| 1. |
- Bjørnland, Kristin, et al.
(författare)
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A Nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid Hirschsprung disease
- 2017
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Ingår i: Journal of Pediatric Surgery. - : Elsevier BV. - 1531-5037 .- 0022-3468. ; 52:9, s. 1458-1464
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVE: Transanal endorectal pull-through (ERPT) is the most popular technique to treat Hirschsprung disease (HD). Still, there is limited knowledge on long-term bowel function. This cross-sectional, multicenter study assessed long-term bowel function in a large HD population and examined predictors of poor outcome.METHODS: Patients older than four years or their parents filled out a validated questionnaire on bowel function. Clinical details were recorded retrospectively from medical records.RESULTS: 73/200 (37%) patients reported absolutely no impaired bowel function, meaning no constipation, fecal accidents, stoma, appendicostomy or need for enemas. Seven (4%) had a stoma, and 33 (17%) used antegrade or rectal colonic enemas. Most disarrangements of fecal control and constipation were significantly less common in older age group, but abnormal defecation frequency and social problems remained unchanged. Syndromic patients (n=31) experienced frequent fecal accidents (46%) more often than nonsyndromic (14%, P<0.001). Having a syndrome (adjusted OR 5.6, 95% CI 2.1-15, P=0.001) or a complete transanal ERPT (adjusted OR 2.4, 95% CI 1.1-5.7, P=0.038) was significantly associated with poor outcome defined as having a stoma, an appendicostomy, daily fecal accidents or need of regular rectal wash outs.CONCLUSION: A significant number of HD patients experience bowel problems many years after definite surgery. Fecal control was significantly better in older than younger HD patients, but some continued to have considerable bowel problems also as adults. A total transanal ERPT was associated with poorer outcome. Long-term follow-up of HD patients is warranted. Prognosis Study: Level II.
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| 2. |
- Hoel, Anders T., et al.
(författare)
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Reaching adulthood with Hirschsprung's disease : Patient experiences and recommendations for transitional care
- 2021
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Ingår i: Journal of Pediatric Surgery. - : Elsevier BV. - 0022-3468. ; 56:2, s. 257-262
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Tidskriftsartikel (refereegranskat)abstract
- Background/Purpose: The need for transitional care has gained increased focus in the treatment of patients with congenital colorectal disorders. We aimed to acquire in-depth knowledge about the experiences of adult patients with Hirschsprung's disease (HD) and their suggestions for transitional care. Methods: Binational study applying gender equal focus group interviews (FGI). Results: Seventeen (9 men) of 52 invited patients with median age 29 (19–43) years participated. Three themes evolved from the FGI. “Scarred body and soul” describes the somatic and psychosocial challenges the patients experienced and “limited health literacy on HD” refers to the patients' lack of HD knowledge. “Absent transition” depicts missing transitional care and the patients' inability to find adult HD specialists. The adult HD patients strongly recommended transitional care from early teens with focus on information about HD and establishment of a peer-to-peer program. They also emphasized the possibility of being referred to a pelvic floor center. Conclusions: HD negatively influences patients' somatic and psychosocial health in childhood, adolescence and adulthood. Adult HD patients strongly recommend transitional care from early teens and the possibility for referral to a center working with pelvic floor dysfunctions. Level of evidence: IV Type of research: Clinical
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| 3. |
- Hukkinen, Maria, et al.
(författare)
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Management strategies and treatment results of pediatric choledochal malformations in the Nordic countries
- 2020
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Ingår i: HPB. - : Elsevier BV. - 1365-182X. ; 22:1, s. 161-168
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Tidskriftsartikel (refereegranskat)abstract
- Background: Incidence and long-term outcomes of choledochal malformations (CMs) in children remain unclear. Methods: Clinical characteristics, operative details, complications, and follow-up data were collected from eight pediatric surgical centers in Sweden, Norway, Denmark, and Finland, which also answered a questionnaire addressing management practices. Results: During 2000–2017, 126 pediatric CMs were diagnosed, corresponding an incidence of 1:37,400. Diagnostic, treatment, and follow-up practices varied markedly. Of patients with complete clinical data (n = 119), 85% and 11% had type I and IV CMs and were managed by open hepaticojejunostomy at median age of 2.5 (interquartile range 0.46–5.8) years. Associated malformations were more common in fusiform and type IV (23%) than cystic CMs (8%, p = 0.043). Pancreaticobiliary maljunction was more frequently confirmed in patients presenting with pancreatitis (26% vs. 7%, p = 0.005) and with fusiform CMs (56% vs. 25%, p = 0.001). Cholangitis/pancreatitis episodes, occurring in 12% during postoperative follow-up of 4.0 (2.0–7.9) years, associated with longer surveillance (OR 1.32, 95% CI 1.13–1.54, p < 0.001). However, only two thirds of centers continued follow-up until adulthood. No malignancies were reported. Conclusions: CM incidence was higher than traditionally reported among Western populations. Although open hepaticojejunostomy carries good short-term outcomes, long-term morbidity is noteworthy. Standardized evidence-based management strategies and long-term follow-up are encouraged.
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| 4. |
- Pakarinen, Mikko P, et al.
(författare)
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Outcomes of biliary atresia in the Nordic countries - a multicenter study of 158 patients during 2005-2016
- 2018
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Ingår i: Journal of Pediatric Surgery. - : Elsevier BV. - 0022-3468 .- 1531-5037. ; 53:8, s. 1509-1515
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Tidskriftsartikel (refereegranskat)abstract
- Background/purpose: Biliary atresia is the most common reason for newborn cholestasis and pediatric liver transplantation. Even after normalization of serum bilirubin after portoenterostomy, most patients require liver transplantation by adulthood due to expanding fibrosis. We addressed contemporary outcomes of biliary atresia in the Nordic countries. Methods: Data on center and patients characteristics, diagnostic practices, surgical treatment, adjuvant medical therapy after portoenterostomy, follow-up and outcomes were collected from all the Nordic centers involved with biliary atresia care during 2005-2016. Results: Of the 154 patients, 148 underwent portoenterostomy mostly by assigned surgical teams at median age of 64 (interquartile range 37-79) days, and 95 patients (64%) normalized their serum bilirubin concentration while living with native liver. Postoperative adjuvant medical therapy, including steroids, ursodeoxycholic acid and antibiotics was given to 137 (93%) patients. Clearance of jaundice associated with young age at surgery and favorable anatomic type of biliary atresia, whereas annual center caseload >. 3 patients and diagnostic protocol without routine liver biopsy predicted early performance of portoenterostomy. The cumulative 5-year native liver and overall survival estimate was 53% (95% CI 45-62) and 88% (95% CI 83-94), respectively. Portoenterostomy age <. 65. days and annual center caseload >. 3 patients were predictive for long-term native liver survival, while normalization of serum bilirubin after portoenterostomy was the major predictor of both native liver and overall 5-year survival. Conclusions: The outcomes of biliary atresia in the Nordic countries compared well with previous European studies. Further improvement should be pursued by active measures to reduce patient age at portoenterostomy. Retrospective prognosis study: Level II.
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| 5. |
- Stenström, Pernilla, et al.
(författare)
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Bowel symptoms in children with anorectal malformation : A follow-up with a gender and age perspective.
- 2014
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Ingår i: Journal of Pediatric Surgery. - : Elsevier BV. - 1531-5037 .- 0022-3468. ; 49:7, s. 1122-1130
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Tidskriftsartikel (refereegranskat)abstract
- Abstract Background Gender specific outcome for children with anorectal malformations (ARM) is rarely reported although it is important for medical care and in parent counseling. Purpose To assess bowel function according to the Krickenbeck system in relation to ARM-subtype, gender and age. Method All children born with ARM in 1998–2008 and referred to two centers in two different countries were followed up. The bowel function in 50 girls and 71 boys, median age 8years, was analyzed. Results Among those with a perineal fistula, incontinence occurred in 42% of the females and in 10% of the males (p=0.005) whereas constipation occurred in 62% of the females and 35% of the males (p<0.001). No bowel symptoms differed between the females with perineal and vestibular fistulas (p>0.3 for every symptom). Sacral malformations were associated with incontinence only in males with rectourethral fistulas. Constipation among the males differed between the age groups: 58% versus 26% (p=0.013). Bowel symptoms did not change with age among the females. Conclusion Gender differences in outcome for children with ARM must be considered. Males with perineal fistulas had less incontinence and constipation than the females with perineal fistulas. The females with perineal and vestibular fistulas had similar outcomes.
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| 6. |
- Stenström, Pernilla, et al.
(författare)
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Lower urinary tract symptoms in children with anorectal malformations with rectoperineal fistulas.
- 2016
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Ingår i: Journal of Pediatric Surgery. - : Elsevier BV. - 1531-5037 .- 0022-3468. ; 51:8, s. 1234-1240
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Tidskriftsartikel (refereegranskat)abstract
- AIM:The aim was to describe the frequency of lower urinary tract symptoms (LUTS) in children with anorectal malformations with rectoperineal fistulas (ARM-P), as compared with healthy controls based on gender.METHOD:LUTS were defined using the 2014 definitions of the International Children's Continence Society. Data were collected at 2 tertiary pediatric surgery centers in 2 countries from all children aged 4-12years who had undergone an operation for ARM-P.RESULTS:A total of 24 girls and 33 boys, with a median age of 8 (4-12)years, were eligible and compared with 165 controls. Of the patient group, 4 (17%) girls had 8 urinary tract anomalies (UTA), and 8 (24%) boys had 13 UTA. There were no gender differences in LUTS among the patients. The frequency of urinary tract infections was higher among the patients (5/24 girls and 7/55 boys) than the controls (1/55 and 4/110) (p=0.009). More patients (5/24 girls and 5/33 boys) than controls (1/55 and 2/110) used daily urinary medications (p=0.009 and p=0.007, respectively). Patients with UTA reported urinary infections more frequently (3/4 girls and 4/8 boys) than those without UTA (2/20 girls and 0/25 boys) (p=0.018 and p=0.002, respectively).CONCLUSION:Children with ARM-P had more LUTS than controls, and patients with concomitant UTA had more LUTS than patients without UTA. Therefore, children with ARM-P are suggested to have routine follow-up for both UTA and LUTS.
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| 7. |
- Stenström, Pernilla, et al.
(författare)
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Total colonic aganglionosis: : a multicenter study of surgical treatment and patient-reported outcomes up to adulthood
- 2020
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Ingår i: BJS Open. - : Oxford University Press (OUP). - 2474-9842. ; 4:5, s. 943-953
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Tidskriftsartikel (refereegranskat)abstract
- 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 center dot 3 per cent) had died and 102 were traced. Over a median follow-up of 12 (range 0 center dot 3-33) years, bowel continuity was established in 75 (73 center dot 5 per cent) at a median age of 11 (0 center dot 5-156) months. Mucosectomy with a short muscular cuff and straight ileoanal anastomosis (SIAA) (29 patients) or with aJpouch (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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| 8. |
- Tofft, Louise, et al.
(författare)
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Key components of successful transition for adolescents born with anorectal malformations-a Nordic focus group study
- 2022
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Ingår i: International Journal of Adolescent Medicine and Health. - : Walter de Gruyter GmbH. - 0334-0139 .- 2191-0278. ; 34:4, s. 211-218
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Tidskriftsartikel (refereegranskat)abstract
- Transitional care for adolescents with congenital malformations, such as anorectal malformations (ARM), is described sparsely in the literature and referred to as being inadequate. In order to organize future successful healthcare structures, knowledge of patient-reported important aspects of transition is required. The aim of the study was therefore to explore the needs and expectations of transitional-and adult healthcare among adolescents and adults born with ARM. Two tertiary paediatric surgical centres, in collaboration with two tertiary pelvic floor centres, in Sweden and Norway, conducted a qualitative study, involving adolescents and adults born with ARM in focus group discussions regarding transitional care. Discussions were analyzed by qualitative content analysis. Ethical approval was obtained. Sixteen participants (10 women) with a median age of 24 (19-47) years, born with mixed subtypes of ARM were included in gender-divided focus groups. Participants emphasized a need for improved knowledge of ARM, both among patients and adult care providers. Participants identified a need for support with coping strategies regarding challenging social-and intimate situations due to impaired bowel function. Participants pin-pointed well-functioning communication between the patient and the paediatric-and adult care providers as a key factor for a successful transitional process. Further, participants emphasized the importance of easy access to specialized adult healthcare when needed, suggested to be facilitated by appointed patient navigators. Adolescents and adults born with ARM identify improved knowledge of ARM, well-functioning communication and easy access to specialized adult care as key components of a successful transition.
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