Meyer s dysplasia : delayed ossification of the femoral head as a differential diagnosis in Perthes disease

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1 At Orthop. Belg., 2010, 76, ORIGINAL STUDY Meyer s ysplsi : elye ossifition of the femorl he s ifferentil ignosis in Perthes isese Nsir MuzAffAR, He-Ryong SONg, Kmlesh DEvMuRARI, Hitesh MODI From the Kore University Meil Center, Seoul, South Kore Meyer s ysplsi strongly mimis Legg-Clve- Perthes isese espite iffering mrkely in eventul outome. This stuy presents the linioriologil fetures whih ifferentite it from Perthes isese in group of 178 hilren with preliminry ignosis of Perthes isese, of whom nine were susequently ignose with Meyer s ysplsi. All h ner norml evelopment of the femorl he ; mil flttening ws seen in two ses. Clinil initil presenttion often resemles Perthes isese. However, the presene of grnulr spet of the femorl he, often ilterl, in mle hil with preliminry ignosis of Perthes isese shoul lert the liniin to the possiility of Meyer s ysplsi. Restitution of norml pitl rhiteture is usul ; there my however e mil resiul hypoplsi. the sixth yer (4). Previous stuies inite tht oys re ffete five times more thn girls (3). Ptients show ontinuous improvement, with stey unifition n growth of the epiphysis, usully over three-yer perio (4). Riogrphi fetures inlue smller ossifition nulei, rks in the ossifition enter, yst formtion n rioluent efets in the femorl he (6). Bone sns re norml in ptients with Meyer s ysplsi (2). Epiphysel ysplsi n hypothyroiism shoul e inlue in the ifferentil ignosis whenever ilterl hnges re present (7). Meyer s ysplsi my e esily onfuse with more serious onitions suh s Perthes isese. Keywors : Meyer s ysplsi ; elye ossifition ; Perthes isese ; ilterl ; grnulr pttern. INTRODUCTION Meyer s ysplsi, lso lle ysplsi epiphyselis pitis femoris (DECF), is rre onition tht ffets the pitl femorl epiphysis in young hilren. This evelopmentl isorer of the hip is hrterize y elye, irregulr ossifition of the femorl epiphysel nuleus (5). Onset usully ours in the seon yer of life, is mostly ilterl n the isorer usully isppers y the en of Nsir Muzffr, MS, Orthopei surgeon Deprtment of Peitri Orthopeis, Bone n Joint Surgery Hospitl, Brzll, Sringr, J&K, Ini. He-Ryong Song. MD, PhD, Orthopei surgeon, Professor of Orthopei Surgery Kmlesh Devmurri, MS, Orthopei surgeon Hitesh Moi, MS, PhD, Orthopei surgeon Deprtment of Orthopei Surgery Kore University Meil Center, Guro Hospitl, Seoul, South Kore. Corresponene : Dr Nsir Muzffr, MS, Bone n Joint Surgery Hospitl, Brzll, Sringr, Kshmir, Ini E-mil : rnsirmuzffr@gmil.om 2010, At Orthopæi Belgi. No enefits or funs were reeive in support of this stuy

2 DElAyED OSSIfICAtION Of the femoral HEAD AS A DIffERENtIAl DIAgNOSIS IN PERtHES DISEASE 609 This stuy ws unertken to ssess n evlute the presene of Meyer s ysplsi in popultion of Legg-Clve-Perthes Disese (LCPD) ptients, etermine the iniene, sex n ge groups of prevlene n ifferentil ignosis. The linioriologil fetures, when ouple with equte knowlege of this onition, n prevent unneessry hospitliztion n tretment. We lso present rief stuy of the relevnt literture n its orreltion with our finings. MATERIALS AND METHODS In the peitri orthopei outptient eprtment, etween 2003 n 2008, 9 hilren with elye ossifition of the he of the femur were ignose with DECF, out of totl of 178 hilren with preliminry ignosis of LCPD. The verge presenting ge ws 28.7 months (rnge months). The verge follow-up ws 3 yers 5 months. The ignosis of DECF ws me on the sis of the riologil pttern n evolution of the pitl femorl epiphysis over perio of time, whih iffere from the rest of the hilren in the group of LCPD ptients. This ws generlly in the form of elye, smller n irregulr ossifition enters of the pitl femorl epiphysel nuleus t out 2 yers of ge tht grully olese n normlize within 2-4 yers. No signs of epiphysel ysplsi were oserve in ny other re in ll ses. MRI ws one in 4 ses. No one sns were one. The hemtologil tests i not revel ny normlity. Bone ge estimte y the Greulih-Pyle metho ws less thn the hronologil ge in ll ptients. Sine the preliminry ignosis ws LCPD, ution ring ws given in 6 hilren presenting limp n trtion in one ptient who h hip pin. These ptients were symptomti within 2-4 weeks. There ws no fmily history of LCPD in ny se. RESULTS Out of 178 ptients with LCPD, 9 ptients or 5% were ignose with DECF. Four hilren h ilterl (44%) n five unilterl (56%) involvement. Eight of the hilren were mle (88.9%). Symptomtilly, six hilren presente with limp, one h mil hip pin, one h n norml stning position n one ws isovere inientlly while eing followe up for n mnioti n synrome. Three ses h mil limite ution n two h limite rottion of the hip. These symptoms resolve in ll ses within 2-4 weeks. Plin riologil exmintion t presenttion showe elye n irregulr ossifition enters in ll pitl femorl epiphyses. Metphysel ysts were seen in two ses (figs 1, 2). Centrl olumn riolueny ws oserve in two ses n slerosis of the pitl femorl epiphysis ws seen in one se (fig 2). No lim length isrepny ws seen t eventul resolution (fig 3). Two ses h mil flttening of the femorl he t follow-up (fig 4). No hip showe ny suluxtion, suhonrl frtures or ny femorl nek eformity. Fusion of the ossifition enters ws seen t n verge ge of 5 yers 10 months (rnge : yers). MRI showe metphysel ysts in one ptient (fig 1), multiple ossifition enter involvement in nother, frgmenttion of the pitl nuleus in the thir n in the fourth se whih ws ilterl, low signl intensity hypoplsi with flttening on the left sie ws oserve (fig 4). No nomly ws oserve in the mrrow signl in ny se. There ws no effusion or synovil thikening on MRI in ny of the ffete hips. No meition ws given. There ws no onversion to LCPD in ny se on follow-up. DISCUSSION Meyer s ysplsi or ysplsi epiphyselis pitis femoris (DECF) is rre isorer often onfuse with Legg-Clve-Perthes isese (LCPD). However, it iffers from LCPD in oth tretment n prognosis. The ossifition nuleus is not seen until 18 to 36 months of ge n osionlly multiple nulei of ossifition my e present, so tht the epiphysis hs grnulte spet ; fusion of the vrious ossifition enters ours t roun 5 yers of ge (3). The epiphysel ensity n struture is mintine n it oes not ollpse. Differentil ignosis shoul minly e me with LCPD (2-5) ut multiple epiphysel ysplsi, hypothyroiism (7), yshonroplsi, rthritis n

610 N. MuzAffAR, H. R. SONg, K. DEvMuRARI, H. MODI e f Fig.

rhiteture (,e). The MRI one initilly is showing metphysel yst (f). infetion must e rule out in outful ses.

stuy in 1960, wherein he esrie 42 ptients in group of 672 ses (6.

Meyer (4), in 1964, reporte his finings of 30 ses in group of 300, lele s LCPD showing elye evelopment of the epiphysel

These 30 ses (10%) showe ossifition only fter 2 yers of ge n the nuleus ppere s iffuse grnulr struture with inonspiuous

one tissue in the epiphysis whih is otherwise norml in shpe n size.

3 610 N. MuzAffAR, H. R. SONg, K. DEvMuRARI, H. MODI e f Fig. 1 Seril riogrphs of hil with DECF of the right hip showing n initil grnulr he pttern (,,) n restortion to norml rhiteture (,e). The MRI one initilly is showing metphysel yst (f). infetion must e rule out in outful ses. The notion tht ysplsi epiphyselis pitis femoris is seprte entity istint from LCPD ws first put forwr y Peersen (5) in stuy in 1960, wherein he esrie 42 ptients in group of 672 ses (6.2%) lele s LCPD, hving typil pttern of riogrphs showing no ollpse or gross frgmenttion of the pitl femorl epiphysis. Meyer (4), in 1964, reporte his finings of 30 ses in group of 300, lele s LCPD showing elye evelopment of the epiphysel nuleus. These 30 ses (10%) showe ossifition only fter 2 yers of ge n the nuleus ppere s iffuse grnulr struture with inonspiuous onenstion, unlike in LCPD where, oring to Meyer, the first riologil sign of the isese is mssive uniform onenstion of the one tissue in the epiphysis whih is otherwise norml in shpe n size. The femorl he n nek struture eventully normlize in out 3 yers. Meyer lso reporte ilterl iniene of 42% in his series. Six ses shifte from DECF to LCPD over perio of time. Khermosh n Wientrou (3) reporte 18 ses of DECF with 50% ilterl involvement n oys ffete five times more often thn girls. They esrie n essentilly similr linioriologil piture ut with slight flttening of the femorl epiphysis, whih they ttriute to fol hypoplsi. Our

DElAyED OSSIfICAtION Of the femoral HEAD AS A DIffERENtIAl DIAgNOSIS IN

2 Seril riogrphs of hil with DECF of the right hip showing metphysel yst

seen in 44% of ses n 89% were mle. The verge ge t presenttion ws 2.4 yers.

The hief presenting omplint ws limp in 6 ses (67%) whih is why they were

Meyer (4) reporte his ptients of DCEF s hving essentilly similr linil

4 DElAyED OSSIfICAtION Of the femoral HEAD AS A DIffERENtIAl DIAgNOSIS IN PERtHES DISEASE 611 Fig. 2 Seril riogrphs of hil with DECF of the right hip showing metphysel yst formtion with slerosis of the pitl femorl epiphysis (,) n mil flttening of the restore femorl epiphysis (,). Fig. 3 Seril riogrphs of hil with DECF of the left hip with full remoelling in 1.5 yers. oservtions showe n iniene of 5% in group of LCPD ptients, ilterl iniene ws seen in 44% of ses n 89% were mle. The verge ge t presenttion ws 2.4 yers. Similr figures were reporte y Rowe et l (6) ; they reporte men ge t presenttion of 2.5 yers, n iniene of 4.4% mong group of LCPD ptients n ilterl involvement in 59%. The hief presenting omplint ws limp in 6 ses (67%) whih is why they were initilly onsiere s LCPD ptients. Meyer (4) reporte his ptients of DCEF s hving essentilly similr linil presenttion s LCPD n Hrel et l (2) lso inite the symptoms of limp n restrite rottion s the presenting fetures. Our stuy tens to onfirm these finings. Hip pin ws seen in one se whih hs lso een reporte erlier (2). This le us to speulte tht DECF might e miler form or preursor of LCPD, tht in fvorle environment oes not eteriorte ut shows resolution over time. The

612 N. MuzAffAR, H. R. SONg, K. DEvMuRARI, H. MODI Fig. 4 Riogrph n MRI of hil with ilterl DECF. There is mil flttening of the left pitl femorl epiphysis with riolueny on the riogrph.

etiology of DECF hs puzzle mny reserhers over the yers n though there re proponents of the ishemi theory propose y Meyer (4) n of the ongenitl vsulr theory propose y Btory (1), onlusive eviene for

5 612 N. MuzAffAR, H. R. SONg, K. DEvMuRARI, H. MODI Fig. 4 Riogrph n MRI of hil with ilterl DECF. There is mil flttening of the left pitl femorl epiphysis with riolueny on the riogrph. The MRI shows low signl intensity hypoplsi with flttening of the left femorl he. etiology of DECF hs puzzle mny reserhers over the yers n though there re proponents of the ishemi theory propose y Meyer (4) n of the ongenitl vsulr theory propose y Btory (1), onlusive eviene for oth is lking. Our stuy inlue four MRI stuies showing in one metphysel yst n in nother low signl intensity hypoplsi in the femorl he, whih eventully hele normlly with mil flttening on followup. The presene of metphysel ysts is one of the he t risk signs in LCPD ; the omplete resolution of the isese proess in these ses points to the ft tht DECF hs fetures whih re oth onform n ontrry to LCPD. We elieve tht further investigtions re neessry, more speifilly into possile hormonl use in view of the preponerne of mle ses. This ws out of the sope of our stuy t the time n my e tken up s prospetive investigtion in the future. The si riologil fetures in ll our ptients showe similr pttern. The ossifi nuleus ppere nerly t 2 yers of ge n usully onsiste of single grnulr struture or multiple smll ossifition enters. With the pssge of time, these enters inrese in size n olese. The en result ws norml ppering pitl femorl epiphysis with mil flttening seen in two ses. Riogrphi stuies were quite emonstrtive, showing elye ossifition giving the impression tht there re rioluent lyti res prouing grnulr pttern of the femorl he, resemling lssi golf ll or s Meyer put it, lkerry. With the urrent knowlege of Meyer s ysplsi, the term ysplsi seems misnomer s the unerlying pthology is lk in evelopment of ossifition of the femorl he. The strit mening of ysplsi is efetive evelopment of the hip joint resulting in eformtion of the shpe or orgniztion of the hip joint. In Meyer s ysplsi, elye ossifition of the he of the femur uring growth of the hil eventully les to seonry ossifie nuleus whih is essentilly norml without ny signifint sequele or eformities. However, our results hve shown tht there my e flttening of the femorl he in DECF n though this my not e the se in every ptient, regulr n prolonge follow-up is neee to ssess the long term results on the he-e - tulr reltionship. REFERENCES 1. Btory I. [Dysplsi epiphyselis pitis femoris of primry hypoplsti vessel-evelopment of the proximl epiphysis of femur] (in Germn). Z Orthop Ihre Grenzge 1982 ; 120 : Hrel L, Kornreih L, Ashkenzi S et l. Meyer ys - plsi in the ifferentil ignosis of hip isese in young hilren. Arh Peitr Aoles Me ; 153 : Khermosh O,Wientrou S. Dysplsi epiphyselis pitis femoris. J Bone Joint Surg 1991 ; 73-B : Meyer J. Dysplsi epiphyselis pitis femoris. A linil -riologil synrome n its reltionship to Legg-Clve -Perthes isese. At Orthop Sn 1964 ; 34 : Peersen EK. Dysplsi epiphyselis pitis femoris. J Bone Joint Surg 1960 ; 42-B : Rowe, SM, Chung, JY, Moon ES et l. Dysplsi Epiphyselis Cpitis Femoris. Meyer Dysplsi. J Peitr Orthop 2005 ; 25 : Sprnger J. The epiphysel ysplsis. Clin Orthop Relte Res 1976 ; 114 :

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