Abstract
This thesis describes outcomes of patients with surgical congenital malformations in multidisciplinary follow-up after surgery. Surgical congenital malformations are birth defects that need surgical correction at infant age, including oesophageal atresia, lung malformations, abdominal wall defects, Hirschsprung disease and anorectal malformations.
The research in the first part of this thesis explores the underlying causes and treatment options for obstructive defecation problems, fecal incontinence or soiling problems, and enterocolitis in patients with Hirschsprung disease. Among the causes of obstructive defecation problems in patients with Hirschsprung disease may be a transition zone anastomosis with a prevalence of 9%. Treatment with intrasphincteric botulinum toxin injections improved defecation in 61-66% of the patients with obstructive defecation problems after surgery for Hirschsprung disease. However, no factors were identified that explained an increased risk of enterocolitis. Moreover, age at surgery was not explanatory for differences in gastrointestinal functional outcome.
The research in the second part of this thesis explores outcomes of patients with surgical congenital malformations after surgery beyond gastrointestinal functional outcome. It describes satisfactory long term health-related quality of life in patients with total colonic aganglionosis, a severe form of Hirschsprung disease. It further describes a risk of impaired neurodevelopmental outcome in patients with surgical congenital malformations, as well as an increased risk of post-traumatic stress disorder in mothers of patients with surgical congenital malformations. It also explores how standardized outcome measurement in multidisciplinary follow-up may contribute to improving individual patient outcome, quality evaluation and research on outcome and prognosis in patients with surgical congenital malformations.
The research in the first part of this thesis explores the underlying causes and treatment options for obstructive defecation problems, fecal incontinence or soiling problems, and enterocolitis in patients with Hirschsprung disease. Among the causes of obstructive defecation problems in patients with Hirschsprung disease may be a transition zone anastomosis with a prevalence of 9%. Treatment with intrasphincteric botulinum toxin injections improved defecation in 61-66% of the patients with obstructive defecation problems after surgery for Hirschsprung disease. However, no factors were identified that explained an increased risk of enterocolitis. Moreover, age at surgery was not explanatory for differences in gastrointestinal functional outcome.
The research in the second part of this thesis explores outcomes of patients with surgical congenital malformations after surgery beyond gastrointestinal functional outcome. It describes satisfactory long term health-related quality of life in patients with total colonic aganglionosis, a severe form of Hirschsprung disease. It further describes a risk of impaired neurodevelopmental outcome in patients with surgical congenital malformations, as well as an increased risk of post-traumatic stress disorder in mothers of patients with surgical congenital malformations. It also explores how standardized outcome measurement in multidisciplinary follow-up may contribute to improving individual patient outcome, quality evaluation and research on outcome and prognosis in patients with surgical congenital malformations.
Original language | English |
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Qualification | Doctor of Philosophy |
Awarding Institution |
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Award date | 4 Apr 2022 |
Print ISBNs | 9789464216738 |
Publication status | Published - 2022 |