Tratamiento quirúrgico conservador en un paciente con invaginación yeyuno-yeyunal secundaria a síndrome de Peutz-Jeghers. Reporte de un caso

Acta pediátrica de México, Jan 2018

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.Keywords : Peutz-Jeghers; gastrontestinal polyps; recurrent intestinal obstructions; acute abdominal pain; jejuno jejunal; adolescents.

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Tratamiento quirúrgico conservador en un paciente con invaginación yeyuno-yeyunal secundaria a síndrome de Peutz-Jeghers. Reporte de un caso

CASO CLÍNICO DE INTERÉS ESPECIAL Acta Pediatr Mex. TH`VQ\UPV" !  Tratamiento quirúrgico conservador en un paciente con invaginación yeyuno-yeyunal secundaria a síndrome de Peutz-Jeghers. Reporte de un caso Conservative surgical treatment in a patient with jejunaljejunal intussusception secondary to Peutz-Jeghers syndrome. Case rport. Rodrigo Ugalde-Resano,1 /ƌŝŶĂ :ĞĂŶĞƩĞ ĞƌĐŚŽůĐͲhƌŝŶŽǁƐŬLJ͕2 Luis Enrique Escalona-Ramírez,1 Juan Miguel ůĂŶĐŽͲdŽƌƌĞƐ͕1DŝŐƵĞůŶŐĞůWĂnjͲDƵŹŽnj͕3 Raúl Escamilla-Roque,4 KĐƚĂǀŝŽĂŝůſŶͲhƌŝĂƌƚĞ4 Resumen INTRODUCCIÓN: LSZxUKYVTLKL7L\[a1LNOLYZLZ\UHLUMLYTLKHKH\[VZ}TPJHKVTP- UHU[LWVJVMYLJ\LU[LJHYHJ[LYPaHKHWVYOPWLYWPNTLU[HJP}UT\JVJ\[mULH`W}SPWVZ NHZ[YVPU[LZ[PUHSLZ"LZ[VZ‚S[PTVZJH\ZHU[LZKLVIZ[Y\JJPVULZPU[LZ[PUHSLZYLJ\YYLU[LZ ZLJ\UKHYPHZHPU]HNPUHJP}U PRESENTACIÓN DEL CASO: HKVSLZJLU[LMLTLUPUHKLH|VZX\LHJ\KP}H\YNLUJPHZ KLIPKVH\UJ\HKYVKLKVSVYHIKVTPUHSPU[LUZVJVU\UJ\HKYVKLVIZ[Y\JJP}UPU[LZ[PUHSZLJ\UKHYPVHPU]HNPUHJP}U`L`\UV-`L`\UHS:PL[LH|VZHU[LZ[\]V\UJ\HKYV JSxUPJVZPTPSHYX\LKLZHWHYLJP}LZWVU[mULHTLU[L(SHL_WSVYHJP}UMxZPJHZLVIZLY]HYVU SLZPVULZOPWLYWPNTLU[HKHZLULSSHIPVPUMLYPVY`HULTPHTPJYVJx[PJHLOPWVJY}TPJHJVU SVZX\LZLPU[LNY}LSKPHNU}Z[PJVKLZxUKYVTLKL7L\[a-1LNOLYZ+LIPKVHX\LLUSH [VTVNYHMxHH_PHSJVTW\[HKHKLHIKVTLUZLL]PKLUJP}PU]HNPUHJP}UPU[LZ[PUHSZLYLHSPa} \UHSHWHYV[VTxHL_WSVYHKVYHJVULSOHSSHaNVKLKVZW}SPWVZOHTHY[VTH[VZVZKL` JTKLKPmTL[YVHJTKLSSPNHTLU[VKL;YLP[a:L[YH[}SHPU]HNPUHJP}UPU[LZ[PUHS `WVZ[LYPVYTLU[LZLLMLJ[\HYVULU[LYV[VTxH`WVSPWLJ[VTxH4LKPHU[LLU[LYVZJVWPH [YHUZVWLYH[VYPHZLKLZJHY[HYVUV[YHZHS[LYHJPVULZ3\LNVKL[YH[HYLSJ\HKYVJSxUPJVSH WHJPLU[LM\LKHKHKLHS[HKLSOVZWP[HSLUI\LUHZJVUKPJPVULZ CONCLUSIÓN: SHPU]HNPUHJP}UPU[LZ[PUHSLUHKVSLZJLU[LZLZ\UHZP[\HJP}UL_JLWJPVUHS X\LYLX\PLYL\UHS[VxUKPJLKLZVZWLJOHWHYHLUMLYTLKHKLZWVSPW}ZPJHZ:LYLWVY[H\U JHZVKLPU]HNPUHJP}UPU[LZ[PUHSX\LZL[YH[}L_PZ[VZHTLU[LJVUJPY\NxHJVUZLY]HKVYH ZPUULJLZPKHKKLYLZLJJPVULZPU[LZ[PUHSLZX\LHSHYNVWSHaVJVUKPJPVUHUZxUKYVTLKL PU[LZ[PUVJVY[V!\UVKLSVZWYPUJPWHSLZWYVISLTHZHSVZX\LZLLUMYLU[HULZ[VZWHJPLU[LZ PALABRAS CLAVE: 7L\[a-1LNOLYZW}SPWVZNHZ[YVPU[LZ[PUHSLZVIZ[Y\JJP}UPU[LZ[PUHSYLJ\YYLU[LKVSVYHIKVTPUHSHN\KV`L`\UV-`L`\UHSHKVSLZJLU[LZ Abstract INTRODUCTION: 7L\[a-1LNOLYZZ`UKYVTLPZHU\UJVTTVUH\[VZVTHSKVTPUHU[KPZLHZL JOHYHJ[LYPaLKI`T\JVJ\[HULV\ZO`WLYWPNTLU[H[PVUHUKNHZ[YVPU[LZ[PUHSWVS`WZ^OPJO HYL[OLJH\ZLVMYLJ\YYLU[PU[LZ[PUHSVIZ[Y\J[PVUZZLJVUKHY`[VPU]HNPUH[PVU;OPZJHZL WYV]PKLZL]PKLUJL[VJVUZPKLYTVYLJVUZLY]H[P]LZ\YNPJHS[YLH[TLU[ZHUKWYL]LU[[OLZL WH[PLU[ZMYVTILPUNJVUZ[HU[S`Z\IQLJ[LK[VL_[LUZP]LIV^LSYLZLJ[PVUZ^OPJOPUTHU` JHZLZJHUJVUKP[PVUZOVY[IV^LSKPZLHZL CASE PRESENTATION: (-`LHY-VSKMLTHSLWH[PLU[^OVJHTL[V[OLLTLYNLUJ`YVVT ^P[OHJ\[LHIKVTPUHSWHPUK\L[VPU[LZ[PUHSVIZ[Y\J[PVUZLJVUKHY`[VQLQ\UV-QLQ\UHS PU[\ZZ\ZJLW[PVU`LHYZHNV[OLWH[PLU[YLWVY[LKZPTPSHYJSPUPJHSTHUPMLZ[H[PVUZ[OH[ ZVS]LKZWVU[HULV\ZS`7O`ZPJHSL_HTPUH[PVUZOV^LKO`WLYWPNTLU[LKSLZPVUZVU[OL SV^LYSPWHUKTPJYVJ`[PJO`WVJOYVTPJHULTPH"7L\[a-1LNOLYZZ`UKYVTL^HZLZ[HISPZOLK 0U[LZ[PUHSPU[\ZZ\ZJLW[PVU^HZL]PKLUJLKI`JVTW\[LKH_PHS[VTVNYHWO`VM[OLHIKVTLUZVL_WSVYH[VY`SHWHYV[VT`^HZWLYMVYTLK^P[O[OLÄUKPUNVM[^VOHTHY[VTH[V\Z 244 1 Departamento de Cirugía general, Hospital Central Norte PEMEX. 2 Departamento de Pediatría, Hospital Central Sur de Alta Especialidad PEMEX. 3 Departamento de Imagenología, Hospital Central Norte PEMEX. 4 Departamento de Cirugía general, ,ŽƐƉŝƚĂůZĞŐŝŽŶĂůĚĞDŝŶĂƟƚůĄŶWDy͘ Recibido: 30 de mayo 2017 Aceptado: 14 de julio 2017 Correspondencia KĐƚĂǀŝŽĂŝůſŶhƌŝĂƌƚĞ ĚƌͺďĂŝůŽŶƵΛůŝǀĞ͘ĐŽŵ ƐƚĞĂƌơĐƵůŽĚĞďĞĐŝƚĂƌƐĞĐŽŵŽ hŐĂůĚĞͲZĞƐĂŶŽ Z͕ ĞƌĐŚŽůĐͲhƌŝŶŽǁƐŬLJ /:͕ ƐĐĂůŽŶĂͲZĂŵşƌĞnj >͕ ůĂŶĐŽͲdŽƌƌĞƐ :D͕ WĂnjͲDƵŹŽnj D͕ ƐĐĂŵŝůůĂͲZŽƋƵĞ Z͕ ĂŝůſŶͲ Uriarte O. Tratamiento quirúrgico conservador en un paciente con invaginación yeyuno-yeyunal secundaria a síndrome de PeutzJeghers. Reporte de un caso. Acta WĞĚŝĂƚƌDĞdž͘ϮϬϭϴ͖ϯϵ;ϯͿ͗ϮϰϰͲϮϰϵ͘ www.actapediatrica.org.mx Ugalde-Resano R et al. 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Rodrigo Ugalde-Resano, lrina Jeanette Bercholc-Urinowsky, Luis Enrique Escalona-Ramírez, Juan Miguel Blanco-Torres, Miguel Ángel Paz-Muñoz, Raúl Escamilla-Roque, Octavio Bailón-Uriarte. Tratamiento quirúrgico conservador en un paciente con invaginación yeyuno-yeyunal secundaria a síndrome de Peutz-Jeghers. Reporte de un caso, Acta pediátrica de México, 2018, pp. 244-249, Volume 39, Issue 3, DOI: 10.18233/apm39no3pp244-2491615