A 3-year-old boy with non-syndromic, rectosigmoid Hirschsprung disease was operated on at 4 weeks of age with total transanal endorectal pull-through. He has had several episodes of mild Hirschsprung-associated enterocolitis (HAEC), with foul-smelling, explosive diarrhea. He has responded well to rectal irrigations and oral metronidazol. Recently, he had one of these episodes. The parents describe that he needs irrigations almost every day, also between the enterocolitis episodes, to pass stools.
A retrospective comparative study of prospectively collected data.To compare clinical, radiographic, and health-related quality of life (Scoliosis Research Society [SRS]-24) outcomes in patients undergoing hemivertebra excision by simultaneous anteroposterior (AP) or posterolateral only approach (PL).AP hemivertebra resection for congenital scoliosis has provided reliable and safe long-term outcomes with respect to clinical and radiographic findings. Recently, PL excision has been described with rather high implant failure and revision rates. There are no studies comparing outcomes of AP and PL hemivertebra excision in 1 center. In addition, studies reporting health-related quality of life after surgery for congenital scoliosis are few.Between 2001 and 2008, 12 AP and 11 PL hemivertebra excisions were conducted in 21 patients (16 males) with congenital scoliosis or kyphoscoliosis due to unbalanced hemivertebrae in our hospital. The average age at surgery was 4.0 years (range, 1.2-11.4) and the mean follow-up time 2.5 years (range, 1-7 years).Before surgery, the mean Cobb angle of the main curve was 33° (range, 25°-45°) in the AP group and 43° (range, 26°-87°) in the PL group. At the final follow-up visit, the main curve had been corrected to a mean of 12° (range, 2°-27°) and 15° (range, 6°-28°), respectively (P = NS). The Scoliosis Research Society-24 total scores were similar, 101 (range, 89-109) for the AP and 100 (range, 85-106) for the PL group at the final follow-up visit. Any complication was observed in 8% (1/12) of patients in the AP group and, whereas the PL group had 40% (4/10) complication rate with 2 temporary neurologic deficits, no implant failures were observed. All patients underwent spinal fusion during follow-up.PL resection is technically more demanding and slightly faster method for hemivertebral resection. It has nearly as good correction rate as the AP-method but more minor complications. Hemivertebrectomy seems to provide a controllable untethering effect in patients with a symptomatic tethered cord.
Sexual dysfunction and impaired quality of life due to fecal incontinence are common after classic operations for anorectal malformations. We hypothesized that modern repairs may result in improved outcomes.Following ethical approval for this single institution cross-sectional study, all patients 16 years or older treated for rectourethral, vestibular or perineal fistula from 1983 onward were sent detailed postal questionnaires on sexual function and quality of life. Each respondent was age and gender matched to 3 controls randomly selected from the general population. Penoscrotal/gynecologic abnormalities were obtained from the records.A total of 41 patients (62%) with a median age of 22 years participated in the study. Of the patients 20 were males with rectourethral fistula (prostatic in 60%), 10 were females with vestibular/perineal fistula and 11 were males with low malformations. Although experience of sexual relationships and orgasmic function were reported in comparable proportions to controls, age at coital debut was significantly delayed in all groups of patients (p ≤0.046). Erectile function was preserved after sagittal repair but absent ejaculations or azoospermia affected 3 males with rectourethral fistula (15%). Penoscrotal/gynecologic abnormalities affected 12% of patients. Overall quality of life scores were comparable to controls but a trend was found for lower scores on emotional items in males with rectourethral fistula (p = 0.06) and for a negative effect on sexual life in females (p = 0.03).While erectile and orgasmic function appear preserved after sagittal repair, further evaluation of fertility issues in males with rectourethral fistula is indicated. Larger multicenter studies are needed to confirm our findings.
Background Anorectal malformations (ARMs) and Hirschsprung's disease (HD) are chronic bowel conditions associated with varying degrees of fecal incontinence. We aimed to discuss the contemporary status of social morbidity associated with ARMs and HD in the long term after contemporary treatments. Materials and Methods The bowel functional outcomes of our recent institutional series up to adulthood were reviewed and compared for ARMs and HD. The Rintala score was used to evaluate bowel function, which includes an assessment of the social effects of the condition. In this study, the social outcomes in our population of patients with ARMs and HD were specifically analyzed in relation to the surveyed functional outcomes, our previously collected data on quality of life (QoL) and the current literature. Results Mild ARMs were associated with a minimal risk of social morbidity consistent with good outcomes. In severe ARMs and HD, social problems were reported steadily among all age groups studied. Impairment of domains of fecal control were present among 53 to 89% of patients with social problems, including frequent symptoms (>1/week) in up to 39%. Involuntary gas leakage contributed to social morbidity in 15 to 27%. Comparison with QoL data suggested that social morbidity experienced during childhood may continue to affect emotional domains of QoL in later life, despite improvements in bowel function by adulthood. Conclusion Patients with severe ARMs and HD are at risk of social restrictions from impairment of bowel function despite contemporary surgical treatments. However, the QoL outcomes in adulthood may be more influenced by self-perceptions of illness formed from childhood than disease-specific factors. Strategies to reduce the psychological morbidity associated with these conditions that includes parental involvement from the outset may improve outcomes.
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