Table 1 Characteristics and quality appraisal of included studies
Author | Year | Country | Study design | Age range of patients (years) | Number of patients older than 10 years | Rectosigmoid aganglionosis (n) | Long-segment (n) | Total colonic aganglionosis (n) | Surgery approach | Age at surgery (Mean, interquartile range), (year) | Associated congenital disease or syndrome (n) | Fecal incontinence (n) | Constipation (n) | Urinary system dysfunction (n) | Quality of appraisal (NOS/AHRQ checklist) a |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Granström et al. [25] | 2015 | Sweden | Case-control | 20–43 | 39 | 37 | / | 2 | Soave, Duhamel, Sphincteromyectomy, Ileostomy, Sigmoid colostomy. | 1 (1, 17) | Down syndrome (n = 1), hypospadias (n = 2), congenital central hypoventilation syndrome (n = 1) and Fallot’s striad (n = 1) | / | / | 3 | S: 3, C: 1, E: 3 Total: 7 |
Jarvi et al. [21] | 2010 | Finland | Case-control | 35–48 | 89 | 79 | 4 | 1 | Soave, Duhamel, State Rehbein, Swenson, Colonal pull-through. | 2.2 (0.8, 2.0) | Down syndrome (n = 1), cartilage hair hypoplasia (n = 1), and multiple endocrine neoplasia type 2 (n = 1). | 12 | 27 | / | S: 3, C: 1, E: 2 Total: 6 |
Neuvonen et al. [22] | 2017 | Finland | Case-control | 18–32 | 33 | 66 | 7 | 3 | Transanal endorectal pull-through (TEPT), TEPT with laparotomy/laparoscopy, Ileoanal pull-through; Definitive endostomy. | 0.35 (0, 9.8) | Down syndrome (n = 11), Mowat-Wilson syndrome (n = 4), cartilage-hairhypoplasia (n = 2), Currarino syndrome (n = 1), Waardenburg-Shah syndrome (n = 1), and marker-chromosome syndrome (n = 1). | 1 | 2 | / | S: 4, C: 1, E: 2 Total: 7 |
Diseth et al. [15] | 1997 | Norway | Case-control | 10–20 | 19 | 12 | / | 3 | Duhamel | 0.7 (0.1, 5) | Unclear | 6 | 3 | / | S: 3, C: 1, E: 3 Total: 7 |
Conway et al. [29] | 2007 | UK | Cohort study | 16–23 | 78 | 63 | 15 | 0 | Duhamel | 0.75 | Unclear | / | / | / | S: 2, C: 0, O: 2 Total: 4 |
Athanasakos et al. [27] | 2006 | Australia | Cross-sectional | 13–24 | 23 | 50 | 8 | 14 | Soave, Duhamel. | Unclear | Down syndrome (n = 2) | 11 | 5 | / | Yes: 6, No: 0, Unclear: 5 |
1995 | Finland & UK | Cohort study | 24–38 | 100 | 95 | 4 | 1 | Duhamel, Swenson, State-Rehbein, Soave. | <  1 year old: 38, 1–3 years old: 37, older than 3 years old: 25 | Down syndrome (n = 1), Chondroectodermal hypoplasia (n = 1), Marfan’s syndrome (n = 1). | 14 | 1 | / | S: 3, C: 1, O: 2 Total: 6 | |
Mills et al. [30] | 2008 | Canada | Cross-sectional | 13–18 | 16 | 34 | 10 | 3 | Duhamel, Soave, Swenson, Transnal. | Unclear | Down syndrome (n = 3), cardiac anomalies (n = 2). Cleft palate (n = 1), ureteric reflux (n = 1), autism (n = 1), inguinal hernia (n = 1), congenital ptosis (n = 1), hypothyroidism (n = 1), and pyloric stenosis (n = 1), | 1 | 1 | 0 | Yes: 8, No: 1, Unclear: 2 |
Catto-Smith et al. [28] | 2007 | Australia | Cross-sectional | 12–27 | 32 | / | / | / | Soave, Duhamel, Swenson, Unknown. | Unclear | Down syndrome (n = 10) | 7 | 18 | 4 | Yes: 8, No: 1, Unclear: 2 |
Ieiri et al. [31] | 2010 | Japan | Cross-sectional | 19–55 | 43 | 34 | 4 | 3 | Z-shaped anastomosis (modified Duhamel procedure), Swenson, Martin. | Unclear | Unclear | 15 | 2 | 5 | Yes: 7, No: 1, Unclear: 3 |
2010 | Sweden | Cross-sectional | 18–45 | 42 | 29 | 8 | 3 | Duhamel, Duhamel-Martin modification, Ileorectal anastomosis. | 0.58 (0,11.6) | Down syndrome (n = 6) | 13 | 5 | 2 | Yes: 9, No: 0, Unclear: 2 | |
Niramis et al. [32] | 2008 | Thailand | Cross-sectional | 10–19 | 111 | 95 | 16 | 0 | Duhamel, Soave, Swenson. | 10–15 years old: 1 (0.5,13); older than 15 years old: 1.3 (0.6, 11) | Down syndrome (n = 9), cerebral palsy (n = 4) b | 17 | 11 | / | Yes: 6, No: 2, Unclear: 3 |