PICTURES IN DIGESTIVE PATHOLOGY

 

Coloduodenal and enterocutaneous fistula in twins with Crohn's disease

 

 

María Pilar Ballester¹, David Martí¹, Marta Maia Bosca-Watts¹, José Martín-Arévalo² and Miguel Mínguez¹

Departments of ¹Digestive Medicine and ²General Surgery. Hospital Clínic de Valencia. Universitat de Valencia. Valencia, Spain

 

 

Case report

We present the case of a pair of 45-year-old monozygotic twins (A and B) diagnosed with Crohn's disease (CD) at age 20 (A) and 22 (B) with similar presenting symptoms: diarrhea, fever and weight loss. Both of them had duodenal and ileocolonic disease (A2, L3 + L4 according to Montreal classification); twin B also presented jejunal involvement and perianal disease (B1p). They received treatment with antibiotics, corticosteroids, 5-ASA, azathioprine and anti-TNF with a poor control of activity. They both developed a coloduodenal fistula (Fig. 1) that required surgery. Twin A developed the fistula 12 years after the first presentation; fistula closure with duodenorraphy and ileocolonic resection with gastrojejunostomy was performed. Twin B developed the fistula 22 years after the first presentation, and right colectomy, partial duodenectomy and duodenorraphy was carried out. Both developed an enterocutaneous fistula (Fig. 2) during the postoperative period. With intensive medical treatment, both twins remain asymptomatic.

 

 

 

Discussion

Family history of CD is considered as a risk factor for developing the disease with a greater clinical concordance between monozygotic twins (20-50%). However, the pathogenesis is complex with the influence of environmental and genetic factors. The transmural nature of the disease can lead to fistula-formation. The most common enteroenteric fistulas are ileoileal, ileocecal or ileosigmoid; while cologastric or coloduodenal fistulas, such as those in our patients, rarely appear (< 1%). They cause a malabsorption syndrome due to the exclusion of large sections of the intestine. Imaging with oral contrast (barium transit, magnetic resonance [MR] or computed tomography [CT]) is used for diagnosis.

This is a case report in a pair of monozygotic twins who developed the same disease with a similar phenotype, evolution and severity, demonstrating the fact that family history plays an important role in the development of CD.

 

References

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