Long‐term outcome and quality of life in patients with total colonic aganglionosis in the Netherlands

Roorda, Daniëlle; Witvliet, M.; Wellens, Lianne M.; Schulten, D.; Sloots, Cornelius E. J.; Blaauw, Ivo de; Broens, Paul M. A.; Oosterlaan, Jaap; Heurn, L. W. Ernest van; Steeg, Alida F. W. van der

doi:10.1111/codi.14095

Cited by 22 publications

(24 citation statements)

References 42 publications

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“…Less often 0 (0) 0 (0) 0 (0) 0 (0) 0 (0) Always/daily 1 (2) 1 (5) 0 (0) 0 (0) 0 (0) Values in parentheses are percentages unless indicated otherwise; *values are median (range). SIAA, straight ileoanal anastomosis; JIAA, J pouch-ileoanal anastomosis; BFS, bowel function score (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20). †P = 0⋅007 versus SIAA and Duhamel procedure (Kruskal-Wallis test); all other comparisons were not significant.…”

Section: Frequency Of Bowel Movementsmentioning

confidence: 99%

“…All definitive surgical procedures in TCA aim to achieve faecal continence by preserving the sphincter musculature, even though the internal anal sphincter remains aganglionic and is a potential cause of obstructive defaecation and HAEC 13,14 . In the long-term, nutritional status and growth may be concerns, owing to loss of intestinal length, dysmotility and malabsorption 1,[15][16][17] . To date, no reconstructive surgical technique has emerged as superior to others with regard to functional outcomes or complications 3,4,18,19 .…”

Section: Introductionmentioning

confidence: 99%

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Total colonic aganglionosis: multicentre study of surgical treatment and patient-reported outcomes up to adulthood

et al. 2020

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Background: Surgery for total colonic aganglionosis (TCA) is designed to preserve continence and achieve satisfactory quality of life. This study evaluated a comprehensive group of clinical and social outcomes. Methods: An international multicentre study from eight Nordic hospitals involving examination of case records and a patient-reported questionnaire survey of all patients born with TCA between 1987 and 2006 was undertaken. Results: Of a total of 116 patients, five (4⋅3 per cent) had died and 102 were traced. Over a median follow-up of 12 (range 0⋅3-33) years, bowel continuity was established in 75 (73⋅5 per cent) at a median age of 11 (0⋅5-156) months. Mucosectomy with a short muscular cuff and straight ileoanal anastomosis (SIAA) (29 patients) or with a J pouch (JIAA) (26) were the most common reconstructions (55 of 72, 76 per cent). Major early postoperative complications requiring surgical intervention were observed in four (6 per cent) of the 72 patients. In 57 children aged over 4 years, long-term functional bowel symptoms after reconstruction included difficulties in holding back defaecation in 22 (39 per cent), more than one faecal accident per week in nine (16 per cent), increased frequency of defaecation in 51 (89 per cent), and social restrictions due to bowel symptoms in 35 (61 per cent). Enterocolitis occurred in 35 (47 per cent) of 72 patients. Supplementary enteral and/or parenteral nutrition was required by 51 (55 per cent) of 93 patients at any time during follow-up. Of 56 responders aged 2-20 years, true low BMI for age was found in 20 (36 per cent) and 13 (23 per cent) were short for age. Conclusion: Reconstruction for TCA was associated with persistent bowel symptoms, and enterocolitis remained common. Multidisciplinary follow-up, including continuity of care in adulthood, might improve care standards in patients with TCA.

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Section: Frequency Of Bowel Movementsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Total colonic aganglionosis: multicentre study of surgical treatment and patient-reported outcomes up to adulthood

et al. 2020

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“…To assess HrQoL in children with BA, we used the internationally validated CHQ. 12,13 This questionnaire has been developed to test both physical and psychosocial HrQoL of children aged 5 years and older. 14 The CHQ consists of a 50item parent-proxy form (CHQ-PF50), and an 87-item childreported form (CHQ-CF87) for children aged 10 years or older.…”

Section: Measurementsmentioning

confidence: 99%

Health-Related Quality of Life in Biliary Atresia Patients with Native Liver or Transplantation

et al. 2020

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Abstract Introduction We aimed to assess health-related quality of life (HrQoL) in biliary atresia (BA) patients, based on original data and a literature review, and to determine factors associated with their HrQoL. Materials and Methods We reviewed available studies describing HrQoL in BA patients. We assessed HrQoL in Dutch BA patients (6–16 years) using the validated Child Health Questionnaire. We compared HrQoL scores in BA patients with healthy peers and with children who had undergone major surgery in infancy or children with chronic conditions. We determined the relationship between specific patient-related factors and HrQoL. Results Literature data indicated that HrQoL in children with BA is lower than in healthy peers. In Dutch BA patients (n = 38; age 10 ± 3 years), parent-proxy physical HrQoL (48 ± 11) was significantly lower compared with two reference groups of healthy peers (59 ± 4 and 56 ± 6, respectively, each p < 0.001), and lower than in children with attention deficit hyperactivity disorder (60 ± 5), asthma (54 ± 6), attending a cardiology clinic (52 ± n/r), congenital diaphragmatic hernia (53 ± 7) or D-transposition of the great arteries (54 ± 6; all p < 0.05). Psychosocial HrQoL (50 ± 9) was lower than in healthy peers (54 ± 6, p = 0.02, and 53 ± 6, p = 0.07) and children with asthma (54 ± 6, p = 0.02), and largely comparable to children with other chronic conditions. Parent-proxy physical HrQoL was adversely related to adverse medical event in the past year, special education, and motor impairments; psychosocial HrQoL was adversely related to behavioral problems. Conclusion Children with BA are at risk of impaired HrQoL. Special attention is warranted for children with adverse medical events and special education.

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“…Long-term followup studies have shown that hypermotility problems persist even when the pull through is done after the child is toilet trained, most frequently presenting involuntary loss of stool during the night, abdominal pain, and a poor quality of life due to daily life limitations. 20 An example of the importance of these symptoms is revealed in one study which showed that the most common questions posted on the Hirschsprung's disease Facebook page are regarding the management of severe diaper rash, followed closely by questions regarding bowel management, long-term outcomes, potty training, and dietary issues. 21 We also emphasize the low growth curve in these patients.…”

Section: Discussionmentioning

confidence: 99%

“…The mean age at pull through was 17.5 months (11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25). At the time of the ileoanal pull through, eight of nine of these patients were not toilet trained for urine.…”

Section: Patients Who Underwent a Primary Pull Through At Our Center mentioning

confidence: 99%

Total Colonic Hirschsprung's Disease: The Hypermotility and Skin Rash Protocol

et al. 2019

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Introduction Total colonic Hirschsprung's disease (TCHD) presents a postoperative challenge due to multiple stools and perineal rash. We propose a protocol developed by pediatric surgeons and ostomy nurses to help prevent and treat hypermotility and severe perineal rash, especially in younger children who are not toilet trained. Materials and Methods We retrospectively reviewed our TCHD patients' charts from 2014 to 2017. All patients received a prescribed protocol for the treatment of hypermotility and perineal rash. We describe patients who underwent their pull through before and after the age of urine toilet training, and assessed the number of bowel movements, the perineal skin status, and growth. Results We treated 25 patients. Out of 25, 9 patients received a straight ileoanal pull through before the age of 18 months. Nine of 25 patients presented for a second opinion and had redo pull through. The remaining seven presented for bowel management after having a pull through at another institution. All these were treated following the hypermotility protocol. In total, 19 of 25 patients were not toilet trained. The mean number of bowel movements in all groups was 4 (3–5). All had a resolution of perineal rash and liquid stools after 3 months. Eleven of the 25 patients presented with failure to thrive. Two older patients experienced severe proctalgia requiring replacement of the ileostomy. Conclusion TCHD patients who underwent definitive pull through had nine high incidence of multiple stool, perineal rash, and low growth. With the implementation of bowel management care to slow the stools and a perineal skin protocol to treat the skin, we believe that these symptoms can be minimized even in patients who are not toilet trained. Since the implementation of this protocol, we have changed our practice to perform the pull through in such patients between the age of 6 and 18 months.

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Long‐term outcome and quality of life in patients with total colonic aganglionosis in the Netherlands

Abstract: Children and adolescents with TCA report lower health-related QoL compared with healthy peers, especially in the physical domain. We suggest standardized follow-up and prospective longitudinal future research on functionality and QoL of these patients.

Cited by 22 publications

References 42 publications

Total colonic aganglionosis: multicentre study of surgical treatment and patient-reported outcomes up to adulthood

Total colonic aganglionosis: multicentre study of surgical treatment and patient-reported outcomes up to adulthood

Health-Related Quality of Life in Biliary Atresia Patients with Native Liver or Transplantation

Total Colonic Hirschsprung's Disease: The Hypermotility and Skin Rash Protocol

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