Advertisement

Pediatric Radiology

, 39:1095 | Cite as

Fulminant limb and retroperitoneal necrotizing fasciitis in a 15-year-old girl with Fanconi anaemia

  • Kevin O’Regan
  • Edward O’Mahony
  • Peter MacEneaney
  • Edward Fitzgerald
  • Michael M. Maher
Case Report

Abstract

Necrotizing fasciitis (NF) is an uncommon soft-tissue infection in children that carries a high mortality rate. We present a 15-year-old girl with chronic pancytopenia secondary to Fanconi anaemia who developed extensive NF of the lower limb, which unfortunately resulted in a fatal outcome. Immunodeficiency is a known risk factor for the development of this condition. The findings in this case demonstrate that patients with Fanconi anaemia may be susceptible to NF and that the clinical course may be more aggressive due to underlying immunosuppression. Prompt diagnosis of NF is vital in order to initiate appropriate treatment and to optimize patient outcome. Radiological investigation demonstrated extensive soft-tissue gas and destruction affecting the entire lower limb, abdominal wall and retroperitoneum, which led to timely definitive diagnosis and management.

Keywords

Necrotising fasciitis Fanconi anaemia CT Child 

References

  1. 1.
    Fustes-Morales A, Gutierrez-Castrellon P, Duran-Mckinster C et al (2002) Necrotizing fasciitis: report of 39 pediatric cases. Arch Dermatol 138:893–899PubMedCrossRefGoogle Scholar
  2. 2.
    Duncan BW, Adzick S, deLorimier AA et al (1992) Necrotizing fasciitis in two children with acute lymphoblastic leukemia. J Pediatr Surg 27:668–671PubMedCrossRefGoogle Scholar
  3. 3.
    Moss RL, Musemeche CA, Kosloske AM (1996) Necrotizing fasciitis in children: prompt recognition and aggressive therapy improve survival. J Pediatr Surg 31:1142–1146PubMedCrossRefGoogle Scholar
  4. 4.
    Edlich RF, Cross CL, Dahlstrom JJ et al (2008) Modern concepts of the diagnosis and treatment of necrotizing fasciitis. J Emerg Med. doi: 10.1016/j.jemermed.2008.06.024
  5. 5.
    Yen ZS, Wang HP, Ma HM et al (2002) Ultrasonographic screening of clinically-suspected necrotizing fasciitis. Acad Emerg Med 9:1448–1451PubMedCrossRefGoogle Scholar
  6. 6.
    Wysoki MG, Santora TA, Shah RM et al (1997) Necrotizing fasciitis: CT characteristics. Radiology 203:859–863PubMedGoogle Scholar
  7. 7.
    Meetei AR, Levitus M, Xue Y et al (2004) X-linked inheritance of Fanconi anemia complementation group B. Nat Genet 36:1219–1224PubMedCrossRefGoogle Scholar
  8. 8.
    Woodburn KR, Ramsay G, Gillespie G et al (1992) Retroperitoneal necrotizing fasciitis. Br J Surg 79:342–344PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Kevin O’Regan
    • 1
    • 2
  • Edward O’Mahony
    • 1
  • Peter MacEneaney
    • 1
  • Edward Fitzgerald
    • 1
  • Michael M. Maher
    • 1
    • 2
  1. 1.Department of RadiologyMercy University HospitalCorkRepublic of Ireland
  2. 2.Department of RadiologyCork University HospitalCorkRepublic of Ireland

Personalised recommendations