Meu SciELO
Serviços Personalizados
Journal
Artigo
Indicadores
- Citado por SciELO
- Acessos
Links relacionados
- Citado por Google
- Similares em SciELO
- Similares em Google
Compartilhar
Revista Española de Enfermedades Digestivas
versão impressa ISSN 1130-0108
Rev. esp. enferm. dig. vol.109 no.1 Madrid Jan. 2017
https://dx.doi.org/10.17235/reed.2016.4452/2016
LETTERS TO THE EDITOR
Multiple perforation of small-intestine diverticula in a patient with Ehlers-Danlos syndrome
Perforación múltiple de divertículos de intestino delgado en paciente con síndrome de Ehlers-Danlos
Key words: Ehlers-Danlos. Diverticulum. Duodenum.
Palabras clave: Ehlers-Danlos. Divertículo. Duodeno.
Dear Editor,
Ehlers-Danlos syndrome represents a group of hereditary connective tissue disorders characterized by ligamentous hyperlaxity, fragile skin and joint hypermobility. Gastrointestinal complications in this syndrome are less well known (1).
Case report
We report the case of a 42-year-old male with this condition who presented with acute abdominal over a 12 hour period after taking anti-inflammatory drugs. A CT scan revealed a right-sided retro-hydro-pneumoperitoneum secondary to duodenal diverticular perforation extending to the falciform ligament. An exploratory laparotomy was performed, and biliary peritonitis from two perforated duodenal diverticula were found: one with three perforations, the other with two. Pathological analysis of the specimen found that it contained duodenal segments with no muscularis propria and parietal necrosis, which confirmed the diagnosis (Fig. 1). The patient had a favorable outcome following the procedure, and was discharged within a few days.
Discussion
Most common gastrointestinal manifestations in patients with Ehlers-Danlos syndrome include abdominal pain, nausea and vomiting (2), and gastrointestinal bleeding and intestinal perforation may be seen in severe cases (3). A higher incidence of colonic diverticulosis has also been described for this condition, a fact thought to result from a lack of distension at the colonic wall as a consequence of collagen impairment, but the specific mechanism still remains unknown (2,4). In this patient, however, diverticula were predominantly located in high numbers within the small bowel. Perforations may be spontaneous or brought about by some external factor (anti-inflammatory drugs, endoscopic instrumentation), and their multiplicity even in one diverticulum or apparently healthy tissue must be highlighted (5).
Therefore, excluding a diagnosis of intestinal diverticulosis and its related complications is crucial for patients diagnosed with Ehlers-Danlos syndrome presenting with abdominal pain.
Raúl Honrubia-López1, Aurora Burgos-García1 and Elena Palacios-Lázaro2
1Departments of Digestive Diseases, and 2Pathology.
Hospital Universitario La Paz. Madrid, Spain
References
1. Yen JL, Lin SP, Chen MR, et al. Clinical features of Ehlers-Danlos syndrome. J Formos Med Assoc 2006;105:475-80. DOI: 10.1016/S0929-6646(09)60187-X. [ Links ]
2. Nelson AD, Mouchli MA, Valentin N, et al. Ehlers Danlos syndrome and gastrointestinal manifestations: A 20-year experience at Mayo Clinic. Neurogastroenterol Motil 2015;27(11)1657-66. DOI: 10.1111/nmo.12665. [ Links ]
3. Beighton PH, Murdoch JL, Votteler T. Gastrointestinal complications of the Ehlers-Danlos syndrome. Gut 1969;10(12):1004-8. DOI: 10.1136/gut.10.12.1004. [ Links ]
4. Gómez Sugrañes JR, Luengo Rodríguez de Ledesma L, Castellote Caixal M, et al. Perforation of small intestine diverticulum in Ehlers-Danlos syndrome. Rev Esp Enferm Dig 1995;87(1):53-5. [ Links ]
5. Solomon JA, Abrams L, Lichtenstein GR. GI Manifestations of Ehlers-Danlos syndrome. Am J Gastroenterol 1996;91(11):2282-8. [ Links ]