2018, Number 3
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Acta Pediatr Mex 2018; 39 (3)
Conservative surgical treatment in a patient with jejunaljejunal intussusception secondary to Peutz-Jeghers syndrome. Case rport
Ugalde RR, Bercholc UIJ, Escalona RLE, Blanco TJM, Paz MMÁ, Escamilla RR, Bailón UO
Language: Spanish
References: 15
Page: 244-249
PDF size: 322.38 Kb.
ABSTRACT
Introduction: Peutz-Jeghers syndrome is an uncommon autosomal dominant disease, characterized by mucocutaneous hyperpigmentation and gastrointestinal polyps, which are the cause of recurrent intestinal obstructions, secondary to invagination. This case provides evidence to consider more conservative surgical treatments and prevent these patients from being constantly subjected to extensive bowel resections, which in many cases, can condition short bowel disease.
Case presentation: A 13-year-old female patient who came to the emergency room with acute abdominal pain due to intestinal obstruction secondary to jejuno-jejunal intussusception. 7 years ago, the patient reported similar clinical manifestations that solved spontaneously. Physical examination showed hyperpigmented lesions on the lower lip, and microcytic hypochromic anemia; Peutz-Jeghers syndrome was established. Intestinal intussusception was evidenced by computed axial tomography of the abdomen, so exploratory laparotomy was performed, with the finding of two hamartomatous polyps 2 and 3 cm in diameter at 15 cm of the Treitz ligament. Intestinal intussusception was resolved with subsequently, enterotomy and polypectomy. Other alterations were ruled out by trans-operative enteroscopy. At the resolution of clinical manifestations, the patient was discharged from the hospital in good clinical conditions.
Conclusion: Intestinal intussusception in adolescents is an exceptional situation, which requires a high index of suspicion for polysposis diseases. We present a case of intestinal intussusception, successfully resolved with conservative surgical treatment, without requiring intestinal resections, which can condition short bowel syndrome in the long-term, one of the major problems these patients face.
REFERENCES
Constantinescu AR, Bitzan M, Weiss LS, et al. Non-enteropathic hemolytic uremic syndrome: causes and short-term course. Am J Kidney Dis. 2004; 43(6): 976-82.
Laurence J. Atypical hemolytic uremic syndrome (aHUS): making the diagnosis. Clin Adv Hematol Oncol. 2012; 10(10 Suppl 17):1-12.
George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(7):654-66.
Noris M, Caprioli J, Bresin E, et al. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol. 2010;5(10):1844-1859.
Fremeaux-Bacchi V, Fakhouri F, Garnier A, et al. Genetics and outcome of atypical hemolytic uremic syndrome: a nation wide French series comparing children and adults. Clin J Am Soc Nephrol. 2013;8(4):554-62.
Ohanian M, Cable C, Halka K. Eculizumab safely reverses neurologic impairment and eliminates need for dialysis in severe atypical hemolytic uremic syndrome. Clin Pharmacol Adv Applications. 2011;3:5-12.
Mannucci PM, Cugno M. The complex differential diagnosis between thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome: Laboratory weapons and their impact on treatment choice and monitoring. Thromb Res. 2015;136(5):851-4.
Sperati CJ, Moliterno AR. Thrombotic microangiopathy: focus on atypical hemolytic uremic syndrome. Hematol Oncol Clin North Am. 2015;29(3):541-59.
Nester CM, Thomas CP. Atypical hemolytic uremic syndrome: what is it, how is it diagnosed, and how is it treated? Hematology Am Soc Hematol Educ Program. 2012; 2012:617-25.
Sullivan M, Erlic Z, Hoffmann MM, et al. Epidemiological approach to identifying genetic predispositions for atypical hemolytic uremic syndrome. Ann Hum Genet. 2010;74(1):17-26.
Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med. 2009:361:1676-87.
Caprioli J, Noris M, Brioschi S, et al. Genetics of HUS: the impact of MCP, CF, and IF mutations on clinical presentation, respond to treatment, and outcome. Blood. 2006; 108:1267-1279.
Legendre CM, Licht C, Muus P, et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013 Jun 6;368(23):2169-81.
Loirat CH, Kakhouri F, Ariceta G, et al. An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2016;31(1):15-39.
Sellier-Lerclerc Al, Fremeaux- Bacchi V, Dragon-Durey MA, et al; French Society of Pediatric Nephrology. Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome. J Am Soc Nephrol.2007; 18(8): 2392-400.