Health-related quality of life in children with Robin sequence following Tübingen palatal plate treatment in infancy

Abstract

Objective

Children with Robin sequence (RS) are characterised by micrognathia/retrognathia, glossoptosis and upper airway obstruction, often resulting in hypoxemic episodes and growth failure, potentially impairing their health-related quality of life (HRQoL). Few studies, however, have yet tried to objectively quantify HRQoL in children with RS. The aim of this cohort study was to investigate HRQoL in children with RS 7–11 years after treatment with the Tübingen palatal plate (TPP) in infancy.

Methods

Using two generic health-related QoL-questionnaires from the parental and child perspective (KINDL-R and KDISCREEN-52) and also the cleft-specific CLEFT-Q questionnaire for children, HRQoL was assessed in 17 children with isolated RS (mean age 8.5 years) and compared with that of 24 children with a cleft palate (± cleft lip, no RS; mean age 8.1 years). Results were also compared with data from a representative European reference cohort.

Results

HRQoL was similar in children with clefts and those with RS and better than that of the reference cohort. In the subscales ‘Bullying’ (proxy 47%, self 65%) and ‘Financial Resources’ (proxy 65%, self 71%) the proportion of children with above average results was even three to four times larger than in the reference population (16%).

Conclusion

These results suggest a good HRQoL at primary school age in these children following TPP therapy in infancy.

WHAT IS ALREADY KNOWN ON THIS TOPIC

• Children with Robin sequence often have a reduced health-related quality of life (HRQoL).

WHAT THIS STUDY ADDS

• Children treated with the Tübingen palatal plate in infancy had a HRQoL at age 7–11 years that was similar to that of children with a cleft palate only and also similar to that of a healthy reference cohort.

HOW THIS STUDY MIGHT AFFECT RESEARCH, PRACTICE OR POLICY

• Robin sequence has a good long-term prognosis if feeding and sleep problems are successfully treated with a functional approach early in life.

Introduction

Robin sequence (RS) is defined as the triad of micrognathia/retrognathia, glossoptosis and upper airway obstruction.^1–3 In 80–90% of cases, a (usually U-shaped) cleft palate (CP) is also present.^{2 4} It may occur in isolation or as part of a syndrome, then also referred to as non-isolated or syndromic RS.^{5 6} The clinical picture is characterised by respiratory problems resulting from obstructive sleep apnoea syndrome (OSAS) and failure to thrive due to feeding problems and an increased work of breathing.^{1 2} Hearing and speech problems, cor pulmonale, developmental delay or even sudden deaths have also been reported.^7–10 This makes appropriate treatment after birth essential. There are various conservative and surgical options, but no standard treatment algorithm has yet been established.^{2 11 12} In addition to objective parameters, such as the mixed-obstructive apnoea–hypopnoea index, Z-scores for weight or IQ, subjective parameters such as health-related quality of life (HRQoL) are increasingly used to assess a treatment’s effectiveness. A guideline on RS published by the relevant European Reference Network (ERN-CRANIO) in 2024 also emphasised the importance of patient-reported outcome measures in evaluating specific treatments.²

In one of the first studies on HRQoL in this patient group, involving parents of 21 infants with RS, HRQoL was determined after mandibular distraction osteogenesis (MDO); 5 infants also had a tracheostomy. The authors reported a subjective improvement in HRQoL following MDO.¹³ Another study investigating the effects of MDO and tongue-lip-adhesion (TLA) found an ‘overall benefit’ in HRQoL for both procedures.¹⁴ A third study determined oral and generic HRQoL in children with RS after different treatment types, including MDO and TLA. While overall HRQoL was found comparable to the reference population, that of children after MDO was found slightly less favourable.¹⁵ In contrast, Basart et al found no significant group differences when comparing the results of RS children treated with MDO with data from the general Dutch population.¹⁶ Another study investigated the relationship between OSAS, HRQoL and RS and found a reduced HRQoL especially in RS children with persistent respiratory problems.¹⁷ Additionally, Skirko et al examined the impact of RS on families through interviews. They described concerns and fears related to the respiratory and nutritional problems associated with RS, which seemed to affect the family’s HRQoL.¹⁸ Finally, a 2022 study investigating oral HRQoL in children with cleft lip and/or palate or RS found that children with RS exhibited slightly lower HRQoL compared with those with cleft lip and/or palate only. Overall, children with cleft lip and/or palate demonstrated significantly lower oral HRQoL than healthy controls.¹⁹

Our group has developed the Tübingen palatal plate (TPP), a functional, non-operative treatment approach for infants with RS that is similarly effective as MDO, yet requires a much shorter hospital stay and has better feeding outcomes.²⁰ It is based on a palatal plate to cover the cleft often present in RS and a pharyngeal extension reaching to just above the epiglottis and moving the tongue forward, thereby opening the airway.²¹ It is usually applied for the first 5–6 months after birth.

To complement the above studies on QoL that mostly involved surgical procedures, we assessed HRQoL in children with RS several years after TPP treatment in infancy.

Methods

Study design and setting

This was a cohort study analysing HRQoL of children with RS treated with the TPP and a control group of children with a CP, CP+cleft lip without alveolus (CP+L) or complete cleft lip and palate (CLP). All patients had been treated at Tübingen University Hospital 7–11 years earlier. This age range was chosen because a new team took over our RS treatment 12 years ago, and the minimum age for questionnaires on HRQoL is typically 7–8 years. Additionally, results from children with RS were compared with published reference data from a European cohort of healthy children of the same age (8–11 years).

Initial contact was established between November 2024 and July 2025 either by telephone or personal contact through our hospital’s outpatient clinic. The invitation, questionnaires and consent form were then sent to interested parents via email. If it was not possible to contact a family, they were invited to participate via ordinary mail. Questionnaires were filled out by children under their parents’ supervision and then mailed to us. Each child received a €15 gift voucher for his/her successful participation.

Participants

Participants were included if they (1) had a diagnosis of RS and received TPP treatment at our institution (RS group) or (2) had a diagnosis of CP, CP+L or CLP (control group) and were also treated at our centre.

Only patients whose parents provided written informed consent and whose primary treatment took place between 1 January 2015 and 31 December 2017 were eligible for inclusion. Exclusion criteria were syndromic disease, other major congenital malformations such as structural heart defects and no command of the German language. There were no other selection criteria, that is, we aimed at enrolling all patients fulfilling the above entry criteria.

Questionnaires

Our primary outcome was the HRQoL, obtained through KINDL-R and KIDSCREEN-52, generic questionnaires that provide a general assessment of the HRQoL; the CLEFT-Q questionnaire was also used to obtain a cleft-specific view. Children (self) were asked to complete all three questionnaires, while their parents (proxy) only answered the KINDL-R and KIDSCREEN-52; questions posed always referred to the last week. All participants completed the questionnaires in full.

KINDL-R

The Kid-KINDL-R questionnaire is intended for 7–13 years old and has been described as reliable and valid to measure HRQoL (Cronbach’s alpha >0.70).^{22 23} It consists of 24 Likert-scale questions that can be summarised in six subscales (‘Physical Well-being’, ‘Emotional Well-being’, ‘Self-esteem’, ‘Family’, ‘Friends’, ‘School’) and one overall scale (‘Total QoL’).^{24 25}

KIDSCREEN-52

The KIDSCREEN-52 questionnaire is also a valid and reliable measurement tool for assessing HRQoL (Cronbach’s alpha=0.76–0.89 and intraclass-correlation (ICC) 0.56–0.77). It was developed for children between the ages of 8 and 18 years. In total, the questionnaire consists of 52 Likert-scaled response options, from which 10 subscales can be formed: ‘Physical Well-being’, ‘Psychological Well-being’, ‘Moods & Emotions’, ‘Self-Perception’, ‘Autonomy’, ‘Parent Relation & Home Life’, ‘Social Support & Peers’, ‘School Environment’, ‘Bullying’ and ‘Financial Resources’. Answers are scaled using the Rasch method and then converted to a T-Score. A value of 50 points represents the mean with a SD of 10 points, with higher values representing a better HRQoL.^{26 27}

Reference data

Reference data (mean and SD) were taken from the KIDSCREEN-52 questionnaire handbook, pp. 152 and 162²⁶ and are based on a representative European group of 8–11 years old children. A comparison of these reference data with the values published by Silva et al for a healthy population showed predominantly higher values in the reference data. Therefore, the KIDSCREEN-52 reference data were considered representative of healthy individuals in this study.^{26 28} The recommended evaluation strategy of the editors of the KIDSCREEN-52 questionnaire divides the children’s data into ‘below average’, ‘average’ and ‘above average’. Data below one SD from the mean are classified as ‘below average’, within one SD from the mean as ‘average’ and above one SD as ‘above average’. The distribution follows a Gaussian normal distribution, meaning that the results of 68% of children are in the ‘average’ range; 16% of responses are classified as ‘below average’ and another 16% as ‘above average’.²⁹

CLEFT-Q

The license for using the CLEFT-Q was granted by its owners (McMaster University, Ontario). Good validity and reliability (Cronbach’s alpha and ICC 0.909) have been reported for its German version.³⁰ The questionnaire consists of 12 or 13 subscales with a total of 119 questions and can be divided into three main categories. Seven subscales measure ‘appearance’ (‘Face’, ‘Lips’, ‘Nose’, ‘Nostrils’, ‘Jaws’, ‘Teeth’, ‘Cleft lip scar’), four subscales examine ‘health-related quality of life’ (‘Psychological function’, ‘School function’, 'Social function’, ‘Speech distress’) and two examine children’s ‘facial function’ (‘Speech function’ and ‘Eating and drinking’). The answers are Likert-scaled. Raw data are converted to a scale from 0 to 100 with higher values representing a better HRQoL. As the ‘Eating and drinking’ subscale employs a different scoring method, it was excluded from the present analysis. The questionnaire is validated for patients aged 8–29 years.^31–34

Statistical methods

Patient data were only used in pseudonymised form. SPSS (SPSS Statistics, V.28.0.1.0, IBM, USA) and Excel (Microsoft Excel 365, V.2507, Microsoft, USA) were used for data analysis. Nominally scaled data were reported as absolute and relative frequencies, while ordinally scaled data were described using mean and SD or median and IQR. The IQR corresponds to the difference between the third and first quartiles. For non-normally distributed data, significance was tested using the Mann-Whitney U test. Results were considered significant at p<0.05. The effect size was indicated by the correlation coefficient r, which was calculated using the following formula: $r=\frac{z}{\sqrt{N}}$. The correlation coefficient r was evaluated according to Cohen (>0.1 small effect, >0.3 medium effect, >0.5 large effect).³⁵ As this is an exploratory study, no correction for multiple testing was performed. Comparisons with the reference data were carried out according to the procedure recommended by the editors and described above.

Results

Participants

From 2015 to 2017, a total of 72 patients with isolated or syndromic RS had been treated in our centre. Before contact was made, 33 patients with RS were excluded based on the above inclusion/exclusion criteria. 39 patients were contacted, of whom 17 participated in the study (participation rate of 44%). 13 children could not be reached, 3 declined participation, 1 did not receive TPP therapy and one subsequently received a diagnosis other than RS (figure 1). Four patients did not return the questionnaires despite several reminders. All patients with RS were treated with a TPP that consists of a base plate to cover the cleft plus a pharyngeal extension that pushes the base of the tongue forward, thereby opening the airway. All but one of the children in the RS group also had a CP.

Figure 1. Patient flow in this study. n, number of patients; RS, Robin sequence; TPP, Tübingen palatal plate.

In the control group, 69 patients were treated concurrently for one of the above cleft disorders (CP, CP+L, CLP). As is standard in Germany, all infants received a feeding plate (‘obturator’) separating the oral from the nasal cavity to facilitate oral feeding. These plates had no pharyngeal extension as characteristic of the TPP and thus could be fitted without endoscopic control. In this group, 19 patients had to be excluded before contact was attempted due to the above exclusion criteria. Of the remaining 50 patients, 6 could not be reached and another 20 did not participate: 6 children did not consent, and in 14, questionnaires were not returned, yielding a participation rate of 48%.

Participants in the RS and control group had a mean age of 8.5 (SD 1.1) and 8.1 (SD 0.9) years, respectively. Gestational age at birth was 38.7 (SD 2.3) and 39.0 (SD 1.5) weeks. Eight children with RS were boys (47.1%), compared with 18 (75%) in controls. As expected, there was an uneven composition of cleft types across groups. In the RS group, all but one child (94.1%) had a CP. The control group consisted of 8 children (33.3%) with isolated CP, 5 (20.8%) with CP+L and 11 (45.8%) with CLP. The length of the postnatal hospital stay was 17.8 days (12–25) in RS children and 5.5 days (1–11) in controls.

The median age at start of TPP therapy was 26 (IQR 40) days. Before therapy, the median Mixed-Obstructive Apnoea Index (defined as the number of obstructive apnoeas per hour of sleep) had been 19.5/hour (29.8/hour).

Health-related quality of life

KINDL-R

From the parents’ perspective, results for both groups showed little difference. Both groups mostly scored >80 points, with only the ‘self-esteem’ subscale showing a mean score of 75.0 points in both groups. Among the children’s questionnaires, results were also largely comparable, with mean scores of >80 points in both groups.

KIDSCREEN-52

Based on the parental report questionnaires, both groups scored >50 points and both showed their lowest scores (48.2 (IQR 6.7) in RS, 51.0 (11.3) points in controls) in the ‘Autonomy’ subscale. Results were also similar in the children’s questionnaires, with scores of >50 points throughout except for the ‘Autonomy’ subscale (48.7 points in controls; table 1).

Table 1. Comparison of QoL between RS and control group (KINDL-R, KIDSCREEN-52, CLEFT-Q).

Quality of life RS group versus CG		Proxy								Self
		Median (IQR)		U	Z	P (exact)	R	N		Median (IQR)		U	Z	P (exact)	R	N
		RS	CG					RS	CG	RS	CG					RS	CG
KINDL-R	Total QoL	84.4 (16.7)	81.3 (12.2)	194.5	−0.252	0.808	0.039	17	24	82.3 (19.3)	82.8 (16.9)	172.5	−0.834	0.412	0.130	17	24
	Physical well-being	87.5 (28.1)	81.3 (18.8)	167.0	−0.988	0.330	0.154	17	24	81.3 (21.9)	71.9 (31.3)	154.5	−1.318	0.192	0.206	17	24
	Emotional well-being	81.3 (21.9)	87.5 (6.3)	180.0	−0.650	0.524	0.102	17	24	81.3 (15.6)	81.3 (18.8)	147.5	−1.514	0.133	0.236	17	24
	Self-esteem	75.0 (15.6)	75.0 (23.4)	198.5	−0.148	0.889	0.023	17	24	87.5 (31.3)	84.4 (29.7)	200.5	−0.093	0.933	0.015	17	24
	Family	81.3 (15.6)	84.4 (17.2)	196.0	−0.215	0.837	0.034	17	24	81.3 (9.4)	81.3 (23.4)	201.0	−0.081	0.941	0.013	17	24
	Friends	81.3 (15.6)	81.3 (12.5)	176.0	−0.755	0.460	0.118	17	24	87.5 (18.8)	87.5 (18.8)	203.0	−0.027	0.983	0.004	17	24
	School	81.3 (25.0)	90.6 (25.0)	179.0	−0.672	0.511	0.105	17	24	87.5 (21.9)	90.6 (29.7)	203.5	−0.013	0.995	0.002	17	24
KIDSCREEN-52	Physical well-being	55.9 (17.2)	55.9 (8.3)	203.5	−0.013	0.995	0.002	17	24	55.6 (13.3)	55.6 (16.6)	175.5	−0.761	0.455	0.119	17	24
	Psychological well-being	58.2 (9.1)	56.7 (9.0)	187.0	−0.455	0.657	0.071	17	24	61.5 (10.5)	54.5 (8.3)	141.0	−1.687	0.093	0.263	17	24
	Moods and emotions	51.3 (16.2)	54.4 (11.3)	178.5	−0.682	0.506	0.106	17	24	57.4 (22.5)	55.7 (23.2)	186.0	−0.482	0.638	0.075	17	24
	Self-perception	56.2 (25.6)	58.8 (11.5)	197.0	−0.188	0.857	0.029	17	24	60.1 (20.0)	55.4 (17.0)	174.5	−0.800	0.431	0.125	17	24
	Autonomy	48.2 (6.7)	51.0 (11.3)	186.5	−0.468	0.648	0.073	17	24	53.2 (7.6)	48.7 (10.3)	158.5	−1.216	0.230	0.190	17	24
	Parent relation and home life	55.1 (11.6)	52.1 (5.7)	180.5	−0.631	0.537	0.099	17	24	54.6 (12.7)	51.8 (11.0)	162.5	−1.113	0.272	0.174	17	24
	Financial resources	65.0 (9.6)	65.0 (5.7)	199.5	−0.138	0.904	0.022	17	24	62.9 (6.5)	62,9 (13.6)	166.5	−1.132	0.258	0.177	17	24
	Social support and peers	55.4 (9.5)	53.0 (4.7)	188.0	−0.427	0.677	0.067	17	24	52.4 (12.9)	52.4 (11.9)	166.0	−1.013	0.318	0.158	17	24
	School environment	57.0 (11.5)	57.0 (12.5)	186.5	−0.466	0.650	0.073	17	24	58.9 (18.6)	57.6 (9.6)	176.5	−0.732	0.472	0.114	17	24
	Bullying	50.6 (19.5)	58.8 (19.5)	181.0	−0.664	0.518	0.104	17	24	58.8 (25.7)	58.8 (19.6)	196.5	−0.226	0.830	0.035	17	24
CLEFT-Q	Face	91.0 (32.5)	79.0 (28.0)	187.0	−0.456	0.656	0.071	17	24
	Lips	92.0 (38.0)	83.5 (34.3)	192.0	−0.323	0.754	0.050	17	24
	Nose	100.0 (22.0)	83.5 (31.5)	147.5	−1.544	0.125	0.241	17	24
	Nostrils	100.0 (9.0)	84.0 (34.8)	129.5	−2.082	0.037	0.325	17	24
	Jaws	100.0 (30.5)	89.0 (22.0)	187.0	−0.479	0.640	0.075	17	24
	Teeth	70.0 (58.0)	60.5 (27.5)	192.0	−0.318	0.758	0.050	17	24
	Psychological function	82.0 (29.0)	71.5 (29.0)	172.0	−0.853	0.401	0.133	17	24
	School function	73.0 (27.0)	71.5 (18.3)	180.5	−0.624	0.541	0.098	17	24
	Social function	80.0 (36.0)	73.0 (26.0)	185.5	−0.493	0.630	0.077	17	24
	Speech distress	100.0 (37.0)	86.5 (23.0)	169.0	−0.964	0.343	0.151	17	24
	Speech function	91.0 (37.0)	82.5 (34.0)	174.5	−0.799	0.432	0.125	17	24

Significant p-values are shown in bold.

CG, control group; N, number of participants; QoL, quality of life; RS, Robin sequence group.

CLEFT-Q

For the CLEFT-Q, higher median values were found in the RS group for all subscales. In the subscales ‘Nose’, ‘Nostrils’, ’Jaws’ and ‘Speech Distress’, the RS group even achieved the highest possible median value of 100 points. The ‘Nostrils’ subscale showed the only significant group difference with a moderate effect (median (IQR): RS 100.0 (9.0) vs CG 84.0 (34.8), p=0.037, U=129.5, Z=−2.082, r=0.325).

Comparison with reference data

A comparison of the KIDSCREEN-52 results with reference data showed that, from a parental point of view, participants in both groups predominantly achieved ‘average’ or ‘above average’ results. Only in the ‘Moods & Emotions’ subscale, a slightly higher proportion of ‘below average’ responses than in the reference population was found (29% in RS (5/17) and 21% in controls (5/24) vs 16% in the reference population; table 2). There was also an increased proportion of ‘above average’ results in the subscales ‘Bullying’ (8/17, 47.1%) and ‘Financial Resources’. Regarding the latter, the RS group had a four-times higher proportion of ‘above average’ results (64.7%; 11/17), suggesting that families with RS children were economically better off than the reference population. No participant was classified as ‘below average’ in this or the ‘Autonomy’ subscale.

Table 2. Comparison of the proportions of below average/average/above average results between RS children, controls and reference data (KIDSCREEN-52).

		Absolute and relative frequencies (proxy)			Absolute and relative frequencies (self)
		RS	CG	All	RS	CG	All
Physical well-being	Below average	1 (5.9%)	2 (8.3%)	3	0 (0.0%)	2 (8.3%)	2
	Average	10 (58.8%)	17 (70.8%)	27	12 (70.6%)	15 (62.5%)	27
	Above average	6 (35.3%)	5 (20.8%)	11	5 (29.4%)	7 (29.2%)	12
	All	17	24	41	17	24	41
Psychological well-being	Below average	1 (5.9%)	0 (0.0%)	1	2 (11.8%)	2 (8.3%)	4
	Average	12 (70.6%)	21 (87.5%)	33	11 (64.7%)	19 (79.2%)	30
	Above average	4 (23.5%)	3 (12.5%)	7	4 (23.5%)	3 (12.5%)	7
	All	17	24	41	17	24	41
Moods and emotions	Below average	5 (29.4%)	5 (20.8%)	10	3 (17.6%)	2 (8.3%)	5
	Average	9 (52.9%)	14 (58.3%)	23	10 (58.8%)	16 (66.7%)	26
	Above average	3 (17.6%)	5 (20.8%)	8	4 (23.5%)	6 (25.0%)	10
	All	17	24	41	17	24	41
Self-perception	Below average	3 (17.6%)	1 (4.2%)	4	2 (11.8%)	0 (0.0%)	2
	Average	8 (47.1%)	18 (75.0%)	26	7 (41.2%)	18 (75.0%)	25
	Above average	6 (35.3%)	5 (20.8%)	11	8 (47.1%)	6 (25.0%)	14
	All	17	24	41	17	24	41
Autonomy	Below average	0 (0.0%)	1 (4.2%)	1	2 (11.8%)	3 (12.5%)	5
	Average	16 (94.1%)	19 (79.2%)	35	13 (76.5%)	19 (79.2%)	32
	Above average	1 (5.9%)	4 (16.7%)	5	2 (11.8%)	2 (8.3%)	4
	All	17	24	41	17	24	41
Parent relation and home life	Below average	1 (5.9%)	3 (12.5%)	4	1 (5.9%)	2 (8.3%)	3
	Average	14 (82.4%)	19 (79.2%)	33	12 (70.6%)	18 (75.0%)	30
	Above average	2 (11.8%)	2 (8.3%)	4	4 (23.5%)	4 (16.7%)	8
	All	17	24	41	17	24	41
Financial resources	Below average	0 (0.0%)	0 (0.0%)	0	1 (5.9%)	2 (8.3%)	3
	Average	6 (35.3%)	9 (37.5%)	15	4 (23.5%)	9 (37.5%)	13
	Above average	11 (64.7%)	15 (62.5%)	26	12 (70.6%)	13 (54.2%)	25
	All	17	24	41	17	24	41
Social support and peers	Below average	1 (5.9%)	1 (4.2%)	2	1 (5.9%)	1 (4.2%)	2
	Average	12 (70.6%)	19 (79.2%)	31	12 (70.6%)	22 (91.7%)	34
	Above average	4 (23.5%)	4 (16.7%)	8	4 (23.5%)	1 (4.2%)	5
	All	17	24	41	17	24	41
School environment	Below average	3 (17.6%)	2 (8.3%)	5	2 (11.8%)	2 (8.3%)	4
	Average	11 (64.7%)	16 (66.7%)	27	9 (52.9%)	18 (75.0%)	27
	Above average	3 (17.6%)	6 (25.0%)	9	6 (35.3%)	4 (16.7%)	10
	All	17	24	41	17	24	41
Bullying	Below average	3 (17.6%)	3 (12.5%)	6	5 (29.4%)	2 (8.3%)	7
	Average	6 (35.3%)	7 (29.2%)	13	1 (5.9%)	8 (33.3%)	9
	Above average	8 (47.1%)	14 (58.3%)	22	11 (64.7%)	14 (58.3%)	25
	All	17	24	41	17	24	41

Reference data: KIDSCREEN Handbook, pp. 152, 162 (below average ≙ 16%, average ≙ 68%, above average ≙ 16%).

CG, control group; RS, Robin sequence group.

Similarly, from the children’s perspective, a high proportion of ‘above average’ results was observed in the ‘Financial Resources’ subscale (70.6%) and in ‘Bullying’ (64.7%). In the latter subscale, however, there was also an increased proportion (29%) of ‘below average’ results (table 2).

There were only two subscales in parental questionnaires where mean values for the RS group were below the average of the reference population: ‘Moods & Emotions’ and ‘Autonomy’. In the subscale ‘Financial Resources’ (mean (SD): proxy 61.1 (5.9) vs 50.3 (10.3) and self 59.0 (7.7) vs 48.9 (10.5)), the difference in mean values was particularly large, with higher values in the RS group compared with the reference group (table 3).

Table 3. Comparison of means and SD for the KIDSCREEN-52 between RS and reference group.

		Group
		RS	Reference
		M (SD)	M (SD)
KIDSCREEN proxy	Physical well-being	56.27 (10.15)	52.65 (9.49)
	Psychological well-being	57.35 (8.01)	51.88 (9.46)
	Moods and emotions	51.33 (11.26)	51.46 (9.62)
	Self-perception	57.36 (11.96)	52.89 (9.91)
	Autonomy	50.14 (6.36)	50.34 (9.03)
	Parent relation and home life	54.02 (7.63)	51.46 (9.38)
	Financial resources	61.06 (5.90)	50.32 (10.29)
	Social support and peers	53.98 (9.52)	50.38 (9.42)
	School environment	56.13 (10.60)	53.54 (9.97)
	Bullying	48.94 (11.79)	47.98 (10.33)
KIDSCREEN self	Physical well-being	57.61 (8.17)	53.72 (9.96)
	Psychological well-being	58.12 (9.11)	53.38 (9.40)
	Moods and emotions	53.97 (12.82)	52.15 (9.97)
	Self-perception	59.60 (11.40)	54.48 (9.74)
	Autonomy	52.76 (7.90)	51.57 (9.67)
	Parent relation and home life	55.65 (7.25)	52.65 (9.18)
	Financial resources	58.99 (7.73)	48.88 (10.45)
	Social support and peers	54.60 (10.95)	50.62 (10.05)
	School environment	59.45 (11.03)	54.52 (10.46)
	Bullying	49.58 (13.30)	47.58 (10.54)

Reference data: KIDSCREEN Handbook, pp. 152, 162.

M, mean; RS, Robin sequence group.

Discussion

This study compared HRQoL of children with RS who underwent TPP treatment in our centre 7–11 years earlier with two control groups: (1) age-matched children with cleft disorders (CP/CP+L/CLP) and (2) a reference population of healthy children. RS children exhibited a QoL comparable to that of children with cleft disorders. Their results were also similar to, or even higher than, those of the reference population for the KIDSCREEN-52 questionnaire. Only in the ‘Bullying’ and ‘Moods & Emotions’ subscales there were proportionally more ‘below average’ values. With scores >80 points from the parents’ perspective in the KINDL-R questionnaire, the results of our study were predominantly above the data published by Ravens-Sieberer for a population of healthy children.²⁵ These results suggest that our patients’ initial hospital stay did not seem to have had a lasting impact on their QoL. Especially when compared with chronic conditions such as asthma or OSAS, which are known to substantially impair QoL, RS in our context did not appear to have a long-term impact on HRQoL, which is in line with other studies investigating HRQoL following the application of other, although often surgical, treatment options.^{9 17 25 36}

The KIDSCREEN-52 questionnaire has already been used in a study on HRQoL of children with RS.¹⁵ However, their age range was 12–18 years. In contrast to the present study, their results were slightly below those of a healthy control group.¹⁵ This may be related to the fact that a higher socio-economic status and younger age are associated with a better HRQoL.³⁷ A cleft disorder has already been identified to influence HRQoL in the CLEFT-Q. For example, children with CLP had a lower HRQoL than those with isolated CP.^{31 33} We chose children with CP as a comparison group to make them as comparable as possible to our RS group (where also all but one had a CP); the only difference being respiratory distress resulting from glossoptosis, leading to the addition of a pharyngeal extension to the base plate that ends just above the epiglottis.²¹ Also, a study from our group showed more externalising behaviour in children born with RS than in healthy controls,³⁸ but it is unclear how this affects HRQoL. There is conflicting data regarding HRQoL of children with isolated and syndromic RS.^{13 15 16} Our study was limited to children with isolated RS, which may partly account for their relatively high HRQoL. The overall favourable outcomes of the RS group may also reflect participation or selection bias, as families with positive treatment experiences and correspondingly good HRQoL are more likely to participate in studies.²² In addition, our cohort consisted of children living in Germany, a country that tends to report higher HRQoL in international comparisons.³⁹ Further limitations include the small number of participants and the lack of comparison with another therapy. However, therapeutic options such as MDO or TLA are not applied at our centre. Furthermore, completion of the questionnaires at home under parental supervision may have allowed for parental influence, but we did not obtain information on whether children were watched by a parent while filling in their questionnaires. In addition, the age ranges of the subjects (7–11 years) did not fully correspond to the recommended age ranges of the questionnaires, some of which are only validated for use starting at 8 years of age.^{26 31} To maximise the number of participants, slightly younger children were also included. Inclusion of older children was considered inappropriate due to changes in the treatment team.

We conclude that these children, treated with our centre’s functional treatment approach, showed values for HRQoL that were similar, if not slightly better, than those of controls with a CP or a healthy reference cohort.

Data availability statement

Data are available upon reasonable request.