Brief report Findings in spontneous intrmurl intestinl hemtom imging F. Mirs Azcón, A. Mrtínez Mrtínez, A. Sntigo Chinchill*, A. Medin Benítez, E. Ochndo Pulido nd M.A. Pérez Rosillo Imging deprtment, Virgen de ls Nieves University Hospitl, Grnd, Spin. Astrct Spontneous intrmurl hemtoms re very rre cuse of cute dominl pin, ut likely dignosis in ptients undergoing nticogultion therpy or with underlying hemtologicl disese. Although verge onset fter eginning nticogultion therpy is 24.4 months, they my develop s erly s 10 dys fter strt of tretment. Clinicl symptoms re very rod (e.g. dominl pin nd nuse) nd typiclly similr to those of n intestinl ostruction, ut this is not lwys the cse, nd dignostic errors my occur. Tretment of spontneous intrmurl hemtom is conservtive in most cses, which is why incorrect dignosis my often led to unnecessry surgery. Ultrsound imging, nd in prticulr CT scns, ply key role in erly nd correct dignosis of this condition. Imging ws used to descrie finding in 5 ptients with cute clinicl dominl pin nd spontneous intrmurl hemtom dignosis, review of the literture ws lso conducted. Min rdiologicl signs included: thickening of the intestinl wll, intrmurl hyperdensity on CT without IV contrst, nd presence of hemoperitoneum. Keywords: Hemtom; Spontneous; Computed tomogrphy scn (CT scn); Ultrsound. 2015 Argentine Assocition of Rdiology. Pulished y Elsevier Spin, S.L.U. This is n Open Access rticle under license CC BY-NC-ND (http://cretivecommons.org/licenses/y-nc-nd/4.0/). Introduction Adominl trum is the min cuse of spontneous intrmurl hemtom nd is responsile for up to 90% of cses 1. Spontneous etiology is rre, lthough the condition cn result from excess nticogultion tretment or e secondry to hemtologicl disese. The condition is more frequent in men thn in women, nd men ge of clinicl presenttion is 58 yers 2. Spontneous intrmurl intestinl hemtom presents in 1 of every 2500 ptients undergoing tretment with Wrfrin. Incidence in reltion to hemtologicl disese is unknown, lthough hemophili,leukemi, lymphom,myelomnd idiopthic immune thromocytopeni re the min relted conditions 3, 4. Other risk fctors include vsculitis, chemotherpy nd pncretitis 5.6. Men time to clinicl onset fter eginning nticogultion therpy is 24.4 months. 2 Neverhteless, it cn develop s erly s 10 dys fter eginning tretment. Spontneous cses re most commonly locted in the jejunum (71.6%), followed y the duodenum (29.8%); however in post trumtic cses, the duodenum is the most common site. 5 Hemtoms locted in the colon re rre 6. Cliniclly, spontneous intrmurl intestinl hemtom cn present wide rry of non-specific signs nd symptoms; this is why heightened dignostic suspicion is importnt. In 90.7% of cses dominl pin is present, followed y nuse in 50% nd symptoms of intestinl ostruction in 45% of cses 5. Although there is no stndrdized protocol for treting this condition, consensus exists over n initil, conservtive pproch: symptoms usully resolve within 4 to 6 dys, nd dispper entirely fter two months. 1.7 In cses cused y excess nticogultion, tretment includes: discontinuing mediction, dministering vitmin K nd plsm nd ultimtely, lood trnsfusions. Surgery is reserved for ptients with owel ischemi, perfortion, ctive intrluminl leeding, filure of conservtive mngement, dignostic douts or lte-onset complictions, which re infrequent, s re recurrences 5,6,8,9. If lesions persist for more thn 2 months, other underlying cuses must e investigted. Plin dominl X-ry nd rium swllow lck specificity nd only show evidence of intestinl ostruction when present 5,10. Up to 92.5% of intrmurl intestinl hemtoms my 39
Findings in spontneous intrmurl intestinl hemtom imging show norml findings using these methods, without necessrily estlishing dignosis 11,12. Ultrsound imging is frequently the first tool used to study dominl pin. Ptients with intrmurl hemtom will present hyperechogenic sumucosl thickening in segment of the gut, with reduced peristlsis. However, this finding is not specific nd cn occur in other conditions, such s inflmmtory owel disese nd infectious or ischemic colitis 1,3,6,8. In ny cse, use of ultrsound s first pproch for evlution of dominl pin of unknown origin is still very vlule, nd under dequte clinicl conditions, should serve s incentive for further testing to confirm its presence. Adominl CT scns re the test of choice to dignose this condition. Symmetricl, hyperdense circumferentil thickening of segment of the gut is the min finding, ssocited on occsion to intestinl stricture or ostruction 1,3. Intrluminl, peritonel or mesenteric leeding of the ffected owel loop my lso e present. No cler consensus exists over use of intrvenous iodinesed contrst, nd some uthors clim it cn ctully msk murl hyperdensity. 2 Use of orl contrst should e voided therefore, since it my cuse intrluminl leeding to e overlooked (if contrst is to e dministered, wter is preferle to rdiopque sustnces) 5. Degree of intrmurl hyperdensity will vry with time, nd e most intense during the first ten dys. It is importnt to note tht CT scn findings should e interpreted with cution, since they cn lso e present in other conditions such s inflmmtory owel disese, infections or neoplsi. Nevertheless, if the involved segment mesures less thn 23 cm nd hs no ssocited extrmurl mss, hemtom is the most likely dignosis 6. When no histologicl confirmtion is possile, clinicl follow up nd imging should suffice to dignose the condition 5. This pper ims to nlyze spontneous intrmurl intestinl hemtom cses dignosed t our center etween Jnury 2014 nd the present, nd descrie imging findings, s well s their correltion to clinicl nd epidemiologicl oservtions. Findings Between Jnury 2014 nd the present, we dignosed eight cses of intrmurl hemtom t our center using imging. Two were secondry to trum, one to injection of drenline to tret upper GI leeding nd the remining five were of spontneous origin. The ltter were confirmed during follow up, fter clinicl nd imging findings resolved. Ultrsound nd CT dt were nlyzed nd found to e key for dignosis of this condition in its spontneous form. Becuse this ws retrospective nlysis, informed consent ws not requested; however, dt collection nd processing ws conducted under specific protocol designed y our Center, following recommendtions mde y the Clinicl Reserch Ethics Committee, who uthorized the study. Men ge of ptients presenting spontneous intrmurl hemtom ws 76.8 yers. From n etiologicl stndpoint, four of the five cses were secondry to nticogultion complictions; only one ws cused y n underlying hemtologicl condition, specificlly diffuse lrge B-cell lymphom. Men time to presenttion ws 23.67 months in ptients with nticogultion prolems, the erliest of which presented fter 4.8 months. In four cses (80%), spontneous hemtom ws locted in the jejunum, the fifth ws identified in the ileum (diffuse lrge B-cell lymphom ptient). From clinicl stndpoint, ll ptients (100%) presented dominl pin: of five, three suffered nuse (60%) nd two vomiting (40%) (tle 1). Two of the five ptients (40%) were referred for imging for suspected intestinl ostruction. In the cse of the lymphom ptient, pin ws focused in the right upper qudrnt, nd nemi nd thromocytopeni were detected on complete lood count (CBC). Tle 1: CT findings in spontneous intrmurl intestinl hemtom ptients. Ptient Intrmurl Intrmurl Intestinl Hemoperi- Intrluminl Bowel Loction Length Nro. hyperdensity Hounsfield mesenteric ft toneum leeding distention ffected units hyperdensity (cm) 1 Yes 44 Yes Yes No Yes Jejunum 10.3 2 Yes 46 Yes Yes No Yes Jejunum 11.5 3 Yes 56 Yes Yes Yes Yes Ileum 6.8 4 Yes 56 No No No No Jejunum 17.8 5 Yes 40 Yes Yes No No Jejunum 7.3 Men/percentge 100% 48.4 80% 80% 20% 60% Jejunum 80% 10.74 presenttion Ileum 20% 40
F. Mirs Azcón et l. Tle 2: Spontneous intrmurl intestinl hemtom: ptient epidemiology. Ptient Nro. Age (yers) Adominl pin Nuse Vomiting Clinicl signs Etiology of ostruction 1 82 Yes Yes Yes Yes Anticogultion 2 85 Yes Yes Yes Yes Anticogultion 3 69 Yes, focused in the No No No Lymphom right upper qudrnt 4 75 Yes No No No Anticogultion 5 73 Yes Yes No No Anticogultion Men /percentge 76.8 100% 60% 40% 40% Anticogultion 80% presenttion Hemtologicl condition 20% Fig 1. Adominl ultrsound in spontneous intrmurl hemtom ptient. () Hyperechogenic thickening of the intestinl wll (etween crosses) () Free floting fluid (sterisk) djcent to owel loop, with hyperechogenic thickening of the intestinl wll (etween crosses). Fig 2. Fig 2. ( nd ) Axil CT imges of two different ptients with spontneous intrmurl hemtom. Symmetricl, uniform hyperdense circumferentil thickening of owel wll segment (rrows), comptile with intrmurl hemtom in oth cses. 41
Findings in spontneous intrmurl intestinl hemtom imging Fig 3. Mle ptient, ge 82 yers, with spontneous intrmurl intestinl hemtom secondry to nticogultion tretment. () Hyperdense free pelvic fluid (rrow), suggesting hemoperitoneum. () Symmetricl, hyperdense circumferentil thickening of owel wll segment (rrow) with intrmurl hemtom nd hyperdense reticulr mesenteric edem (rrowhed) consistent with intestinl leeding. All five ptients were sumitted to CT scns without IV or orl contrst; three hd een previously exmined with ultrsound, two of which presented distended owel loops nd free dominl fluid with suspended echoic mteril. This oservtion ws considered non specific for dignosis, nd therefore CT ws performed. The remining cse presented echogenic thickening of the intestinl wll with free dominl fluid, (fig. 1) which lso led to complementry CT scn. All CT scns (100%) reveled symmetricl, hyperdense circumferentil thickening of segment of intestinl wll (fig. 2, tle 2). Men density ws 48.4 Hounsfield Units (HU) nd verge length of intestine ffected 10.74 cm. Additionlly, four of the five cses (80%) exhiited hyperdense mesenteric ft nd free floting fluid in reltion to the hemoperitoneum (fig. 3), wheres only one cse presented miniml intrluminl leeding (20%). In three of five ptients (60%) CT scn identified smll owel loop distension. All ptients were mnged in conservtive mnner, nd none required surgicl intervention. Resolution ws confirmed cliniclly nd in follow-up imging. No cse presented recurrence or complictions. Discussion Spontneous intrmurl hemtom is rre cuse of cute dominl pin; no lrge series of pulished cses exists given its low, ut growing incidence. Compred to pulished literture men ge of presenttion ws considerly higher in our study thn in Sorello et l.2 (76.8 vs. 58 yers). However, men time to presenttion from eginning of nticogultion tretment ws similr, s ws hemtom loction in the 5ptient s descried nd in studies where the jejunum ws the segment most often ffected 5. In line with pulished literture, dominl pin ws the min deut symptom, followed y nuse nd signs of ostruction 2. Also in line with pulished literture the most chrcteristic ultrsound finding ws echogenic thickening of the intestinl wll, nd in CT the most common finding ws symmetricl, hyperdense circumferentil thickening of segment of owel wll 1,3,5,13. In this study, this CT finding ws the most chrcteristic sign of intrmurl intestinl hemtom, present in 100% of cses. In ddition to these oservtions, presence of hyperdense mesenteric ft ccompnied y leeding (present in 80% of cses) nd hemoperitoneum (80%) were complementry signs tht significntly incresed dignostic certinty. Use of intrvenous contrst for studying this pthology remins controversil 1.2. In our experience, imging without contrst is sufficient for correct dignosis, voiding unnecessry risks linked to contrst use s well incresed rdition exposure. More importntly, injection of contrst nd its sorption y owel surrounding the hemtom cn ctully hinder detection of hyperdense wll thickening, which hs lredy een descried s key for spontneous hemtom dignosis. Our current policy is to only consider its use for cses presenting significnt dignostic difficulty. Among the spontneous origin cses, the ptient with diffuse lrge B-cell lymphom presented certin specific findings in reltion to lesion site nd symptoms. The hemtom ws found in the ileum, with pin in the right upper qud- 42
F. Mirs Azcón et l. rnt. Also, CBC reveled nemi nd thromocytopeni. This ptient ws the only cse presenting intrluminl leeding. Although these oservtions were seen in one ptient only, they my suggest prticulr clinicl nd imging findings tht would ssist in the differentil dignosis of other cuses of intrmurl intestinl hemtom. This group of ptients should e suject to further investigtions. Conclusion Spontneous intrmurl intestinl hemtoms re very rre ut incresing cuse of dominl pin. Although the condition does not present with specific set of symptoms, its presence should e suspected in the right clinicl context, specificlly in ptients receiving nticogultion therpy or suffering from hemtologicl conditions who present cute clinicl dominl symptoms simulting n intestinl ostruction. A CT scn without contrst is the dignostic method of choice for this condition, in which the min finding is symmetricl hyperdense circumferentil thickening of the intestinl wll. Additionlly, hyperdense mesentery or presence of hemoperitoneum re dditionl findings present in significnt numer of cses nd of gret dignostic vlue. Ethicl responsiilities Protection of nimls nd individuls. Authors declre no experiments were conducted in humns or nimls during this study. Dt confidentility. Authors declre tht institutionl study protocols relting to ptient dt puliction were followed. Right to privcy nd informed consent. Authors otined informed consent from ptients/individuls referred to in this rticle. Author for correspondence is in possession of rights to this rticle. Conflict of interest Authors hve no conflicts of interest to declre. References 1. Adel Smie A, Theilmnn L. Detection nd mngement of spontneous intrmurl smll owel hemtom secondry to nticogulnt therpy. Expert Rev Gstroenterol Heptol. 2012;6:553---8. 2. Sorello MP, Utiym EM, Prreir JG, Birolini D, Rssln S. Spontneous intrmurl smll owel hemtom induced y nticogulnt therpy: review nd cse report. Clinics (So Pulo). 2007;62:785---90. 3. Altiky N, Prlkgumus A, Demir S, Alkn O, Yildirim T. Smll owel ostruction cused y intrmurl hemtom secondry to wrfrin therpy: report of two cses. Turk J Gstroenterol. 2011;22:199---202. 4. Jimenez J. Adominl pin in ptient using wrfrin. Postgrd Med J. 1999;75:747---8. 5. As MA, Collins JM, Olden KW. Spontneous intrmurl smllowel hemtom: imging findings nd outcome. AJR Am J Roentgenol. 2002;179:1389---94. 6. Ktsumi A, Mtsushit T, Hirshim K, Iwski T, Adchi T, Ymmoto K, et l. Recurrent intrmurl hemtom of the smll intestine in severe hemophili A ptient with high titer of fctor VIII inhiitor: cse report nd review of the literture. Int J Hemtol. 2006;84:166---9. 7. Blthzr EJ, Hulnick D, Megiow AJ, Opulenci JF. Computed tomogrphy of intrmurl intestinl hemorrhge nd owel ischemi. J Comput Assist Tomogr. 1987;11:67---72. 8. Rios R, Grulet P, Rodriguez M, León C, Limones M. Spontneous intrmurl hemtom of the smll intestine. Cir Esp. 2005;78:275. Documento descrgdo de http://www.elsevier.es el 14/03/2016. Copi pr uso personl, se prohíe l trnsmisión de este documento por culquier medio o formto. 44 F. Mirs Azcón et l 9. Reyes Gry H, Tgle Arrospide M. Spontneous intrmurl hemtom of the smll owel due to use of orl nticogulnts: cse report nd review of the literture. Rev Gstroenterol Peru. 2010;30:158---62. 10. Chen YW, Chen MH, Wng LM, Chen YC. Spontneous intrmurl intestinl hemtom. Intern Med J. 2007;37:661---2. 11. Hhn PF, Strk DD, Vici LG, Ferrucci JT Jr. Duodenl hemtom: the ring sign in MR imging. Rdiology. 1986;159:379---82. 12. Hughes CE 3rd, Conn J Jr, Shermn JO. Intrmurl hemtom of the gstrointestinl trct. Am J Surg. 1977;133:276---9. 13. Liu Y, Yng S, Tong Q. Spontneous intrmurl hemtom of colon. Clin Gstroenterol Heptol. 2012;10:e38. 43