Interstitial lung disease (ILD) is a common pulmonary manifestation of rheumatoid arthritis. There is lack of clarity around predictors of mortality and disease behaviour over time in these ...patients.We identified rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients evaluated at National Jewish Health (Denver, CO, USA) from 1995 to 2013 whose baseline high-resolution computed tomography (HRCT) scans showed either a nonspecific interstitial pneumonia (NSIP) or a "definite" or "possible" usual interstitial pneumonia (UIP) pattern. We used univariate, multivariate and longitudinal analytical methods to identify clinical predictors of mortality and to model disease behaviour over time.The cohort included 137 subjects; 108 had UIP on HRCT (RA-UIP) and 29 had NSIP on HRCT (RA-NSIP). Those with RA-UIP had a shorter survival time than those with RA-NSIP (log rank p=0.02). In a model controlling for age, sex, smoking and HRCT pattern, a lower baseline % predicted forced vital capacity (FVC % pred) (HR 1.46; p<0.0001) and a 10% decline in FVC % pred from baseline to any time during follow up (HR 2.57; p<0.0001) were independently associated with an increased risk of death.Data from this study suggest that in RA-ILD, disease progression and survival differ between subgroups defined by HRCT pattern; however, when controlling for potentially influential variables, pulmonary physiology, but not HRCT pattern, independently predicts mortality.
Three patients presented with a colo-cutaneous fistula from the neo-rectum to previous laparoscopic port sites, following an otherwise technically correct pull-through for Hirschsprung disease. We ...evaluated each case to provide guidance to pediatric surgeons on how to avoid this previously unreported complication.
All three patients were male and had a laparoscopic-assisted pull-through for Hirschsprung disease as neonates. All patients underwent intra-operative colonic biopsies above the transition zone, that were closed using an Endoloop ligature (Ethicon), which later proved to be the source of the fistula. The fistulas manifested at variable times: ten days, six months, and five years after the index pull-through. Clinical findings were skin changes, abscess or drainage at previous laparoscopic port sites. Contrast studies were performed to diagnose the underlying pathology. Two patients had primary fistula closure with a diverting ileostomy, followed by ileostomy closure. One patient with delayed symptom presentation had a temporary ileostomy, redo pull-through, and ultimately an ileostomy closure. All three patients recovered without long term sequelae.
The technical error in all of these cases was the utilization of an endoloop ligature for the biopsy site. Furthermore, when the biopsy is taken above a transition zone, it can be postulated that the increased physiologic back-pressure from the sphincter could lead to fistula development and propagation. As more cases are performed with minimally invasive techniques, we must be adamant about evaluating for new, previously un-encountered complications such as this one. An adequate closure of the full-thickness biopsy site could prevent this complication.
Postoperative anal dilations (PAD) are the standard of care for patients after a posterior sagittal anorectoplasty (PSARP) for anorectal malformation (ARM) or a transanal pull-through (TP) procedure ...for Hirschsprung disease (HD). This study assessed the psychosocial impact of PAD among caregivers of children with ARM or HD, which may inform postoperative care strategies.
Caregivers of patients with ARM and HD who underwent PSARP or TP within five years participated in the online survey. Questions included demographics, patient and caregiver experiences with PAD, and baseline psychosocial functioning. Quantitative results were reported descriptively, while qualitative responses were summarized as major themes.
The survey indicated a response rate of 26% caregivers, with most being female (91%) and biological mothers (85%). Patients were mostly male (65%), born with ARM (74%), and were five months old on average when PAD began. Caregivers reported that during PAD, children experienced distress (56%), pain (44%), and fear (41%), while a third noted no negative reactions. Over time, their child's ability to cope with PAD got easier (38%) or stayed the same (41%). Caregivers reported worry/anxiety (88%), guilt (71%), stress (62%), and frustration (35%), noting that additional coping strategies to manage the emotional and logistical challenges of daily PAD would be helpful.
Although PAD is necessary, it can be highly stressful for the patients and their caregivers. Key findings emphasized the need for additional coping strategies and highlighted the importance of integrating psychosocial support into the postoperative care regimen.
Previous studies have examined characteristics critical to make a successful applicant in the pediatric surgery match. No previous study has focused on what applicants consider important as they ...interview pediatric surgery training programs.
Successfully matched applicants to our training program from 2018 to 2020 were identified and a 49-question survey sent electronically. In addition to a descriptive analysis of responses, comparisons between those who matched into one of their top three programs and those who matched outside of their top three programs were made. A Likert scale was used and data are reported as mean (95% confidence intervals).
One hundred and thirty individuals were surveyed. Sixty-three (48%) responded. The most important characteristics identified were total index case volume 1.94 (1.87, 2.00), interactions with faculty on the interview day 1.84 (1.74, 1.94), neonatal case volume 1.75 (1.61, 1.88) and degree of autonomy 1.63 (1.50, 1.77). Respondents also placed a high importance on the in-person interview experience 1.63 (1.49, 1.77). The least important training program characteristics were quantity of vacation time -0.24 (-0.52, 0.05), opportunity to participate in medical mission trips -0.25 (-0.54, 0.06), paid accommodation at the interview -0.29 (-0.57, 0.00), and access to a simulation laboratory -0.51 (-0.78, -0.24).
Clinical volume, neonatal volume, faulty interactions and the experience on the interview day are the most important characteristics to applicants. There were few, if any, characteristics applicants felt were truly unimportant as they weighed their rank lists. Applicants also placed a high level of importance on an in-person interview
Level IV
Enemas have become a common practice for treating fecal incontinence and severe constipation. Several patients receiving enemas complained of severe, colicky, abdominal pain during enema ...administration and complained that the duration for fluid to pass was progressively increasing. Contrast studies showed a startling picture of severe right colon dilatation and a spastic, narrow, left colon. An investigation was started to seek the origin and possible management of this condition.
Medical and radiologic records were reviewed retrospectively, with emphasis on the type and ingredients of enemas used, the duration the patients had been receiving enemas, and their original diagnosis. A literature review was done on previous reports of this condition and publications related to long-term use of enemas.
This series included 22 patients (average age, 19.6 years; range, 8–54) with fecal incontinence due to anorectal malformations (10 cases), myelomeningocele (5), cloaca (2), severe colonic dysmotility (2), Hirschsprung's disease (2), and sacrococcygeal teratoma (1). The average duration of enema use was 13.7 years (range, 4–45). The composition of the enemas included saline/glycerin (six cases), only saline solution (five), saline/glycerin/soap (four), plain water (three), and one case each of molasses/milk, saline/glycerin/soap/phosphate, saline/phosphate, and only phosphate. The enemas were performed in an antegrade fashion in 21 cases and rectally in 1. All patients had a dilated right colon and a narrow, spastic, left, transverse, and descending colon. Four patients underwent colonoscopy, colonic manometry, and mucosal biopsies, which did not help in explaining the etiology of the problem. In the literature, 43 reports mentioned a “long-term follow-up” for the administration of enemas, but we could not find a description of symptoms, such as in our cases.
An intriguing and, to our knowledge, previously unreported complication of chronic enema use is presented. We call attention to an overly concerning complication and report our findings in the hope that they will aid and stimulate more investigations into this condition. Several hypotheses to explain the cause are presented, as well as potential treatment options.
Rectal prolapse after repair of an anorectal malformation (ARM) occurs at a frequency of 3.8% to 60.0%. Different techniques have been described for repair, with a recurrence rate of up to 33%. We ...aimed to describe a new technique for rectal prolapse and present its results.
A retrospective review of our database identified 14 patients with rectal prolapse after posterior sagittal anorectoplasty (PSARP) for an ARM. The study was performed from January 2014 until March 2020. All patients underwent transanal rectal mucosectomy and muscular plication (TRMMP).
All but one patient had PSARP, and the remaining patient had laparoscopic-assisted PSARP. The mean age at repair was 4 years and 8 months (range, 1–12 years). One patient had three previous repair attempts, and another five had only one attempt. The mean follow-up was 2 years and 5 months (range, 3 months to 5 years and 7 months). None of the patients experienced prolapse recurrence or postoperative complications.
This preliminary report showed that TRMMP can be used for rectal prolapse, without the risk of recurrence or complications. Pediatric surgeons may consider this technique as another option for the treatment of rectal prolapse in patients with an ARM.
Purpose
Despite medical advances, individuals with anorectal malformations (ARM) experience significant medical and psychosocial challenges due to their complex conditions. This study aimed to obtain ...the perspectives of adults with ARM throughout their lifetime regarding their medical, school/vocational, and psychosocial functioning.
Methods
A 40-item survey was administered electronically to members of an international ARM Facebook group (56% response rate;
n
= 125). Survey items included demographics, medical diagnosis/treatment, school/workplace accommodations, mental health diagnosis/treatment, and life perspectives.
Results
Majority of respondents were female (73%), aged 25–34 years (31%), Caucasian (92%), US residents (60%), and attended public school (86%). 53% of respondents are currently employed. 32% of respondents received school-based accommodations and 24% at work. 58% of respondents had a mental health diagnosis, with depression (82%) and anxiety (81%) being the most common.
Conclusions
Results suggest that adults with ARM experience ongoing difficulties related to schooling, employment, and mental health, in addition to medical complications. It is becoming increasingly clear that improving patients’ physical well-being is not enough; psychosocial concerns must also be addressed directly. Thus, it is important for clinicians to be aware of and partner with psychosocial providers to support these challenges associated with ARM, to maximize patients’ overall health and well-being.
Purpose
To evaluate individual and community sociodemographic factors that predict bowel regimen adherence in youth and young adults with Spina Bifida (SB) following participation in a bowel ...management program (BMP).
Methods
Participants were drawn from clinical cases seen through an International Center for Colorectal and Urogenital Care. Area deprivation index (ADI) scores were extracted from participant addresses and bowel regimen adherence data were collected from the electronic medical record (EMR).
Results
Participants’ mean age was 8.06 years old, 51.7% were male, 72.4% white, 37.9% Hispanic, 56.9% government insurance, 89.7% myelomeningocele, 15.5% non-adherent. Average neighborhood disadvantage was 5.19 (SD:2.83, range:1–10). After controlling for variables correlated with adherence (
p
< .20), every one decile higher neighborhood disadvantage score was associated with a 48% decrease in the odds of being adherent (OR = 0.52,
p
= .005, 95% CI: − 101.90, − 0.21).
Conclusion
Our results suggest that neighborhood disadvantage is a strong predictor of medical adherence following a BMP, more so than other sociodemographic and health-related variables. These results may assist with identifying which individuals may be at higher risk for poor health outcomes due to neighborhood socioeconomic disadvantage and help health care systems intervene proactively.
Purpose
To present the functional results after a transanal proximal rectosigmoidectomy in patients with severe idiopathic constipation in which medical treatment has failed.
Methods
Patients with ...severe idiopathic constipation who underwent transanal proximal rectosigmoidectomy (TPRS) at Children’s Hospital Colorado between June 2019 and March 2024 were included in the study. We compared multiple pre- and post-operative outcome measures and the patient’s bowel regimen before and after resection.
Results
Fourteen patients underwent TPRS, 10 of whom were male. The average age at the time of surgery was 10.1 years (range 5–19). Seven patients have moderate to severe autism. Constipation-related clinic visits, family calls, procedural intervention, emergency room visits, and hospitalizations notably decreased frequency after TPRS. Laxative dosages and enema volume requirements were also reduced after surgery. Before surgery, all the patients suffered from daily fecal accidents, while post-operatively, all were completely free of stool accidents.
Conclusion
In our experience, for patients who suffer from severe medically refractory idiopathic constipation, TPRS has provided improvement in their symptoms and decreased the complications inherent to this chronic disease. Parents and patients attest to a profound positive transformation in their quality of life after surgery.
Fertility and pregnancy outcome data in female patients with anorectal malformations is limited. Those with complex anatomy, mullerian anomalies, or extensive surgical histories may face fertility ...challenges. The purpose of this study was to report fertility concerns and describe pregnancy outcomes in this patient population.
This was an IRB approved, prospective study of patients in the Adult Colorectal Research Registry who completed surveys between November 2021 and August 2022. Reproductive health surveys were administered through REDCap. Female patients between the ages of 18 and 80 years with ARM were included.
Fifty-four patients with ARM were included in the study (Table 1), ages ranged from 22 - 80 years with a median age of 32.5 years. Fertility concerns were highest (59%) in patients with a diagnosis of cloaca (Table 1). Twenty-nine (54%) reported that they have not tried to conceive, of which 45% were concerned about fertility (Figure 1). In the 25 females who tried to conceive, four were unsuccessful (16%) and 21 (84%) reported pregnancy ending in either miscarriage or delivery. Concerns about fertility were reported in one third of patients that were able to conceive compared to 75% of those that were unsuccessful. There were 15 miscarriages reported by 10 women and 29 deliveries reported by 17 women in the cohort. Duration of trying to get pregnant ranged from one month to over 20 years (median 12 months). Of the 23 women with fertility concerns, 57% (13/23) were seen by a fertility specialist including four women who have not yet tried to conceive. Six females reported needing assisted reproductive technologies (IVF, donor egg, medical stimulated ovulation, surrogacy). Fourteen females (26%) were diagnosed with fertility problems, which included two with ovulation problems, four with damaged or blocked tubes, nine with abnormalities of the uterus, three with diminished ovarian reserve and two with unexplained infertility.
Providers should be aware of fertility concerns in patients with ARM. Proactive counseling should be considered in patients who desire future fertility, especially those with a history of cloaca or complex ARM. There should be a low threshold to refer these patients to a fertility specialist.
Supporting Figures or Tables
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