Health-Related Quality of Life and Psychosocial Morbidity in Anorectal Malformation and Hirschsprung's Disease

 

 Buy Article Permissions and Reprints

Abstract

Anorectal malformation (ARM) and Hirschsprung's disease (HD) are the most common congenital colorectal anomalies. Despite advances in surgical techniques and improvements in postoperative clinical care, short- and long-term physical and psychosocial morbidity of these patients remains high. This review outlines the current literature on the physical and psychosocial aspects of health-related quality of life (HrQoL) and its confounders in patients with ARM/HD. Moreover, important coping strategies are summarized to further improve HrQoL of ARM/HD patients and their families.

• References

• 1 Levitt MA, Peña A. Anorectal malformations. Orphanet J Rare Dis 2007; 2: 33
  CrossrefPubMedGoogle Scholar
• 2 Cassina M, Fascetti Leon F, Ruol M. , et al. Prevalence and survival of patients with anorectal malformations: a population-based study. J Pediatr Surg 2019; 54 (10) 1998-2003
  CrossrefPubMedGoogle Scholar
• 3 Bradnock TJ, Knight M, Kenny S, Nair M, Walker GM. ; British Association of Paediatric Surgeons Congenital Anomalies Surveillance System. Hirschsprung's disease in the UK and Ireland: incidence and anomalies. Arch Dis Child 2017; 102 (08) 722-727
  CrossrefPubMedGoogle Scholar
• 4 Oyania F, Ogwal A, Nimanya S. , et al. Long term bowel function after repair of anorectal malformations in Uganda. J Pediatr Surg 2019; DOI: 10.1016/j.jpedsurg.2019.11.015.
  CrossrefPubMedGoogle Scholar
• 5 Judd-Glossy L, Ariefdjohan M, Curry S, Ketzer J, Pena A, Bischoff A. A survey of adults with anorectal malformations: perspectives on educational, vocational, and psychosocial experiences. Pediatr Surg Int 2019; 35 (09) 953-961
  CrossrefPubMedGoogle Scholar
• 6 Dai Y, Deng Y, Lin Y, Ouyang R, Li L. Long-term outcomes and quality of life of patients with Hirschsprung disease: a systematic review and meta-analysis. BMC Gastroenterol 2020; 20 (01) 67
  CrossrefPubMedGoogle Scholar
• 7 Nah SA, Ong CCP, Saffari SE. , et al. Anorectal malformation & Hirschsprung's disease: a cross-sectional comparison of quality of life and bowel function to healthy controls. J Pediatr Surg 2018; 53 (08) 1550-1554
  CrossrefPubMedGoogle Scholar
• 8 Constitution of the World Health Organization. World Health Organization Handbook of Basic Documents. 5th Ed. Ginebra: Palais des Nations; 1955
  Google Scholar
• 9 Coons SJ, Rao S, Keininger DL, Hays RD. A comparative review of generic quality-of-life instruments. Pharmacoeconomics 2000; 17 (01) 13-35
  CrossrefPubMedGoogle Scholar
• 10 Karimi M, Brazier J. Health, health-related quality of life, and quality of life: what is the difference?. Pharmacoeconomics 2016; 34 (07) 645-649
  CrossrefPubMedGoogle Scholar
• 11 Haraldstad K, Wahl A, Andenæs R. , et al; LIVSFORSK network. A systematic review of quality of life research in medicine and health sciences. Qual Life Res 2019; 28 (10) 2641-2650
  CrossrefPubMedGoogle Scholar
• 12 Rigueros Springford L, Connor MJ, Jones K, Kapetanakis VV, Giuliani S. Prevalence of active long-term problems in patients with anorectal malformations: a systematic review. Dis Colon Rectum 2016; 59 (06) 570-580
  CrossrefPubMedGoogle Scholar
• 13 Neuvonen MI, Kyrklund K, Rintala RJ, Pakarinen MP. Bowel function and quality of life after transanal endorectal pull-through for Hirschsprung disease. Ann Surg 2017; 265 (03) 622-629
  CrossrefPubMedGoogle Scholar
• 14 Grano C, Aminoff D, Lucidi F, Violani C. Long-term disease-specific quality of life in adult anorectal malformation patients. J Pediatr Surg 2011; 46 (04) 691-698
  CrossrefPubMedGoogle Scholar
• 15 Kyrklund K, Neuvonen MI, Pakarinen MP, Rintala RJ. social morbidity in relation to bowel functional outcomes and quality of life in anorectal malformations and Hirschsprung's disease. Eur J Pediatr Surg 2018; 28 (06) 522-528
  Article in Thieme ConnectPubMedGoogle Scholar
• 16 Boemers TM. Urinary incontinence and vesicourethral dysfunction in pediatric surgical conditions. Semin Pediatr Surg 2002; 11 (02) 91-99
  CrossrefPubMedGoogle Scholar
• 17 Neuvonen M, Kyrklund K, Taskinen S, Koivusalo A, Rintala RJ, Pakarinen MP. Lower urinary tract symptoms and sexual functions after endorectal pull-through for Hirschsprung disease: controlled long-term outcomes. J Pediatr Surg 2017; 52 (08) 1296-1301
  CrossrefPubMedGoogle Scholar
• 18 Witvliet MJ, van Gasteren S, van den Hondel D, Hartman E, van Heurn L, van der Steeg A. Predicting sexual problems in young adults with an anorectal malformation or Hirschsprung disease. J Pediatr Surg 2018; 53 (08) 1555-1559
  CrossrefPubMedGoogle Scholar
• 19 Rosen RC, Fisher WA, Eardley I, Niederberger C, Nadel A, Sand M. ; Men's Attitudes to Life Events and Sexuality (MALES) Study. The multinational Men's Attitudes to Life Events and Sexuality (MALES) study: I. Prevalence of erectile dysfunction and related health concerns in the general population. Curr Med Res Opin 2004; 20 (05) 607-617
  CrossrefPubMedGoogle Scholar
• 20 Kyrklund K, Taskinen S, Rintala RJ, Pakarinen MP. Sexual function, fertility and quality of life after modern treatment of anorectal malformations. J Urol 2016; 196 (06) 1741-1746
  CrossrefPubMedGoogle Scholar
• 21 Kyrklund K, Pakarinen MP, Rintala RJ. Long-term bowel function, quality of life and sexual function in patients with anorectal malformations treated during the PSARP era. Semin Pediatr Surg 2017; 26 (05) 336-342
  CrossrefPubMedGoogle Scholar
• 22 Huibregtse ECP, Draaisma JMT, Hofmeester MJ, Kluivers K, van Rooij IALM, de Blaauw I. The influence of anorectal malformations on fertility: a systematic review. Pediatr Surg Int 2014; 30 (08) 773-781
  CrossrefPubMedGoogle Scholar
• 23 Fanjul M, Lancharro A, Molina E, Cerdá J. Gynecological anomalies in patients with anorectal malformations. Pediatr Surg Int 2019; 35 (09) 967-970
  CrossrefPubMedGoogle Scholar
• 24 Grano C, Aminoff D, Lucidi F, Violani C. Disease-specific quality of life in children and adults with anorectal malformations. Pediatr Surg Int 2010; 26 (02) 151-155
  CrossrefPubMedGoogle Scholar
• 25 Grano C, Bucci S, Aminoff D, Lucidi F, Violani C. Feelings of depression in people with ARM: the role of critical incidents and perceived difficulties in close and sexual relationships. Pediatr Surg Int 2014; 30 (08) 823-828
  CrossrefPubMedGoogle Scholar
• 26 Grano C, Bucci S, Aminoff D, Lucidi F, Violani C. Transition from childhood to adolescence: quality of life changes 6 years later in patients born with anorectal malformations. Pediatr Surg Int 2015; 31 (08) 735-740
  CrossrefPubMedGoogle Scholar
• 27 Wigander H, Nisell M, Frenckner B, Wester T, Brodin U, Öjmyr-Joelsson M. Quality of life and functional outcome in Swedish children with low anorectal malformations: a follow-up study. Pediatr Surg Int 2019; 35 (05) 583-590
  CrossrefPubMedGoogle Scholar
• 28 Collins L, Collis B, Trajanovska M. , et al. Quality of life outcomes in children with Hirschsprung disease. J Pediatr Surg 2017; 52 (12) 2006-2010
  CrossrefPubMedGoogle Scholar
• 29 Meinds RJ, van der Steeg AFW, Sloots CEJ. , et al. Long-term functional outcomes and quality of life in patients with Hirschsprung's disease. Br J Surg 2019; 106 (04) 499-507
  CrossrefPubMedGoogle Scholar
• 30 Mille E, Dariel A, Louis-Borrione C. , et al. Quality of life and neuropsychological development at school age in Hirschsprung's disease. J Pediatr Surg 2020; DOI: 10.1016/j.jpedsurg.2020.01.005.
  CrossrefPubMedGoogle Scholar
• 31 Jarvi K, Laitakari EM, Koivusalo A, Rintala RJ, Pakarinen MP. Bowel function and gastrointestinal quality of life among adults operated for Hirschsprung disease during childhood: a population-based study. Ann Surg 2010; 252 (06) 977-981
  CrossrefPubMedGoogle Scholar
• 32 Sood S, Lim R, Collins L. , et al. The long-term quality of life outcomes in adolescents with Hirschsprung disease. J Pediatr Surg 2018; 53 (12) 2430-2434
  CrossrefPubMedGoogle Scholar
• 33 Drissi F, Meurette G, Baayen C. , et al. Long-term outcome of Hirschsprung disease: impact on quality of life and social condition at adult age. Dis Colon Rectum 2019; 62 (06) 727-732
  CrossrefPubMedGoogle Scholar
• 34 Grano C, Bucci S, Aminoff D, Lucidi F, Violani C. Quality of life in children and adolescents with anorectal malformation. Pediatr Surg Int 2013; 29 (09) 925-930
  CrossrefPubMedGoogle Scholar
• 35 Hartman EE, Oort FJ, Aronson DC, Sprangers MA. Quality of life and disease-specific functioning of patients with anorectal malformations or Hirschsprung's disease: a review. Arch Dis Child 2011; 96 (04) 398-406
  CrossrefPubMedGoogle Scholar
• 36 Grano C, Fernandes M, Bucci S, Aminoff D, Lucidi F, Violani C. Self-efficacy beliefs, faecal incontinence and health-related quality of life in patients born with anorectal malformations. Colorectal Dis 2018; 20 (08) 711-718
  CrossrefPubMedGoogle Scholar
• 37 Gunnarsdóttir A, Sandblom G, Arnbjörnsson E, Larsson LT. Quality of life in adults operated on for Hirschsprung disease in childhood. J Pediatr Gastroenterol Nutr 2010; 51 (02) 160-166
  CrossrefPubMedGoogle Scholar
• 38 Michel G, Bisegger C, Fuhr DC, Abel T. ; KIDSCREEN group. Age and gender differences in health-related quality of life of children and adolescents in Europe: a multilevel analysis. Qual Life Res 2009; 18 (09) 1147-1157
  CrossrefPubMedGoogle Scholar
• 39 Tran VQ, Mahler T, Dassonville M. , et al. Long-term outcomes and quality of life in patients after Soave pull-through operation for hirschsprung's disease: an observational retrospective study. Eur J Pediatr Surg 2018; 28 (05) 445-454
  Article in Thieme ConnectPubMedGoogle Scholar
• 40 Khalil M. Long-term health-related quality of life for patients with Hirschsprung's disease at 5 years after transanal endorectal pull-through operation. Qual Life Res 2015; 24 (11) 2733-2738
  CrossrefPubMedGoogle Scholar
• 41 Versteegh HP, van den Hondel D, IJsselstijn H, Wijnen RMH, Sloots CEJ, de Blaauw I. Cloacal malformation patients report similar quality of life as female patients with less complex anorectal malformations. J Pediatr Surg 2016; 51 (03) 435-439
  CrossrefPubMedGoogle Scholar
• 42 Tannuri ACA, Ferreira MAE, Mathias AL, Tannuri U. Long-term evaluation of fecal continence and quality of life in patients operated for anorectal malformations. Rev Assoc Med Bras (1992) 2016; 62 (06) 544-552
  CrossrefPubMedGoogle Scholar
• 43 Raman VS, Agarwala S, Bhatnagar V. Correlation between quality of life and functional outcomes in operated children with anorectal malformations using the Krickenbeck consensus. Indian J Pediatr 2017; 84 (03) 177-182
  CrossrefPubMedGoogle Scholar
• 44 Townley OG, Lindley RM, Cohen MC, Murthi GV. Functional outcome, quality of life, and 'failures' following pull-through surgery for Hirschsprung's disease: a review of practice at a single-center. J Pediatr Surg 2020; 55 (02) 273-277
  CrossrefPubMedGoogle Scholar
• 45 Stoll C, Dott B, Alembik Y, Roth M-P. Associated anomalies in cases with anorectal anomalies. Am J Med Genet A 2018; 176 (12) 2646-2660
  PubMedGoogle Scholar
• 46 Pini Prato A, Rossi V, Mosconi M. , et al. A prospective observational study of associated anomalies in Hirschsprung's disease. Orphanet J Rare Dis 2013; 8: 184
  CrossrefPubMedGoogle Scholar
• 47 Banu T, Karim A, Adel MG. , et al. Multicenter study of 342 anorectal malformation patients: age, gender, Krickenbeck subtypes, and associated anomalies. Eur J Pediatr Surg 2019; DOI: 10.1055/s-0039-1695789.
  Article in Thieme ConnectPubMedGoogle Scholar
• 48 Akin Sari B, Demiroğullari B, Ozen O, Iseri E, Kale N, Başaklar C. Quality of life and anxiety in Turkish patients with anorectal malformation. J Paediatr Child Health 2014; 50 (02) 107-111
  CrossrefPubMedGoogle Scholar
• 49 Senel E, Akbiyik F, Atayurt H, Tiryaki HT. Urological problems or fecal continence during long-term follow-up of patients with anorectal malformation. Pediatr Surg Int 2010; 26 (07) 683-689
  CrossrefPubMedGoogle Scholar
• 50 Bjørsum-Meyer T, Lund L, Christensen P, Jakobsen MS, Asmussen J, Qvist N. Impact of spinal defects on urinary and sexual outcome in adults with anorectal malformations-a cross-sectional study. Urology 2020; 139: 207-213
  CrossrefPubMedGoogle Scholar
• 51 van Tilburg MAL, Murphy TB. Quality of life paradox in gastrointestinal disorders. J Pediatr 2015; 166 (01) 11-14
  CrossrefPubMedGoogle Scholar
• 52 Athanasakos EP, Kemal KI, Malliwal RS. , et al. Clinical and psychosocial functioning in adolescents and young adults with anorectal malformations and chronic idiopathic constipation. Br J Surg 2013; 100 (06) 832-839
  CrossrefPubMedGoogle Scholar
• 53 Georgeson KE, Inge TH, Albanese CT. Laparoscopically assisted anorectal pull-through for high imperforate anus--a new technique. J Pediatr Surg 2000; 35 (06) 927-930
  CrossrefPubMedGoogle Scholar
• 54 Georgeson KE, Fuenfer MM, Hardin WD. Primary laparoscopic pull-through for Hirschsprung's disease in infants and children. J Pediatr Surg 1995; 30 (07) 1017-1021
  CrossrefPubMedGoogle Scholar
• 55 Wong CWY, Chung PHY, Tam PKH, Wong KKY. Quality of life and defecative function 10 years or longer after posterior sagittal anorectoplasty and laparoscopic-assisted anorectal pull-through for anorectal malformation. Pediatr Surg Int 2020; 36 (03) 289-293
  CrossrefPubMedGoogle Scholar
• 56 Han Y, Xia Z, Guo S, Yu X, Li Z. Laparoscopically assisted anorectal pull-through versus posterior sagittal anorectoplasty for high and intermediate anorectal malformations: a systematic review and meta-analysis. PLoS One 2017; 12 (01) e0170421
  CrossrefPubMedGoogle Scholar
• 57 Langer JC. Laparoscopic and transanal pull-through for Hirschsprung disease. Semin Pediatr Surg 2012; 21 (04) 283-290
  CrossrefPubMedGoogle Scholar
• 58 Hartman EE, Oort FJ, Aronson DC. , et al. Children with anorectal malformations, Hirschsprung disease, and their siblings: proxy reports and self-reports. J Pediatr Gastroenterol Nutr 2015; 61 (06) 630-635
  CrossrefPubMedGoogle Scholar
• 59 Witvliet M, Sleeboom C, de Jong J, van Dijk A, Zwaveling S, van der Steeg A. Anxiety and quality of life of parents with children diagnosed with an anorectal malformation or Hirschsprung disease. Eur J Pediatr Surg 2014; 24 (01) 70-74
  PubMedGoogle Scholar
• 60 Kaugars A, Shields C, Brosig C. Stress and quality of life among parents of children with congenital heart disease referred for psychological services. Congenit Heart Dis 2018; 13 (01) 72-78
  CrossrefPubMedGoogle Scholar
• 61 Puka K, Tavares TP, Anderson KK, Ferro MA, Speechley KN. A systematic review of quality of life in parents of children with epilepsy. Epilepsy Behav 2018; 82: 38-45
  CrossrefPubMedGoogle Scholar
• 62 Cano-Garcinuño A, Mora-Gandarillas I, Bercedo-Sanz A. , et al. Looking beyond patients: can parents' quality of life predict asthma control in children?. Pediatr Pulmonol 2016; 51 (07) 670-677
  CrossrefPubMedGoogle Scholar
• 63 Lazarus RS, Folkman S. Stress, Appraisal, and Coping. New York: Springer; 1984
  Google Scholar
• 64 Grano C, Fernandes M, Aminoff D, Bucci S, Lucidi F, Violani C. The role of coping strategies on health-related quality of life in adults with anorectal malformations. Pediatr Surg Int 2016; 32 (08) 759-765
  CrossrefPubMedGoogle Scholar
• 65 Monteiro AMF, Santos RL, Kimura N, Baptista MAT, Dourado MCN. Coping strategies among caregivers of people with Alzheimer disease: a systematic review. Trends Psychiatry Psychother 2018; 40 (03) 258-268
  CrossrefPubMedGoogle Scholar
• 66 Knowles SR, Tribbick D, Connell WR, Castle D, Salzberg M, Kamm MA. Exploration of health status, illness perceptions, coping strategies, psychological morbidity, and quality of life in individuals with fecal ostomies. J Wound Ostomy Continence Nurs 2017; 44 (01) 69-73
  CrossrefPubMedGoogle Scholar
• 67 Cappe E, Bolduc M, Rougé M-C, Saiag M-C, Delorme R. Quality of life, psychological characteristics, and adjustment in parents of children with attention-deficit/hyperactivity disorder. Qual Life Res 2017; 26 (05) 1283-1294
  CrossrefPubMedGoogle Scholar
• 68 Xiong X, Chen X, Wang G, Feng J. Long term quality of life in patients with Hirschsprung's disease who underwent heart-shaped anastomosis during childhood: a twenty-year follow-up in China. J Pediatr Surg 2015; 50 (12) 2044-2047
  CrossrefPubMedGoogle Scholar
• 69 Espeso L, Coutable A, Flaum V. , et al. Persistent soiling affects quality of life in children with Hirschsprung's disease. J Pediatr Gastroenterol Nutr 2020; 70 (02) 238-242
  CrossrefPubMedGoogle Scholar
• 70 Granström AL, Danielson J, Husberg B, Nordenskjöld A, Wester T. Adult outcomes after surgery for Hirschsprung's disease: evaluation of bowel function and quality of life. J Pediatr Surg 2015; 50 (11) 1865-1869
  CrossrefPubMedGoogle Scholar
• 71 Witvliet MJ, Bakx R, Zwaveling S, van Dijk TH, van der Steeg AFW. quality of life and anxiety in parents of children with an anorectal malformation or Hirschsprung disease: the first year after diagnosis. Eur J Pediatr Surg 2016; 26 (01) 2-6
  PubMedGoogle Scholar