The Pediatric Gastrointestinal Tract: What Every Radiologist Needs to Know

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1 The Peditric Gstrointestinl Trct: Wht Every Rdiologist Needs to Know 15 Emily A. Dunn, Øystein E. Olsen, nd Thierry A. G. M. Huismn Lerning Ojectives To compre imging modlities commonly used in the ssessment of peditric gstrointestinl disese. To understnd gstrointestinl pthology ffecting children of different ge groups. To recognize clssic imging fetures of gstrointestinl diseses commonly encountered in infnts nd children Ultrsound Ultrsound (US) is one of the most vlule imging tools for the initil evlution of gstrointestinl disese, due to its high-resolution, rel-time cpility, wide vilility nd cceptnce, nd lck of ionizing rdition nd ecuse it rrely requires sedtion. There re two key fctors for success: (1) knowing how to del with children of ll ges nd (2) using high-end US equipment/high-frequency proes Imging Techniques Plin Film Rdiogrphy nd Fluoroscopy Conventionl rdiogrphy remins the first-line imging in most hospitls for infnts nd children presenting with suspected gstrointestinl pthology. Fluoroscopy is prticulrly useful when evluting dynmic processes such s gstrointestinl motility. Fluoroscopy cn lso e used for guiding procedures/interventions such s foreign ody removl nd intussusception reduction. E. A. Dunn (*) Thierry A. G. M. Huismn Division of Peditric Rdiology, Russell H. Morgn Deprtment of Rdiology nd Rdiologicl Science, Johns Hopkins University School of Medicine, Chrlotte R. Bloomerg Children s Center, Bltimore, MD, USA e-mil: Edunn18@jhmi.edu; Thuism1@jhmi.edu Ø. E. Olsen Rdiology Deprtment, Gret Ormond Street Hospitl for Children NHS Foundtion Trust, London, UK e-mil: oystein.olsen@gosh.nhs.uk Computed Tomogrphy In children, CT should e used with cution nd is usully reserved for trouleshooting equivocl findings mde in the initil dignostic workup of cutely nd criticlly sick children. CT is consequently often performed s first-line imging for emergent indictions, such s lunt nd penetrting trum. As generl rule, only one cquisition is needed, nd multiphse contrst enhnced sequences should e voided Mgnetic Resonnce Imging MRI is rrely used in the first-line evlution of gstrointestinl pthology; however, it my serve s n djunct in chllenging/unequivocl cses. In children, MRI is typiclly performed in the ssessment of intestinl pthology such s inflmmtory owel disese nd ppendicitis, focl nd diffuse disese of the dominl viscer, nd diseses of the iliry tree Intestinl Ostruction In neontes nd young children, there is no rdiogrphic signture for the lrge nd smll owel. Plin film rdiogrphs of the norml neworn demonstrte swllowed ir The Author(s) 2018 J. Hodler et l. (eds.), Diseses of the Adomen nd Pelvis , IDKD Springer Series, 157

2 158 reching the rectum y 24 h of life. In cliniclly ostructed neworn, the numer of dilted loops ( dilted mening roughly wider thn the verterl interpediculr spce) my e more helpful. Whether there is one gs ule (gstric outlet ostruction), doule ule (duodenl ostruction), 3 4 ules/loops (other high smll owel ostruction), or more (low ostruction), the owel gs pttern cn help decide which contrst study is pproprite. A owel gs pttern suspicious for high ostruction my e ssessed further with n upper GI contrst study. A owel gs pttern suggesting low ostruction should e followed y contrst enem Neontl Ostruction Neontl intestinl ostruction cn e clssified y loction, with high ostructions occurring through the level of the proximl jejunum nd low ostructions occurring fter the proximl jejunum. Etiologies of neontl intestinl ostruction re commonly congenitl. In most cses, fluoroscopic contrst study is required. E. A. Dunn et l. The differentil dignosis for high intestinl ostruction includes tresi of the esophgus, stomch, duodenum nd jejunum, duodenl stenosis from nnulr pncres, duodenl we, nd mlrottion possily complicted y midgut volvulus. The differentil dignoses for low intestinl ostruction include ilel nd colonic tresi, norectl mlformtion, meconium ileus, colonic dysmotility syndromes, nd Hirschsprung s disese. Congenitl dominopelvic cysts or msses cn cuse oth high nd low (usully prtil) ostruction. An understnding of clinicl presenttion s well s the expected postntl owel gs progression throughout the domen on rdiogrphs cn help to detect the level of ostruction [1]. Esophgel tresi (Fig. 15.1) is clinicl dignosis. If the dignosis is estlished, then ny gs in the gstrointestinl system distl to the esophgus on plin rdiogrphs mens there is lso trcheoesophgel fistul. Rdiology hs greter role in the follow-up of these children. Fistule my recur, nd the contrst esophgogrm is the imging test of choice for dignosis of recurrence; this is performed y trined opertors s there is considerle spirtion risk. c Fig Esophgel tresi nd trcheoesophgel fistul represent spectrum of nomlies elieved to result from norml formtion nd seprtion of the emryologicl foregut. These nomlies rnge from isolted EA to isolted TEF. Clssifiction of congenitl trcheoesophgel nomlies depends on the presence nd loction of the fistulous communiction etween oth structures. () Rdiogrph of neworn with isolted esophgel tresi (no fistul) shows no owel gs distlly. Those with isolted EA my hve polyhydrmnios in utero, nd t irth, there is filure of pssge of the orogstric tue eyond the level of the tresi. () Rdiogrph of neworn with esophgel tresi nd fistul etween the irwy nd the distl tretic segment demonstrtes gs in the gstrointestinl trct despite filure to pss feeding tue. (c) Fluoroscopic imge in n infnt with isolted trcheoesophgel fistul (rrow) following instilltion of wter-solule contrst medium in the esophgus

3 15 The Peditric Gstrointestinl Trct: Wht Every Rdiologist Needs to Know 159 Fig Duodenl ostruction (complete or prtil) is commonly cused y duodenl tresi, duodenl stenosis, duodenl we, nnulr pncres, nd midgut volvulus. () Rdiogrph of 12-hour-old neworn with prentlly dignosed duodenl tresi. Adominl rdiogrph shows the doule ule ppernce of the ir-distended stomch nd proximl duodenum nd no gs distlly. These findings suggest duodenl ostruction. It should e noted tht nnulr pncres nd midgut volvulus my produce similr rdiogrphic ppernce. () Neworn with vomiting. The first nd second portions of the duodenum re distended. A curviliner filling defect is oserved t the trnsition point (rrows), representing the windsock ppernce of the duodenl we For suspected nstomotic stricture, contrst swllow is performed. There is lmost lwys reltive nrrowing t the nstomosis, so it is functionl holdup which suggests potentil need for dilttion [2, 3]. Esophgel tresi my e oserved s prt of the VACTERL ssocition. Additionl nomlies should e ctively serched for. Duodenl ostruction (Fig. 15.2) my hve severl cuses. In duodenl tresi the clssic rdiogrphic findings re doule ule nd no distl intestinl gs. Duodenl tresi is thought to result from filure or incomplete recnliztion of the intestine in utero. Annulr pncres is cused y filed migrtion of the nlgen, nd it my e evident sonogrphiclly (or with CT/MRI). Duodenl stenosis nd duodenl we cuse prtil ostruction nd my e difficult to distinguish. The clssic sign of we on contrst study is the windsock sign [4, 5]. Mlrottion with midgut volvulus (Fig. 15.3) is surgicl emergency in the neonte s well s in infnts nd children. The filed intestinl rottion mnifests s right-sided/midline low duodenojejunl flexure nd high cecum. This is ssocited with short mesenteric root, which predisposes for volvulus. The norml position of the duodenojejunl flexure is t the height of the pylorus nd to the left of the midline, t lest s fr s the left verterl pedicles. Volvulus is suggested y ilious vomiting nd is verified y the finding of spirling outline of mlrotted duodenum/proximl jejunum on the contrst study [6, 7]. On US whirlpool of vessels is typiclly seen. Smll owel tresi nd stenosis (Fig. 15.4) re reltively common cuses of complete nd incomplete ostruction in the neonte. Multiple tresis my coexist; the etiology is thought to e in utero mesenteric vsculr ccidents. Accurte dignosis is criticl, since erly surgicl invention is usully performed. Bilious vomiting my e oserved in those with ostruction eyond the level of the pncretic mpull. The typicl findings of tresi on contrst enem re smll-clier (unused) colon nd distl smll owel nd the inility to reflux contrst into dilted smll owel loops (distinguishing it from meconium ileus; see elow) [4, 5]. Meconium ileus (Fig. 15.5) hs very high ssocition (>80%) with cystic firosis. The distl ileum is impcted with sticky meconium. The findings on contrst enem re similr to those in smll owel tresi except tht when contrst refluxes into the ileum, it outlines pellets of meconium, nd it is usully possile to eventully fill dilted smll owel loops. In uncomplicted cses, (repeted) contrst enems using wter solule contrst mteril re used therpeuticlly to soften nd wsh out meconium pellets. Both meconium ileus nd intestinl tresis my e complicted y in utero owel necrosis/perfortion nd meconium peritonitis [8, 9].

4 160 E. A. Dunn et l. Fig Intestinl mlrottion results from incomplete rottion nd fixtion of the owel in utero, with the duodenojejunl junction nd cecum lying closer. This shortening of the mesenteric pedicle predisposes to midgut volvulus, in which the normlly fixed owel loops twist out the xis of the superior mesenteric rtery nd my ostruct the duodenum. While mlrottion with midgut volvulus my occur t ny ge, the gret mjority present within their first month nd mny within their first week. Midgut volvulus cn led to intestinl ischemi necessitting owel resection. Emergent nd ccurte imging dignosis is therefore criticl. Bilious vomiting nd dominl distention in neworn infnt should rise the suspicion for n ostruction distl to the mpull of Vter, nd imging should e pursued. Rdiogrphs re norml in mny cses of mlrottion, nd fluoroscopic upper gstrointestinl series, which is considered the gold stndrd for dignosis, should e performed in highly suspect cses. () Mlrottion with midgut volvulus. In the cse of mlrottion with midgut volvulus, contrst mteril fils to pss eyond the ostructed third portion of the duodenum. () Mlrottion with midgut volvulus in the setting of n incomplete ostruction. There is corkscrew configurtion of contrst within the twisted midgut owel loops Fig Ilel tresi is thought to result from mesenteric vsculr events in utero. () Rdiogrph of neworn infnt with ilel tresi shows distention of multiple owel loops throughout the domen, suggesting low ostruction. () Fluoroscopic contrst enem in the sme child demonstrtes typicl microcolon (unused colon) nd retrogrde filling of non-dilted distl ileum to the level of the tresi (rrow). More proximl owel loops re distended nd ir filled

5 15 The Peditric Gstrointestinl Trct: Wht Every Rdiologist Needs to Know 161 Fig Meconium ileus represents ostruction of the distl ileum y viscous, impcted meconium pellets. There is high ssocition with cystic firosis (presenting symptom in 15% of neontes with CF). () Fluoroscopic contrst enem demonstrtes contrst opcifiction of microcolon (unused colon due to proximl ostruction). Contrst hs refluxed into the distl ileum (rrowheds) nd outlines numerous impcted meconium pellets. () Adominl rdiogrph demonstrtes punctte clcifictions scttered throughout the domen, suggestive of meconium peritonitis which cn occur with intestinl tresis s well s meconium ileus, nd results from perfortion of meconium-contining owel Colonic tresi is exceedingly rre, nd the dignosis is often mde cliniclly (vomiting nd filure to pss meconium) on plin rdiogrphs (no gs in the rectum). Contrst enem revels filed reflux of contrst proximl to the level of the colonic tresi [10]. In Hirschsprung s disese (Fig. 15.6), intermusculr nd sumucosl nerve plexuses re sent due to rrested migrtion of intestinl gnglion cells, producing functionl intestinl ostruction. Since this migrtion occurs from proximl to distl lrge owel, the gnglionic segment extends proximlly from the nus. The typicl clinicl presenttion is delyed pssge of meconium in the neonte eyond 24 h of ge. Most ffected ptients demonstrte short-segment involvement, in which the trnsition point etween norml nd gnglionic owel is locted in the rectosigmoid colon. Rrely, the entire colon my e involved. Meconium/fecl impction, zone of trnsition from dilted to non-dilted owel, nd norml peristlsis of the ffected colon segment with swtooth ppernce of the wll re clssicl findings t contrst enem. Very short-segment gnglionosis lcks cler trnsition zone nd is therefore difficult to dignose rdiologiclly. Hirschsprung s disese cn only e definitively dignosed y rectl (suction) iopsy [4, 11]. Functionl immturity of the colon, smll left colon, nd meconium plug syndrome (Fig. 15.7) re entities with considerle overlp. The risk is greter in ies of dietic mothers. Rdiologiclly there is reltively smller left colon nd proximl meconium impction. It is difficult to mke ctegoricl dignosis s similr findings my e seen in Hirschsprung s disese [12, 13]. Anorectl mlformtion is clinicl dignosis. These mlformtions re clssified s high or low depending on the loction of the distl-most owel segment reltive to the puorectlis sling, with high lesions terminting ove nd low lesions terminting elow this level. The role of rdiology is to ssess the length of the tretic norectl segment nd to mp the fistul(e) [14] Intestinl Ostruction in the Older Neonte nd Infnt In older infnts nd children, ostruction my e relted to other etiologies, such s dhesions, hypertrophic pyloric stenosis, intussusception, incrcerted intestinl herni, ppendicitis, sigmoid volvulus, nd Meckel s diverticulum. Plin film rdiogrphs cn occsionlly suggest the level of ostruction in older infnts nd children. In mny cses, however, ultrsound nd, occsionlly, CT or MRI my e necessry to rech the dignosis. Hypertrophic pyloric stenosis (Fig. 15.8) clssiclly presents with forceful projectile vomiting nd filure to gin weight in n infnt. The dignosis is mde sonogrphiclly

6 162 E. A. Dunn et l. Fig Functionl immturity of the colon, long with meconium plug nd smll left colon, represent syndromes of colonic dysmotility. Those ffected typiclly present with delyed pssge of meconium. There is reported incresed incidence in ies with mothers who re dietic or who hve received mgnesium sulfte during pregnncy for preeclmpsi. This imge from contrst enem revels smll clier of the descending nd sigmoid colon, which re filled with meconium, proximl dilttion, nd norml-clier rectum Differentil dignoses for dominl cystic lesions tht re rrely ostructive include choledochl, mesenteric, nd ovrin cysts, renl cystic dysplsi, nd hydroureter [16]. Fig A fluoroscopic contrst enem in 2-dy-old with iopsyproven Hirschsprung s disese shows smll-clier rectum with mild distention of more proximl lrge owel loops. Note the swtooth ppernce of the gnglionic segment (rrowheds), which reflects irregulr contrctions where the typicl findings re (1) thickened musculris (>4 mm); (2) elongted pylorus (>15 mm), with redundnt mucos occsionlly protruding into the ntrum of the stomch; (3) gstric hyperperistlsis; nd (4) persistent closed pyloric chnnel fter test feed. A contrst mel is rrely indicted except in few equivocl cses [15]. Incrcerted intestinl herni is surgicl emergency. The presenttion nd imging findings re similr to those in dults. Congenitl dominopelvic msses (Fig. 15.9) comprise spectrum of normlities where gstrointestinl ostruction is reltively unusul. Scrococcygel msses (e.g., germ cell tumors, such s tertoms, or nterior meningoceles) my cuse rectl ostruction. A cystic ostructive mss should rise suspicion for meconium pseudocyst or enteric dupliction cyst. Dupliction cysts my occur nywhere long the gstrointestinl trct nd my hve reltively thick lyered wll (kin to owel) nd mucus-fluid level Intestinl Ostruction in the Infnt nd Older Child Ileocolic intussusception (Fig ) is surgicl emergency, usully occurring in infnts nd toddlers with mximum incidence t just under 1 yer of ge. The typicl presenttion is with dominl pin, loody stools, nd plple mss. A cler cuse (i.e., led point, such s mesenteric lymph node, lymphoid hyperplsi, dupliction cyst, murl inflmmtion cused y Henoch-Schonlein purpur, or Meckel s diverticulum) my or my not e estlished. The sonogrphic dignosis is firly strightforwrd with the finding of concentric doughnut shpe in the trnsverse plne nd sndwiching of the involved owel segments (the pseudokidney sign) in the longitudinl plne [17]. Long-stnding intussusception, trpped fluid in the involved segments, nd trpped lymph nodes re thought to e ssocited with lower success rtes for reduction with pressurized gs or fluid. Colonic volvulus results from twisting of the colon round its mesenteric root nd is rre cuse of ostruction in children. In children, cecl volvulus is more common compred with volvuli occurring in the sigmoid nd trnsverse colon.

7 15 The Peditric Gstrointestinl Trct: Wht Every Rdiologist Needs to Know 163 Fig Hypertrophic pyloric stenosis is n cquired ostruction of the gstric outlet, chrcterized y elongtion of the ntropyloric chnnel nd thickening of the pyloric musculris with vrying degrees of mucosl hypertrophy. Infnts typiclly present with non-ilious, projectile vomiting nd occsionlly plple hypertrophied pyloric olive. Hypertrophic pyloric stenosis is usully not present t irth. Infnts usully present with symptoms t 2 12 weeks of ge. The dignosis is mde sonogrphiclly. () High-frequency ultrsound exmintion of the ntropyloric region shows thickening of the musculris lyer to more thn 3 mm (rrowheds) nd elongtion of the pyloric chnnel to more thn 15 mm in length (dshed line). Dynmic imging cn detect the presence or sence of trnsit through the pyloric chnnel nd my lso identify hyperperistltic gstric wves (sterisk) in the presence of gstric outlet ostruction. Redundnt pyloric mucos my protrude into the gstric lumen (rrow), producing the ntrl nipple sign. () High-frequency detiled ultrsound often demonstrtes strition within the thickened musculris (etween clipers) Fig Dupliction cysts represent closed congenitl duplicted segments of ny prt of the gstrointestinl trct nd my e dignosed incidentlly or s result of ostruction. () Coronl reformt of computed tomogrphy scn following orl nd intrvenous contrst medium dministrtion demonstrtes smll owel dupliction cyst (rrowhed). () Sgittl sonogrm shows cyst posterior to the urinry ldder (u). The dignosis of rectl dupliction cyst is redily mde y demonstrting owel wll ppernce of the cyst wll (rrowheds)

8 164 E. A. Dunn et l. Fig Intussusception, most commonly ileocolic, hs pek incidence in lte infncy nd presents with dominl distention nd loody stools. () Rdiogrphs my show, s in this exmple, well-defined opcity (rrows) representing the invginte. () Ultrsound is dignostic showing typicl doughnut configurtion (rrowheds) of the telescoping owel segments with trpped mesenteric ft (hyperechoic content) Clinicl presenttion includes dominl pin, vomiting, nd distention. Depending on the type of twist, plin films my demonstrte mrked distention of the colon nd/or ir-fluid levels resemling either ird s ek or coffee en. CT demonstrtes the swirling ppernce of the sigmoid mesentery nd its vsculture. While clinicl nd imging findings re suggestive, sigmoid volvulus remins n often missed dignosis which cn e complicted y owel ischemi, gngrene, perfortion, shock, nd even deth [18 20]. Henoch-Schonlein purpur is n cute smll-vessel vsculitis in young children typiclly involving the skin, kidneys, synovium, nd owel. Sonogrphiclly there is nonspecific murl inflmmtion [21] Infectious, Ischemic, nd Inflmmtory Intestinl Pthology Necrotizing enterocolitis (Fig ) is the most common gstrointestinl emergency in the neworn. The etiology of NEC is incompletely understood nd is thought to involve multiple fctors, including immturity of the lood-gut rrier, erly feeding, nd cteril coloniztion which ultimtely led to hemorrhgic nd ischemic necrosis of the intestines. NEC typiclly occurs in preterm neontes especilly with very low irthweight nd less commonly term or lte preterm neontes with congenitl crdic disese or Hirschsprung s disese. Although somewht insensitive, plin films my demonstrte intestinl pneumtosis, portl venous ir, nd pneumoperitoneum. Intestinl ultrsound hs proven useful in the dignosis of NEC nd my demonstrte the presence of free fluid nd norml owel peristlsis, incresed wll thickness, echogenicity, nd vsculrity nd my suggest impending complictions such s perfortion. Additionlly, US hs een shown to detect smll mounts of intrmurl nd portl venous ir ules not demonstrted on plin film [22, 23]. Acute ppendicitis is the most common surgicl indiction in children. Appendicitis results from ostruction of the ppendix which leds to distention, mucosl ischemi, cteril overgrowth, inflmmtion, nd potentilly wll compromise nd perfortion. Erly dignosis is therefore essentil in order to void such complictions. US is the initil test of choice due to its lck of ionizing rdition nd is optiml for imging children. With n experienced sonogrpher nd use of grded compression technique, US is highly sensitive nd specific for the dignosis of ppendicitis nd its complictions nd pproches the ccurcy of CT. Use of MRI in the dignosis of ppendicitis is incresing, prticulrly for children with equivocl ultrsound [24, 25].

9 15 The Peditric Gstrointestinl Trct: Wht Every Rdiologist Needs to Know 165 Fig Necrotizing enterocolitis is clinicl dignosis ut my nevertheless hve typicl rdiologicl fetures. () Adominl rdiogrph demonstrtes thick-wlled gs-distended owel loops with intrmurl gs seen s liner lucencies (rrowheds) nd portl venous gs (rrows). () Intrmurl gs demonstrted sonogrphiclly s string of hyperechoic perls (rrowheds) in the owel wll Inflmmtory owel disese my e seen t ny ge, ut erly-onset disese is often more complex with ssocited immunologicl disorders. In older children nd dolescents, the clinicl picture nd the imging chrcteristics re more similr to those in dults. Conventionl fluoroscopic contrst follow-through is rrely indicted. Both ultrsound nd MR enterogrphy re excellent for ssessing the mcroscopic disese: owel wll thickening, mesenteric inflmmtion, mesenteric lymphdenopthy, hyperemi, nd fistultion [26]. Meckel s diverticulitis is inflmmtion of Meckel s diverticulum, n omphlomesenteric remnnt contining gstric/pncretic mucos. Adominl pin nd melen re typicl symptoms. The dignosis is extremely difficult to mke with rdiogrphs nd cross-sectionl imging lone, which re often insensitive. 99mTc-pertechnette scintigrphy is typiclly dignostic [27]. imging of choice in stle children presenting with trum. US my e used in unstle ptients nd is especilly helpful in the detection of hemoperitoneum. The liver, spleen, nd kidneys re mong the most common solid orgn injuries oserved in ccidentl trum. In non-ccidentl trum, the liver nd pncres re the most commonly injured solid orgns. Intestinl injury is uncommon overll, yet occurs with more frequency in non-ccidentl versus ccidentl trum. Children with compensted shock following initil resuscittion efforts my demonstrte chrcteristic dominl CT findings known s the hypoperfusion complex, which includes collpse of the ort nd inferior ven cv nd hyperenhncement of the drenls, kidneys, mesentery, nd owel wlls. These findings signl impending hemodynmic collpse, nd their immedite recognition is criticl [28 30] Gstrointestinl Trum Accidentl nd non-ccidentl trums, prticulrly lunt trum, re common cuses of moridity nd mortlity in children. Clinicl nd lortory findings my suggest injury to the hollow nd solid dominl viscer, necessitting imging follow-up. CT performed with intrvenous contrst provides super detil of vsculr, solid orgn, intestinl, nd musculoskeletl ntomy, thus mking it the Tke-Home Messges The differentil dignosis for neontl high intestinl ostruction includes tresi of the esophgus, stomch, duodenum nd jejunum, duodenl stenosis from nnulr pncres, duodenl we, nd mlrottion with midgut volvulus.

10 166 The differentil dignoses for neontl low intestinl ostruction include ilel nd colonic tresi, norectl mlformtion, meconium ileus, colonic dysmotility syndromes, nd Hirschsprung s disese. Congenitl dominopelvic cysts my led to high or low intestinl ostruction. In older infnts nd children, intestinl ostruction my e relted to dhesions, pyloric stenosis, intussusception, incrcerted intestinl herni, ppendicitis, sigmoid volvulus, nd Meckel s diverticulum. Rdiogrphic findings of NEC including free, intrmurl, nd portl venous ir re clssic yet infrequently oserved. Ultrsound improves sensitivity. In the hnds of n experienced sonogrpher nd with use of grded compression technique, sensitivity nd specificity of intestinl ultrsound for cute ppendicitis pproches tht of CT. Contrst enhnced CT is the imging stndrd in the evlution of stle children with lunt or penetrting trum. References 1. Gro M. Intestinl ostruction in the neworn infnt. Arch Dis Child. 1960;35: Hrmon C, Corn A. 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