Kate Abrahamsson

BLADDER DYSFUNCTION: AN INTEGRAL PART OF THE ECTOPIC URETEROCELE COMPLEX

PURPOSE We evaluate whether bladder dysfunction is common in patients with ectopic ureterocele and, if so, whether it is an integral part of the ectopic ureterocele complex or a result of surgery. MATERIALS AND METHODS From 1986 to 1995, 34 patients with a mean age of 10 months were treated for large or medium ectopic ureteroceles at our institution and 32 participated in postoperative followup. Bladder function was investigated by a careful history and repeat uroflowmetry, and residual urine estimation was assessed by ultrasound and cystometry. RESULTS Of the 32 patients 19 had infrequent voiding and 3 had incontinence. Cystometric bladder capacity was increased to greater than 150% of the normal value for age in 15 of 27 patients (55%). Uroflowmetry revealed greater than 5 ml. residual urine in 15 patients (56%). Postoperatively no radiological signs of bladder neck obstruction were found. Increased bladder capacity and residual urine did not correlate with ureterocele size or location, or surgical procedure. There was no progression of bladder dysfunction with age. CONCLUSIONS Children with ectopic ureterocele are at high risk for a high capacity bladder with incomplete emptying. This bladder dysfunction associated with ectopic ureterocele does not seem to be the result of surgery but an integral part of the disorder.

Hypospadias repair with tubularized incised plate: Does the obstructive flow pattern resolve spontaneously?

OBJECTIVE The aim of this prospective study was to evaluate whether urinary flow improves with time after tubularized incised plate (TIP) repair. PATIENTS AND METHOD Between 1999 and 2003, primary TIP was performed in 126 boys. In patients old enough (48 boys, mean age at surgery 46 months, range 18-103), uroflowmetry was performed 1 year and 7 (median, range 3-10) years post surgery. Miskolc nomograms were used to compare results from the two follow ups (Q(max) in relation to voided volume and age). RESULTS Eleven boys had symptoms of obstruction resulting in intervention. For the other 37 boys, the mean Q(max) was 13.6 ± 5.6 ml/s 1 year postoperatively (mean voided volume 107 ± 43 ml) and 49% had flows below the 5th percentile. Seven years postoperatively the mean Q(max) was 19.0 ± 8.1 ml/s (mean voided volume 235 ± 112 ml) and 32% had flows below the 5th percentile. In the group with flows below the 5th percentile at 1 year, all improved and 28% improved to above the 25th percentile. Proximal hypospadias was more often associated with obstructive flow than distal (75%/75% compared to 43%/21% 1/7 years postoperatively). CONCLUSION We found spontaneous improvement (P = 0.00022) 7 years after TIP repair, although many boys still had a Q(max) in the low normal or obstructive range.

Self-catheterization during adolescence.

Objective. It has been suggested that clean intermittent catheterization (CIC) during the teenage years is associated with poor motivation to comply with the treatment, recurrent urinary tract infections and epididymitis. The aim of this study was to identify the main problems and complications associated with self-CIC in a group of adolescents with no overt neurological problems. Material and methods. The medical records of 24 boys and girls aged ≥12 years on self-CIC with severe bladder dysfunction and intact perineal sensibility and motor function in the lower limbs were reviewed. The median age of the patients was 16.5 years (range 12–24 years) and they had been on CIC for a median of 11 years (range 2–16 years). Results. No urethral damage was reported. Of the 11 patients (46%) who had good CIC routines, two had cystitis and none had epididymitis. Thirteen patients (54%) reported a poor CIC regimen at a median age of 11.5 years (range 9–16 years). In this group, 10 had recurrent cystitis, five had pyelonephritis, four had increasing creatinine levels and three of nine boys had epididymitis. Conclusions. One of the main problems associated with CIC during adolescence is poor compliance with the treatment. Epididymitis and recurrent urinary tract infections were seen more frequently in connection with poor CIC routines.

Half Century of Followup After Ureterosigmoidostomy Performed in Early Childhood

PURPOSE We studied clinical outcomes, especially regarding colorectal adenocarcinoma, in patients who underwent ureterosigmoidostomy in early childhood between 1944 and 1961. MATERIALS AND METHODS A total of 25 consecutive patients underwent ureterosigmoidostomy at a mean age of 3.1 years. The most common indication for ureterosigmoidostomy was bladder exstrophy-epispadias complex. The study period ended in 2010. Patient files were retrospectively evaluated, personal telephone interviews were performed and colorectal histology was reevaluated. One girl who died 4 days postoperatively was excluded. RESULTS Of the 24 patients 17 were alive in 2010 with a mean age of 59 years (range 48 to 67), and 2 still had a functioning ureterosigmoidostomy. A total of 20 patients with a mean age of 33 years had undergone re-diversion at a mean of 30 years postoperatively. Invasive colorectal adenocarcinoma developed in 7 patients and colorectal adenocarcinoma in situ in 1. Five patients died due to generalized colorectal adenocarcinoma. Mean time from ureterosigmoidostomy to diagnosis of invasive colorectal adenocarcinoma was 38 years (range 23 to 55). Three cases were diagnosed at 1, 21 and 25 years after re-diversion. One patient with colorectal adenocarcinoma in situ was 22 years old at polyp resection, which was 20 years after re-diversion. A carcinoid tumor developed in 1 patient. Of the 7 cases of invasive colorectal adenocarcinoma 6 were low differentiated. CONCLUSIONS After a half century of followup in 25 individuals undergoing ureterosigmoidostomy during childhood 17 were still alive and 20 had undergone re-diversion. Compared to the general Swedish population, the risk of colorectal adenocarcinoma was increased 42 times and the incidence of low differentiation was extremely high.

Ultrasound Assessment of Detrusor Thickness in Children and Young Adults with Myelomeningocele

PURPOSE We determine by ultrasonography the range of dT in carefully treated and followed children with myelomeningocele, and evaluate the role of such measurements for the understanding of bladder abnormalities in these patients. MATERIALS AND METHODS We studied 66 children and young adults with MMC (34 males and 32 females, median age 8.1 years, range 1.1 to 20.1). Detrusor thickness was measured with a previously established ultrasonographic technique and the results were compared to those in normal children. The variation in detrusor thickness with degree of bladder dysfunction as well as with bladder wall trabeculation, kidney function and anticholinergic treatment was studied. RESULTS The detrusor of the ventral wall was slightly thinner in children with MMC compared to normal. No significant variation in dT was found for different degrees of bladder dysfunction, bladder wall trabeculation, kidney function or anticholinergic treatment. Boys had thicker detrusor of the ventral wall than girls. CONCLUSIONS Children with MMC, followed closely and treated according to international standards, do not acquire detrusor thickening as measured by ultrasonography. The detrusor thickness did not correlate with the degree of bladder dysfunction or renal function, or with anticholinergic treatment. Bladder wall trabeculation at VCU was not associated with bladder wall thickening on ultrasonography. We postulate that in a closely monitored and actively treated population of patients with MMC muscular hypertrophy and the development of connective tissue in the bladder wall is kept to a minimum.

Estimation of Renal Function in Children and Adolescents With Spinal Dysraphism

PURPOSE In children with spinal dysraphism such as myelomeningocele the relation between muscle mass and body composition varies considerably. Therefore, it is difficult to evaluate the relevance of renal function assessments done with serum creatinine. Since serum cystatin C has been suggested to be independent of body size and composition, this evaluation was compared to chromium(51) edetic acid clearance. MATERIALS AND METHODS Simultaneous measurements of cystatin C and chromium(51) edetic acid clearance were performed prospectively in 65 patients 2 to 19 years old with spinal dysraphism. RESULTS Cystatin C values were within the normal range in all patients, while chromium(51) edetic acid clearance was reduced in 10. A significant relation was seen. CONCLUSIONS Using chromium(51) edetic acid clearance as a gold standard, children with spinal dysraphism and slightly to moderately reduced renal function may remain undiagnosed if cystatin C is used for evaluation.

Psychosocial factors in teenagers and young adults with myelomeningocele and clean intermittent catheterization.

Objective. The aim was to analyse the psychosocial factors of teenagers and young adults with myelomeningocele with at least 5 years’ experience of clean intermittent catheterization (CIC). Material and methods. A qualitative interview technique with semi-structured questions was used in 22 participants aged 13–26 years (median 17) and issues addressed were information given to others, integrity, attitudes to other disabilities, friendship, partnership and sexuality. Results. The participants wanted to inform their peers about the disability but often did not have the courage. Ignorance of the principle of self-CIC among medical staff was confusing. Urinary incontinence after the introduction of the self-CIC regimen was not seen as a problem in comparison with other major disabilities. Half of the participants had peers that they could call a best friend. The subjects wanted more specific information regarding their own sexual function. Finding a partner and becoming a parent were strongly desired but considered problematic. Conclusions. The participants wished to acquire more knowledge about CIC as a bladder-emptying method from the medical staff and also to have the respect to perform self-CIC at examinations. This study did not find any negative psychosocial factors associated with CIC.

Health-related quality of life experiences among children and adolescents born with esophageal atresia: Development of a condition-specific questionnaire for pediatric patients

BACKGROUND/PURPOSE The aims were to present the framework for the development a condition-specific health-related quality-of-life (HRQOL) questionnaire for children with esophageal atresia (EA) and to describe HRQOL experiences reported by children and by their parents. METHODS Utilizing the well-established DISABKIDS methodology, standardized focus group discussions were held and transcribed. HRQOL experiences were identified, content analyzed and evaluated using descriptive statistics. RESULTS 30 families (18 children 8-17years, 32 parents of children 2-17years) participated in ten focus group discussions. 1371 HRQOL experiences were identified referencing social, emotional and physical aspects of eating and drinking (n=368), relationships with other people (n=283), general life issues; physical activity, sleep and general health (n=202), communicative/interactive processes of ones health condition (n=161), body issues (n=109), bothersome symptoms (n=81), impact of health care use/medical treatment (n=78), confidence in oneself and in the future (n=65) and difficulties because of concomitant anomalies (n=24). A basis of two age-related HRQOL questionnaires for children with EA (2-7years, 8-17years) was subsequently constructed. CONCLUSIONS EA interacts with various aspects of the childs life. In addition to HRQOL issues of eating and drinking, social dimensions like relationships and interactions with other people seem to be prominent condition-specific HRQOL parameters. The settings for the development of the first condition-specific HRQOL questionnaires for patients with EA are established.

Physiological Studies at 7 Years of Age in Children Born with Esophageal Atresia.

OBJECTIVE For many years, esophageal atresia (EA) has been curable by surgery. However, severe respiratory morbidity and gastroesophageal reflux (GER) symptoms remain a problem in many patients. The purpose of this study was to describe respiratory and esophageal morbidity, esophageal function, and lung function, including the small airways, in patients with the most common type of the malformation (EA with a distal fistula). METHODS The study comprised 26 children undergoing surgery for EA, who had performed respiratory and esophageal function studies at the age of 7 years in a follow-up program. The study design was retrospective analysis of both these 7-year functional investigations and esophageal and respiratory morbidity from birth to the age of 7 years, as documented in medical records. Pulmonary function was evaluated mainly by spirometry and multiple breath washout (MBW), whereas esophageal function was evaluated by 24-hour pH studies. RESULTS We found a high prevalence of both respiratory (69%) and esophageal (62%) morbidity between birth and 7 years among the EA children. Examination with MBW (peripheral airway function) revealed few abnormal results, whereas spirometry revealed high airway obstruction in half the children, which also correlated well with overall respiratory symptoms (p = 0.047), as well as recurrent pneumonias (p = 0.035). However, no association with GER symptoms was found. In addition, 46% of the children had GER according to pH measurements, which were correlated to clinical GER symptoms but not to respiratory symptoms. CONCLUSION This study confirms a high prevalence of respiratory and esophageal morbidity. In terms of respiratory function, the high proportion with a spirometric abnormality indicated an associated developmental delay/dysfunction in the central airways, whereas the peripheral airways appeared to have normal function at this age. Tracheomalacia may explain the spirometric abnormalities, but this need to be studied in more detail.

Treatment and Follow-Up of Patients with Long-Gap Esophageal Atresia: 15 Years' of Experience from the Western Region of Sweden.

OBJECTIVE This retrospective study aims to report treatment results in patients with long-gap esophageal atresia (LGEA), gross A + B type, and discuss the value of different clinical findings and physiological tests in the follow-up. METHODS This retrospective observational study comprises all patients with LGEA admitted to our department between 1995 and 2010. RESULTS A total of 16 patients were included. Their mean gestational age was 35(+2) weeks and their mean birth weight was 1,945 g (-2.5 standard deviation scores). No catch-up growth in height could be seen and they remained smaller than the average population during the study period. Gastrostomy was performed as the first surgical procedure. Overall, 11 of the 16 patients had a delayed primary anastomosis. Elongation of the distal esophageal segment was required in 3 of the 16 patients and a colonic interposition in 2 of the 16 patients. The median age at definitive surgery was 150 days. All the patients had gastroesophageal reflux after their definitive surgery. Three of the 16 patients required surgery due to aspiration and all 3 had a pathological lung clearance index (LCI) at multiple-breath washout (MBW). At the age of 1 or 7 years, the LCI was pathological in 4 of the 14 patients, and spirometry showed an obstruction in 9 of the 14 patients. CONCLUSION LGEA is a severe congenital malformation, with severe morbidity. No mortality was seen. MBW could be a useful tool for the early detection of progressive pulmonary damage.