presenting as a distinct clinical disorder or as an initial presentation of Crohn's disease [Ghandour and Issa, 1991; Challacombe, 1997; Sanderson et al., 2005; Grave et al., 2009]. However childhood OFG seems to be more frequently related to systemic disease, with intestinal inflammation significantly more likely if OFG onset is under 30 years of age [Sanderson et al., 2005; Saalman et al., 2009]. Appropriate systemic investigations and careful review are therefore indicated [Grave et al., 2009].
Crohn's disease (CD) This is a chronic inflammatory granulomatous disorder of unknown aetiology, which can affect any part of the gastrointestinal tract including the oral cavity [Wiesenfeld et al., 1985; Challacombe, 1997]. Males and females are equally affected and the incidence is approximately 4-6 per 100,000 per year [Hildebrand et al., 2003]. There is a bimodal age presentation with peaks in early and late adulthood [Bradley et al., 2004]. Oral lesions may be the initial and only clinical presentation and have been reported to be present in up to 50% of patients recently diagnosed with CD [Pittock et al., 2001; Harty et al., 2005]. The clinical oral signs show similarity to those seen in OFG [Wiesenfeld et al., 1985]. A diagnosis of CD is made following clinical evaluation and a combination of endoscopic, histological, radiological, and biochemical investigations.
Clinical Outcomes OFG and CD currently have no cure, so treatment is aimed at controlling symptoms and limiting recurrence [Rampton, 1999]. The swelling associated with OFG can cause significant cosmetic and functional problems, however appropriate management can substantially reduce the adverse effects on health and quality of life [Mignogna et al., 2003; Al-Johani et al., 2009; Riordain et al., 2011]. Treatment options range from diets excluding cinnamon and benzoates to intra-lesional or systemic steroids and in unresponsive or severe cases, surgery or immunomodulatory medications such as azathioprine, Infliximab and thalidomide may be considered [Rampton, 1999; Hegarty et al., 2003; Leao et al., 2004; Elliott et al., 2011]. Topical medications such as chlorhexidine, benzydamine hydrochloride (Difflam), hyaluronan (Gengigel) and corticosteroids are also used. The clinical outcome of OFG patients is unpredictable as current therapies are not reliably effective [Leao et al., 2004; Grave et al., 2009]. Mignogna et al.  reported that a delay in treatment of OFG leads to disease that is more refractory to first-line medications signifying the importance of prompt diagnosis and commencement of treatment [Mignogna et al., 2003].
Service evaluation. This approach to care is gaining popularity in the UK National Health Service as a way to define or judge current care through analysis of existing data [NRES Ethics Consultation E-Group, 2006]. It can also involve administration of a simple questionnaire, such as a patient satisfaction survey, providing an opportunity for patients to express opinions of the hospital-care they receive in order to maintain and improve quality of care [Cohen, 1996]. Furthermore, the involvement of children and young people in decisions regarding service development is endorsed by government and healthcare policies [Department for Education and Skills 2003; Department of Health 2003].
The aim of this service evaluation was to establish a profile of the symptoms, treatments and outcomes experienced by patients with OFG and OCD, attending the joint paediatric dentistry and oral medicine clinic at Charles Clifford Dental Hospital, Sheffield. The evaluation also aimed to record patient/parent satisfaction with their management in order to highlight areas for service improvement.
Materials and methods
Study Population. The study population consisted of all patients under 16 years of age with OFG or OCD seen on the monthly joint paediatric dentistry and oral medicine clinic at Charles Clifford Dental Hospital (CCDH), Sheffield. Patients were identified from clinic attendance and histopathology records at CCDH over the past 14 years.
Study Design. The study design was a service evaluation. It was registered with the Clinical Effectiveness Unit at Sheffield Teaching Hospitals NHS Foundation Trust (Registration number 3029). The first part was a retrospective case note analysis to establish a patient profile. The second part was a patient satisfaction questionnaire to gain patient/parent perspectives on their attendance at the clinic.
Data Collection and Analysis: Data were collected in two phases:
Patient Profile. Hospital notes were retrospectively reviewed and a proforma was used to record patient demographics, clinical features, investigations, diagnosis, treatment and outcomes of treatment. Data were analysed using Microsoft Excel 2003.
Patient Satisfaction Questionnaire. The Healthcare Satisfaction Generic Module of the Paediatric Quality of Life Inventory (PedsQLTM) was used. It consists of Likert scales along with free text boxes to allow participants to make comments. Questionnaires were distributed to the participants by mail with an explanatory letter and a pre-paid envelope for return. Six weeks later, questionnaires were sent again to those participants who had not responded. Questionnaires were sent to the parent/guardian of patients below 14 years of age; those age 14 years or above were encouraged to complete the questionnaire themselves. All questionnaires were anonymous. Results were analysed in Microsoft Excel 2003.
Patient profile. Demographic Data. A total of 24 patients (13 females, 11 males) were identified over a 14-year period. The median age at presentation was 11 years (SD 3.79, range 2-15 years).
Referral of 14 patients was by paediatric specialists including those in gastroenterology, dermatology, infectious diseases and immunology. Eight patients were referred by general dental practitioners, one by a general medical practitioner and one by a nurse specialist.
Diagnosis. Of the sample 63% (n=15/24) were diagnosed with OCD and the remaining 37% (n=9/24) with OFG. The majority of the sample were under the care of other medical specialists and had already received a diagnosis for their condition, following oral and gastrointestinal biopsies, prior to referral to the joint paediatric oral medicine clinic (79%, n=19/24). Children were referred for management of their oral symptoms. Almost half the sample (46%, n=11/24) suffered from another medical problem, most commonly atopic conditions (42%, n=10/24) such as asthma and eczema. Arthritis, idiopathic angio-oedema, Turner's syndrome and nut allergy were also observed in individual patients.
Orofacial features. A range of orofacial features classically associated with OCD and OFG were seen (Figure 1). Overall, the most common presentation was oral ulceration (75%, n=18/24), followed by lip/facial swelling (71%, n=17/24). When OCD and OFG are considered separately, a different trend was being observed; oral ulceration were the predominant feature for OCD (87%, n=13/15) but it was only experienced by 56% (n=5/9) of patients with OFG, however this difference did not reach statistical significance OR = 5.20 (95% CI 0.71 to 37.90, p=0.10). Half (n=9/18) of patients with oral ulceration reported associated pain, more commonly in those with OCD (54%, n=7/13), than with OFG (40%, n=2/5). Lip swelling (78%, n=7/9) and angular cheilitis (67%, n=6/9) were the most common orofacial features of OFG. The total number of oral features reported for each patient ranged from 1 to 8. One patient with OCD experienced 8 different features, while the maximum number of features reported for OFG patients was 5. The majority of patients with OCD had 4 or more features.
Investigations. In 21% (n=5/24) of patients referred to the clinic, had been given no diagnosis for their condition. Investigations were organised including blood tests for 4/5 patients, as one had already had all necessary haematological investigations, and biopsy (100%, n=5/5; one oral, three gastrointestinal and one both types). Three patients were subsequently diagnosed with OFG and two with OCD. One patient with OFG had patch testing (33%, n=1/3).
Treatment. Topical preparations were the most common treatment option (65%, n=16/24) followed by placement on a cinnamon and benzoate exclusion diet (58%, n=14/24). Figure 2 shows a comparison between the treatments provided for the two disease entities. Systemic medications were frequently given to patients with OCD as part of their systemic disease management, in particular prednisolone (53%, n=8/15). These were prescribed both on the oral medicine clinic and by other medical specialists involved in the patients' care. In contrast, only one patient with OFG was given a systemic medication. Topical steroids were commonly used for both conditions (42%, n=10/24). No patients received intra-lesional steroids.
Patient-reported symptom improvement was documented in the case notes of 14 of the 24 patients (58%) at some point in their care with the following treatments; infliximab (75%, n=3/4), exclusion diet (43%, n=6/14), azathioprine (33%, n=1/3), topical agents (19%, n=3/16) and predniso lone (11%, n=1/9).
Results of patient satisfaction questionnaire
Response rate. A total of 15 out of 24 questionnaires were returned (63%). Two were excluded as the information given was deficient for consideration. Of the remaining 13 (54%), 10 were completed by parent/guardians and three by the patients themselves.
Information. All participants reported that they had received information on their/their child's diagnosis and that it was in language that was easy to understand. In the majority of cases, verbal information, either alone or in combination with written information, was provided on their condition, treatments, side-effects of drugs and test results. For 62% (n=8/13) it was felt that they were 'Always' updated on their/ their child's condition. Other responses to this question were 'Often' 15% (n=2/13), 'Sometimes' 8% (n=1/13) and 'Not Applicable' 15% (n=2/13).
Communication. When asked questions relating to communication, over 90% (n=12/13) of participants felt the paediatric oral medicine staff were 'Always' friendly, helpful and understanding. They thought staff answered questions, listened to their concerns and explained what to expect during tests or procedures.
Treatment and overall satisfaction. All respondents (n=13) reported that they were happy with their current treatment and with the progress/improvement with their condition. They rated staff as either 'Excellent' or 'Good' when dealing with patient needs, oral symptoms and emotional needs. There were 85% (n=11/13) of participants who rated the overall care received as 'Excellent'. Furthermore, the majority of participants (85%, n=11/13) felt they had been given a choice of appointment times.
Comments. Two main themes emerged from the comments written by the participants; appointment/waiting times and overall experience. The vast majority were positive remarks (Table 1).
OFG and OCD are chronic, debilitating conditions with potential physical, emotional, and psycho-social implications for both children and adults. The literature on the symptoms and quality of life of this group of patients is currently sparse.
The majority of patients were referred by paediatric specialists highlighting the importance of a multidisciplinary approach to the diagnosis and management of these conditions. One third of children were referred by their general dental practitioner (33%, n=8/24). This result is promising as awareness of these conditions is vital to ensure early diagnosis as treatment outcome has been reported to be related to the time between onset of swelling and initiation of therapy [Mignogna et al., 2003; Elliott et al., 2011].
The OFG and OCD cases in this service evaluation reflect the varied clinical presentation reported in the literature [Wiesenfeld et al.,1985; Mignogna et al., 2003]. Similarities to the literature are seen, with oral ulceration and lip swelling presenting as the most prominent features [Wiesenfeld et al., 1985; Saalman et al., 2009]. The findings for labial swelling +in OFG patients (78%) are comparable to those of other studies in the UK (75.5%) [Al-Johani et al., 2010] and Ireland (77%) [McCartan et al., 2011]. Oral ulceration was present in 56% of our OFG patients. This is higher than in other reports from the UK (36.7%) [Al-Johani et al., 2010] and Ireland (36%) [McCartan et al., 2011]. Oral ulceration was more common in OCD patients than OFG patients, a pattern which was also reported in a study by Campbell et al. . Importantly 50% of those with oral ulceration reported associated pain which may be an underestimation given the age group of the sample and their possible inability to articulate their symptoms. Swelling associated with OFG can cause significant cosmetic problems [Mignogna et al., 2003; Al-Johani et al., 2009], but chronic oral mucosal diseases can also impact upon the daily life of patients in areas from physical health and functioning, to concerns about their future [Riordain et al., 2011]. A chronic oral mucosal disease questionnaire (COMDQ) has recently been developed as a tool for measuring quality of life in this group of patients [Riordain and McCreary, 2011]. This is yet to be validated for use in children.
In 46% of the total sample (n=11/24) other medical conditions occured. Ten of these patients had atopic conditions, implying OFG may be an unusual form of allergic or hypersensitivity reaction. There are many suggestions of a possible allergic aetiology in the literature, in particular to foods containing cinnamon and benzoates [Patton et al., 1985; Endo and Rees 2007]. Significant improvements in symptoms have been reported following commencement on a cinnamon and benzoate free diet [White et al., 2006].
A cinnamon and benzoate exclusion diet was a common management approach for both clinical entities (67% OFG, 53% CD). Other treatment modalities included topical applications of medications and systemic medication. Intra-lesional injection of steroids or surgical treatment of swollen lips were not undertaken in any children during the 14 years observation period of this study, but 58% (n=14/24) of the sample reported improvement in their oral symptoms, in line with the literature [Leao et al., 2004; Grave et al., 2009]. Of note, symptom improvement was only reported in 11% (n=1/9) of patients treated with systemic steroids, reflecting the potential recording bias of this retrospective study. Interestingly, all the respondents in the patient satisfaction questionnaire stated they were happy with their current treatments. This could be due to patients accepting their diagnosis and symptoms or it could reflect the intermittent nature of these conditions whereby the acute symptoms are often managed despite the underlying disease never being fully resolved.
The postal questionnaire was an anonymous, retrospective patient view of previous hospital appointments and management. This had the advantage that respondents might feel freer to voice dissatisfaction. Disadvantages are that memory may dilute or distort feelings experienced at the time of hospital episodes. However, the respondents gave positive comments across all categories of the questionnaire. The response rate of 63% may reflect the fact that although all patients from the last 14 years were sent a questionnaire many may have since moved away and so are no longer contactable.
In terms of service improvement, appointment and waiting times were the main themes that emerged. Remarks regarding appointment times were generally encouraging, although one participant felt appointment times conflicted with work and school and another would have liked a choice of appointment times. Other constructive comments included reducing patient waiting times between arrival at the clinic and being seen by a dentist. Following discussion of these findings, changes have been made to increase the flexibility of appointment booking. Furthermore, patient information leaflets are now routinely given to patients to help reinforce information given verbally at consultations.
The main limitation of this service evaluation was its relatively small sample size. As the service evaluation only involved patients with symptoms referred to the paediatric oral medicine clinic, it may not reflect the true population of paediatric OFG/OCD patients in this region of the UK. It does however provide important information on the patient demographics, symptoms and management of this group and a valuable insight into their perspective of the service provided.
This service evaluation highlights the variety of presenting features of OFG and OCD in paediatric patients. Oral ulceration and lip/facial swelling were the most frequently displayed orofacial features for the group as a whole. These conditions are often difficult to manage and only a moderate number of patients reported improvement in their symptoms in response to treatment. Despite this, patient satisfaction with the service provided by the paediatric oral medicine clinic was high, emphasising the importance of good communication when managing children with chronic, debilitating conditions.
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J.L. Howell *, R.M. Bussell *, A.M. Hegarty **, H. Zaitoun *
* Department of Paediatric Dentistry, ** Department of Oral Medicine, Charles Clifford Dental Hospital, Wellesley Road, Sheffield, S10 2SZ, England
Postal address: Dr. H. Zaitoun, Department of Paediatric Dentistry, Charles Clifford Dental Hospital, Wellesley Road, Sheffield, S10 2SZ, UK
Table 1. Selected comments made by participants in the free text boxes of the patient satisfaction questionnaire Appointment/waiting times: Overall experience: "The only thing that needs to be "I have received excellent care improved is the waiting times" and advice about my child's condition. The OFG/ Crohn's was identified almost immediately after months of anxiety and lack of understanding from our GP" "Appointment times conflict with "The treatment my son receives is work and school but this was OK, I outstanding, they are all so very was offered the earliest possible" friendly and accommodating, nothing is too much trouble" "Appointment times were convenient "In my opinion nursing staff are and they were happy to change them overworked and underpaid, they if there was a problem" deserve medals for what they do" "We would have liked a choice of "My experience at the dental appointments as we travel for 1 hospital has been superb. My hour to get to the hospital" daughter is at ease, relaxed, happy about treatment and looks forward to visiting" Figure 1. Comparison of the orofacial features experienced by patients with orofacial granulomatosis (OFG) and oral Crohn's disease (OCD). Other orofacial features included geographic tongue (n=1), gingival hyperplasia (n=1) and oral candidosis (n=2). OFG OCD Glossitis 0 13 Other 22 15 Fissured lips 11 33 Gingivitis 22 53 Mucosaltags 22 53 Cobblestoning 44 80 Angular cheilitis 67 67 Lip/facial swelling 78 67 Oral ulceration 56 87 Note: Table made from bar graph. Figure 2. Comparison of the treatments provided for patients with orofacial granulomatosis (OFG) and oral Crohn's disease (OCD). Topical preparations included chlorhexidine, benzydamine hydrohloride (Difflam), hyaluronan (Gengigel), emolients and antimicrobials. OFG OCD Azathioprine 0 20 Sulpha salazine 0 27 Infliximab 0 27 Topical steroids 33 47 Prednisolone 11 53 Exclusion diet 67 53 Topical preparations 56 73 Note: Table made from bar graph.
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|Author:||Howell, J.L.; Bussell, R.M.; Hegarty, A.M.; Zaitoun, H.|
|Publication:||European Archives of Paediatric Dentistry|
|Date:||Aug 1, 2012|
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