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Esophageal Disconnect for Severe GERD in Neurologically-Impaired Children:...
Chapter 1 of 6 · Case-Based Learning
Case 1 presentation
Case 1: 4-year-old with recurrent reflux after fundoplication
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Redo fundoplication with hiatal hernia repair is a 30-minute operation without cutting the stomach or dissociating the esophagus, which have significant morbidity
True hiatal hernias in 6-month-old infants are very unusual and not common
Large hiatal hernia at first operation may complicate esophagogastric disconnect by allowing small bowel to herniate into chest
The Kansas technique for fundoplication, which preserves esophagophrenic ligament, may leave better options for later disconnect compared to wide hiatal dissection
Continuous gastrostomy feeds may eliminate vomiting but patients can still have severe reflux with erosive esophagitis and discomfort
After 2-3 failed fundoplications, disconnect becomes a very attractive third option
Many neurologically impaired children have life expectancy of 20-40 years, and adult surgeons may not be excited to inherit patients who have had 4 redo fundoplications
Primary disconnect patients seem to have a bumpier postoperative course compared to those done after multiple fundoplications, though this is anecdotal without statistical power
Both vagus nerves can be preserved during disconnect and no pyloroplasty is needed, with no gastric emptying problems observed
One patient had bile refluxing into stomach post-operatively, confirmed on contrast study, due to inadequate Roux limb length
Roux limb length should be 25-30 centimeters; shorter limbs risk bile reflux into the stomach
Mesenteric defects, particularly Peterson's defect, must be closed to prevent internal hernias
Prophylactic antibiotics for several days post-operatively are used, treating the case like perforated appendicitis, due to theoretical contamination when crossing the esophagus near liver parenchyma
Contrast study is typically done at 3 days post-operatively, though some de novo patients have been fed without imaging if doing very well
Low threshold for re-imaging or returning to OR if concerned about leak, similar to bariatric surgery practice
Some patients can start feeds immediately post-operatively since feeds go into stomach and downstream, not past the esophagojejunostomy
Operating from patient's right side (as in bariatric surgery) rather than between legs allows better access and avoids lithotomy position
Hand-sewn esophagojejunostomy with interrupted sutures is preferred over stapled anastomosis, especially in smaller children, for security
4-0 Vicryl suture is used for esophagojejunostomy; PDS or silk could also be used
One complication involved colon flipping around Roux limb causing obstruction in a patient with neurologic impairment and global motility problems; resolved by pulling colon back and tacking it to Roux limb
In neurologically normal patients who want to eat by mouth after disconnect, they will be able to eat but probably cannot maintain weight without supplemental nighttime feeds
Patients who have had total gastrectomy with Roux-en-Y reconstruction can still eat by mouth but cannot maintain weight without supplementation
In 24-patient series, average age was 9 years (range 14 months to 17 years), average weight 8-57 kg, average operative time 474 minutes, average length of stay 12 days
Long length of stay (12 days average) is related to complex patient population with respiratory issues, need for pulmonary toilet, and mobilization challenges, not the operation itself
Results show minimal post-operative retching and vomiting in the series
Caregivers report dramatic improvement in quality of life, with some mothers in tears saying 'you've given me my child back'
No patients in the series were readmitted for aspiration-related events or respiratory problems related to reflux
Complications in the series include internal hernias (one requiring bowel resection after delayed presentation), one death from gram-negative sepsis with no leak on contrast study, and several readmissions
Patients can continue to take food by mouth for pleasure after disconnect, including ice cream and supplemental feeds, though they may not maintain weight on oral intake alone
Having a Roux limb is not necessarily benign long-term and carries significant potential complications
Redo Nissen fundoplication in neurologically impaired children is typically a 2-hour operation with discharge in 24-48 hours, significantly less morbidity than disconnect
Patients who fail fundoplication may receive GJ tubes and not return to surgeon, so true long-term fundoplication failure rates may be underestimated
Neurologically impaired children have a higher incidence of reflux disease and higher failure rate when treated with fundoplication
Esophageal disconnect was introduced by Bianchi in a 1997 paper as a rescue operation for children with failed fundoplication
European results from Manchester show disconnect effectively cures reflux and respiratory complications, improves nutrition, and dramatically improves quality of life for patients and caregivers
Retrospective comparison shows dissociation is associated with increased OR time, increased length of stay, and increased time to full feeds, but improved reflux results and lower failure rates compared to fundoplication
Danielson's Rochester series of 27 patients (mix of children and adults) showed disconnect is definitive treatment, but only 3 had prior fundoplication so 24 were de novo procedures