3D Imaging in Unilateral Primary Pulmonary Hypoplasia in an Adult Case report Aristida Georgescu, Crinu Nuta, Simona Bondari

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1 3D Imging in Unilterl Primry Pulmonry Hypoplsi in n Adult Cse report Aristid Georgescu, Crinu Nut, Simon Bondri Aristid Georgescu, MD, PhD Emergency Clinicl County Hospitl of Criov Prim Medicl SRL Criov No. 1, Tci Street, Criov, Romni RO ristid_georgescu@yhoo.com Crinu Nut, MD Clinicl Infectious Diseses nd Pneumo-ftysiology Hospitl V. Bes Criov No. 126, Cle Bucuresti Street, Criov, Romni RO nutcrinu2001@yhoo.com Simon Bondri, MD, PhD Emergency Clinicl County Hospitl of Criov Prim Medicl SRL Criov No. 1, Tci Street, Criov, Romni RO simonondri@yhoo.com 1

2 ABSTRACT Unilterl primry pulmonry hypoplsi is rre in dulthood (UPHA); it is chrcterized y decresed numer of ronchil segmenttion nd decresed/sent lveolr ir spce. Clssicl chest X-Ry my e confusing, nd the iologicl tests re unspecific. We present cse of UPHA in 60-yer-old femle, smoker, with 3 term norml deliveries, who presented with lte recurrent pneumonis nd ronchiectsis-type symptomthology, rteril hypertension nd oesity. Chest X-rys reveled opcity in the left lower pulmonry zone, n pprent hypoerted upper left loe nd left devition of the medistinum. Preopertory multidetector computer tomogrphy (MDCT) presented smll retro crdic left lung with 5-6 ronchil segmenttion rnge nd cystic ppernce. After pneumonectomy the gross specimen showed smll lung with multiple ronchiectsis nd smll cysts, lined y hyperplsic epithelium, surrounded y stroml firo-sclerosis. We concluded this UPHA occurred in the 4-7 emryonic weeks, nd the 3D MDCT reconstructions offered the est noninvsive dignostic. INTRODUCTION The development nomlies of the lung etween the 4th nd 24th gesttionl weeks my cuse functionl dmge usully discovered in neworns nd infnts nd it cn e rrely present in dulthood [1]. Prcticlly, the erlier the nomly is present, the rnching of the trchel- ronchil tree is reduced (Tle 1). EMBRYONIC STAGE: 4-7 WEEKS PSEUDOGLANDULAR: 5-17 WEEKS CANALICULAR: WEEKS MATURATION STAGE Bronchil segmenttion: up to 10 genertions Bronchi, ronchioli, respirtory ronchioli: up to 18 genertions Respirtory ronchioli, lveolr ducts: up to 22 genertions Scullus: up to 23 genertions; lveoli Extrpulmonr rtery: 34 dys Loulr rteries: 44 dys Precinr rteries: 5-17 weeks Intr-cinr rteries: weeks, lveolr duct rteries Alveolr duct rteries: 25 weeks - 18 months postntl Alveolr cpillries: 36 weeks 18 yers Tle 1: Development of the lung (Bsed on Hislop A: Developmentl iology of the pulmonry circultion. Peditr Respir Rev 6:35-43, 2005). The degree of lung involvement ws clssified into three groups y Boyden [3]: - Pulmonry genesis: crin, min ronchi, lung tissue, nd vsculr structures re sent; - Pulmonry plsi: pouch-like, lind-ending min ronchus nd crin re present; - Pulmonry hypoplsi: the destroyed ronchil structures cuse mldevelopment in lveolr tissue, lung tissue is seen s medistinl structure. Pthologiclly, the hypoplstic lung hs reduced lung weight, fewer genertions of irwys, nd hypoplsi of the corresponding pulmonry rteries. Epithelil differentition is delyed, nd surfctnt deficiency is ssocited. In pulmonry hypoplsi, medistinl shift to the side of homogenous density my e depicted, with compenstory hernition of the uninvolved lung. Etiologiclly, the pulmonry hypoplsi cn e clssified s primry, if there is not ny ovious cuse of hypoplsi; respirtory distress cn e seen immeditely fter irth, nd the higher mortlity rte is generlly due to severe pulmonry circultory normlities. If there re other fetl nd mternl nomlies, the pulmonry hypoplsi is clssified s secondry; 2

3 spce-occupying lesions in the chest (including diphrgmtic herni), developmentl nomlies of the chest wll, nd urogenitl nd neuromusculr diseses cn e ssocited nd they represent supplementry risk fctors for erly complictions nd deth. Clinicl presenttion in dult is highly vrile, depending in lrge mesure on history of smoking nd repeted respirtory infections. The lmost symptomtic cses with long survivl in dulthood re explined y compenstory hypertrophy of the contr lterl lung filling of the ipsilterl hemithorx, s in pneumonectomy; the est survivl is for the left lung hypoplsi, ecuse of the good compenstory hypertrophy of the lrger right lung. Unilterl pulmonry hypoplsi in dult (UPHA) cn e found incidentlly in routine exmintions for other resons, ut rther this pthology is long time misdignosed possily ecuse the symptoms nd the clssicl rdiologicl slient nomlies re ttriuted to some old infections, such s were considered in the cse presented in the following. Chest MDCT is t present the dignostic tool of choice [4], ecuse it llows multiplnr nd 3D reconstructions of the lung, including oth the ronchil nd the vsculr tree. CASE PRESENTATION This pper presents cse of UPHA in 60-yer-old femle, whose dignosis ws misinterpreted up to 58-yer-old. History: The ptient ws symptomtic t irth nd in the childhood; she presented pneumopthy t 19-yer-old, nd the chest X-rys reveled dense opcity in the left lower pulmonry zone extended to the pleurl sl sinuses, nd 3 months tuerculosttic preventive therpy ws pplied. The ptient hd good condition during the most prt of life, with 3 term pregnncies finished with norml deliveries. She supported lso some surgicl tretments without complictions: ovrin cystectomy t 30-yer-old nd lproscopic cholecistectomy t 54-yer-old. The occsionlly chest X-Rys were interpreted s left sl pleurl thickening nd chronic pneumopthy, sechel of the previous infections. Clinicl presenttion: The ptient of 58-yer-old, smoker for 20 yers, presented oesity nd rteril hypertension with the men lood pressure 180/100 mm Hg. During the lst yers she presented recurrent cteril roncho-pneumonis with high fever, chills, cough with mucous-purulent expectortion, left chest pin, rethlessness one exertion nd wheezing. Investigtions: The white cells lood count presented infectious formul nd the erythrocyte sedimenttion rte (ESR) ws rised up to 46mm/hr nd 68mm/2hr. The electrocrdiogrm presented left crdic xis devition with QRS +50, micro voltge of the QRS complexes in the stndrd devitions nd negtive T wves in V3-V6. The dominl nd pelvic Ultrsonogrphy did not revel ny congenitl normlity nd confirmed the stts post cholecistectomy. The posterior-nterior chest roentgenogrm offered n erroneous dignosis, presenting n pprently opcity in the left lower pulmonry zone, hypoerted upper left loe nd left devition of the medistinum, s we present in this CT scnogrm (Fig. 1). We cn mention tht despite the pprent telectsis of the left lung, there is not significnt retrction of the left hemithorx, such s in the postntl cquired pthology (Fig. 1). The MDCT demonstrted smll left lung consisting of multiple cysts, hyperinfltion of the right lung with left side hernition mostly in the upper zone nd left medistinl devition. The xil scns in lung nd medistinl windows imges (Fig.2; 3) were considered essentil for the counting of the ronchil segmenttion rnge, ut not enough for the understnding of the ntomicl compenstory development of the right lung nd of the medistinl structures. 3

4 Fig. 1: The rdiologicl spect could suggest left sl pchypleuritis nd left lower loe telectsis, with ipsilterl medistinl devition nd right lung hernition (); 3D one reconstruction reveled symmetricl thorcic cge, discordnt with n cquired retrctile pleurl-pulmonry pthology (). c d Figure 2,, c, d: The xil scns in lung window imging reconstruction efore (,, c) nd fter (d) the left pneumonectomy. 4

5 c d Figure 3,, c, d: The xil scns in medistinl window imging reconstruction efore (,, c) nd fter (d) the left pneumonectomy. Indeed, there ws not only n ipsilterl medistinl devition, ut we dmit specil dpted development of the functionl thorcic orgns nd tissues; the multiplnr reconstructions demonstrted the depression of the right hemidiphrgm tht vs lnced to llow the right lung hypertrophy (not only hyperinfltion) with norml position of the left hemidiphrgm (Fig. 4, ). The est understnding of the ntomicl reltions ws offered y the volumic reconstructions of the MDCT cquisitions, using dedicted medicl imging softwre tht offered superior computer ssisted dignostics (CAD) pltform [5]; indeed, 3D views cn revel normlities tht my otherwise e overlooked y rdiologists nd other medicl professionls. In this cse, the right lung development ws dimorphic, the loes were oriented in different directions to empty the thorcic volume, the pleurl fissures nd the right ronchil tree re not proportionl, while the left lung ppers s cluster of smll cysts connected to the lor ronchi, without ny lveolr structure. 5

6 Figure 4: The coronl () nd sgittl () reconstructions in medistinl window imging llow etter understnding of the ntomicl position of the supr nd underdiphrgmtic orgns. For the reconstructions we mintined conventionlly the left loe on the left-side of the screen in the posterior-nterior view, for etter recognition when compring with the xil nd the coronl scns (Fig. 5). Figure 5, : 3D Reconstruction in the surfce mode, visulize oth the hypoplsic dysplstic left lung, with multicystic rchitecture of smll rnge segmenttion (), nd the right hypertrophic lung with the pleurl fissures nd the norml lor orienttion (): the upper loe is in the left-posterior loction, the middle loe is in the left-nterior position nd the lower loe is emptying the retrosternl spce nd the right hemithorx. The medistinl spce is reduced nd posterior locted, surrounded y the horseshoe right lung. 6

7 Moreover, in the trnsprency reconstruction mode, we cn see the lrge development nd the specil orienttion of the right pulmonry vessels, while the hypoplsic lung hs not slient vsculture (Fig. 6). c Figure 6: The pprent lind-ended hypoplsic left pulmonry rtery (lck rrow) nd the dilted right pulmonry rtery re ssocited to the ronchil pthology in UPHA (); coronl reconstruction visulizes the hypoplsic left pulmonry rtery () nd 3D reconstruction in the trnsprency mode (c) demonstrtes the min ronchil orienttion, the cystic structure of the left lung nd the presence of the functionl vsculr rchitecture only in the developed lung (white rrow). Tretment: A left pneumonectomy ws performed without compliction (Fig. 7) nd the gross specimen exmintion reveled smll, pprently retrcted densified lung, with low elsticity nd olished crepitus; on section mny ronchiectsis mucus-filled, some with cystic ppernce. The microscopic exmintion of the surgicl specimen showed pulmonry structure chrcterized y reshuffle res with dilted ronchi with thick wlls lined y hyperplsic epithelium, surrounded y stroml firo-sclerosis nd vsculr stsis. There were not ny lveoli nd ronchioli. 7

8 Figure 7: Post opertory MDCT with 3Dwiew reconstruction showing the lind-ended left min ronchi nd the compenstory hyperplsic right lung. The follow-up exms reveled fter 2 yers pulmonry function test with severe restriction ecuse of low FVC representing only 47% from the predicted vlue, nd FEV1 eqully decresed representing 44% of the predicted vlue (Fig. 8). In this intervl there were no more respirtory infectious signs. The white cells lood count presented norml vlue of /μl, ut the red cells lood count sketched compenstory chnges with n elevted VEM of 96.3fL (norml fL), Hemogloin of 15.5g/dl (norml 12-15g/dl), while the erythrocyte sedimenttion rte (ESR) decresed towrd 24mm/hr nd 42mm/2hr. Fig. 8: The Pulmonry Function Test (Spirometry) 8

9 DISCUSSION: Dignosis: From the clinicl point of view, most of the pulmonry hypoplsi cuse severe respirtory filure; in dult individuls the dignosis is difficult, since there re very few relevnt symptoms nd signs. Bsed on the ntomicl spects we concluded this UPHA occurred in the first 7-10 weeks of fetl development, llowed contr lterl pulmonry hypertrophy with good compenstion ut the dysplstic lung determined repeted ronchitis followed y unuseful drugs tretments due to the flse X-Ry dignostic. MDCT with the clssicl xil views nd especilly the multiplnr nd the 3D reconstructions llowed the more ccurte dignostic, prcticlly the ngiogrphy ecoming unnecessry. Indeed, in the left hypoplsic lung, there ws no more thn 5-6 rnge ronchil segmenttion. This erly mlformtion ws simultneous with the left pulmonry rtery extremely hypoplsi, concordnt with the oservtion of Kurkcuoglu et l [1] who presented left pulmonry hypoplsi nd showed y pulmonry rteriogrphy norml coursing of the right, dilted pulmonry rtery nd the sence of the ipsilterl (left) pulmonry rtery. This erly event explins the sence of the lveoli in the pthologic report nd lso the overextension nd the reorienttion in the whole thorcic remnnt spce of the right lung, so the symmetry of the thorcic cge ws preserved. The sence of the ssocited emryologicl pthology rgues the primry lung hypoplsi, with etter life prognostic thn the secondry pulmonry hypoplsi; the left-sided involvement llowed good survivl despite of the risk fctors (smoking, oesity, nd rteril hypertension), due to the lrger compenstory possiility of the right lung composed of 3 loes [6]. Differentil dignosis - The first differentil dignosis is secondry (cquired) pneumopthy with non-congenitl ronchiectsis, s ws considered the wrong initil dignosis sed on the lte history, the clinicl exm nd the chest roentgenogrms. The first rgue is the smll rnge of ronchil segmenttion in smll, hypoplsic lung, while norml neworn lung hs developed lveoli (Tle 1) nd they re lwys present with or without consolidtion in the secondry smll lung (pseudo-hypoplsi) (Fig. 9). Fig. 9: Smll left lung fter loectomy nd telectsis of the remnnt loe due to lveolr collpse y pleurl fluid collection nd lveolr consolidtion. There is not significnt medistinl shift towrd the involved side nd the rnches of the left pulmonry rtery re present. 9

10 - Cystic denomtoid mlformtion (CAM) C is developmentl hmrtomtous normlity of the lung, with denomtoid prolifertion of cysts resemling ronchioles. Cystic denomtoid mlformtion ccounts for 25% of ll congenitl lung mlformtions [6]. By contrrily to UPHA, the rdiogrphic pttern ppers s n expnsile soft-tissue mss contining multiple ir-filled cystic msses of vrying size nd shifting of the medistinum. The involved lung my pper honeycomed or spongy, ut occsionlly, 1 lrge cyst my overshdow the others. Respirtory distress occurs in the neontl period, when collterl pores of Kohn ventilte the lveolr tissue present. Ptients my hve medistinl shift nd pneumothorx. The ffected re is dull on percussion, nd ir entry is decresed. The rdiogrphic depiction of solid or cystic mss on one side of the thorx were considered utile for the dignosis, ut we consider MDCT or chest Mgnetic Resonnce Imging (MRI) with multiplnr nd 3D reconstructions using the performing soft progrms disposle nowdys re the methods of choice to chrcterize this normlities. Moreover, different types of cystic denomtoid mlformtion cn e differentited more ccurtely with CT thn with chest rdiogrphy; lesions tht my pper to hve resolved on rdiogrphy cn still e identified on the chest CT scn. CAM is rre in dulthood nd could e confused with UPHA or secondry ronchil pthology; Vicidomini et l [7] reported cse in 62-yer-old mle, who presented with recurrent cteril pneumonis nd rethlessness one exertion. Other reports descried the clinicl nd MDCT imge chrcteristics of CAM of the lung in dults [8]. The min elements of dignosis of CAM, lthough rre, would e recurrent productive cough in dult nd multiloculted cystic mss in one loe with norml vsculr imges in MDCT. - Pulmonry sequestrtion Pulmonry sequestrtion ccounts for 6% of ll congenitl lung mlformtions nd mostly occurs in the lower loes. A sequestrtion is ronchopulmonry mss without norml ronchil communiction nd with norml or nomlous vsculr supply. The involved lung segments cn e clssified on the sis of their pleurl coverge into intrpulmonry or extr pulmonry types. The similrities with pulmonry hypoplsi re: recurrent respirtory prolems in the sme ntomic loction, ssocited nomlies including diphrgmtic herni nd eventrtion. The dignostic is sed on MDCT, which llows the chrcteriztion of the unusul solid ttenution without ronchil connection nd the nomlous vsculr supply cn e visulized with vsculr contrst enhncement. - Congenitl lor emphysem Mssive overinfltion of one or more lung loes occurs postntl in congenitl lor emphysem (CLE) nd it could mimic the compenstory overinfltion of the contrlterl lung in UPHA. Cuses of the CLE include intrinsic sence or normlity (ronchomlci) of crtilginous rings or externl compression y lrge pulmonry rtery, which is eqully present in UPHA. Hyperexpnsion of pulmonry loe is present fter irth when, with negtive inspirtory pressure, ir cn enter the lung. However, the ir cnnot exit esily ecuse positive pressure cuses the softened irwy to collpse. The remining norml lung is then compressed. Tht results on the Spirometry pulmonry function with severe ostruction. Moreover, the CLE primrily involves the upper loes nd most ptients with congenitl lor emphysem present efore 6 months of life. On MDCT the involved loe nd its vsculrity cn e esily outlined s compred to norml lung prenchym, nd the contrlterl loe presents norml rchitecture of the ronchil nd of the vsculr tree. Conclusion Although congenitl lung mlformtions re rre, they re importnt disorders ecuse they my led to considerle moridity nd mortlity (infection, hemorrhge, respirtory filure). Prognosis depends on the size of the lesion, the degree of functionl impirment nd the ssocite congenitl mlformtions. 10

11 Helthy lung is composed of n orderly system of tues (irwys) nd scs (irspces or lveoli) in strict reltionship to pulmonry lood vessels (rteril from the right ventricle nd venous return to the left trium). In UPHA, oth the ronchil tree nd the pulmonry vsculrity re simultneously ffected nd the rnge of the ronchil tree could determine the gesttionl ge of the mlformtion occurrence. UPHA my remin symptomtic or misinterpreted for mny yers; the unspecific clinicl signs nd the clssicl chest X-Rys my led to lte dignosis of the pulmonry hypoplsi, primry or secondry. Filure to recognize mlformtion my led to inpproprite tretment nd delyed surgicl intervention such s in this cse. In some cses, the dignosis even fter Computed Tomogrphy with the usul protocol ws précised post-opertory: Aydi-Kddour et l [9] reported 2 from 3 cses nd Oh et l mentioned 1 from 7 cses with lte confirmtion [8]. Some uthors consider the lte dignosis of the UPHA [3], possily ecuse the nomlies oserved re ttriuted to old infections nd the clinicl presenttion is highly vrile, depending in lrge mesure on history of smoking nd repeted respirtory infections. Chest MDCT is t present the dignostic tool of choice, llowing the Computed Aided Dignosis techniques, with the est noninvsive chrcteriztion of the ntomy. REFERENCES 1. Kurkcuoglu IC, Eroglu A, Kroglnoglu N, Polt P. Pulmonry Hypoplsi in 52- Yer-Old Womn. Ann Thorc Surg 2005;79: Hislop A. Developmentl iology of the pulmonry circultion. Peditr Respir Rev 6:35-43, Boyden EA. Developmentl nomlies of the lungs. Am J Surg. 1955;89(1): Comet R, Mirpeix RM, Mrín A, Cstñer E, Sns J, Domingo C. Pulmonry hypoplsi in dults: emryology, clinicl presenttion nd dignostic methods. Our experience nd review of the literture. Arch Bronconeumol 1998 Jn;34(1): *** Unic3DView, 6. Schittny CJ, Burri HP. Development nd Growth of the Lung in Fishmn s Pulmonry Diseses nd Disorders, Fourth Ed, Copyright C 2008, 1998, 1988, 1980 y The McGrw-Hill Compnies, Inc; p Vicidomini G, Sntini M, Bldi A, Cesrno T, Di Mrino MP, Bldi F. Cystic denomtoid mlformtion of the lung in n dult. Minerv Chir Apr; 52(4): Oh BJ, Lee JS, Kim JS, Lim CM, Koh Y. Congenitl cystic denomtoid mlformtion of the lung in dults: clinicl nd CT evlution of seven ptients. Respirology Jul; 11(4): Aydi-Kddour A, Chouni S, Merï S, Ben Mrd S, Djilni H, Tritr F, El Mezni F. Congenitl cystic denomtoid mlformtion of the lung. Report of 3 cses with lte presenttion. Rev Ml Respir Mr; 25(3):