Rectal Stenosis

Case Summary

Recurrent Stenosis and X-Linked Agamaglobulinemia

• Despite initial dilation attempts and biopsy, stenosis reoccurred, leading to a second dilation and biopsy.
• The cause of the stenosis remained unclear, but the patient was found to have X-linked agamaglobulinemia, a rare immuno-suppressive condition.
• Highlighted were the child's immunosuppressive condition and the need for further investigation into the cause of the stenosis.
• It was proposed that the idea of a distal proctectomy to better understand and remove the problem. Also mentioned was that a sigmoid colostomy as a temporary solution until the underlying issue was resolved.
• The team expressed a need for further discussion and guidance on the best management approach for this complex case. They discussed the surgical intervention carried out on a child with perineal distal stenosis and explained the procedure, which involved a full-thickness dissection and a distal pull-through with no diversion elected.
• He shared the results of the postoperative period, where the child was managed conservatively with clear fluids and presented the pathological findings from the specimen, which included smooth muscle proliferation, architectural distortion of the colonic mucosa, and inflammation.
• These features were consistent with a diagnosis of solidarity syndrome in the appropriate clinical context.
• A lack of evidence for conditions like IBD or Crohn disease, the cause of the stenosis remained unclear. The team shared their experiences and doubts regarding the use of topical mitomycin or steroids for the stenosis. The team also discussed the need for colonoscopy surveillance for the child and the challenges of managing an immunocompromised child with an anastomosis.
• The case discussion ended with no clear resolution on the cause of the stenosis, but stated the need for further follow-up.