Generic and disease-specific health-related quality of life in patients with Hirschsprung disease: A systematic review and meta-analysis

Table 1 Characteristics of included studies

Ref.	Study design	Sample size	Self- report	Parental proxy report	HRQoL instrument	Age range (yr)	Mean age (yr)	Sex (% male)	Operation-technique	Segment- length (% short)	Syndromal comorbidity (%)	Stoma present (%)	Postoperative complications (%)
Amin et al[50], 2018	Cohort	46	0	46	PedsQL	0-18	4.2	71		80
Baayen et al[70], 2017	Cohort	50	50	0	HAQL	12-16						6
Cavusoglu et al[46], 2012	Case-control	12	6	12	PedsQL	2-12	4.7	84
Collins et al[26], 2017	Cohort	60	0	60	PedsQL	2-10	6.4	82	13% Duhamel 80% Trans- anal PT	78	12	13
Espeso et al[21], 2019	Cohort	63			HAQL	6-18	10.8	70	22% Duhamel 78% Trans- anal PT	79	0	0	44
Hartman et al[53], 2007	Cohort	121	121	0	HAQL / TACQoL	8-16	11.4	82		69	0	4
Hartman et al[49], 2008	Cohort	152	152	0	TACQoL	8-16	11.6	80		68	0	3
Khalil et al[47], 2015	Cohort	53	0	53	PedsQL	5-7	5.9	70					3
Lane et al[52], 2016	Cohort	118	0	118	PedsQL	0-17
Meinds et al[17], 2019	Cohort	150	0	150	CHQ-CF87	8-17		79	62% Duhamel 16% Trans- anal PT	84		0	31
Mills et al[25], 2008	Cohort	51	37	44	PedsQL	3-21	9.8	82	15% Duhamel 77% Trans- anal PT	66	6
Nah et al[33], 2018	Case-control	44	21	42	PedsQL	2-20	9.1	75		73
Neuvonen et al[23], 2017	Case-control	39	27	12	PedsQL	0-17		75	96% Trans- anal PT	84	24	5	47
Roorda et al[61], 2018	Cohort	18 /14	7	7	HAQL / CHQ-CF87 / CHQ-PF50	4-17		77	67% Duhamel	0	13	6
Sood et al[48], 2018	Cohort	58	38	58	PedsQL	11-18	14.5	84	7% Duhamel 86% Trans- anal PT	84	12	7	28
Townley et al[67], 2019	Cohort	56	0	56	PedsQL	0-13	5.4	80	84% Duhamel 16% Trans- anal PT	77		14
Xi et al[50], 2018	Case-control	50	0	50	TACQoL	6	6.0	76	100% Trans- anal PT

HRQoL: Health-related quality of life; HAQL: Hirschsprung disease/anorectal malformation quality of life.

Table 2 Differences in health-related quality of life scores in subgroup analyses

Factor associated with HRQoL		Cohen’s d [95%CI], P value1	Q value, P value2
Age	[0-12 yr]	d = -0.206 [-0.721, 0.310], P = 0.434	Q = 1.727, P = 0.422
	[12-16 yr]	d = 0.609 [-0.492, 1.711], P = 0.278
	[16+ yr]	d = -0.033 [-1.170, 1.103], P = 0.954
Type of questionnaire	PedsQL	d = -0.043 [-0.281, 0.195], P = 0.724	Q = 6.370, P = 0.041a
	TACQoL	d = -0.562 [-2.271, 1.147], P = 0.519
	CHQ-CF87	d = -0.412 [-0.575,-0.249], P < 0.001b
Reference data	Normative scores	d = 0.028 [-0.278, 0.334], P = 0.859	Q = 1.768, P = 0.184
	Selected controls	d = -0.793 [-1.965, 0.378], P = 0.184
Overall HRQoL scores	Reported overall HRQoL scores	d = -0.440 [-0.817,-0.063], P = 0.022a	Q = 4.078, P = 0.043a
	Constructed overall HRQoL scores	d = 0.245 [-0.303, 0.792], P = 0.381

¹The difference in scores between patients with Hirschsprung disease and normative or control groups was calculated for each study and expressed as the standardized difference in means (Cohen’s d) and aggregated across studies.

²A Q-test was done to test for significant heterogeneity between groups.

^aP < 0.05.

^bP < 0.01. HRQoL: Health-related quality of life.

Table 3 Summary estimates Hirschsprung disease / Anorectal malformation Quality of Life scores

Summary estimates HAQL scores	Aggregated HAQL domain scores[21,61,70]1	Hanneman et al[4], 2001
Domain	Mean (SD)	Mean rank
Laxative diet	88.41 (19.63)	64.67
Constipating diet	91.67 (17.39)	63.77
Diarrhea	73.32 (26.86)	61.22
Constipation	83.33 (36.51)	64.75
Urinary continence	94.82 (13.57)	65.72
Fecal incontinence	78.70 (24.47)	68.58
Social functioning	93.77 (15.40)	65.33
Emotional functioning	84.42 (19.92)	65.11
Body image	84.70 (20.08)	65.48
Physical symptoms	69.33 (19.09)	58.52

¹The average of mean scores was calculated across domain scores of each studies and aggregated into a weighted mean (SD). HAQL: Hirschsprung disease/anorectal malformation quality of life.

Table 4 Study quality assessment according to the Newcastle-Ottawa Scale criteria

Ref.	Selection	Comparability	Outcome	Assessment
Amin et al[50], 2018	2 points		2 points	Poor
Baayen et al[70], 2017	1 point		2 points	Poor
Cavusoglu et al[46], 2012	3 points	2 points	2 points	Good
Collins et al[26], 2017	3 points	1 point	3 points	Good
Espeso et al[21], 2019	1 point		2 points	Poor
Hartman et al[53], 2007	2 points		2 points	Poor
Hartman et al[49], 2008	3 points	1 point	3 points	Good
Khalil et al[47], 2015	2 points		3 points	Poor
Lane et al[52], 2016	2 points		3 points	Poor
Meinds et al[17], 2019	3 points	1 point	2 points	Good
Mills et al[25], 2008	3 points	1 point	2 points	Good
Nah et al[33], 2018	3 points	2 points	2 points	Good
Neuvonen et al[23], 2017	3 points	2 points	2 points	Good
Roorda et al[61], 2018	2 points	1 point	2 points	Fair
Sood et al[48], 2018	3 points	1 point	3 points	Good
Townley et al[67], 2019	3 points	1 point	3 points	Good
Xi et al[50], 2018	3 points	2 points	3 points	Good