Catastrophic wound dehiscence in early onset scoliosis secondary to hyperinflammatory response: a case report

Area of Science:

• Pediatric Orthopedics
• Immunology
• Genetics

Background:

• Early onset scoliosis (EOS) treatment is challenging, with postoperative wound complications being a significant concern.
• Hyperinflammatory wound complications can arise, particularly in children with specific genetic mutations.
• Non-infectious inflammatory processes can mimic or exacerbate surgical site infections.

Purpose of the Study:

• To present a novel case of hyperinflammatory wound complications in a child with early onset scoliosis and an NFKB1 mutation.
• To demonstrate the successful management of these complications using targeted immunomodulation.
• To highlight the importance of considering genetic predispositions and immune dysregulation in refractory wound healing issues.

Main Methods:

• A 6-year-old boy with EOS developed wound dehiscence and persistent inflammation post-rod placement, unresponsive to conventional treatments.
• Genetic testing revealed a mutation in the NFKB1 gene, associated with hyperinflammatory states.
• Treatment was initiated with Anakinra, an interleukin-1 (IL-1) receptor antagonist.

Main Results:

• Anakinra treatment led to rapid wound healing and normalization of inflammatory markers.
• Subsequent surgical procedures (serial rod expansions) were performed without complications after pretreatment with Anakinra.
• The patient remained stable for 4 years following the immunomodulatory treatment.

Conclusions:

• NFKB1 mutations can predispose patients to severe postoperative hyperinflammatory wound complications in EOS.
• Targeted immunomodulation with Anakinra is effective in managing dysregulated IL-1β activity and promoting wound healing.
• Immunologic evaluation should be considered for patients with refractory wound complications, especially those with disproportionate inflammatory responses.