Does Length of Extended Resection Beyond Transition Zone Change Clinical Outcome for Hirschsprung Pull-Through?

INTRODUCTION: A proximal resection margin greater than 5 cm from the intra-operative histologically determined transition zone has been deemed necessary to minimize the risk of transition zone pull-through. This extended resection may require the sacrifice of vascular supply and even further bowel resection. The impact of extended proximal resection margin on post-operative complications and functional outcomes is unclear. METHODS: A retrospective chart review of patients who underwent primary pull-through for Hirschsprung disease at a single institution between January 2008 and December 2022 was performed. An adequate proximal margin was defined by a circumferential normally ganglionated ring and absence of hypertrophic nerves. The extended margin was defined as the total length of proximal colon with normal ganglion cells and without hypertrophic nerves. Fecal incontinence severity was assessed with the Pediatric Fecal Incontinence Severity Score (PFISS). RESULTS: Eighty seven patients met criteria for inclusion. Median age at primary pull-through was 17 days (IQR 10-92 days), 55% (n = 48) of patients had an extended proximal margin (EPM) ≤ 5 cm, and 45% (n = 39) had an EPM > 5 cm. An EPM ≤5 cm was not associated with increased rates of Hirschsprung associated enterocolitis (≤5 cm 43%, >5 cm 39%, P = 0.701), diversion post pull-through (≤5 cm 10%, >5 cm 5%, P = 0.367) or reoperation for transition zone pull-through (≤5 cm 3%, >5 cm 0%, P = 0.112). EPM ≤5 cm had more frequent involuntary daytime bowel movements (P = 0.041) and more frequent voluntary bowel movements (P = 0.035). There were no differences in other measures of fecal incontinence severity. CONCLUSIONS: Shorter proximal extended margins beyond the adequate ganglionated margin do not significantly impact post-operative complication rates and have an unclear effect on fecal incontinence. TYPE OF STUDY: Case Control. LEVEL OF EVIDENCE: Level III.

Sacral Nerve Stimulation in Children with Medically Refractory Fecal Incontinence or Severe Constipation.

INTRODUCTION: The goal of this study was to evaluate outcomes in patients treated with sacral nerve stimulation (SNS) for medically refractory fecal incontinence or severe constipation. METHODS: We performed a retrospective cohort study of all patients treated with SNS after failed medical management at a single center between 9/1/2015 and 6/30/2022. Demographic and clinical data was extracted from the electronic medical record. Rates of involuntary bowel movements were evaluated using a bowel severity score questionnaire and compared pre- and post-SNS using McNemar and McNemar-Bowker tests. RESULTS: 70 patients underwent SNS placement. The median age was 12.8 years (IQR 8.6-16.0) and 61.4% were male. The most common diagnosis was idiopathic constipation (67.1%), followed by anorectal malformation (15.7%), and others. 43 patients had severity scores recorded both pre- and at least 90 days post-SNS insertion. The rates of daytime and nighttime involuntary bowel movements were significantly different pre-compared to post-SNS placement (p = 0.038 and p = 0.049, respectively). The rate of daytime and nighttime fecal continence increased from 44% to 58.1% and 53.5%-83.7%, respectively. The rate of at least weekly daytime and nighttime fecal incontinence decreased from 48.8% to 18.7% and 34.9%-7.0%, respectively. Minor pain/neurological symptoms occurred in 40% of patients, while 5.7% developed a wound infection. Further surgery for the SNS was required in 40% of patients. CONCLUSIONS: SNS placement can be an effective treatment for medically refractory fecal incontinence. Minor complications and the need for further procedures are common, while more serious complications like wound infections are rare. TYPE OF STUDY: Retrospective Cohort Study. LEVEL OF EVIDENCE: Level 3.

What parents need to know about Hirschsprung disease.

Hirschsprung's disease (HD) is a complex colorectal disorder that can be challenging to understand and overwhelming to many parents. Counseling parents is critical for ensuring they understand their child's condition, how it must be treated, pitfalls that can occur during treatment, and how they will do in the long term. This article breaks down this process into a series of informally worded questions and reviews evidence-based discussion points around the most common concerns parents will have: survival, pathophysiology, surgical correction and complications, etiology and genetic basis, associated issues, outcomes, and familial nature. Where evidence is unclear, expert opinion is included. Discussion is geared towards the parents of a newly diagnosed and otherwise healthy neonate, with a brief section on complex situations including associated syndromes and late diagnosis. Embarking on the longitudinal care of a child with HD is a complex undertaking and requires significant parental education to establish a proper parent-surgeon relationship, an essential factor for optimizing outcome and family satisfaction.

Damaged anal canal as a cause of fecal incontinence after surgical repair for Hirschsprung disease - a preventable and under-reported complication.

INTRODUCTION: Fecal incontinence after the surgical repair of Hirschsprung disease is a potentially preventable complication that carries a negative impact on patient's quality of life. METHODS: Patients that were previously operated for Hirschsprung disease and presented to our bowel management clinic with the complaint of fecal incontinence were retrospectively reviewed. All patients underwent a rectal examination under anesthesia looking for anatomic explanations for their incontinence. RESULTS: One hundred three patients were identified. 54 patients had a damaged anal canal. 22 patients also had a patulous anus. The operative reports mentioned the pectinate line in 32 patients, in 12 it was not mentioned, and in 10 patients the operative report was not available. All patients with a damaged anal canal suffered from true fecal incontinence; 45 of them are on daily enemas (41 are clean and 4 are still having "accidents"), 7 are not doing bowel management due to noncompliance and 2 patients have a permanent ileostomy. 49 patients did not have a damaged anal canal, 25 of those responded to changes in diet and medication and are having voluntary bowel movements. CONCLUSION: Fecal incontinence may occur after an operation for Hirschsprung disease. When the anal canal is damaged, incontinence is always present, severe, and probably permanent. The preservation of the anal canal may avoid this complication.

Surgical outcomes, bowel habits and quality of life in young patients after ileoanal anastomosis for ulcerative colitis.

PURPOSE: We aim to investigate the postoperative outcomes, bowel habits and quality of life (QoL) of younger pediatric ulcerative colitis (UC) patients following surgical intervention compared to an older pediatric population. METHODS: Medical records of UC patients after colectomy with ileoanal reconstruction (2002-2013) at our institution were reviewed. Patients/parents completed a QoL, bowel habits and disease course questionnaire. Surgical outcomes, bowel habits and QoL were reported comparing the younger (≤11years old, n=26) to older (>11years old, n=38) cohorts. RESULTS: The mean age at colectomy was 7.04±0.63years vs 14.71±0.32years in the two groups. Patients had a significant (P<0.001) reduction in stooling frequency after surgery in both age groups and had favorable rates of fecal continence. The frequency of pouchitis and postoperative small bowel obstruction was similar in both cohorts. Dehydration was slightly increased in the younger population but not significant. Anastomotic leak and stricture rates were slightly reduced in younger patients. Postoperative QoL was favorable and similar regardless of age at surgery. CONCLUSIONS: Colectomy with ileoanal anastomosis for young children (≤11years old) with UC is without increased complications relative to older patients and maintains a postoperative QoL and stool patterns.

Treatment of fecal incontinence with a comprehensive bowel management program.

PURPOSE: Many articles describe the antegrade continence enemas (ACEs), but few refer to a bowel management program. A successful ACE may not help a patient without such management. Valuable lessons were learned by implementation of bowel management in 495 fecally incontinent patients. METHODS: We previously reported 201 patients. Thereafter, another 294 patients participated in our program. On the basis of a contrast enema and symptoms, they were divided as follows: (a) 220 constipated patients and (b) 74 patients with tendency toward diarrhea. Colonic stool was monitored with abdominal radiographs, modifying the management according to the patient's response and radiologic findings. For constipated patients, the emphasis was on using large enemas. For patients with tendency toward diarrhea, we used small enemas, a constipating diet, loperamide, and pectin. Diagnoses included anorectal malformation (223), Hirschsprung's (36), spina bifida (12), and miscellaneous (23). RESULTS: The management was successful in 279 patients (95%)-higher in constipated patients (98%) and less successful in patients with tendency toward diarrhea (84%). CONCLUSIONS: The key to a successful bowel management program rests in tailoring the type of enema, medication, and diet to the specific type of colon. The best way to determine the effect of an enema is with an abdominal film. The ACE procedures should be recommended only after successful bowel management.