Introduction
Cleft lip and/or palate (CL/P) is the most common malformation occurring in approximately 1:600 live births [
18]. Cleft malformation can arise in different combinations, in varying degrees of severity as well as visibility of the cleft, such as uni- (U) or bilateral (B) CL/P, cleft palate only (CP) or cleft lip with or without alveolus (CL ± A).
A craniofacial malformation that is associated in 80–90% of the cases with a CP is the Robin sequence (RS) [
16,
49]. The prevalence of RS is 11.3:100,000 of live births [
46]. This malformation involves the triad of mandibular retrognathia, glossoptosis, and resultant upper airway obstruction [
61,
62]. Patients with CL/P as well as RS exhibit severe functional difficulties such as feeding problems and failure to thrive in the first weeks after birth. Duration and intensity of interdisciplinary rehabilitation therapy that includes neonatologists, craniomaxillofacial surgeons, ear, nose and throat specialists, speech therapists, orthodontists, and psychologists depend on the severity of the craniofacial malformation. Therapy begins at birth and can last until adolescence. Furthermore, hearing and speech development are also strongly influenced by a cleft in the soft palate. Problems with oral hygiene, missing or malpositioned teeth, arch form deformation, oro-nasal fistulas, nasal deformity, lip scar, facial appearance, and distinctive skeletal discrepancies between the lower and upper jaw and velopharyngeal insufficiencies are additional concerns that affect therapy.
These patients do not only present physical challenges. The malformation can also influence comprehension, cognition, and communication [
33]. These in turn impact well-being, self-esteem and eventually the psyche of patients, thus, affecting social life, social interaction, and quality of life (QoL) [
1,
39,
40,
45,
57]. In the current literature, we found only two studies on the QoL of RS patients and none comparing them with nonsyndromal CL/P patients [
8,
21].
In a society dominated by improved living conditions, beauty ideals, and personal well-being, the term QoL has become increasingly emphasized in many areas of science [
29]. In medicine, QoL was introduced in 1975 [
7]. Topolsky et al. showed a difference in QoL in adolescents with facial conspicuity [
71]. In dentistry, QoL and its impact on health has only recently been considered relevant. Reisine et al. were the first to demonstrate the importance of QoL in relation to oral disease [
60]. In 2003, the World Health Organization (WHO) recognized oral health-related quality of life (OHRQoL) as a segment of the Global Oral Health Program [
56]. OHRQoL is more specific than QoL as a factor in determining the functional and psychosocial implications emanating from oral diseases [
44]. The effects of these diseases impact social life, functional well-being, satisfaction, and expectations concerning care of the afflicted persons and their caregivers [
66,
76].
In contrast to the clinical assessment of oral health by dentists, John et al. described this measure as revealing how patients themselves assess the status of their oral health [
35], including factors such as orofacial function, pain, appearance, and psychosocial effects [
35,
36]. These in turn have significant implications for everyday clinical practice and dental research. The need for scales to measure OHRQoL has therefore been growing in the last 20 years in dentistry [
66]. In 1976, Cohen developed sociodental indicators [
19], which led to the development of instruments for measuring OHRQoL [
40,
50,
67].
To assess OHRQoL, validated and standardized questionnaires are mandatory [
9]. Various questionnaires have been developed by several authors and tested for their psychometric properties. The most frequently used questionnaire for children and adolescents is the Child Oral Health Impact Profile (COHIP) questionnaire [
26,
27]. Broder et al. designed the original COHIP questionnaire to assess the self-reported OHRQoL of children and adolescents aged 8–15 years. It was subsequently adjusted to the ages of 7–18 years for easier handling by patients and for better comparison of the results between age groups [
12,
14]. The authors even published a short version of 19 questions of the original 34-question COHIP questionnaire that provides similar results regarding reliability, validity, and sensitivity of the data. Because of its broad applicability and coverage of psychometric properties, the COHIP-19 questionnaire is best suited for assessing OHRQoL [
15,
22,
26].
The aim of this study was to evaluate a possible difference in OHRQoL between and within a group of patients with craniofacial disorders and a control group, using the COHIP-G19 questionnaire. Factors such as age, gender, visibility of the cleft, cleft type, as well as the severity of the malformation were also considered. The following null hypotheses were investigated:
1.
Patients with craniofacial disorders do not differ in OHRQoL compared with a healthy reference group.
2.
There is no age-related difference in OHRQoL in either group.
Discussion
Our results show a significantly lower OHRQoL in patients with craniofacial disorders compared to a control group without craniofacial disorders who, though, were receiving orthodontic treatment. The outcome was revealed in a statistically higher COHIP total score of the control group. This finding refutes the first of our null hypotheses. A few studies have been published on the OHRQoL using a COHIP questionnaire in patients with craniofacial disorders or at least CL/P. The results of our study corroborate with those of Aravena et al. [
5], Ali et al. [
2], Broder and Wilson-Genderson [
12], and Ward et al. [
76], in terms of a population of American and Chilean children with CL/P. But overall, due to differences in study methods, sample sizes, and standardization, it is difficult to combine the results of previous studies and compare them with our own. Antonarakis et al. [
4] evaluated in a review the OHRQoL of nonsyndromic patients with CL/P in comparison to a general noncleft population in children and adults. In 2 of the 3 studies, the OHRQoL was found to be significantly lower in patients with CL/P. In the third study, there were no significant differences between noncleft and cleft populations [
4]. The COHIP total for the oral-health subscale showed a significantly 2 points lower value in patients with a craniofacial disorder. Patients with a craniofacial disorder had a lower OHRQoL, especially regarding the question of discolored teeth, crooked teeth, and bleeding gums. Stelzle et al. found a statistically significant correlation between gingival esthetics and OHRQoL in patients with CL/P [
69]. In terms of functional well-being, a statistically significantly lower COHIP score was reported in patients with a craniofacial disorder in response to questions on pronunciation and the difficulty saying certain words. These results were consistent with those described in a study from Chetpakdeechit et al. [
17] and Aravena et al. [
5]. They reported that patients with CL/P felt different because of their speech difficulties. This fact is due to the cleft of the soft palate, which is a very important part of the palate, influencing pronunciation, and the emphasis of certain sounds. If there is a velopharyngeal insufficiency, marked by the soft palate not being closed tightly between the mouth and the nasal cavity, the affected patient will suffer from strong hypernasal resonance and problems with pronunciation. In order to ensure sufficient closure and to facilitate speaking at a young age, the palate is sealed as early as possible in our interdisciplinary center by cleft palate repair following Sommerlad’s technique. In addition, a speech pathologist is consulted for successful rehabilitation [
70]. Hypernasal resonance can influence psychological and social factors in the lives of affected patients [
32]. According to our study, and regarding the socioemotional subscale, bullying increased and confidence levels were significantly lower in patients with a craniofacial disorder. Berk et al. showed that patients with a CL/P had much lower self-esteem compared to their unaffected siblings [
10]. Furthermore, patients with CL/P have reported that their self-confidence had been affected by their disorder [
37,
73]. This is in line with the results of our study. There is evidence that patients with craniofacial disorder or those with extreme malocclusion have higher occurrences of being bullied among children and adolescents [
72]. This may lead to major psychosocial problems and difficult social relationships [
31,
52]. These findings reflect the importance of communication skills and the need for early rehabilitation. A cooperative family environment for patients is especially important here. Relatives play a major role in language learning and offer critical support in the development of a psychologically stable individual. It is important to remember that the occurrence of a craniofacial disorder affects the lives of parents as well as a wider family circle. Research indicates that parents can suffer from depression, anxiety, and psychological distress [
42,
43]. This reflects the fact that parents struggle with their own emotions regarding a child’s malformation, its effects on their child’s speech, the social reaction of others, and the concerns regarding cleft treatment [
53,
54,
63]. Treatment of the patient is not the only concern, as the initiation of treatment for a craniofacial anomaly begins at a prenatal stage [
68]. Early diagnosis, education of the parents, and prenatal counselling can reduce parental anxiety associated with this [
48]. Furthermore, providing psychiatric or psychologic counseling and treatment to the parents can prove critical in supporting those with cranial disorders [
42,
55]. High levels of positive reinforcement, support from family and friends, lower psychological distress, and a harmonious parent–child relationship all lead to a better coping strategy for the affected parents. This invariably carries over into the care of the patients [
6,
28,
38].
In a 2009 study, Bos et al. determined the OHRQoL of Dutch orthodontic patients and their parents. They presented lower values for the socioemotional and the well-being subscales in the girls group as compared to the boys group [
11]. This contradicts the results of our present study. In the descriptive analysis of gender, the girls showed higher COHIP scores in total and in the three subscales, though without statistical significance. This result is in line with the studies by Kramer et al. [
41] and van Roy et al. [
74]. Feragen and Stock conducted a psychological evaluation of patients with CL/P at the ages of 10 and 16 years. They determined that male patients at 10 years of age showed lower psychological adjustment than females, while the reverse became true at the age of 16 years [
23,
24]. We can conclude that, in terms of gender, psychological adjustment is dependent on age. The results of the COHIP total score of the older age group of 12–18 years showed significantly lower values in group 1. We can assume that the older patients are more self-reflective, observing, and comparing themselves more with pubertal development. Puberty was the criteria to split the age groups in this study between 11 and 12 years.
The second hypothesis was rejected for group 1. Speech and esthetic concerns seem to have been important factors affecting the health-related quality of life for children with CL/P [
20,
58]. These factors seem to be more important as children approach adolescence (ages 8–12 years), when acceptance by peers becomes more critical. Chetpakdeechit et al. determined that during childhood patients with CLP are not as aware of their condition, feeling more like children without craniofacial disorders. As they grow older, these patients become aware of their malformation. Their concerns included the following: being treated differently, appearing different, lack of recognition, and wanting most of all to be treated like children without a malformation [
17]. Growing older increased the importance of the OHRQoL, with patients suffering from craniofacial disorders expressing the negative side of their appearance. This can lead to developing functional, social, emotional, and speech problems during the transition from childhood to adolescence [
3].
In our study, patients with CL ± A showed the highest OHRQoL, followed by those with CP and those with a unilateral cleft. The validity of this result has to be regarded carefully due to the small case number of patients with CL ± A and RS. RS and bilateral clefts had the lowest COHIP total score values. Dulfer et al. described the HRQoL in RS children [
21]. Parents of RS patients reported a lower HRQoL than parents of children unaffected by RS. This was due to respiratory problems such as upper airway obstruction of the patients with RS. In contrast, a study of Basart et al. demonstrated that the HRQoL in patients with RS was comparable with an unaffected control group, although parental distress was higher in the syndromic RS group than in the nonsyndromic group [
8]. In the study of Basart et al., no significant difference was determined in the visibility of the cleft. This is in line with findings from the current literature [
23,
24].
Many studies have reported on patient assessments with CL/P based on questionnaires, interviews, or observations by self-report or others [
13,
30,
47,
51,
59,
65]. In our study, we used the COHIP-G19 questionnaire to evaluate OHRQoL. Sierwald et al. proved that the German version of COHIP-G19 is a sufficient tool in assessing psychometric properties in children and adolescents [
64]. The test score reliability coefficient, or Cronbach’s α, to evaluate internal consistency of the COHIP-SF19 questionnaire provided good values for nearly all subscales. Only the functional and socioemotional subscales of group 2 revealed unacceptable values. This might be due to the fact that, regarding group 1 versus group 2, group 1 shows better values of internal consistency, suggesting that patients with craniofacial disorder may be more reliable in their answers. Furthermore, the COHIP-G19 questionnaire is designed for patients with craniofacial health problems and has proven reliable specifically for these patients. For investigations concerning children and the effects of dental treatments, or in epidemiologic studies on oral health outcome, the use of condition-specific QOL measures like the COHIP has the advantage of increased patient responsiveness, since the assessment is focused on a specific condition, namely oral health, and it involves increased sensitivity to treatment effects.
Only a few interdisciplinary cleft teams routinely carry out regular psychological assessment, as recommended by the American Cleft Palate Craniofacial Association [
28]. Adequate interdisciplinary therapy from a multidisciplinary team that is always interested in improvement is the basis of successful treatment for these complex and sensitive patients who are in rehabilitation throughout their early years, and even longer. The results of this study reveal that an early start in oral hygiene instruction and prevention, speech therapy, prosthetic and conservative rehabilitation, and psychological support—not only for the affected patients but for the entire family—are imperative, making up a fundamental part of therapy in general. These findings are similar to those published by Feragen and Stock [
23]. Overall, the aim of therapy for patients with craniofacial disorder is optimizing function in terms of feeding, eating, speech, and hearing, as well as achieving the best esthetic results while also providing social and socioemotional support to patients and their families, especially before patients begin school [
42]. Only by looking at all these factors can we reduce the development of deeper social and emotional problems and the risk of bullying or social exclusion. The constant improvement of therapy is essential in raising awareness on how to identify and deal with these patients and to improve rehabilitation and patient care.
Study limitations and outlook
The small sample size limits the power of this study. The smaller the sample size, the more difficult it becomes to predict the meaningfulness of the received COHIP scores. To compare reliably patients with RS versus nonsyndromic patients with CLP, the sample size of the RS population needs to be increased. To assess the impact of the craniofacial disorder on the QoL within the family environment of the affected patient, one could use the Coping Health Inventory for Parents (CHIP).
In reviewing the current literature, we found several gaps in the fields of cleft care [
34,
53]. Significantly lower values in the oral-health subscales for patients with craniofacial malformation compared to the healthy control group are a limiting factor. Future studies need to address the effects of orthodontic treatment or secondary alveolar bone grafting on OHRQoL. For this purpose, patients should answer in future studies the COHIP-G19 questionnaire before and after treatment in order to acquire comparative results. The subject matter of this study is interesting across many disciplines, regardless of the location. Thus, a multicenter study would be critical for future research as a means of producing a longitudinal approach in clarifying differences in protocols.
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