Surgical management of complicated Crohn's disease.

Surgical management of pediatric Crohn's disease is fundamentally palliative, aiming to treat the sequalae of complicated disease while preserving intestinal length. Multidisciplinary discussion of risk factors and quality of life should take place prior to operative intervention. Though the surgical management of pediatric Crohn's disease is largely based on the adult literature, there are considerations specific to the pediatric population - notably disease and treatment effects on growth and development. Intrabdominal abscess is approached with percutaneous drainage when feasible, reserving surgical intervention for the patient who is unstable or failing medical therapy. Pediatric patients with fibrostenotic disease should be considered for strictureplasty when possible, for maximum preservation of bowel length. Patients with medically refractory Crohn's proctocolitis should be treated initially with fecal diversion without proctocolectomy.

Does Delayed Diagnosis of Hirschsprung Disease Impact Post-operative and Functional Outcomes? A Multi-Center Review From the Pediatric Colorectal and Pelvic Learning Consortium.

BACKGROUND: Hirschsprung Disease (HD) is a rare cause of functional bowel obstruction in children. Patients are typically diagnosed in the neonatal period and undergo pull-through (PT) soon after diagnosis. The optimal management and post-operative outcomes of children who present in a delayed fashion are unknown. METHODS: A multi-center retrospective review of children with HD was performed at participating Pediatric Colorectal and Pelvic Learning Consortium sites. Children were stratified by age at diagnosis (neonates <29 days; infants 29 days-12 months; toddler 1 year-5 years and child >5 years). RESULTS: 679 patients with HD from 14 sites were included; Most (69%) were diagnosed in the neonatal period. Age at diagnosis was not associated with differences in 30-day complication rates or need for PT revision. Older age at diagnosis was associated with a greater likelihood of undergoing fecal diversion after PT (neonate 10%, infant 12%, toddler 26%, child 28%, P < 0.001) and a greater need for intervention for constipation or incontinence postoperatively (neonate 56%, infant 62%, toddler 78%, child 69%, P < 0.001). CONCLUSION: Delayed diagnosis of HD does not impact 30-day post-operative outcomes or need for revision surgery but, delayed diagnosis is associated with increased need for fecal diversion after pull-through. LEVEL OF EVIDENCE: III.

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.

Rectal Prolapse Following Repair of Anorectal Malformation: Incidence, Risk Factors, and Management.

BACKGROUND: The incidence and optimal management of rectal prolapse following repair of an anorectal malformation (ARM) has not been well-defined. METHODS: A retrospective cohort study was performed utilizing data from the Pediatric Colorectal and Pelvic Learning Consortium registry. All children with a history of ARM repair were included. Our primary outcome was rectal prolapse. Secondary outcomes included operative management of prolapse and anoplasty stricture following operative management of prolapse. Univariate analyses were performed to identify patient factors associated with our primary and secondary outcomes. A multivariable logistic regression was developed to assess the association between laparoscopic ARM repair and rectal prolapse. RESULTS: A total of 1140 patients met inclusion criteria; 163 (14.3%) developed rectal prolapse. On univariate analysis, prolapse was significantly associated with male sex, sacral abnormalities, ARM type, ARM complexity, and laparoscopic ARM repairs (p < 0.001). ARM types with the highest rates of prolapse included rectourethral-prostatic fistula (29.2%), rectovesical/bladder neck fistula (28.8%), and cloaca (25.0%). Of those who developed prolapse, 110 (67.5%) underwent operative management. Anoplasty strictures developed in 27 (24.5%) patients after prolapse repair. After controlling for ARM type and hospital, laparoscopic ARM repair was not significantly associated with prolapse (adjusted odds ratio (95% CI): 1.50 (0.84, 2.66), p = 0.17). CONCLUSION: Rectal prolapse develops in a significant subset of patients following ARM repair. Risk factors for prolapse include male sex, complex ARM type, and sacral abnormalities. Further research investigating the indications for operative management of prolapse and operative techniques for prolapse repair are needed to define optimal treatment. TYPE OF STUDY: Retrospective cohort study. LEVEL OF EVIDENCE: II.

Long-Term Male Sexual Function and Fecal Incontinence Outcomes for Adult Patients with Hirschsprung Disease or Anorectal Malformation.

BACKGROUND: This study sought to characterize sexual function and fecal incontinence related quality of life (QOL) outcomes for adult males with anorectal malformation (ARM) or Hirschsprung's Disease (HD). METHODS: We conducted a cross-sectional survey study of male patients ≥18 years with ARM or HD. Patients were identified from our institutional database, contacted and consented by telephone, and sent a REDCap survey via email. The International Index of Erectile Function (IIEF-5) and Male Sexual Health Questionnaire (MSHQ) evaluated erectile dysfunction (ED) and ejaculatory dysfunction (EjD), respectively. The Cleveland Clinic Incontinence Score (CCIS) and the Fecal Incontinence Quality of Life Scale (FIQLS) assessed fecal incontinence-related outcomes. A linear regression analysis of IIEF-5 scores compared to CCIS scores was used to evaluate for an association between ED and incontinence. RESULTS: Of 63 patients contacted, 48 completed the survey. The median age for respondents was 22.5 years (IQR 20-25). There were 19 patients with HD and 29 patients with ARM. On the IIEF-5 survey, 35.3% report some level of ED. On the MSHQ-EjD survey, the median score was 14 out of 15 (IQR 10.75-15), indicating few EjD concerns. The median CCIS was 5 (IQR 2.25-7.75) and the median FIQL scores ranged from 2.7 to 3.5 depending on the domain assessed, demonstrating some QOL challenges secondary to fecal incontinence. On linear regression analysis, IIEF-5 and CCIS scores were weakly associated (B = -0.55, p = 0.045). CONCLUSIONS: Male adult patients with ARM or HD may have ongoing concerns with sexual function and fecal incontinence. LEVEL OF EVIDENCE: Level 4. TYPE OF STUDY: Cross-Sectional Survey Study.

Hirschsprung-Associated Enterocolitis at a Referral Institution: A Retrospective Review.

BACKGROUND: Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality amongst patients with Hirschsprung disease (HD); rectal Botulinum toxin (Botox) has been reported a possible prevention strategy. We aimed to evaluate our institution's historic cohort of HD patients, first to determine our incidence of HAEC and second to begin assessing the effect of Botox on HAEC incidence. METHODS: Patients with HD seen at our institution between 2005 and 2019 were reviewed. Incidence of HD and frequencies of HAEC and Botox injections were tallied. Associations between initial Botox treatment or transition zone and HAEC incidence were evaluated. RESULTS: We reviewed 221 patients; 200 were included for analysis. One hundred thirteen (56.5%) patients underwent primary pull-through at a median age of 24 days (IQR 91). Eighty-seven (43.5%) patients with initial ostomy had their intestinal continuity reestablished at a median of 318 days (IQR 595). Ninety-four (49.5%) experienced at least one episode of HAEC and 62 (66%) experienced multiple episodes of HAEC. Nineteen (9.6%) patients had total colonic HD and had an increased total incidence of HAEC compared to patients without total colonic HD (89% vs 44%, p < 0.001). Six (2.9%) patients received Botox injections at the time of pull-through or ostomy takedown; one experienced an episode of HAEC (versus 50.7% of the patients who were confirmed to have not received Botox injections at their surgery, p = 0.102). CONCLUSION: Further prospective study on Botox's effect on Hirschsprung-associated enterocolitis is required and is the next step in our investigation. 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.

Antithrombin III infusion improves anticoagulation in congenital diaphragmatic hernia patients on extracorporeal membrane oxygenation.

PURPOSE: Achieving effective anticoagulation during neonatal extracorporeal membrane oxygenation (ECMO) without increasing the risk of hemorrhage remains challenging. The use of antithrombin III (AT-III) for this purpose has been examined, but studies have been limited to intermittent bolus dosing. We aimed to evaluate the efficacy and safety of an institutionally developed AT-III continuous infusion protocol in neonates receiving ECMO for the treatment of congenital diaphragmatic hernia (CDH). METHODS: In this single center, retrospective study, all neonates with a CDH who received ECMO support during the study period were included. Data on anticoagulation labs and therapy, life-threatening bleeding, and circuit changes were analyzed. RESULTS: Eleven patients were divided into two groups: patients with AT-III continuous infusion (n = 5) and without (n = 6). There were no differences in the gestational age (p = 0.29), sex (p = 1.00), ECMO duration (p = 0.59), or initial AT-III levels (p = 0.76) between groups. Patients in the AT-III infusion group had on average 18.5% higher AT-III levels (p < 0.0001). Patients receiving continuous AT-III infusions spent a significantly higher percentage of ECMO time within the therapeutic range, measured using anti-Factor Xa levels (64.9±4.2% vs. 29.1±8.57%, p = 0.008), and required fewer changes to the heparin infusion rate (6.48±0.88 vs 2.38±0.36 changes/day changes/day, p = 0.005). Multivariate analysis revealed continuous infusion of AT-III did not increase the rate of intracranial or surgical bleeding (p = 0.27). CONCLUSION: AT-III as a continuous infusion in CDH neonates on ECMO provides a decreased need to modify heparin infusion and more consistent therapeutic anticoagulation without increasing the risk of life-threatening bleeding.

Hirschsprung's disease in low- and middle-income countries.

Hirschsprung's disease (HD) is one of the most common causes of pediatric bowel obstruction in low- and middle-income countries (LMICs). This paper describes the unique aspects of presentation, diagnosis, management and post-operative care and outcomes of HD in LMICs. In LMICs, patients with HD are much more likely to present in a delayed fashion with subsequent increased morbidity and mortality including higher rates of chronic obstruction, malnutrition with failure to thrive, complete obstruction and perforation. There are multifactorial causes for delay, with opportunities to improve initial timely diagnosis and referral, support families to address socioeconomic and cultural barriers, and improve workforce and infrastructure resources to provide definitive care. In LMICs, the diagnosis is often made based on clinical presentation and radiographic findings as pathological services may be limited. Initial diversion with multi-stage procedure, instead of a single-stage pull-through, predominates. This is also a result of multifactorial causes, including initial presentation to general surgeons at first-level hospitals instead of pediatric surgeons, delayed presentation with sick, malnourished children with significantly distended bowel, and a lack of fresh-frozen pathological services to guide the extent of resection. Post-operatively, HD patients in LMICs experience higher complication and mortality rates - likely stemming from sicker baseline presentations and more limited resources. Significant recent advances in care have occurred for patients with HD in LMICs, while opportunities to continue to improve care remain.

Surgical history of Hirschsprung disease.

Harald Hirschsprung presented the first comprehensive and detailed description of clinical outcomes in patients with congenital megacolon. In the century following his discovery, we have seen realization of the etiology, diagnosis, and treatment of Hirschsprung disease. In this article we describe the surgical management of Hirschsprung disease starting with Orvar Swenson, who pioneered the field with the first full thickness transrectal dissection followed by several additional innovative surgeons who have contributed to the modifications that have brought us to the modern surgical management of Hirschsprung Disease.

Hospital and Professional Charges and Reimbursement Patterns in Pediatric Extracorporeal Membrane Oxygenation.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) is an expensive therapeutic modality. We sought to identify the main charge contributors to patient bills and analyze their patterns of reimbursement. We additionally sought to evaluate the impact of 2015 Current Procedural Terminology (CPT) code changes in professional billing for pediatric surgeons. METHODS: A retrospective review of ECMO cases at a standalone quaternary children's hospital between 2008-2017 was performed. Itemized hospital and professional bills were analyzed. RESULTS: Top charges included room rates, nitric oxide, medications, invasive support and monitoring, and laboratory testing. Average reimbursement was ∼60% for hospital and ∼36% for professional bills. CPT code changes in 2015 represented a 65% reduction in RVUs and 46% reduction in professional charges. Medicaid reimbursement for professional billing remained stable at 9%, and commercial reimbursement fell from 70% to 59% during the study period. CONCLUSIONS: The main drivers of ECMO charges are unrelated to ECMO supplies or surgery. Evidence-based guidelines for ECMO management could make a difference in healthcare expenditure. Modern CPT codes depreciate RVUs and professional charges, compromising revenue. As the infrastructure required to provide this service is costly, diminishing returns may limit access to this therapy.

Prospective quality of life outcomes in pediatric fecal incontinence following bowel management.

BACKGROUND: Severe fecal incontinence (FI) is common in patients both with and without anorectal malformations. Whether a formal bowel management program (BMP) has significant effects on FI, psychosocial development of the child, and caregiver stress is poorly understood. We hypothesize that BMP participation results in long-term clinical and quality of life (QOL) improvements for patients and caregivers. METHODS: Using a prospective cohort study over three years, 342 children (age 3-12 years) and caregivers were followed for one year after attending a week-long BMP, during which a regimen was tailored to promote daily stool evacuation.FI QOL was measured with the validated Cincinnati Fecal Incontinence Scale (CINCY-FIS), evaluating multiple subscales, including parental stress. Scores were obtained at multiple timepoints following BMP (baseline, 2 weeks, 3 months, 1 year). RESULTS: Within 2 weeks, BMP participation significantly improved FI with increased frequency of daily daytime voluntary bowel movements (20%-70%, p < 0.001) and decreased daily daytime and nighttimeinvoluntary bowel movements (60%-20%, p < 0.001; 30%-10%, p < 0.05). Marked improvements in CINCY-FIS were observed across multiple QOL subscales, with the greatest in parental stress, and sustained through one year. CONCLUSIONS: BMP results in significant and sustained improvement in FI and QOL for patients and caregivers.

A child presents with perianal symptoms - how often is this Crohn's disease?

BACKGROUND: The cumulative incidence and predictors of future diagnosis of Crohn's disease (CD) following presentation with perianal symptoms, such as anorectal abscess, fistula or fissure, is unknown. METHODS: A 5-year retrospective review of children presenting with perianal symptoms without prior CD diagnosis was performed. Institutional cumulative incidence of CD was calculated to determine the risk of CD presenting with perianal symptoms. RESULTS: 1140 children presented for evaluation of an anorectal abscess (n = 232), fistula (n = 49), or fissure (n = 859). Thirty-five were later diagnosed with CD, resulting in an incidence of 3%. Prognostic indicators of future CD diagnosis included increased age per every additional year (RR 1.19, 95% CI: 1.14-1.25, p < 0.001), male sex (RR 2.12, 95% CI 1.07-4.22, p = 0.024), or perianal fistula (RR 4.67, 95% CI 2.26-9.67, p = 0.022). Among those diagnosed with CD, 57% experienced and had a documented history of a CD-associated symptom prior to perianal symptom onset. Absence of symptoms resulted in delayed diagnosis (43 vs 3 days, p < 0.02). CONCLUSION: Of children presenting with a perianal symptom, three percent will eventually be diagnosed with CD. At highest risk (35%) were males aged 10 years or older with a perianal fistula; which should prompt expeditious workup.

Creating a collaborative program for the care of children with colorectal and pelvic problems.

The treatment of patients with colorectal disorders and their associated urologic, gynecologic, gastrointestinal, spinal, and orthopedic anomalies requires care from various medical and surgical specialties over the course of their lifetime. This is ideally handled by a collaborative center which facilitates the assessment and development of a long-term patient care plan among multiple specialties which can enhance the quality of care, improve communication among different specialties, and improve patient satisfaction and outcomes. We describe the process, as well as lessons learned in developing such a center.

A comparison of surgical complications after appendicostomy and neoappendicostomy in pediatric patients.

PURPOSE: There are limited data on neoappendicostomy complications owing to small patient populations. This study compares appendicostomy and neoappendicostomy procedures with an emphasis on major postoperative complications requiring either a surgical or interventional radiology procedure. METHOD: A single-institution retrospective review included all patients with complete medical charts in the Cincinnati Children's Colorectal Database who underwent either an appendicostomy or neoappendicostomy from August 2005 through December 2016. Demographics, details of the procedure, and major postoperative complications were evaluated. RESULTS: 261 patients (appendicostomy n = 208, neoappendicostomy n = 53) with a median follow up time of 2.5 years resulted in 84 patients (appendicostomy n = 60, neoappendicostomy n = 24) experiencing a total of 118 complications requiring surgical or radiologic intervention with a significant difference between the groups (29% vs 45%, RR = 1.79 (95% CI: 1.24-2.60), p < 0.01). Skin level stricture was the most common complication (20% appendicostomies vs 30% neoappendicostomies, p = 0.13). CONCLUSIONS: Appendicostomies and neoappendicostomies can be an effective way to manage fecal incontinence; however, 32% of our patients experienced a complication that required either a surgical or interventional radiology procedure. Patients need to be informed of the possible complications that are associated with appendicostomy and neoappendicostomy construction. TYPE OF STUDY: Single institution retrospective review. LEVEL OF EVIDENCE: IV.

Hemorrhage after on-ECMO repair of CDH is equivalent for muscle flap and prosthetic patch.

BACKGROUND/PURPOSE: Prosthetic patch (patch) and muscle flap (flap) techniques are utilized for severe congenital diaphragmatic hernia (CDH) repair; however, when performed on extracorporeal membrane oxygenation (ECMO), the risk of hemorrhage increases. We sought to compare bleeding complications between repair types. METHODS: We retrospectively reviewed 2010-2016 on-ECMO CDH repairs. RESULTS: Twenty-nine patients met criteria: 13 patch (44.8%) and 16 flap (55.2%). Eight patch (61.5%) and 13 flap (81.2%) patients had left-sided defects (p = 0.223). All defects were Type C or D (Type C: patch 53.8%, flap 56.2%, p = 0.596). There was no difference in gestational age at delivery (patch 37.5 ±â€¯0.9 weeks, flap 37.2 ±â€¯1.3 weeks, p = 0.390) or age at repair (patch 7.46 ±â€¯6.6 days, flap 6.00 ±â€¯4.3 days, p = 0.476). Seven patch (53.8%) and 9 flap (56.2%) patients survived to discharge (p = 0.596). Estimated intraoperative blood loss was equivalent (patch 35.3 ±â€¯53.9 mL, flap 24.2 ±â€¯18.4 mL, p = 0.443). One patch patient (7.6%) and two (12.5%) flap patients required reoperation in the first 48 h for bleeding (p = 0.580). 48-h postoperative transfusions were the same for those that required reoperation (patch 282.0 mL/kg, flap 208.5 ±â€¯21.9 mL/kg, p = 0.054) and those that did not (patch 120.7 ±â€¯111.7 mL/kg, flap 118.4 ±â€¯89.9 mL/kg, p = 0.561). CONCLUSIONS: On-ECMO bleeding complications are equivalent for both flap and patch CDH repair. LEVEL OF EVIDENCE: Type III (retrospective comparative study).

Initial development and validation of a fecal incontinence-specific quality of life measure.

BACKGROUND/PURPOSE: Fecal incontinence is a prevalent pediatric condition with psychosocial impacts on both children and their caregivers. We sought to develop and validate the Cincinnati Fecal Incontinence Scale (CINCY-FIS) as a psychometrically valid measure to assess the quality of life and caregiver impacts of pediatric fecal incontinence. METHODS: Items were generated through review of previous measures, expert consensus, and pilot testing with feedback from 8 families. Initial study measures were completed by 222 caregivers. Following item reduction, 18 items were subjected to confirmatory factor analysis. Convergent and criterion validity were assessed using correlation. Reliability was established using internal consistency statistics and test-retest reliability at baseline and 2-week follow-up. RESULTS: A five factor first-order structure with two higher-order factors demonstrated acceptable fit to the data, was consistent with a priori hypotheses, and was more parsimonious than the alternative model. Convergent validity and criterion-related validity were established for all of the CINCY-FIS scales. Reliability was high and consistent across both measurement occasions. CONCLUSIONS: The CINCY-FIS is a reliable and valid assessment of pediatric fecal incontinence-specific quality of life and parenting stress. The score is highly sensitive to patient changes making it suitable for both clinical and research purposes. TYPE OF STUDY: Prospective observational. LEVEL OF EVIDENCE: Study of Diagnostic Test Level II.

Extracorporeal Membrane Oxygenation in a Patient with Biliary Atresia: Case and Review of Extracorporeal Life Support Organization Data.

Biliary atresia is a newborn cholangiopathy that may lead to portopulmonary hypertension and cirrhosis-induced cardiomyopathy while awaiting liver transplantation. Extracorporeal life support and hepatic toxin filtration are life-saving interventions that provide cardiopulmonary support and hepatic dialysis to allow resolution of a child's illness. We utilized a combination of these extreme measures to bridge an infant with biliary atresia to transplantation. We reviewed cases of extracorporeal life support utilization in transplantation recipients in the Extracorporeal Life Support Organization database and determined that ours was the only use of pretransplant extracorporeal life support in biliary atresia.

Complications in colorectal surgery.

Colorectal pediatric surgery is a diverse field that encompasses many different procedures. The pullthrough for Hirschsprung disease, the posterior sagittal anorectoplasty for anorectal malformations including complex cloaca reconstructions and the ileal pouch anal anastomosis for ulcerative colitis and familial adenomatous polyposis present some of the most technically challenging procedures pediatric surgeons undertake. Many children prevail successfully following these surgical interventions, however, a small number of patients suffer from complications following these procedures. Anticipated postoperative problems are discussed along with medical and surgical strategies for managing these complications.

Extensive heterotopic gastric mucosa of the small intestine: imaging with 99mTc-sodium pertechnetate SPECT/CT enterography.

Extensive heterotopic gastric mucosa of the small intestine is a rare, but potentially life-threatening condition characterized by multifocal or long-segment heterotopic gastric mucosa within the bowel lumen that is often associated with other anomalies including malrotation and annular pancreas. Although the imaging findings are characteristic, this entity may be unrecognized due to its unusual imaging appearance and rarity. CT or MR enterography and 99mTc-sodium pertechnetate scintigraphy can provide complementary information that enables specific diagnosis and accurate assessment of disease extent. We present a case of extensive heterotopic gastric mucosa of the small intestine imaged by simultaneous, combined 99mTc-sodium pertechnetate single photon-emission computed tomography (SPECT)/CT enterography to both familiarize the reader with the condition and describe an imaging strategy that enables specific diagnosis and assists with treatment planning.