Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2020:66:381-384.
doi: 10.1016/j.ijscr.2019.12.037. Epub 2020 Jan 9.

Pediatric Cullen gangrene: Case report of postoperative pyoderma gangrenosum in a preterm infant with a complex gastrointestinal malformation

Sonja Diez  1 , Julia Syed  2 , Hanna Müller  3 , Manuel Besendoerfer  2 , Vera Schellerer  2
Affiliations
Case Reports

Pediatric Cullen gangrene: Case report of postoperative pyoderma gangrenosum in a preterm infant with a complex gastrointestinal malformation

Sonja Diez et al. Int J Surg Case Rep. 2020.

Abstract

Introduction: Pyoderma gangrenosum (PG) is a rare condition in infants, children and adolescents. The postoperative variant of this rapidly progressive ulceration is known as Cullen gangrene. Only a few case reports of pediatric Cullen gangrene have been published.

Presentation of case: We present a male infant (gestational age 31 + 6 weeks) with gastroschisis and intrauterine volvulus. After multiple operations due to his complex gastrointestinal malformation, the patient showed classical symptoms of postoperative PG (PPG) 4 weeks after birth. Diagnosis could be made early in an interdisciplinary approach, differentiating intraabdominal infection (vancomycin resistant enterococcus (VRE), candida albicans) from epifascial occurrence. Treatment with high-dose steroids and immunoglobulins was initiated immediately and led to rapid recovery.

Discussion: The unusual combination of a complex gastrointestinal malformation and an intraabdominal infection with VRE and candida albicans made the diagnosis difficult. However, early diagnosis is crucial in the treatment of Cullen gangrene.

Conclusion: This is the first case report of Cullen gangrene presenting in an infant (at 4 weeks of age).

Keywords: Cullen gangrene; Gastroschisis; Pediatric surgery; Pyoderma gangrenosum.

PubMed Disclaimer

Conflict of interest statement

Declaration of Competing Interest Non to declare.

Figures

Fig. 1
Fig. 1
Intraoperative findings of the second surgery, presenting a complex gastrointestinal malformation, including an intrauterine volvulus and accordingly twisted mesenteric vessels in combination with a duodenal atresia and atresia of the ascending colon. Ischemic discoloration was already notable (surgery at an absolute age of 5 days).
Fig. 2
Fig. 2
Clinical presentation of the ulcerous development of the Cullen gangrene in the presented infant. First picture was taken after one day of abdominal erythema. Every further picture shows development after 12 hours, indicating enlargement of the lesion to a size measuring 5 × 3 cm of maximal diameters.
Fig. 3
Fig. 3
After surgery for biopsies and careful exploration of wound defect, secondary closure via biological patch was performed. The cutaneous edges were not excised, but tension-free attached to the patch.
Fig. 4
Fig. 4
The infant’s abdominal wall at the age of 7 months after secondary closure of abdominal wall. Therapy with low-dose prednisolone was continued to this time.
See this image and copyright information in PMC

References

    1. Bennett M.L., Jackson J.M., Jorizzo J.L., Fleischer A.B., Jr., White W.L., Callen J.P. Pyoderma gangrenosum. A comparison of typical and atypical forms with an emphasis on time to remission. Case review of 86 patients from 2 institutions. Medicine. 2000;79(1):37–46. - PubMed
    1. Graham J.A., Hansen K.K., Rabinowitz L.G., Esterly N.B. Pyoderma gangrenosum in infants and children. Pediatr. Dermatol. 1994;11(1):10–17. - PubMed
    1. Binus A.M., Qureshi A.A., Li V.W., Winterfield L.S. Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients. Br. J. Dermatol. 2011;165(6):1244–1250. - PubMed
    1. Shahi V., Wetter D.A. Pyoderma gangrenosum associated with solid organ malignancies. Int. J. Dermatol. 2015;54(9):e351–e357. - PubMed
    1. Cullen T.S. A progressively enlarging ulcer of the abdominal wall involving the skin and fat, following drainage of an abdominal abscess apparently of appendiceal origin. Surg. Gynecol. Obstet. 1924;38:579–582.

Publication types